REVIEW ARTICLE


Approach to the History
C O N T I N UU M A UD I O
I NT E R V I E W A V AI L A B L E
ONLINE
and Evaluation of Vertigo
and Dizziness
Downloaded from http://journals.lww.com/continuum by wqLe/H3/oM2vx1Qs5A5qMemKKHLPqE4qkmGNpdheG3ikf1o85ttJSkFL2oTkgOkhMEmawHvsuTTE5xJG2ZjqccytIAhVn2qegy060KLcKJuPZsx5htaYiopRjp2zor2egUzWJAEWYNo65TEub0G9tZtPaMachiqn on 04/28/2022

By Terry D. Fife, MD, FAAN, FANS

ABSTRACT
PURPOSE OF REVIEW: This article reviews a method of obtaining the medical
history of patients presenting with dizziness, vertigo, and imbalance. By
combining elements of the history with examination, the goal is to identify
patterns and an effective differential diagnosis for this group of patients
to help lead to an accurate diagnosis.

RECENT FINDINGS:Studies over the past dozen years have changed the
historical approach to patients with dizziness from one based primarily
on how the patient describes the sensation of dizziness. This older
approach can lead to misdiagnosis, so a preferred method puts greater
emphasis on whether the dizziness is acute or chronic, episodic or
continuous, or evoked by or brought on by an event or circumstance so that
a pattern may be derived that better narrows the differential diagnosis
and focused examination can further narrow to a cause or causes.

SUMMARY: Dizziness is a common symptom of many possible causes. This

article will help clinicians navigate gathering the history and examination to
formulate a working diagnosis in patients affected by dizziness.

CITE AS: INTRODUCTION

D
CONTINUUM (MINNEAP MINN)
2021;27(2, NEURO-OTOLOGY):
izziness is frequently encountered in neurology, otolaryngology, and
306–329. general medical practice, and generates many visits to emergency
departments and other medical clinics. The lifetime prevalence of
Address correspondence to having significant dizziness is between 17% and 30%.1 The lifetime
Dr Terry D. Fife, Barrow
Neurological Institute, 240 W prevalence of dizziness due to vestibular disease was 7.4% in one
Thomas Rd, Ste 301, Phoenix, population-based survey.2 A multinational observational registry of patients
AZ 85013, [email protected]. with some form of a peripheral vestibular symptom found that 65% were female
edu.
and that those aged 51 to 60 years were the most commonly affected. The age
RELATIONSHIP DISCLOSURE: group between 41 and 70 years accounted for almost two-thirds of all patients
Dr Fife reports no disclosure.
with dizziness whereas patients older than 70 accounted for 18.4%.3 Another
UNLABELED USE OF study in a neuro-otology clinic found that the peak age group for having dizziness
PRODUCTS/INVESTIGATIONAL was 60 to 69 years, and women were more likely to have nonperipheral
USE DISCLOSURE:
Dr Fife reports no disclosure.
vestibular causes of dizziness.4 This suggests, perhaps contrary to a widely held
view, that people 70 years and older are not the age group most commonly
seen for peripheral vestibular disorders. Meanwhile, in a study of all referred
© 2021 American Academy
of Neurology. patients with a chief complaint of dizziness at an academic otolaryngology

306 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

neuro-otology clinic where one might think peripheral vestibular causes would
be especially common, the researchers found 42% of patients did not have
peripheral vestibular disorders. This underscores the breadth of possible causes
of dizziness well beyond just inner ear disorders. Furthermore, approximately
one in six patients presents with two causes of dizziness at the same time.4
TABLE 1-1 lists these and some additional examples of common types of dizziness
that often occur together. The most common combinations of two diagnoses
found in a cohort study of an academic otolaryngology clinic were (1) vestibular
migraine and benign paroxysmal positional vertigo (BPPV) and (2) vestibular
migraine and Ménière disease.4
Many patients with chronic dizziness have an expensive and often long
sojourn before an accurate diagnosis is made. One survey study found that many

Common Combinations of Simultaneous or Overlapping Disorders Causing TABLE 1-1

Dizziness or Vertigo

Combined disorders Typical associated history

Vestibular migraine and benign paroxysmal Episodes of spinning, rocking swaying, tilting, and frequent motion sensitivity
positional vertigo (BPPV) with nausea that last minutes to many hours and frequent visually induced
vertigo, photophobia, and periodic migraine headaches AND intermittent
spells of vertigo triggered by the head tilting back or looking up or when turning
in bed

Ménière disease and vestibular migraine Random prolonged spells of spinning lasting hours and associated with
unilateral fluctuating hearing and tinnitus and low-frequency hearing loss on
audiometry AND episodes of spinning, rocking swaying, tilting, and frequent
motion sensitivity with nausea that last minutes to many hours and frequent
visually induced vertigo, photophobia, and periodic migraine headaches

Vestibular migraine and persistent postural Episodic spells of vertigo, rocking swaying, tilting, floating, and frequent
perceptual dizzinessa motion sensitivity with nausea, visually induced vertigo, photophobia, periodic
migraine headaches, and associated generalized anxiety

Vestibular neuritis and vestibular migraine Acute onset of spinning vertigo lasting several weeks and gradually abating
over time but overtaken by ongoing motion sensitivity with nausea, visually
induced vertigo, photophobia, and periodic migraine headaches

Vestibular neuritis and BPPV Acute onset of spinning vertigo lasting several weeks and gradually abating
over time but punctuated by intermittent spells of vertigo triggered by head
tilting back or looking up or when turning in bed

Vestibular neuritis and persistent postural Acute onset of spinning vertigo lasting days and gradually abating over time
perceptual dizziness but overtaken by constant rocking and/or floating sensations without nausea,
with visually induced vertigo and associated generalized anxiety

BPPV and persistent postural perceptual Intermittent spells of vertigo triggered by head tilting back or looking up or
dizziness when turning in bed with rocking and/or floating sensations without nausea,
with visually induced vertigo and possibly associated generalized anxiety

BPPV and orthostatic intolerance or Intermittent spells of vertigo triggered by head tilting back or looking up or
orthostatic hypotension when turning in bed and also intermittent near-fainting lightheaded sensations
on standing

a
This combination is fairly common, and determining which disorder accounts for most of the dizziness requires close monitoring of the response
to treatments.

CONTINUUMJOURNAL.COM 307

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

were referred to multiple health care providers, but one-third still felt frustrated,
misdiagnosed, or misdirected.5 This article aims to guide practicing clinicians
in their approach to patients with dizziness and to use the data from the history
and examination to discern a pattern associated with a more specific causative
diagnosis.

CLASSIFICATION OF VESTIBULAR SYMPTOMS

TABLE 1-2 outlines definitions of common vestibular symptoms as determined by
the International Classification of Vestibular Disorders of the Bárány Society.6
These definitions were developed to standardize nomenclature used in clinical
and research communications among those who care for patients with vestibular

TABLE 1-2 Definition of Common Vestibular Symptoms as Determined by International

Consensusa,b

Symptom Definition Subtypes

Vertigo Sensation of self-motion when no self-motion is Spontaneous: occurs without obvious trigger
occurring or the sensation of distorted self-motion
during an otherwise normal head movement Triggered: occurs with an obvious trigger

• Positional: triggered after changing head

position

• Head motion: occurs only during head motion

• Visually induced: triggered by seeing objects

in motion in the visual surround

• Sound-induced: triggered by sound

• Valsalva-induced: triggered by Valsalva

maneuver or straining

• Orthostatic: triggered by change in body

position from lying or sitting to standing

• Other triggered forms

Dizziness Sensation of disturbed or altered spatial Spontaneous: occurs without obvious trigger
orientation without the feeling of false motion
Triggered: occurs with an obvious trigger

• Positional: triggered after changing head position

• Head motion: occurs only during head motion

• Visually induced: triggered by seeing objects

in motion in the visual surround

• Sound-induced: triggered by sound

• Valsalva-induced: triggered by Valsalva

maneuver or straining

• Orthostatic: triggered by change in body

position from lying or sitting to standing

• Other triggered forms

CONTINUED ON PAGE 309

308 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

and balance disorders. Unlike common teaching in the past, the International
Classification of Vestibular Disorders system does not consider vertigo to be a
subtype of the broader rubric of dizziness but rather a separate descriptor from
dizziness. Vertigo designates the false perception of motion in any direction
whereas dizziness designates distortion of spatial orientation without the
perception of motion. Thus, by using this construction, a patient with a single
disorder, such as BPPV, could simultaneously describe vertigo, dizziness, and
postural symptoms even though he or she has a single cause of symptoms. In the
International Classification of Vestibular Disorders definitions, the term
vestibular symptoms does not mean that the symptoms are necessarily caused by
or directly related to vestibular structures or physiology; rather, it is meant to be

CONTINUED FROM PAGE 308

Symptom Definition Subtypes

Vestibulovisual Visual symptoms resulting from vestibular External vertigo: illusion that the visual surround is
symptoms dysfunction or from the interaction of the visual spinning or flowing
and vestibular systems; examples include visual
illusions that the environment is tilted or blurring of Oscillopsia: the perception that the visual
visual lag during head movements; categorized as surround is oscillating or bouncing
external vertigo
Visual lag: the illusion that the visual surround lags
behind during head movement

Visual tilt: the illusion that the visual surround is

not true vertical

Movement-induced blur

Postural symptoms Balance-related symptoms that occur when Unsteadiness: the feeling of being unstable when
upright (seated, standing, walking); examples seated, standing, or walking
include feeling unsteady, swaying, or rocking only
when upright Directional pulsion: unsteadiness with a feeling of
veering or falling to a particular direction

Balance-related near fall: a feeling of imminent or

nearly falling due to vestibular symptoms, pulsion,
or unsteadiness

Balance-related fall: a complete fall due to

vestibular symptoms, pulsion, or unsteadinessc

a
Data from Bisdorff A, et al, J Vestib Res.6
b
The use of the term vestibular symptoms in this table is meant to refer to the symptoms often used but does not mean to imply that all conditions
causing these symptoms have a basis in vestibular pathways or mechanisms.
c
Not used are terms such as drop attack, otolithic crisis, otolith crisis of Tumarkin. Instead, the consensus panel opted for the terms balance-related
falls or near falls.

CONTINUUMJOURNAL.COM 309

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

a broader term of phenomenological descriptions only some of which have

vestibular causes.6 When terms such as vestibular disorders, processes, mechanisms,
or causes are mentioned, then this does refer to conditions that are due to
vestibular structures or mechanisms. Although this set of definitions is intended
to permit physicians to speak a common language, it should be expected that
patients will continue to describe symptoms in their own way.

WAYS TO EVALUATE PATIENTS WITH DIZZINESS

A patient with dizziness can be approached in several ways, and what seems
easiest or most useful for each clinician may vary by background, practice
setting, specialty, and experience. No system is necessarily ideal for all specialties
in all clinical settings. All the approaches are similar and involve data gathering in
the history and examination, but which data and which steps follow vary slightly
in each approach. Following are four examples:

1 Pattern recognition. This approach focuses on using data from the history and examination
to identify patterns (or overlapping patterns) that best fit with a specific cause or causes.
The disadvantage is that it takes time to gather details of the history and experience to
become familiar with common clinical patterns of disorders that cause vertigo or dizziness.
The advantage for the neurologist is that this approach is what neurologists do every day in
evaluating patients. For example, neurologists routinely use these methods for evaluating
patients with headache, muscle weakness, or unexplained spells. It is the nature of the

TABLE 1-3 Common Vestibular Disorders Associated With Major Syndrome Categoriesa

Syndrome Description Examples of disorders

Acute vestibular A syndrome of acute-onset, continuous vertigo, Vestibular neuritis, stroke causing vertigo, acute
syndrome dizziness, and unsteadiness lasting days to weeks drug toxicity, demyelinating disease
often associated with nausea, vomiting, nystagmus, vestibulopathy, Wernicke syndrome, selective
and vertigo or dizziness aggravated by head motion in serotonin reuptake inhibitor (SSRI) or serotonin
any direction norepinephrine reuptake inhibitor (SNRI)
discontinuation

Episodic A syndrome of recurrent spells of vertigo, dizziness, Spontaneous: vestibular migraine, Ménière
vestibular or unsteadiness lasting seconds to hours, disease, transient ischemic attack (vertebrobasilar
syndrome occasionally days. The episodes may be associated insufficiency), vestibular paroxysmia, cardiac
with brief periods of nausea, nystagmus, loss of causes (aortic stenosis, arrhythmia), episodic
balance, headache, central nervous system ataxias
symptoms, or hearing symptoms
Triggered: benign paroxysmal positional vertigo,
orthostatic intolerance or hypotension, motion
sickness, central positional vertigo

Chronic A syndrome of chronic vertigo, dizziness, or Persistent postural perceptual dizziness, bilateral
vestibular unsteadiness lasting months to years; symptom vestibulopathy, late effects of stroke, cerebellar
syndrome descriptions may include gait unsteadiness, ataxia, ataxias, posterior fossa neoplasms, chronic
hearing loss, nausea, nystagmus, or oscillopsia; may visually induced vertigo or dizziness, mal de
result from a progressive neurodegenerative débarquement
disorder, a static deficit in vestibular function, or
evolving symptoms between episodic vestibular
episodes

a
Data from Bisdorff A, et al, J Vestib Res6 and Bisdorff AR, et al, Neurol Clin.9

310 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 specialty (and other medical specialties as well) to identify patterns of symptoms and signs KEY POINTS
that point to a specific disorder. This is the approach discussed in this article and is not
really dissimilar to the International Classification of Vestibular Disorders approach or ● Dizziness is a common
those guided by TiTrATE (Timing, Triggers, Associated symptoms and Targeted symptom that occurs at all
Examination)7 or ATTEST (Associated symptoms, Timing, Triggers, Examination Signs and ages but especially in
Testing)8 described later in this list. patients aged between 41
2 International Classification of Vestibular Disorders classification. This is not so much an and 70 years.
approach as it is an organizational scheme to proceed from symptoms and signs to syndromes
to mechanisms in the cause of vertigo and dizziness. It consists of using information from the ● Peripheral vestibular
history and examination to place the patient’s symptoms into one of three syndrome patterns: disorders are common, but
(1) acute vestibular syndrome; (2) episodic vestibular syndrome (episodic or triggered); or (3) half of patients with
chronic vestibular syndrome. Once categorized in one of these syndrome patterns, each dizziness have a
syndrome being associated with a finite list of specific diagnoses or mechanisms (TABLE 1-3),6,9 nonvestibular mechanism,
the differential diagnosis can be narrowed and pattern recognition is used to arrive at a and approximately one in six
specific diagnosis. A limit of this system is that some vestibular diagnoses involve symptoms patients present with two
that cross boundaries between episodic vertigo and chronic vertigo because disorders do not different causes of dizziness
always fit neatly into the syndromic category. In addition, when a patient has more than one at the same time.
form of dizziness simultaneously, which occurs in nearly 20% of cases, this must be recognized
and the approach must be applied to each type of dizziness. ● Many patients with
dizziness see multiple health
3 TiTrATE and ATTEST. TiTrATE is an acronym for Timing, Triggers, Associated symptoms and care providers in evaluation
Targeted Examination.7 ATTEST is an acronym for Associated symptoms, Timing, Triggers, of the dizziness and feel
Examination Signs and Testing.8 These algorithms use memory aids to help clinicians recall frustrated, misdiagnosed, or
which parts of the history are most essential. However, some pattern recognition is misdirected.
needed; pattern recognition is still very helpful for the most common urgent care causes of
dizziness such as BPPV, vestibular neuritis, and stroke presenting with isolated dizziness or ● Overreliance on a
vertigo. This system was developed as a way of improving diagnosis, particularly for those patient’s description of the
in primary care and emergency settings who need to keep their history abbreviated. dizziness and using it as the
4 Symptom description. This now-outdated approach introduced in the early 1970s is based main piece of information to
on categorizing the patient’s description of dizziness as fitting into one of four categories: choose among causes leads
vertigo, presyncope, impaired equilibrium, or nonspecific dizziness. Data over the past to mistakes in the diagnosis.
dozen years have made apparent that using a symptom characterization as the sole
algorithmic branch point leads to more misdiagnosis when compared with using timing ● All aspects of history
and trigger information. This is especially true in the acute care setting such as in (symptom description,
emergency departments. The symptom description approach was introduced at a time onset, frequency, duration,
when it was believed that the most common single cause of dizziness was “hyperventilation,” and provoking or aggravating
which was said to account for 22% of dizziness. This was tested by having the patient circumstances) should be
hyperventilate, and, if the sensation resembled his or her dizzy sensation, then questioned until understood
“hyperventilation can often thus be diagnosed without further ado.”10 This is no longer as well as possible because
considered valid. Although some value remains in obtaining a description of the patient’s any part of the history can be
sensation, its value in diagnosis tended to be overemphasized by using this approach. miscommunicated by a
The description should be combined with other features of the history including onset, patient or misunderstood by
duration, triggers, factors that aggravate symptoms, and so on. the health care provider.

It is not necessary to abandon any aspect of the history that a patient can
describe consistently and reliably. All aspects of history (symptom description,
onset, frequency, duration, and provoking or aggravating circumstances) are
prone to being incorrectly conveyed by the patient or incorrectly interpreted by
the clinician (CASE 1-1). Clinicians should probe until the question is understood
and answered as well as possible. At times, the history may still fit no pattern or
simply remain unclear. Limitations in recalling and describing sensations,
discerning triggers, and recalling durations are understandably susceptible to
some unreliability. Memory is malleable and can be influenced by the questions
asked or by friends and family, prior life experiences, anxiety and emotional
links to the memory, and other factors.12 Taking a history is still partly an art. In
addition, health care providers must also watch for their own bias errors in
concluding a diagnosis (TABLE 1-4).

CONTINUUMJOURNAL.COM 311

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

CASE 1-1 A 37-year-old woman presented with dizziness that came on suddenly
4 weeks earlier as a feeling of abrupt intense spinning. She believed the
dizziness was related to her neck because she had some neck pain on and
off for which she sought chiropractic care in the past that helped. She
said the dizziness lasted for minutes to hours, and she had some nausea,
as well. At times, she felt like her “head is swimming,” and, when she got
up, sometimes she felt off balance or tilted to one side briefly. When
asked what she was doing at the time of the first episode, she indicated
she was getting out of bed. Another time she had straightened up after
putting away some shoes on the closet floor. She also indicated that none
of the spells occurred when she had remained completely motionless but
seemed triggered by moving her neck.
Examination was normal including tandem gait and no spontaneous or
gaze-evoked nystagmus was seen. She had paroxysmal upbeating
torsional nystagmus with Dix-Hallpike positioning to the right; that is,
nystagmus fast phases caused the upper pole of her eyes to beat toward
the right ear during straight-ahead gaze so that the upper pole of the right
eye extorted and the upper pole of the left eye intorted. This gave the
appearance from the examiner’s viewpoint of counterclockwise torsional
nystagmus, characteristic of right posterior canal benign paroxysmal
positional vertigo (BPPV).

COMMENT In this case, the patient gave a clear history of a spinning feeling but also of
a swimming sensation. This was a clue for a vestibular mechanism;
however, as an isolated piece of the history, a vestibular mechanism was
suspected but certainly not assured. The patient also thought the neck
movement was the trigger, but with further questioning, it was actually
head movement relative to gravity that was the trigger, and neck
movement, by necessity, also occurred. Finally, the patient gave imprecise
durations of the symptoms because she lumped the spinning feeling
together with the vague swimming-head after-effect she felt at times
when active. This makes the point that patients may have several durations
and sensations from the same disorder and may misattribute the triggering
circumstance and conflate several sensations, resulting in incorrect timing
or duration of dizziness or vertigo. It also points out why examining with the
Dix-Hallpike test is advisable in patients with vestibular symptoms even if
their history seems to steer one away from the diagnosis of BPPV.
Throughout this issue, the term Dix-Hallpike test is used rather than
Dix-Hallpike maneuver (even though both are correct) to distinguish it from
treatment maneuvers such as canalith repositioning or the Epley maneuver,
which are discussed in more detail in the article “Episodic Positional
Dizziness” by Kevin A. Kerber, MD, MS,11 in this issue of Continuum.

312 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Taking the History
Good history-taking both conveys empathy and gathers important information
about the medical symptoms.13 It is often best to start with an open-ended question
about when the symptoms began and what the patient is experiencing. Some
patients give a very good and cogent history of their symptoms and prefer to be
listened to. If the patient begins by talking about prior tests or what others have told
him or her, try to redirect the patient to get the key details of the actual symptoms.
Once the current symptoms and symptom evolution up to the time of the visit are
obtained, then one can review the test results, prior diagnoses, and treatments tried.

SYMPTOM DESCRIPTION. As mentioned earlier, overemphasis of the description of

the sensation of dizziness or vertigo can lead to misdiagnosis, especially in the
emergency department setting.14 Nevertheless, one should not ignore this part of
the history but rather take it in context along with the other historical
information. Patients may describe spinning, whirling, rotational sensations,
tilting, sinking, free-falling, or rising, and all these symptoms defined as vertigo
imply a higher likelihood of a vestibular process. Such symptoms are quite
uncommon in presyncope, for example.15 Dizziness and lightheadedness,
nausea, yawning, visible pallor observed by others, diaphoresis, and a feeling as
though one is about to pass out are much more characteristic of cardiovascular
causes of dizziness.15 Patients with vestibular migraine may report a multitude of
sensations, including spinning, rocking, floating, motion sickness, and visually
induced vertigo and dizziness, whereas patients with Ménière disease are much
more likely to describe vertigo as a spinning feeling with nausea and vomiting,
often to the point they do not want to even move during attacks.

SYMPTOM ONSET. The mode of onset can be helpful in some cases; some causes
can lead to abrupt symptoms and some can evolve more gradually, and others
may have a clear relation to a specific event. For example, acute vertigo after
significant head trauma suggests a traumatic vestibulopathy. When the onset
occurs after minor trauma or a mild concussion and symptoms are subjective and

Error Avoidance in Diagnosis of Dizzinessa TABLE 1-4

Error Remedy

Incorrect data Probe the answers and take a careful history. Take a repeat history on follow-up to reaffirm the
diagnosis or revise the diagnosis. Recollection is itself influenced by prior questioning as
patients begin to think about their previous responses and may give somewhat different
answers with sequential questioning.

Lack of familiarity with the Lack of familiarity with the pattern of a cause or encountering a rare pattern is part of training
patterns and experience and why lifelong learning is crucial. Be aware of and keep up with
evidence-based guidelines where they exist to expand one’s sphere of competence.

Cognitive bias We all have a tendency to draw conclusions that reinforce our first impression or to be
influenced by the historian or by the way the history is delivered (eg, haphazard, tangential,
fraught with an anxious affect, having an overly inclusive symptom list). Care should be
exercised to avoid dismissing patient symptoms because the patient relays the history poorly
or with a great deal of superimposed anxiety.

a
Errors in identifying the diagnosis can arise from several sources, only some of which are listed in this table.

CONTINUUMJOURNAL.COM 313

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

signs are absent, one must view this with circumspection and in full context. The
onset of rocking or swaying dizziness without nausea after a cruise suggests
mal de débarquement. Onset of dizziness after turning in bed or tilting the head
can suggest BPPV as this may indicate that position changes are actual triggers
(see the Triggers section). Abrupt onset of vertigo and loss of equilibrium without
known provocation can suggest a vascular mechanism, especially if accompanied
by the simultaneous onset of other neurologic features. Symptoms that begin after
initiation of a new medication can indicate a medication is the cause (CASE 1-2).

SYMPTOM PERIODICITY. It can be helpful to ascertain whether symptoms are

episodic or constant or whether the symptoms are constant at a low ebb but
periodically intensify and whether they are influenced by a particular
circumstance. For example, some patients with vestibular migraine have
episodes of vertigo but no symptoms in between. A bit more commonly,
however, some patients with vestibular migraine have a continuing susceptibility
to motion sickness and visually induced vertigo but try to avoid those triggers
and then, in addition, have some periodic spontaneous vertigo spells. Ménière
disease is characteristically episodic often with some residual symptoms for a
few days after attacks if they are severe, but patients usually recover before the
next attack. Occasionally, Ménière disease may cause attacks with enough

CASE 1-2 A 63-year-old woman reported nearly 1 year of dizziness and feeling
unsteady. She described feeling off balance when she was up and
walking or with activity on her feet. She had no spinning or sensations of
motion and speculated that it may be her vision or her neck, but
evaluations by an ophthalmologist and cervical spine MRI performed by
an orthopedic surgeon did not confirm her suspicions. She had tried
acupuncture, chiropractic treatment, and meclizine. Acupuncture
seemed to make her neck feel better, but none of these measures
improved the dizziness. She said her dizziness was simply present all the
time. She had no focal weakness, numbness, change in hearing, slurred
speech, or visual symptoms. She said her neck pain “acts up” sometimes,
and she avoided lying on her left side when sleeping. Her past medical
history was notable for a 10-year history of type 2 diabetes mellitus and
moderate neuropathy.
Examination was normal except for neuropathy in a stocking
distribution to the ankles. Dix-Hallpike positioning to the left revealed
paroxysmal positional nystagmus, which was treated.

COMMENT Many patients try to identify the causes of their symptoms and sometimes
develop incorrect conclusions. In this case, the patient gave little reason to
suspect benign paroxysmal positional vertigo (BPPV) except that she could
not lie on her left side. Her imbalance was probably a combination of mild
neuropathy, and her dizziness was likely related to BPPV during motion
activities. As in the previous case, Dix-Hallpike positioning can reveal BPPV
even when the history reported lacks appropriate timing and triggers.

314 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

frequency that patients barely recover from one spell before another occurs. KEY POINTS
Vestibular neuritis usually has a monophasic course of abrupt severe symptoms
● History-taking is best
with slow and gradual resolution over a week to several months depending on started with an open-ended
its severity. BPPV is characteristically episodic, but if a patient has some question to allow patients to
proneness to motion sickness, some degree of dizziness might be reported as relay how the symptoms
seeming to be nearly constant with periodic worsening. began and what they
experience, although
DURATION OF SYMPTOMS. Particularly in the case of vertigo, BPPV should be high patients may need to be
redirected in some cases.
on the list of possible causes of spells that are shorter than 1 minute. For spells
lasting minutes, a transient ischemic attack affecting vestibular structures ● Excessive reliance on the
and vestibular migraine are considerations. For spells lasting 2 to 6 hours, patient’s description of
Ménière disease and vestibular migraine can be causes. Chronic symptoms can be dizziness or vertigo leads to
mistakes in diagnosis, but
seen with persistent postural perceptual dizziness, mal de débarquement,
some patients accurately
bilateral vestibulopathy, and cerebellar degeneration. describe spinning, whirling,
rotational sensations that do
TRIGGERS. A trigger should be obvious and reproducible to qualify as a trigger and indeed imply a higher
not just a possible exacerbating circumstance. For example, Dix-Hallpike likelihood of a vestibular
positioning is a trigger for active BPPV because it results in vertigo most of the process.

time it is performed. However, someone with vestibular neuritis will feel ● For patients who describe
dizzier during the Dix-Hallpike test but will also feel increased dizziness with clear vertigo (spinning,
any kind of head motion until compensation has taken place. The latter is whirling, rotation), if spells
therefore not really a trigger as much as an aggravating circumstance. Vestibular last less than 1 minute, then
benign paroxysmal
migraine can sometimes be aggravated by fragrances, certain visual stimulation, positional vertigo may be
or excessive head motion, but these do not actually always trigger a spell as much the cause. If the spells last
as increase the likelihood of a spell or making ongoing dizziness worse. Dizziness minutes, transient ischemic
can be triggered by orthostatic postural changes in someone severely prone to attack or vestibular migraine
should be considered. If the
orthostatic hypotension or postural tachycardia syndrome (POTS).
spells last hours, Ménière
disease or vestibular
ASSOCIATED FEATURES. Sometimes the associated features can be characteristic or migraine may be the cause.
very helpful. Ménière disease attacks can be preceded by or associated with
unilateral ear fullness and muffled hearing and louder low-pitched roaring ● It is helpful to ask patients
tinnitus related to one ear. Transient ischemic attacks may be associated with about the impact the
dizziness or vertigo has on
focal hemisensory symptoms, dysarthria, diplopia, or hemiataxia. Superior canal their quality of life and
dehiscence syndrome may be accompanied by autophony, a heightened hearing ascertain their goal for the
of internal body sounds. Dizziness or postural symptoms from cerebellar ataxia visit and evaluation because
may be accompanied by dysarthria, gait ataxia, and ocular motor abnormalities some patients just want to
be reassured that the cause
causing blurry vision during gaze changes. Anxiety is commonly associated with is benign but can live with
many conditions that lead to dizziness, sometimes as part of the underlying cause but the symptom if need be,
often as a reaction to the symptoms, the loss of control, and feelings of uncertainty whereas others are
about when symptoms will occur or whether they will become severe. desperate for treatment to
relieve the symptoms.
IMPACT ON QUALITY OF LIFE. Establishing the impact on the patient’s life is
important in gauging how aggressive to be in the workup and treatment.
Along with this assessment should be a discussion on what the patient expects
or would like from the visit. Some patients may have what seems to be fairly
minor dizziness, but they are very worried about it due to their own concerns
with having something serious. In some of those cases, once they feel it has been
adequately established to be benign, they do not want any medication but would
consider other approaches. Some patients, particularly those with persistent
postural perceptual dizziness (also referred to as PPPD), may have a normal
examination but view their lives as severely negatively impacted by the

CONTINUUMJOURNAL.COM 315

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

symptoms. Knowing this will help produce a better treatment plan and a more
agreeable experience and outcome for the patient (CASE 1-3).

PREVIOUS TREATMENTS TRIED. In some cases, if no treatment has yet been tried or
it is the patient’s first medical evaluation, this is a brief conversation. In other
cases, many opinions and tests have been offered but no actual treatments have
been attempted. If a patient has been treated, the previous treatment can be
noted; if it failed in the past, perhaps it should not be repeated. Some patients
may have symptoms amenable to physical therapy but say it did not work in
the past. It can be helpful to ask the patient to recount what was done by the
therapist and how many sessions were attended. Some patients fail to respond
because the therapist mistakenly tried to treat for BPPV but the patient actually
has vestibular neuritis or another condition. Other patients may not have
complied with a reasonable trial because they were not convinced it would help.
In other cases, a medication may have been used but at such a low dosage or for
such a short therapeutic trial it should be reconsidered.

CASE 1-3 A 41-year-old man presented with 3 years of intermittent dizziness and
vertigo. He was not really sure when the dizziness began, but the first
time he recalled it occurring was when he was exercising vigorously at a
gym training for a triathlon and he developed sudden wooziness and a
feeling of spatial disorientation that lasted several minutes. He also
described an occasional spontaneous sensation of spinning often lasting
just seconds, and he had a constant low-grade motion sickness
aggravated by repetitive head movements and by seeing pattern and
object motion around him. Nausea and a low-grade sense of rocking or
swaying dizziness became a daily problem, and he took ondansetron
most days to mitigate this. He stopped the training he used to do because
of the dizziness and nausea. He had a history of migraine and got about six
severe migraine headaches per year and much more frequent lower-level
headaches that he worked through.
Examination was normal. Brain MRI, a hearing test, and
videonystagmography were all unremarkable.

COMMENT This is most likely vestibular migraine, although rocking/swaying and

visually induced dizziness as the patient described can also be seen with
persistent postural perceptual dizziness. He also had so-called quick spins
(that is, fragments of vertigo consisting of a partial turn for a brief moment
that then stops without continuing but may recur many times). He also had
significant nausea and migraine headaches. This patient’s quality of life
was diminished by the symptoms, and despite 3 years of seeing physicians,
he had not received a diagnosis or been tried on any therapy aside from
ondansetron. After discussion with the patient about migraine treatment
options, nortriptyline was started and titrated to 75 mg daily with
significant improvement so that he was able to return to all previous
activities.

316 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Examination
Note whether the patient came in with a walking assistive device and observe
whether the device is worn from use and inquire how long has the patient used
it and why. For example, a cane may be used due to knee arthritis or perhaps
because of imbalance or recurrent falling. Consider whether the reported severity of
vertigo, dizziness, or imbalance is proportional to observed balance and function.
If the patient reports constant nausea and inability to walk, does that fit with his or
her appearance? Does the patient sit straight and use his or her arms to stand, and is
it done with little or significant effort? Take note of the interaction with others in
attendance and the patient’s affect, train of thought, attention to details, and the
logic of chronology of history as relayed by the patient.
The neurologic examination of the patient with dizziness or vertigo should
include evaluation for potential related signs such as Horner syndrome,
hemisensory deficits, unilateral facial weakness, dysarthria, limb ataxia,
dysconjugate gaze, head tilt, spasticity, abnormal reflexes, or distal
somatosensory deficits, which may be clues to localizing lesions in the
cerebellum, brainstem, spinal cord, or peripheral nerves. Such signs can localize
the vertigo or dizziness to a central nervous system (CNS) location or explain
unrelated comorbid conditions that contribute to imbalance or tendency to fall.
Otoscopy is not part of the standard examination for many neurologists
but can be helpful in excluding obvious ceruminous impaction; a bulla or cyst;
or perforation, darkness, or coloration changes of the tympanic membrane.
Orthostatic vital signs are indicated when orthostatic hypotension or
intolerance is suspected.

Areas of Special Focus for Patients With Vertigo and Dizziness

Although much can be garnered from the neurologic examination, several areas
of the examination (eye movements, speech articulation, cerebellar functions,
vestibular function, Romberg sign, gait, and tandem gait) are particularly
important in evaluating patients with dizziness or vertigo. A more detailed
coverage of the bedside examination is included in the articles “Vestibular
Testing” by Timothy C. Hain, MD, and Marcello Cherchi, MD, PhD, FAAN,16
and “Episodic Positional Dizziness” by Kevin A. Kerber, MD, MS,11 in this issue
of Continuum.

STANCE, GAIT, AND ROMBERG SIGN. Imbalance and gait difficulty overlap but are
not the same. Balance entails the maintenance of steady weight distribution by
adjusting the center of gravity in all positions whether stationary or while in
motion. Balance uses CNS integration mostly via the cerebellum to maximize
control of weight distribution by using somatosensory, visual, and vestibular
inputs. Stability in gait requires the integration of balance and motor control but
is also influenced by cognitive judgment and anticipation and orthopedic
considerations. For patients with dizziness overall, balance is judged by gait
speed, gait base, and the ability to walk in tandem and maintain balance during
and after quick turns. Other elements of gait, including cadence (steps per
minute), stride length, floor clearance, gait ignition, arm swing, and foot strike
location, may provide additional information. Examination of gait should be
included whenever possible in patients with dizziness or vertigo.
Some patterns can steer toward a possible diagnosis. Falls during Romberg
testing may indicate bilateral vestibulopathy or somatosensory dysfunction. A

CONTINUUMJOURNAL.COM 317

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

wide-based gait may be seen in chronic communicating hydrocephalus, ataxic

neuropathy, or hypothyroidism but is most characteristic of cerebellar
dysfunction. Patients with acute vertigo (less than 10 days from onset) due to
vestibular neuritis commonly have a slight change in gait mechanics, including a
longer stride length and longer stance time using the ipsilesional leg and an
overall tendency to minimize head movements,17 although this usually

CASE 1-4 A 57-year-old man was in good health until about 8 months earlier when
he developed a feeling of spinning when getting out of bed. He staggered
to the bathroom, the vertigo continued, and, within 20 minutes, he
started vomiting. He stayed home from his job as a claims inspector for a
commercial trucking company and sat, hoping it would resolve, but, after
an hour, he went to a local emergency department. There, a head CT, a
head and neck CT angiogram, ECG, and laboratory values were all
negative, and he was sent home with instructions to see an
otolaryngologist the next day. He saw an otolaryngologist 9 days later,
had a normal audiogram, and was told he may have a viral inner ear
infection and that it would resolve. In the weeks that followed, he
noticed some improvement, but his improvement plateaued and
eventually worsened such that he developed continual dizziness. He
described no nausea but felt a continuous swaying and floating sensation
that seemed to be constant and diminished his ability to focus at work. He
was bothered by environments with fluorescent lights whenever in
crowds or stores where there seemed to be too much visual stimulation.
A trial of 15 sessions of vestibular physical therapy 4 months after the
onset of vertigo did not help. He was functioning at work but felt he could
not go on living like this and was desperate. Brain MRI was normal.
Videonystagmography that had been performed 4 weeks after the onset
of vertigo showed 38% right reduced vestibular responses and another
videonystagmography 5 months after the onset of vertigo was normal.
Examination was normal, including head impulse testing. When
exposed to an optokinetic strip, he appeared to be bothered by it,
although his optokinetic responses were normal.

COMMENT This patient most likely has had two types of dizziness. He had vestibular
neuritis at the outset that accounted for his early symptoms, but, over time,
the dizziness changed from vertigo and dizziness with quick movements to
a feeling of constant swaying and floating. One could speculate that he did
not compensate for the unilateral vestibular loss, but this is less likely
because vestibular function by head impulse testing clinically normalized
after several months as occurs in nearly half of cases of vestibular neuritis.
The dizziness that has bothered him for the past 5 to 6 months is persistent
postural perceptual dizziness, a condition that sometimes follows a
vestibular event. Despite a normal examination, this patient was greatly
troubled by the ongoing dizziness, which is common in persistent postural
perceptual dizziness as well.

318 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

normalizes in 6 to 9 weeks after onset as the patient achieves compensation.18 KEY POINTS
This manifests as a gait that appears cautious, a bit slower, and with some
● Spontaneous downbeat
minimization of normal head movements (CASE 1-4). nystagmus should be
Between bouts of vertigo due to BPPV and Ménière disease and with considered a central finding
vestibular migraine and most cases of PPPD, the gait is either normal or mostly that localizes to the
normal with some guardedness. Most patients with PPPD have a normal gait or cerebellar vermis or
cervicomedullary junction.
occasionally appear to walk cautiously.
● In peripheral vestibular
EYE MOVEMENTS, NYSTAGMUS. Examination of eye movement abnormalities can horizontal nystagmus, the
be very helpful in localization. For patients with dizziness and vertigo, eye nystagmus stays in one
direction, intensifying with
movement findings can help in clarifying the cause of vertigo. For example, gaze in the direction of the
lateropulsion (or ipsipulsion) of saccades, in which saccades toward the side of fast phase and diminishing
the lesion tend to overshoot the target whereas saccades to the opposite side tend or abating with gaze in the
to undershoot the target, is a feature seen in lateral medullary syndrome direction away from the fast
phase of nystagmus.
(Wallenberg syndrome). Saccadic dysmetria, in which bidirectional
overshooting of the target and occasional undershooting occurs, localizes to the
cerebellar vermis and fastigial nucleus. Skew deviation of the eyes is
characterized by acquired misalignment of the eyes due to a disturbance of the
otolith ocular pathways that project to the interstitial nucleus of Cajal. Skew
deviation is more common in central vestibular lesions but may be seen in
peripheral vestibular lesions, as well. Skew deviation from peripheral
vestibular lesions has a small amplitude and abates within days as
compensation of the acute peripheral vestibular asymmetry occurs.19 The
article “Acute Vestibular Syndrome” by Kristen K. Steenerson, MD,20 in this
issue of Continuum discusses skew deviation further in the context of acute
continuous vertigo.
Nystagmus is a hallmark sign of vestibular disorders. Nystagmus may be
categorized in many ways, but for practical clinical purposes, it may be
spontaneous, gaze related, or positional.21 If nystagmus is present in primary
gaze, then it is considered spontaneous nystagmus. If nystagmus is not
spontaneous but occurs with a gaze to any direction, then is it gaze related or
gaze evoked. If it is not spontaneous or gaze-related nystagmus but position
changes induce the nystagmus, it is positional nystagmus.
Examination for nystagmus can provide valuable information in patients with
acute, episodic, and chronic vestibular syndromes.

SPONTANEOUS NYSTAGMUS. Spontaneous downbeating nystagmus should be

considered of central origin and localizes to the cerebellar vermis and
cervicomedullary junction. The most common conditions causing it are
cerebellar ataxias, less commonly Chiari malformation or multiple sclerosis.
Spontaneous upbeating nystagmus is encountered much less frequently but
may be seen in cerebellar ataxias, multiple sclerosis, Wernicke syndrome,
autoimmune encephalitis, or lesions of the medial dorsal medulla. Spontaneous
horizontal nystagmus can be central or, more commonly, a result of peripheral
vestibular lesions. In peripheral vestibular horizontal nystagmus, the nystagmus
stays in one direction. That is, if it is spontaneous right-beating nystagmus, it
intensifies with gaze in the direction of the fast phase (to the right) and
diminishes or goes away with gaze to the left. Although clinicians test for this by
assessing the effect of gaze direction changes, it is still a form of spontaneous
nystagmus. When the nystagmus adheres to this pattern, referred to as the

CONTINUUMJOURNAL.COM 319

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

Alexander law, it indicates the nystagmus is of peripheral vestibular origin. When

the spontaneous nystagmus is right beating and changes to left beating with gaze
to the left, it is very likely central in origin.

GAZE-EVOKED NYSTAGMUS. Gaze-evoked nystagmus occurs when no spontaneous

nystagmus is present but when the patient gazes to the right, right-beating
nystagmus occurs, and when the patient looks to the left, left-beating nystagmus
occurs. Gaze-evoked nystagmus occurs when the gaze-holding mechanism
is impaired so that the elastic forces of the orbits draw the eyes to drift back
toward midline, which is then followed by a corrective saccade. For horizontal
gaze holding, the neural integrator is mediated by the nucleus prepositus
hypoglossi and medial vestibular nucleus, as well as by inputs from the
flocculonodular lobe of the cerebellum. For vertical and torsional gaze holding,
the interstitial nucleus of Cajal in the midbrain mediates gaze holding.
Gaze-evoked nystagmus is a CNS finding and should be distinguished from
so-called end point nystagmus. End point nystagmus occurs when the patient
attempts to look at the extremes of horizontal gaze, usually approximately
45 degrees from the vertical meridian, and a small degree of nystagmus occurs.22
Pathologic gaze-evoked nystagmus is usually evident by 30 degrees of eccentric
gaze or less.

POSITIONAL NYSTAGMUS. Positional nystagmus is key to diagnosing the most

common cause of recurrent vertigo, BPPV. In patients with dizziness or vertigo,
examination with Dix-Hallpike positioning elicits paroxysmal positional
nystagmus that is a combination of upbeating and torsional nystagmus with the
top pole of rotation (fast-phase) beating toward the downward ear, indicating
BPPV related to the posterior semicircular canal of the ear that is downward.
That is, for right posterior canal BPPV, the nystagmus is counterclockwise
from the examiner’s perspective (top pole nystagmus beats torsionally toward
the right ear) and upbeating from the patient’s perspective.
Positional testing may also result in horizontal nystagmus, which is most
commonly a variant of BPPV related to one of the lateral canals. Positional
downbeating nystagmus may be seen with variants of BPPV, but central
causes should be considered if it does not resolve with positioning treatments.
Static positional nystagmus is common and may be seen with video goggles
that allow visual fixation to be removed. Spontaneous static positional upbeating
nystagmus less than approximately 6 degrees per second is seen in vestibular
migraine but may occasionally occur in other conditions and so should not
be considered reliably localizing or indicative of a central versus peripheral
etiology.
Examination for vestibular function helps identify the absence or presence of
peripheral vestibular function on one or both sides (CASE 1-5).

Synthesis of History and Examination

Once relevant information from the history, observations, and examination is
compiled, the next steps are integration and discernment of patterns or possible
patterns to generate a differential diagnosis (TABLE 1-5).23-26 As already
mentioned, sometimes symptoms and signs are from two or more conditions
that cause dizziness or vertigo with features of both disorders at the same
time (TABLE 1-1).

320 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Laboratory Testing
Blood tests for vertigo are rarely helpful as a matter of routine but may be
indicated in some cases. Patients taking antiepileptic drugs that may account for
dizziness or abnormal eye movements may need drug levels assessed. For
patients with impaired balance, vitamin B12, methylmalonic acid, hemoglobin
A1c, and thyroid function studies may be warranted. For patients with possible
orthostatic dizziness, a complete blood cell count and comprehensive metabolic
panel may be ordered. In patients with bilateral fluctuating hearing with or
without vertigo and in whom luetic otitis or autoimmune inner ear disease is
suspected, an antinuclear antibody screen, erythrocyte sedimentation rate, and

A 48-year-old man was seen for recurrent falling and constant dizziness. CASE 1-5
His past medical history was notable for type 2 diabetes mellitus,
hypertension, and chronic renal failure due to autosomal dominant
polycystic kidney disease. He said the symptoms had been ongoing for
the past 4 months since he was hospitalized for peritonitis complicating
his peritoneal dialysis. He recalled developing some dizziness in the
hospital but was discharged to an extended care facility as antimicrobial
therapy was continued. When it came time for discharge, it was apparent
he could not walk well even with a cane, whereas he had been walking
independently before hospitalization.
An otolaryngologist found normal hearing on his audiogram and felt it
was not likely an otologic issue. Brain MRI was normal. The patient had 12
sessions of home physical therapy but still felt dizzy and off balance.
When standing, he felt severely off balance, saying, “I don’t know where I
am in space,” but this improved if he touched something with his hand.
When he walked or rode in a car, his vision jostled and he had trouble
focusing until he stopped moving. He had no nausea or spinning
sensation, and he was not aware of much change in his hearing.
Examination revealed evidence for neuropathy to the midlegs. His head
impulse test was abnormal to the right and left. No nystagmus was
detected. Dynamic visual acuity was impaired. Romberg sign was
consistently positive. Bilateral vestibulopathy was suspected and
confirmed by videonystagmography that showed severely reduced
caloric vestibular responses (summed caloric responses of 11 degrees per
second).

This is a case in which the history and bedside examination strongly COMMENT
pointed to bilateral vestibulopathy, most likely caused by an
aminoglycoside such as gentamicin that was likely given as treatment of the
peritonitis. Gentamicin is more selectively vestibulotoxic, so hearing was
not significantly affected. This case also points out how having concurrent
neuropathy with attendant somatosensory loss severely reduces the ability
to walk because vestibular and somatosensory signals are important
sensory inputs for balance, so when the patient’s vision was removed by
closing his eyes, he consistently fell.

CONTINUUMJOURNAL.COM 321

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

TABLE 1-5 Clinical Features of Some Disorders Causing Dizziness and Vertigo

International
Classification
of Vestibular
Common dizziness/ Timing/ Associated Disorders
vertigo descriptions Onset duration Triggers features syndromea Disorder

Vestibular disorders

Spinning, rotating, Abrupt 5–60 seconds Tilting head Occasionally Episodic Benign
whirling, tilting, back, rolling nausea, vestibular paroxysmal
floating, or falling in bed, nystagmus with syndrome positional
straightening Dix-Hallpike vertigo
after bending test on
affected side

Spinning, whirling, Abrupt or Days to No reliable Worse with any Acute Vestibular
rotating, tilting evolving over weeks trigger, 15% head motion, vestibular neuritis
30 minutes with nausea, syndrome
with some antecedent direction-fixed
variability upper nystagmus
respiratory (early on),
infection abnormal head
symptoms impulse test to
the side
affected

Spinning, whirling, Abrupt or Days to No reliable Worse with any Acute Labyrinthitis
rotating, tilting evolving over weeks trigger, 15% head motion, vestibular
30 minutes with nausea, acute syndrome
with some antecedent unilateral
variability upper hearing loss,
respiratory direction-fixed
infection nystagmus
symptoms (early on),
abnormal head
impulse test to
the side
affected

Severe spinning, Abrupt or 30 minutes to No reliable Unilateral Episodic Ménière

whirling, rotating, evolving over 12 hours trigger in tinnitus and vestibular disease
imbalance 30 minutes most cases hearing loss syndrome
with some that may
variability fluctuate on
the affected
side; worse
during head
motion;
low-frequency
hearing loss on
the affected
side

CONTINUED ON PAGE 323

322 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CONTINUED FROM PAGE 322

International
Classification
of Vestibular
Common dizziness/ Timing/ Associated Disorders
vertigo descriptions Onset duration Triggers features syndromea Disorder

Reduced equilibrium, Usually Continuous No trigger Bilateral Chronic Bilateral

unsteadiness worse insidious, symptoms abnormal head vestibular vestibulopathy
during movements occasionally worse during impulse test, syndrome
more abrupt head positive
depending movements Romberg sign,
on or in darkness reduced
mechanism dynamic visual
acuity

Spinning, tilting, Insidious but Spinning, Sounds may Autophony, Episodic Superior canal
oscillopsia, floating occasionally tilting, trigger spells unilateral ear vestibular dehiscence
sometimes induced patients oscillopsia, of worse pressure or syndrome
by sounds23 describe a floating may symptoms fullness and
sensation of last seconds hearing
“popping” at to minutes reduction,
onset recurrently; tinnitus;
autophony,b occasionally
tinnitus, ear nystagmus can
fullness, and be induced by
hearing may noise or
be fairly vibration on
continuous examination

Hemodynamic
disorders

Near-faintness, Abrupt, Minutes, may Most events Pallor, Episodic Orthostatic

“about to pass out,” usually when be recurrent occur or are diaphoresis, vestibular dizziness
“lightheadedness”24 standing on standing; evoked when nausea, may syndrome (orthostatic
may upright culminate in intolerance,
culminate in syncope, orthostatic
syncope or symptoms hypotension)
abate in relieved by
minutes lying flat;
abnormal
orthostatic
heart rate/
blood pressure

CONTINUED ON PAGE 324

CONTINUUMJOURNAL.COM 323

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

CONTINUED FROM PAGE 323

International
Classification
of Vestibular
Common dizziness/ Timing/ Associated Disorders
vertigo descriptions Onset duration Triggers features syndromea Disorder
Central nervous
system and related
disorders

Spinning, tilting, Abrupt or Spinning, No reliable Migraine Episodic Vestibular

rocking, floating, more tilting, triggers headache vestibular migraine
visually induced gradual, rocking, history, syndrome
vertigo/dizziness, sometimes floating may periodic
motion sensitivity discrete vary from photophobia or
spells, brief quick phonophobia;
sometimes spins lasting a examination is
constant but few seconds usually normal
varying in recurrently to
intensity spells lasting
minutes to
much of the
day; visually
induced
vertigo/
dizziness,
motion
sensitivity
may be nearly
constant

Rocking, swaying, Often Often No trigger Sometimes less Chronic Persistent

floating, no spinning, insidious, constant, severe when vestibular postural
and minimal or no may ensue varying in distracted; syndrome perceptual
nausea25 after a intensity; minimally dizziness
vestibular occasionally affected by
disorder or comes and head
emotional goes for movements;
event hours or days disability and
at a time distress seem
out of
proportion to
normal
examination

Vertigo, poor Abrupt Usually No trigger Transient Acute Transient

equilibrium or ataxia resolves in nystagmus and vestibular ischemic
5–20 minutes ataxia but syndrome attack
resolve after
minutes

CONTINUED ON PAGE 325

324 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CONTINUED FROM PAGE 324

International
Classification
of Vestibular
Common dizziness/ Timing/ Associated Disorders
vertigo descriptions Onset duration Triggers features syndromea Disorder

Vertigo, poor Abrupt Continues, No trigger Nausea, poor Acute Stroke

equilibrium or ataxia may see some gait balance; vestibular
gradual possibly syndrome
improvement gaze-evoked
in days to nystagmus,
months diplopia,
depending on dysarthria,
size and normal head
location of impulse test,
infarction possibly
hemiataxia and
other central
nervous system
signs26,c

Impaired balance Usually Continuous No trigger Balance Chronic Cerebellar

worse with head insidious; for worsens with vestibular dizziness
movement, less abrupt onset degenerative fatigue, syndrome (from a variety
commonly with may occur and alcohol, of cerebellar
position changes27 with lesion-based sedation; disorders)
cerebellar cerebellar gaze-evoked or
stroke or disease vertical
hemorrhage nystagmus;
or with limb and
episodic truncal ataxia,
ataxias or dysarthria,
acute abnormal
cerebellitis pursuit, and
saccadic eye
movements

a
By using the International Classification of Vestibular Disorders construct of syndromes, the term vestibular does not necessarily mean the
conditions under each rubric have an actual vestibular mechanism of origin.
b
Autophony is the abnormally increased perception of one’s own internal body sounds (one’s own voice, blinking, heart sounds, etc).
c
Cerebellar stroke confined to the cerebellar tonsil, flocculus, nodulus, superior vermis, and cerebellar peduncles may present only with vertigo
and imbalance lacking other prominent signs.26

CONTINUUMJOURNAL.COM 325

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

tests for syphilis can be obtained. Patients presenting with ataxia should have a
test for anti–glutamic acid decarboxylase 65 (GAD65) antibodies and possibly
vitamin E level. Although anti-GAD65 antibodies are associated with stiff person
syndrome, in some people high levels of anti-GAD65 antibodies may manifest
primarily with cerebellar ataxia with imbalance and “dizziness,” oftentimes with
downbeating nystagmus that is amenable to treatment with immunotherapy (eg,
mycophenolate, cyclosporine, rituximab, cyclophosphamide). For anyone
suspected of having dizziness in association with Wernicke syndrome, assessment
and possible empiric treatment for this condition should be considered.

CASE 1-6 A 52-year-old woman was previously seen for benign positional vertigo
on and off for the past 4 years. She had recently seen the physical
therapist who treated her and said no benign paroxysmal positional
vertigo could be found at that time. The patient had recurrent, mostly
random spells of dizziness that did not seem clearly triggered by
anything, lasted a few minutes, and mild to severe wobbliness,
sometimes being so severe that she had to sit down or would fall. The last
severe spell occurred while standing in the produce section of a grocery
store, and she had to sit on the floor. It lasted 3 to 4 minutes, and she
became sweaty and felt a little queasy but denied a feeling of spinning.
She had been in good health and took only a thyroid supplement,
estrogen, and rosuvastatin. Recent head CT was normal, and ECG,
complete blood cell count, and comprehensive metabolic panel ordered
by her primary physician were all normal.
On examination, her sitting blood pressure was 96/62 mm Hg with a
heart rate of 93 beats/min; on standing, it was 94/63 mm Hg with a heart
rate of 96 beats/min, and she had no symptoms. The remainder of the
neurologic examination was normal, and cardiac auscultation was also
normal. Postural hypotension was suspected, and a tilt-table study was
performed. On the test, she had no change in heart rate or blood pressure
until 19 minutes of head-up tilt at which time she exhibited a systolic
blood pressure drop to 61 mm Hg, and she passed out while strapped to
the tilt table. Her blood pressure normalized soon after resumption to a
recumbent position.
She responded to fludrocortisone, liberalized dietary sodium intake,
and pyridostigmine. The pyridostigmine was successfully discontinued
after 6 weeks, and the episodes did not return. Over the subsequent year,
she was able to discontinue the fludrocortisone as well.

COMMENT This case illustrates delayed orthostatic hypotension, a nonvestibular

cause of recurrent dizziness. In this case, routine orthostatic vital signs did
not reveal immediate changes in blood pressure. Rather, the decline in
blood pressure occurred after a time in the upright position. The delay in
onset made the episodes seem entirely random. However, as she
described the circumstances of the episodes, they were all while she was
upright.

326 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Cardiac and Hemodynamic Testing KEY POINT
If cardiogenic near-syncope is suspected, an ECG, echocardiogram, Holter
● Delayed orthostatic
monitoring, and cardiology referral can be obtained. Tilt-table testing can be hypotension (having onset
helpful in patients with unexplained syncope but also in those with suspected beyond 3 minutes after
recurrent, unexplained near-syncopal dizziness. Patients in whom recurrent standing or head-up tilt) may
spells of dizziness occur mostly or exclusively when upright may have be missed by routine
orthostatic vital signs but
dizziness caused by orthostatic hypotension. Delayed orthostatic hypotension
can be detected by a
(defined as having onset beyond 3 minutes of standing or head-up tilt) may be tilt-table test.
missed by routine orthostatic vital signs but can be detected by a tilt-table test.
In one study, only 46% of patients demonstrated orthostatic hypotension
within the first 3 minutes, and 39% did not exhibit orthostatic hypotension
until more than 10 minutes of head-up tilt,28 so a tilt-table study for this
indication should assess vital signs and symptoms for 30 minutes. Nearly
one-third of patients who have delayed orthostatic hypotension have a 10-year
mortality of approximately 29%, and nearly one-third eventually develop
an α-synucleinopathy such as Parkinson disease, dementia with Lewy bodies,
or multiple system atrophy (CASE 1-6).29

Vestibular Testing
Vestibular testing is performed to determine the functionality and integrity of
the peripheral vestibular apparatus and pathways, so it should generally be done
to confirm or refute a hypothesis. Of course, like many other tests in medicine, it
can be sometimes helpful in situations in which the cause is very uncertain to at
least ascertain that the vestibular structures and reflexes remain intact. The role
of diagnostic vestibular testing is discussed in detail in the article “Vestibular
Testing” by Timothy C. Hain, MD, and Marcello Cherchi, MD, PhD, FAAN,16 in
this issue of Continuum.

Imaging Studies
Imaging may be indicated when the cause of dizziness is uncertain or the
examination reveals findings of CNS dysfunction. For patients with dizziness
and vertigo, a noncontrast head CT has a very low yield of identifying a cause
when patients with headache, trauma to the head and neck, altered mental
status, focal neurologic deficits, or recent head or neck surgery are excluded. In
one prospective analysis of patients presenting to an emergency department with
dizziness, none of the 200 studies found a causative lesion, and head CT in this
setting was deemed not cost effective.30 Temporal bone CT is indicated to
identify lesions such as cholesteatoma or lesions within the labyrinth, including
canal dehiscence. Brain MRI without contrast is a reasonable first step, and
MRI with and without contrast is warranted if a vestibular schwannoma or
other structural lesion of the cerebellopontine angle is a consideration.31 Head
and neck CT angiography (CTA) or head and neck magnetic resonance
angiography (MRA) may be appropriate when dizziness or vertigo may have a
vascular cause.

CONCLUSION
Dizziness and vertigo by newer definitions are distinct and separate descriptors
(TABLE 1-2). Vertigo is not considered a subset of dizziness but a separate
symptom descriptor that indicates illusory motion. Dizziness describes a feeling

CONTINUUMJOURNAL.COM 327

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO AND DIZZINESS

of impaired spatial orientation without the illusion of motion. The causes of

dizziness and vertigo are many, but peripheral vestibular disorders are common.
A thorough history is paramount in making a diagnosis, and, in most cases, the
examination and laboratory testing are confirmatory rather than primary
diagnostic tools. All aspects of the patient’s history have importance, and
no reliably relayed part of the history should be ignored. Key examination focus
points include eye movements and especially observations of nystagmus and
bedside tests of vestibular function and symmetry. Derivation of a logical
differential diagnosis requires recognizing patterns or near patterns in the
clinical history, which is something neurologists do routinely. The following
articles in this issue guide the evaluation of this group of patients, many of whom
can enjoy substantial improvement in their quality of life by proper evaluation
and treatment.

REFERENCES

1 Murdin L, Schilder AGM. Epidemiology of 10 Reilly BM. Dizziness. In: Walker HK, Hall WD, Hurst
balance symptoms and disorders in the JW, editors. Clinical methods: the history,
community: a systematic review. Otol Neurotol physical, and laboratory examinations. 3rd ed.
2015;36(3):387-392. doi:10.1097/ Boston: Butterworths, 1990.
MAO.0000000000000691
11 Kerber KA. Episodic positional dizziness.
2 Neuhauser HK, von Brevern M, Radtke A, et al. Continuum (Minneap Minn) 2021;
Epidemiology of vestibular vertigo: a 27(2, Neuro-otology):348-368.
neurotologic survey of the general population.
12 Lacy JW, Stark CEL. The neuroscience of
Neurology 2005;65(6):898-904. doi:10.1212/01.
memory: implications for the courtroom. Nat Rev
wnl.0000175987.59991.3d
Neurosci 2013;14(9):649-658. doi:10.1038/nrn3563
3 Agus S, Benecke H, Thum C, Strupp M. Clinical
13 Ohm F, Vogel D, Sehner S, et al. Details acquired
and demographic features of vertigo: findings
from medical history and patients' experience of
from the REVERT Registry. Front Neurol 2013;
empathy—two sides of the same coin. BMC Med
4:48. doi:10.3389/fneur.2013.00048
Educ 2013;13:67. doi:10.1186/1472-6920-13-67
4 Muelleman T, Shew M, Subbarayan R, et al.
14 Newman-Toker DE, Cannon LM, Stofferahn ME,
Epidemiology of dizzy patient population in a
et al. Imprecision in patient reports of dizziness
neurotology clinic and predictors of peripheral
symptom quality: a cross-sectional study
etiology. Otol Neurotol 2017;38(6):870-875.
conducted in an acute care setting. Mayo Clin
doi:10.1097/MAO.0000000000001429
Proc 2007;82(11):1329-1340. doi:10.4065/82.11.1329
5 To-Alemanji J, Ryan C, Schubert MC. Experiences
15 Wieling W, Thijs RD, van Dijk N, et al. Symptoms
engaging healthcare when dizzy. Otol Neurotol
and signs of syncope: a review of the link
2016;37(8):1122-1127. doi:10.1097/MAO.
between physiology and clinical clues. Brain
0000000000001145
2009;132(pt 10):2630-2642. doi:10.1093/
6 Bisdorff A, von Brevern M, Lempert T, Newman- brain/awp179
Toker DE. Classification of vestibular symptoms:
16 Hain TC, Cherchi M. Vestibular testing. Continuum
towards an international classification of
(Minneap Minn) 2021;27(2, Neuro-otology):330-347.
vestibular disorders. J Vestib Res 2009;19(1-2):
1-13. doi:10.3233/VES-2009-0343 17 Kim SC, Kim JY, Lee HN, et al. A quantitative
analysis of gait patterns in vestibular neuritis
7 Newman-Toker DE, Edlow JA. TiTrATE: a novel
patients using gyroscope sensor and a
approach to diagnosing acute dizziness and
continuous walking protocol. J Neuroeng Rehabil
vertigo. Neurol Clin 2015;33(3):577-599.
2014;11:58. doi:10.1186/1743-0003-11-58
doi:10.1016/j.ncl.2015.04.011
18 Allum JHJ, Honegger F. Recovery times of stance
8 Edlow JA, Gurley KL, Newman-Toker DE. A new
and gait balance control after an acute unilateral
diagnostic approach to the adult patient with
peripheral vestibular deficit. J Vestib Res 2016;
acute dizziness. J Emerg Med 2018;54(4):469-483.
25(5-6):219-231. doi:10.3233/VES-150561
doi:10.1016/j.jemermed.2017.12.024
19 Eggers SDZ, Kattah JC. Approaching acute
9 Bisdorff AR, Staab JP, Newman-Toker DE.
vertigo with diplopia: a rare skew deviation in
Overview of the international classification of
vestibular neuritis. Mayo Clin Proc Innov Qual
vestibular disorders. Neurol Clin 2015;33:541-550.
Outcomes 2020;4(2):216-222. doi:10.1016/j.
doi:10.1016/j.ncl.2015.04.010
mayocpiqo.2019.12.003

328 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

20 Steenerson KK. Acute vestibular syndrome. 26 Kim SH, Kim HJ, Kim JS. Isolated vestibular
Continuum (Minneap Minn) 2021; syndromes due to brainstem and cerebellar
27(2, Neuro-otology):402-419. lesions. J Neurol 2017;264(suppl 1):63-69.
doi:10.1007/s00415-017-8455-6
21 Eggers SDZ, Bisdorff A, von Brevern M, et al.
Classification of vestibular signs and examination 27 Zwergal A, Feil K, Schniepp R, Strupp M.
techniques: nystagmus and nystagmus-like Cerebellar dizziness and vertigo: etiologies,
movements. J Vestib Res 2019;29(2-3):57-87. diagnostic assessment, and treatment. Semin Neurol
2020;40(1):87-96. doi:10.1055/s-0039-3400315
22 Shallo-Hoffmann J, Schwarze H, Simonsz HJ,
Mühlendyck H. A reexamination of end-point 28 Gibbons CH, Freeman R. Delayed orthostatic
and rebound nystagmus in normals. Invest hypotension: a frequent cause of orthostatic
Ophthalmol Vis Sci 1990;31(2):388-392. intolerance. Neurology 2006;67(1):28-32.
doi:10.1212/01.wnl.0000223828.28215.0b
23 Naert L, Van de Berg R, Van de Heyning P, et al.
Aggregating the symptoms of superior 29 Gibbons CH, Freeman R. Clinical implications of
semicircular canal dehiscence syndrome. delayed orthostatic hypotension: a 10-year
Laryngoscope 2018;128(8):1932-1938. doi:10.1002/ follow-up study. Neurology 2015;85(16):1362-1367.
lary.27062 doi:10.1212/WNL.0000000000002030
24 Stewart JM, Clarke D. “He's dizzy when he stands 30 Wasay M, Dubey N, Bakshi R. Dizziness and yield
up.” An introduction to initial orthostatic of emergency head CT scan: is it cost effective?
hypotension. J Pediatr 2011;158(3):499-504. Emerg Med J 2005;22(4):312. doi:10.1136/
doi:10.1016/j.jpeds.2010.09.004 emj.2003.012765
25 Holle D, Schulte-Steinberg B, Wurthmann S, et al. 31 Expert Panel on Neurologic Imaging; Sharma A,
Persistent postural-perceptual dizziness: a Kirsch CFE, et al. ACR Appropriateness Criteria®
matter of higher, central dysfunction? PLoS One hearing loss and/or vertigo. J Am Coll Radiol
2015;10(11):e0142468. doi:10.1371/journal. 2018;15(11S):S321-S331. doi:10.1016/j.
pone.0142468 jacr.2018.09.020

CONTINUUMJOURNAL.COM 329

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 REVIEW ARTICLE


Vestibular Testing
C O N T I N UU M A UD I O By Timothy C. Hain, MD; Marcello Cherchi, MD, PhD, FAAN
I NT E R V I E W A V AI L A B L E
ONLINE


Downloaded from http://journals.lww.com/continuum by wqLe/H3/oM2vx1Qs5A5qMemKKHLPqE4qkmGNpdheG3ikf1o85ttJSkFL2oTkgOkhMEmawHvsuTTE5xJG2ZjqccytIAhVn2qegy060KLcKJuPZsx5htaYiopRjp2zor2egUzWJAEWYNrnNdb1tgu/p0M6EMGzm2Aj on 04/28/2022

VIDEO CONTENT
ABSTRACT
A VA I L A B L E O N L I N E PURPOSE OF REVIEW: Vestibular testing, both at the bedside and in the
laboratory, is often critical in diagnosing patients with symptoms of
vertigo, dizziness, unsteadiness, and oscillopsia. This article introduces
readers to core concepts, as well as recent advances, in bedside and
instrumented vestibular assessments.

RECENT FINDINGS: Vestibular testing has improved immensely in the past

2 decades. While history and bedside testing is still the primary method of
differential diagnosis in patients with dizziness, advances in technology
such as the ocular vestibular-evoked myogenic potential test for superior
canal dehiscence and the video head impulse test for vestibular neuritis
have capabilities that go far beyond the bedside examination. Current
vestibular testing now allows clinicians to test all five vestibular sensors in
the inner ear.

SUMMARY: Contemporary vestibular testing technology can now assess the

entire vestibular periphery. Relatively subtle conditions, such as superior
canal dehiscence or a subtle vestibular neuritis, can now be diagnosed
with far greater certainty.

INTRODUCTION

V
estibular testing is defined as the quantification of the function of
CITE AS:
CONTINUUM (MINNEAP MINN) the motion-sensing portions of the inner ear (semicircular canals
2021;27(2, NEURO-OTOLOGY): and otoliths). Vestibular testing is generally performed in the
330–347.
context of an evaluation of the symptom of dizziness, and such
Address correspondence to
evaluations often benefit from information about hearing.
Dr Marcello Cherchi, 645 N Accordingly, although this article mainly focuses on vestibular assessments, it also
Michigan Ave, Chicago, IL 60611, includes content about how hearing testing contributes to forming a diagnosis.
[email protected].
edu.
Vestibular testing has improved immensely over the past 30 years. Five
motion sensors are located in each inner ear: three semicircular canals and two
RELATIONSHIP DISCLOSURE: otolith organs (the utricle and saccule). Ideally, one should be able to quantify the
Dr Hain has served as an
associate editor for Audiology function of all five.
and Neurotology. Dr Cherchi In 1914, Robert Bárány was awarded the Nobel Prize in Physiology or
reports no disclosures.
Medicine for the development of a test of the lateral semicircular canal.1 Since
UNLABELED USE OF then, and especially in the past decade, new tests have come into clinical use that
PRODUCTS/INVESTIGATIONAL can quantify the remaining two canals (anterior/superior and posterior), as well
USE DISCLOSURE:
Drs Hain and Cherchi report
as both otolith organs.
no disclosures. The main goal of vestibular testing is to determine whether vestibular function
is normal or abnormal, testing in each sensor of the peripheral vestibular
© 2021 American Academy apparatus, which may identify when central vestibular and ocular motor
of Neurology. pathways exhibit dysfunction. For example, if examination identifies subtle

330 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

spontaneous downbeating nystagmus but testing shows normal canal and otolith KEY POINTS
function, then this would suggest that peripheral vestibular function is intact and
● The function of all five
the observed nystagmus must therefore be arising from a central vestibular vestibular sensors in the
abnormality. inner ear, including the
otolith organs (saccule and
BEDSIDE TESTING utricle) and all three
semicircular canals, can now
Dizziness has numerous causes, many of which are unrelated to the peripheral
be tested.
vestibular system (eg, blood pressure fluctuations). A thorough neurologic
history and general neurologic evaluation are desirable. The approach to the ● The assessment of the
patient with dizziness when time does not permit a complete neurologic balance of a patient with
evaluation has been discussed elsewhere2 and is discussed in the article dizziness starts when the
patient is met in the waiting
“Approach to the History and Evaluation of Vertigo and Dizziness” by Terry D. room and walked to the
Fife, MD, FAAN, FANS,3 in this issue of Continuum. examination room.
In this section, the repertoire of relevant bedside tests is briefly described.

Balance Assessment
The goal of the assessment of balance is to quantify imbalance, look for
inconsistency, and to separate vestibular patterns of imbalance from other
neurologic problems, such as cerebellar ataxia, sensory loss in the feet,
movement disorders, and simulated unsteadiness.

GAIT OBSERVATION. The assessment of the balance of a patient with dizziness starts
when the patient is met in the waiting room and walked to the examination
room. Informal observations should be made concerning how the patients arise
from their chair, as well as how they lower themselves into the examination room
chair, their speed of locomotion, whether they swing their arms, and whether
they have a wide-based gait or use the wall or a caregiver’s arm to steady
themselves. To screen for a functional disorder, it is helpful to compare informal
observation and formal balance testing such as the Romberg test, which is
described in the following section. Most patients with acute vestibular problems
are unsteady, and most patients with chronic vestibular problems are not
unsteady. Inconsistencies should be noted.

EYES-CLOSED TANDEM ROMBERG TEST. The tandem Romberg test, also referred to as
sharpened Romberg, is quick and useful, albeit also nonspecific and somewhat
insensitive.4 It is a test for sensory ataxia. Borderline normal performance
consists of the ability to stand heel-to-toe, with eyes closed, for 6 to 30 seconds.
The test can be made easier and thus quantified to some extent by allowing the
eyes to be open or by allowing the feet to be in parallel but next to one another
(standard Romberg test) rather than in tandem. Variants of the test involve
standing on a foam pad, rather than in tandem, or on a narrow rail.5,6
High-normal performance, defined as the time before a step is required to
prevent a fall, is generally found in young adults, who can often perform the
eyes-closed tandem variant of the Romberg test for 30 seconds. Performance
declines greatly with age, especially in patients in their seventies and older.6
Many middle-aged patients with chronic inner ear disorders will have no
difficulty standing in tandem with their eyes open, but they may need to take a
step before 6 seconds passes with eyes closed.
It is helpful to develop a judgment of how much impairment of the Romberg
test is appropriate for a given degree of ear injury. Patients with bilateral

CONTINUUMJOURNAL.COM 331

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VESTIBULAR TESTING

vestibular loss have moderate ataxia; they rely heavily on their vision and are
unsteady when their eyes are closed when standing with a narrow base, whether
together in parallel or tandem. Most patients with substantial bilateral vestibular
loss cannot stand in the eyes-closed tandem Romberg test for 6 seconds. Patients
with bilateral vestibular deficits with an additional superimposed position sense
deficit, such as peripheral neuropathy, lose balance when standing with a
narrowed base even with eyes open. Patients with chronic unilateral vestibular
loss are only mildly ataxic, and they usually perform normally on the eyes-closed
tandem Romberg test. Patients with acute unilateral vestibular hypofunction
with nystagmus may be much more off balance but can adapt in weeks to a few
months to show fairly normal balance.

THE FUKUDA STEPPING TEST. The Fukuda stepping test (FIGURE 2-1) and assessment
for past-pointing are measurements of vestibulospinal function. They are rarely
used in contemporary clinical practice. In the Fukuda stepping test, the patient is
asked to march in place with eyes closed for approximately 30 seconds; and the
clinician then notes rotation and translation on a calibrated mat.7 The Fukuda
stepping test fell from popularity after it was shown that it has very wide
variability in subjects without balance problems. Honaker and Shepard8
concluded, “Overall, the [Fukuda stepping test] provides little benefit to
clinicians when used in the vestibular bedside examination.”
The past-pointing test is also called the Quix test.9 During the test, the patient
and examiner assume mirror-image postures with outstretched hands so that the
fingers almost touch, and the examiner assesses whether the patient’s fingers drift
after their eyes are closed. The Quix test has not undergone rigorous scrutiny in the
literature, and it is rarely used. Practically, much stronger tools are available to
detect vestibular imbalance based on nystagmus, ie, Frenzel goggle testing.

Hearing Assessment at the Bedside

Because many vestibular conditions share
an underlying pathology with the hearing
apparatus, it is prudent to examine hearing
and evaluate the status of the external ear
and tympanic membrane. High-frequency
hearing can be screened quickly with the
rubbed-fingers test, during which
examiners use their own hearing as a
control. Young patients should be able to
perceive the finger rubbing at an arm’s
length, whereas many older patients
cannot hear the rubbing sound until it gets
as close as 0.3 m (1 ft). Tuning fork tests
can also be used for a similar purpose, but
formal audiometry is the preferred next
step if the patient fails the finger-rub test.
FIGURE 2-1 Low-frequency hearing loss is not well
The Fukuda test. Patients march in assessed with the finger-rub test, and
place for 30 seconds with their eyes
closed. The amount of rotation and
patients with Ménière disease, for
translation is documented and example, may pass this bedside test. In
interpreted from markings on the mat. other words, one cannot avoid ordering

332 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

audiometry even if the finger-rub test is normal if Ménière disease is a possible KEY POINTS
diagnosis.
● Frenzel goggles are
Otoscopy can determine if the ear canal is occluded by cerumen, if a critical to the rapid and
perforation or scarring of the tympanic membrane has occurred, or if a mass is efficient evaluation of
present as may be seen with a cholesteatoma or glomus tumor. patients with dizziness
Finally, some bedside maneuvers are useful in patients with tinnitus, which because they improve the
clinician’s ability to detect
often accompanies dizziness. Rarely, objective tinnitus deriving from the tensor
vestibular nystagmus.
tympani muscle of the tympanic membrane can be diagnosed by careful
observation for intermittent dimpling of the eardrum during otoscopy. In ● Vestibular spontaneous
addition, rarely in patients with pulsatile tinnitus, the examiner will be able to nystagmus is suppressed by
hear the high-flow bruit of a dural arteriovenous malformation with a fixation.

stethoscope. For more information about tinnitus, refer to the article, “Tinnitus,
Hyperacusis, Otalgia, and Hearing Loss” by Terry D. Fife, MD, FAAN, FANS,
and Roksolyana Tourkevich, MD,10 in this issue of Continuum.

Nystagmus Testing
Evaluation of nystagmus is very useful in a patient with dizziness. Optimally, this
requires the use of Frenzel goggles (FIGURE 2-2), which are worn by the patient to
reduce fixation, as well as to magnify the examiner’s view of the patient’s eyes.
Frenzel goggles are useful because most inner ear causes of dizziness produce
nystagmus that can be suppressed by fixation. To see nystagmus roughly 1 week
after onset of an acute vestibular syndrome such as vestibular neuritis, the
patient’s eyes must not be allowed to fixate when being viewed.
Of the two available variants of Frenzel goggles (optical and infrared video),
the infrared video goggles are far superior, but the optical goggles are more
affordable. Without a method of viewing the eyes without fixation, some types
of nystagmus may not be
observable. The ophthalmoscope
can be used for making
inferences about spontaneous
nystagmus if Frenzel goggles are
not available; this is discussed in
more detail in the following
sections.

NYSTAGMUS ASSESSMENT TESTS

THAT DO NOT REQUIRE FRENZEL
GOGGLES. Some types of
nystagmus may be observable
even without Frenzel goggles.

SPONTANEOUS NYSTAGMUS. The

assessment for spontaneous
nystagmus is important for
diagnosing conditions
characterized by vestibular FIGURE 2-2
imbalance, such as vestibular Frenzel goggles. A, Optical Frenzel goggles, which
reduce fixation through the use of +20 diopter
neuritis. It should not be omitted lenses over the eyes. B, Video Frenzel goggles,
in the assessment of a patient which use infrared illumination to prevent fixation;
with dizziness. With either the image of the eye can be made very large.

CONTINUUMJOURNAL.COM 333

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VESTIBULAR TESTING

KEY POINTS Frenzel goggles placed on the

patient or using the
● Congenital nystagmus is
enhanced by fixation.
ophthalmoscope, the eyes are
observed for spontaneous
● The ophthalmoscope can nystagmus. FIGURE 2-3
be used to assess illustrates the two most common
spontaneous nystagmus if
types of spontaneous
Frenzel goggles are not
available. nystagmus. The nystagmus
typical of inner ear dysfunction
● The Alexander law can be is jerk nystagmus in primary
used to assess for gaze in which the eyes deviate
spontaneous jerk
nystagmus.
slowly (slow phase) in one
direction followed by a rapid
correction (fast phase) in the
opposite direction. The direction
of nystagmus is named for the
fast phase (VIDEO 2-1). The
more rapidly the eyes deviate off
center (the slow phase), the
more frequently the corrective FIGURE 2-3
Illustration of how the eyes move from side to side.
jerks (the fast phase) occur, By convention, the upward direction on the tracing
making the nystagmus appear corresponds to rightward eye movement, while the
faster. Most nystagmus of other downward direction on the tracing corresponds to
patterns (eg, pendular or leftward eye movement. Pendular nystagmus is
less common but may be seen in some central
saccadic) are of central origin nervous system causes or congenital nystagmus.
(VIDEO 2-2). For the most part, The far more common jerk nystagmus (in this case,
if a jerk-type nystagmus can be to the left because it is named after the fast phase)
detected without the use of some is typical of vestibular nystagmus.
method of blocking fixation, the
patient has either acute dizziness
or a disorder that impairs fixation, such as a cerebellar or brainstem problem. These
central cases are relatively rare compared with inner ear types of dizziness.
An exception to the preceding general rule is congenital nystagmus. Unlike
vestibular nystagmus, congenital nystagmus is often reduced by removal of
fixation, and this is one of the ways it can be recognized. The most common
congenital nystagmus is latent nystagmus, generally found in people with
congenital strabismus (CASE 2-1). Latent nystagmus is a jerk nystagmus that
changes direction according to the viewing eye (VIDEO 2-3). Patients with latent
nystagmus also have no stereovision and will fail bedside tests for stereopsis,
such as the Titmus Fly test.
When Frenzel goggles are not available, the ophthalmoscopic examination can
help with obtaining some information about spontaneous nystagmus. The examiner
monitors movement of the back of the eye while obscuring vision by covering the
other eye. As the back of the eye moves in the direction opposite of the front of the
eye, horizontally and vertically, the examiner must be sure to invert the direction of
the nystagmus when making notes. If an ophthalmoscope is also not available, the
presence of a unidirectional nystagmus can sometimes be inferred from an
evaluation of the effect of gaze nystagmus. According to the Alexander law,
vestibular nystagmus nearly always increases when the patient looks in the
direction of the fast phase, so an asymmetric frequency or intensity of nystagmus

334 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

will be seen depending on whether the patient looks to the right or left (although
the nystagmus does not change direction) in peripheral vestibular nystagmus.11

DIX-HALLPIKE TEST. Ideally, the Dix-Hallpike test is performed with Frenzel

goggles, optical or video (FIGURE 2-4), although in many cases, the nystagmus of
benign paroxysmal positional vertigo (BPPV) is seen readily with the naked eye.
The sensitivity of the test improves when performed with the goggles. For the
Dix-Hallpike test to the right (VIDEO 2-4), the patient’s head is turned 45 degrees
to the right and then the patient is moved quickly from the sitting position to this
head-hanging position. If the patient feels no dizziness or if nystagmus is not
appreciated after 15 seconds, then the patient is moved back up to the sitting
position. For the Dix-Hallpike test to the left (VIDEO 2-5), the patient’s head is
turned 45 degrees to the left and then the patient is quickly laid back to the
head-hanging left position. After 15 seconds, the patient is again moved to a
sitting position. This may be repeated if BPPV is still suspected based on history.
In a positive test, a burst of nystagmus is provoked by either the head-right or
the head-left position. Further information on BPPV is described in the article
“Episodic Positional Dizziness” by Kevin A. Kerber, MD, MS,12 in this issue
of Continuum.

A 50-year-old man was born with congenital esotropia; as a child, he CASE 2-1
frequently squinted and eventually was able to describe that he was
experiencing double vision, so at age 5 years he underwent surgery to
correct the esotropia.
Videonystagmography recordings (with the camera placed over the
right eye while the patient had initially viewed out of the left eye)
documented a spontaneous left-beating nystagmus; however, caloric
tests were normal. Because he had spontaneous nystagmus but no caloric
weakness, the audiologist concluded that the patient had a central
vestibular disorder.
In the office, the patient had no depth perception (stereopsis) as
determined by the Titmus Fly test in which the patient wore polarized
glasses while looking at a specially formatted picture of a housefly that
should appear to pop out from the page if depth perception is intact. The
patient also had amblyopia in the right eye and weak left-beating
horizontal nystagmus in the light. When the left eye was covered, the
nystagmus reversed direction and became right beating. The nystagmus
stopped in complete darkness (with the use of video Frenzel goggles).
Close inspection of the videonystagmography pursuit traces showed that
the eye actually tracked faster than the target to the left, and backup
saccades were present.

This case illustrates some of the examination findings in a patient with a COMMENT
type of congenital nystagmus called latent nystagmus, which often results
from congenital esotropia, and points out how some of the findings taken
in isolation can lead to an incorrect diagnosis.

CONTINUUMJOURNAL.COM 335

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VESTIBULAR TESTING

The most common type of positional

nystagmus, posterior canal BPPV, beats
upward and has a rotatory component,
such that the top part of the eye beats
toward the undermost ear (VIDEO 2-4 and
VIDEO 2-5). The nystagmus typically has a
latency of 2 to 5 seconds and lasts 5 to
60 seconds, and an unwinding
downbeat/rotatory nystagmus may be
seen when the patient is sat up again
(VIDEO 2-6). Variations of BPPV have
different vectors. The lateral canal variant
of BPPV is typified by a strong horizontal
nystagmus that reverses direction between
head left and right (VIDEO 2-7 and
VIDEO 2-8). The rare anterior canal variant
is associated with a downbeating
nystagmus elicited by the Dix-Hallpike
test. The Dix-Hallpike test is a high-yield
portion of the dizziness bedside
assessment and should almost never be
omitted. For more information on
positional vertigo, refer to the article,
“Episodic Positional Dizziness” by
Kevin A. Kerber, MD, MS,12 in this issue of
Continuum.

NYSTAGMUS ASSESSMENT TESTS THAT

REQUIRE FRENZEL GOGGLES. In general, the
following additional maneuvers should
not be used if Frenzel goggles are not
available. More detail about these
maneuvers can be found elsewhere.13

NECK-VIBRATION TEST.
The neck-vibration
FIGURE 2-4
Dix-Hallpike positional maneuver. To test (FIGURE 2-5) is very useful as a
precipitate the characteristic bedside test because it is a robust and
nystagmus of benign paroxysmal durable test for unilateral vestibular
positional vertigo, the patient is rapidly
brought from sitting (A) into a
weakness.14 In the neck-vibration test, the
head-hanging position (B and C) that patient’s eyes are observed in complete
makes the posterior canal vertical and darkness (ie, with video, not optical,
brings it through a large angular Frenzel goggles) while vibration
displacement.
(typically from a massaging device) is
applied to the sternocleidomastoid muscle
for 10 seconds. The vibration is applied first on one side and then on the other
(VIDEO 2-9). One looks for direction-fixed nystagmus (that is, it does not change
direction with changes in the direction of gaze) with fast-phase beating to the
side opposite the ear with vestibular hypofunction. The neck-vibration test
requires less expertise than the head impulse test (see the Head Impulse Test
section), requires almost no subjective judgment, and is not greatly affected by

336 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 the passage of time since the onset of the KEY POINT
lesion. Provided that video Frenzel goggle
● The neck-vibration test is
equipment is available, the neck- a sensitive and durable test
vibration test can be helpful in patients of unilateral vestibular
with suspected unilateral vestibular weakness.
hypofunction.

HEAD-SHAKE TEST. With the patient

wearing Frenzel goggles and sitting, the
patient's head is rotated by the examiner
in the horizontal plane for 20 cycles. The
examiner should aim for a 30- to
45-degree turn of the head to either side
(if the patient is safely able) and a
frequency of 2 cycles per second. A
nystagmus that lasts 5 seconds or longer
FIGURE 2-5 indicates an organic disorder of the ear or
Neck-vibration test. While the central nervous system and indicates
examiner applies vibration to the further investigation is warranted. The
sternocleidomastoid muscle, video
Frenzel goggles are used to detect
head-shake test is neither as reliable nor as
nystagmus. A positive test elicits strong durable as the neck-vibration test. False
unidirectional horizontal nystagmus positives are common.
from both sides.
VALSALVA TEST.
The Valsalva test is
optional and mainly performed if a
pressure or sound sensitivity symptom is elicited from the history. While
wearing the Frenzel goggles, the patient inhales a deep breath and strains for
10 seconds while the examiner observes for nystagmus. Nystagmus at the onset
and release of pressure indicates a positive test. The glottis can be open or closed.
The Valsalva test is used mainly to assess for superior canal dehiscence. Because
far more sensitive laboratory tests for superior canal dehiscence (ie,
vestibular-evoked myogenic potential) are available, performing this test at the
bedside has little benefit. It is also possible, in a small subset of patients with
superior canal dehiscence, to elicit nystagmus in response to sound. This is called
the Tullio test, but it is also highly insensitive and rarely performed.

HYPERVENTILATION TEST. The hyperventilation test is also optional. It is mainly

performed if the examination has been entirely normal or if a vestibular
schwannoma or other partial lesion of the vestibular nerve is suspected. The
patient takes 30 deep, forceful breaths. Immediately after hyperventilation and
with the use of the Frenzel goggles, the patient’s eyes are inspected for
nystagmus. Hyperventilation-induced nystagmus suggests a partially
conducting eighth nerve or central vestibular pathways caused by, for example,
a tumor of the eighth cranial nerve, gamma knife radiosurgery for vestibular
schwannoma, or multiple sclerosis. The hyperventilation test is both insensitive
and nonspecific.

VESTIBULO-OCULAR REFLEX GAIN TESTING

The vestibulo-ocular reflex mediates eye movements in response to head
rotation, such that eyes move equally in amplitude but in the direction opposite

CONTINUUMJOURNAL.COM 337

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VESTIBULAR TESTING

of the head rotation, which serves to stabilize the image of a viewed target on the
fovea during movement.

Head Impulse Test

The head impulse test is a bedside vestibular test, first described in 1988.15 It is
useful for detecting vestibular damage and most helpful in documenting
vestibular neuritis. The test requires no special equipment. Standing directly
in front of the patient, the examiner holds the patient’s head firmly on each
side and instructs the patient to look at a fixed point, usually the examiner’s
nose. The examiner then abruptly rotates the patient’s head rapidly but only a
small distance to the left and right (approximately 10 degrees); this brisk
rotation (the impulse) should be in a pattern that is unpredictable in the
timing and direction of the head turn; several impulses toward each side
should be assessed. In a person with an intact vestibular system, the
vestibulo-ocular reflex will keep the eyes on target and the patient will still be
looking at the examiner’s nose after the impulse. In a patient with a recent
unilateral vestibular deficit, especially involving the vestibular nerve, the
eyes move with the head (due to an impaired vestibulo-ocular reflex on the
side to which the head was turned), and this is followed by a corrective rapid
eye movement to bring the eyes back to the target (the examiner’s nose)
(CASE 2-2). The head impulse test is most useful when video Frenzel goggles
are not available. The next two maneuvers are aimed at documenting bilateral
vestibular loss.

Dynamic Illegible E Test

This is a test of dynamic visual acuity. Using an eye chart positioned at least
3 meters (10 feet) from the patient, preferably calibrated in LogMAR units
(FIGURE 2-6), the examiner records visual acuity while the patient’s head is still.
Then, the examiner gently turns the patient’s head 30 degrees to the left and right
at approximately 1 to 2 Hz and again records the visual acuity. Patients with no
loss of vestibular function drop from 0 to 2 lines per LogMAR of acuity with
turning of the head. Patients with complete or partial bilateral loss of vestibular

CASE 2-2 A 40-year-old man reported acute dizziness and unsteady gait and had
been vomiting for several hours. Although he was ambulatory, he was
unsteady and preferred to hold onto his wife’s shoulder as he was taken
from the waiting room to the examination room.
On examination, he could not stand with his eyes closed in a tandem
Romberg stance. He had a left-beating spontaneous nystagmus readily
seen in both eyes. The nystagmus increased on left gaze and stopped on
right gaze. His head impulse test was positive to the right and normal to
the left.

COMMENT This is the presentation of acute right-sided vestibular neuritis. This case
illustrates how a unilateral vestibular weakness presents with spontaneous
unidirectional horizontal nystagmus that obeys the Alexander law.

338 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 function drop from 3 to 7 lines
per LogMAR of acuity. Patients
with acute complete bilateral loss
generally can read only the top
line (ie, the F and P).

Ophthalmoscope Test
The ophthalmoscope test is used
to obtain objective corroboration
when a patient has a positive
dynamic illegible E test. The
examiner focuses on the optic
disk and then gently moves the
patient’s head as described for
the illegible E test. The vestibulo-
ocular reflex gain is abnormal if
the disk moves with the head, ie,
moves back and forth from the
examiner’s perspective.16 The
ophthalmoscope test is less
sensitive than the dynamic
FIGURE 2-6 illegible E test and should be
Dynamic illegible E test. This test of dynamic performed with the patient’s
visual acuity is performed with the examiner
spectacles on to avoid interaction
oscillating the patient’s head side to side about
the vertical axis at approximately 1 to 2 Hz; the with the effects of spectacles on
lowest line that can be read is ascertained. A vestibulo-ocular reflex gain.
decline in visual acuity before and during
movement of at least three lines is abnormal,
Hearing Testing
indicating bilateral vestibulopathy.
© 2007 Timothy C. Hain. TABLE 2-1 lists the indications for
common laboratory procedures
used for evaluating hearing in
patients with vertigo and dizziness. Not all of these tests are useful for every patient.
To be time- and cost-efficient, tests should be chosen according to each patient’s
specific set of symptoms (TABLE 2-2). For more information on hearing loss, refer to

Hearing-Related Laboratory Testing for Dizziness and Vertigo TABLE 2-1

Test Indications

Audiogram Hearing symptoms, dizziness, vertigo, Ménière disease suspected, superior canal
dehiscence suspected

Otoacoustic emissions Hearing symptoms, functional hearing loss

Brainstem auditory evoked Suspicion of vestibular schwannoma with no access to MRI

response

Electrocochleography Secondary test for Ménière disease

MRI = magnetic resonance imaging.

CONTINUUMJOURNAL.COM 339

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VESTIBULAR TESTING

the article, “Tinnitus, Hyperacusis, Otalgia, and Hearing Loss” by Terry D. Fife,
MD, FAAN, FANS, and Roksolyana Tourkevich, MD,10 in this issue of Continuum.
When hearing symptoms are present or when a disorder such as Ménière
disease or vestibular schwannoma is reasonably suspected, then an audiogram is
the most useful initial test. An audiogram is recommended even for patients
who have few symptoms of hearing loss because some patterns of hearing loss
cannot be determined at the bedside (eg, low-frequency hearing loss) and may
not be readily noticed by the patient. TABLE 2-2 outlines four common hearing
patterns that may be documented on audiometry. FIGURE 2-7 shows the typical
low-tone hearing loss seen in early Ménière disease.
It is incumbent for the clinician seeing patients with dizziness to recognize
each of the abnormal patterns above because they should trigger important
actions on the part of the clinician.

AUDIOGRAM. The audiogram is a subjective test (requiring cooperation from the

patient) that measures hearing and mainly tests the cochlea. Certain
abnormalities suggest otologic vertigo (TABLE 2-2). It is nearly always indicated
in patients with dizziness. Hearing declines symmetrically in both ears with
age, mainly at high pitches. In some cases, it is helpful to combine the audiogram
(a subjective test) with the otoacoustic emissions test (an objective test) to
look for inconsistency when factitious hearing loss is a concern.

OTOACOUSTIC EMISSIONS. Otoacoustic emissions testing is an objective test based

on registration of sound elicited from the inner ear itself in response to an external
sound. Otoacoustic emissions are a quick and simple automated procedure. In
newborns or others who cannot cooperate with formal audiometry, otoacoustic
emissions are valuable because, when present, they show that cochlear function is
present. Otoacoustic emissions are usually not helpful in people older than
60 years old because otoacoustic emissions are reduced with age. In adults younger
than 60 years, otoacoustic emissions as objective tests are useful in detecting
functional hearing loss through the inconsistency between otoacoustic emissions
and audiometry. For example, if a patient claims to be deaf on one side but has a
robust otoacoustic emission in that ear, functional hearing loss is a reasonable
possibility. Otoacoustic emissions can also be normal in central hearing deficits,
such as a brainstem or cortical site of hearing loss, but these cases are very rare.
Regarding functional hearing loss, a large assortment of audiologic procedures can
detect psychogenic hearing loss, including the excellent Stenger test. The Stenger
effect refers to the psychoacoustic phenomenon in which a tone, presented
simultaneously to both ears but with a greater intensity in one ear, will only be
perceived in the ear receiving the louder stimulus; the Stenger effect is leveraged
by the Stenger test to detect functional unilateral hearing loss.

TABLE 2-2 Common Audiometric Findings in Selected Disorders Causing Dizziness

◆ Ménière disease: unilateral low-frequency sensorineural loss at the onset of disease

◆ Vestibular schwannoma: unilateral progressive high-frequency sensorineural loss
◆ Superior canal dehiscence (conductive hyperacusis): bone conduction better than air
conduction at 500 Hz

340 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 BRAINSTEM AUDITORY EVOKED KEY POINTS
RESPONSE TESTING. Brainstem
● Hearing testing is critical
auditory evoked response, also to assess for Ménière
referred to as auditory brainstem disease and contributes
responses among audiologists, is an greatly to the diagnosis of a
evoked potential test measuring vestibular schwannoma.
brainstem responses to sound. In
● The combination of a
patients with dizziness, it is most subjective hearing test such
useful to detect vestibular as the audiogram with an
schwannomas. Brainstem auditory objective test such as
evoked response testing has fallen otoacoustic emissions can
help with the diagnosis of
out of favor because MRI testing functional hearing loss.
has far superior diagnostic
sensitivity and specificity.

ELECTROCOCHLEOGRAPHY.
FIGURE 2-7
An audiogram showing right-sided low-frequency Electrocochleography is an
sensorineural hearing loss. Low-frequency evoked potential test in which the
sensorineural hearing loss on the right side is recording electrode is positioned
depicted, which is fairly specific for right-sided
on the eardrum to get a better
Ménière disease. Here, X depicts hearing
definition of the cochlear potential
thresholds for the left ear, and O and the triangle
indicate thresholds for the right ear. from the inner ear. An abnormal
electrocochleogram may suggest
Ménière disease in patients with a
clinical history that is consistent with Ménière disease. Electrocochleography is
technically difficult and relatively unrewarding in diagnostic power and should
not be considered a useful screening test in all patients with vertigo. For these
reasons, electrocochleography has fallen out of favor and is not widely available.

Vestibular Laboratory Testing

Vestibular laboratory testing can now assess all five sensory organs in the inner
ear. Three tests—videonystagmography (VENG), rotary chair, and video head
impulse—assess the semicircular canals. VENG and rotary chair test only
horizontal canal function, whereas the video head impulse test can test all three
semicircular canals on each side. These tests are mainly helpful in patients
with dizziness when no clear diagnosis is evident after history and bedside
examination (TABLE 2-3). Two vestibular tests, cervical vestibular-evoked
myogenic potential and ocular vestibular-evoked myogenic potential, assess the
otolith organs (saccule and utricle, respectively). Vestibular tests are sometimes
unreliable and, to confirm abnormal results, it is often helpful to obtain several
independent measurements, usually entailing two different techniques (ie, video
head impulse test and VENG or rotary chair). One can also use a combination
of a good bedside examination (with video Frenzel goggles) and one or more
laboratory tests to increase reliability of the diagnostic process. More detail about
the older vestibular tests (ie, VENG and rotary chair) can be found in an
American Academy of Neurology (AAN) assessment of vestibular testing.17

VIDEONYSTAGMOGRAPHY. VENG is a battery of eye-movement recordings that can

identify vestibular asymmetry, such as that caused by vestibular neuritis, and
document spontaneous or positional nystagmus, such as that caused by BPPV.

CONTINUUMJOURNAL.COM 341

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VESTIBULAR TESTING

VENG includes a caloric test, wherein the vestibular system is stimulated by

warming or cooling the eardrum with water or air. VENG is a long and difficult
test, both for the patient and the technician, and patients sometimes call it the
“water torture test” because part of the test may induce vertigo and, in some
people, motion sickness.
Laboratories sometimes attempt to avoid the strong dizziness that can be
encountered in the caloric test by replacing water irrigation with air, resulting in
less stimulating and messy procedures but lower-quality data. False-positive
interpretations, especially “bilateral vestibular weakness,” are common if the
person performing the air caloric testing does not point the stream of warm or cool
air directly at the eardrum (CASE 2-3). The interpretation of “central vestibular
disturbance,” is sometimes applied to patients with a VENG finding outside of the
usual distribution found in inner ear disorders. For example, patients with weak
but measurable spontaneous upbeating or downbeating nystagmus may be given
the diagnosis of central vestibular disturbance and referred for neurologic
evaluation. Another source of testing error can be a partial cerumen impaction of
the ear being tested, which results in the false-positive diagnosis of unilateral
vestibular weakness. VENG results are usually interpreted by audiologists, whose
training mainly covers inner ear disorders; consequently, some audiologists
characterize as a “central finding” any results they do not recognize deriving from
an inner ear disturbance, leading to a neurology referral.
As is the case with other vestibular tests, an abnormal result that does not
fit the clinical picture should be supplemented by rotary chair testing or video
head impulse testing, ideally, in combination with vestibular-evoked myogenic
potential testing (see the following section). VENG remains the vestibular test of
choice but can be associated with some discomfort, including nausea and/or
headache, especially in patients with motion sickness or migraine. Similar clinical
information can be gained by combining the video head impulse test and bedside
nystagmus testing with video Frenzel goggles. It should be noted that, currently,
Centers for Medicare & Medicaid Services and many other insurance plans do
not cover video head impulse test procedures (TABLE 2-4).

VESTIBULAR-EVOKED MYOGENIC POTENTIAL. Vestibular-evoked myogenic potential

testing measures the function of the otolith organs (the utricle and saccule) and

TABLE 2-3 Vestibular Laboratory Tests (in Order of Usefulness)

Test Indication(s)
Videonystagmography (VENG) Vertigo

Video head impulse test (vHIT) Vertigo

Vestibular-evoked myogenic potential (cervical Vertigo, sound sensitivity, pulsatile tinnitus

[cVEMP] and ocular [oVEMP])

Rotary chair test (Rchair) Bilateral vestibular loss suspected, secondary test to confirm abnormal
caloric responses or video head impulse test suggesting unilateral or
bilateral vestibular loss

Posturography (CDP) Assess for functional disorder, assess fall risk

342 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

was mentioned briefly in the 2000 AAN vestibular assessment,17 but now it is
mainstream and fairly widely available. Vestibular-evoked myogenic potential
testing is sensitive to the superior semicircular canal dehiscence syndrome18 and
has a minor role in supporting the diagnosis of bilateral vestibular loss, vestibular
neuritis, and conductive hearing loss.
The two variants of the vestibular-evoked myogenic potential test most
commonly used in clinical practice are the ocular vestibular-evoked myogenic
potential and cervical vestibular-evoked myogenic potential. The ocular
vestibular-evoked myogenic potential quantifies utricular function, whereas the
cervical vestibular-evoked myogenic potential quantifies saccular function.
Vestibular-evoked myogenic potential testing is sensitive to the superior
semicircular canal dehiscence syndrome. Either the ocular or cervical test may
show larger than normal responses and lower than normal sound thresholds on
the affected side(s). While thin-slice CT scans of the temporal bone images
document dehiscence, this procedure both exposes the patient to radiation as
well as may be falsely positive, as asymptomatic dehiscence is found in about 1%
of the population.19 The combination of a visible dehiscence (abnormal opening
of the bony canal) on CT of the temporal bone with abnormal vestibular-evoked
myogenic potential responses (either cervical or ocular) strongly points to the
diagnosis of superior semicircular canal dehiscence syndrome. For more
information on superior canal dehiscence syndrome, refer to the article,
“Selected Otologic Disorders Causing Dizziness” by Gail Ishiyama, MD,20 in this
issue of Continuum.

A 70-year-old woman underwent videonystagmography for evaluation of CASE 2-3

chronic dizziness. The air caloric testing technique was used. Warm air
caloric stimulation was applied in each ear sequentially, then cool air
caloric stimulation was applied in each ear sequentially; the total response
(sum of the peak slow-phase velocity responses from all four caloric
stimuli, warm and cool in each ear) was only 8 degrees per second whereas
normal is between 20 and 100 degrees per second. The patient was
referred for a neurologic evaluation for potential bilateral vestibular loss.
On examination, she could stand in the tandem Romberg stance with her
eyes closed and had no loss of visual acuity on the dynamic illegible E test.
Her ophthalmoscope and head impulse tests were normal.

This case illustrates a discrepancy between clinical examination and an COMMENT

inadequate vestibular test. On physical examination, the facts that the
patient could maintain a tandem Romberg stance with her eyes closed and
she had normal performance on dynamic illegible E testing are
incompatible with a diagnosis of bilateral vestibular loss. In contrast, air
caloric testing is very dependent on pointing the column of air directly at
the tympanic membrane to properly evoke good caloric vestibular
responses. The conclusion of “bilateral vestibular loss” was a false-positive
result.

CONTINUUMJOURNAL.COM 343

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VESTIBULAR TESTING

Vestibular-evoked myogenic potential responses, like balance, normally

decline with advancing age but generally are repeatable and stable. Cervical
vestibular-evoked myogenic potentials (whose pathway travels through the
inferior division of the vestibular nerve) are often normal in patients with
vestibular neuritis (which more commonly affects the superior division of the
vestibular nerve), and thus can be helpful in making this diagnosis.21
Vestibular-evoked myogenic potential testing now has a Current Procedural
Terminology (CPT) code (TABLE 2-4).

ROTARY CHAIR. Rotary chair testing measures vestibular function of both inner ears
together. It is sensitive to bilateral loss of vestibular function and performs better
than VENG for this purpose.17 In unilateral vestibular loss, rotary chair testing is
sensitive but nonspecific because it is poor at identifying the side of the lesion.

TABLE 2-4 Common Vestibular and Auditory Tests and Their Corresponding CPT Codesa

Test CPT code

Comprehensive audiometry threshold evaluation and speech recognition 92557
(92553 and 92556 combined)

Auditory evoked potentials; screening of auditory potential with broadband 92635

stimuli, automated analysis neurodiagnostic, with interpretation and report

Caloric vestibular test with recording, bilateral; bithermal (ie, one warm and 92537
one cool irrigation in each ear for a total of four irrigations)

Electrocochleography 92584

Distortion product evoked otoacoustic emissions; limited evaluation (to 92587

confirm the presence or absence of hearing disorder, 3-6 frequencies) or
transient evoked otoacoustic emissions, with interpretation and report

Distortion product evoked otoacoustic emissions; comprehensive diagnostic 92588

evaluation (quantitative analysis of outer hair cell function by cochlear
mapping, minimum of 12 frequencies), with interpretation and report

Computerized dynamic posturography sensory organization test (CDP-SOT), 92548

6 conditions (ie, eyes open, eyes closed, visual sway, platform sway, eyes
closed platform sway, platform and visual sway), including interpretation
and report

Vestibular-evoked myogenic potential (VEMP) testing, with interpretation 92517

and report; cervical (cVEMP)

Vestibular-evoked myogenic potential (VEMP) testing, with interpretation 92518

and report; ocular (oVEMP)

Vestibular-evoked myogenic potential (VEMP) testing, with interpretation 92519

and report; cervical (cVEMP) and ocular (oVEMP)

Video head impulse test None; bill as 92700 and have patient
sign an advanced beneficiary notice

Sinusoidal vertical axis rotational testing 92546

a
Current Procedural Terminology (CPT) codes maintained by the American Medical Association and approved by the Centers for Medicare and
Medicaid Services in the United States as of January 1, 2021.
CPT © 2021 American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.

344 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Rotary chair testing is most useful when caloric vestibular testing done as part of KEY POINT
VENG falsely suggests that vestibular responses are reduced or absent on both sides
● False-positive
because of small ear canals, cerumen in the external canal, or inadequate irrigation videonystagmography
technique. Rotary chair testing can clarify matters because it does not determine findings of bilateral
vestibulo-ocular reflex function by caloric stimulation via the external ear canal vestibular weakness or
but rather by actual movement of the head. Rotary chair testing is almost always central vestibular
disturbance are common
covered by health insurance, but it requires costly equipment. In addition, rotary
sources of referrals to
chair testing requires considerable technician and patient time to perform the test. neurologists.

VIDEO HEAD IMPULSE TEST. This recently available vestibular test can quickly diagnose
both severe bilateral vestibular loss and complete unilateral vestibular loss, especially
when due to vestibular nerve injury. The video head impulse test is less sensitive
to vestibular damage due to hair cell disease, such as Ménière disease, whereas
VENG testing is more accurate. The video head impulse test is more resistant to
false-positive findings in patients with functional symptoms than tests of
nystagmus such as VENG and the rotary chair.
Variants of the video head impulse test purport to measure function of the
posterior and anterior semicircular canals, but they should not be relied on for
this because current commercially available eye-movement monitoring technology
is not able to quantify the entire three-dimensional vector of eye movement. As
a consequence, the results of video head impulse tests done for the anterior or
posterior canal planes are often puzzling. Despite the high utility of video head
impulse testing, many insurances do not currently cover it.

POSTUROGRAPHY. This discussion specifically refers to the computerized dynamic

posturography device. Posturography attempts to evaluate vestibular,
proprioceptive, and visual contributions to balance and is similar to the Romberg
test. It requires cooperation from the patient. Posturography involves a series of
six testing conditions, progressing through permutations of normal/absent/
misleading visual input and normal/misleading proprioceptive input. The series
of conditions become gradually more difficult, and a patient’s performance
should decline with progressively more difficult conditions. However, if the
conditions are presented in a random order, and if the patient performs normally
on difficult conditions but poorly in easy conditions, then such inconsistency22
may raise suspicion for a functional disorder. Posturography is also a method of
assessing fall risk and is usually reimbursed by Medicare insurance (TABLE 2-4),
but often it is not covered by other types of insurance.

SUMMARY OF THE ROLE OF VESTIBULAR TESTING. In patients with dizziness,

vestibular testing assists in the diagnostic process by supplementing the clinical
history and examination. Vestibular tests can usually identify unilateral
vestibular loss, bilateral vestibular loss, and superior canal dehiscence. When
combined with hearing testing, they can assist in the identification of Ménière
disease. When comprehensive vestibular testing is all normal, it goes a long way
toward excluding primary peripheral vestibular disorders that leave persisting
dysfunction. Most other conditions, including vestibular migraine, dizziness
related to anxiety, dizziness from cardiovascular disturbances (eg, orthostatic
hypotension), and conditions such as persistent postural perceptual dizziness
have normal results on vestibular assessments at the bedside and in the
laboratory. In these cases, vestibular tests may assist the clinician in avoiding

CONTINUUMJOURNAL.COM 345

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VESTIBULAR TESTING

fruitless management strategies, such as vestibular-suppressant medication or

vestibular rehabilitation for patients who have no vestibular disturbance.

CODING OF VESTIBULAR TESTING. Bedside testing (without instrumentation and

recording), such as the Dix-Hallpike test for BPPV or the bedside head impulse
test for unilateral and bilateral vestibular loss, has no billing code that is
reimbursed in the United States. Instrumented testing, often involving the same
basic process as bedside testing, but with a recording, is usually reimbursed by
insurance (TABLE 2-4).

CONCLUSION
For a clinician to diagnose a patient with dizziness, a careful history and an
examination that includes specific bedside vestibular tests are crucial. In some
instances, this may need to be supplemented with audiometric tests and with
instrumented vestibular testing, the latter of which has seen significant advances
in the past decade as it is now possible to evaluate the entire labyrinth (all
semicircular canals and otolith organs).

VIDEO LEGENDS
VIDEO 2-1 VIDEO 2-6
Weak jerk nystagmus in a patient with resolving Left-posterior canal benign paroxysmal positional
vestibular neuritis. The eyes are first in primary vertigo (BPPV). The patient is in the left Dix-Hallpike
position of gaze, and modest spontaneous position, and upbeat and left-torsional nystagmus is
left-beat nystagmus is present. When the patient present. This pattern is consistent with left-posterior
directs gaze rightward, no nystagmus is present. canal BPPV. The patient then sits up and is put in the
When the patient directs gaze leftward, left-beat right Dix-Hallpike position, and downbeat and right-
nystagmus is more pronounced than it had been on torsional nystagmus is present. This pattern is
primary position of gaze. This pattern is in accordance compatible with “unwinding” of left-posterior canal BPPV.
with the Alexander law for a right-sided vestibular
weakness. © 2021 American Academy of Neurology

© 2021 American Academy of Neurology VIDEO 2-7

Geotropic direction-changing positional
VIDEO 2-2 nystagmus. When the patient is first in the left
Vertical pendular nystagmus in a patient with Dix-Hallpike position, left-beat nystagmus is
pontine bleed and palatal myoclonus (not shown). present. When the patient is moved to the right
Dix-Hallpike position, right-beat nystagmus is
© 2021 American Academy of Neurology present. This pattern is geotropic
direction-changing positional nystagmus.
VIDEO 2-3
Latent nystagmus, a variety of congenital © 2006 Timothy C. Hain, MD
nystagmus. At first, the camera is over the right eye,
the patient is viewing out of the left eye, and left-beat VIDEO 2-8
nystagmus is present. Then, the camera is switched Apogeotropic direction-changing positional
from the right eye to the left eye (noted as Switch nystagmus. When the patient is first in the left
Cover in the video), and the patient is viewing out of Dix-Hallpike position, right-beat nystagmus is
the right eye, and right-beat nystagmus is present. present. When the patient is moved to the right
The fast phase of the nystagmus is ipsiversive to the Dix-Hallpike position, left-beat nystagmus is
eye out of which the patient is viewing. This pattern is present. This pattern is apogeotropic
characteristic of latent nystagmus. direction-changing positional nystagmus.

© 2021 American Academy of Neurology © 2006 Timothy C. Hain, MD

VIDEO 2-4 VIDEO 2-9

Dix-Hallpike test and right benign paroxysmal Vibration-induced nystagmus in a patient with a
positional vertigo from the posterior canal. vestibular schwannoma that was removed 40 years
ago. Vibration is applied on the right mastoid
© 2021 American Academy of Neurology process and then on the left mastoid process.
Vibration on either side elicits left-beat nystagmus,
VIDEO 2-5 consisting of rightward drift (slow phase of
Dix-Hallpike test and nystagmus from left benign nystagmus toward the side of vestibular weakness)
paroxysmal positional vertigo from the posterior and leftward saccade (fast phase of nystagmus
canal. away from the side of vestibular weakness).

© 2021 American Academy of Neurology © 2005 Timothy C. Hain, MD

346 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

REFERENCES

1 Baloh RW. Robert Bárány and the controversy 14 Dumas G, Perrin P, Ouedraogo E, Schmerber S.
surrounding his discovery of the caloric reaction. How to perform the skull vibration-induced
Neurology 2002;58(7):1094-1099. doi:10.1212/ nystagmus test (SVINT). Eur Ann Otorhinolaryngol
wnl.58.7.1094 Head Neck Dis 2016;133(5):343-348. doi:10.1016/
j.anorl.2016.04.002
2 Hain T. Approach to the patient with dizziness
and vertigo. In: Biller J, editor. Practical 15 Halmagyi GM, Curthoys IS. A clinical sign of canal
neurology. 4th ed. Philadelphia, PA: Wolters paresis. Arch Neurol 1988;45(7):737-739.
Kluwer, Lippincott, Williams & Wilkins 2017. doi:10.1001/archneur.1988.00520310043015
3 Fife TD. Approach to the history and evaluation 16 Kheradmand A, Zee DS. The bedside examination
of vertigo and dizziness. Continuum (Minneap of the vestibulo-ocular reflex (VOR): an update.
Minn) 2021;27(2, Neuro-otology):306-329. Rev Neurol (Paris) 2012;168(10):710-719.
doi:10.1016/j.neurol.2012.07.011
4 Longridge NS, Mallinson AI. Clinical Romberg
testing does not detect vestibular disease. Otol 17 Fife TD, Tusa RJ, Furman JM, et al. Assessment:
Neurotol 2010;31(5):803-806. doi:10.1097/ vestibular testing techniques in adults and
MAO.0b013e3181e3deb2 children: report of the Therapeutics and
Technology Assessment Subcommittee of the
5 Graybiel A, Fregly AR. A new quantitative ataxia
American Academy of Neurology. Neurology
test battery. Acta Otolaryngol 1966;61(4):292-312.
2000;55(10):1431-1441. doi:10.1212/wnl.55.10.1431
6 Agrawal Y, Carey JP, Hoffman HJ, et al. The
18 Fife TD, Colebatch JG, Kerber KA, et al. Practice
modified Romberg balance test: normative data
guideline: cervical and ocular vestibular evoked
in US adults. Otol Neurotol 2011;32(8):1309-1311.
myogenic potential testing: report of the
doi:10.1097/MAO.0b013e31822e5bee
Guideline Development, Dissemination, and
7 Fukuda T. The stepping test: two phases of the Implementation Subcommittee of the American
labyrinthine reflex. Acta Otolaryngol 1959;50(2): Academy of Neurology. Neurology 2017;89(22):
95-108. doi:10.3109/00016485909129172 2288-2296. doi:10.1212/WNL.0000000000004690

8 Honaker JA, Shepard NT. Performance of Fukuda 19 Klopp-Dutote N, Kolski C, Biet A, et al. A
stepping test as a function of the severity of caloric radiologic and anatomic study of the superior
weakness in chronic dizzy patients. J Am Acad semicircular canal. Eur Ann Otorhinolaryngol
Audiol 2012;23(8):616-622. doi:10.3766/jaaa.23.8.6 Head Neck Dis 2016;133(2):91-94. doi:10.1016/
j.anorl.2015.11.001
9 Hart CW. The Quix test. Laryngoscope 1983;93(9):
1160-1161. doi:10.1288/00005537-198309000-00010 20 Ishiyama G. Selected otologic disorders causing
dizziness. Continuum (Minneap Minn) 2021;
10 Fife TD, Tourkevich R. Tinnitus, hyperacusis,
27(2, Neuro-otology):468-490.
otalgia, and hearing loss. Continuum (Minneap
Minn) 2021;27(2, Neuro-otology):491-525. 21 Oh SY, Kim JS, Yang TH, et al. Cervical and ocular
vestibular-evoked myogenic potentials in
11 Robinson DA, Zee DS, Hain TC, et al. Alexander's
vestibular neuritis: comparison between air- and
law: its behavior and origin in the human
bone-conducted stimulation. J Neurol 2013;
vestibulo-ocular reflex. Ann Neurol 1984;16(6):
260(8):2102-2109. doi:10.1007/s00415-013-6953-8
714-722. doi:10.1002/ana.410160614
22 Cevette MJ, Puetz B, Marion MS, et al.
12 Kerber KA. Episodic positional dizziness.
Aphysiologic performance on dynamic
Continuum (Minneap Minn) 2021;27(2,
posturography. Otolaryngol Head Neck Surg
Neuro-otology):348-368.
1995;112(6):676-688. doi:10.1016/s0194-5998(95)
13 Cherchi M, Hain T. Provocative maneuvers for 70175-3
vestibular disorders. In Eggers S, Zee DS, eds.
Vertigo and imbalance: clinical neurophysiology
of the vestibular system. Amsterdam,
Netherlands: Elsevier, 2010.

CONTINUUMJOURNAL.COM 347

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 REVIEW ARTICLE


Chronic Dizziness
C O N T I N UU M A U D I O By Yoon-Hee Cha, MD, FAAN
INTERVIEW AVAILABLE
ONLINE
Downloaded from http://journals.lww.com/continuum by wqLe/H3/oM2vx1Qs5A5qMemKKHLPqE4qkmGNpdheG3ikf1o85ttJSkFL2oTkgOkhMEmawHvsuTTE5xJG2ZjqccytIAhVn2qegy060KLcKJuPZsx5htaYiopRjp2zor2egUzWJAEWYNo/R1NuKT7SYsuBdzpo0ePV on 04/28/2022

ABSTRACT
PURPOSE OF REVIEW: Determining the etiology of disorders that manifest
with chronic dizziness can seem a daunting task, but extracting some basic
elements of the patient’s history can reduce the differential diagnosis
significantly. This includes determining initial triggers, timing of
symptoms, associated features, and exacerbating factors. This article
covers distinct causes of chronic dizziness including persistent postural
perceptual dizziness, mal de débarquement syndrome, motion
sickness and visually induced motion sickness, bilateral vestibulopathy,
and persistent dizziness after mild concussion.
CITE AS:
CONTINUUM (MINNEAP MINN) RECENT FINDINGS: Todate, none of the disorders above has a cure but are
2021;27(2, NEURO-OTOLOGY):
420–446.
considered chronic syndromes with fluctuations that are both innate and
driven by environmental stressors. As such, the mainstay of therapy for
Address correspondence to chronic disorders of dizziness involves managing factors that exacerbate
Dr Yoon-Hee Cha, University of symptoms and adding vestibular rehabilitation or cognitive-behavioral
Minnesota, 717 Delaware St SE,
Minneapolis, MN 55414, therapy alone or in combination, as appropriate. These therapies are
[email protected]. supplemented by serotonergic antidepressants that modulate sensory
gating and reduce anxiety. Besides expectation management, ruling out
RELATIONSHIP DISCLOSURE:
Dr Cha has received concurrent disorders and recognizing behavioral and lifestyle factors
research/grant support from that affect symptom severity are critical issues in reducing morbidity for
the Brain & Behavior Research
Foundation, the Mal de
each disorder.
Debarquement Syndrome
(MdDS) Balance Disorder SUMMARY: Many syndromes of chronic dizziness can be diagnosed by
Foundation, MnDrive Scholars, recognition of key features, although many symptoms overlap between
National Institutes of
Health/National Institute of these groups. Symptoms may be manageable and improve with time, but
General Medical Sciences they are often incompletely relieved.
(P20 GM121312), the National
Science Foundation/
Established Program
to Stimulate Competitive INTRODUCTION

P
Research (R2 Track-2 1539068),
Springbank Foundation, and
atients who present with chronic dizziness can be clinically
Totts-Gap Foundation. challenging because of the wide range of potential causes, but
methodical determination of factors such as timing, triggers,
UNLABELED USE OF
PRODUCTS/INVESTIGATIONAL
associated symptoms, and the presence of any asymptomatic periods
USE DISCLOSURE: can quickly narrow the differential diagnosis. It is helpful to
Dr Cha discusses the determine whether the patient has episodic dizziness with asymptomatic periods
unlabeled/investigational use of
selective serotonin reuptake versus chronic constant symptoms with episodic exacerbations. This article
inhibitors in the treatment of discusses five major syndromes that cause chronic constant dizziness:
mal de débarquement
persistent postural perceptual dizziness (PPPD), mal de débarquement
syndrome and persistent
postural perceptual dizziness. syndrome, motion sickness and visually induced motion sickness, bilateral
vestibulopathy, and persistent dizziness after mild concussion.
© 2021 American Academy
The terms dizziness and vertigo are used according to definitions outlined in the
of Neurology. International Classification of Vestibular Disorders established by the Bárány

420 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Society Classification Committee.1 Dizziness and vertigo are nonhierarchical terms KEY POINT
that are used distinctly to relate a person’s subjective experience. Dizziness is
● Persistent postural
defined as, “the sensation of disturbed or impaired spatial orientation without a perceptual dizziness is a
false or distorted sense of motion,” whereas vertigo is defined as, “the sensation chronic disorder of postural
of self-motion when no self-motion is occurring or the sensation of distorted instability that lasts at least
self-motion during an otherwise normal head movement.”1 Dizziness and vertigo 3 months but can have
fluctuations that are both
can and frequently do occur together, but being able to distinguish different
innate as well as driven by
components of the patient’s experience can be helpful, especially because environmental stimuli such
many of the most common disorders of dizziness do not involve any symptoms as passive or active motion
of vertigo. and visual stimuli.

PERSISTENT POSTURAL PERCEPTUAL DIZZINESS

This section reviews the clinical features, diagnostic criteria, causes, time course,
and treatment for PPPD, a syndrome characterized by persistent postural
instability and visually induced dizziness. It has become a major diagnostic entity
that encompasses many triggers of chronic dizziness. PPPD and its precursors
represented a shift away from thinking about vestibular disorders as involving
only measurable damage to pathways along the peripheral or central vestibular
system.

Clinical Syndrome
The term PPPD represented the culmination of efforts to bring previous
designations such as chronic subjective dizziness, space and motion discomfort,
and visual vertigo into a common diagnostic framework.2 All of these disorders
were originally described as entailing a combination of dizziness, postural
instability, and discomfort in visually complex or motion-rich environments,
although with differing degrees of emphasis in each. Of note, visual vertigo was a
term referenced for historical purposes in the designation of PPPD.3 After the
presentation of the International Classification of Vestibular Disorders in 2009,
the visual vertigo term was replaced by visually induced dizziness because the
phenomenon is related to visually induced feelings of disorientation rather than
an actual feeling of motion.1 A closely related disorder, phobic postural vertigo
was not included under PPPD because phobic postural vertigo was defined as
including primary mood or anxiety diagnoses or obsessive-compulsive
personality traits as inherent components.4 In contrast, in PPPD, any mood or
anxiety disorder or personality trait is considered a comorbid condition that may
be a risk factor but not the primary driver of symptoms.

Criteria
Criteria for PPPD were established by the Bárány Society for International
Classification of Vestibular Disorders in 2017 (TABLE 6-1).2 Patients are
diagnosed with PPPD when they experience a syndrome complex lasting at least
3 months that is characterized by dizziness, unsteadiness, or nonspinning
vertigo that is present for most of the time and is worsened by being upright, by
being in motion, or during exposure to visual motion or complex visual patterns.
Significant distress or functional impairment must be present and caused by
the associated symptoms. As is the case for all Bárány Society criteria, a diagnosis
of PPPD requires that symptoms are not better accounted for by another disease
or disorder. PPPD can, however, coexist with the disorder that initially
triggered it. Because the triggering events for PPPD can be quite varied, patients

CONTINUUMJOURNAL.COM 421

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

may initially present to a variety of physicians including primary care

physicians, neurologists, psychiatrists, ophthalmologists, or otolaryngologists
before ultimately being diagnosed with PPPD.

Causes
PPPD can be precipitated by severe homeostatic derangements such as
psychological distress, a medical illness, or a neurologic or vestibular disorder. PPPD
can develop after varied causes such as vestibular neuritis, concussion, autonomic
dysfunction, and severe panic attacks; it is a diagnosis that is independent of the
initial trigger. PPPD can be present with other vestibular disorders such as vestibular
migraine, Ménière disease, vestibular neuritis, or benign paroxysmal positional
vertigo (BPPV) but is diagnosed separately and in addition to those disorders
(CASE 6-1). Even if the precipitating factor has resolved, PPPD can continue without
an ongoing trigger. Thus, PPPD can be diagnosed as a sequela to or concurrently
with vestibular, cardiac, autonomic, or other neurologic disorders as long as all of
the symptom components of the diagnostic criteria are met.

Time Course
The time course for PPPD development can vary depending on the initial
trigger, but PPPD generally develops in relation to an acute event rather than

TABLE 6-1 Diagnostic Criteria for Persistent Postural Perceptual Dizzinessa

Persistent postural perceptual dizziness is a chronic vestibular disorder defined by criteria A

through E below. All five criteria must be fulfilled to make the diagnosis.
A One or more symptoms of dizziness, unsteadiness, or nonspinning vertigo are present on
most days for 3 months or more.
1 Symptoms last for prolonged (hours-long) periods of time but may wax and wane in severity.
2 Symptoms need not be present continuously throughout the entire day.
B Persistent symptoms occur without specific provocation but are exacerbated by three
factors:
1 Upright posture,
2 Active or passive motion without regard to direction or position, and
3 Exposure to moving visual stimuli or complex visual patterns.
C The disorder is precipitated by conditions that cause vertigo, unsteadiness, dizziness, or
problems with balance including acute, episodic, or chronic vestibular syndromes, other
neurologic or medical illnesses, or psychological distress.
1 When the precipitant is an acute or episodic condition, symptoms settle into the pattern of
criterion A as the precipitant resolves, but they may occur intermittently at first, and then
consolidate into a persistent course.
2 When the precipitant is a chronic syndrome, symptoms may develop slowly at first and
worsen gradually.
D Symptoms cause significant distress or functional impairment.
E Symptoms are not better accounted for by another disease or disorder.

a
Reprinted with permission from Staab JP, et al, J Vestib Res.2 © 2017 IOS Press and the authors.

422 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

insidiously. The initial acute event can be of varied etiology (eg, after vestibular
neuritis), or it can have an initial stuttering course if the trigger is episodic
(eg, vestibular migraine). In all cases, PPPD is defined as the chronic state that
develops after these initial triggers and typically does not include any
asymptomatic periods. PPPD can be diagnosed without a known trigger,
however, which is often the case in patients who have had prolonged symptoms
and cannot remember the circumstances surrounding the symptom onset.
However, a slowly progressive time course that does not have a clear onset
should raise suspicion for another process, such as a neurodegenerative disorder.

A 36-year-old man experienced a bout of severe spinning vertigo and CASE 6-1
nausea with no hearing loss that lasted several days. He had recovered
from a viral illness several weeks prior. He was seen in the emergency
department and had a normal cranial nerve examination except for a
positive head impulse test to the right side, signifying a right-sided
vestibulopathy. He tended to veer to the right on gait examination. He
was diagnosed with a right-sided vestibular neuritis and sent home with
instructions to take meclizine as needed. The patient was able to
ambulate in about 1 week and was able to return to work in 2 weeks.
Despite continued recovery, at 3 months he was still experiencing a
persistent sense of imbalance, head motion–induced nausea,
unsteadiness, and a reduced ability to tolerate visually busy
environments, particularly the grocery store. He developed agoraphobia
and anticipatory anxiety going to social events and subsequently severely
curtailed outings with his friends.
His neurologic examination was normal, including resolution of an initially
abnormal head impulse test to the right side. He walked with a stiffened
gait, however.
His neurologist diagnosed him with persistent postural perceptual
dizziness (PPPD) and prescribed him vestibular rehabilitation, a selective
serotonin reuptake inhibitor (SSRI), and cognitive-behavioral therapy.
The vestibular therapist worked with the patient on habituation exercises
to increase his tolerance for visual stimulation. The clinical psychologist
helped the patient develop reappraisal strategies when he started to feel
trapped in crowded environments. Within about 6 months, his symptoms
decreased to the point that he was able to socialize with friends again.

PPPD can develop after a variety of homeostatic perturbations, such as an COMMENT

episode of vestibular neuritis. A prolonged recovery can become a setup
for maladaptive postural responses and behavioral inhibition, which can
lead to multiple layers of functional impairment. Vestibular rehabilitation
can promote habituation to greater degrees of head movement and visual
stimulation while cognitive-behavioral therapy can help avoid the
development of maladaptive emotional responses that can lead to social
isolation. The addition of a serotonergic antidepressant may be helpful in
raising tolerance for sensory stimuli and the threshold for developing anxiety.

CONTINUUMJOURNAL.COM 423

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

Treatment
The three strategies for treating PPPD are vestibular rehabilitation,
cognitive-behavioral therapy (CBT), and serotonergic antidepressants.5
Providing patients with a positive diagnosis of PPPD and an explanation of the
interplay between acute triggers and the persistence of maladaptive responses
can itself be therapeutic.
One mechanism for the development of PPPD is overreliance on the visual and
somatosensory systems after a vestibular perturbation has occurred, as well as
the activation of high-vigilance mechanisms of postural control that are no longer
adaptive during the recovery phase.6 These mechanisms may have been
appropriate in the initial triggering event, such as when active vertigo creates a
real threat of falls or vestibular input is not reliable (eg, during a Ménière
disease attack). However, as the triggering event resolves, these initial postural
strategies are not only no longer appropriate, they can lead to worsened balance.
Some inappropriate strategies include shortening of stride length, stiffening of
posture, and co-contraction of agonist and antagonist muscles. These behaviors
may persist after a balance perturbation as a conditioned response to threatened
balance. During nonstressed gait, these behaviors can increase the energy
expenditure of normal walking while providing no protection against falls.

VESTIBULAR REHABILITATION. The reweighting of sensory inputs through

vestibular, visual, and somatosensory systems can be accomplished through
focused vestibular rehabilitation. Vestibular therapy must be done gently,
however, as overaggressive treatment can lead to greater anxiety and
perpetuation of an abnormally strong conditioned fear response to motion.
Despite the strong fear of falling, patients with PPPD generally do not fall; this
may reflect an impairment of higher cognitive appraisal of actual fall risk. If a
patient has frequent falls despite resolution of the initial triggering event, an
alternative diagnosis to PPPD should be sought.7
In general, patients who do well with vestibular rehabilitation have a resilient
mindset, have higher satisfaction with life, and are generally optimistic. Negative
prognostic factors include anxious temperament, introversion, hypervigilance
about physical symptoms at the time of injury, and catastrophic thinking about
the outcome of therapy.8-11

COGNITIVE-BEHAVIORAL THERAPY. CBT has been found to be helpful in PPPD, even

in the long term. Anticipatory anxiety to potential threats to postural control
can reduce the threshold for engaging in high-threat postural strategies that are
contextually inappropriate. Developing awareness of this pattern, reappraising
environmental threats to balance, overcoming the fear of falling, and being able
to actively think through strategies to deal with feelings of disorientation
(rather than trying to escape) are all tasks that can be addressed with regular
focused CBT. CBT can help address some of the cognitive and emotional barriers
to improvement in a vestibular rehabilitation program.

ANTIDEPRESSANTS. Serotonergic antidepressants (selective serotonin reuptake

inhibitors [SSRIs]) or those that also include action on the norepinephrine system
(SSRIs/serotonin norepinephrine reuptake inhibitor [SNRIs]) can be helpful in
PPPD. The mechanism of efficacy is unclear because the serotonergic system
in the brainstem projects nearly ubiquitously to the cerebrum. Sensory gating

424 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

and reweighting of visual versus vestibular inputs are theoretical mechanisms, KEY POINTS
however. Patients with balance disorders are generally sensitive to additional
● Persistent postural
homeostatic perturbations, and this includes sensitivity to medication side perceptual dizziness may be
effects. It is prudent, therefore, to start these agents at one-quarter to one-half of triggered by any severe
the usual starting doses used to treat depression and to proceed with a gentle homeostatic perturbation
titration. Clinical effects can take 2 to 3 months, particularly because the titration such as a vestibular disorder
or medical, neurologic, or
phase is usually slower than is typical for these medications when used
psychological process.
for other disorders. Symptoms may continue
despite resolution of the
MAL DE DÉBARQUEMENT SYNDROME initial trigger or can coexist
with an ongoing trigger.
This section reviews the clinical features, diagnostic criteria, causes, time course,
and treatment for mal de débarquement syndrome. Mal de débarquement ● A slowly progressive
translates to sickness of disembarkation and has been recognized as a post–motion disorder without a clear
exposure phenomenon for centuries. However, it has only been in recent precipitant is not consistent
history that this phenomenon has become well known because of increased with persistent postural
perceptual dizziness.
travel opportunities of humans to boats, planes, and cars.
● The mainstays of therapy
Clinical Syndrome for persistent postural
Mal de débarquement syndrome refers to the chronic feeling of oscillating perceptual dizziness include
vestibular rehabilitation,
vertigo, generally described as, “rocking,” “bobbing,” or “swaying,” that occurs
cognitive-behavioral
after prolonged exposure to motion. The term oscillating denotes the actual therapy, and serotonergic
feeling of motion rather than a temporal pattern of vertigo coming and going. antidepressants.
Most cases occur after water-based travel, such as after disembarking from a
cruise. However, a growing number of cases are being seen after air travel and ● Mal de débarquement
syndrome is a disorder of
prolonged land travel. Patients with mal de débarquement syndrome frequently post–motion-induced
report feeling like they are “still on the boat.” In many cases, the exact persistent oscillating vertigo
frequency of the oscillation can be measured. A hallmark feature of mal de lasting more than 48 hours.
débarquement syndrome is the reduction in motion perception with reexposure
● The perception of motion
to motion, such as driving a car or getting back on the boat. Because of the in mal de débarquement
modulating effect of motion, some patients find that walking or running is better syndrome is usually
than standing still. The temporary reprieve from symptoms with motion is described as rocking,
often associated with a temporary exacerbation once the motion stops, however. bobbing, or swaying. This
perception decreases when
This is particularly apparent when the patient has a reduction in symptoms
the individual is back in
when driving a car but feels like he or she is still moving when the car stops. motion such as when driving.
Mal de débarquement syndrome is associated with a variable number of other
symptoms including visually induced dizziness, cognitive difficulties (patients ● Symptoms associated
use the term “brain fog”), new or exacerbation of headaches, chronic fatigue, with mal de débarquement
syndrome include chronic
anxiety, sleep disturbance, tinnitus, general sensory hypersensitivity, and leg fatigue, visually induced
heaviness. The feeling of a gravitational, or “G-force,” on the body is a common dizziness, headaches,
description. It is not typical for mal de débarquement syndrome to be tinnitus, and anxiety. It is not
associated with nystagmus, extraocular movement abnormalities, hearing loss, typical for mal de
débarquement syndrome to
or spinning vertigo, however. The presence of any of these features should be associated with
prompt a search for an alternative diagnosis. Typical cases of mal de nystagmus, extraocular
débarquement syndrome are not associated with any abnormalities in movement abnormalities,
neuroimaging or vestibular or auditory testing (CASE 6-2).12 hearing loss, or spinning
vertigo.
Mal de débarquement syndrome typically occurs in women and has a peak age
of onset between 40 and 50 years with a bell-shaped curve around this peak
(CASE 6-2). The proportion of men with mal de débarquement syndrome has
been reported to be as high as 25% to as low as 0%.12 In all case series, women
significantly outnumber men. Hormonal state may be relevant because the

CONTINUUMJOURNAL.COM 425

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

majority of women who develop mal de débarquement syndrome are

perimenopausal, and the majority of premenopausal women with mal de
débarquement syndrome note that their symptoms are worse before menses.13
New-onset headaches, worsened existing headaches, and hypersensitivity to
light and sound can develop along with mal de débarquement syndrome. The
prevalence of headaches that meet criteria for migraine is almost 50% in mal de
débarquement syndrome.14 Mal de débarquement syndrome does not meet
the current criteria for vestibular migraine.15 Mal de débarquement syndrome

CASE 6-2 A 50-year-old woman went on a 7-day cruise and felt well on the trip.
While waiting at the airport to catch her flight home after disembarking
from the cruise, she noticed a feeling of rocking, as if she were still on
the cruise ship. She did not notice this so much during the flight itself or
the car ride home after the flight. Once she got home, however, she
noticed a stronger sense of motion. She nearly fell over while taking a
shower that evening. The next morning, she woke up with a strong sense
of rocking, as if she were still on the cruise ship. The rocking feeling
only subsided when she was driving a car again. In addition, she felt
fatigued, had slowed cognitive processing, and had a difficult time
tolerating visual stimuli at the grocery store. She noticed heightened light
and sound sensitivity and a persistent headache. She was otherwise in
excellent health with no chronic illnesses. She did note the recent onset
of hot flashes that raised concerns of oncoming menopause. Her
neurologic examination was normal.
Her neurologist ordered neuroimaging and vestibular and auditory
testing, which all returned within normal limits. Her symptoms were
persistent at 3 months, and she had to take a leave of absence from work.
She was eventually started on clonazepam 0.25 mg 2 times a day and
venlafaxine extended release 75 mg every morning. This medication
combination decreased her symptoms enough to allow her to return to
her work, but they were not completely relieved.

COMMENT The persistent oscillating vertigo of mal de débarquement syndrome is

generally described as a rocking sensation that decreases with reexposure
to passive motion, usually with driving a car. The most common trigger
for mal de débarquement syndrome is ocean travel on a cruise, and the
most common demographic affected with mal de débarquement
syndrome is middle-aged women. Mal de débarquement syndrome is
associated with a variety of symptoms such as cognitive slowing, fatigue,
headache, and visually induced dizziness that can be as debilitating as the
persistent vertigo. Patients with mal de débarquement syndrome can
have symptoms exacerbated in closed spaces such as showers or in very
wide-open spaces with poor focal points such as grocery stores.
Medications such as selective serotonin reuptake inhibitors (SSRIs) and
benzodiazepines can be helpful, but they rarely lead to a complete
resolution of symptoms.

426 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

occurs without a history of migraine in more than 50% of affected individuals
and is a persistent syndrome that has fluctuations but no significant periods
of reprieve. Vestibular migraine requires time-limited vestibular symptoms that
occur in episodes in someone who meets criteria for migraine headaches.

Criteria
Diagnostic criteria for mal de débarquement syndrome have been published by
the Classification Committee for the Bárány Society (TABLE 6-2).16 Mal de
débarquement syndrome is diagnosed when oscillating vertigo occurs after
disembarkation from a moving vessel such as a boat, plane, or car with symptoms
lasting for at least 48 hours. Symptoms temporarily improve with exposure to
passive motion. Symptoms that last for more than 48 hours but less than 1 month
are designated as transient mal de débarquement syndrome. Symptoms that last
for more than 1 month are designated as persistent mal de débarquement syndrome.
If at least 1 month of observation time has not passed, mal de débarquement
syndrome is designated as in evolution. Postmotion unsteadiness lasting less than
48 hours should be termed land sickness and not mal de débarquement syndrome.
Land sickness is extremely common, affecting up to three-fourths of healthy
adults, and shows an equal sex distribution.17–20

Causes
The most common triggers for mal de débarquement syndrome relate to
water-based travel, followed by air- and then land-based travel. However, any
kind of persistent passive motion exposure can lead to mal de débarquement
syndrome. Patients have described triggers such as sleeping on waterbeds, living
on houseboats, running on treadmills, and spending the day in a swaying
tower as all preceding the onset of very typical mal de débarquement syndrome
symptoms.12 Although the trigger itself is important, individual factors may
raise susceptibility. These factors include age, sex, low estrogen state, and stress
during motion exposure.13

Diagnostic Criteria for Mal de Débarquement Syndromea TABLE 6-2

A Nonspinning vertigo characterized by an oscillatory perception (“rocking,” “bobbing,”

“swaying”) present continuously or for most of the day
B Onset occurs within 48 hours after the end of exposure to passive motion
C Symptoms temporarily reduce with exposure to passive motion
D Symptoms continue for >48 hours
D0: Mal de débarquement syndrome in evolution: symptoms are ongoing, but the
observation period has been less than 1 month
D1: Transient mal de débarquement syndrome: symptoms resolve at or before 1 month, and
the observation period extends at least to the resolution point
D2: Persistent mal de débarquement syndrome: symptoms last longer than 1 month
E Symptoms not better accounted for by another disease or disorder.

a
Reprinted with permission from Cha YH, et al, J Vestib Res.16 © 2020 IOS Press and the authors.

CONTINUUMJOURNAL.COM 427

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

Time Course
The duration of mal de débarquement syndrome in any individual is difficult to
predict, but the longer the symptoms persist, the lower the probability of
resolution. Symptoms lasting longer than 6 months generally have a low chance
of spontaneous remission. Curiously, when symptoms remit, they can do so over
a very short period of time. Patients may even say that the symptom went away,
“like a light switch.” Recurrent episodes of mal de débarquement syndrome tend
to be either the same length or get progressively longer with repeated motion
exposure.20 In any given episode, symptoms do tend to get better with time.
Symptoms that get worse with time should trigger a search for a concurrent
diagnosis such as severe anxiety or another cause for balance dysfunction.

Treatment
No cure for mal de débarquement syndrome exists because symptoms arise from
the brain’s natural ability to entrain to periodic motion. Some experimental
protocols have been able to put mal de débarquement syndrome into remission
or reduce symptoms to more manageable levels. These experimental
procedures involve neuromodulation methods such as transcranial magnetic
stimulation, transcranial electrical current stimulation, or readaptation of the
vestibular ocular reflex.21–24 In the clinical realm, however, treatments consist of
lifestyle modifications including precautions taken before travel, as well as
serotonergic antidepressants and benzodiazepines.

LIFESTYLE MODIFICATIONS. Mal de débarquement syndrome symptoms are

exacerbated by emotional stress and poor sleep; these two factors are the most
important for successful symptom management. Patients with mal de
débarquement syndrome frequently feel overwhelmed by sensory stimuli in
places such as grocery stores, shopping malls, and large open warehouse-type
stores; conversely, very small spaces such as bathrooms and shower stalls
can make them feel unstable. Thus, finding the right balance of open spaces and a
tolerable amount of visual stimulation can be challenging. Generally, noisy
environments, low-light conditions, and excessive visual motion stimulation
should be avoided. No evidence has been found that severe dietary restrictions or
low-salt diets are helpful for mal de débarquement syndrome symptoms.13
Patients frequently find that their tolerance for alcohol is reduced because it
impairs their balance. The effect of caffeine is variable.

SEROTONERGIC ANTIDEPRESSANTS. Antidepressants in the SSRI category are

typically much more effective than those that primarily work on the
norepinephrine system, for example, tricyclic antidepressants and SNRIs, for
mal de débarquement syndrome symptoms.13 Commonly used SSRIs include
citalopram, escitalopram, sertraline, paroxetine, and fluoxetine. The choice of
agent depends on tolerance and side effects. Of the antidepressants that have
activity on both the serotonin and norepinephrine systems, venlafaxine is the
most widely used among neuro-otologists, but no head-to-head trials have been
conducted to support its use above others in its class. Patients with mal de
débarquement syndrome are typically very sensitive to medication side effects
and should be started at lower than the usual starting doses of these medications
(generally at one-quarter to one-half of the usual adult dose) with a slow titration.
When done slowly, they can usually reach a standard adult therapeutic dose.

428 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

BENZODIAZEPINES. Benzodiazepines are the most quickly acting and effective KEY POINTS
symptomatic treatment for mal de débarquement syndrome.13,20 Because mal de
● Clinically available
débarquement syndrome symptoms are typically chronic and are present to treatments for mal de
some degree all day, most patients who are put on benzodiazepines use débarquement syndrome
clonazepam because of its long half-life (12 to 24 hours). A history of medication include serotonergic
sensitivity should be assessed in patients with mal de débarquement syndrome. antidepressants and
benzodiazepines; vestibular
A typical starting dose of clonazepam is 0.25 mg 1 time a day with a titration
therapy is generally not
up to no higher than 0.5 mg 2 times a day. Dosing higher than this level is helpful.
typically not additionally effective.
Agents with intermediate half-lives such as lorazepam or diazepam can ● Motion sickness and
be used before traveling to reduce post–motion exposure exacerbation of visually induced motion
sickness are generally
symptoms. Doses as low as 0.5 mg to 1 mg of diazepam can be sufficient for this self-limited processes that
particular application. Flights that are longer than 6 hours may require a second end when the stimulus is
dose midflight. Benzodiazepines should not be used in combination, however over. Symptoms may
(eg, diazepam on top of clonazepam). Patients should be advised not to drive or include nausea/vomiting,
stomach awareness,
consume alcohol when they are taking benzodiazepines. headache, sweating/pallor,
dizziness, drowsiness, or
MOTION SICKNESS AND VISUALLY INDUCED MOTION SICKNESS eyestrain.
This section reviews the clinical features, diagnostic criteria, causes, time
course, and treatment for motion sickness and visually induced motion
sickness. These phenomena are normal physiologic responses to motion that
become defined as disorders depending on severity. These nuances are
discussed in this section.

Clinical Syndrome
Motion sickness is a polysymptomatic disorder that can be experienced by all
individuals who have a functioning vestibular system given a strong enough
stimulus. The induction of sick feelings in the form of nausea, stomach awareness
or discomfort, thermoregulatory dysfunction, headache, dizziness, or
drowsiness is a normal physiologic response and may be accompanied by signs
such as vomiting, cold sweating, or pallor. However, when the threshold of
experiencing these symptoms is very low and habituation to repeated stimuli is
lacking, the morbidity from motion sickness can be extremely high. This can
lead to restrictions in social, personal, and professional activities (CASE 6-3).
Motion sickness can be divided into sickness induced by physical motion of
the person or by visual motion. Susceptibility to one kind of motion sickness does
not necessarily predict susceptibility to the other. For practical purposes in this
article, the term motion sickness is used for physical motion of the self, whereas
visually induced motion sickness refers to sickness caused by visual motion.25,26

MOTION SICKNESS. Motion sickness is generally a benign self-limited condition

that starts during exposure to motion, whether active or passive. Most causes of
motion sickness are due to passive motion exposure such as occurs on boats,
airplanes, or cars. The sickness symptoms gradually arise during the motion
exposure and abate after the motion stops. Most cases of motion sickness resolve
within hours if not within minutes of the end of stimulation. The exception is
headache. When a headache is triggered by motion, it can last well after the
motion has ended and only resolve after it has been specifically treated. Motion
sickness symptoms can continue past the cessation of motion to some degree, but
symptoms that arise only after the motion has ended should not be considered

CONTINUUMJOURNAL.COM 429

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

motion sickness; these individuals may have land sickness or may progress to
developing mal de débarquement syndrome.
Nausea is the most ubiquitous symptom of motion sickness. If retching or
vomiting is to occur during a motion sickness episode it is generally preceded by
stomach awareness and worsening nausea. People who are very susceptible to
motion sickness can experience the “avalanche phenomenon,” which is
characterized by a rapid onset of vomiting after motion exposure. In very rare
cases, typically in situations in which an individual has learned to ignore the
premonitory symptoms of motion sickness, he or she can go straight to
vomiting.26 Motion exposure can lead to drowsiness in some individuals; they
may fall asleep before developing any other symptom. This has been called the
sopite syndrome and can also take the form of motion-triggered tiredness,
lethargy, fatigue, or yawning.27

VISUALLY INDUCED MOTION SICKNESS. Visually induced motion sickness is caused

by visual stimuli such as occurs with simulation equipment, IMAX screens,
virtual reality displays, computers, televisions, and even smartphone screens.26
In addition to the usual symptoms of motion sickness, visually induced motion
sickness is associated with eyestrain and blurred vision. Nausea to the point of

CASE 6-3 A 24-year-old woman took the shuttle from the park-and-ride to her
office every day. The ride took a lot of turns and made several stops along
the route. She noticed that, around 10 minutes into the ride, she always
started to feel nauseated and developed a slight headache. It was
worsened by the smell of the exhaust from the vehicle when the shuttle
briefly stopped along the route. She never vomited from the nausea, but
she could feel a discomfort in her stomach before frank nausea began.
The ride was over before her symptoms got too severe. She found that if
she looked at her smartphone during the ride, the nausea was worse.
The nausea got better as soon as the bus ride was over, and she
generally recovered within 15 minutes of getting off the bus. Because this
happened so frequently, the woman eventually decided to ride her bike
to work and avoid taking the shuttle. She was otherwise in good health
and had no neurologic deficits.

COMMENT The presence of a normally functioning vestibular system creates a risk for
the development of motion sickness. A normal response to predictable
vestibular stimulation that induces nausea is to eventually habituate to the
motion. However, when the features of the motion stimulus are beyond the
person’s adaptive capability, motion sickness symptoms can arise. These
include nausea, stomach awareness, sweating, pallor, headache, dizziness,
and drowsiness. Nausea can increase to the point of vomiting. Motion
sickness symptoms usually decrease after the stimulus is over, although
headache can persist until it is specifically treated. In many cases, such as
in this example, people modify their behavior to avoid sickness-inducing
situations.

430 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

vomiting is unusual in visually induced motion sickness, namely because the KEY POINT
individual can close his or her eyes to avoid continued stimulation. Headache and
● Motion sickness
eyestrain are generally more common with visually induced motion sickness susceptibility peaks at the
than physical motion–induced motion sickness. Visually induced motion ages of 7 to 12 years, is
sickness is different from visually induced dizziness and from the phenomenon stable through adult years,
of vection.28 Although these symptoms can all coexist, they refer to different and declines after age
60 years. Visually induced
aspects of the experience. Visually induced dizziness specifically refers to a
motion sickness generally
sense of spatial disorientation during exposure to visually complex or moving worsens with age.
visual stimuli (see the Persistent Postural Perceptual Dizziness section),
whereas vection refers to the illusion of self-motion that is induced by motion of
the visual field.
In healthy individuals, repeated exposures to the same kind of motion,
whether physical motion or visual motion, lead to habituation and a higher
threshold for inducing sickness symptoms with subsequent exposures.
Individuals very susceptible to motion sickness and visually induced motion
sickness do not habituate to the motion and frequently develop aversive
emotions before motion exposures and may change behaviors to avoid further
motion exposures.
The prevalence of motion sickness in the general population is difficult to
assess because susceptibility varies by age and sex and can be very specific to
each kind of motion. Infants and toddlers younger than 2 years old seem
relatively protected against motion sickness. Thereafter, susceptibility rises until
it peaks between the ages of 7 and 12 years and then gradually declines. In
primary school–aged children before puberty, the rate of carsickness is about
30% to 40%.29 Susceptibility in adults hovers between 14% and 25% and is stable
until around the age of 60 years, at which time susceptibility goes down
significantly to about 7%.30,31 One difference between motion sickness and
visually induced motion sickness is that visually induced motion sickness
susceptibility tends to increase with age, with the highest prevalence in those
older than 60 years.32 These statistics represent general population trends,
whereas individuals can follow a specific course.
Susceptibility is generally higher in women than men, particularly around the
menstrual cycle.33 Even at the same level of motion sickness severity, women are
more likely to experience vomiting.34 The age effect is much stronger than the
sex effect in motion sickness susceptibility, however.35

Criteria
Criteria for motion sickness and visually induced motion sickness have been
drafted by the Bárány Society for both an episode of motion sickness/visually
induced motion sickness and for motion sickness and visually induced motion
sickness as disorders.36 Because motion sickness induction can be very specific to
a specific trigger (eg, sickness in boats but not in cars), the criteria require that
the sickness is induced by the same or similar kind of trigger.
Many scales for motion sickness severity have been developed over the past
60 years, generally motivated by the armed forces and space exploration. Some
severity scales include the Simulator Sickness Questionnaire (SSQ),37 the
Motion Sickness Assessment Questionnaire (MSAQ),38 the Nausea Profile,39 the
Misery Scale,40 and Fast Motion Sickness Scale,41 among several others. Several
motion sickness susceptibility scales have also been developed, although far
fewer of these exist. The Motion Sickness Susceptibility Questionnaire (MSSQ)

CONTINUUMJOURNAL.COM 431

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

Short-form is the most widely used susceptibility scale because it has been
validated through time and translated into multiple languages.42 Because
susceptibility changes dramatically with age (reducing with older age), the
Motion Sickness Susceptibility Questionnaire divides susceptibility into subscales
for individuals 12 years old or younger and for those older than 12 years.
A specific scale can be used for its individual strengths. However, from a
practical standpoint, a short Likert-style scale that queries whether the patient is
“always,” “often,” “sometimes,” or “never” affected by motion sickness can
be used in the office setting. Because individuals can also modify activities to
avoid becoming motion sick, this simple scale can also be used in the context of
how often the patient avoids motion exposure to prevent becoming sick.

Causes
Many theories exist for why motion sickness persists in the modern day. The
most common theory is the sensory conflict and mismatch theory. This refers to
the discrepancy between expected versus experienced sensory inputs through
the visual, vestibular, and somatosensory systems.43 These systems may be in
conflict with each other (eg, vision versus vestibular), may be in conflict with
itself (eg, canal versus otolith input), or reflect a mismatch in perceived versus
expected verticality. It is also possible that the overlap between vestibular and
autonomic pathways makes autonomic activation an unfortunate byproduct of
vestibular stimulation that serves no purpose.44
Certain disorders such as migraine, vestibular migraine, and Ménière disease
are associated with much higher rates of motion sickness susceptibility than
other vestibular disorders.45 The rate of motion sickness is no higher in BPPV or
compensated vestibular neuritis than in healthy controls without vestibular
disorders.46 A functioning vestibular system may be a critical component of
motion sickness because susceptibility is much lower in individuals with bilateral
vestibulopathy.47,48

Time Course
Because motion sickness susceptibility generally declines with age, if this trend
does not follow for an individual, an underlying vestibular asymmetry or
metabolic disorder should be investigated. In contrast, visually induced motion
sickness may increase with age. When severe, however, an underlying ocular
motility issue or other cause of increased eyestrain should be investigated.

Treatment
Both motion sickness and visually induced motion sickness can be treated with
habituation exercises to slowly increase the threshold for developing symptoms
with motion exposure. Oral treatments include anticholinergic, antimuscarinic,
benzodiazepine, and antihistaminergic medications. Common options include
meclizine, dimenhydrinate, promethazine, prochlorperazine, diazepam, and
scopolamine.49 Scopolamine can be given in oral form for rapid action or as a
patch placed on the mastoid and changed every 72 hours. No agent should be
used chronically, however, because they interfere with vestibular compensation
and can be associated with side effects such as sedation, confusion, dry eyes,
dry mouth, and urinary retention. If medications cannot be taken and exposure
to motion is inevitable, controlled breathing exercises, listening to music, or
exposure to pleasant smells can be helpful.50–52

432 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

BILATERAL VESTIBULOPATHY KEY POINTS
This section reviews the clinical features, diagnostic criteria, causes, time course,
● Certain disorders such as
and treatment for bilateral vestibulopathy. Although this is an uncommon migraine, vestibular
disorder, it is important to recognize because of the extremely high degree of migraine, and Ménière
morbidity, the lack of effective treatments, and the wide range of causes. disease can increase
susceptibility to motion
sickness. Motion sickness
Clinical Syndrome
susceptibility can increase
Bilateral vestibulopathy refers to significant impairment of both peripheral with vestibular neuritis but
vestibular systems. It may occur with or without hearing loss. The typical patient return to normal if the
with bilateral vestibular loss experiences chronic unsteadiness in the upright vestibular paresis is
compensated. Individuals
position that is absent when sitting or lying down. Imbalance is particularly severe in
with bilateral vestibulopathy
low-light settings and walking on uneven surfaces when other body position– have very low motion
orienting sensory inputs (eg, vision, somatosensory) are challenged (CASE 6-4).53 sickness susceptibility.
Patients may report that their “eyes are not keeping up with my head,” which
relates to impaired conduction of head acceleration information through a ● Habituation exercises,
medications (antimuscarinic,
slowed vestibulo-ocular reflex (VOR). Oscillopsia (“bouncing vision”) with anticholinergic,
walking or blurred vision with head movement may occur. Even though antihistaminergic, or
oscillopsia is most severe with walking, milder symptoms may be present in the diazepam), controlled
seated position and induced by a strong heartbeat or head movement during breathing, music, or
pleasant smells can modify
speaking or chewing. Patients may prefer to keep their hand on their chin to motion sickness severity.
stabilize the head during these situations.
Imbalance can be compounded by the loss of vestibulospinal reflexes that are ● Core symptoms of
critical in translating head position information into spinal reflexes that bilateral vestibulopathy
include gait unsteadiness,
maintain balance in the upright position. Chronic loss of vestibular input can also
postural instability, visual
lead to degeneration of central vestibular projections to the hippocampus that blurring with head
are involved in spatial navigation.54 movement, and sometimes
Reduced ability to stabilize vision from a weakened VOR, diminished postural oscillopsia.
reflexes from a weakened vestibulospinal reflex, and degraded spatial orientation
● Bilateral vestibulopathy
sense from reduced vestibular input to the hippocampus can synergistically can be diagnosed by
worsen balance impairment and risk for falls in bilateral vestibulopathy. rotational chair testing,
caloric irrigation, or video
Criteria head impulse testing; the
most reliable method is
Diagnostic criteria for bilateral vestibulopathy were established by the Bárány rotational chair testing.
Society in 2017 and are presented in TABLE 6-3.55 Individuals with bilateral
vestibulopathy are diagnosed by a combination of symptoms as well as diagnostic
vestibular function metrics. Because the angular vestibular ocular system
operates at different velocities and testing procedures are tuned for different
frequencies, each testing procedure has different criteria. Video head impulse
testing is tuned for high-acceleration head movements, rotational chair testing
detects medium-frequency head movements, and caloric testing detects
very-low-frequency activity. In recognition of these differences, the criteria for
definite bilateral vestibulopathy as set forth by the Bárány Society allow for a
diagnosis based on any of these measurements. A diagnosis of probable bilateral
vestibulopathy can be made if the clinical symptoms are met and only an abnormal
bedside head impulse testing response is present.
Rotational chair testing is the most physiologic method of assessing bilateral
vestibulopathy because both vestibular systems are tested at the same time. It is
not affected by structural issues such as a narrowed external ear canal, a perforated
eardrum, middle ear fluid, or a temporal bone abnormality (all of these
abnormalities can affect caloric irrigation studies). A reduced gain of the VOR

CONTINUUMJOURNAL.COM 433

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

CASE 6-4 A 70-year-old man had a history of Ménière disease about 20 years prior
that had left him with poor hearing and a compensated vestibular deficit
in his right ear. One morning, he woke up with severe rotational vertigo,
left ear tinnitus, and aural fullness. The symptoms lasted about 5 hours.
Over the next year, he had three more episodes of similar vertigo. He
noticed gradually reduced balance function in between each spell of
vertigo. In particular, he noticed difficulty walking at night. He also felt
that when he turned his head quickly, his vision had a short lag catching
up to his head movement. He noted that his vision kept “bouncing”
whenever he walked.
His examination was remarkable for normal visual acuity in both eyes.
Hearing was absent in the right ear. He could detect conversational
speech in the left ear but required frequent repeating. Bilateral catch-up
saccades were present on the head impulse maneuver. His baseline gait
was widened, and he had a positive Romberg sign.
He started a vestibular rehabilitation program to help with the visual
blurring, was referred to otorhinolaryngology for a cochlear implant
evaluation, and was counseled on taking additional precautions when
walking at night or on uneven surfaces.

COMMENT Because each peripheral vestibular system can detect motion in both
directions, the loss of one vestibular system can be compensated for
readily. However, when both peripheral vestibular systems are damaged,
leading to bilateral vestibulopathy, the functional consequences can be
severe. The dysfunction occurs because of the loss of the vestibulo-ocular
reflex that drives compensatory eye movements for head motion and
vestibulospinal reflexes that adjust posture for head motion. Thus,
symptoms of bilateral vestibulopathy include imbalance, visual lag, and
oscillopsia when severe. Bilateral vestibulopathy has many causes, such as
sequential inner ear dysfunction, which can occur in Ménière disease, like
in the case above. It can also happen with sequential vestibular neuritis,
vestibulotoxic medications, or infiltration of the inner ear space from
contents of the intracranial space such as blood (eg, subarachnoid
hemorrhage), inflammatory cells (eg, meningitis), or cancerous cells (eg,
carcinomatous meningitis). It can occur idiopathically, in combination with
other peripheral neuropathies, or in CANVAS (cerebellar ataxia,
neuropathy, vestibular areflexia syndrome). When some residual vestibular
function remains, vestibular therapy can be helpful, but the mainstay of
treatment is to protect other systems that contribute to postural control
(ie, vision, proprioception, cognition, physical conditioning) and to be
aware of situations that challenge these other pathways and create
dangerous circumstances (eg, walking in low light).

434 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

(eye movement velocity relative to chair movement velocity in sinusoidal harmonic
acceleration) and a phase lead of eye position relative to chair position are
indicative of vestibular impairment. Caloric testing is the most widely available
test of inner ear function but is the least relevant because activation of one vestibular
system at a time with a temperature stimulus is not physiologic and only corresponds
to a natural stimulus frequency of 0.003 Hz. It can also be limited by structural
abnormalities of the external and middle ears, as noted earlier. Abnormal caloric
testing that is suggestive of bilateral vestibulopathy should be verified with
rotational testing because spuriously low-caloric-induced responses are common.
Video head impulse testing has gained in use in some clinical contexts, but the
limitations in reimbursement for incorporating video head impulse testing into a
regular vestibular function testing battery has curtailed more widespread clinical use.
Bedside screening for bilateral vestibulopathy can be done by either performing
the head impulse test or checking dynamic visual acuity. The head impulse test
maneuver is a test of the VOR. During a head impulse test, the patient’s head is
held by the examiner with both hands and the patient is instructed to focus on a
point on the examiner, such as his or her nose. The patient’s head is then thrust to
either side with short-excursion (10 degrees) high-acceleration movements. If the
VOR is intact, the patient’s eyes should be focused on the examiner at all times. If
the VOR is impaired, the patient’s eyes will passively follow the head movement
during testing due to slowed conduction through the vestibular system. The
patient will then make a corrective saccade back to midline to refocus on the
examiner. The head thrusts must be done in a random manner so that the patient
does not make predictive saccades ahead of the corrective saccade. The head
impulse test is most sensitive for new-onset vestibular dysfunction and for severe

Diagnostic Criteria for Bilateral Vestibulopathya,b TABLE 6-3

A Chronic vestibular syndrome with the following symptoms:

1 Unsteadiness when walking or standing plus at least one of 2 or 3 below
2 Movement-induced blurred vision or oscillopsia during walking or quick head/body
movements and/or
3 Worsening of unsteadiness in darkness and/or on uneven ground
B No symptoms while sitting or lying down under static conditions
C Bilaterally reduced or absent angular vestibulo-ocular reflex (VOR) function documented by:
Bilaterally pathologic horizontal angular VOR gain <0.6, measured by the video head impulse
test or scleral-coil technique and/or
Reduced caloric response (sum of bithermal maximum peak slow phase velocity on each
side <6 degrees per second) and/or
Reduced horizontal angular VOR gain <0.1 upon sinusoidal stimulation on a rotatory chair
(0.1 Hz, maximum velocity = 50 degrees per second) and a phase lead >68 degrees (time
constant <5 seconds)
D Not better accounted for by another disease

a
Modified with permission from Strupp M, et al, J Vestib Res.55 © 2017 IOP Press and the authors.
b
Probable bilateral vestibulopathy may be diagnosed if criteria A, B, and D are met but only if the pathologic
horizontal bedside head impulse test is abnormal and no laboratory testing is available.

CONTINUUMJOURNAL.COM 435

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

deficits in which the angular VOR gain is less than 0.4.56 The overall sensitivity
(84%) and specificity (82%) are fairly good for diagnosing bilateral
vestibulopathy.57 Over time, however, the corrective saccade can occur so early
that it partially overlaps with the head thrust maneuver and is thus not visible to
the naked eye.58,59 Therefore, when suspicion for a vestibulopathy is high but the
head thrust maneuver appears to be normal, follow-up with video head impulse
testing or rotational testing should be performed.
Dynamic visual acuity can be checked at the bedside to screen for bilateral
vestibulopathy. The patient is asked to read a Snellen chart with both eyes open,

TABLE 6-4 Etiologies of Bilateral Vestibulopathy

Primary inner ear

◆ Sequential or concurrent bilateral Ménière disease
◆ Sequential or concurrent bilateral labyrinthitis
◆ Sequential vestibular neuritis (rare)
Infectious
◆ Meningitis
◆ Encephalitis
◆ Cerebellitis
◆ Note common infectious agents: herpes simplex, varicella, mumps, Treponema pallidum
(syphilis), Borrelia burgdorferi (Lyme disease), Creutzfeldt-Jakob disease
Autoimmune
◆ Cogan syndrome
◆ Susac syndrome
◆ Antineutrophil cytoplasmic antibody–associated vasculitides
◆ Polyarteritis nodosa
◆ Relapsing polychondritis
◆ Neuro Behçet disease
◆ Neurosarcoidosis
◆ Sjögren syndrome
◆ Systemic lupus erythematosus
◆ Antiphospholipid syndrome
◆ Graves and Hashimoto thyroid disease
◆ Vogt-Koyanagi-Harada syndrome
Genetic
◆ Neurofibromatosis 2
◆ CANVAS (cerebellar ataxia, neuropathy, vestibular areflexia syndrome)
◆ COCH (DFNA9), MYO7A (DFNA11), ESPN (DFNB36), PTPRQ (DFNB84A)
◆ Spinocerebellar ataxia 3

CONTINUED ON PAGE 437

436 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

and the best level of acuity is assessed. The patient’s head is then passively
oscillated at 2 Hz, and acuity is reassessed. Losing more than two lines of acuity
suggests an impaired VOR with specificity going up with more lines of acuity
lost.60

Causes
Bilateral vestibulopathy may be idiopathic in 20% to 50% of cases or may be
caused by a variety of identifiable inner ear–specific or systemic disorders that
involve the vestibular system (TABLE 6-4 and TABLE 6-5).61–69 Intracranial

CONTINUED FROM PAGE 436

◆ Spinocerebellar ataxia 6
◆ Episodic ataxia 2
◆ Hereditary sensorimotor neuropathy
◆ MELAS (mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes)
Degenerative
◆ Superficial siderosis
◆ Multiple system atrophy
Toxic/metabolic
◆ Medication-related ototoxicity (see TABLE 6-5)
◆ Wernicke encephalopathy (partially reversible)
◆ Vitamin B12/folate deficiency
◆ Hypothyroidism
◆ Phenobarbital, phenytoin
◆ Miscellaneous: styrene, Jet Propellant-8 jet fuel, butyl nitrate, carbon disulfide, lead,
mercury, manganese, tin
Oncologic
◆ Vestibular schwannomas
◆ Carcinomatosis–metastatic disease
◆ Paraneoplastic syndromes (anti-Yo, anti-Hu)
◆ Lymphoma
Congenital
◆ Usher syndrome
◆ Pendred syndrome
◆ Enlarged vestibular aqueduct syndrome
◆ CHARGE (coloboma of the eye, heart defects, atresia of the choanae, renal abnormalities
and retardation of growth and/or development, genital abnormalities, and ear
abnormalities) syndrome
◆ Other congenital malformations (eg, Mondini dysplasia)
Iatrogenic
◆ Surgical vestibular injury (eg, cochlear implant)

CONTINUUMJOURNAL.COM 437

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

processes that involve blood, inflammatory mediators, tumor cells, or iron in the
subarachnoid space can lead to passage of these elements through the cochlear
and vestibular aqueducts into the inner ear, leading to hearing loss and vestibular
dysfunction. Metabolic abnormalities (eg, deficiencies in vitamin B1, vitamin B12,
folate, thyroid) can affect both the peripheral and central vestibular systems.
Thus, although bilateral vestibulopathy is defined by its peripheral vestibular
diagnostic abnormalities, it can be associated with systemic and central nervous
system disorders with all contributing to balance dysfunction.
Vestibulotoxicity from aminoglycoside antibiotics should be suspected when
an insidious onset of balance problems occurs after their use (TABLE 6-5). Of
particular relevance is gentamicin, which is strongly vestibulotoxic and widely
used to treat gram-negative bacterial infections for prolonged periods of time.
The lack of auditory warning symptoms (eg, tinnitus or hearing loss), the
systemic accumulation of gentamicin with prolonged use, and concurrent
nephrotoxicity have been recognized as contributors to the high risk of
gentamicin-related vestibular damage.69 Other aminoglycosides to be aware of
include streptomycin and amikacin, which are used to treat tuberculosis, and
tobramycin, which is used to treat infections common in cystic fibrosis
(eg, Pseudomonas aeruginosa).70 Commonly used antibiotics in the penicillin,
cephalosporin, macrolide, or fluoroquinolone classes are rarely vestibulotoxic.71
Several other drug classes are known to cause ototoxicity but cause significantly
greater hearing loss and tinnitus than vestibular loss. These drug classes

TABLE 6-5 Vestibulotoxicity of Antibiotics

Strongly vestibulotoxic
◆ Gentamicin
◆ Streptomycin
◆ Tobramycina
Weakly vestibulotoxic
◆ Neomycina
◆ Kanamycina
◆ Amikacina
◆ Netilmicin
◆ Vancomycin
Rarely vestibulotoxic
◆ Penicillins
◆ Cephalosporins
◆ Macrolides
◆ Fluoroquinolones
◆ Metronidazole

a
Also cochleotoxic.

438 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

include loop diuretics, salicylates, quinine, cisplatin (which causes permanent KEY POINTS
hearing loss), carboplatin, paclitaxel, and nitrogen mustard.
● Bilateral vestibulopathy
Regular screening for vestibulopathy, such as with the head impulse, the video may occur after sequential
head impulse, or dynamic visual acuity testing, should be done for a patient inner ear injury such as from
who is put on repeated doses of vestibulotoxic medications. Because some Ménière disease, vestibular
vestibulotoxic medications can cause concurrent peripheral neuropathy and neuritis, or vestibular
schwannomas, from
exacerbate imbalance (eg, platinum-based chemotherapeutics), vigilance should
extension of intracerebral
be particularly high in patients receiving these agents. Other medications such as processes such as
vincristine, nitrogen mustard, loop diuretics, salicylates, and nonsteroidal meningitis, carcinomatosis,
anti-inflammatory drugs are also potentially ototoxic but generally cause more or other processes in the
subarachnoid space into the
hearing loss and tinnitus than vestibular dysfunction and are thus more
inner ear, or secondary to
readily detected as a cause for ototoxicity. vestibulotoxic medications
In 2011, a disorder called CANVAS (cerebellar ataxia, neuropathy, vestibular such as aminoglycoside
areflexia syndrome) was described in 27 patients with late-onset cerebellar antibiotics.
ataxia, bilateral vestibulopathy, and a non–length-dependent sensory
● Vestibular rehabilitation,
neuropathy.72 Histopathology showed cerebellar Purkinje cell and dorsal root protecting vision, and
ganglion loss. Clinical cerebellar abnormalities included the presence of avoiding deconditioning are
downbeat and gaze-evoked nystagmus, poor visual suppression of the VOR, helpful in reducing
rebound nystagmus, saccadic dysmetria, and greater axial compared with limb morbidity from bilateral
vestibulopathy. Safety
dysmetria. Brain MRI showed atrophy of the vermis as well as crus I of the measures should emphasize
cerebellar hemisphere. Most cases of CANVAS present later in life (in the sixth care in low-light settings.
decade), but the range of age at onset of initial symptoms has subsequently
been reported to be as wide as 19 to 76 years.73 The earliest symptom is a chronic
cough, with unsteadiness, sensory loss, and dysautonomia as subsequent
symptoms. The phenotype can be incomplete and the order of onset of symptoms
variable among patients, however. An autosomal recessive inheritance pattern
was confirmed to be a biallelic pentameric repeat expansion (AAGGG) that was
observed in 90% of cases in which all three systems were involved (sensory,
vestibular, cerebellar) and in 14% of cases in which the phenotype was incomplete.
Of the individuals for whom the clinical information was known, the sex
distribution was 55% female and 45% male; 45% of the cases were familial, and 55%
were sporadic. All cases were in patients of Caucasian origin, which raised the
possibility of a commonly shared distant ancestor.73

Time Course
Patients with inner ear disorders, such as Ménière disease or vestibular neuritis,
may do fine with a unilateral vestibulopathy because one vestibular system can
detect head motion in both directions, but if the second inner ear function is lost,
the clinical effects can be quite devastating. Vestibulopathy can also occur
insidiously and may be asymptomatic until significant dysfunction is present. This is
particularly true for idiopathic cases and those occurring after exposure to ototoxic
medications.

Treatment
Currently, no standardized treatments can reverse damage to the inner ear due to
the causes of bilateral vestibulopathy. Intensive research on vestibular implants
is in preliminary stages at the time of this writing.74 Patients with bilateral
vestibulopathy may respond well to vestibular rehabilitation measures, especially
if they have some residual vestibular function left. Protecting vision, treating
potential causes of peripheral sensory loss, staying cognitively intact, and

CONTINUUMJOURNAL.COM 439

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

maintaining good muscle tone are important for reducing morbidity from
bilateral vestibulopathy. Patients should also be warned not to swim alone
because they can become easily disoriented when their eyes are closed and may
swim the wrong way. They should be particularly vigilant in low-light settings
in all circumstances.

PERSISTENT DIZZINESS AFTER MILD CONCUSSION

This section reviews the clinical features, time course, and treatment for
persistent dizziness after a mild concussion. This clinical syndrome represents a

CASE 6-5 A 20-year-old woman who played collegiate hockey was knocked
particularly hard against the wall of the ice rink and felt a bit dazed after
the impact. She continued to play the rest of the game but felt that
she couldn’t quite predict the position of the puck as well as she used to.
In the days after the game, she developed a persistent headache,
difficulty with shifting from reading the blackboard to reading her
notebook, and a feeling of lightheadedness and “wooziness” when
running. When she was particularly tired, she noticed slight double vision.
She had a difficult time watching movies with her friends because the
motion on the screen made her feel like she was moving.
She saw a neurologist when she was still symptomatic after a month.
On examination, she had a slight slowness of response to questions but
with normal informational content. Visual acuity was normal, but she had
a convergence insufficiency. Hearing and head impulse testing were
normal. Baseline gait was normal, but she had difficulty balancing on one
foot. This was abnormal given her age and high baseline athleticism. She
did not develop vertigo or nystagmus on positional (Dix-Hallpike) testing.
Her neurologist started her on nortriptyline for the headaches and
referred her for visual and vestibular rehabilitation therapy. The
vestibular therapist started her on a graduated exercise program of
increasing head movement and physical exertion. An optometrist with
experience in vision rehabilitation helped her with eye muscle–
strengthening exercises. She stayed off the ice that season but did train in
the gym. Her symptoms gradually improved over the next 3 months until
she was able to play hockey again.

COMMENT Although concussions are otherwise known as mild traumatic brain injury,
the symptoms can be quite debilitating. Benign paroxysmal positional
vertigo, vestibular migraine, exertional dizziness, spatial disorientation, and
visual dysfunction can follow concussions. Severe structural injury to the
inner ear is uncommon after concussions, but a screen for benign
paroxysmal positional vertigo with the Dix-Hallpike test should always be
performed after a concussion because it is common and treatable.
Vestibular rehabilitation, ocular motor rehabilitation, treatment of
headaches, and graduated return to activity are the mainstays of
treatment.

440 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

major public health issue because it affects every age group, has a wide number KEY POINT
of potential causes, and can manifest with a variety of both visually triggered
● Postconcussion dizziness
and head motion–triggered symptoms. includes categories such as
positional vertigo, exertional
Clinical Syndrome dizziness, vestibular
The term concussion refers to mild traumatic brain injury in which biomechanical migraine, spatial
insult from blunt head trauma leads to brain dysfunction. Blunt head trauma occurs disorientation, and visual
disorders.
in combat, sports, whiplash, motor vehicle accidents, altercations, falls, and various
accidents. Dizziness is a frequent symptom of concussion, affecting between 59%
and 98% of patients depending on the mechanism and time since injury; it can
accompany headaches, cognitive dysfunction, emotional dysregulation, and
ocular-motor problems.75,76 Concussions can lead to both peripheral and central
vestibular dysfunction as well as dizziness due to a variety of ocular and visual
processing problems (CASE 6-5). Penetrating head injuries or blast injuries are
more likely to be associated with hemorrhages and structural brain problems,
causing additional symptoms that are beyond the scope of this article.
The most common categories of dizziness after concussion are positional
vertigo, exertional dizziness, vestibular migraine, spatial disorientation,
and dizziness secondary to visual disorders.

POSITIONAL VERTIGO. BPPV can occur after a concussion due to mechanical

dislodgement of otoliths from the utricle and can be present in up to 61% of
individuals after head trauma.77 This results in typical BPPV as occurs from
nontraumatic causes. However, when BPPV follows head trauma, care should be
taken to rule out bilateral and multiple canal involvement. BPPV is treated
with the canalith repositioning maneuver appropriate to each canal (ie, the Epley
head-hanging roll maneuver for posterior canal and the Lempert roll maneuver
for horizontal canal).

EXERTIONAL DIZZINESS. Patients with concussion can experience dizziness during

and after exercise. This is treated with graduated increases in exercise duration
to build up tolerance.

VESTIBULAR MIGRAINE. Headache is one of the most frequent symptoms of

concussions and can be associated with light and sound sensitivity and dizziness.
General migraine therapies are effective (adequate sleep, exercise, stress
moderation, healthy diet) as well as vestibular rehabilitation. Preventative
migraine medications may be added in cases that become chronic.

SPATIAL DISORIENTATION. This symptom is typically the most protracted because it

can be associated with abnormalities in the VOR. Patients can experience
unsteadiness of gait, postural instability, and even symptoms when they are lying
down. Gentle graded vestibular therapy is the mainstay of therapy, but
symptoms can become chronic (ie, lasting more than 3 months).

VISUAL DISORDERS. Patients who develop ocular or visual-processing dysfunction

after concussion may describe their symptoms as “dizziness.” The most
common concussion-related visual disorders include convergence insufficiency
(inability to move eyes inward, required for near work), accommodation
dysfunction (inability to flexibly change focus), stereopsis (depth perception),
and heterophorias (latent misalignment of the visual axes). Higher-order visual

CONTINUUMJOURNAL.COM 441

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

processing can be affected in concussions because much of the cerebral cortex

is dedicated to visual function or ocular motility. After concussion, basic
problems with smooth pursuit and saccadic control can occur, leading to
problems with reading and a propensity for eyestrain. Susceptibility to visually
induced dizziness and visually induced motion sickness can occur, as well as
reduced figure-ground segregation ability (difficulty separating visual targets
of interest from the background). With sufficient head trauma, more severe
ocular motility disorders caused by injury or entrapment of the oculomotor,
trochlear, or abducens nerves can lead to diplopia.78
Mild traumatic brain injury is typically not associated with severe structural
damage to the inner ear, but concern should be raised when concussions are
associated with severe prolonged spells of vertigo or hearing loss. In these cases,
the following less common but more serious disorders should be considered.

SUPERIOR CANAL DEHISCENCE. Superior canal dehiscence is a disorder in which a

likely congenitally thin roof of the superior (also called anterior) semicircular
canal that separates the labyrinth from the intracranial space is dehisced. About
half of cases of superior canal dehiscence occur after head trauma or barotrauma.
Superior canal dehiscence symptoms include sound-induced spells of vertigo
(ie, Tullio phenomenon), conductive and/or sensorineural hearing loss, tinnitus,
and autophony. In rare cases, superior canal dehiscence can be caused by
fracture of the temporal bone, but this is usually in the setting of severe head
injury rather than concussion.79

PERILYMPHATIC FISTULAS. Perilymphatic fistulas are difficult to diagnose but

should be suspected when head trauma leads to sensorineural hearing loss,
vertigo, and tinnitus. They occur when the inner ear is connected to the middle
ear through a ruptured oval or round window.80
ENDOLYMPHATIC HYDROPS. Primary endolymphatic hydrops is referred to as
Ménière disease. Secondary or delayed endolymphatic hydrops can occur after
head trauma when the force is strong enough to disrupt the membranous
labyrinth and affect fluid pressure. Delayed endolymphatic hydrops
symptoms include vertigo spells associated with hearing loss, tinnitus, and
aural pressure.81

Criteria
Efforts are undergoing to categorize the different components of posttraumatic
dizziness into symptom domains, and these categories may help focus therapy.

Causes
Concussion-related dizziness is diagnosed by the temporal profile of symptoms
following the acute event, keeping in mind that multiple concurrent
contributors to symptoms may be present. Unwitnessed events or associations
made with a long temporal delay can make attribution of chronic symptoms
difficult, however. Some symptoms from concussion may not be apparent at the
time of injury but may occur after a period of posttraumatic inflammation.

Time Course
Balance problems generally resolve in a few days after a concussion, but
abnormalities in the VOR predict longer recovery.82 Dizziness that occurs

442 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

immediately upon impact, the presence of migraine headaches, preexisting KEY POINTS
mood or anxiety disorders, and impending litigation are predictors of slower
● Concurrent ocular motor
recovery.83 Symptoms that last beyond 4 weeks are considered part of the dysfunction and visual
postconcussive syndrome, and the most difficult vestibular symptoms to treat processing disorders may
are persistent spatial disorientation and unsteadiness. occur with postconcussion
dizziness and can
significantly add to
Treatment
morbidity.
After a concussion, all patients should undergo positional testing for BPPV, a
screen for auditory dysfunction, and an assessment for migraine headaches. ● It is important to rule out
Treatment should be tailored for these initial high-yield areas. Structural injury to structural injury to the inner
the inner ear is uncommon, but red flags such as sound-induced vertigo and ear after head trauma,
particularly if severe vertigo
hearing loss should be ruled out. In most cases, concussions will not be associated or concurrent hearing loss is
with any measurable abnormalities despite severe symptoms. Vestibular present.
rehabilitation should be offered to patients with persistent symptoms,
particularly those with abnormalities in the VOR.84,85 An optometrist trained in ● Graded vestibular
rehabilitation is generally
vision rehabilitation can help with ocular motility issues. Concussions can lead required for postconcussive
to complicated symptoms, including cognitive slowing, irritability, and chronic dizziness along with a
pain, all of which can impact recovery from dizziness. These additional multipronged approach to
symptoms can require management assistance from other specialties. address concurrent
cognitive slowing,
headache, anxiety, and
mood dysregulation.
CONCLUSION
Chronic dizziness can be caused by homeostatic perturbations, prolonged motion
exposure, concussion, impaired vestibular function, or innate difficulties with
habituation to motion. Although symptoms may initially seem intractable,
methodically identifying modifiable factors can make untenable problems more
manageable. Many of these chronic disorders can be treated with education,
lifestyle modifications, vestibular rehabilitation, serotonergic antidepressants,
and treatment of concurrent maladaptive thought processes. Giving patients a
clear diagnosis, educating them on the nature of these disorders, and
communicating reasonable expectations for the tempo of recovery are critical
factors in the successful management of chronic dizziness.

REFERENCES

1 Bisdorff A, Von Brevern M, Lempert T, 4 Brandt T. Phobic postural vertigo. Neurology

Newman-Toker DE. Classification of vestibular 1996;46(6):1515-1519. doi:10.1212/wnl.46.6.1515
symptoms: towards an international
5 Popkirov S, Staab JP, Stone J. Persistent
classification of vestibular disorders. J Vestib Res
postural-perceptual dizziness (PPPD): a
2009;19(1–2):1-13. doi:10.3233/VES-2009-0343
common, characteristic and treatable cause of
2 Staab JP, Eckhardt-Henn A, Horii A, et al. chronic dizziness. Pract Neurol 2018;18(1):5-13.
Diagnostic criteria for persistent doi:10.1136/practneurol-2017-001809
postural-perceptual dizziness (PPPD): consensus
6 Staab JP. Persistent postural-perceptual
document of the committee for the
dizziness. Semin Neurol 2020;40(1):130-137.
Classification of Vestibular Disorders of the
doi:10.1055/s-0039-3402736
Bárány Society. J Vestib Res 2017;27(4):191-208.
doi:10.3233/VES-170622 7 Nada EH, Ibraheem OA, Hassaan MR. Vestibular
rehabilitation therapy outcomes in patients
3 Bronstein AM. The visual vertigo syndrome.
with persistent postural-perceptual dizziness.
Acta Otolaryngol Suppl 1995;520 pt 1:45-8.
Ann Otol Rhinol Laryngol 2019;128(4):323-329.
doi:10.3109/00016489509125186
doi:10.1177/0003489418823017

CONTINUUMJOURNAL.COM 443

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

8 Godemann F, Siefert K, Hantschke-Bruggemann 23 Cha YH, Urbano D, Pariseau N. Randomized single

M, et al. What accounts for vertigo one year after blind sham controlled trial of adjunctive
neuritis vestibularis - anxiety or a dysfunctional home-based tDCS after rTMS for mal de
vestibular organ? J Psychiatr Res 2005;39(5): debarquement syndrome: safety, efficacy, and
529-34. doi:10.1016/j.jpsychires.2004.12.006 participant satisfaction assessment. Brain Stimul
2016;9(4):537-544. doi:10.1016/j.brs.2016.03.016
9 Hallam RS, Hinchcliffe R. Emotional stability; its
relationship to confidence in maintaining 24 Cha YH, Deblieck C, Wu AD. Double-blind
balance. J Psychosom Res 1991;35(4-5):421-30. sham-controlled crossover trial of repetitive
transcranial magnetic stimulation for mal de
10 Kroenke K, Lucas CA, Rosenberg ML, et al.
debarquement syndrome. Otol Neurotol 2016;
Causes of persistent dizziness. A prospective
37(6):805-812. doi:10.1097/MAO.
study of 100 patients in ambulatory care.
0000000000001045
Ann Intern Med 1992;117(11):898-904.
doi:10.7326/0003-4819-117-11-898 25 Golding JF. Motion sickness. Handb Clin Neurol
2016;137:371-390. doi:10.1016/B978-0-444-
11 Yardley L, Beech S, Weinman J. Influence of
63437-5.00027-3
beliefs about the consequences of dizziness on
handicap in people with dizziness, and the effect 26 Keshavarz B, Hecht H, Lawson BH. Visually
of therapy on beliefs. J Psychosom Res 2001; induced motion sickness: characteristics,
50(1):1-6. doi:10.1016/S0022-3999(00)00202-6 causes, and countermeasures. In: Hale KS,
Stanney KM, editors. Handbook of virtual
12 Cha YH. Mal de debarquement. Semin Neurol
environments: design, implementation, and
2009;29(5):520-527. doi:10.1055/s-0029-1241038
applications. 2nd ed. Boca Raton, FL: CRC Press,
13 Cha YH, Cui YY, Baloh RW. Comprehensive 2014:648-697.
clinical profile of mal de debarquement
27 Lawson BD, Mead AM. The sopite syndrome
syndrome. Front Neurol 2018;9:261.
revisited: drowsiness and mood changes during
doi:10.3389/fneur.2018.00261
real or apparent motion. Acta Astronaut 1998;
14 Cha YH, Cui Y. Rocking dizziness and headache: a 43(3-6):181-192. doi:10.1016/s0094-5765(98)00153-2
two-way street. Cephalalgia 2013;33(14):1160-1169.
28 Keshavarz B, Riecke BE, Hettinger LJ, Campos JL.
doi:10.1177/0333102413487999
Vection and visually induced motion sickness:
15 Lempert T, Olesen J, Furman J, et al. Vestibular how are they related? Front Psychol 2015;6:472.
migraine: diagnostic criteria. J Vestib Res doi:10.3389/fpsyg.2015.00472
2012;22(4):167-172. doi:10.3233/VES-2012-0453
29 Henriques IF, Douglas de Oliveira DW,
16 Cha YH, Baloh RW, Cho C, et al. Mal de Oliveira-Ferreira F, Andrade PM. Motion sickness
debarquement syndrome diagnostic criteria. prevalence in school children. Eur J Pediatr
Consensus document of the Classification 2014;173(11):1473-1482. doi:10.1007/s00431-014-2351-1
Committee of the Barany Society [published
30 Hromatka BS, Tung JY, Kiefer AK, et al. Genetic
online September 22, 2020]. J Vestib Res.
variants associated with motion sickness point to
doi:10.3233/VES-200714
roles for inner ear development, neurological
17 Gordon CR, Spitzer O, Doweck I, et al. Clinical processes and glucose homeostasis. Hum Mol
features of mal de debarquement: adaptation Genet 2015;24(9):2700-2708. doi:10.1093/hmg/
and habituation to sea conditions. J Vestib Res ddv028
1995;5(5):363-369.
31 Paillard AC, Quarck G, Paolino F, et al. Motion
18 Nachum Z, Shupak A, Letichevsky V, et al. Mal de sickness susceptibility in healthy subjects and
debarquement and posture: reduced reliance on vestibular patients: effects of gender, age and
vestibular and visual cues. Laryngoscope 2004; trait-anxiety. J Vestib Res 2013;23(4-5):203-209.
114(3):581-586. doi:10.1097/00005537- doi:10.3233/VES-130501
200403000-00036
32 Kawano N, Iwamoto K, Ebe K, et al. Slower
19 Cohen H. Mild mal de debarquement after adaptation to driving simulator and simulator
sailing. Ann N Y Acad Sci 1996;781:598-600. sickness in older adults. Aging Clin Exp Res 2012;
doi:10.1111/j.1749-6632.1996.tb15734.x 24(3):285-289. doi:10.1007/BF03325260

20 Cha YH, Brodsky J, Ishiyama G, et al. Clinical 33 Golding JF, Kadzere P, Gresty MA. Motion
features and associated syndromes of mal de sickness susceptibility fluctuates through the
debarquement. J Neurol 2008;255(7):1038-1044. menstrual cycle. Aviat Space Environ Med 2005;
doi:10.1007/s00415-008-0837-3 76(10):970-973.
21 Cha YH, Gleghorn D, Doudican B. Occipital and 34 Turner M, Griffin MJ. Motion sickness incidence
cerebellar theta burst stimulation for mal de during a round-the-world yacht race. Aviat
debarquement syndrome. Otol Neurotol 2019; Space Environ Med 1995;66(9):849-856.
40(9):e928-e937. doi:10.1097/MAO.
35 Golding JF, Gresty MA. Motion sickness.
0000000000002341
Curr Opin Neurol 2005;18(1):29-34.
22 Dai M, Cohen B, Smouha E, Cho C. Readaptation doi:10.1097/00019052-200502000-00007
of the vestibulo-ocular reflex relieves the mal de
debarquement syndrome. Front Neurol 2014;
5:124. doi:10.3389/fneur.2014.00124

444 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

36 Cha YH, Golding JF, Keshavarz B, et al. Motion 50 Keshavarz B, Hecht H. Pleasant music as a
sickness: diagnostic criteria. consensus countermeasure against visually induced motion
document of the Classification Committee of the sickness. Appl Ergon 2014;45(3):521-527.
Barany Society. J Vestib Res. Forthcoming 2021. doi:10.1016/j.apergo.2013.07.009
37 Kennedy R, Lane N, Berbaum K, Lilienthal MG. 51 Keshavarz B, Stelzmann D, Paillard A, Hecht H.
Simulator sickness questionnaire: an enhanced Visually induced motion sickness can be
method for quantifying simulator sickness. Int J alleviated by pleasant odors. Exp Brain Res 2015;
Aviat Psychol 1993;3:203-220. doi:10.1207/ 233(5):1353-1364. doi:10.1007/s00221-015-4209-9
s15327108ijap0303_3
52 Yen Pik Sang FD, Golding JF, Gresty MA.
38 Gianaros PJ, Muth ER, Mordkoff JT, et al. A Suppression of sickness by controlled breathing
questionnaire for the assessment of the multiple during mildly nauseogenic motion. Aviat Space
dimensions of motion sickness. Aviat Space Environ Med 2003;74(9):998-1002.
Environ Med 2001;72(2):115-119.
53 Strupp M, Feil K, Dieterich M, Brandt T. Bilateral
39 Muth ER, Stern RM, Thayer JF, Koch KL. vestibulopathy. Handb Clin Neurol 2016;137:
Assessment of the multiple dimensions of 235-240. doi:10.1016/B978-0-444-63437-5.00017-0
nausea: the Nausea Profile (NP). J Psychosom
54 Göttlich M, Jandl NM, Sprenger A, et al.
Res 1996;40(5):511-520. doi:10.1016/0022-3999(95)
Hippocampal gray matter volume in bilateral
00638-9
vestibular failure. Hum Brain Mapp 2016;37(5):
40 Bos JE, MacKinnon SN, Patterson A. Motion 1998-2006. doi:10.1002/hbm.23152
sickness symptoms in a ship motion simulator:
55 Strupp M, Kim JS, Murofushi T, et al. Bilateral
effects of inside, outside, and no view. Aviat
vestibulopathy: diagnostic criteria consensus
Space Environ Med 2005;76(12):1111-1118.
Document of the Classification Committee of
41 Keshavarz B, Hecht H. Validating an efficient the Bárány Society. J Vestib Res 2017;27(4):
method to quantify motion sickness. 177-189. doi:10.3233/VES-170619
Hum Factors 2011;53(4):415-426. doi:
56 Yip CW, Glaser M, Frenzel C, et al. Comparison of
10.1177/0018720811403736
the bedside head-impulse test with the video
42 Golding JF. Motion sickness. Predicting individual head-impulse test in a clinical practice setting: a
differences in motion sickness susceptibility by prospective study of 500 outpatients. Front
questionnaire. Person Individ Diff 2006;41: Neurol 2016;7:58. doi:10.3389/fneur.2016.00058
237-248. doi:10.1016/j.paid.2006.01.012
57 Schubert MC, Tusa RJ, Grine LE, Herdman SJ.
43 Reason JT. Motion sickness adaptation: a neural Optimizing the sensitivity of the head thrust test
mismatch model. J R Soc Med 1978;71(11):819-829. for identifying vestibular hypofunction. Phys
Ther 2004;84(2):151-158. doi:10.1093/ptj/84.2.151
44 Oman CM. Are evolutionary hypotheses for
motion sickness “just-so” stories? J Vestib Res 58 Weber KP, Aw ST, Todd MJ, et al. Head impulse
2012;22(2):117-127. doi:10.3233/VES-2011-0432 test in unilateral vestibular loss: vestibulo-ocular
reflex and catch-up saccades. Neurology 2008;
45 Murdin L, Chamberlain F, Cheema S, et al. Motion
70(6):454-463. doi:10.1212/01.wnl.0000299117.
sickness in migraine and vestibular 45. J Neurol
48935.2e
Neurosurg Psychiatry 2015;86(5):585-587.
doi:10.1136/jnnp-2014-308331 59 Tjernstrom F, Nystrom A, Magnusson M. How to
uncover the covert saccade during the head
46 Paillard AC, Quarck G, Paolino F, et al. Motion
impulse test. Otol Neurotol 2012;33(9):1583-1585.
sickness susceptibility in healthy subjects and
doi:10.1097/MAO.0b013e318268d32f
vestibular patients: effects of gender, age and
trait-anxiety. J Vestib Res 2013;23:203-209. 60 Fife TD, Tusa RJ, Furman JM, et al. Assessment:
doi:10.3233/VES-130501 vestibular testing techniques in adults and
children: report of the Therapeutics and
47 Strupp M, Brandt T, Huppert D, Grill E.
Technology Assessment Subcommittee of the
Prevalence of motion sickness in various
American Academy of Neurology. Neurology
vestibular disorders: a study on 749 patients.
2000;55(10):1431-1441. doi:10.1212/wnl.55.10.1431
J Neurol 2018;265(suppl 1):95-97. doi:10.1007/
s00415-018-8961-1 61 Kattah JC. Clinical characteristics and etiology of
bilateral vestibular loss in a cohort from Central
48 Kennedy RS, Graybiel A, McDonough RC,
Illinois. Front Neurol 2018;9:46. doi:10.3389/
Beckwith FD. Symptomatology under storm
fneur.2018.00046
conditions in the North Atlantic in control
subjects and in persons with bilateral 62 Lucieer F, Vonk P, Guinand N, et al. Bilateral
labyrinthine defects. Acta Otolaryngol 1968; vestibular hypofunction: insights in etiologies,
66(6):533-540. doi:10.3109/00016486809126317 clinical subtypes, and diagnostics. Front Neurol
2016;7:26. doi:10.3389/fneur.2016.00026
49 Golding JF, Gresty MA. Pathophysiology and
treatment of motion sickness. Curr Opin Neurol 63 Zingler VC, Cnyrim C, Jahn K, et al. Causative
2015;28(1):83-88. doi:10.1097/WCO. factors and epidemiology of bilateral
0000000000000163 vestibulopathy in 255 patients. Ann Neurol 2007;
61(6):524-532. doi:10.1002/ana.21105

CONTINUUMJOURNAL.COM 445

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHRONIC DIZZINESS

64 Zingler VC, Weintz E, Jahn K, et al. Causative 75 Alkathiry AA, Sparto PJ, Kontos AP, Furman JM.
factors, epidemiology, and follow-up of bilateral Chapter 10: vestibular dysfunction associated
vestibulopathy. Ann N Y Acad Sci 2009;1164: with mild traumatic brain injury. In: Hoffer ME,
505-508. doi:10.1111/j.1749-6632.2009.03765.x Balaban CD, eds. Neurosensory disorders in mild
traumatic brain injury. London, United Kingdom:
65 Girasoli L, Cazzador D, Padoan R, et al. Update on
Academic Press, 2019:133-148.
vertigo in autoimmune disorders, from diagnosis
to treatment. J Immunol Res 2018;2018:5072582. 76 Lumba-Brown A, Teramoto M, Bloom OJ, et al.
doi:10.1155/2018/5072582 Concussion guidelines step 2: evidence for
subtype classification. Neurosurgery 2020;86(1):
66 Cronin T, Arshad Q, Seemungal BM. Vestibular
2-13. doi:10.1093/neuros/nyz332
deficits in neurodegenerative disorders:
balance, dizziness, and spatial disorientation. 77 Davies RA, Luxon LM. Dizziness following head
Front Neurol 2017;8:538. doi:10.3389/ injury: a neuro-otological study. J Neurol 1995;
fneur.2017.00538 242(4):222-230. doi:10.1007/BF00919595
67 Fife TD, Robb MJA, Steenerson KK, Saha KC. 78 Singman E, Quaid P. Vision disorders in mild
Bilateral vestibular dysfunction associated with traumatic brain injury. In: Hoffer ME, Balaban CD,
chronic exposure to military jet propellant ed. Neurosensory disorders in mild traumatic
type-eight jet fuel. Front Neurol 2018;9:351. brain injury. London, United Kingdom: Academic
doi:10.3389/fneur.2018.00351 Press, 2019:223-244.
68 Gürkov R. Amiodarone: a newly discovered 79 Ward BK, Carey JP, Minor LB. Superior canal
association with bilateral vestibulopathy. Front dehiscence syndrome: lessons from the first
Neurol 2018;9:119. doi:10.3389/fneur.2018.00119 20 years. Front Neurol 2017;8:177. doi:10.3389/
fneur.2017.00177
69 Hain TC, Cherchi M, Yacovino DA. Bilateral
vestibular weakness. Front Neurol 2018;9:344. 80 Maitland CG. Perilymphatic fistula. Curr Neurol
doi:10.3389/fneur.2018.00344 Neurosci Rep 2001;1(5):486-491. doi:10.1007/
s11910-001-0111-x
70 Peloquin CA, Berning SE, Nitta AT, et al.
Aminoglycoside toxicity: daily versus 81 Lopez-Escamez JA, Carey J, Chung WH, et al.
thrice-weekly dosing for treatment of Diagnostic criteria for Menière's disease
mycobacterial diseases. Clin Infect Dis 2004; according to the Classification Committee of the
38(11):1538-1544. doi:10.1086/420742 Bárány Society [in German]. HNO 2017;65(11):
887-893. doi:10.1007/s00106-017-0387-z
71 Baloh RW, Kerber K. Clinical neurophysiology of
the vestibular system. 4th ed. New York, NY.: 82 Master CL, Master SR, Wiebe DJ, et al. Vision and
Oxford University Press, 2011. vestibular system dysfunction predicts
prolonged concussion recovery in children. Clin J
72 Szmulewicz DJ, Waterston JA, MacDougall HG,
Sport Med 2018;28(2):139-145. doi:10.1097/
et al. Cerebellar ataxia, neuropathy, vestibular
JSM.0000000000000507
areflexia syndrome (CANVAS): a review of the
clinical features and video-oculographic 83 Sandel N, Collins MW. Diagnosis and
diagnosis. Ann N Y Acad Sci 2011;1233:139-147. management of mild traumatic brain injury. Curr
doi:10.1111/j.1749-6632.2011.06158.x Trauma Rep 2018;4:127-137. doi:10.1007/s40719-
018-0120-8
73 Cortese A, Tozza S, Yau WY, et al. Cerebellar
ataxia, neuropathy, vestibular areflexia syndrome 84 Kleffelgaard I, Soberg HL, Tamber AL, et al.
due to RFC1 repeat expansion. Brain 2020;143(2): The effects of vestibular rehabilitation on
480-490. doi:10.1093/brain/awz418 dizziness and balance problems in patients after
traumatic brain injury: a randomized controlled
74 Boutros PJ, Schoo DP, Rahman M, et al.
trial. Clin Rehabil 2019;33(1):74-84.
Continuous vestibular implant stimulation
doi:10.1177/0269215518791274
partially restores eye-stabilizing reflexes.
JCI Insight 2019;4(22):e128397. doi:10.1172/jci. 85 Gottshall KR, Whitney SL. Vestibular
insight.128397 rehabilitation for mild traumatic brain injury
(mTBI). In: Hoffer ME, Balaban CD, eds.
Neurosensory disorders in mild traumatic brain
injury. London, United Kingdom: Elsevier, Inc,
2019:339-354.

446 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 REVIEW ARTICLE


Acute Vestibular
CONTINUUM AUDIO
INTERVIEW AVAILABLE
ONLINE
Syndrome
By Kristen K. Steenerson, MD
Downloaded from http://journals.lww.com/continuum by wqLe/H3/oM2vx1Qs5A5qMemKKHLPqE4qkmGNpdheG3ikf1o85ttJSkFL2oTkgOkhMEmawHvsuTTE5xJG2ZjqccytIAhVn2qegy060KLcKJuPZsx5htaYiopRjp2zor2egUzWJAEWYNo65TEub0G9tZtPaMachiqn on 04/28/2022

ABSTRACT
PURPOSE OF REVIEW: This article provides a practical approach to acute
vestibular syndrome while highlighting recent research advances.

RECENT FINDINGS:Acute vestibular syndrome is defined as sudden-onset,

continuous vertigo lasting longer than 24 hours with associated nausea
and vomiting, all of which are worsened with head movement. Acute
vestibular syndrome is provoked by a variety of central and peripheral
causes, the most common of which are vestibular neuritis and acute stroke
(posterior circulation). A clinical approach focusing on timing, associated
history, and ocular motor findings can improve diagnostic accuracy and
is more sensitive and specific than early neuroimaging. Because of the
shared neurovascular supply, both peripheral and central vestibular
disorders can manifest overlapping signs previously considered solely
peripheral or central, including vertical skew, nystagmus, abnormal
vestibular ocular reflex, hearing loss, and gait instability. Although acute
vestibular syndrome is typically benign, stroke should be considered in
every person with acute vestibular syndrome because it can act as a
CITE AS: harbinger of stroke or impending cerebellar herniation. Treatment is
CONTINUUM (MINNEAP MINN) focused on physical therapy because the evidence is minimal for the
2021;27(2, NEURO-OTOLOGY):
402–419.
long-term use of medication.

Address correspondence to SUMMARY: The diagnosis of acute vestibular syndrome first requires the
Dr Kristen K. Steenerson, Stanford elimination of common medical causes for dizziness. Next, underlying
University, 2452 Watson Ct,
Ste 1700, Palo Alto, CA 94303, pathology must be determined by distinguishing between the most
[email protected]. common causes of acute vestibular syndrome: central and peripheral
RELATIONSHIP DISCLOSURE:
vestibular disorders. Central vestibular disorders are most often the result
Dr Steenerson reports no of ischemic stroke affecting the cerebellar arteries. Peripheral vestibular
disclosure. disorders are assumed to be caused mostly by inflammatory sources, but
UNLABELED USE OF
ischemia of the peripheral vestibular apparatus may be underappreciated.
PRODUCTS/INVESTIGATIONAL By using the HINTS Plus (Head Impulse test, Nystagmus, Test of Skew with
USE DISCLOSURE: Plus referring to hearing loss assessment) examination in addition to a
Dr Steenerson discusses the
unlabeled/investigational use of comprehensive neurologic examination, strokes are unlikely to be missed.
betahistine and cinnarizine, For nearly all acute vestibular disorders, vestibular physical therapy
neither of which is approved by
contributes to recovery.
the US Food and Drug
Administration for the
treatment of peripheral
vestibular disorders.

© 2021 American Academy

of Neurology.

402 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 KEY POINTS
INTRODUCTION

O
riginally coined by Hotson and Baloh1 in 1998, acute vestibular ● Acute vestibular
syndrome describes the sudden onset of continuous vertigo lasting syndrome describes the
longer than 24 hours and associated with nausea, head motion sudden onset of continuous
vertigo lasting longer than
intolerance, and unstable balance.1 Although vertigo and nausea 24 hours and associated
are the driving symptoms of acute vestibular syndrome, patients with nausea, head motion
often describe additional spatial disorientation and gait instability that may be intolerance, and unstable
confused with nonvestibular sources of dizziness ranging from hyponatremia to balance.
gastroenteritis. When acute vestibular syndrome is strictly defined as ● Nausea, vomiting, and
sudden-onset, continuous vertigo and common medical causes for dizziness such unsteadiness are symptoms
as arrhythmia, hypotension, and toxic or metabolic derangements have been of acute vestibular
ruled out, key examination findings can help determine the cause. The most syndrome that are common
to several causes, so
common causes are inflammatory events of the peripheral labyrinth followed in additional measures are
frequency by ischemic events of the posterior cerebral circulation.2 needed to narrow the
Previous work has focused heavily on bedside ocular motor testing protocols diagnostic possibilities.
because of their high specificity and sensitivity for differentiating central from
● The posterior circulation
peripheral causes. Properly performed, clinical examination using HINTS (Head
(vertebral arteries, posterior
Impulse test, Nystagmus, Test of Skew) is more sensitive in detecting small inferior cerebellar artery,
strokes causing isolated acute vertigo than is early MRI diffusion-weighted anterior inferior cerebellar
imaging.3 artery, and less often
superior cerebellar artery)
Acute care clinicians should maintain a high level of suspicion for posterior
supplies the brainstem and
circulation stroke because its presentation may perfectly overlap the much more cerebellar regions causing
common clinical picture of vestibular neuritis. Posterior circulation strokes that stroke that might present
are small and cause isolated acute vertigo tend to allow for a good recovery, but with acute vestibular
syndrome.
they are important to recognize because they represent a forewarning of
vertebrobasilar disease and a larger stroke to follow that might be preventable. ● Cerebellar strokes that
Posterior circulation strokes that are larger and involve the cerebellum are can present with vertigo,
essential to recognize because they can lead to life-threatening edema. nystagmus, and imbalance if
left unrecognized and
Life-threatening posterior circulation strokes more commonly involve multiple
untreated could lead to
neurologic deficits on presentation including ataxia, dysarthria, hemianopsia, brain edema that can rarely
diplopia, Horner syndrome, or hemisensory deficits. lead to herniation and death.
Some factors serve as obstacles to full recognition of posterior circulation
● CT may miss posterior
stroke as the cause of acute vertigo. Posterior circulation stroke may be missed
fossa strokes because of
because of emergency department algorithms' bias toward anterior circulation considerable bony artifact
stroke.4 The National Institutes of Health Stroke Scale neglects many debilitating from the skull, and MRI may
aspects of posterior circulation stroke, including vertigo, and can fail to detect be diffusion negative up to
the first 48 hours of
posterior circulation stroke entirely.5 Many stroke centers, therefore, have other
symptoms.
protocols to account for acute vertigo and possible stroke. Furthermore, posterior
circulation stroke comprises only 15% to 20% of all strokes, and only 20% to 25% ● Strictly defined acute
of posterior circulation stroke causes acute dizziness, limiting clinician vestibular syndrome is most
commonly due to acute
experience and compounding uncertainty. Perhaps in part because of these
unilateral vestibulopathy
factors, neuroimaging is overused in acute care settings despite evidence (vestibular neuritis or
showing its limitations in the first 24 to 48 hours of symptoms.6,7 ischemic labyrinthopathy). If
By understanding the methodology behind the existing protocols, simplifying all types of acute dizziness
are included, about
their interpretation, and updating their accuracy of use with the most recent
one-third are due to
research, providers can improve their diagnostic accuracy in patients presenting vestibular causes.
with acute vertigo.
More than 3 million emergency department visits per year are for dizziness,8
and about 1 in 5 of those are because of acute vestibular syndrome.9 Like other

CONTINUUMJOURNAL.COM 403

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

ACUTE VESTIBULAR SYNDROME

acute syndromes seen in the emergency setting, the number of cases of acute
vestibular syndrome continues to increase over time; however, it is considerably
outpaced by rates of defensive neuroimaging,8 perhaps reflecting the
misconception that CT or MRI can definitively rule out posterior circulation
stroke. In a population-based study by Kerber and colleagues,10 of patients
presenting with any dizziness, 3.2% (53 of 1666) of cases were attributed to
stroke; of patients presenting with isolated dizziness, only 0.7% (9 of 1297) of
cases could be attributed to acute stroke.
Although it is important in the acute setting to frame the initial workup of
acute dizziness in the dichotomy of determining “stroke” or “not stroke,” the
other causes of acute dizziness must be considered. A comprehensive study
looking at all causes of acute dizziness in patients presenting to the emergency
department found otovestibular causes account for approximately one-third, and
neurologic causes (including stroke) account for 11%. Other causes are delineated
in TABLE 5-1.11 Of otovestibular causes, the most likely are acute unilateral
vestibulopathy, migraine, benign paroxysmal positional vertigo (BPPV), and
Ménière disease.11
When acute vestibular syndrome is held to the strict syndrome criteria of
longer than 24 hours of the illusion of movement, imbalance, nystagmus, nausea,
and vomiting, 10% of all causes of acute dizziness can be attributed to acute
vestibular syndrome according to one single-center study.12 The vast majority of
strictly defined cases are due to acute unilateral vestibulopathy (vestibular
neuritis or ischemic labyrinthopathy) and a smaller fraction are due to posterior
circulation stroke.13,14 The remaining few can be attributed to a first-ever
vestibular migraine attack and possibly Ménière disease, although strict criteria
for Ménière disease limit attacks to 12 hours.12 Other otovestibular causes are
easily distinguishable from true acute vestibular syndrome and are discussed in
further detail.

TABLE 5-1 Rate of Various Etiologies for Dizziness in the Acute Care Settinga,b

Dizziness source Rate, %

Otovestibular 32.9

Cardiovascular 11.5

Neurologic (stroke) 11.2 (4)

Metabolic 11

Poisoning 10.6

Psychiatric 7.2

Gastrointestinal 7

a
Data from Newman-Toker DE, et al, Mayo Clin Proc.11
b
Notably, acute dizziness was not further delineated into a true acute vestibular syndrome, which is
narrowly defined as spontaneous, continuous vertigo lasting longer than 24 hours. Other large-scale,
population-based studies have found acute vestibular syndrome as the cause of 19% of dizziness cases in
the acute care setting.9

404 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

APPROACH TO THE PATIENT WITH ACUTE DIZZINESS KEY POINTS
As already discussed, various medical causes can result in a patient with acute
● Orthostatic hypotension
dizziness. Understanding the key differentiators of medical causes of acute and other cardiac causes of
dizziness can help quickly rule out their role in acute vestibular syndrome. By dizziness can classically
emphasizing the core characteristics of acute vestibular syndrome in cause near-syncope and
history-taking, including quality, timing, triggers, and associated symptoms, the possibly brief bouts of
vertigo but rarely sustained
interviewer can more reliably identify true acute vestibular syndrome. Finally,
vertigo as is classic for acute
key examination techniques narrow the causes of true acute vestibular syndrome vestibular syndrome.
to central and peripheral etiologies.
● Vitamin B1 deficiency
Cardiac and Metabolic Causes of Acute Dizziness causing Wernicke
encephalopathy can rarely
Orthostatic hypotension, defined as a greater than 20-mm Hg drop in systolic present as acute vestibular
blood pressure in a normotensive patient on standing, can cause global cerebral syndrome, although often
hypoperfusion, generating dizziness that can be exacerbated by orthostatic with additional neurologic
positional changes. Rarely, hypotension in the setting of asymmetric posterior findings.
circulation vascular supply may cause relative hypoperfusion of select areas of ● Phenytoin toxicity may
the vestibulocerebellum and/or peripheral vestibular end organs, resulting in present with acute vertigo,
vestibular asymmetry and, thus, brief vertigo15; however, spinning vertigo is nausea, ataxia, and
certainly not the most common description of dizziness from orthostatic gaze-evoked nystagmus.
hypotension. Overall, it is rare and unlikely for hypotension to present with
● Acute intoxication with
sustained vertigo as seen in acute vestibular syndrome.16 alcohol, phencyclidine,
Arrhythmia often has a sudden, unpredictable onset with rapid recovery. opiates, marijuana, and
Neurally mediated syncope generally occurs while standing and has a prodrome. barbiturates can cause
Structural heart defects, heart failure, or aortic stenosis can all cause dizziness. nystagmus and vertigo.

Lack of chest pain, palpitations, shortness of breath with a normal physical ● Vertigo is a disorder of
examination devoid of murmur or fluid overload in the setting of normal motion perception and
orthostatic blood pressure measurements and normal electrocardiogram (ECG) encompasses false spinning
are reassuring that a cardiovascular cause is not the source of acute dizziness. sensations (spinning vertigo)
and other false sensations
Toxic and metabolic abnormalities can be quickly considered by reviewing such as swaying, tilting,
basic serum electrolyte levels, glucose, thyroid function, and a drug screen, if bobbing, bouncing, or
needed, for acute dizziness. All of these conditions can present with acute sliding (nonspinning vertigo).
dizziness, but none of them causes sustained vertigo for longer than 24 hours
● Spontaneous vertigo
with nausea and vomiting worsened by head motion. Among metabolic causes,
classic to acute vestibular
Wernicke encephalopathy can be the exception. It can lead to neuronal and/or syndrome continues even
neuropil destruction and endothelial swelling affecting the mammillary bodies, when the patient is
periaqueductal gray matter, thalamus, inferior olives, and cerebellum and may motionless but worsens with
present with acute vertigo, ataxia, nausea, and vomiting although often with any kind of head movement;
in contrast, the vertigo of
other neurologic findings such as gaze paresis. benign paroxysmal
positional vertigo ensues
Medications, Toxins, and Substances That Cause Acute Dizziness after only certain
Nearly any medication can cause a predictable or idiosyncratic side effect of provocative head
maneuvers that evoke
dizziness. Antihypertensives that are newly prescribed or recently increased can vertigo lasting less than
cause various forms of light-headedness, orthostasis, and fatigue. Psychoactive 1 minute rather than
medications are prone to causing dizziness, especially at initiation or dose continuously for 24 hours.
increases. Certain medications may be associated with vertigo, imbalance, or eye
● Recurrent attacks that are
movement abnormalities. Antiepileptic medications, such as phenytoin,
new and increasing may
carbamazepine, and primidone, can cause dizziness but rarely vertigo. rarely be a sign of stuttering
Phenytoin, however, may cause a central-pattern nystagmus (gaze-evoked transient ischemic attack of
nystagmus), vertigo, and ataxia, particularly at toxic levels. Aminoglycosides, the posterior circulation.
particularly gentamicin, which is vestibulotoxic, can cause acute vestibular

CONTINUUMJOURNAL.COM 405

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

ACUTE VESTIBULAR SYNDROME

syndrome. Toxins such as organophosphates and carbon monoxide have been

associated with acute vestibulocochlear symptoms.17 Substances including
alcohol, phencyclidine, opiates, marijuana, and barbiturates can cause nystagmus
and vertigo.18
After a comprehensive review of toxic-metabolic and medical causes of acute
dizziness, the diagnosis of acute vestibular syndrome can be more reliably
focused by targeted questions (TABLE 5-2).6,19

Is There Spontaneous Movement?

According to the International Classification of Vestibular Disorders,2 vertigo
encompasses false spinning sensations (spinning vertigo) and other false
sensations such as swaying, tilting, bobbing, bouncing, or sliding (nonspinning
vertigo). By highlighting a sensation of false motion or movement, vertigo can be
more accurately identified. Spontaneous vertigo is vertigo that occurs without an
obvious trigger and may be exacerbated by movements (especially head
movements). Spontaneous vertigo is the defining feature of strictly defined acute
vestibular syndrome.

Is the Movement Continuous?

Vertigo worsens during head movement, so when a patient’s vertigo worsens
with position changes, it does not necessarily mean a diagnosis of BPPV is
assured unless characteristic nystagmus induced by canal-plane–specific
positioning is present, for example, with the Dix-Hallpike test. An important
distinction for the interviewer is determining if dizziness is exquisitely triggered
by position change or if it is primarily spontaneous and constant and only
aggravated by movement. Classic BPPV is triggered by certain head positioning;
however, a minority of patients describe constant underlying dizziness between
positional attacks, which can lead to confusion.

Is This the First-Ever Attack?

Patients with a long history of recurrent dizziness attacks are less likely to have a
serious cause of their dizziness. Recurrent attacks that are new and increasing
may rarely be a sign of stuttering transient ischemic attack (TIA) of the posterior

TABLE 5-2 Targeted Questions in the History That Can Help Narrow the Differential
Diagnosis for Acute Vestibular Syndrome Causesa

Question Target

Is there spontaneous Determines if a false sense of motion (vertigo) is the driving symptom
movement?

Is the movement Noncontinuous vertigo should raise suspicion this is not acute vestibular syndrome
continuous?

Is this the first-ever attack? Recurrent attacks should point toward episodic vestibular syndrome causes instead of acute
vestibular syndrome; rarely represent stuttering transient ischemic attacks

a
Data from Kerber KA and Newman-Toker DE, Neurol Clin6 and Young AS, et al, Handbook of Clinical Neurology.19

406 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

circulation. More commonly, stereotyped recurrent dizziness attacks are
classified as episodic vestibular syndrome and generate a different category of
differential diagnoses from acute vestibular syndrome, including vestibular
migraine, Ménière disease, and BPPV. Although vestibular neuritis can recur, the
lifetime recurrence rate is only 2%, so it is unlikely to explain multiple recurrent
vertigo attacks.

EXAMINATION TECHNIQUES
When the above questions are satisfactorily assessed and have confirmed
sudden-onset, continuous, spontaneous vertigo, certain examination techniques
help narrow the short list of differential diagnoses of acute vestibular syndrome.
The classic distinction of peripheral versus central causes is an excellent starting
framework. Peripheral causes resulting from acute vestibular syndrome are
referred to as acute unilateral vestibulopathy and typically result from two main
pathologies that affect the vestibulocochlear nerve and vestibular end organs:
vestibular neuritis (labyrinthitis if hearing is also involved) and ischemic
labyrinthopathy. Central causes refer to mainly posterior circulation stroke but
can, of course, involve any lesions along the central vestibular circuitry.
The original HINTS examination (Head Impulse test, Nystagmus, Test of
Skew) is the earliest collection of physical examination findings developed to
reliably differentiate a central cause, such as stroke, from a peripheral cause, such
as vestibular neuritis (FIGURE 5-1).20,21 This collection refers to the following:

u Head impulse: A bedside head impulse test with a catch-up saccade during rapid
acceleration of the head to one side is a sign of peripheral vestibular loss on the side of the
turn associated with the catch-up saccade.
u Nystagmus: Nystagmus that is spontaneous, horizontal, and unidirectional that attenuates
with visual fixation is another peripheral sign. Using fixation blocking techniques such as a
bright penlight to blind the patient’s ability to fixate or bedside Frenzel goggles improves
detection of (peripheral-pattern) nystagmus.
u Test of skew: A lack of vertical misalignment, or skew, is reassuring for peripheral
pathology.

Any other findings should raise concern for a central cause, and stroke workup
should be performed. For central causes, HINTS is 96.8% sensitive and 98.5%
specific compared with 14.3% falsely negative MRI in the first 48 hours.22 HINTS

FIGURE 5-1
Adapted algorithm for the approach to acute vestibular syndrome. The algorithm focuses
on the strict definition of acute vestibular syndrome as acute, continuous vertigo lasting
longer than 24 hours followed by HINTS (Head Impulse test, Nystagmus, Test of Skew)
examination plus additional useful differentiators including sensorineural hearing loss (SNHL)
evaluation, truncal ataxia assessment, and timing.49
AUV = acute unilateral vestibulopathy; HIT = head impulse test; PCS = posterior circulation stroke.
Modified with permission from Venhovens J, et al, J Neurol.20 © 2016 Springer-Verlag.

CONTINUUMJOURNAL.COM 407

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

ACUTE VESTIBULAR SYNDROME

outperforms MRI and the ABCD2 (age, blood pressure, clinical features,
duration, presence of diabetes) score in the acute care setting.22 The ABCD2 score
is a risk-stratification tool with points ranging from 0 to 7 used to identify
patients at high risk of stroke that includes an age of 60 or older (1 point), blood
pressure 140/90 mm Hg or higher (1 point), unilateral weakness (2 points) or
impaired speech (1 point), symptom duration of 10 to 59 minutes (1 point) or
longer than or equal to 60 minutes (2 points), and presence of diabetes (1 point).
The higher the score, the greater the risk of stroke.
Although HINTS has been in academic use for more than 10 years, it has
limitations in its practical use. Many providers in acute care settings lack the
confidence to use their own examination and interpretation of eye movements to
make a critical decision about whether a patient has had a stroke and tend to fall
back on neuroimaging.23 This means the utility of HINTS is strongly influenced
by the level of expertise of the person performing it.24 The uncertainty arises
from a few sources but most commonly results from a lack of practice. This may
improve in time. Stroke teams are increasingly using HINTS regularly because
patients with isolated vertigo will not be recognized by the National Institutes of
Health Stroke Scale as having a possible stroke. Some interesting innovations are
being explored to circumvent this. Portable video-oculography has been shown
to improve the accuracy of ocular motor assessments both in person and
telemedically.25 Although not evidence based, using slow-motion recording on
smartphones can, in this author’s experience, help capture difficult-to-interpret
catch-up saccades on bedside head impulse testing.
HINTS Plus is the same as HINTS but with the addition of audiometry
(TABLE 5-326). New-onset hearing loss with acute vestibular syndrome was
identified as an additional predictor of stroke based on a 2013 population-based
study; its addition increased sensitivity for stroke detection to 99%.22 With this
added value, hearing should be assessed in every patient with acute vestibular
syndrome. Although formal audiometry is not feasible in most emergency
department settings, bedside testing with the finger-rub test or
smartphone-based applications can help identify some patients. If a hearing
assessment cannot be obtained in the emergency setting, an outpatient

TABLE 5-3 Classic HINTS Plus (Head Impulse Test, Nystagmus, Test of Skew With
Audiometry) Examination Findings in Central and Peripheral Causes
for Acute Vestibular Syndromea

Head impulse test Nystagmus Test of skew Plus hearing loss

Central No corrective Direction-changing, pure Abnormal vertical Present (anterior inferior

saccade vertical, pure torsional or ocular alignment cerebellar artery), absent
vertical-torsional, (posterior inferior cerebellar
nonfatiguing artery)

Peripheral Corrective saccade Unidirectional, horizontal- Absent or rarely Absent (vestibular neuritis),
with head turn torsional, attenuates with time-limited vertical present (labyrinthitis)
toward side of lesion fixation point ocular alignment

a
Data from Kattah JC, et al, Stroke3; Kung NH, et al, J Neuroophthalmol21; and Kattah JC, J Neurol Phys Ther.26

408 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

audiogram can be arranged to aid in risk stratification and secondary prevention
after all acute care decisions have been made.
Another study examining refining of acute vestibular syndrome examination
techniques added ataxia and truncal instability ratings to HINTS and found
ataxia grade 2 to 3 added to overall sensitivity, resulting in a perfect 100%
sensitivity.27 Truncal ataxia, measured by using independent observers, was
scored as grade 1, mild to moderate imbalance with walking independently;
grade 2, severe imbalance with standing, but cannot walk without support; grade
3, falling at an upright posture (TABLE 5-4). The ataxia rating alone had a
sensitivity of 92.9% and was easier to perform reliably compared with HINTS.27
Another group found that adding the ABCD2 score also improved stroke
detection: ABCD2 score of less than 4 (low risk) had a stroke frequency of 1%
(5 of 512 patients) compared with 8.1% (32 of 395 patients) in the high-risk group
(ABCD2 score ≥4).28 HINTS Plus combined with ataxia and vascular risk factor
assessment such as the ABCD2 score can reduce the risk of missing stroke
diagnosis to near nil.24 An algorithmic approach using these concepts is shown in
FIGURE 5-1.

ACUTE STROKE
If the HINTS Plus examination demonstrates a central pattern consisting of no
catch-up saccade on head impulse testing, central pattern nystagmus
(direction-changing, vertical, unaffected by fixation), vertical skew deviation,
and/or new, sudden asymmetric hearing loss, stroke is likely. If high-grade
truncal ataxia is present, stroke is nearly certain. The two most common types of
stroke stem from the posterior inferior cerebellar artery (PICA) and anterior
inferior cerebellar artery (AICA) territories with ischemic stroke outnumbering
hemorrhagic stroke 8:1.24 The presence of acute unilateral hearing loss with acute
vestibular syndrome suggests that a lateral pontine stroke in the distribution of
AICA is somewhat more probable. Head or neck pain with sudden-onset vertigo
should prompt evaluation for vertebral dissection (CASE 5-1).
PICA territory strokes are the most common in acute vestibular syndrome and
most affect the cerebellum followed by the medulla, pons, and thalamus.24
Because the PICA supplies the posterior inferior cerebellum (including the
nodulus, uvula, and flocculus) and ipsilateral pontomedullary vestibular nucleus,
the main symptoms and signs of a PICA territory infarction may consist only of

Truncal Ataxia Gradations Used to Distinguish Peripheral Versus Central TABLE 5-4
Causes of Acute Vestibular Syndromea

Ataxia grade Definition

1 Mild to moderate imbalance with walking independently

2 Severe imbalance with standing, but cannot walk without support

3 Falling at upright posture

a
In a study evaluating acute vestibular syndrome presentations of posterior circulation stroke, all patients at
grade 3 had stroke, and all at grade 1 had peripheral causes.27

CONTINUUMJOURNAL.COM 409

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

ACUTE VESTIBULAR SYNDROME

CASE 5-1 A 33-year-old right-handed man presented to the emergency

department for evaluation of new-onset continuous dizziness. He
described the sudden onset of room-spinning vertigo shortly after
beginning skiing that morning. He became so dizzy that he could no longer
ski. He had no vascular risk factors but did have dull head and neck pain.
His vital signs and neurologic examination were normal except for gait
instability and truncal ataxia such that he could no longer walk
unassisted. HINTS Plus (Head Impulse test, Nystagmus, Test of Skew with
hearing assessment) revealed absent catch-up saccade on head impulse
testing, gaze-evoked nystagmus, vertical skew deviation, and normal
hearing on the finger-rub test. The emergency department physician
ordered vascular imaging, which showed a right vertebral dissection and
a posterior inferior cerebellar artery (PICA) territory infarct.

COMMENT This patient’s postural and ocular motor examination findings are quite
concerning for central vestibular pathology, particularly cerebellar.
Although lacking conventional vascular risk factors, vascular risk factors
that can lead to stroke presenting as acute vestibular syndrome in young
people are more likely related to trauma, increasing the risk of dissection.
PICA territory strokes are more common in younger patients, of which
dissections are common causes.

CASE 5-2 A 75-year-old man presented to his primary care physician for evaluation
of sudden-onset continuous dizziness. He had no vascular risk factors
except for age. On further questioning, he described spontaneous vertigo
attacks that began in the past 1 to 2 weeks and were getting progressively
longer.
His examination revealed catch-up saccade on head impulse testing to
the right and peripheral-pattern nystagmus that was spontaneously
left-beating and attenuated with fixation. He was able to ambulate but
required assistance. Bedside finger-rub testing demonstrated
asymmetric right hearing loss. His primary care physician recommended
immediate emergency evaluation. Central imaging revealed anterior
inferior cerebellar artery territory ischemia with intracranial
atherosclerosis.

COMMENT This case demonstrates that new vertigo that abruptly begins and quickly
intensifies is concerning for stuttering transient ischemic attack. It also
highlights that, by the original HINTS (Head Impulse test, Nystagmus, Test
of Skew) evaluation, he may have been incorrectly diagnosed with a
peripheral cause of acute unilateral vestibulopathy and sent home. HINTS
Plus (HINTS with audiometry) demonstrated hearing loss that helped
stratify the cause of symptoms correctly as a posterior circulation stroke.

410 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

vertigo and truncal ataxia without the other classic cerebellar signs of gaze-evoked KEY POINTS
nystagmus or dysmetria.29
● In patients with acute
Because the internal auditory artery off of the AICA supplies the peripheral vertigo, nystagmus may be
vestibular and auditory structures in addition to the AICA supply of the lateral obvious with the naked eye,
pons, middle cerebellar peduncle, and anterior and inferior cerebellum, strokes but when it is not, removal
in this distribution can affect peripheral or central vestibular structures or both. of fixation by some means
(eg, Frenzel goggles, Fresnel
Although labyrinthine ischemia cannot be detected on MRI, the possibility
lenses, magnifying sheet,
should be considered in a patient with a peripheral-pattern acute vestibular bright penlight, or
syndrome and considerable vascular risk factors. fundoscopy) improves
Rarely, AICA infarction can affect only the vestibular root entry zone and detection of more subtle
nystagmus.
seventh cranial nerve fascicle, resulting in isolated vertigo and lower motor
neuron–pattern facial nerve palsy. The concept of isolated root entry zone, ● Although ideally hearing
cerebellum, or vestibular nucleus infarct causing peripheral-pattern symptoms should be assessed in every
is captured in the terms vestibular pseudoneuritis and pseudolabyrinthitis30 patient with suspected
(CASE 5-2). A collection of longitudinal studies suggests that patients who present acute vestibular syndrome,
emergency settings are
to the emergency department with acute vestibular syndrome have a higher rarely equipped for formal
relative risk of stroke during the following year.31 Although the rate of stroke audiometry. Bedside testing
remained low, the increased relative risk may indicate missed early stroke or TIA with hearing test
presenting as acute vestibular syndrome. Other studies have found less smartphone applications or
finger-rub testing followed
significant associations,32 but the importance of carefully ruling out posterior by an eventual outpatient
circulation stroke nonetheless remains as smaller strokes may serve as harbingers formal audiogram can
of more disabling, larger strokes to come. Acute vestibular syndrome without provide valuable vascular
clearly identified stroke may still benefit from vascular risk factor optimization risk factor information.
and a time-limited course of aspirin because of the known association of
● Grading truncal ataxia in
acute vestibular syndrome with ischemic labyrinthopathy and/or a punctate acute vestibular syndrome
central ischemic event and a higher likelihood of stroke in the subsequent patients can increase
90 days. anterior inferior cerebellar
artery stroke detection
sensitivity to 100%.
ACUTE UNILATERAL VESTIBULOPATHY
If the HINTS Plus and ataxia examination reveal a peripheral pattern, does ● If the HINTS Plus (Head
that mean it must be vestibular neuritis? The answer is not necessarily. Impulse test, Nystagmus,
Vestibular neuritis has become a catch-all term for the more accurate Test of Skew plus hearing
loss assessment)
descriptor: acute unilateral vestibulopathy. Acute unilateral vestibulopathy examination demonstrates a
indicates a peripheral asymmetry in vestibular function, disrupting normal central pattern consisting of
vestibular tone. Of acute unilateral vestibulopathy causes, vestibular neuritis no catch-up saccade on
is likely the most common.14 Vestibular neuritis refers to inflammation of the head impulse testing,
central pattern nystagmus
vestibulocochlear nerve, which thus results in acute unilateral vestibulopathy.
(direction-changing,
Vestibular neuritis is classically understood to derive from a viral infection. vertical, unaffected by
Association with a viral prodrome such as upper respiratory infections is not fixation), vertical skew
always present, and the absence of a previous upper respiratory infection deviation, and/or new,
sudden asymmetric hearing
should not exclude vestibular neuritis as a cause. Some evidence supports
loss, stroke is likely.
herpes simplex virus type 1 involvement because herpes simplex virus DNA
has been detected in as many as 60%33 or as few as 18%34 of sampled vestibular
ganglia of patients with vestibular neuritis. Additionally, histopathologic
studies of vestibular neuritis show similar pathology to what is seen in herpes
zoster oticus, linking viral etiologies.14 However, herpes simplex virus type 1 is
very common in the general population, and, because of the inability to assay
in live, symptomatic individuals, causation cannot yet be proven. Because of
the prevalence of neurotropic viruses in the general population and relative
rarity of acute unilateral vestibulopathy, acute unilateral vestibulopathy may

CONTINUUMJOURNAL.COM 411

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

ACUTE VESTIBULAR SYNDROME

just as likely derive from demyelination, ischemia, or autoimmune or other

inflammatory conditions preferentially affecting cranial nerves. Indeed, a lack
of significant response to antivirals35 may be a surrogate sign of involvement
of other etiologies beyond viral neuritis.
In contrast, herpes zoster oticus, known by the eponym Ramsay Hunt
syndrome,36 is a syndrome associated with facial palsy, vestibulocochlear
dysfunction, mouth pain, and vesicular rash over the external auditory canal and
concha. Because herpes zoster oticus is a potentially severely disabling and
disfiguring syndrome, antiviral treatment is effective and should not be delayed.
Famciclovir may be more effective than acyclovir.37 Other infectious causes
should be considered in patients with a history of chronic middle ear infections or
meningitis because these may progress to bacterial vestibular neuritis or
labyrinthitis, in which hearing is affected as well.
Acute unilateral vestibulopathy has similar physical examination findings for
most causes. Because of the asymmetry in vestibular tone, patients have

CASE 5-3 A 55-year-old man presented to the emergency department with

sudden-onset vertigo for the past 25 hours. He had severe nausea and
vomiting and felt considerable exacerbation with head movements. His
past medical history was significant for anxiety and depression, and he
was recently started on a new medication for anxiety: alprazolam 0.5 mg
as needed. He recalled taking the medication the day before onset
without difficulty.
His examination revealed spontaneous, horizontal-torsional,
right-beating unidirectional nystagmus that attenuated with fixation.
Head impulse testing demonstrated catch-up saccade with head
accelerated to the left. His hearing was normal. He was able to walk
unassisted, although he felt very uncomfortable doing so. The rest of his
neurologic examination was normal.
He was discharged with instructions to begin vestibular therapy as
soon as possible. At a 3-month follow-up, his symptoms were
considerably improved, but he still had a mild sense of head lag with
quick movements and prominent brain fog. He continued to take
alprazolam daily.

COMMENT This case demonstrates a classic presentation of acute vestibular

syndrome due to acute unilateral vestibulopathy, most likely viral vestibular
neuritis. The patient’s new medication was not implicated in the onset of
dizziness because benzodiazepines are not associated with new-onset,
acquired vertigo and nystagmus in adults; in fact, they are potent vestibular
suppressants, which may help reduce symptom burden initially. This case
highlights two important concepts on recovery from acute vestibular
syndrome: (1) previous history of mood disorder is a negative
prognosticator for recovery and (2) chronic use of vestibular suppressants
contributes to incomplete central compensation.

412 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

spontaneous nystagmus with the fast phase beating away from the affected side. KEY POINTS
It intensifies with gaze in the direction of the fast phase and diminishes or abates
● The internal auditory
with gaze in the direction of the slow phase (known as the Alexander law). As artery, also known as the
central compensation can quickly begin, the spontaneous nystagmus may labyrinthine artery, supplies
diminish in some within hours to a few days when patients can visually fixate on the cochlea, saccule, and
their surroundings. The pattern of the nystagmus should mirror the affected posterior semicircular canal.
Greater than 90% of anterior
parts of the vestibular labyrinth. Because the vestibular nerve has two major
inferior cerebellar artery
divisions (superior and inferior), it is possible to have one or both divisions infarctions affect hearing
affected. The superior division is the most commonly affected,38 and, because it and have evidence of
innervates the anterior and horizontal canals, spontaneous nystagmus reflects peripheral vestibulopathy
superimposed on central
those planes, resulting in horizontal-torsional, unidirectional nystagmus (beating
vestibulopathy.
away from the affected side). A similar appearance is seen when both superior
and inferior vestibular nerves are affected. In both isolated superior vestibular ● Antivirals have not been
neuritis and combined superior and inferior vestibular neuritis, head impulse found to be effective
testing results in a catch-up saccade when the head is turned toward the side medications and are not
recommended in acute
of hypofunction. If, however, the inferior division is affected in isolation (the vestibular syndrome or
least common type), spontaneous downbeating torsional nystagmus may be acute unilateral
observed. Isolated inferior vestibular neuritis is rare, accounting for only about vestibulopathy in isolation.
2% of cases.38 Ramsay Hunt syndrome,
which may cause acute
Skew deviation is the vertical misalignment of the eyes caused by a vestibular syndrome in
supranuclear disorder involving the otolith-ocular pathways. Although vertical addition to vesicles around
skew deviation is usually associated with central lesions of the brainstem or the ear and multiple cranial
cerebellum, it can rarely occur in peripheral acute unilateral vestibulopathy as neuropathies, requires
immediate antiviral therapy.
part of the ocular tilt reaction (see collections.lib.utah.edu/details?id=1253805 for
representative ocular motor findings of acute vestibular syndrome with an ocular ● Peripheral-pattern
tilt reaction due to bacterial labyrinthitis); thus, vertical skew findings must be nystagmus for the majority
interpreted with caution. Abnormalities in the pathways, peripheral to central, of patients with acute
can manifest as skew deviation. Acute unilateral vestibulopathy is a rare but unilateral vestibulopathy is
direction-fixed, horizontal
possible cause of vertical skew39 because of involvement of these same pathways. nystagmus that beats away
Skew deviation from peripheral causes is usually mild and short-lived to no more from the affected side. It
than 1 or 2 days, although this timing does not help with differentiating in the intensifies with gaze in the
acute care setting. direction of the fast phase
and diminishes or abates
The classic pattern of HINTS Plus in acute unilateral vestibulopathy is the with gaze in the direction of
presence of an abnormal head impulse test with a catch-up saccade and the slow phase (known as
spontaneous unidirectional nystagmus (ie, it does not change direction with the Alexander law).
changes in the direction of gaze) that attenuates with fixation, increases with
● A catch-up saccade on
gaze in the direction of the fast phase, lessens or abates in the opposite direction
head impulse testing is an
(the Alexander law), and is devoid of vertical skew deviation on cover-uncover abnormality that indicates
testing. ipsilateral peripheral
Most patients with acute unilateral vestibulopathy will improve with time, but vestibular hypofunction. It is
a cortically generated
up to 50% of patients have some chronic residual symptoms.40 Hypotheses for
response to the loss of the
prolonged and incomplete recovery range from more intuitive theories such as normal vestibular ocular
age-related poor central compensation, severity of the degree of vestibular loss,41 reflex. A catch-up saccade
vestibular suppressant use, and physical inactivity to unexpected negative with a quick turn to the right
prognosticators such as increased visual dependence, anxiety/depression, fear of indicates the right is
hypofunctional, and a
bodily sensations, and autonomic arousal (CASE 5-3).42 catch-up saccade with a
Evidence-based guidelines support the use of vestibular physical therapy in quick turn to the left
the rehabilitation of acute unilateral vestibulopathy43 with little risk of harm to indicates the left is
the patient. Corticosteroids do not appear to change long-term outcomes, but hypofunctional.
they may hasten recovery.44 Function at 12 months after acute unilateral

CONTINUUMJOURNAL.COM 413

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

ACUTE VESTIBULAR SYNDROME

vestibulopathy is similar despite combined vestibular therapy and steroids or

steroids alone. Betahistine and cinnarizine are two vestibular suppressants
available outside of the United States about which conflicting studies exist
arguing for and against their use in peripheral vestibular disorders. Betahistine
does appear to have a favorable safety profile and may be effective, but the
quality of evidence is low.45 The US Food and Drug Administration (FDA) has
not approved betahistine and precludes its distribution across state lines, but it is
available for compounding in the United States. Cinnarizine in combination with
betahistine may be more effective46 than betahistine alone, although quality
studies are lacking. Because of concerns for increased risk of parkinsonism from
cinnarizine, the FDA has not approved its use,47 although its use is prevalent in
Europe and Oceania.

CASE 5-4 A 33-year-old right-handed woman presented to the emergency

department with new-onset, continuous room-spinning dizziness that
came on suddenly while sitting in front of her computer. She had no
history of stroke, vascular risk factors, or chronic medical conditions.
Her examination revealed normal vital signs. She had no cranial nerve,
motor, sensory, or reflex deficits. Her gait was unstable, and she veered
to her left with a near fall and needed minimal gait assistance. Her ocular
motor examination performed with fixation was normal. When the
penlight was shined in her eyes, a subtle jerk nystagmus was detected,
which was horizontal with the fast phase to the patient’s right. A head
impulse test demonstrated a corrective saccade with head impulse to the
left. She had no vertical skew on a cover-uncover test.
She was provided reassurance and discharged home with plans for
vestibular therapy. She did well with therapy and was symptom free on
follow-up 3 months later. Five months after the initial presentation, she
presented to the emergency department again with sudden-onset eye
pain. MRI revealed demyelinating plaques of the optic nerve, brainstem,
and cerebellum. A lumbar puncture was normal, but serum aquaporin-4
was positive, confirming the diagnosis of neuromyelitis optica spectrum
disorder.

COMMENT This is a rare case of neuromyelitis optica spectrum disorder with a lesion
initially in the root entry zone of the vestibulocochlear nerve that appeared
compatible with isolated peripheral vestibulopathy. However, with further
time, symptom evolution, and neuroimaging, neuromyelitis optica
spectrum disorder was suspected and confirmed by serology. This case
demonstrates the confusing presentation of root entry zone disease as
seemingly peripheral. It also demonstrates the broad differential of acute
vestibular syndrome, including various central pathologies, and the
importance of long-term follow-up, particularly when other neurologic
symptoms evolve.51

414 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

RARE ETIOLOGIES OF ACUTE VESTIBULAR SYNDROME KEY POINTS
Situational clues may highlight some of the rare causes of acute vestibular
● Abnormalities in the
syndrome. Blunt trauma and whiplash have been documented to cause central as pathways, peripheral to
well as peripheral vestibulopathy. Lesser recognized blast trauma can also cause central, of the ocular tilt
acute vestibular syndrome and may have different symptomatology than classic reaction can result in skew
posttraumatic syndromes with more exercise intolerance inducing dizziness.48 deviation; although skew
deviation is usually a central
Demyelinating diseases such as multiple sclerosis49 and neuromyelitis optica
finding, it can sometimes be
spectrum disorder50 may impact the vestibulocerebellum or vestibulocochlear present with acute unilateral
nerve and its root entry zone (CASE 5-4). vestibular loss.
Autoimmune inner ear disease is typically aggressive and bilateral, but this
may be the first of a sequential presentation. Other autoimmune disorders such ● HINTS Plus in acute
vestibular syndrome due to
as autoimmune cerebellitis, rhombencephalitis, paraneoplastic syndromes, and vestibular neuritis consists
anti-GQ1b52 syndromes have also been associated with acute vestibular of an abnormal head impulse
syndrome. Typically, though, these involve more than a singular episode of test with a catch-up
vertigo and have a somewhat subacute progression and other associated saccade toward the side
affected, spontaneous
neurologic symptoms. An empiric steroid trial for autoimmune inner ear disease unidirectional nystagmus
should be considered in patients with aggressive, subacute, fluctuating, bilateral with fast phases away from
vestibular, and cochlear symptoms. FIGURE 5-2 shows the rare and common the affected ear, and the
differential diagnoses to consider in acute vestibular syndrome. absence of skew eye
deviation on cover-uncover
testing.
TRENDS
Although considerable advances in the understanding of acute vestibular ● Mood disorders and
syndrome have developed over the past decade, many exciting topics of research inactivity can prolong or
cause incomplete recovery
are still emerging. New technologies allowing for on-demand interpretation of
from symptoms of acute
eye movements may, at some point, play a larger role as the number of unilateral vestibulopathy.
neuro-otologists cannot keep up with disease burden; telemedicine and machine
learning may help substitute for expertise.25 Devices can focus on interpretation ● Evidence-based
of spontaneous nystagmus as well as head impulse testing to pick up subtleties guidelines support the use
of vestibular physical
the untrained, inexperienced, or naked eye may miss. In the direction opposite of therapy alone in the
technologic advances, a case report found the simple bucket test of the subjective treatment of classic acute
visual vertical may be quite useful in acute vestibular syndrome differentiation,53 unilateral vestibulopathy
but further study is needed. The subjective visual vertical is the patient’s ability resulting from acute
vestibular syndrome.
to perceive true vertical accurately. Defects in subjective visual vertical are
associated with otolithic dysfunction. Subjective visual vertical can be tested by ● Physical and barotrauma
simply marking a line in a bucket, offsetting the line from true vertical, and then can cause central and
asking the patient to realign with their perception of vertical. Based on how peripheral vestibular
dysfunction.
accurately (in degrees of rotation) the patient aligns with true vertical, subjective
visual vertical can be measured. ● An empiric steroid trial for
The video head impulse test is undergoing scrutiny as evidence suggests it can autoimmune inner ear
be abnormal in cerebellar (and potentially other central) conditions and may not disease should be
be a purely peripheral assessment.54 Understanding the nuances of central considered in patients with
aggressive, subacute,
findings is key to accurate assessment of acute vestibular syndrome. fluctuating, bilateral
Outside of eye movement evaluations, as previously discussed, truncal ataxia vestibular, and cochlear
scores may provide another measure for central versus peripheral causes for symptoms.
acute vestibular syndrome. One study has already found that truncal ataxia
scoring is an effective screen that requires less expertise for reliable retesting27
and paves the way for an ongoing clinical trial comparing HINTS with
STANDING (SponTAneous, Nystagmus, Direction, head Impulse test,
standiNG), an algorithm that consists of two ocular motor examinations (head
impulse test and detection of nystagmus), detection of ataxia, and practice of

CONTINUUMJOURNAL.COM 415

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

ACUTE VESTIBULAR SYNDROME

FIGURE 5-2
Schematic to frame differential diagnoses of acute vestibular syndrome.
AEDs = antiepileptic drugs; CO = carbon monoxide; MS = multiple sclerosis; mTBI = mild traumatic brain
injury; NMOSD = neuromyelitis optica spectrum disorder.
Modified with permission from Kerber KA and Newman-Toker DE, Neurol Clin.6 © 2015 Elsevier Inc.

release maneuvers. STANDING was proposed in 2015 for diagnosis of the central
causes of acute vestibular syndrome in emergencies in a 1-year prospective
monocentric study.55 The ongoing study seeks to aid in understanding the
accuracy of HINTS when nonspecialists perform it, as well as comparing it with
STANDING.
Medications as treatment for acute vestibular syndrome, particularly
peripheral acute vestibular syndrome, are still controversial. Of medications that
are available in the United States, only supportive medications, mostly
antiemetic medications and antihistamines, have been studied for symptom
management in the first 24 to 48 hours of onset. Few quality trials exist, but
evidence suggests that promethazine 25 mg IM or IV, as well as dimenhydrinate
50 mg to 100 mg IV, are more effective and less sedating than IV
benzodiazepines.56-58 Outside of the United States, the debate surrounding the
utility and safety of betahistine, cinnarizine, and flunarizine continues.

CONCLUSION
Acute vestibular syndrome may seem intimidating because of considerable
overlap of symptoms with vestibular and nonvestibular causes, including both
serious and benign causes. A commonsense approach followed by targeted
HINTS Plus examination and vascular risk factor assessment can help improve
diagnostic accuracy. Stroke is common enough and variable enough in
presentation to be considered in every patient with acute vestibular syndrome;
however, when no acute unilateral hearing loss, central pattern nystagmus, or
severe truncal ataxia is present, this is reassuring for a benign acute unilateral
vestibulopathy process. Acute unilateral vestibulopathy can stem from etiologies
ranging from inflammation to demyelination and improves with time and
vestibular physical therapy. When applied systematically, the preceding
recommendations can demystify dizziness, improving provider confidence and
patient outcomes.

416 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

USEFUL WEBSITES AND APPLICATIONS
VESTIBULAR DISORDERS ASSOCIATION aVOR APPLICATION
This website serves as a patient education and This application is a teaching, training, and test tool
resource center regarding vestibular disorders. for the vestibulo-ocular reflex (VOR) system and its
vestibular.org disorders.

NEURO-OPHTHALMOLOGY VIRTUAL EDUCATION LIBRARY MIMI HEARING TEST APPLICATION

This video library features representative videos of This application provides a hearing test that
ocular motor findings in vestibular disorders can be used for the bedside audiometry in
including acute vestibular syndrome. HINTS Plus.
novel.utah.edu

REFERENCES

1 Hotson JR, Baloh RW. Acute vestibular syndrome. 10 Kerber KA, Brown DL, Lisabeth LD, et al. Stroke
N Engl J Med 1998;339(10):680-685. doi:10.1056/ among patients with dizziness, vertigo, and
NEJM199809033391007 imbalance in the emergency department: a
population-based study. Stroke 2006;37(10):
2 Bisdorff AR, Staab JP, Newman-Toker DE.
2484-2487. doi:10.1161/01.STR.0000240329.
Overview of the International Classification of
48263.0d
Vestibular Disorders. Neurol Clin 2015;33(3):
541-550, vii. doi:10.1016/j.ncl.2015.04.010 11 Newman-Toker DE, Hsieh Y-H, Camargo CA Jr,
et al. Spectrum of dizziness visits to US
3 Kattah JC, Talkad AV, Wang DZ, et al. HINTS to
emergency departments: cross-sectional
diagnose stroke in the acute vestibular
analysis from a nationally representative sample.
syndrome: three-step bedside oculomotor
Mayo Clin Proc 2008;83(7):765-775.
examination more sensitive than early MRI
doi:10.4065/83.7.765
diffusion-weighted imaging. Stroke 2009;40(11):
3504-3510. doi:10.1161/STROKEAHA.109.551234 12 Lopez-Escamez JA, Carey J, Chung W-H, et al.
Diagnostic criteria for Menière's disease. J Vestib
4 Caplan L, Chung C-S, Wityk R, et al. New England
Res 2015;25(1):1-7. doi:10.3233/VES-150549
Medical Center Posterior Circulation Stroke
Registry: I. Methods, data base, distribution of 13 Sekitani T, Imate Y, Noguchi T, Inokuma T.
brain lesions, stroke mechanisms, and outcomes. Vestibular neuronitis: epidemiological survey by
J Clin Neurol 2005;1(1):14-30. doi:10.3988/ questionnaire in Japan. Acta Otolaryngol Suppl
jcn.2005.1.1.14 1993;503:9-12. doi:10.3109/00016489309128061
5 Siniscalchi A, Sztajzel R, Malferrari G, Gallelli L. 14 Choi J-H, Park M-G, Choi SY, et al. Acute transient
The National Institutes of Health Stroke Scale: its vestibular syndrome: prevalence of stroke and
role in patients with posterior circulation stroke. efficacy of bedside evaluation. Stroke 2017;48(3):
Hosp Top 2017;95(4):79-81. doi:10.1080/ 556-562. doi:10.1161/STROKEAHA.116.015507
00185868.2017.1322888
15 Kim HA, Yi HA, Lee H. Recent advances in
6 Kerber KA, Newman-Toker DE. Misdiagnosing orthostatic hypotension presenting orthostatic
dizzy patients: common pitfalls in clinical dizziness or vertigo. Neurol Sci 2015;36(11):
practice. Neurol Clin 2015;33(3):565-575. 1995-2002. doi:10.1007/s10072-015-2363-2
doi:10.1016/j.ncl.2015.04.009
16 Gorelik O, Feldman L, Cohen N. Heart failure and
7 Saber Tehrani AS, Kattah JC, Mantokoudis G, orthostatic hypotension. Heart Fail Rev 2016;
et al. Small strokes causing severe vertigo: 21(5):529-538. doi:10.1007/s10741-016-9541-z
frequency of false-negative MRIs and
17 Seale B, Ahanger S, Hari C. Subacute carbon
nonlacunar mechanisms. Neurology 2014;83(2):
monoxide poisoning presenting as vertigo and
169-173. doi:10.1212/WNL.0000000000000573
fluctuating low frequency hearing loss. J Surg
8 Kerber KA, Meurer WJ, West BT, Fendrick AM. Case Rep 2018;2018(8):rjy205. doi:10.1093/jscr/
Dizziness presentations in U.S. emergency rjy205
departments, 1995-2004. Acad Emerg Med 2008;
18 Peragallo J, Biousse V, Newman NJ. Ocular
15(8):744-750. doi:10.1111/j.1553-2712.2008.00189.x
manifestations of drug and alcohol abuse. Curr
9 Ljunggren M, Persson J, Salzer J. Dizziness and Opin Ophthalmol 2013;24(6):566-573. doi:10.1097/
the acute vestibular syndrome at the emergency ICU.0b013e3283654db2
department: a population-based descriptive
19 Young AS, Rosengren SM, Welgampola MS.
study. Eur Neurol 2018;79(1-2):5-12.
Disorders of the inner ear balance organs and
doi:10.1159/000481982
their pathways. In: Day BL, Lord SR, eds.
Handbook of clinical neurology. Amsterdam,
Netherlands: Elsevier, 2018:385-401.

CONTINUUMJOURNAL.COM 417

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

ACUTE VESTIBULAR SYNDROME

20 Venhovens J, Meulstee J, Verhagen WIM. Acute 32 Kerber KA, Zahuranec DB, Brown DL, et al. Stroke
vestibular syndrome: a critical review and risk after nonstroke emergency department
diagnostic algorithm concerning the clinical dizziness presentations: a population-based
differentiation of peripheral versus central cohort study. Ann Neurol 2014;75(6):899-907.
aetiologies in the emergency department. doi:10.1002/ana.24172
J Neurol 2016;263(11):2151-2157. doi:10.1007/
33 Arbusow V, Schulz P, Strupp M, et al. Distribution
s00415-016-8081-8
of herpes simplex virus type 1 in human
21 Kung NH, Van Stavern GP, Gold DR. HINTS in the geniculate and vestibular ganglia: implications
acute vestibular syndrome: pearls and pitfalls. for vestibular neuritis. Ann Neurol 1999;46(3):
J Neuroophthalmol 2018;38(2):244-250. 416-419. doi:10.1002/1531-8249(199909)46:3<416::
doi:10.1097/WNO.0000000000000608 aid-ana20>3.0.co;2-w
22 Newman-Toker DE, Kerber KA, Hsieh Y-H, et al. 34 Himmelein S, Lindemann A, Sinicina I, et al.
HINTS outperforms ABCD2 to screen for stroke in Differential involvement during latent herpes
acute continuous vertigo and dizziness. Acad simplex virus 1 infection of the superior and
Emerg Med 2013;20(10):986-996. doi:10.1111/ inferior divisions of the vestibular ganglia:
acem.12223 implications for vestibular neuritis. J Virol 2017;
91(14):e00331-17. doi:10.1128/JVI.00331-17
23 Quimby AE, Kwok ESH, Lellie D, et al. Usage of the
HINTS exam and neuroimaging in the assessment 35 Strupp M, Zingler VC, Arbusow V, et al.
of peripheral vertigo in the emergency Methylprednisolone, valacyclovir, or the
department. J Otolaryngol Head Neck Surg 2018; combination for vestibular neuritis. N Engl J Med
47(1):54. doi:10.1186/s40463-018-0305-8 2004;351(4):354-361. doi:10.1056/NEJMoa033280
24 Kerber KA, Meurer WJ, Brown DL, et al. Stroke risk 36 Jackler RK. Letter to the editor comment on
stratification in acute dizziness presentations: a “Ramsay Hunt syndrome.” Otol Neurotol 2017;
prospective imaging-based study. Neurology 38(10):1549. doi:10.1097/MAO.0000000000001606
2015;85(21):1869-1878. doi:10.1212/WNL.
37 Kim HJ, Jung J, Kim SS, et al. Comparison of
0000000000002141
acyclovir and famciclovir for Ramsay Hunt
25 Mantokoudis G, Gold DR, Newman-Toker DE. syndrome. Otol Neurotol 2017;38(5):754-758.
Video-oculography in the emergency doi:10.1097/MAO.0000000000001367
department: an “ECG” for the eyes in the acute
38 Taylor RL, McGarvie LA, Reid N, et al. Vestibular
vestibular syndrome. In: Shaikh A, Ghasia F, eds.
neuritis affects both superior and inferior
Advances in translational neuroscience of eye
vestibular nerves. Neurology 2016;87(16):
movement disorders. Cham, Switzerland:
1704-1712. doi:10.1212/WNL.0000000000003223
Springer International Publishing, 2019:283-307.
39 Safran AB, Vibert D, Issoua D, Häusler R. Skew
26 Kattah JC. Update on HINTS plus, with discussion
deviation after vestibular neuritis. Am J
of pitfalls and pearls. J Neurol Phys Thery 2019;
Ophthalmol 1994;118(2):238-245. doi:10.1016/
43(suppl 2):S42-S45. doi:10.1097/NPT.
s0002-9394(14)72904-6
0000000000000274
40 Halmagyi GM, Weber KP, Curthoys IS. Vestibular
27 Carmona S, Martínez C, Zalazar G, et al. The
function after acute vestibular neuritis. Restor
diagnostic accuracy of truncal ataxia and HINTS
Neurol Neurosci 2010;28(1):37-46. doi:10.3233/
as cardinal signs for acute vestibular syndrome.
RNN-2010-0533
Front Neurol 2016;7:125. doi:10.3389/
fneur.2016.00125 41 Navari E, Casani AP. Lesion patterns and possible
implications for recovery in acute unilateral
28 Navi BB, Kamel H, Shah MP, et al. Application of
vestibulopathy. Otol Neurotol 2020;41(2):
the ABCD2 score to identify cerebrovascular
e250-e255. doi:10.1097/MAO.
causes of dizziness in the emergency
0000000000002476
department. Stroke 2012;43(6):1484-1489.
42 Cousins S, Kaski D, Cutfield N, et al. Predictors of
29 Kim HA, Lee H, Kim J-S. Vertigo due to vascular
clinical recovery from vestibular neuritis: a
mechanisms. Semin Neurol 2020;40(1):67-75.
prospective study. Ann Clin Transl Neurol 2017;4:
doi:10.1055/s-0039-3402737
340-346. doi:10.1002/acn3.386
30 Cnyrim CD, Newman-Toker D, Karch C, et al.
43 Hall CD, Herdman SJ, Whitney SL, et al. Vestibular
Bedside differentiation of vestibular neuritis
rehabilitation for peripheral vestibular
from central “vestibular pseudoneuritis.”
hypofunction: an evidence-based clinical
J Neurol Neurosurg Psychiatry 2008;79(4):
practice guideline. From the American Physical
458-460. doi:10.1136/jnnp.2007.123596
Therapy Association Neurology Section. J Neurol
31 Atzema CL, Grewal K, Lu H, et al. Outcomes Phys Ther 2016;40(2):124-155. doi:10.1097/
among patients discharged from the emergency NPT.0000000000000120
department with a diagnosis of peripheral
44 Ismail EI, Morgan AE, Abdel Rahman AM.
vertigo. Ann Neurol 2016;79(1):32-41. doi:10.1002/
Corticosteroids versus vestibular rehabilitation in
ana.24521
long-term outcomes in vestibular neuritis.
J Vestib Res 2018;28(5-6):417-424. doi:10.3233/
VES-180645

418 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

45 Murdin L, Hussain K, Schilder AGM. Betahistine 52 Lee S-U, Kim H-J, Choi J-Y, et al. Acute vestibular
for symptoms of vertigo. Cochrane Database syndrome associated with anti-GQ1b antibody.
Syst Rev 2016;(6):CD010696. doi: Neurology 2019;93(11):e1085-e1092. doi:10.1212/
10.1002/14651858.CD010696.pub2 WNL.0000000000008107
46 Asadi P, Zia Ziabari SM, Majdi A, et al. 53 Nateghi Gillberg B, Panizo Benito CM, Tjernström
Cinnarizine/betahistine combination vs. the F. “The bucket test” can be helpful to distinguish
respective monotherapies in acute peripheral central from peripheral acute vestibular
vertigo: a randomized triple-blind placebo- syndrome—a case report. Acta Otolaryngol Case
controlled trial. Eur J Clin Pharmacol 2019; Rep 2019;4:17-20. doi:10.1080/23772484.
75(11):1513-1519. doi:10.1007/s00228-019-02741-x 2019.1601495
47 Fabiani G, Pastro PC, Froehner C. Parkinsonism 54 Halmagyi GM, Chen L, MacDougall HG, et al. The
and other movement disorders in outpatients in video head impulse test. Front Neurol 2017;8:258.
chronic use of cinnarizine and flunarizine. doi:10.3389/fneur.2017.00258
Arq Neuropsiquiatr 2004;62(38):784-788. doi:
55 ClinicalTrials.gov. Eye-ECG approach to
10.1590/s0004-282x2004000500008
emergencies: diagnostic performance of the
48 Gottshall KR, Whitney SL. Vestibular HINTS test. Updated October 8, 2019. Accessed
rehabilitation for mild traumatic brain injury December 29, 2020. clinicaltrials.gov/ct2/show/
(mTBI). In: Hoffer ME, Balaban CD, eds. NCT04118361
Neurosensory disorders in mild traumatic brain
56 Saberi A, Pourshafie SH, Kazemnejad-Leili E, et al.
injury. Cambridge, England: Academic Press,
Ondansetron or promethazine: which one is
2019:339-355.
better for the treatment of acute peripheral
49 Pula JH, Newman-Toker DE, Kattah JC. Multiple vertigo? Am J Otolaryngol 2019;40(1):10-15.
sclerosis as a cause of the acute vestibular doi:10.1016/j.amjoto.2018.09.010
syndrome. J Neurol 2013;260:1649-1654.
57 Shafipour L, Goli Khatir I, Shafipour V, et al.
doi:10.1007/s00415-013-6850-1
Intravenous promethazine versus diazepam for
50 Dhakar MB, Sivakumar S, NavidSeraji-Bozorgzad, treatment of peripheral vertigo in emergency
et al. Brain MRI lesions in neuromyelitis optica department. J Mazandaran Univ Med Sci 2017;
spectrum disorders. Accessed February 26, 27:88-98.
2020. austinpublishinggroup.com/clinical-
58 Amini A, Heidari K, Asadollahi S, et al. Intravenous
neurology/ajcn-v2-id1064.php
promethazine versus lorazepam for the
51 Guo Q, Han D, Liu J, et al. Neuromyelitis optica treatment of peripheral vertigo in the emergency
spectrum disorders with vertigo as the initial department: a double blind, randomized clinical
symptom: a case report. Medicine 2019;98(34): trial of efficacy and safety. J Vestib Res 2014;
e16906. doi:10.1097/MD.0000000000016906 24(1):39-47. doi:10.3233/VES-130506

CONTINUUMJOURNAL.COM 419

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 REVIEW ARTICLE


Episodic Positional
C O N T I N UU M A UD I O
I NT E R V I E W A V AI L A B L E
ONLINE
Dizziness
By Kevin A. Kerber, MD, MS
Downloaded from http://journals.lww.com/continuum by wqLe/H3/oM2vx1Qs5A5qMemKKHLPqE4qkmGNpdheG3ikf1o85ttJSkFL2oTkgOkhMEmawHvsuTTE5xJG2ZjqccytIAhVn2qegy060KLcKJuPZsx5htaYiopRjp2zor2egUzWJAEWYNo65TEub0G9tZtPaMachiqn on 04/28/2022

ABSTRACT
PURPOSE OF REVIEW: This article provides a summary of the evaluation and
treatment of patients presenting with episodic positional dizziness.

RECENT FINDINGS: Positional components are nearly ubiquitous among

diagnoses of dizziness, so it can be challenging to classify patients with
episodic positional dizziness simply based on the history of present illness.
Overreliance on the presence of a report of positional components has
likely resulted in misapplication or misinterpretation of positional testing
and negative experiences with maneuvers to treat positional dizziness. The
prototypical episodic positional dizziness disorder is benign paroxysmal
positional vertigo (BPPV). BPPV is caused by free-floating particles in a
semicircular canal that move in response to gravity. The diagnosis is made
by identifying the characteristic patterns of nystagmus on the Dix-Hallpike
test. Particle repositioning for BPPV is supported by randomized
CITE AS:
CONTINUUM (MINNEAP MINN) controlled trials, meta-analyses, and practice guidelines. Other disorders
2021;27(2, NEURO-OTOLOGY): that can present with episodic positional dizziness are migraine dizziness,
348–368.
central lesions, and light cupula syndrome.
Address correspondence to
Dr Kevin Kerber, Department of SUMMARY: Episodic positional dizziness is a common presentation of
Neurology, University of dizziness. Neurologists should prioritize identifying and treating BPPV;
Michigan Health System, 1500 E
Medical Center Dr, Ann Arbor, doing so provides an important opportunity to deliver effective and
MI 48109, [email protected]. efficient care. Providers should also recognize that positional components
are common in most causes of dizziness and, therefore, should not
RELATIONSHIP DISCLOSURE:
Dr Kerber has served as a over-rely on this part of the history of presentation when considering the
section editor for Neurology diagnosis and management plan.
and as a consultant for Bind, Inc;
has received research/grant
support from the Agency for
Healthcare Research and
Quality (R18 HS02225), American INTRODUCTION

E
Academy of Neurology, and pisodic positional dizziness is a label used to describe a category of
National Institutes of Health
(R01DC012760-06A1, R01DC012760, dizziness presentations characterized by recurrent events and
U01DC013778-01A1); and has prominent positional components or triggers. The dizziness can be a
received publishing royalties
variety of types, including spinning, tilting, and other sensations of
from Oxford University Press.
movement, lightheadedness, or imbalance. The positional components
UNLABELED USE OF are also broad, including standing up, turning the head, tilting the head back, or
PRODUCTS/INVESTIGATIONAL
USE DISCLOSURE:
rolling over in bed. Other categories of dizziness presentations include the acute
Dr Kerber reports no disclosure. vestibular syndrome, episodic spontaneous dizziness, and chronic constant
dizziness. Episodic positional dizziness has a variety of causes. A priority in these
© 2021 American Academy presentations is identifying people who may have benign paroxysmal positional
of Neurology. vertigo (BPPV) and, therefore, benefit from a canalith repositioning maneuver,

348 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

which is evidence-based and guideline supported.1-4 Despite this, providers KEY POINT
continue to have misconceptions about BPPV and underuse or misuse the
● Patients with any cause of
canalith repositioning maneuver.5-7 This article provides a summary of the dizziness typically report
evaluation and management of episodic positional dizziness. positional components. As a
result, providers should be
EPIDEMIOLOGY cautious in relying on the
self-report of positional
Approximately 15% of adults in the United States report problems with dizziness
components to make a
in the past 12 months inclusive of symptoms such as imbalance/unsteadiness, diagnosis of benign
lightheadedness, feeling as if passing out/fainting, spinning/vertigo, paroxysmal positional
floating/tilting sensation, and blurring of vision when moving the head.8 The vertigo (BPPV).
lifetime prevalence of BPPV has been estimated to be 10% by using data from a
random–digit dial national health survey in Germany.9 Self-reported positional
components are extremely common among all patients with dizziness in general,
which can make it challenging to classify a case as episodic positional dizziness as
opposed to episodic spontaneous dizziness or even the acute vestibular
syndrome. More than 90% of individuals who report problems with dizziness in
the past 12 months describe one or more of the following triggers: turning the
head side to side, looking up or down, rolling over in bed, getting up after sitting
or lying down, or standing or being on their feet for a long time (data from the
2008 National Health Interview Survey).10 Approximately 33% of all patients
with problems with dizziness in the past 12 months report typical BPPV triggers
of turning the head side to side, looking up or down, and rolling over in bed. The
very high frequency of positional components makes it a challenge to rely on
the patient's report of positional components to make a diagnosis or even classify
the category of dizziness presentation.

TAKING THE HISTORY

The history of present illness in patients with episodic positional dizziness is
important. However, the history of the dizziness should not be overemphasized
when making management decisions. It has been demonstrated that frontline
doctors generally overemphasize the history of present illness in patients with
dizziness, and this may be a key factor in negative experiences with treating
patients with dizziness. Frontline providers specifically report negative
experiences in using the Dix-Hallpike test and canalith repositioning
maneuvers.6 Providers should not use types of dizziness as rigid branch points in
diagnostic considerations because verbatim descriptions of dizziness are
generally vague, most patients select more than one type of dizziness, the
selection of a primary type of dizziness is typically not reliable, and types of
dizziness are actually poor discriminators among causes of dizziness.8,11 Patients
are generally more reliable in their report of timing and triggers of symptoms.11
However, positional triggers are nearly ubiquitous among all causes of dizziness.
Experience in clinical practice has found that the history of dizziness symptoms
can be nearly identical in patients who present acutely with either BPPV or an
acute unilateral vestibulopathy (eg, vestibular neuritis). Patients with either
diagnosis often report the onset of symptoms after getting out of bed, severe
symptoms with subsequent movements, and improvement when still, with
milder constant dizziness.
The evaluation of patients with dizziness generally starts by asking them to
use their own words to describe the symptom; though, as previously noted, the
neurologist should be careful not to overemphasize the description. Next, it is

CONTINUUMJOURNAL.COM 349

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

important to obtain details about the timing and triggers of symptoms. Defining
how long ago the dizziness started helps in considering whether this is an acute,
subacute, or chronic disorder. The frequency and duration of the episodes should
be determined. In considering the self-report of timing and triggers, providers
also need to be careful to not overemphasize these components. For example,
BPPV is considered a brief dizziness, typically lasting less than 1 minute. However,
many patients with BPPV report more prolonged symptoms that can even be
constant,4 although usually the duration of the most intense symptoms is still brief.
Therefore, even patients who report prolonged symptoms should be assessed for
BPPV unless another clear cause is identified before positional testing.
Lastly, providers should also gather information about the course of the
symptoms over time in terms of whether they have improved, worsened, or
stabilized. It can also be helpful to ask about any patterns with the symptoms,
such as whether they tend to occur at certain times of the day or in association
with certain foods, stress, or sleep.

EXAMINATION
For patient evaluations in typical office or emergency department settings, the
most important diagnostic information is usually obtained on the physical
examination. Emphasizing the examination is a strategy that can prevent
focusing on the often unreliable or overlapping components of the history of
present illness.
The examination should start with a focused general medical and general
neurologic assessment. The neuro-otologic components can be incorporated into
the general neurologic examination or grouped at the end. The neuro-otologic
examination consists of the ocular motor assessment, coordination tasks, balance
assessment, and positional testing. Providers may find it useful to incorporate
these elements into routine examinations so they can establish internal
thresholds of normal versus abnormal findings.

Ocular Motor Examination

For some patients with episodic positional dizziness, the key examination
findings are obtained on the bedside ocular motor assessment. For example,
some patients who report episodic positional dizziness can have mild cerebellar
disorders that are rapidly identified on the ocular motor assessment. These
findings include spontaneous downbeat nystagmus, bidirectional gaze-evoked
nystagmus, and pathologically impaired smooth pursuit. The ocular motor
examination starts with observing the eyes in primary gaze for 5 to 10 seconds
and specifically looking for any spontaneous movements such as nystagmus or
saccadic intrusions. Nystagmus has alternating slow and fast components that
give the appearance that the eyes are beating in the direction of the fast phase.
Saccadic intrusions are involuntary saccades that intrude upon gaze. Square-
wave jerks are the most common type of saccadic intrusion. Square-wave jerks
consist of an involuntary saccade to one side, a brief intersaccade delay, and then
a saccade back, giving the appearance of shifting, as opposed to the rhythmic
beating of nystagmus. Square-wave jerks are nonspecific and generally not
pathologic when an isolated finding; they are particularly common when people
are anxious. Other types of saccadic intrusions are ocular flutter and opsoclonus,
which are pathologic and commonly occur in people with autoimmune
cerebellitis and Friedreich ataxia.

350 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 After observing for a spontaneous movement, the clinician should then have KEY POINTS
the patient follow the examiner’s finger to lateral gaze to each side and then up
● Patients with dizziness
and down. In each position, the eyes are held at approximately 30 degrees off are often not reliable in their
center and observed for nystagmus for about 5 to 10 seconds. The clinician should self-report of the type of
take advantage of the transitions of gaze testing to also assess smooth pursuit. dizziness. As a result, BPPV
Smooth pursuit is normal when the patient can track the finger back and forth should be considered a
diagnostic possibility even in
very smoothly. Impaired smooth pursuit is considered a nonlocalizing finding.
patients who do not report
However, when impaired smooth pursuit is identified in patients who are vertigo.
cognitively intact and without other large motor deficits (eg, hemiplegia), it is a
strong indicator of cerebellar dysfunction. When smooth pursuit is impaired, the ● The ocular motor
individual must use the saccade system to move the eyes to the side. Therefore, examination starts with
observing the eyes in
impaired smooth pursuit is typically saccadic pursuit. The saccade system can primary gaze for 5 to
be assessed by having a patient look back and forth from one finger to the next 10 seconds and specifically
and observing for the speed of the movement and the accuracy. Hypometric looking for any spontaneous
saccades are generally not a pathologic finding if they are isolated. Consistent movements such as
nystagmus or saccadic
hypermetric saccades are suggestive of cerebellar dysfunction. This ocular motor intrusions.
assessment generally takes less than 1 minute to perform and is important to
perform before proceeding to positional testing. Patients with vestibular neuritis ● When impaired smooth
may have a history suggestive of episodic positional dizziness but, on close pursuit is identified in
patients who are cognitively
inspection, will have unidirectional (ie, does not change direction) horizontal
intact and without other
nystagmus in primary gaze and lateral gaze. Many providers do not appreciate large motor deficits (eg,
the physiology of BPPV is such that nystagmus is present only when the particles hemiplegia), it is a strong
are in motion, and the particles are typically at rest when the head is still. When indicator of cerebellar
dysfunction.
shown videos of a typical acute unilateral vestibulopathy pattern of nystagmus in
a patient with vestibular neuritis, one study found that primary providers nearly ● Eye movement testing,
always erroneously selected BPPV as the cause.6 In the same study, these including pursuit tracking,
providers also typically reported negative experiences using the Dix-Hallpike test gaze testing, and saccadic
and canalith repositioning maneuvers. It is likely that their negative experiences eye movement observation,
takes very little time but can
in using the Dix-Hallpike test and Epley maneuver are based on an incorrect be the key factor in
selection of patients and interpretation of the results. identifying a cerebellar or
vestibular disorder.
Head Impulse Test
● The Dix-Hallpike test is
The head impulse test is another component of the ocular motor assessment. It is
designed to identify
a bedside measure of the vestibular ocular reflex (described further in the section posterior canal BPPV but
Evaluation and Management of the Patient With Episodic Positional Dizziness) can also identify the
and an important part of the evaluation of patients with the acute vestibular horizontal and anterior canal
variants and central causes
syndrome. This is generally less applicable in patients who have episodic
of dizziness.
positional dizziness such as BPPV.

Coordination and Balance Assessment

General gait and coordination assessments are used to identify cerebellar
dysfunction, which can be a key indicator of a central disorder.

Positional Testing
Positional testing includes the Dix-Hallpike test and supine positional testing
(FIGURE 3-112 and FIGURE 3-2). Different strategies can be used for the approach
to positional testing. A common strategy is to start with the Dix-Hallpike test to
one side and then, if negative, performing the test on the other side. The reason
for the testing positions of the Dix-Hallpike test is that particles can be evaluated
one side at a time based on the plane of the canal. The Dix-Hallpike test primarily

CONTINUUMJOURNAL.COM 351

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

FIGURE 3-1
Dix-Hallpike test for the diagnosis of right posterior canal benign paroxysmal positional
vertigo (BPPV). The patient’s head is turned 45 degrees to the side to be tested (step 1) and
then laid back quickly (step 2). If BPPV is present, nystagmus ensues usually within seconds.
Reprinted with permission from Fife TD, et al, Neurology.12 © 2008 American Academy of Neurology.

FIGURE 3-2
The supine roll test to detect horizontal canal benign paroxysmal positional vertigo (BPPV).
The patient may be moved from sitting to a straight supine position (step 1). The head is
turned to the right side (step 2) with observation of nystagmus and then turned back to face
up (step 1). Then the head is turned to the left side (step 3). The side with the most prominent
nystagmus is understood to be the affected horizontal semicircular canal. The direction of
nystagmus in each position determines whether the horizontal canal BPPV is of the geotropic
or apogeotropic type.
Reprinted with permission from Barrow Neurological Institute. © 2020 Barrow Neurological Institute, Phoenix,
Arizona.

352 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

assesses for particles in the posterior canal, although it can also identify the
horizontal and anterior canal variants. The outline of the patient’s ear on one side
can be used as a model of the general anatomy of the posterior canal to teach
patients and trainees about BPPV. When the outline of the ear is used as a model,
the particles can be described as accumulating in the lobule area of the ear. It can
then be explained that the goal in particle repositioning is to move the particles
up and around the helix to the superior portion of the ear and then down and out
(FIGURE 3-3). To perform the Dix-Hallpike test on the left side, the patient’s head
is turned approximately 45 degrees to the left side. This puts the plane of the
posterior canal in the direction in which the patient is going to move. The right
posterior canal is out of the plane such that particles in the right posterior canal
will not generally move during the left-side Dix-Hallpike test. The patient is
brought back down to a head-hanging position either over the edge of the bed or
over some pillows placed behind the upper back. The head should be tilted back
approximately 20 degrees so that the particles move enough in the posterior canal
to trigger the symptoms and nystagmus. In addition, at least 20 degrees of
extension is needed for the particles to move far enough to the superior portion
of the posterior canal for the eventual treatment to be successful. Once the
patient is in the head-hanging position, the eyes are observed for approximately
10 to 15 seconds. Because many patients want to close their eyes, the examiner
should be prepared to open them with the explanation that any eye movements
need to be observed by the neurologist. If the test to the left is negative, the
patient should be asked to sit back up and then turn their head to the right side
approximately 45 degrees; the patient should then lie back down with the head
tilted back approximately 20 degrees. The test is considered positive when
nystagmus is triggered by the test and the nystagmus is transient in duration and
accompanied by symptoms. Some patients report symptoms, but no nystagmus
is observed in which case BPPV can still be suspected but not confirmed. The
nystagmus pattern of posterior canal BPPV is upbeat torsional.
Supine positional testing is a test more designed to specifically move particles
in the horizontal canal. However, the positional nystagmus of horizontal canal
BPPV will generally be elicited even on the Dix-Hallpike test. If a horizontal
nystagmus is triggered by the Dix-Hallpike test, then posterior canal BPPV is
excluded. To perform supine positional testing, the patient starts in the supine
position (FIGURE 3-2). Next, the head is turned to one side and held there for 10 to
15 seconds. The head is then brought back to midline and turned to the opposite
side and held for approximately 10 to 15 seconds.

COMMON CAUSES OF EPISODIC POSITIONAL DIZZINESS

The most common vestibular causes of episodic positional dizziness are BPPV,
migraine dizziness, and structural disorders that affect central vestibular pathways.

Benign Paroxysmal Positional Vertigo

BPPV is the most common peripheral vestibular disorder and the prototypical
episodic positional dizziness disorder. Benign is not synonymous with trivial
because 85% of patients with BPPV report missing work, stop driving, or
present for a medical evaluation.9 Patients with BPPV generally report a spinning
type of dizziness, but this is not required. The symptoms are generally brief
and triggered by lying down or rolling over in bed or looking up to reach
for something.

CONTINUUMJOURNAL.COM 353

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

FIGURE 3-3
Canalith repositioning maneuver (ie, the Epley maneuver) for right-sided benign paroxysmal
positional vertigo. Steps 1 and 2 are identical to the Dix-Hallpike test. The patient is held in
the right head-hanging position (step 2) for 20 to 30 seconds and then in step 3 the head is
turned 90 degrees toward the unaffected side. Step 3 is held for 20 to 30 seconds before
turning the head another 90 degrees (step 4) so the head is nearly in the face-down position.
Step 4 is held for 20 to 30 seconds, and then the patient is brought to the sitting up position
(step 5). The movement of the otolith material within the labyrinth is depicted with each step,
showing how otoliths are moved from the semicircular canal to the vestibule. Although it is
advisable for the examiner to guide the patient through these steps, it is the patient’s head
position that is the key to a successful treatment.
Reprinted with permission from Fife TD, et al, Neurology.12 © 2008 American Academy of Neurology.

354 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 BPPV is caused by otoconia, calcium carbonate particles that have detached KEY POINTS
from the utricular macula and enter a semicircular canal on one side (or
● Some patients with BPPV
occasionally both). The reason the particles detach is not clear, but an animal report a constant milder
study found that as rats age the otoconia in both maculae become pitted, dizziness before and even
fissured, and fragmented with weakened or broken linking filaments.13 It is for a time after treatment for
likely that this degeneration makes the otoconia susceptible to detaching or at unclear reasons.
least eroding. It is also believed that the otoconia can detach after head trauma
● The gold standard test for
or an acute unilateral vestibulopathy from either neuritis or ischemia. The BPPV is the Dix-Hallpike
particles are more dense than the endolymph and therefore will move in the test. A positive finding is a
canal in response to gravity. When enough particles in the canal move at a triggered and transient
sufficient speed, fluid drag is created, resulting in aberrant endolymph flow nystagmus.

that deflects the cupula and then modulates the activity of the vestibular
afferents of the affected canal. This is referred to as canalithiasis. It is also
possible for the particles to attach to the cupula of a semicircular canal and
render it sensitive to gravity, which is referred to as cupulolithiasis. The
modulation of the vestibular afferents results in a brief burst of nystagmus and
the associated symptoms. The positional nystagmus can “fatigue” on repeated
testing likely because the particles disperse in the canal. CASE 3-1 describes a
case of BPPV of the posterior canal, and CASE 3-2 describes a case of acute
unilateral vestibulopathy.
Some patients with BPPV report a constant milder dizziness before and even
for a time after treatment for unclear reasons.4 However, the severe recurrent
positional attacks of dizziness occur only when the particles are in the
semicircular canal. Particles can enter any of the three canals: the anterior, the
horizontal, and the posterior (TABLE 3-1). BPPV of the posterior canal is by far
the most common type reported in large case series.2 However, these case series
are derived from outpatient specialty clinics comprising patients who have had
symptoms for days to weeks or even longer. No particular reason exists for the
particles to be more likely to enter the posterior canal than other canals; however,
the reasons are clear why particles are more likely to be stuck in the posterior
canal rather than the anterior or horizontal canal. If the particles enter the
anterior or horizontal canal, natural head positions such as sitting up, lying down,
or rolling over are generally adequate for the particles to fall out of the canal.
However, natural head positions are much less likely to guide particles out of
the posterior canal, which is why the Dix-Hallpike test uses deep extension to
guide the particles to the superior aspect of the posterior canal and then around
and out.
The gold standard test for benign positional vertigo is the Dix-Hallpike test.2,4
A positive finding is a triggered and transient nystagmus and associated
symptoms. The key feature is that the nystagmus is not present when the patient
is sitting still but is then triggered and transient after the patient is placed in the
head-hanging position. The pattern of nystagmus in the posterior canal is
upbeating torsional in the head-hanging position as the particles move into the
superior portion of the posterior canal, creating drag that deflects the cupula
(TABLE 3-1). If the patient is then brought back up to the sitting position without
being treated for the BPPV, generally a burst of downbeat-torsional nystagmus
occurs as the particles move in the opposite direction. The nystagmus usually has
a latency to onset of 1 or a few seconds with the Dix-Hallpike test, a peak velocity
of 30 to 50 degrees per second, and a duration of approximately 10 to 30
seconds.14

CONTINUUMJOURNAL.COM 355

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

Variants of Benign Paroxysmal Positional Vertigo

The main variants of BPPV are the horizontal canal variant and the anterior canal
variant. The pattern of nystagmus in horizontal canal BPPV is direction-changing
horizontal positional nystagmus, which can be either geotropic (a label used to
describe that the fast phase of nystagmus beats toward the ground in each
position: the head positioned to right while supine triggers nystagmus to right,
and the head to left while supine triggers nystagmus to left) or apogeotropic
(a label used to describe that the fast phase of nystagmus beats away from the
ground in each position: the head positioned to the right while supine triggers
nystagmus to left, and the head to left while supine triggers nystagmus to right)
(TABLE 3-1 and FIGURE 3-415). Note that direction-changing positionally triggered
nystagmus is different from direction-changing gaze-evoked nystagmus,
which is a central sign.

CASE 3-1 A 65-year-old woman presented for dizziness symptoms that started
approximately 3 weeks before. The dizziness was described as an odd
sensation, and, on specific questioning, she reported a feeling of spinning
and lightheadedness. She also had a mild constant sense of imbalance. The
most significant symptoms occurred primarily after rolling over in bed during
the night, getting up from bed in the morning, and looking up to reach for
something during the day. Her past medical history was unremarkable.
General medical and neurologic examinations were normal. No
spontaneous or gaze-evoked nystagmus was present. Smooth pursuit was
intact. No coordination abnormalities were noted. She walked with a narrow
base with a good heel strike and stride length.
The Dix-Hallpike test performed with her head turned to the right side did
not trigger symptoms or nystagmus. The Dix-Hallpike test to the left side,
however, triggered a robust burst of upbeat-torsional nystagmus. The
patient was substantially symptomatic and attempted to close her eyes;
therefore, the examiner needed to open the patient’s eyes to be able to see
the nystagmus and assess its duration. The nystagmus slowly dissipated and
was gone within 15 seconds.

COMMENT This is a classic presentation of benign paroxysmal positional vertigo (BPPV)

of the posterior canal, which is the most common variant. Posterior canal
BPPV can last for weeks or months because the particles are trapped in the
inferior portion of the canal. The Dix-Hallpike test is negative on the
unaffected side because the affected canal is out of the plane of movement
such that the particles do not move during that test. However, when that
affected canal is lined up with the plane in which the head is moving in the
left Dix-Hallpike test, a robust burst of upbeat-torsional nystagmus occurs in
a pattern that is consistent with stimulation of the posterior canal vestibular
afferents. When performing the Dix-Hallpike test, the examiner should be
prepared to open the patient’s eyes if necessary so that nystagmus can be
identified. The provider can then complete the Epley maneuver to guide the
particles around and out of the canal.

356 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 A 65-year-old woman presented to the emergency department with a CASE 3-2
new onset of dizziness symptoms described as a spinning and a
woozy-type sensation in addition to a feeling of imbalance. She reported
feeling fine the night before. While getting out of bed in the morning, she
felt a rapid onset of severe spinning that was soon followed by nausea
and vomiting. She collapsed back into bed and felt better after
approximately 10 minutes. However, with any subsequent attempts to
move and get up, the symptoms substantially worsened. She denied any
other focal symptoms. Her past medical history was unremarkable.
She was examined in the emergency department. The general medical
and neurologic assessments were unremarkable. On the ocular motor
assessment, she was noted to have a spontaneous right-beating
nystagmus. The velocity of nystagmus increased when she looked to the
right and decreased, but did not change direction, when she looked to
the left. On the head impulse test, an abnormal corrective saccade was
present after quick movements to the left side but not to the right side.
No coordination abnormality was noted. The patient had mild
unsteadiness when walking with difficulty taking 10 steps in tandem. On
positional testing, right-beating nystagmus was present on the right
Dix-Hallpike test. On the left Dix-Hallpike test, the right-beating
nystagmus was also present. The same nystagmus was noted on supine
positional testing to the right side and the left sides.

This is a case of an acute unilateral vestibulopathy, which typically arises COMMENT

from vestibular neuritis. The case illustrates just how closely the history of
present illness can be in patients with an acute unilateral vestibulopathy
and patients with benign paroxysmal positional vertigo (BPPV). On the first
day of symptoms, and particularly among patients who present acutely to
the emergency department, the history of present illness in these two
disorders can substantially overlap. In this case and in CASE 3-1, the patients
reported a sudden onset of symptoms after getting out of bed. Patients
often report initial symptoms that last longer than would typically be
expected for BPPV but also describe a substantial improvement from
being still that is more so than expected for acute unilateral vestibulopathy.
What discriminates acute unilateral vestibulopathy from BPPV in this
circumstance is the pattern of nystagmus. Patients with acute unilateral
vestibulopathy have spontaneous unidirectional horizontal nystagmus.
However, typically patients with BPPV have only the typical triggered and
transient nystagmus observed with positional testing. In this current
patient, no indication for attempted particle repositioning was present,
and, in fact, this would just likely exacerbate symptoms. The focus is
instead on symptomatic management and vestibular rehabilitation for
acute unilateral vestibulopathy. A trial of corticosteroids can also be
considered, although meta-analyses of clinical trials concluded that
currently the evidence is insufficient to support the use of corticosteroids
in acute unilateral vestibulopathy.

CONTINUUMJOURNAL.COM 357

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

With the geotropic pattern, the patient has left-beating nystagmus after
turning the head to the left and right-beating nystagmus after turning the head to
the right. The geotropic pattern occurs when the particles are in the posterior
aspect of the horizontal canal; therefore, a head turn to the affected side while
supine results in particle movement toward the cupula, and a head turn away
from the affected side while supine results in particle movement away from the
cupula. The velocity of the nystagmus in the horizontal canal variant can be very
high (greater than 100 degrees per second).14 The duration of the nystagmus is
also usually longer than that of the posterior canal, typically approximately from
30 to 60 seconds.14 In the apogeotropic form, the particles are in the anterior
segment of the horizontal canal on or near the cupula. With the apogeotropic
variant, the patient has left-beating nystagmus after turning the head to the right
while supine and right-beating nystagmus after turning the head to the left while
supine. The velocity of the nystagmus in the apogeotropic variant is usually in the
moderate range of 10 to 40 degrees per second, whereas the duration may last
longer than 1 minute.14 In the horizontal canal variant, the velocity of the
nystagmus is often greater with movement to one side compared with the other
and corresponds with the particles moving toward the cupula resulting in an
excitation deflection. In the geotropic variant, the affected side is typically the
one that generates the higher-velocity nystagmus. However, the affected side in
the apogeotropic variant is typically the side that generates the lower-velocity
nystagmus (CASE 3-3).
Anterior canal BPPV is the least common variant, likely because the
particles should easily fall out of the canal with natural head positions. The
nystagmus pattern with anterior canal BPPV is a burst and taper of
downbeat-torsional nystagmus. The Dix-Hallpike test also evaluates for the
anterior canal variant.

TABLE 3-1 Patterns of Positional Nystagmus

Type of episodic positional dizziness Characteristic nystagmus patterns

Posterior canal benign paroxysmal Burst and taper of upbeat-torsional nystagmus on the Dix-Hallpike test
positional vertigo (BPPV)

Horizontal canal BPPV, geotropic Positionally triggered direction-changing horizontal nystagmus beating toward the
ground triggered by the Dix-Hallpike test or supine positional testing; the
nystagmus is right beating on head turns to the right and then left beating on head
turns to the left

Horizontal canal BPPV, apogeotropic Positionally triggered direction-changing horizontal nystagmus beating away from
the ground triggered by the Dix-Hallpike test or supine positional testing; the
nystagmus is left beating on head turns to the right and then right beating on head
turns to the left

Anterior canal BPPV Burst and taper of downbeat-torsional nystagmus on the Dix-Hallpike test

Central Persistent positional downbeating nystagmus, apogeotropic unidirectional

positional nystagmus, typically associated with other ocular motor abnormalities,
speech, or other motor problems

Migraine A variety of patterns of nystagmus usually less in velocity than BPPV and seen
primarily with positional testing in the dark

358 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Central Positional Dizziness KEY POINTS
Central nervous system disorders
● Direction-changing
can also cause positional positional nystagmus refers
nystagmus.16 Therefore, to nystagmus that changes
clinicians should not think of the direction with changes in
Dix-Hallpike test as a test only head position and should
not be confused with
for peripheral disorders. No
gaze-evoked nystagmus in
studies have assessed the which nystagmus changes
prevalence of central positional direction with changes in the
nystagmus; however, case series direction of gaze.
from specialty clinics have
● Clinicians should not think
reported that approximately 10% of the Dix-Hallpike test as a
of cases with positional test only for peripheral
nystagmus are central in origin.16 disorders; it is also a test for
A 2017 systematic review central positional dizziness.
identified 28 studies that focused ● The most common
on central positional patterns of central
nystagmus.16 The studies were positional nystagmus are
FIGURE 3-4 downbeat, apogeotropic
generally small, ranging
Head and horizontal canal position in the horizontal, geotropic
anywhere from single cases to a geotropic and apogeotropic variants of horizontal horizontal, and multiplanar.
series of 14 patients. This review canal benign paroxysmal positional vertigo
in total identified only 82 (HC-BPPV) affecting the right side. The curved
participants. In most cases, the arrows along the canal show the direction of
otolithic debris movement after a head turn. A and
nystagmus was purely vertical B demonstrate debris position within the canal
(nearly all downbeating), purely when a patient with right HC-BPPV is lying supine.
horizontal, purely torsional, or a C and D demonstrate debris movement, the
combination involving a different effects on the cupula, and the direction
of the fast phase of horizontal nystagmus when
downbeating component. The the patient turns to the right side. The size of the
patients who had central arrow in front of the subject’s nose also indicates
horizontal positional nystagmus nystagmus intensity (amplitude). The density of
with supine positional testing lines in the afferent vestibular nerve indicates the
firing rate, which accounts for the stronger
differed from the typical pattern nystagmus when debris moves toward the cupula
of horizontal canal BBPV (ampullopetal) as opposed to away from it
because the nystagmus in the (ampullofugal). E and F demonstrate debris
central cases was usually only movement, the effect on the cupula, and the
direction of the fast phase of horizontal
present on one side, which was
nystagmus when the patient turns the head to the
typically apogeotropic. The left side.
duration of the positional Reprinted with permission from Nuti D, et al, Ann NY
nystagmus could be brief or Acad Sci.15 © 2009 New York Academy of Sciences.

persistent in cases with central

causes. About half of the patients
with central positional nystagmus had at least one other central symptom or
abnormality such as weakness, ataxia, or speech change. A large majority (85%)
of those with reported ocular motor assessments had gaze-evoked nystagmus,
abnormal saccades, or impaired smooth pursuit. In 75% of the central positional
nystagmus cases, the lesion was in the cerebellum, 9% had isolated brainstem
lesions and 15% had lesions involving the fourth ventricle.
In a 2019 case series from a single center of 27 patients with central positional
nystagmus, the most common pattern of positional nystagmus was downbeat
nystagmus in 69%, followed by apogeotropic horizontal in 42%, geotropic

CONTINUUMJOURNAL.COM 359

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

horizontal in 8%, and multiplanar in 23%.17 In this series, 85% of the patients had
additional neurologic symptoms, and 70% had other ocular motor abnormalities.
The central lesions were most frequently identified in the cerebellar tonsil,
nodulus, uvula, or other midline vermis regions.
Degenerative cerebellar ataxia (eg, spinocerebellar type 6) can also have
prominent episodic positional dizziness that requires patients to avoid provocative
positions by sleeping propped up and avoiding lying flat.18 The nystagmus pattern
can comprise a persistent positional downbeat. These patients inevitably have
other central ocular motor findings and ataxia (CASE 3-4).

CASE 3-3 A 65-year-old man presented to the emergency department with

new-onset dizziness. The dizziness was described as a spinning-type
sensation in addition to lightheadedness, wooziness, and mild imbalance.
The patient stated that, when he woke up in the morning of presentation
and went to get out of bed, he experienced a substantial spinning
sensation that seemed to last 5 to 10 minutes. He felt much better when
being still, but with any subsequent movement the symptoms would
recur. His past medical history was unremarkable.
On examination, no general medical or neurologic abnormalities were
present. On the ocular motor assessment, no spontaneous or gaze-
evoked nystagmus was observed. On the Dix-Hallpike test to the right,
a burst of high-velocity right-beating nystagmus and associated
symptoms were present. The nystagmus slowly decreased in velocity but
persisted for longer than 30 seconds. The patient’s head was returned to
a flat supine position and then turned to the left side, which triggered a
significant but lower-velocity left-beating nystagmus lasting
approximately 30 seconds.

COMMENT This case is consistent with horizontal canal benign paroxysmal positional
vertigo (BPPV) of the geotropic type. The affected side is likely the right
side because having the patient turn to the right elicited the highest-
velocity nystagmus. The nystagmus of horizontal canal BPPV can be
triggered by the Dix-Hallpike test, although supine positional testing is
more specifically designed to assess for horizontal canal BPPV. In this case,
it was clearly seen as a horizontal pattern in the Dix-Hallpike test on the
right side. Because only an observed prolonged right-beating nystagmus
on the Dix-Hallpike test to the right was present, it would be conceivable
that the patient could have a mild or recovering left-side acute unilateral
vestibulopathy with the left-beating nystagmus best seen on the positional
test. The fact that the nystagmus changed to left beating on supine left
positional testing, however, is incompatible with an acute unilateral
vestibulopathy. The affected side was likely the right side because the
highest-velocity nystagmus of this geotropic pattern was triggered by
turning the head to the right side. The Gufoni maneuver was used to treat
this patient’s right geotropic horizontal canal BPPV and resolved the
symptoms and positional nystagmus. No further testing was indicated.

360 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Migraine Dizziness
A presumed migrainous etiology is a common cause of many types of dizziness
presentations. Although more commonly characterized by recurrent
spontaneous dizziness, migraine dizziness can also present as episodic positional
dizziness.19,20 Vestibular migraine can closely mimic BPPV by presenting with
purely positional dizziness.19-22 When assessed in the acute setting, a study of 26
patients with migraine dizziness found that 19% had spontaneous nystagmus
whereas 100% had nystagmus provoked with positional testing with fixation
removed.21 The direction of the positional nystagmus varied among the patients.
The most common direction of the nystagmus was horizontal in 69% of the
patients. This horizontal positional nystagmus remained constant in direction in
72% of the patients when positional testing was performed on both sides. Vertical
nystagmus was seen in 19% of the patients either as upbeat nystagmus or
downbeat nystagmus.
In a study of 20 patients with migrainous vertigo, positional nystagmus
greater than 3 degrees per second was present in 40%.23 In five of the patients,
the nystagmus was present only on positional testing. The positional nystagmus
evolved over minutes in the provocative position and persisted in all the patients
as long as that head position was maintained. In three patients, positional
nystagmus changed direction with different supine positions. Another study of
13 patients found that 8 had a positional upbeat nystagmus and 5 had positional

A 75-year-old man presented with approximately 3 years of mild CASE 3-4

progressive imbalance symptoms and episodes of dizziness after lying
down. The symptoms were gradual in onset, and he had recently required
the use of a cane for walking stability. His older brother also developed
similar symptoms late in life. He had some possible slurring of speech,
but he denied frank coordination problems.
On the general medical and neurologic assessment, mild dysarthria
was detected. Mild dysmetria on the finger-nose-finger test was also
present. On the ocular motor assessment, square-wave jerks were noted
in addition to gaze-evoked nystagmus with right-beating nystagmus on
right gaze and left-beating nystagmus on left gaze. He also had
pathologically impaired smooth pursuit. On the Dix-Hallpike test,
downbeating nystagmus was seen, which was persistent for as long as the
position was held, with mild associated dizziness symptoms.

This patient’s presentation is consistent with a degenerative neurologic COMMENT

disorder such as spinocerebellar ataxia type 6 or multiple system atrophy
cerebellar type. The patient had prominent episodic positional dizziness
but also a constant sense of imbalance and other symptoms of cerebellar
dysfunction. The ocular motor examination is clearly consistent with
cerebellar dysfunction. Given the reports of episodic positional dizziness,
positional testing was performed, but the pattern of nystagmus was typical
of cerebellar dysfunction as opposed to benign paroxysmal positional
vertigo. MRI revealed mild cerebellar atrophy.

CONTINUUMJOURNAL.COM 361

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

downbeat nystagmus.22 The nystagmus was persistent and nonfatigable, and the
mean velocity was 17 degrees per second (standard deviation [SD], 9.5 degrees
per second; range, 7–40 degrees per second).

Light Cupula Syndrome

Light cupula syndrome is uncommon but important to be aware of because it can
closely mimic horizontal canal BPPV.24,25 Patients with light cupula syndrome
typically have profound positional vertigo and a constant sense of imbalance. The
nystagmus is persistent geotropic direction-changing positional nystagmus
without latency or fatigability. Typically, a null point to the nystagmus exists
while the patient is in a supine position with the head rotated about 20 to
30 degrees toward the affected side. The precise cause of light cupula syndrome is
unclear, but the mechanisms appear similar to that of positional alcohol
nystagmus. If the cupula becomes light, it becomes responsive to gravity such
that the head turned to the side will persistently deflect the cupula. A mild
spontaneous nystagmus while sitting can be seen in patients with light cupula
syndrome, but the nystagmus stops when the head has been tilted slightly
forward, about 30 degrees, placing the lateral canal parallel to the horizontal
plane. In some patients, accompanying unilateral hearing loss is present,
suggesting that they have a full labyrinthitis disorder. When apogeotropic
nystagmus is present instead, it could be caused by heavy endolymph rather than
a light cupula. Patients with light cupula syndrome are refractory to attempts at
particle repositioning, and the recovery course is slow, occurring over days or
weeks, similar to an acute unilateral vestibulopathy.

EVALUATION AND MANAGEMENT OF THE PATIENT WITH EPISODIC

POSITIONAL DIZZINESS
The most important parts of the assessment in patients presenting with episodic
positional dizziness are the details of the history and particularly the examination
when in the office setting. Once the history is obtained, the clinician should
perform the general neurologic examination including the ocular motor
examination. It is probably a mistake to jump straight to positional testing
without first inspecting the ocular motor system at rest, because in acute
presentations, patients can actually have a fixed lesion (eg, acute unilateral
vestibulopathy or stroke) even when the history might suggest episodic
positional dizziness.
Positional testing usually begins with the Dix-Hallpike test. Some patients can
identify one side that is the most likely affected. A strategy in that circumstance is
to start by testing the presumed unaffected side, which serves as the internal
control. But ultimately, the order should not matter. If the test starts with the right
side, the clinician should turn the patient’s head to the right side approximately
45 degrees and then lay the patient back. The key parts for eliciting the nystagmus
are to move patients a little faster than they would normally lie down and
to make sure that the head is tilted back approximately 20 degrees.
Either moving too slowly or not tilting the head back far enough may not
result in sufficient particle movement to initiate the drag force required to
deviate the cupula and elicit nystagmus. The patient should stay in the
head-hanging position for approximately 10 to 20 seconds. If no nystagmus is
seen, then the clinician should have the patient sit back up, turn the patient’s
head to the other side, and perform the test. If either side is positive for triggered

362 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

and transient nystagmus, the examination can immediately proceed with the KEY POINTS
canalith repositioning maneuver. With the Epley maneuver, the clinician simply
● Vestibular migraine can
turns the patient’s head to the opposite side approximately 90 degrees, waits closely mimic BPPV by
approximately 20 to 30 seconds, and then has the patient roll over until they are presenting with purely
looking down to the ground. It is important to make sure the patient does not sit positional dizziness.
up during this rollover because many patients do try. The patient stays in that
● Patients with light cupula
position for approximately 20 to 30 seconds and then sits up. After treating
syndrome typically have
patients with the Epley maneuver, the clinician can generally spend some time profound positional vertigo
explaining what was being done and showing the maneuver on a diagram. It is and a constant sense of
usually helpful to then repeat the Dix-Hallpike test and Epley maneuver because imbalance. The nystagmus is
it sometimes takes more than one maneuver to get all the particles out and persistent geotropic
direction-changing
because doing so can also clearly demonstrate to the patient that the treatment positional nystagmus
was effective. A Cochrane collaboration meta-analysis of eight randomized without latency or
controlled trials with a total of 507 participants found that conversion from a fatigability.
positive to a negative Dix-Hallpike test was substantially more common in the
● The Dix-Hallpike test
group treated with the Epley maneuver compared with the group treated with a should be performed by
sham maneuver (odds ratio, 9.62; 95% confidence interval, 6.0 to 15.42) moving the patient from the
(TABLE 3-223-35).1 Each study had a statistically significant benefit of the sitting to head-hanging
treatment compared with the control. However, the effect size of the maneuver position at a pace a bit more
quickly than he or she would
varied from study to study with the largest effect sizes occurring when the
ordinarily lie down and
diagnosis and treatment were performed in specialty outpatient clinics, more trying to tilt the head back
than one maneuver was allowed at the initial treatment, and the outcome was approximately 20 degrees.
measured in the first week. In a study that used a 24-hour outcome assessment
and up to three maneuvers performed in a specialty clinic, 80% (28 of 35) of ● A Cochrane collaboration
meta-analysis of eight
patients with BPPV randomly assigned to expert treatment with the canalith randomized controlled trials
repositional maneuver were cured compared with 10% (3 of 31) of the patients found that conversion from
randomly assigned to sham treatment (number needed to treat, 1.4; P < 0.001).26 a positive to a negative
It has also been demonstrated that patients can be taught to self-perform the Dix-Hallpike test
significantly favored the
Epley maneuver or other maneuvers and successfully treat their BPPV.36-39 The Epley treatment group when
self-treatment studies, however, were limited in their ability to generalize compared with a sham
because the patients received in-person instructions and performance feedback maneuver or control.
from expert providers. The Dix-Hallpike test uses angles of the head specifically
oriented to the plane of the posterior semicircular canal, but particles that are
instead in the horizontal canal will also typically move enough to trigger
horizontal nystagmus. However, if the Dix-Hallpike test is negative, the next step
in a patient with a high suspicion of BPPV is to perform supine positional testing,
which is more specifically designed to move particles in the horizontal canal.
When the horizontal direction-changing positional nystagmus of horizontal
canal BPPV is identified, three treatment options are supported by randomized
controlled trials: the barbeque roll maneuver (also known as the Lempert roll
maneuver) (FIGURE 3-5), the Gufoni maneuver, and the head-shaking
maneuver.34,35 The barbecue roll maneuver is similar to the Epley maneuver, but
the head is resting on the bed or examination table rather than extended back. It
can be used to treat both the geotropic and apogeotropic variants. The maneuver
starts with the patient in the supine position, and then head rotations are made in
30- to 60-second intervals toward the healthy side for treating the geotropic
variant and toward the affected side for treating the apogeotropic variant.
To use the Gufoni maneuver in the geotropic variant, the patient starts in the
sitting position and then is quickly moved to a side-lying position onto the
unaffected side, which moves the particles toward the utricle. The patient stays in

CONTINUUMJOURNAL.COM 363

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

this position for approximately 2 minutes and then turns their head to a face-down
direction for 1 minute before sitting back up. To use the Gufoni maneuver in the
apogeotropic variant, the patient starts in the seated position and then is quickly
moved to a side-lying position on the affected side, which moves the particles away
from the cupula and toward the utricle. The patient stays in this position for
approximately 2 minutes and then turns their head to look up and hold the position
for approximately 1 minute before sitting up. The maneuver generally either
resolves the BPPV or converts it to the geotropic variant, which can then be treated
with the Gufoni maneuver for the geotropic variant. Surprisingly, the Gufoni
maneuver did not demonstrate evidence of being significantly statistically better
than a simple head-shaking maneuver in the apogeotropic variant.35 The

TABLE 3-2 Selected Data From Clinical Trials of Canalith Repositioning Maneuver
Treatments for Benign Paroxysmal Positional Vertigo

Control, %
Study Treatment, % (n/N) (n/N) Notes
Posterior canal benign paroxysmal
positional vertigo (BPPV) clinical trialsa

Lynn et al,27 1995 89 (16/18) 27 (4/15) Audiologist vestibular clinic; up to 4

maneuvers; outcome at 1 month.

Froehling et al,28 2000 67 (16/24) 38 (10/26) General medicine clinic; up to 5

maneuvers; outcome at 1–2 weeks.

Yimtae et al,29 2003 88 (22/25) 65 (13/20) Neuro-otology clinic, up to 5

maneuvers, outcome at 1 week

Munoz et al,30 2007 34 (13/38) 15 (6/41) Family practice clinic, single maneuver,
outcome within hours

Von Brevern et al,26 2006 80 (28/35) 10 (3/31) Neuro-otology clinic, up to 3

maneuvers, outcome at 24 hours

Bruintjes et al,31 2014 91 (20/22) 45 (10/22) Dizziness outpatient clinic, up to 2

maneuvers, outcome at 1 month

Liang et al,32 2010 98 (42/43) 77 (34/44) 1 maneuver, outcome at 4 days

Xie et al,33 2012 93 (54/58) 34 (11/45) 1 maneuver, outcome at 4 days

Horizontal canal BPPV clinical trials

Kim et al,34 2012, geotropic Barbeque roll 35 (17/48) 10 outpatient dizziness clinics, up to 2
maneuver: 69 (38/55) maneuvers, outcome at 2 hours
Gufoni maneuver: 61
(39/64)

Kim et al,35 2012, apogeotropic Gufoni maneuver: 73 35 (17/49) 10 outpatient dizziness clinics, up to 2
(38/52) maneuvers, outcome at 2 hours
Head-shake
maneuver: 62 (33/53)

n = number of patients with positive outcome; N = total number of patients in trial group.
a
Selected from the Cochrane collaboration meta-analysis.1

364 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 KEY POINT

● Horizontal canal BPPV

can be treated by using the
barbeque roll maneuver, the
Gufoni maneuver, or the
forced prolonged position.

FIGURE 3-5
Barbeque roll maneuver, also referred to as the Lempert roll maneuver, for right-sided
horizontal canal benign paroxysmal positional vertigo (BPPV). When it is determined to be
horizontal canal BPPV affecting the right side, the patient is taken through a series of
stepwise 90-degree turns away from the affected side in steps 1 through 5, holding each
position for 10 to 30 seconds. From step 5, the patient positions their body to the back (6) in
preparation for the rapid and simultaneous movement from the supine face up to the sitting
position (7).
Reprinted with permission from Fife TD, et al, Neurology.12 © 2008 American Academy of Neurology.

head-shaking maneuver is performed with the patient in the sitting position. The
head is first pitched forward approximately 30 degrees and then moved
sinusoidally at a rate of approximately 3 Hz for 15 seconds.
Another simple option to treat the horizontal canal variant is the forced
prolonged position, which is performed by having patients lie on their healthy
side for the geotropic variant or the affected side for the apogeotropic variant for
approximately 12 hours.40 It is also worth noting that horizontal canal BPPV has a
natural history of spontaneous resolution within days, which is different from
the typical weeks to months for the posterior canal variant.41-43 In addition, more
than one-third of the patients treated with sham maneuvers in clinical trials of
treatments for horizontal canal BPPV had resolution of the BPPV within hours,
and more than 80% were cured within 7 days.34,35
If a patient is suspected of having BPPV but the positional testing does not
trigger nystagmus, several possibilities for this exist. First, a spontaneous
resolution may have occurred, and the particles are already out of the canal.
Second, it is possible that the positional testing was not adequately performed.
This can occur if the patient is moved too slowly into the head-hanging position
or if the head was not tilted back far enough. If the particles do not move fast

CONTINUUMJOURNAL.COM 365

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

KEY POINT enough or far enough, then the drag force of endolymph flow in the canal will not
be sufficient to deviate the cupula. Lastly, if the patient reports symptoms but no
● If a patient is suspected
of having BPPV but the
nystagmus is observed, then it is possible that the particles are out of the canal
positional testing does not but the patient has developed an anxiety response to previously provocative
trigger nystagmus, it is positions. In all these circumstances, it is still recommended to perform the
possible that the patient had repositioning maneuver for patient self-treatment education.
spontaneous resolution, the
If the findings on examination instead suggest a central lesion, then the
positional testing was not
adequately performed, or appropriate evaluation should follow. This typically includes an MRI of the brain
the patient developed an to assess for a structural abnormality such as a mass lesion, stroke, demyelinating
anxiety response to previous lesion, or Chiari malformation.
BPPV.
For patients suspected of having migraine-associated episodic positional
dizziness, the treatment is usually supportive with symptomatic medications,
rest, and hydration. Patients who have many recurrent episodes may benefit
from a migraine prophylactic medication. However, randomized controlled trials
of migraine preventative medications in patients with migraine-related dizziness
have not been conducted.
Patients suspected of having light cupula syndrome require supportive care
and symptomatic medications. After the acute phase, they may also benefit from
vestibular rehabilitation, although clinical trials are lacking in this uncommon
presentation. It is reasonable to attempt a particle repositioning maneuver
because horizontal canal BPPV is typically in the differential diagnosis, but this
would not be expected to treat light cupula syndrome.
Consensus clinical practice guidelines recommend limiting brain imaging
studies to patients with symptoms or signs of concurrent brainstem or cerebellar
dysfunction or when positional vertigo and nystagmus have atypical features or
fail to resolve with repeated therapeutic positional maneuvers.2,4

CONCLUSION
Episodic positional dizziness is a common category of dizziness presentations.
Properly placing patients in this category is a challenge because positional
components are common in nearly all types of dizziness presentations. For this
reason, providers should not overemphasize the presence of positional
components in determining the management plan. BPPV is the prototypical
episodic positional dizziness disorder and can be readily identified and treated at
the bedside. The canalith repositioning maneuvers are supported by numerous
randomized controlled trials, meta-analyses, and clinical guideline statements.
Therefore, identifying and treating BPPV is a priority in routine practice. Central
disorders are much less common causes of episodic positional dizziness but need
to be considered when signs of central dysfunction are present, including central
patterns of nystagmus. Providers should also be aware of migraine and light
cupula syndrome as potential causes of episodic positional dizziness.

REFERENCES

1 Hilton MP, Pinder DK. The Epley (canalith 2 Bhattacharyya N, Gubbels SP, Schwartz SR, et al.
repositioning) manoeuvre for benign paroxysmal Clinical practice guideline: benign paroxysmal
positional vertigo. Cochrane Database Syst Rev positional vertigo (update). Otolaryngol Head
2014;12:CD003162. doi:10.1002/14651858. Neck Surg 2017;156(3 suppl):S1-S47.
CD003162.pub3 doi:10.1177/0194599816689667

366 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

3 Brown MD. Evidence-based emergency 15 Nuti D, Mandala M, Salerni L. Lateral canal
medicine. Is the canalith repositioning maneuver paroxysmal positional vertigo revisited. Ann NY
effective in the acute management of benign Acad Sci 2009;1164:316-323. doi:10.1111/j.1749-
positional vertigo? Ann Emerg Med 2011;58(3): 6632.2008.03720.x
286-287. doi:10.1016/j.annemergmed.2011.02.024
16 Macdonald NK, Kaski D, Saman Y, et al. Central
4 von Brevern M, Bertholon P, Brandt T, et al. positional nystagmus: a systematic literature
Benign paroxysmal positional vertigo: diagnostic review. Front Neurol 2017;8:141. doi:10.3389/
criteria. J Vestib Res 2015;25(3–4):105-117. fneur.2017.00141
doi:10.3233/VES-150553
17 De Schutter E, Adham ZO, Kattah JC. Central
5 Kerber KA, Burke JF, Skolarus LE, et al. Use of positional vertigo: a clinical-imaging study. Prog
BPPV processes in emergency department Brain Res 2019;249:345-360. doi:10.1016/bs.
dizziness presentations: a population-based pbr.2019.04.022
study. Otolaryngol Head Neck Surg 2013;148(3):
18 Jen JC, Yue Q, Karrim J, et al. Spinocerebellar
425-430. doi:10.1177/0194599812471633
ataxia type 6 with positional vertigo and
6 Kerber KA, Forman J, Damschroder L, et al. acetazolamide responsive episodic ataxia.
Barriers and facilitators to ED physician use of J Neurol Neurosurg Psychiatry 1998;65(4):
the test and treatment for BPPV. Neurol Clin 565-568. doi:10.1136/jnnp.65.4.565
Pract 2017;7(3):214-224. doi:10.1212/CPJ.
19 von Brevern M, Radtke A, Clarke AH, Lempert T.
0000000000000366
Migrainous vertigo presenting as episodic
7 Meurer WJ, Johnson P, Brown D, et al. An positional vertigo. Neurology 2004;62(3):469-472.
educational intervention for acute dizziness doi:10.1212/01.wnl.0000106949.55346.cd
care: a randomized, vignette-based study. Otol
20 Lempert T, Olesen J, Furman J, et al. Vestibular
Neurotol 2019;40(8):e830-e838. doi:10.1097/
migraine: diagnostic criteria. J Vestib Res 2012;
MAO.0000000000002338
22(4):167-172. doi:10.3233/VES-2012-0453
8 Kerber KA, Callaghan BC, Telian SA, et al.
21 Polensek SH, Tusa RJ. Nystagmus during attacks
Dizziness symptom type prevalence and overlap:
of vestibular migraine: an aid in diagnosis. Audiol
a US nationally representative survey. Am J Med
Neurootol 2010;15(4):241-246. doi:10.1159/
2017;130(12):1465.e1-1465.e9. doi:10.1016/j.
000255440
amjmed.2017.05.048
22 El-Badry MM, Samy H, Kabel AM, et al. Clinical
9 von Brevern M, Radtke A, Lezius F, et al.
criteria of positional vertical nystagmus in
Epidemiology of benign paroxysmal positional
vestibular migraine. Acta Otolaryngol 2017;137(7):
vertigo: a population based study. J Neurol
720-722. doi:10.1080/00016489.2017.1318220
Neurosurg Psychiatry 2007;78(7):710-715.
doi:10.1136/jnnp.2006.100420 23 von Brevern M, Zeise D, Neuhauser H, et al. Acute
migrainous vertigo: clinical and oculographic
10 Centers for Disease Control and Prevention.
findings. Brain : a journal of neurology. 2005;
National Health Interview Survey: 2008 data
128(pt 2):365-374. doi:10.1093/brain/awh351
release. Accessed February 18, 2021. cdc.gov/
NCHS/nhis/nhis_2008_data_release.htm 24 Kim CH, Choi JM, Jung HV, et al. Sudden
sensorineural hearing loss with simultaneous
11 Newman-Toker DE, Cannon LM, Stofferahn ME,
positional vertigo showing persistent geotropic
et al. Imprecision in patient reports of dizziness
direction-changing positional nystagmus. Otol
symptom quality: a cross-sectional study
Neurotol 2014;35(9):1626-1632. doi:10.1097/
conducted in an acute care setting. Mayo Clin
MAO.0000000000000457
Proc 2007;82(11):1329-1340. doi:10.4065/82.11.1329
25 Kim CH, Kim MB, Ban JH. Persistent geotropic
12 Fife TD, Iverson DJ, Lempert T, et al. Practice
direction-changing positional nystagmus with a
parameter: therapies for benign paroxysmal
null plane: the light cupula. Laryngoscope 2014;
positional vertigo (an evidence-based review):
124(1):E15-19. doi:10.1002/lary.24048
report of the Quality Standards Subcommittee of
the American Academy of Neurology. Neurology 26 von Brevern M, Seelig T, Radtke A, et al. Short-
2008;70(22):2067-2074. doi:10.1212/01.wnl. term efficacy of Epley's manoeuvre: a
0000313378.77444.ac double-blind randomised trial. J Neurol
Neurosurg Psychiatry 2006;77(8):980-982.
13 Jang YS, Hwang CH, Shin JY, et al. Age-related
doi:10.1136/jnnp.2005.085894
changes on the morphology of the otoconia.
Laryngoscope 2006;116(6):996-1001. 27 Lynn S, Pool A, Rose D, et al. Randomized trial of
doi:10.1097/01.mlg.0000217238.84401.03 the canalith repositioning procedure.
Otolaryngol Head Neck Surg 1995;113(6):712-720.
14 Aw ST, Todd MJ, Aw GE, et al. Benign positional
doi:10.1016/s0194-5998(95)70010-2
nystagmus: a study of its three-dimensional
spatio-temporal characteristics. Neurology 28 Froehling DA, Bowen JM, Mohr DN, et al. The
2005;64(11):1897-1905. doi:10.1212/01. canalith repositioning procedure for the
WNL.0000163545.57134.3D treatment of benign paroxysmal positional
vertigo: a randomized controlled trial. Mayo Clin
Proc 2000;75(7):695-700. doi:10.4065/75.7.695

CONTINUUMJOURNAL.COM 367

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC POSITIONAL DIZZINESS

29 Yimtae K, Srirompotong S, Srirompotong S, 37 Radtke A, von Brevern M, Tiel-Wilck K, et al.

Sae-Seaw P. A randomized trial of the canalith Self-treatment of benign paroxysmal positional
repositioning procedure. Laryngoscope 2003; vertigo: Semont maneuver vs Epley procedure.
113(5):828-832. doi:10.1097/00005537- Neurology 2004;63(1):150-152. doi:10.1212/01.
200305000-00011 wnl.0000130250.62842.c9
30 Munoz JE, Miklea JT, Howard M, et al. Canalith 38 Tanimoto H, Doi K, Katata K, Nibu KI.
repositioning maneuver for benign paroxysmal Self-treatment for benign paroxysmal positional
positional vertigo: randomized controlled trial in vertigo of the posterior semicircular canal.
family practice. Can Fam Physician 2007;53(6): Neurology 2005;65(8):1299-1300. doi:10.1212/01.
1048-1053. wnl.0000180518.34672.3d
31 Bruintjes TD, Companjen J, van der Zaag-Loonen 39 Foster CA, Ponnapan A, Zaccaro K, Strong D. A
HJ, van Benthem PP. A randomised comparison of two home exercises for benign
sham-controlled trial to assess the long-term positional vertigo: half somersault versus Epley
effect of the Epley manoeuvre for treatment of maneuver. Audiol Neurotol Extra 2012;2:16-23.
posterior canal benign paroxysmal positional doi:10.1159/000337947 doi:10.1212/WNL.
vertigo. Clin Otolaryngol 2014;39(1):39-44. 0b013e31823fcd26
doi:10.1111/coa.12217
40 Chiou WY, Lee HL, Tsai SC, et al. A single therapy
32 Liang SB, Li L, He HY. The efficacy of Epley for all subtypes of horizontal canal positional
procedure for treatment of benign paroxysmal vertigo. Laryngoscope 2005;115(8):1432-1435.
positional vertigo of the posterior semicircular doi:10.1097/01.mlg.0000168092.91251.d3
canal. J Youjiang Med Univ Nationalities 2010;2:7.
41 Shim DB, Ko KM, Lee JH, et al. Natural history of
33 Xie K, Du S, Gao JJ, et al. Clinical efficacy of Epley horizontal canal benign paroxysmal positional
procedure for treatment of benign paroxysmal vertigo is truly short. J Neurol 2015;262(1):74-80.
positional vertigo of posterior semicircular canal. doi:10.1007/s00415-014-7519-0
Chin J Gen Pract 2012;2:20.
42 Imai T, Ito M, Takeda N, et al. Natural course of
34 Kim JS, Oh SY, Lee SH, et al. Randomized clinical the remission of vertigo in patients with benign
trial for geotropic horizontal canal benign paroxysmal positional vertigo. Neurology 2005;
paroxysmal positional vertigo. Neurology 2012; 64(5):920-921. doi:10.1212/01.WNL.0000152890.
79(7):700-707. doi:10.1212/WNL. 00170.DA
0b013e3182648b8b
43 Imai T, Takeda N, Ito M, Inohara H. Natural course
35 Kim JS, Oh SY, Lee SH, et al. Randomized clinical of positional vertigo in patients with
trial for apogeotropic horizontal canal benign apogeotropic variant of horizontal canal benign
paroxysmal positional vertigo. Neurology 2012; paroxysmal positional vertigo. Auris Nasus Larynx
78(3):159-166. 2011;38(1):2-5. doi:10.1016/j.anl.2010.05.011
36 Radtke A, Neuhauser H, von Brevern M, Lempert
T. A modified Epley's procedure for self-
treatment of benign paroxysmal positional
vertigo. Neurology 1999;53(6):1358-1360.
doi:10.1212/wnl.53.6.1358

368 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 Episodic Spontaneous REVIEW ARTICLE


Dizziness CONTINUUM AUDIO
INTERVIEW AVAILABLE
ONLINE
By Scott D. Z. Eggers, MD
Downloaded from http://journals.lww.com/continuum by wqLe/H3/oM2vx1Qs5A5qMemKKHLPqE4qkmGNpdheG3ikf1o85ttJSkFL2oTkgOkhMEmawHvsuTTE5xJG2ZjqccytIAhVn2qegy060KLcKJuPZsx5htaYiopRjp2zor2egUzWJAEWYNrnNdb1tgu/p0M6EMGzm2Aj on 04/28/2022

ABSTRACT CITE AS:

CONTINUUM (MINNEAP MINN)
PURPOSE OF REVIEW: Conditions causing recurrent spontaneous episodes
2021;27(2, NEURO-OTOLOGY):
of dizziness or vertigo span several medical specialties, making it 369–401.
challenging for clinicians to gain confidence in evaluating and managing
the spectrum of episodic vestibular disorders. Patients are often Address correspondence to
Dr Scott Eggers, Department of
asymptomatic and have normal examinations at the time of evaluation. Neurology, Mayo Clinic,
Thus, diagnosis depends heavily on eliciting key features from the history. 200 First St SW, Rochester,
MN 55905, eggers.scott@
Overreliance on symptom quality descriptions commonly leads to mayo.edu.
misdiagnosis. The goal of this article is to provide the reader with a
straightforward approach to the diagnosis and management of conditions RELATIONSHIP DISCLOSURE:
Dr Eggers has received
that cause episodic spontaneous dizziness. research/grant support from
the National Institutes of
RECENT FINDINGS:Consensus diagnostic criteria have been established for Health/National Institute on
Deafness and Other
vestibular migraine, Ménière disease, vestibular paroxysmia, and Communication Disorders
hemodynamic orthostatic dizziness/vertigo. Vertigo has been recognized (U01 DC13256), publishing
royalties from UpToDate, Inc,
as a common symptom in vertebrobasilar ischemia, cardiogenic
and technology intellectual
dizziness, and orthostatic hypotension. Treatment recommendations for property royalty payments from
vestibular migraine still lack high-quality evidence, but controlled trials the Mayo Clinic.
are occurring. UNLABELED USE OF
PRODUCTS/INVESTIGATIONAL
SUMMARY: The evaluation should start with a detailed description of the USE DISCLOSURE:
Dr Eggers discusses the
episodes from the patient and any observers. Rather than focusing first on unlabeled/investigational use
whether the symptom quality is most consistent with vertigo, dizziness, of amitriptyline, atenolol,
lightheadedness, or unsteadiness, the clinician should clarify the timing betahistine, cinnarizine,
cyproheptadine, diazepam,
(episode frequency and duration), possible triggers or circumstances diltiazem, dimenhydrinate,
(eg, position changes, upright posture), and accompanying symptoms. flunarizine, gabapentin,
lamotrigine, lomerizine,
History should identify any auditory symptoms, migraine features, lorazepam, metoclopramide,
posterior circulation ischemic symptoms, vascular risk factors, clues for metoprolol, nortriptyline,
anxiety, and potentially relevant medications. Carefully selected testing pizotifen, venlafaxine, and
verapamil, none of which are
can help secure the diagnosis, but excessive and indiscriminate testing can approved by the US Food and
lead to more confusion. Treatments for these conditions are vastly Drug Administration (FDA) for
different, so an accurate diagnosis is critical. the treatment of vestibular
migraine; carbamazepine,
lacosamide, and oxcarbazepine,
none of which are FDA
approved for the treatment of
INTRODUCTION vestibular paroxysmia; and

A
rizatriptan, which is not FDA
s with any type of spell, recurrent episodes of dizziness or vertigo approved for the treatment of
can be challenging to diagnose for several reasons. Because patients motion sickness.
are often asymptomatic at the time of evaluation, diagnosis
depends primarily on eliciting a detailed description of the episodes © 2021 American Academy
from the patient and observer. Commonly, the physical of Neurology.

CONTINUUMJOURNAL.COM 369

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

examination is normal between events. Tests must be carefully chosen because

overreliance on indiscriminate and costly investigations risks uncovering
irrelevant or diagnostically misleading abnormalities. Conditions that cause
episodic dizziness span several areas of practice, including neurology,
otolaryngology, cardiology, physical therapy, psychiatry, and general medicine.
This creates a challenge for clinicians to gain expertise in evaluating and
managing the spectrum of vestibular disorders.
Ambiguous symptom terminology and excessive focus on symptom quality
both contribute to diagnostic errors. Patients and clinicians use the terms
dizziness, vertigo, lightheadedness, faintness, presyncope, unsteadiness, and
disequilibrium in different and arbitrary ways. Many providers have been taught
to first classify the character of the “dizziness” symptom into a rotational
sensation, a sensation of impending faint, disequilibrium, or ill-defined
“lightheadedness” with the rationale that the symptom quality will appropriately
dictate the diagnostic categories and evaluation.1 This approach is no longer
advocated and results in premature disregard for many diagnostic possibilities
because spinning vertigo does not always imply a vestibular disorder just as a
sensation of impending faint does not rule one out.2 Furthermore, patient reports
of symptom quality have been shown to be both inconsistent (mismatched
across different question formats) and unreliable (mismatched on test-retest of
the same question).3 Instead, the history should focus on the timing, triggers, and
accompanying symptoms before placing too much significance in the symptom
quality description.
Despite the risks of overreliance on symptom quality, establishing uniform
symptom definitions is important to promote clear communication among
clinicians. The International Classification of Vestibular Disorders4 defines
vertigo, dizziness, and unsteadiness as separate vestibular symptoms that do not
necessarily predict the underlying cause or localization (TABLE 4-1). Often the
term dizziness is used more broadly (as in this article’s title) for any vestibular
symptom, with vestibular also being used here in the broadest sense and not
merely for the inner ear.

TABLE 4-1 Definition of Vestibular Symptoms According to the International

Classification of Vestibular Disordersa

Term Definition

Vertigo (Internal) vertigo is the sensation of self-motion when no self-motion is occurring or the sensation of distorted
self-motion during an otherwise normal head movement. This “internal” vestibular sensation is distinguished
from the “external” visual sense of motion referred to as either external vertigo or oscillopsia. The term
encompasses false spinning sensations (spinning vertigo) and also other false sensations such as swaying,
tilting, bobbing, bouncing, or sliding (nonspinning vertigo).

Dizziness (Nonvertiginous) dizziness is the sensation of disturbed or impaired spatial orientation without a false or
distorted sense of motion.

Unsteadiness Unsteadiness is the feeling of being unstable while seated, standing, or walking without a particular directional
preference.

a
Data from Bisdorff A, et al, J Vestib Res.4

370 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 This article focuses on conditions that cause a spontaneous “episodic KEY POINTS
vestibular syndrome,” which the World Health Organization in the forthcoming
● Patients with episodic
International Classification of Diseases, Eleventh Edition5 defines as the following: spontaneous dizziness are
often asymptomatic at the
…a clinical syndrome of transient vertigo, dizziness, or unsteadiness time of evaluation and most
lasting seconds to hours, occasionally days, and generally including often have normal
features suggestive of temporary, short-lived vestibular system examinations. The
dysfunction (e.g., nausea, nystagmus, sudden falls). There may also be diagnostic history should
symptoms or signs suggesting cochlear or central nervous system focus on the timing, triggers,
circumstances, and
dysfunction. Episodic vestibular syndrome usually connotes multiple, accompanying symptoms
recurrent events caused by an episodic disorder with repeated spells rather than placing too much
(triggered or spontaneous), but may initially present after the first event. emphasis on the patient’s
description of the quality of
An episodic vestibular syndrome should always be specified as either dizziness.
spontaneous or triggered (eg, by position change or Valsalva maneuver)
● Dizziness is a common
because the differential diagnosis and diagnostic evaluations are quite distinct. accompaniment of migraine
Triggers should be distinguished from occasional precipitants (eg, red wine) that is not associated with
or exacerbating factors (eg, head movement worsening already-present other headache types.
dizziness). Many of the disorders herein now have formal consensus
definition papers by the Bárány Society that are freely available at jvr-web.
org/ICVD.html.
The majority of patients with episodic spontaneous (nontriggered) dizziness
can be diagnosed with one of a handful of disorders (TABLE 4-2). In addition to
the circumstances and accompanying symptoms, the duration of the episodes
helps narrow the differential diagnosis:

u Seconds: vestibular paroxysmia, cardiogenic dizziness

u Minutes: vertebrobasilar transient ischemic attack (TIA), vestibular migraine, panic
disorder, delayed orthostatic hypotension, hypoglycemia
u Hours: vestibular migraine, Ménière disease, toxic/metabolic
u Days: vestibular migraine

It is important to distinguish the duration of the core vestibular symptoms

from any residual symptoms just as it is important to distinguish the overall
duration of the condition from the duration of individual episodes.

VESTIBULAR MIGRAINE
Dizziness is a common symptom in patients with migraine. Vestibular migraine
is a term used to specifically describe episodic vestibular symptoms attributed to
migraine. It is the most common but underdiagnosed cause of recurrent
spontaneous episodes of vestibular symptoms. Several factors have hindered the
study of vestibular migraine, including the lack of standardized nomenclature,
incomplete understanding of the pathophysiology, clinical manifestations that
vary and overlap with other conditions, absence of specific tests or biologic
markers, and only recently developed consensus diagnostic criteria. Prior terms
include migraine-associated vertigo, migraine-associated dizziness, migraine-related
vestibulopathy, and migrainous vertigo. Benign paroxysmal vertigo is considered a
childhood precursor to migraine,6 and benign recurrent vertigo is an entity with
strong links to migraine.7,8 Only a tiny fraction of patients with migraine and
vertigo meet the criteria for migraine with brainstem aura.

CONTINUUMJOURNAL.COM 371

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

Epidemiology and Associations

A large German study that screened a representative sample of the general
population with a validated questionnaire found that vertigo and migraine
co-occurred 3 times more often than would be expected by chance and estimated
a lifetime vestibular migraine prevalence of 1%, making it the most common
cause of spontaneous episodes of vertigo.9 A subsequent nationwide US
population-based survey found a 1-year vestibular migraine prevalence of
2.7%.10 As with migraine headache, women are diagnosed with vestibular
migraine more often than men, making up 60% to 83% of patients in case
series.10–12 The mean age of vertigo onset is around 40 years but ranges from the
first to seventh decades. Migraine headache typically precedes vertigo onset by
several years; although they may begin concurrently, headaches may have
resolved decades before vertigo begins.9,11,13,14
Many clinical series report a high prevalence of vestibular symptoms and signs
in patients with migraine but not in patients with other headache types.
Similarly, the prevalence of migraine among patients being evaluated or treated
for episodic vertigo has been estimated between 38% and 87%, compared with
10% to 24% in control groups.7,14,15 Half to two-thirds of patients with migraine
have motion sickness (2 to 5 times greater than the general population) and
appear more susceptible to visually induced motion sickness.13,16

TABLE 4-2 Disorders and Key Features of Episodic Spontaneous Dizziness

Disorder Key features

Vestibular migraine Episodes of vestibular symptoms lasting 5 min to 72 hours (may be spontaneous,
positional, visually induced, or head motion–induced), history of migraine, migraine
features during episodes

Ménière disease Episodes of spontaneous vertigo lasting 20 min to 12 hours accompanied by fluctuating
sensorineural hearing loss, tinnitus, and aural fullness

Vertebrobasilar transient Episodes of spontaneous vertigo lasting minutes to 1 to 2 hours, either isolated or
ischemic attack accompanied by diplopia, dysarthria, dysphagia, limb dysmetria, or visual field defects;
affects mainly older adults with vascular risk factors

Vestibular paroxysmia Brief attacks of vertigo (seconds) multiple times per day with or without tinnitus; typically
responsive to carbamazepine

Benign recurrent vertigo Episodes of spontaneous vertigo without migrainous, neurologic, or otologic features that
does not go on to cause any persistent vestibular or hearing loss

Panic disorder Recurrent panic attacks lasting minutes that may have prominent dizziness,
lightheadedness, or unsteadiness along with other symptoms

Delayed orthostatic Dizziness, lightheadedness, or vertigo developing more than 3 minutes after assuming
hypotension upright posture, associated with significant blood pressure drop, relieved by sitting or
lying down

Cardiogenic dizziness Dizziness or vertigo lasting seconds to minutes due to cerebral hypoperfusion from
low cardiac output, most often from a paroxysmal arrhythmia (bradycardia less than
40 beats/min or tachycardia greater than 170 beats/min)

Hypoglycemia Dizziness or vertigo due to transient drop in serum glucose; affects mainly patients with
diabetes who are on insulin

372 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Pathophysiology KEY POINTS
The pathophysiology of vestibular migraine is incompletely understood. The
● Vestibular migraine is the
wide spectrum of clinical manifestations and vestibular laboratory findings most common cause of
suggests heterogeneous mechanisms that are not mutually exclusive. episodic spontaneous
Vertigo in vestibular migraine could sometimes represent a migraine vertigo, affecting between
aura, either as a brainstem aura or as cortical spreading depression affecting 1% and 2.7% of the
population.
the posterior parietal cortex and, via direct connections, influencing the
brainstem vestibular nuclei.17 However, only 2% to 30% of patients have ● Migraine headache
vertigo meeting the aura criteria of 5 to 60 minutes preceding a migraine typically precedes vertigo
headache.11,14,18 onset by several years;
Altered neural activity in the trigeminovascular system is implicated as a although they may begin
concurrently, headaches
primary mechanism for migraine headache. Some of the same neurotransmitters may have resolved decades
that cause vasodilation and neurogenic inflammation (calcitonin gene-related before vertigo begins.
peptide and serotonin) are expressed in the vestibular system and reciprocally
connected to the trigeminal nuclei and from there to thalamocortical projections ● The wide spectrum of
clinical manifestations and
and nociceptive brainstem areas. Thus, longer-lasting vestibular symptoms vestibular laboratory
could stem from processes paralleling the headache phase of migraine related findings in vestibular
to the trigeminovascular system. migraine suggests
Patients who have migraines have reduced thresholds to a variety of heterogeneous
pathophysiologic
sensory stimuli, such as light, sound, smell, and tactile stimuli. This sensory
mechanisms.
hypersensitivity appears to extend to vestibular and motion stimuli. They
have higher rates of optokinetic stimulation-induced motion sickness,19
enhanced perceptual sensitivity to head motion in the roll plane,20 and larger
errors in spatial orientation during lateral head tilts,21 implying abnormal
integration of semicircular canal and otolith inputs. These and other findings
suggest patients with vestibular migraine have alterations in sensory
processing and integration of inputs contributing to the perception of spatial
orientation.
The P/Q-type calcium channelopathy episodic ataxia type 2 manifests
clinically with episodic vertigo, and half of the patients with that condition have
migraine.22 Other kindreds with apparently dominantly inherited syndromes
that include both migraine and vertigo have been described, but a similar ion
channel defect has not been identified for those or others with vestibular
migraine.23 It has been suggested that a channelopathy causing migraine could
also cause an osmotic disequilibrium in the inner ear that results in
endolymphatic hydrops, the pathologic process underlying Ménière disease.
The vertigo attacks of vestibular migraine and Ménière disease have similar
duration, and one study found that vertigo attacks in Ménière disease have
migrainous features (headache, photophobia, or visual aura) in 45%
of patients.24

Clinical Features
Numerous case series have described the clinical features of vestibular migraine.
Evolving diagnostic criteria and referral bias in neuro-otology clinics likely
influence some of the findings. Clinical features may be divided into ictal
(episodic) symptoms and signs, interictal symptoms and signs, and other patient
characteristics and comorbidities.

EPISODIC SYMPTOMS. Patients may describe vertigo that is internal (a false

sensation of self-motion) or external (a false sensation that the visual surround is

CONTINUUMJOURNAL.COM 373

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

spinning or flowing). The character is often that of spinning but may also
commonly be rocking, tilting, swaying, falling, or floating.11,13 Frequently,
accompanying postural unsteadiness or nonvertiginous lightheadedness is
present. Although vestibular migraine most commonly causes spontaneous
episodes of dizziness, patients frequently describe their vestibular symptoms as
being triggered by (and aggravated by) head motion, visual stimuli (such as
complex motion–rich visual environments, busy patterns, optic flow, or
screen motion), or changing head positions such as to supine or side-lying.13,25,26
Patients may also report episodes provoked by other common migraine triggers
such as stress, sleep deprivation, menses, bright lights, specific foods, or
weather changes.
The temporal relationship between headaches and vertigo is quite variable.
Migrainous headaches are associated with at least some of the vestibular episodes
in between 50% and 94% of patients.13,14,27 Although some patients rarely
experience vertigo and headache together, some report a consistent pattern with
their episodes, with vertigo occurring before, during, or after the headache
phase. Few patients experience vertigo consistently as a typical aura lasting
5 to 60 minutes before headache onset.9,18
Other migrainous symptoms appear to be more common than headache
during vertigo attacks. Case series report photophobia in 70% to 90% of patients,
phonophobia in 60% to 90% of patients, and migraine auras (usually visual) in
13% to 36% of patients.13,14,18,28 Nausea with or without vomiting occurs in the
majority of patients but is not specific for migraine because nausea is a symptom
of most vestibular disorders. Auditory symptoms beyond phonophobia occur in
40% to 60% of patients.9,13,28,29 These most often include tinnitus and aural
pressure/fullness but may also include muffled hearing or other subjective
hearing impairment. Usually binaural, these symptoms can be monaural and
thus do not immediately discriminate conditions such as Ménière disease from
vestibular migraine, although objective low-frequency sensorineural hearing loss
is not a feature of vestibular migraine. Other nonspecific symptoms such as
cognitive slowing, fatigue, visual blurring, word-finding difficulty, extrapersonal
misperceptions, or visual distortions are reported not uncommonly.13
The duration of a vestibular migraine episode is variable, but most last
minutes to hours. Roughly one-third of patients report episodes lasting minutes,
one-third lasting hours, and one-third lasting longer than 1 day.18,30 Occasionally,
patients report brief paroxysmal vestibular symptoms lasting seconds, ranging
from vertigo to directional pulsion to postural unsteadiness, without other
accompanying symptoms. Those symptoms alone would not meet criteria for
vestibular migraine. Like other migraine types, the frequency of vestibular
migraine episodes is also highly variable, but most occur a few times per year to a
few times per month.11,12,27,31

EXAMINATION FINDINGS DURING EPISODES. Examination findings during episodes of

vestibular migraine are highly variable. Case series describing ocular motor signs
typically use video-oculography with visual fixation removed, which is more
sensitive at detecting subtle abnormalities than routine bedside examination.
One series found 70% of patients to have pathologic nystagmus during episodes,
which could be spontaneous, gaze-evoked, or positional and have either central
or peripheral features.32 An abnormal head impulse test can sometimes
accompany spontaneous horizontal nystagmus, supporting a unilateral deficit in

374 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

the vestibulo-ocular reflex during episodes. Most patients have impaired gait and KEY POINTS
stance during episodes, showing progressively increasing rates of difficulty with
● The character of
standard gait, Romberg test, tandem gait, and tandem Romberg test. A study vestibular symptoms varies
capturing ictal nystagmus in patients with episodic vertigo found 44 of 67 widely in vestibular
patients with vestibular migraine to have spontaneous horizontal (n = 28), migraine. Vertigo may be
upbeat (n = 6), or downbeat (n = 10) nystagmus; 16 had positional nystagmus external or internal spinning,
rocking, tilting, swaying,
only, and 7 had no nystagmus.33 Strong spontaneous horizontal nystagmus
falling, or floating.
(>12 degrees per second) was much more sensitive and specific for Ménière Symptoms may be
disease than vestibular migraine, but spontaneous vertical nystagmus was highly spontaneous or may be
specific for vestibular migraine. As opposed to paroxysmal positional nystagmus triggered or aggravated by
position changes, head
with benign paroxysmal positional vertigo (BPPV), positional nystagmus in
movements, or visual
vestibular migraine persisted for the duration the head position was maintained. stimuli.
Another study found that a sustained, low-velocity positional nystagmus could
be elicited in all 26 patients examined during vestibular migraine attacks with ● The temporal relationship
visual fixation blocked, and this could be horizontal, vertical, or torsional.34 between headaches and
vertigo is quite variable in
Spontaneous nystagmus was observed in only 19% but could be provoked by vestibular migraine, but few
horizontal headshaking in 35%. patients experience vertigo
consistently as a typical
INTERICTAL SYMPTOMS. Although vestibular migraine is considered an episodic aura.
vestibular disorder, patients report a high prevalence of symptoms between
● Auditory symptoms occur
attacks. It is sometimes unclear whether these should be considered within the during episodes in about half
spectrum of vestibular migraine or represent comorbid vestibular disorders. Most of patients with vestibular
patients have a past or concurrent history of more typical headache-predominant migraine and can create
diagnostic confusion with
migraine with or without aura. But beyond the episodic vestibular symptoms
Ménière disease.
meeting the criteria for vestibular migraine, other vestibular symptoms are common.
Motion sickness affects those with migraine much more commonly than those ● Most patients with
without. One study found 61% of 131 patients with vestibular migraine experience vestibular migraine have
motion sickness,13 although it is uncertain whether patients with vestibular migraine nystagmus during episodes,
although tools to block
have an even higher susceptibility than other patients with migraine.35,36 visual fixation may be
Patients with vestibular migraine frequently report sensitivity to head motion needed to appreciate it. It
and visual stimuli beyond that of classic motion sickness, even when such may be present in the
triggers do not provoke a full episode of vestibular migraine. One series reported upright position or only
during positional testing,
visually induced dizziness in 89%, including sensitivity to supermarket aisles, may look central or
busy visual patterns, motion on large movie screens (three-dimensional, IMAX), peripheral, and may be
scrolling on electronic screens, video games, optic flow in vehicles, or visual horizontal, vertical, or
motion (windshield wipers, ceiling fans). The same series reported dizziness torsional. However, very
intense horizontal
induced by quick head movements in 66%, beyond the already high 13% of
nystagmus is more
patients with vestibular migraine with coexisting BPPV.13 In fact, 51% of this suggestive of Ménière
cohort with vestibular migraine reported persistent, almost constant dizziness. disease.
When these visually and motion-induced symptoms are so pervasive as to be
present throughout most days, some authors have considered whether this may ● Between episodes,
patients with vestibular
represent a form of chronic vestibular migraine. However, it may be more migraine experience higher
appropriate to make a coexisting diagnosis of persistent postural perceptual rates of motion sickness,
dizziness (PPPD) if criteria for that condition are met.37 Vestibular migraine appears head motion–induced
to be among the most common conditions leading to the development of comorbid dizziness, and visually
induced dizziness with
PPPD, with up to 35% of patients with vestibular migraine ultimately developing complex or moving visual
PPPD by the time they reach a tertiary referral center for evaluation of dizziness.18,38 stimuli.
In addition to motion sickness and chronic dizziness of PPPD, patients with
vestibular migraine have higher rates of psychiatric comorbidities and
psychological strain compared with those with other vestibular disorders or

CONTINUUMJOURNAL.COM 375

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

controls. This appears independent of any measurable degree of vestibular

dysfunction on laboratory testing. Anxiety or depression affects more than half
of patients with vestibular migraine.13,39,40 Rates of insomnia, phobic disorders,
and functional disorders also appear to be increased.

INTERICTAL EXAMINATION AND LABORATORY TEST FINDINGS. The general neurologic

examination of patients with vestibular migraine is normal between episodes, so
any abnormalities suggest a comorbid condition or alternative diagnosis. Even a
detailed bedside ocular motor and vestibular examination is typically normal.
Among 131 patients assessed in a retrospective chart review study, none had
any abnormalities of alignment, extraocular range, saccades, pursuit, dynamic
visual acuity, vestibulo-ocular reflex by head impulse test, or vestibulo-ocular
reflex suppression nor any spontaneous nystagmus, and only 3.8% had
gaze-evoked nystagmus.13 However, minor nonspecific peripheral or central
vestibular and ocular motor findings are common during the symptom-free
period when carefully evaluated with quantitative tools, such as
videonystagmography, that allow for eliminating visual fixation to examine
nystagmus. Case series found that 33% to 70% of patients with vestibular
migraine have at least one of the following test abnormalities: spontaneous,
gaze-evoked, positional, or other triggered (head-shaking– or vibration-induced)
nystagmus; impaired pursuit; or vestibulo-ocular reflex abnormalities.11,12,41–45
Positional nystagmus is most often persistent, asymptomatic, and central
appearing. Studies have also found increased rates of such abnormalities in
patients with migraine without vestibular symptoms.41,42
A variety of vestibular laboratory tests are more commonly abnormal in
patients with vestibular migraine than in controls, but none of them is specific
for the diagnosis. Caloric testing is reportedly abnormal in about 20% of patients,
most often a reduced unilateral response or abnormal directional preponderance
but occasionally a bilateral reduced response.11,12,41,43,46 Video head impulse
testing may be abnormal in about 10% of patients.46 Studies measuring
vestibular-evoked myogenic potentials to test otolith pathway function have
shown conflicting findings of various parameters in patients with vestibular
migraine. Although the rate of vestibular-evoked myogenic potential
abnormalities is higher in these patients than in controls, overall they are not
specific to allow or refute a diagnosis of vestibular migraine nor can they
discriminate between vestibular migraine and Ménière disease as the cause of
episodic vertigo in individual patients.47–49

Differential Diagnosis
The differential diagnosis for vestibular migraine is broad and largely consists of
other conditions that cause episodic dizziness (TABLE 4-2), particularly those
causing vertigo with a similar temporal profile. Most of these disorders are
discussed in subsequent sections, but a few comments about them are
particularly relevant to the differential diagnosis of vestibular migraine.

u The diagnosis of migraine with brainstem aura requires two or more brainstem symptoms
acting as an aura lasting 5 to 60 minutes before a migraine headache. Only a small fraction
of patients with vestibular migraine meets such criteria.11,18
u Ménière disease is about 1/10 as common as vestibular migraine but can be difficult to
distinguish from vestibular migraine because of overlapping clinical features.9,50 Just as

376 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 auditory symptoms are common during attacks of vestibular migraine (see the earlier section KEY POINTS
Episodic Symptoms), migrainous symptoms such as headache are common during attacks
of Ménière disease.18,24,29,51 Occasionally, patients meet the criteria for both conditions. ● Patients with vestibular
u Vertebrobasilar TIAs can cause episodes of isolated vertigo lasting from minutes to 1 to migraine have higher rates of
2 hours without other brainstem symptoms. These would typically not be accompanied other coexisting vestibular
by migrainous symptoms and would be an unlikely cause of vertigo attacks spread weeks disorders such as benign
or months apart. paroxysmal positional
vertigo and persistent
u BPPV is the most common vestibular disorder (although uncommon in the teens and postural perceptual
twenties) and is comorbid with vestibular migraine more often than would be expected by dizziness, as well as higher
chance. Vestibular migraine can sometimes be mistaken for BPPV when it causes primarily rates of anxiety and
positional-induced symptoms. Typically, the two can be distinguished by history based depression than the general
on the temporal profile. During episodes of vestibular migraine, patients may experience population.
persisting vertigo for the duration the offending position is maintained, but the entire
episode lasts hours to 1 or 2 days, with episodes weeks to months apart. ● The general neurologic,
u PPPD is a chronic vestibular disorder of daily fluctuating nonvertiginous dizziness. As ocular motor, and vestibular
discussed earlier in the section Interictal Symptoms, it can sometimes be difficult to know examinations are typically
whether to attribute vestibular symptoms to PPPD or vestibular migraine, particularly in normal between episodes of
patients with chronic migraine headaches who have long-standing episodic unsteadiness vestibular migraine,
superimposed on a strong background of chronic nonvertiginous dizziness.18 although minor nonspecific
peripheral or central
u Vestibular paroxysmia causes spontaneous episodes of vertigo lasting less than 1 minute,52 vestibular and ocular motor
although patients with episodes lasting up to 5 minutes may meet criteria for probable findings are common during
vestibular paroxysmia but also reach the duration qualifying for vestibular migraine. the symptom-free period
when carefully evaluated
with quantitative tools such
Diagnostic Process as videonystagmography.
In the absence of any specific clinical examination signs or diagnostic tests,
● A variety of vestibular
vestibular migraine is diagnosed based on symptoms meeting diagnostic criteria,
laboratory tests are more
with consideration of the differential diagnosis mentioned earlier and commonly abnormal in
sometimes investigations to exclude those conditions. patients with vestibular
migraine than in controls,
DIAGNOSTIC CRITERIA. Building on the criteria originally proposed by Neuhauser but none of them is specific
14 for the diagnosis.
and colleagues in criteria from 2001, the International Headache Society and
the Bárány Society jointly developed and published diagnostic criteria in 2012 ● Vestibular migraine is
and 2013 (TABLE 4-3).53,54 Essentially, these require having migraine with or diagnosed based on
without aura, recurrent episodes of vestibular symptoms lasting 5 minutes to symptoms meeting
diagnostic criteria, with
72 hours, and at least one migrainous feature besides nausea with more than half consideration of the
of vestibular episodes. The Bárány Society also published criteria for probable differential diagnosis and
vestibular migraine for when the relationship between episodic vestibular sometimes investigations to
symptoms and migraine is less clear.54 exclude those conditions.
Unsurprisingly in practice, patients with recurrent spontaneous episodes of
● Diagnosing vestibular
vestibular symptoms more commonly meet the criteria for probable vestibular migraine requires a patient
migraine than vestibular migraine. In that case, it is appropriate to consider the meets International
differential diagnosis, perform any investigations needed to exclude other Headache Society criteria
causes, identify and manage any coexisting vestibular disorders (eg, BPPV, for migraine with or without
aura, have recurrent
PPPD), treat as vestibular migraine, and reconsider the diagnosis if the patient episodes of vestibular
does not respond to treatment or if clinical features change. Over time, some symptoms lasting 5 minutes
patients with probable vestibular migraine will eventually meet criteria for to 72 hours, and have at
vestibular migraine and some will not, but it is uncommon for patients with least one migrainous feature
besides nausea with more
probable vestibular migraine to ultimately develop an alternative disorder such than half of vestibular
as typical Ménière disease.55 episodes.

EVALUATION. For many or most patients, a characteristic symptom history

meeting diagnostic criteria, a normal examination between episodes, and

CONTINUUMJOURNAL.COM 377

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

absence of any red flags to suggest an alternative diagnosis permit the clinician
to diagnose vestibular migraine in the office without any special diagnostic tests,
as is done with other forms of migraine. Some further evaluation may be
necessary to investigate alternative conditions:

u Positional testing in the office should routinely be performed to identify and treat BPPV,
especially in patients reporting any positional vestibular symptoms. Without special
equipment such as video goggles, such testing may trigger vertigo and nystagmus in
patients with BPPV, but it is virtually always normal in patients with vestibular migraine
between attacks.
u Audiometric evaluation should be obtained for patients with any auditory symptoms
during or between attacks to look for characteristic features of Ménière disease or, less
likely, superior canal dehiscence syndrome mimicking (or coexisting with) vestibular
migraine.
u Neuroimaging should be considered for patients with recent-onset episodes lasting
minutes, especially those with vascular risk factors, to exclude the possibility of
vertebrobasilar TIAs presenting as isolated episodes of vertigo. MRI of the brain with
diffusion-weighted imaging and magnetic resonance angiography (MRA) or CT
angiography of the posterior circulation can exclude a completed stroke or critical
stenosis. Vestibular schwannoma, best identified with gadolinium-enhanced MRI of the
internal auditory canals, would rarely cause episodic vertigo mimicking vestibular
migraine, especially in the absence of progressive monaural sensorineural
hearing loss.

TABLE 4-3 Diagnostic Criteria for Vestibular Migraine and Probable Vestibular Migrainea

Vestibular migraine
A At least five episodes with vestibular symptomsb of moderate or severe intensity,c lasting
5 minutes to 72 hours
B Current or previous history of migraine with or without aura according to the International
Classification of Headache Disorders (ICHD)
C One or more migraine features with at least 50% of the vestibular episodes:
• Headache with at least two of the following characteristics: one-sided location, pulsating
quality, moderate or severe pain intensity, aggravation by routine physical activity
• Photophobia and phonophobia
• Visual aura
D Not better accounted for by another vestibular or ICHD disorder
Probable vestibular migraine
A At least five episodes with vestibular symptomsb of moderate or severe intensity,c lasting
5 minutes to 72 hours
B Only one of the criteria B and C for vestibular migraine is fulfilled (migraine history or
migraine features during the episode)
C Not better accounted for by another vestibular or ICHD disorder

a
Reprinted from Lempert T, et al, J Vestib Res.54 © 2012 IOS Press and the authors.
b
Qualifying vestibular symptoms include spontaneous vertigo, positional vertigo, visually induced vertigo,
head motion–induced vertigo, or head motion–induced dizziness with nausea.
c
Vestibular symptoms are moderate or severe if they interfere with daily activities.

378 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

u Vestibular laboratory testing is not required to diagnose vestibular migraine and commonly
reveals minor nonspecific central or peripheral abnormalities that create diagnostic
confusion. Testing can be helpful in select circumstances when concern exists for an
alternative cause of episodic vertigo such as Ménière disease or superior canal dehiscence
syndrome or when additional persistent symptoms raise the question of a unilateral or
bilateral vestibulopathy. Neurologists are generally skilled at detecting signs of central
nervous system dysfunction at the bedside (downbeat or gaze-evoked nystagmus,
impaired pursuit, dysmetric saccades), although vestibular testing can evaluate these
ocular motor signs in a more sensitive and quantitative way.

Treatment
Treatment for vestibular migraine has not been evaluated in large, well-designed,
controlled trials. Most data come from numerous case series and retrospective
reviews vulnerable to placebo effect, spontaneous improvement, or potentially
biased un-blinded investigators. Thus, current recommendations represent a
synthesis of these available studies, anecdotal experience, expert opinion, and
adaptation from the much larger and scientifically solid migraine headache
literature (TABLE 4-4).

NONPHARMACOLOGIC MANAGEMENT. Education and reassurance may be the

most important aspects of management. Many patients with vestibular

Treatment Options for Vestibular Migraine TABLE 4-4

Nonpharmacologic management
◆ Education and reassurance
◆ Sleep hygiene
◆ Trigger avoidance (eg, dietary restriction)
◆ Stress reduction
◆ Regular exercise
Acute symptomatic or abortive medications
◆ Antihistamines (eg, meclizine, dimenhydrinate)
◆ Benzodiazepines (eg, diazepam, lorazepam)
◆ Antiemetics (eg, promethazine, prochlorperazine, metoclopramide)
◆ Triptans
Prophylactic medications
◆ Antiepileptic drugs (valproic acid, topiramate, gabapentin, lamotrigine)
◆ Beta-blockers (propranolol, atenolol, metoprolol)
◆ Calcium channel blockers (flunarizine,a cinnarizine,a verapamil, diltiazem, lomerizinea)
◆ Serotonin norepinephrine reuptake inhibitors (SNRIs) (venlafaxine)
◆ Tricyclic antidepressants (amitriptyline, nortriptyline)
◆ Cyproheptadine
◆ Pizotifena

a
Not approved by the US Food and Drug Administration (FDA) for use in the United States.

CONTINUUMJOURNAL.COM 379

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

migraine have had unexplained attacks of vertigo for years or decades

without a diagnosis or with an incorrect diagnosis, most often Ménière
disease, BBPV, or a symptomatic label such as recurrent vestibulopathy.
Educating patients that, although episodes can be temporarily disabling, they
are not going to permanently lose hearing or vestibular function or have a
stroke can help frame the treatment discussion toward symptom control and
shared treatment decision making. Because patients often seek care when
their attacks are most frequent or severe, the expected disease fluctuations
over time commonly improve. Patients should examine modifiable
lifestyle factors that could be contributing to migraine (eg, stress,
insufficient/irregular sleep, triggering foods, insufficient exercise) and
address those where possible to take an active role in management. If
vestibular migraine episodes are mild, short, or infrequent, pharmacologic
management may be unnecessary unless indicated for other migraine
symptoms such as headache.

ACUTE EPISODE TREATMENT. Antihistamines (eg, meclizine, dimenhydrinate),

benzodiazepines (eg, diazepam, lorazepam), or antiemetics (eg, promethazine,
prochlorperazine, metoclopramide) can be used to treat significant vertigo and
nausea when episodes tend to last more than 30 minutes (long enough for the
medication to begin to work). Most patients prefer oral administration, although
some medications are available in a rectal formulation if vomiting makes oral
use impractical. In the acute care setting, the IV route is preferred.
Triptans have not been rigorously studied in vestibular migraine but should be
considered when headache symptoms accompany vertigo attacks or when
vertigo acts as a migraine aura. A pilot study of zolmitriptan for vestibular
migraine found a nonsignificantly higher response rate with treatment versus
placebo (38% versus 22%) but was underpowered because of low enrollment.56
Rizatriptan pretreatment reduced vestibular-induced motion sickness in patients
with migraine in one small trial.57 A multicenter, randomized, double-blind,
placebo-controlled trial of rizatriptan for treating vertigo attacks of vestibular
migraine is currently underway. Noninvasive vagal nerve stimulation and
external trigeminal nerve stimulation have also been reported in single-center
retrospective case series as potentially shortening attack duration or reducing
vertigo intensity.58,59

PROPHYLACTIC MEDICATIONS. Migraine prophylactic medications may be

appropriate for patients with frequent episodes of vestibular migraine when
acute treatments are insufficient. The approach is similar to that for migraine
headache, with the goal of reducing the frequency and severity of the attacks.
Because prospective trials are limited and almost every migraine preventative has
been reported as potentially effective in some case series, medication choice is
driven more by side effect profiles and comorbidities than efficacy data. A small
randomized controlled trial found the calcium channel blocker flunarizine plus
betahistine significantly reduced the frequency of vertiginous episodes in
vestibular migraine compared with betahistine alone.60 Another small
randomized controlled trial compared venlafaxine to propranolol (without a
placebo group) in vestibular migraine and found significant reductions in
vestibular symptom severity and attack frequency at 4 months; the treatment
outcome was similar in both groups, although venlafaxine also reduced

380 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

depression symptoms.61 A single-blinded randomized trial compared KEY POINTS
venlafaxine, flunarizine, and valproic acid in 25 patients per group and found
● Patients can often be
no difference among groups in total Dizziness Handicap Inventory score diagnosed with vestibular
reductions (although only venlafaxine improved the emotional domain); migraine based on a
valproic acid and venlafaxine but not flunarizine were associated with reduced characteristic history
vertigo attack frequency.62 Metoprolol was compared with placebo over meeting diagnostic criteria,
a normal examination
6 months in a multicenter, randomized, controlled trial but found no difference
between episodes, and an
(with both groups showing the same reduction in attack frequency over time) absence of any red flags.
before being terminated prematurely because of poor participant accrual.63 Case Further investigations to
series have reported many other medications used to treat migraine can be exclude alternative
diagnoses may include
helpful in vestibular migraine, including other calcium channel blockers
positional testing,
(cinnarizine, verapamil, diltiazem, lomerizine), beta-blockers (propranolol, audiometric evaluation,
atenolol, metoprolol), antiepileptic drugs (topiramate, gabapentin, lamotrigine, neuroimaging, or vestibular
valproic acid), tricyclic antidepressants (amitriptyline, nortriptyline), and laboratory testing.
medications from other classes (cyproheptadine, pizotifen).12,17,64–72 Among
● Treatment
these prophylactic medications studied, flunarizine, cinnarizine, betahistine, recommendations for
lomerizine, and pizotifen are not US Food and Drug Administration (FDA) vestibular migraine come
approved in the United States. largely from case series,
retrospective reviews,
expert opinion, a few small
VESTIBULAR THERAPY. Vestibular rehabilitation exercises probably have little role controlled trials, and
in the management of vestibular migraine for patients who only have adaptation from the much
spontaneous episodes of vestibular symptoms with no interictal symptoms or larger migraine headache
other vestibular comorbidities that would benefit from vestibular rehabilitation. literature.
A review of five retrospective and prospective studies examining the role of
● Identifying and treating
vestibular rehabilitation in patients with vestibular migraine (along with other comorbid conditions is
conditions but generally without a control group) found the current evidence critical in the management
inconclusive.73 of vestibular migraine.

● Ménière disease is an
TREATING COMORBID CONDITIONS. Identifying and treating comorbid conditions inner ear disorder whose
are critical in the management of vestibular migraine.38 In addition to clinical syndrome consists
considering and ruling out alternative diagnoses in the differential (discussed in of spontaneous episodes of
the earlier Differential Diagnosis section), some conditions not infrequently vertigo associated with
typically unilateral
coexist with vestibular migraine, including BPPV, PPPD, motion sickness, and fluctuating sensorineural
anxiety (CASE 4-1). Some patients meet criteria for both vestibular migraine and hearing loss, tinnitus, and
Ménière disease and require careful medical management for both conditions, aural fullness.
sometimes using headache behavior as an indicator of migraine treatment
response, before considering any ablative procedure for Ménière disease.29
Serotonin norepinephrine reuptake inhibitors (SNRIs), particularly venlafaxine,
may be ideal when vestibular migraine coexists with PPPD, anxiety, or
depression.

MÉNIÈRE DISEASE
Ménière disease is an inner ear disorder whose clinical syndrome consists of
spontaneous episodes of vertigo associated with typically unilateral fluctuating
sensorineural hearing loss, tinnitus, and aural fullness. Although
histopathologically associated with endolymphatic hydrops within the labyrinth,
it remains uncertain precisely how this relates to the clinical findings.75 This
article discusses the clinical features and diagnosis of Ménière disease. For more
information about its pathophysiology and treatment, refer to the articles
“Selected Otologic Disorders Causing Dizziness” by Gail Ishiyama, MD,76 and

CONTINUUMJOURNAL.COM 381

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

“Tinnitus, Hyperacusis, Otalgia, and Hearing Loss” by Terry D. Fife, MD, FAAN,
FANS, and Roksolyana Tourkevich, MD,77 in this issue of Continuum.

Clinical Features
The prevalence of Ménière disease in the United States has been reported as
190 per 100,000, with slightly more women affected than men.78 It most
commonly begins in the fourth to sixth decades, with prevalence increasing with
advancing age. It is rare in children (9 per 100,000 children). Most patients
present with vertigo and cochlear symptoms together, although some may
experience only vertigo or hearing loss alone initially. If sensorineural hearing
loss predates the onset of episodic vertigo by many months or years, it is called
delayed endolymphatic hydrops or delayed Ménière disease. At disease onset, the
frequency of attacks averages 4 to 6 times per year but rapidly declines in the
first 5 to 10 years to reach 1 to 2 times per year, slowly declining thereafter until
eventually “burning out.”79 However, the clinical course can vary considerably
among patients, with intervals of unrelenting attacks separated by long periods
of only occasional attacks.

CASE 4-1 A 37-year-old woman presented with episodic and chronic vestibular
symptoms. She had a 20-year history of occasional migraine headaches
without aura. She had previously been treated for generalized anxiety
disorder. Four years ago, she began having spontaneous episodes of
rocking or spinning vertigo, aggravated by head movement or
environmental motion and usually accompanied by nausea, photophobia,
and phonophobia. She experienced binaural tinnitus with them but not
aural fullness or hearing loss. With a few of the dozen episodes, she
developed a unilateral throbbing headache. Episodes typically lasted
half a day, although some lasted 2 days.
Last year, she also began experiencing a feeling of floating woozy
lightheadedness and mild unsteadiness almost every day, aggravated by
her own movements, walking through busy stores or streets, scrolling on
her computer or smartphone, or watching action movies. She felt better
when she was recumbent. She had repeatedly normal neuroimaging,
audiograms, and vestibular laboratory testing. She worried about
overlooked multiple sclerosis or brain tumor. She had restricted many
activities because of her daily symptoms and concern that she may
become incapacitated by an attack. Neurologic, ocular motor, and
vestibular examinations were normal.

COMMENT This case illustrates the challenge of coexisting episodic and chronic
vestibular syndromes along with the diagnosis and management of
relevant comorbidities. The patient’s episodic vestibular symptoms along
with long-standing migraine headaches are consistent with vestibular
migraine. Although tinnitus might raise the possibility of Ménière disease,
the binaural nature and absence of fluctuating or progressive hearing loss

382 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 Attacks of Ménière disease typically begin with cochlear symptoms such as
roaring or rushing tinnitus, a sensation of aural fullness or pressure, and hearing
loss (or distorted, muffled hearing). Within minutes to sometimes hours, vertigo
(most commonly spinning) rapidly develops and quickly peaks in intensity
before declining over the course of 20 minutes to several hours, averaging 2 to
3 hours. If examined during an attack, most patients have intense spontaneous
horizontal jerk nystagmus that ultimately reverses direction during the attack.
Generally, the nystagmus initially beats toward the affected ear (excitatory or
irritative phase) before reversing direction within 12 hours (inhibitory or paretic
phase). Subsequently, another reversal representing “recovery nystagmus” may
occur.33,80 During episodes, patients commonly experience nausea, vomiting,
sweating, diarrhea, severe unsteadiness, and such head motion intolerance that
they must lie perfectly still until the episode subsides. They often have some mild
residual unsteadiness for 1 to 2 days.
Although the sensorineural hearing loss fluctuates and initially recovers after
attacks, it progressively worsens over time, generally still affecting lower
frequencies but eventually flattening out to affect all frequencies and becoming

after 4 years is inconsistent with Ménière disease. The diagnostic criteria

for vestibular migraine require that at least half of the episodes be
accompanied by migrainous symptoms, but they do not need to be
headache. Probable vestibular migraine does not require a temporal
relationship between episodic vestibular symptoms and migrainous
symptoms.
This patient developed daily nonvertiginous dizziness and unsteadiness
consistent with the entity persistent postural perceptual dizziness (PPPD).
Vestibular migraine and generalized anxiety disorder are among the most
common conditions leading to the development of PPPD. For her, although
episodes of vestibular migraine a few times per year were briefly
debilitating, she was much more affected by daily symptoms of PPPD,
catastrophic thinking, and avoidance behavior. Management must
consider her migraine headaches, episodic vertigo, PPPD, and anxiety.
Acute treatment for vestibular migraine episodes may include vestibular
suppressants, antiemetics, or triptans. Because her migraine episodes
(vestibular and headache) occurred less than 1 time per month, they may
not warrant prophylactic medication, although it could be considered if
they were particularly severe, prolonged, or difficult to treat acutely. Her
PPPD would be best treated with a selective serotonin reuptake inhibitor
(SSRI) or serotonin norepinephrine reuptake inhibitor (SNRI). Although any
such medication may also help treat her anxiety, venlafaxine may be an
ideal choice because it has some supporting data for migraine and
vestibular migraine, as well. Habituation-type vestibular therapy should be
initiated to help desensitize her to head motion, visual motion, and
complex visual environments.74 Cognitive-behavioral therapy may help
address aspects of illness anxiety and avoidance behavior.

CONTINUUMJOURNAL.COM 383

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

less varying. Tinnitus also generally becomes persistent but may still fluctuate in
intensity. Estimates of eventual contralateral ear involvement vary widely, but
ultimately roughly 30% (10% to 50%) of patients develop some bilateral hearing
involvement.81 Bilateral Ménière disease may produce slightly more severe (and
bilateral) hearing loss with a flatter audiometric curve.82
Sudden, unexplained falls without vertigo or loss of consciousness can occur in
a minority (less than 10%) of patients with Ménière disease. These otolithic crises
of Tumarkin, or drop attacks, are often described by patients as if they have been
forcefully shoved or thrown to the ground or as if the floor has been pulled out
from under them.83 They often occur within the first few years of the disease,
with most affected patients having two or three attacks over the course of a year
or less before they remit. Similar drop attacks have been described in patients
with migraine, cardiac, or cerebrovascular disorders.84

Diagnosis
Ménière disease is a clinical diagnosis. Diagnostic criteria include specific
audiometric findings. Imaging and vestibular laboratory testing sometimes play
a role.

DIAGNOSTIC CRITERIA. In 2015, the Bárány Society, in collaboration with other

international societies, jointly developed and published updated international
consensus diagnostic criteria for Ménière disease (TABLE 4-5).50 These updates
included four major changes from the widely used 1995 American Academy of
Otolaryngology–Head and Neck Surgery criteria: (1) elimination of the histologically
“certain” and the “possible” categories, (2) requirement for audiometrically
documented low- to medium-frequency hearing loss in the definite category only,

TABLE 4-5 Diagnostic Criteria for Ménière Diseasea

Definite Ménière disease

A Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours
B Audiometrically documented low- to medium-frequency sensorineural hearing lossb in one
ear, defining the affected ear on at least one occasion before, during, or after one of the
episodes of vertigo
C Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear
D Not better accounted for by another vestibular diagnosis
Probable Ménière disease
A Two or more episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours
B Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear
C Not better accounted for by another vestibular diagnosis

a
Reprinted from Lopez-Escamez JA, et al, J Vestib Res.50 © 2015 IOS Press and the authors.
b
Low-frequency sensorineural hearing loss is defined as increases in pure tone thresholds for
bone-conducted sound that are worse in the affected ear than in the contralateral ear by at least
30 decibels hearing loss (dB HL) at each of two contiguous frequencies below 2000 Hz, or if bilateral, hearing
loss must be worse than 35 dB HL or more at each of two contiguous frequencies below 2000 Hz.
Demonstrating recovery of hearing loss at some point in time further supports the diagnosis of Ménière
disease.

384 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

(3) auditory symptoms should be KEY POINTS
fluctuating, and (4) vertigo
● Ménière disease attacks
attack duration with an upper typically produce severe
limit of 12 hours (definite) or spinning vertigo lasting 2 to
24 hours (probable).85 3 hours on average. If
examined during an attack,
most patients have intense
AUDIOMETRIC TESTING.
spontaneous horizontal jerk
Audiometrically documenting nystagmus that ultimately
low- to medium-frequency reverses direction during the
sensorineural hearing loss attack.
(FIGURE 4-1) is important for
● Although the
establishing the diagnosis early sensorineural hearing loss in
on, especially because tinnitus Ménière disease fluctuates
and even subjective hearing loss and initially recovers after
can occur with episodes of attacks, it progressively
FIGURE 4-1 worsens over time,
vestibular migraine,29,51 and Typical audiometric pattern in Ménière disease. Pure generally still affecting
other vestibular disorders such tone thresholds for air conduction (circles), masked lower frequencies but
as superior canal dehiscence air conduction (triangles), and masked bone eventually flattening out to
and vestibular schwannoma conduction (brackets) in the right ear indicate affect all frequencies and
moderately severe low- and medium-frequency (250 becoming less varying.
are associated with different
to 1000 Hz) sensorineural hearing loss compared with
patterns of hearing loss. Later normal air conduction hearing in the left ear (X). ● Audiometrically
on, audiometry can help documenting low- to
determine the progression of medium-frequency
sensorineural hearing loss is
hearing loss, involvement of the contralateral ear, potential benefit of hearing
important for establishing
amplification, or candidacy for cochlear implantation. the diagnosis of Ménière
disease early on, especially
ROLE OF IMAGING AND VESTIBULAR LABORATORY TESTING. Ménière disease remains because tinnitus and even
a clinical diagnosis supported by audiometric findings (CASE 4-2). Given subjective hearing loss can
occur with episodes of
its overlapping symptoms with other conditions such as vestibular migraine, vestibular migraine.
several tests have been investigated in hope of identifying markers that
would discriminate patients with Ménière disease from those without or
those with other vestibular disorders. Lack of a gold standard and a high
frequency of some test abnormalities in other conditions have made this
challenging.
MRI has been investigated to identify the presence of endolymphatic hydrops,
particularly expansion of the saccule. However, conflicting results in patients
with Ménière disease may be due to differences in MRI sequence parameters,
hydrops grading methods, and patient inclusion criteria. It appears that saccular
hydrops is associated with hearing loss and may be a function of disease duration,
but endolymphatic hydrops on MRI may also be seen in some patients with
vestibular migraine, tinnitus, autoimmune inner ear disease, or acute
sensorineural hearing loss without vertigo, as well as frequently in the
contralateral unaffected ear in patients with Ménière disease.86 At this time, MRI
does not yet have an established role in the diagnosis of Ménière disease,
although MRI of the internal auditory canal with gadolinium may be used to
exclude other retrocochlear causes of hearing loss and vertigo.
Vestibular laboratory testing may be normal or abnormal in patients with
Ménière disease. Caloric testing is often normal early in the disease course, but in
the first several years, caloric responsiveness in the affected ear frequently
deteriorates, with a functional reduction of about 35% to 50%.81 Video head

CONTINUUMJOURNAL.COM 385

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

impulse testing might provide a diagnostic complement to caloric testing in

Ménière disease.87,88 One study found that, among 606 subjects in a
neuro-otology clinic with various vestibular disorders who underwent both
tests, 27 of 73 patients with Ménière disease had discordant results, all with
asymmetric caloric testing but normal symmetric video head impulse testing.
This is in contrast to the 532 patients without Ménière disease, only 9 of whom

CASE 4-2 A 44-year-old man was evaluated for a 5-year history of episodic vertigo
and auditory symptoms. Initially, 5 years ago he developed progressive
right-sided hearing loss and intermittent tinnitus. Then 4 years ago, he
began having spontaneous attacks 2 times per month of external spinning
vertigo, generally lasting about 2 hours, accompanied by nausea and
vomiting and preceded by right aural fullness and buzzing tinnitus, but he
had no decrease in hearing during attacks. More recently, he had been
experiencing episodic focal throbbing headaches accompanied by
nausea about 2 times per month, but they were not temporally associated
with his vertigo attacks. Neurologic examination, including detailed
ocular motor and vestibular examination and otoscopy, was normal. With
video-oculography and visual fixation removed, both horizontal
headshaking and mastoid vibration produced left-beating nystagmus.
Audiometric testing was normal on the left but revealed moderate
low- and medium-frequency sensorineural hearing loss on the right, with
normal tympanogram and acoustic reflexes and 55% word recognition.
Videonystagmography demonstrated normal ocular motor and gaze
stability testing. A 39% right caloric weakness was observed. Video head
impulse testing of both lateral canals was normal. Ocular and cervical
vestibular-evoked myogenic potentials were normal and symmetric with
500-Hz tone bursts, but the right ear showed an abnormal upward shift in
the most sensitive cervical vestibular-evoked myogenic potential
threshold from 500 Hz to 1000 Hz.

COMMENT In this patient with right-sided sensorineural hearing loss and episodic
vertigo accompanied by tinnitus and aural fullness, evaluation
demonstrated supportive findings for a diagnosis of Ménière disease.
Diagnosing definite unilateral Ménière disease requires, in part,
audiometrically documented low- to medium-frequency sensorineural
hearing loss in the affected ear in which pure tone thresholds for
bone-conducted sound are at least 30 decibels hearing loss (dB HL) higher
at each of two contiguous frequencies below 2000 Hz compared with the
contralateral ear. Function of the vestibulo-ocular reflex pathways serving
the lateral canals was assessed two different ways. Dissociation between
normal video head impulse test and abnormal ipsilesional caloric testing
has been proposed as a potentially useful diagnostic finding in Ménière
disease that may be the result of enlargement of the membranous duct
causing a reduced response to caloric stimulation, as local convective flow
dissipates hydrostatic pressure across the cupula.

386 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

showed dissociation. Thus, more than two-thirds of patients with caloric video
head impulse testing dissociation had Ménière disease.89 Vestibular-evoked
myogenic potentials have been studied in Ménière disease. Asymmetrically reduced
cervical and ocular vestibular-evoked myogenic potential amplitude to
air-conducted sound but not bone-conducted vibration has been reported to be the
most common profile in Ménière disease, although it is still abnormal in only 30%
to 50% of patients compared with 10% to 20% of controls.90 Other
vestibular-evoked myogenic potential response metrics have been studied,
including shifts in tuning frequency, that could ultimately be useful to
discriminate Ménière disease from other causes of episodic vertigo or hearing
loss.91,92 Electrocochleography currently has little to no role in the diagnosis of
Ménière disease.

Differential Diagnosis
The differential diagnosis of Ménière disease includes other conditions causing
episodic vertigo or fluctuating progressive hearing loss (TABLE 4-6).

VERTEBROBASILAR TRANSIENT ISCHEMIC ATTACKS

Vertebrobasilar insufficiency from stenosis, embolism, or dissection may cause a
stroke, producing an acute vestibular syndrome (discussed elsewhere in this issue).
But vertebrobasilar ischemia may also manifest as TIAs of spontaneous vertigo.
The notion that episodes of isolated vertigo can be the sole symptom of a TIA,
preceding up to half of posterior circulation infarctions, has growing evidence in
the literature.93 Thus, although TIAs are an uncommon cause of episodic vertigo
across the population, they are an important and dangerous cause to consider,
especially in older patients with recent-onset symptoms and vascular risk factors.

Differential Diagnosis of Ménière Disease TABLE 4-6

◆ Autosomal dominant sensorineural hearing loss type 9 from the COCH gene or type 6/14
from the WSF1 gene
◆ Autoimmune inner ear disease
◆ Cerebellopontine angle tumor (eg, meningioma)
◆ Cogan syndrome
◆ Endolymphatic sac tumor
◆ Neuroborreliosis
◆ Otosyphilis
◆ Paraneoplastic encephalitis with Kelch-like protein 11 antibodies and testicular cancer
◆ Perilymphatic fistula
◆ Superior canal dehiscence syndrome
◆ Susac syndrome
◆ Vertebrobasilar transient ischemic attack or stroke
◆ Vestibular migraine
◆ Vestibular paroxysmia (neurovascular compression syndrome)
◆ Vestibular schwannoma

CONTINUUMJOURNAL.COM 387

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

Pathophysiology
The posterior circulation supplies both the central and peripheral structures of
the vestibular system. The posterior inferior cerebellar artery branches from the
vertebral artery and irrigates the caudal cerebellum (including the inferior
vermis, nodulus, and uvula) and lateral medulla (including the caudal portion of
the vestibular nuclei). The anterior inferior cerebellar artery (AICA) branches
from the basilar artery to irrigate the anterior inferior cerebellum (including the
flocculus), middle cerebellar peduncle, lateral pons, and inner ear. Transient
ischemia of vestibular structures may arise from several mechanisms, such as
atherosclerotic stenosis/thrombosis, artery-to-artery embolism, or arterial
dissection. Cardioembolism would be less likely to cause multiple identical
TIAs to the same vascular distribution. Very rarely, dynamic spondylotic
compression of the vertebral arteries during extreme head rotation can produce
vertebrobasilar ischemia with vertigo or near syncope, known as rotational
vertebral artery syndrome or bow hunter syndrome.94

Clinical Features
Vertebrobasilar ischemia may produce a variety of brainstem, cerebellar,
occipital lobe, medial temporal lobe, and thalamic symptoms, and such transient
neurologic symptoms appear to be more common in the immediate days or
weeks before vertebrobasilar territory strokes than carotid territory strokes.
Although attacks of multiple simultaneous posterior circulation symptoms
should alert the clinician to possible vertebrobasilar TIAs, a large
population-based study showed that sudden spontaneous episodes of vertigo
are the most common vertebrobasilar TIA symptom preceding a stroke and
consist more often of isolated vertigo (preceding 51% of posterior circulation
strokes) than accompanied by other posterior circulation symptoms, mimicking
peripheral vertigo.95 Of those isolated vertigo episodes, 52% lasted more than
1 hour, which is longer than typical TIAs.
Among patients in an emergency department whose acute transient isolated
vertigo had resolved within 24 hours and who were asymptomatic when
evaluated, other investigators found that 32% had a posterior circulation stroke
based on diffusion-weighted imaging (55%) or only by cerebellar hypoperfusion
on perfusion-weighted imaging (45%).96 Although vertebrobasilar ischemia
still represents a minority of patients presenting to the emergency department
with vertigo, a population-based study found a 9.3-times increased relative risk
of 30-day stroke in patients discharged with a diagnosis of peripheral vertigo
compared with those with renal colic, with the highest relative risk of 50 at
7 days.97 A large cohort study found that patients hospitalized for isolated vertigo
from all causes had a threefold greater risk of stroke in the subsequent 4 years
than a hospitalized control group, and patients with vertigo with three or more
vascular risk factors had a 5.5-fold higher stroke risk than those without risk
factors.98 Among patients with vertebrobasilar TIAs before stroke, up to
21% may have isolated episodes of vertigo for at least 4 weeks as the only
presenting symptom.99
Because the AICA supplies the internal auditory artery to irrigate the cochlea
and vestibular labyrinth, sudden or fluctuating hearing loss in the context of
vertigo does not automatically imply a benign peripheral vestibular disorder
such as Ménière disease or labyrinthitis. Sudden hearing loss occurring in
proximity to vertigo is associated with a significantly higher subsequent stroke

388 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

risk compared with hearing loss or vertigo alone, based on a large KEY POINTS
population-based study.100 Recurrent transient hearing loss and tinnitus alone
● Transient ischemic
can be the prodrome days before an AICA infarction producing hearing loss, attacks are an uncommon
tinnitus, vertigo, facial palsy, and hemiataxia.101,102 In fact, mounting evidence cause of episodic vertigo
suggests that new hearing loss occurring with an acute vestibular syndrome across the population, but
favors a stroke as the most likely cause.103 they are an important and
dangerous cause to
consider, especially in older
Evaluation patients with recent-onset
Identifying the small number of patients with a cerebrovascular cause among all symptoms and vascular risk
patients with spontaneous episodes of dizziness or vertigo is a challenge. The factors.
possibility of vertebrobasilar ischemia should be considered in any patient
● Isolated vertigo is the
presenting with a recent onset of spontaneous episodes of vertigo with or without most common warning
auditory symptoms, especially with episodes lasting minutes to 1 or 2 hours in symptom before
older patients or those with vascular risk factors. Eliciting other posterior vertebrobasilar stroke. Most
circulation symptoms may be diagnostically helpful, but they are often absent. such vertebrobasilar TIAs
last minutes to 1–2 hours.
Vertebrobasilar TIAs are an unlikely cause of recurrent vertigo that has been
occurring for more than 6 months. The patient group at greatest risk of an ● New hearing loss
overlooked cerebrovascular cause is young patients without vascular risk factors, accompanied by vertigo can
although that group also has the highest prevalence of vestibular migraine, a occur in lateral pontine or
inner ear stroke.
much more common benign cause. New headache, neck pain, or recent neck
trauma with vertigo may suggest the possibility of vertebral artery dissection.104
If the patient is still symptomatic at the time of evaluation, the HINTS Plus
(Head Impulse, Nystagmus, and Test of Skew plus bedside hearing test by finger
rub) battery can be used to examine the acute vestibular syndrome and
differentiate a central from peripheral cause to guide further workup and
management.93,105
If the patient with possible vertebrobasilar TIAs is not symptomatic at the time
of examination and the neurologic, ocular motor, and vestibular examinations
including positional testing are normal, then the diagnostic approach must be
based on the index of suspicion for a vascular cause. Patients with probable TIAs
based on symptom features or risk factors (eg, ABCD2 [age, blood pressure,
clinical features, duration, presence of diabetes mellitus] score greater than 3)
should be admitted or undergo rapid outpatient evaluation with MRI (including
diffusion-weighted imaging) and MRA or CT angiography of the head and neck
vessels. If available, perfusion-weighted MRI may increase the yield of
demonstrating ischemia in the acute transient vestibular syndrome.96

VESTIBULAR PAROXYSMIA
Occasionally patients report very brief (1 to a few seconds) episodes of vertigo
occurring up to dozens of times per day. These can be a diagnostic mystery
because often no other accompanying symptoms are present, and the
examination between episodes is normal. Originally reported by Jannetta106 as
disabling positional vertigo attributed to neurovascular cross-compression of
the vestibulocochlear nerve, this clinical syndrome is now referred to as
vestibular paroxysmia.52

Clinical Features
Vestibular paroxysmia represents a clinical syndrome that is most often
attributed to microvascular compression of the eighth cranial nerve, although
identical symptoms can occur from other compressive lesions or without

CONTINUUMJOURNAL.COM 389

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

evidence of a lesion at all. As with trigeminal neuralgia, the mechanism is thought

to be ephaptic transmission, which refers to coupling of the action potentials
via current flow through the extracellular space between adjacent axons. The
temporal profile is similar to that of trigeminal neuralgia. The frequency of
episodes varies widely, from one every few months to dozens per day. Most
attacks occur spontaneously, although they can sometimes be triggered by
position changes, head turns, loud noise, or hyperventilation, requiring
differentiation from BPPV, rotational vertebral artery syndrome, superior
canal dehiscence syndrome, or vestibular schwannoma, respectively. Attacks
generally last only a few seconds or up to 1 minute. Patients often report an
individually stereotyped type of spinning or nonspinning vertigo, fine
shimmering binocular oscillopsia, and/or directional pulsion that can be
associated with abrupt unsteadiness and falls. The simultaneous occurrence
of monaural tinnitus or hyperacusis, or rarely hemifacial spasm, can occasionally
localize the affected side.

Diagnosis
The vestibular and ocular motor examination is typically normal between
attacks, although hyperventilation (while removing visual fixation) can
sometimes induce nystagmus beating toward the affected side, suggesting an
excitatory nystagmus.107 In patients with such frequent attacks that they are
occurring during the office examination, brief bursts of spontaneous
horizontal-torsional nystagmus beating toward the affected ear may be seen
coinciding with the patient’s spontaneous episodes. Often, the nystagmus
amplitude is so low that it is best seen by patiently waiting to observe fine
intermittent jiggling of the eye’s conjunctival vessels or shimmering of the optic
disc during ophthalmoscopy time-locked to the patient’s reported symptoms.
Diagnostic criteria for vestibular paroxysmia were established in 2016 by the
Bárány Society based on clinical features and treatment response (TABLE 4-7).52

TABLE 4-7 Diagnostic Criteria for Vestibular Paroxysmiaa

Vestibular paroxysmia (each point needs to be fulfilled)

A At least 10 attacks of spontaneous spinning or nonspinning vertigo
B Duration less than 1 minute
C Stereotyped phenomenology in a particular patient
D Response to a treatment with carbamazepine or oxcarbazepine
E Not better accounted for by another diagnosis
Probable vestibular paroxysmia (each point needs to be fulfilled)
A At least five attacks of spinning or nonspinning vertigo
B Duration less than 5 minutes
C Spontaneous occurrence or provoked by certain head movements
D Not better accounted for by another diagnosis

a
Reprinted from Strupp M, et al, J Vestib Res.52 © IOS Press and the authors.

390 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 MRI with high-resolution constructive interference in steady-state (CISS) or KEY POINTS
fast imaging employing steady-state acquisition (FIESTA) sequences through
● Vestibular paroxysmia
the brainstem should be performed in patients with vestibular paroxysmia, in refers to recurrent
part to exclude a cerebellopontine angle tumor, arachnoid cyst, demyelinating spontaneous or sometimes
lesion, or other structural cause for the episodes. Demonstrating neurovascular triggered episodes of
cross-compression is not necessary for the diagnosis, and the precise role of vertigo lasting seconds to
1 minute that can occur up to
imaging in this regard requires further study. Although high-resolution MRI
dozens of times per day. It is
appears very sensitive at visualizing eighth cranial nerve compression, most most often attributed to
often from the AICA near the nerve root entry zone, that finding is not specific neurovascular cross-
for vestibular paroxysmia, as one study found 35% of unaffected control compression of the
vestibulocochlear nerve.
patients had similar neurovascular compression and another study found 42% of
Sometimes time-locked
patients with vestibular paroxysmia had bilateral neurovascular compression tinnitus aids localization.
despite presumably unilateral symptoms.108,109
The differential diagnosis for short attacks includes BPPV, otolithic crises of ● Most patients with
Tumarkin (in Ménière disease), paroxysmal brainstem attacks (from multiple vestibular paroxysmia
respond to carbamazepine
sclerosis or stroke), or superior canal dehiscence syndrome. In longer-lasting or oxcarbazepine.
attacks (longer than 5 minutes), vestibular migraine, Ménière disease,
vertebrobasilar TIAs, and panic attacks should be considered

Treatment
Medical therapy is generally effective at reducing or eliminating attacks of
vestibular paroxysmia. Treatment responsiveness is one of the diagnostic criteria.
The majority of patients respond to carbamazepine 200 mg/d to 800 mg/d or
oxcarbazepine 300 mg/d to 900 mg/d in divided doses.110 Lacosamide may be
another treatment option, particularly if contraindications to or side effects
with carbamazepine or oxcarbazepine are present.111 Microvascular
decompression of the eighth cranial nerve could be considered for patients in
whom the affected side is clearly established when they have responded to
medical treatment (further supporting the diagnosis) but cannot tolerate an
effective dose (CASE 4-3).

BENIGN RECURRENT VERTIGO

The entity known as benign recurrent vertigo is a condition of spontaneous episodes
of vertigo without migrainous, neurologic, or otologic features that do not go on to
cause any persistent vestibular or hearing loss.112 No consensus diagnostic criteria
exist, so the literature is quite sparse and variable. The temporal profile is generally
similar to that of vestibular migraine, with recurrent spontaneous episodes of
vestibular symptoms lasting minutes to 1 or 2 days.113 Strong links to migraine
exist, and today many such patients would meet the criteria for probable
vestibular migraine if a current or past history of migraine exists.7,8,15
Clinical features and outcomes were examined in 66 patients (73% women)
with benign recurrent vertigo.113 Vertigo attack duration varied from minutes to
72 hours. Family history of migraine or recurrent vertigo was reported in 51%.
Four of the 66 patients developed vestibular migraine at a median 32.5-month
follow-up. Among 338 patients with benign recurrent vertigo, other investigators
identified a subgroup of 35 with a new diagnostic entity they called recurrent
spontaneous vertigo with interictal headshaking nystagmus.114 These patients, with
robust post–head-shaking nystagmus as an interictal marker of central vestibular
dysfunction within the velocity storage mechanism, had a higher susceptibility to
motion sickness than the other patients with benign recurrent vertigo.

CONTINUUMJOURNAL.COM 391

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

CASE 4-3 A 57-year-old woman was evaluated for a 2-year history of brief
stereotyped paroxysms of tinnitus and oscillopsia. Her spells would
begin with sudden static-like left tinnitus. Within 5 seconds, she
developed fine shimmering binocular horizontal oscillopsia, without a
clear sense of spinning, pulsion, or unsteadiness. The visual symptoms
and then the tinnitus subsided within 20 seconds. Spells occurred
spontaneously, without any clear trigger, hundreds of times per day.
She experienced several spells during examination, but it was difficult
to appreciate any nystagmus upon direct inspection or even with infrared
video-oculography. However, with fundoscopy, within a few seconds of
her indicating the onset of tinnitus, she developed fine shimmering of the
optic disc indicative of nystagmus lasting 10 seconds that correlated with
the room oscillopsia experienced in the other eye. The tinnitus subsided a
few seconds after the nystagmus stopped. The only other abnormal
examination finding was an abnormal leftward head impulse test.
Caloric testing revealed a 54% left caloric weakness. Left ocular
vestibular-evoked myogenic potential was absent. Mastoid vibration
produced right-beating nystagmus. Previous MRI of the brain and internal
auditory canals had been interpreted as normal. However, scrutinizing
the T2-weighted images suggested signal abnormality within the
prepontine cistern and cerebellopontine angles, with the left
vestibulocochlear nerve taking a distorted path as it traversed through
the cisternal segment. Repeat imaging confirmed what was felt to
represent an epidermoid cyst in the prepontine cistern and
cerebellopontine angles encasing and displacing the left seventh and
eighth cranial nerves.

COMMENT This case illustrates several important points. The syndrome of vestibular
paroxysmia, although most commonly attributed to microvascular
compression of the eighth cranial nerve, may be associated with normal
neuroimaging or caused by other structural lesions, such as this epidermoid
cyst. The temporal profile of this case is typical, with very brief but
frequent spells. The character of vertigo is sometimes not one of spinning
but rather of a fine shimmering binocular oscillopsia from very rapid,
low-amplitude nystagmus (not unlike the spells of monocular oscillopsia
seen in superior oblique myokymia). Sometimes, sudden directional pulsion
can lead to falls. Although isolated vestibular symptoms are most common,
concurrent monaural tinnitus during spells can add localizing value. Tests
of vestibular function are generally normal in vestibular paroxysmia,
although this patient with a structural lesion had evidence of
accompanying vestibular dysfunction.
The consulting neurosurgeon estimated a 50% chance of helping her
symptoms with resection of the cyst and a 10% to 20% chance of injuring her
hearing or vestibular function. She was instead treated with
carbamazepine 300 mg 2 times daily and quickly noted complete resolution
of the spells of oscillopsia and a 10-fold reduction in the frequency of the
now-isolated tinnitus spells.

392 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

PANIC ATTACKS
Panic attacks commonly cause dizziness, unsteadiness, or lightheadedness. Such
vestibular symptoms are second only to cardiopulmonary symptoms (eg, dyspnea
or chest pain) as a manifestation of panic attacks. Vertigo occurs less commonly
and is usually less dramatic than the intense spinning of acute peripheral vestibular
disorders. Panic attacks may be identified by their characteristic features listed in
TABLE 4-8. They may occur spontaneously or be triggered by identifiable
115

fear-provoking stimuli. Although panic attacks are part of panic disorder, they
may also occur in other anxiety, trauma-related, and obsessive-compulsive

Panic Disorder Diagnostic Criteria From the Diagnostic and Statistical Manual TABLE 4-8
of Mental Disorders, Fifth Editiona

A Recurrent unexpected panic attacks. A panic attack is an abrupt surge of intense fear or
intense discomfort that reaches a peak within minutes, during which time four (or more) of
the following symptoms occur:
• Palpitations, pounding heart, or accelerated heart rate
• Sweating
• Trembling or shaking
• Sensations of shortness of breath or smothering
• Feelings of choking
• Chest pain or discomfort
• Nausea or abdominal distress
• Feeling dizzy, unsteady, light-headed, or faint
• Chills or heat sensations
• Paresthesias (numbness or tingling sensations)
• Derealization (feelings of unreality) or depersonalization (being detached from oneself)
• Fear of losing control or “going crazy”
• Fear of dying
B At least one of the attacks has been followed by 1 month (or more) of one or both of the
following:
• Persistent concern or worry about additional panic attacks or their consequences (eg,
losing control, having a heart attack, “going crazy”)
• A significant maladaptive change in behavior related to the attacks (eg, behaviors designed
to avoid having panic attacks, such as avoidance of exercise or unfamiliar situations)
C The disturbance is not attributable to the physiologic effects of a substance (eg, a drug of
abuse, a medication) or another medical condition (eg, hyperthyroidism, cardiopulmonary
disorders).
D The disturbance is not better explained by another mental disorder (eg, the panic attacks do
not occur only in response to feared social situations, as in social anxiety disorder; in
response to circumscribed phobic objects or situations, as in specific phobia; in response to
obsessions, as in obsessive-compulsive disorder; in response to reminders of traumatic
events, as in posttraumatic stress disorder; or in response to separation from attachment
figures, as in separation anxiety disorder).

a
Reprinted from the American Psychiatric Association.115 © 2013 American Psychiatric Association.

CONTINUUMJOURNAL.COM 393

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

disorders, in medical conditions such as hyperthyroidism, and during substance

intoxication or withdrawal. In late adolescence and early adulthood, panic
attacks are among the most common causes of episodic dizziness.
Patients with panic disorder are much more likely than control subjects to
have uneasiness with heights, space and motion discomfort, and constant or
fluctuating dizziness.116 The dizziness from panic attacks is not related to
underlying vestibular dysfunction, although patients with panic disorder are
more likely than controls to have one or more nonspecific, nondiagnostic
abnormalities on tests of basic vestibular reflexes that do not point toward any
consistent pattern of central or peripheral vestibular dysfunction.117 However,
patients with panic disorder do sway more than controls on static and dynamic
posturography that assesses higher-order integrated balance function, and the
degree of postural instability correlates with the severity of phobic avoidance and
anticipatory anxiety of situations associated with dizziness.118 Patients with
anxiety disorders and space and motion discomfort have more difficulty
integrating visual, vestibular, and somatosensory cues, becoming overreliant on
visual cues that are problematic in busy complex visual environments.119
Psychiatric and vestibular disorders interact in complex ways. Anxiety
disorders, including panic and phobic disorders, are estimated to be the primary
cause of vestibular symptoms in 8% to 10% of patients evaluated in specialty
neuro-otology centers120; rates of psychiatric comorbidity in patients with
structural or functional vestibular disorders are much higher still. Just like
panic attacks can manifest with dizziness, acute vestibular syndromes frequently
cause high levels of anxiety including panic attacks. High anxiety during and after
acute vestibular episodes is associated with poor long-term outcomes and is more
predictive of development of chronic dizziness than is the degree of peripheral
vestibular injury.121 Sometimes, preexisting psychiatric disorders that were not
associated with dizziness or unsteadiness increase in severity with onset of a
vestibular disorder and interact to contribute to overall morbidity.122 Psychiatric
disorders may also trigger functional vestibular disorders such as PPPD.
The most important part of the evaluation for patients with spontaneous
episodes of dizziness is a careful history, which includes listening for symptoms
or triggers that might suggest panic disorder as a cause. Clinicians should
inquire about diagnoses or previous symptoms suggesting an anxiety disorder,
particularly generalized anxiety disorder, panic disorder, or specific phobias.
Such conditions can be the cause of vestibular symptoms, the result of a
vestibular disorder, or a comorbidity that is necessary to identify and manage
simultaneously. Evaluation of suspected panic attacks may need to rule out drug
intoxication or withdrawal as well as medical conditions mimicking panic
attacks, such as arrhythmia, angina, hyperthyroidism, pheochromocytoma,
hypoglycemia, or temporal lobe epilepsy. When episodic dizziness is the
prominent symptom, other vestibular disorders must be considered and
potentially investigated. Panic disorder is typically managed with cognitive
behavioral therapy or medications (mainly selective serotonin reuptake
inhibitors [SSRIs]), or both.

DELAYED ORTHOSTATIC HYPOTENSION

Orthostatic hypotension typically presents as a triggered cause of dizziness,
defined as a drop in blood pressure of at least 20 mm Hg systolic or 10 mm Hg
diastolic when going from lying to sitting, lying to standing, or sitting to

394 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

standing. Classic orthostatic hypotension develops within 3 minutes of assuming KEY POINTS
upright posture, whereas initial (immediate) orthostatic hypotension is
● Panic attacks commonly
associated with a transient decrease in blood pressure within 15 seconds of cause dizziness,
standing. However, patients with delayed orthostatic hypotension have a gradual unsteadiness, or
fall in blood pressure that takes more than 3 minutes of upright posture to lightheadedness, but
develop.123 In this case, the relationship between the trigger (upright position) intense vertigo is
uncommon.
and dizziness may be less clear, and patients may present with what appears to be
episodic spontaneous dizziness. Of course, with orthostatic hypotension, the ● Anxiety disorders,
symptoms should resolve on sitting or lying down. including panic disorder,
Patients experience the symptom quality of orthostatic dizziness in various can be the cause of
ways. Although clinicians often expect a description of lightheadedness or vestibular symptoms, the
result of a vestibular
faintness (ie, presyncope), patients may describe the symptoms instead or disorder, or a comorbidity
additionally as vertigo, unsteadiness, generalized weakness, tiredness, difficulty that is necessary to identify
in thinking or concentrating, tremulousness, fading vision, or posterior head and manage simultaneously.
or neck discomfort, as well as experiencing tachycardia or palpitations.124,125 Psychiatric disorders may
also trigger functional
Thus, clinicians should focus on the timing after the patient assumes an upright vestibular disorders such as
posture rather than the quality of symptom to appropriately suspect and persistent postural
diagnose delayed orthostatic hypotension. perceptual dizziness.
In addition to orthostatic hypotension, the differential diagnosis for dizziness,
● Patients with delayed
vertigo, or unsteadiness with upright posture is broad and includes the following:
orthostatic hypotension
have a gradual fall in blood
u postural tachycardia syndrome (POTS) pressure that takes more
than 3 minutes of upright
u PPPD posture to develop. Thus,
u bilateral vestibulopathy the relationship between
the trigger (upright position)
u orthostatic tremor/myoclonus and dizziness may be less
u sensory neuropathy clear, and patients may
present with what appears
u other gait disorders to be episodic spontaneous
u dizziness/vertigo due to cardiac problems dizziness.

u anxiety and depressive disorders ● Patients with orthostatic

hypotension may describe
symptoms of vertigo or
Evaluation of suspected delayed orthostatic hypotension begins by querying unsteadiness rather than
for other symptoms that might suggest autonomic impairment (urinary lightheadedness or
incontinence, erectile dysfunction, anhidrosis), review of potentially causative faintness.
medications (alpha-blockers, beta-blockers, diuretics), and office measurement
● Dizziness is a prominent
of orthostatic blood pressure and heart rate with prolonged standing or while
symptom in patients with
symptomatic. The head-up tilt test can be used to further evaluate forms of bradycardia, tachycardia, or
orthostatic hypotension, although reproducibility is suboptimal.126 Additional other low cardiac output
autonomic testing may be required. states, and it is commonly
experienced as vertigo
lasting seconds to minutes.
CARDIOGENIC DIZZINESS
Syncope, along with presyncope of various qualities (dizziness, vertigo), can be
caused by several cardiac disorders. Bradycardia, tachycardia, or low cardiac
output from heart failure or outflow obstruction can all lead to dizziness from
global cerebral hypoperfusion. Dizziness is a prominent or presenting symptom
in about 10% of patients with acute myocardial infarction.127 A review of the
limited literature found that, among patients with primary cardiovascular
disorders who experience dizziness, actual vertigo is quite common (63%,
including 37% for whom vertigo was the only dizziness type).128 Paroxysmal

CONTINUUMJOURNAL.COM 395

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

arrhythmias typically cause episodic dizziness/vertigo lasting seconds to

minutes, which may be accompanied by fading vision, bilateral tinnitus or
hearing loss, palpitations, chest pain, or dyspnea and progress to syncope. Unlike
with orthostatic hypotension, episodes may occur in any position. Arrhythmias
may be suspected by the presence of an underlying cardiac disorder, medical
condition promoting arrhythmias (thyrotoxicosis, hyperkalemia), or
arrhythmogenic drugs. Evaluation is guided by a suspected cause but typically
begins with an ECG and Holter monitor.

HYPOGLYCEMIA
Acute hypoglycemia may cause dizziness or vertigo along with confusion,
anxiety, drowsiness, tremor, palpitations, sweating, and paresthesia.
Hypoglycemia occurs most commonly in patients being treated for diabetes,
particularly with insulin. Symptomatic hypoglycemia is rare in otherwise healthy
individuals, although insulinomas or other causes of endogenous
hyperinsulinism, as well as other drugs or hormone deficiencies, can produce
hypoglycemia. Evaluation first requires demonstrating low serum glucose while
symptomatic and relief of those symptoms when the serum glucose is raised.
Further evaluation seeks to identify the specific cause.

CONCLUSION
Diagnosing patients with spontaneous episodes of dizziness or vertigo does not
need to be a daunting task. Only a handful of conditions are commonly
encountered in clinical practice. Because most patients are asymptomatic when
evaluated in the office and have normal examinations, diagnosis relies heavily on
the history. More important than the vestibular symptom quality are the timing,
triggers, accompanying symptoms, and associated comorbidities. Many
disorders are diagnosed based on clinical diagnostic criteria alone. Carefully
selected tests including audiometric evaluation, neuroimaging, vestibular
laboratory testing, or cardiovascular evaluation may be helpful, but overreliance
on indiscriminate and costly investigations risks uncovering irrelevant or
diagnostically misleading abnormalities. Although most of the conditions are
treatable, the treatments are vastly different, and some, such as vertebrobasilar
TIAs or arrhythmias, are potentially deadly. Thus, it is important to strive for a
specific diagnosis to guide ongoing care.

USEFUL WEBSITE
INTERNATIONAL CLASSIFICATION OF VESTIBULAR
DISORDERS
This web page presents a collection of freely
available formal consensus definition articles by the
Bárány Society.
jvr-web.org/ICVD.html

REFERENCES

1 Drachman DA, Hart CW. An approach to the dizzy 2 Edlow JA, Gurley KL, Newman-Toker DE. A new
patient. Neurology 1972;22(4):323-334. diagnostic approach to the adult patient with
doi:10.1212/wnl.22.4.323 acute dizziness. J Emerg Med 2018;54(4):469-483.
doi:10.1016/j.jemermed.2017.12.024

396 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

3 Newman-Toker DE, Cannon LM, Stofferahn ME, 18 Eggers SD, Staab JP, Neff BA, et al. Investigation
et al. Imprecision in patient reports of dizziness of the coherence of definite and probable
symptom quality: a cross-sectional study vestibular migraine as distinct clinical entities.
conducted in an acute care setting. Mayo Clin Otol Neurotol 2011;32(7):1144-1151.
Proc 2007;82(11):1329-1340. doi:10.4065/82.11.1329 doi:10.1097/MAO.0b013e31822a1c67
4 Bisdorff A, Von Brevern M, Lempert T, Newman- 19 Drummond PD. Motion sickness and migraine:
Toker DE. Classification of vestibular symptoms: optokinetic stimulation increases scalp
towards an international classification of tenderness, pain sensitivity in the fingers and
vestibular disorders. J Vestib Res 2009;19(1-2): photophobia. Cephalalgia 2002;22(2):117-124.
1-13. doi:10.3233/VES-2009-0343 doi:10.1046/j.1468-2982.2002.00332.x
5 Episodic vestibular syndrome. International 20 King S, Wang J, Priesol AJ, Lewis RF. Central
classification of diseases 11th revision. Accessed integration of canal and otolith signals is
December 17, 2020. icd.who.int/browse11/l-m/ abnormal in vestibular migraine. Front Neurol
en#/http://id.who.int/icd/entity/1402706403 2014;5:233. doi:10.3389/fneur.2014.00233
6 Olesen J. International classification of headache 21 Winnick A, Sadeghpour S, Otero-Millan J, et al.
disorders. Lancet Neurol 2018;17(5):396-397. Errors of upright perception in patients with
doi:10.1016/S1474-4422(18)30085-1 vestibular migraine. Front Neurol 2018;9:892.
doi:10.3389/fneur.2018.00892
7 Cha YH, Lee H, Santell LS, Baloh RW. Association
of benign recurrent vertigo and migraine in 208 22 Jen J, Kim GW, Baloh RW. Clinical spectrum of
patients. Cephalalgia 2009;29(5):550. episodic ataxia type 2. Neurology 2004;62(1):
doi:10.1016/S1474-4422(18)30085-1 17-22. doi:10.1212/01.wnl.0000101675.61074.50
8 Oh AK, Lee H, Jen JC, et al. Familial benign 23 Baloh RW, Foster CA, Yue Q, Nelson SF. Familial
recurrent vertigo. Am J Med Genet 2001;100(4): migraine with vertigo and essential tremor.
287-291.doi:10.1002/ajmg.1294 Neurology 1996;46(2):458-460. doi:10.1212/
wnl.46.2.458
9 Neuhauser HK, Radtke A, von Brevern M, et al.
Migrainous vertigo: prevalence and impact on 24 Radtke A, Lempert T, Gresty MA, et al. Migraine
quality of life. Neurology 2006;67(6):1028-1033. and Ménière's disease: is there a link? Neurology
doi:10.1212/01.wnl.0000237539.09942.06 2002;59(11):1700-1704. doi:10.1212/01.
wnl.0000036903.22461.39
10 Formeister EJ, Rizk HG, Kohn MA, Sharon JD. The
epidemiology of vestibular migraine: a 25 Huang TC, Wang SJ, Kheradmand A. Vestibular
population-based survey study. Otol Neurotol migraine: an update on current understanding
2018;39(8):1037-1044. doi:10.1097/ and future directions. 2020;40(1):107-121.
MAO.0000000000001900 doi:10.1177/0333102419869317
11 Dieterich M, Brandt T. Episodic vertigo related to 26 von Brevern M, Radtke A, Clarke AH, Lempert T.
migraine (90 cases): vestibular migraine? J Neurol Migrainous vertigo presenting as episodic
1999;246(10):883-892. doi:10.1007/s004150050478 positional vertigo. Neurology 2004;62(3):469-472.
doi:10.1212/01.wnl.0000106949.55346.cd
12 Johnson GD. Medical management of
migraine-related dizziness and vertigo. 27 Brantberg K, Trees N, Baloh RW. Migraine-
Laryngoscope 1998;108(1 pt 2):1-28. associated vertigo. Acta Otolaryngol 2005;125(3):
doi:10.1097/00005537-199801001-00001 276-279. doi:10.1080/00016480510003165
13 Beh SC, Masrour S, Smith SV, Friedman DI. The 28 Zhang Y, Kong Q, Chen J, et al. International
spectrum of vestibular migraine: clinical features, Classification of Headache Disorders 3rd edition
triggers, and examination findings. Headache beta-based field testing of vestibular migraine in
2019;59(5):727-740. doi:10.1111/head.13484 China: demographic, clinical characteristics,
audiometric findings and diagnosis statues.
14 Neuhauser H, Leopold M, von Brevern M, et al.
Cephalalgia 2016;36(3):240-248.
The interrelations of migraine, vertigo, and
doi:10.1177/0333102415587704
migrainous vertigo. Neurology 2001;56(4):436-441.
29 Neff BA, Staab JP, Eggers SD, et al. Auditory and
15 Lee H, Sohn SI, Jung DK, et al. Migraine and
vestibular symptoms and chronic subjective
isolated recurrent vertigo of unknown cause.
dizziness in patients with Ménière's
Neurol Res 2002;24(7):663-665. doi:10.1179/
disease, vestibular migraine, and Ménière's
016164102101200726
disease with concomitant vestibular
16 Drummond PD. Triggers of motion sickness in migraine. Otol Neurotol 2012;33(7):1235-1244.
migraine sufferers. Headache 2005;45(6): doi:10.1097/MAO.0b013e31825d644a
653-656. doi:10.1111/j.1526-4610.2005.05132.x
30 Lempert T, Neuhauser H. Epidemiology
17 Furman JM, Marcus DA, Balaban CD. Migrainous of vertigo, migraine and vestibular
vertigo: development of a pathogenetic model migraine. J Neurol 2009;256(3):333-338.
and structured diagnostic interview. doi:10.1007/s00415-009-0149-2
Curr Opin Neurol 2003;16(1):5-13. doi:10.1097/
31 Cutrer FM, Baloh RW. Migraine-associated
01.wco.0000053582.70044.e2
dizziness. Headache 1992;32(6):300-304.
doi:10.1111/j.1526-4610.1992.hed3206300.x

CONTINUUMJOURNAL.COM 397

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

32 von Brevern M, Zeise D, Neuhauser H, et al. Acute 46 Kang WS, Lee SH, Yang CJ, et al. Vestibular function
migrainous vertigo: clinical and oculographic tests for vestibular migraine: clinical implication of
findings. Brain 2005;128(pt 2):365-374. video head impulse and caloric tests. Front
doi:10.1093/brain/awh351 Neurol 2016;7:166. doi:10.3389/fneur.2016.00166
33 Young AS, Lechner C, Bradshaw AP, et al. 47 Inoue A, Egami N, Fujimoto C, et al. Vestibular
Capturing acute vertigo: a vestibular event evoked myogenic potentials in vestibular
monitor. Neurology 2019;92(24):e2743-e2753. migraine: do they help differentiating from
doi:10.1212/WNL.0000000000007644 Ménière's disease? Ann Otol Rhinol Laryngol
2016;125(11):931-937. doi:10.1177/0003489416665192
34 Polensek SH, Tusa RJ. Nystagmus during attacks
of vestibular migraine: an aid in diagnosis. Audiol 48 Taylor RL, Zagami AS, Gibson WP, et al. Vestibular
Neurootol 2010;15(4):241-246. doi:10.1159/000255440 evoked myogenic potentials to sound and
vibration: characteristics in vestibular migraine
35 Jeong SH, Oh SY, Kim HJ, et al. Vestibular
that enable separation from Ménière's disease.
dysfunction in migraine: effects of associated
Cephalalgia 2012;32(3):213-225. doi:10.1177/
vertigo and motion sickness. J Neurol 2010;257(6):
0333102411434166
905-912. doi:10.1007/s00415-009-5435-5
49 Zuniga MG, Janky KL, Schubert MC, Carey JP. Can
36 Murdin L, Chamberlain F, Cheema S, et al. Motion
vestibular-evoked myogenic potentials help
sickness in migraine and vestibular disorders.
differentiate Ménière disease from vestibular
J Neurol Neurosurg Psychiatry 2015;86(5):
migraine? Otolaryngol Head Neck Surg 2012;
585-587. doi:10.1136/jnnp-2014-308331
146(5):788-796. doi:10.1177/0194599811434073
37 Staab JP, Eckhardt-Henn A, Horii A, et al.
50 Lopez-Escamez JA, Carey J, Chung WH, et al.
Diagnostic criteria for persistent
Diagnostic criteria for Ménière's disease. J Vestib
postural-perceptual dizziness (PPPD): consensus
Res 2015;25(1):1-7. doi:10.3233/VES-150549
document of the Committee for the
Classification of Vestibular Disorders of the 51 Lopez-Escamez JA, Dlugaiczyk J, Jacobs J, et al.
Barany Society. J Vestib Res 2017;27(4):191-208. Accompanying symptoms overlap during attacks
doi:10.3233/VES-170622 in Ménière's disease and vestibular migraine. Front
Neurol 2014;5:265. doi:10.3389/fneur.2014.00265
38 Eggers SD, Neff BA, Shepard NT, Staab JP.
Comorbidities in vestibular migraine. J Vestib Res 52 Strupp M, Lopez-Escamez JA, Kim JS, et al.
2014;24(5-6):387-395. doi:10.3233/VES-140525 Vestibular paroxysmia: diagnostic criteria.
J Vestib Res 2016;26(5-6):409-415. doi:10.3233/
39 Best C, Eckhardt-Henn A, Tschan R, Dieterich M.
VES-160589
Psychiatric morbidity and comorbidity in
different vestibular vertigo syndromes. 53 Headache Classification Committee of the
Results of a prospective longitudinal study International Headache Society (IHS). The
over one year. J Neurol 2009;256(1):58-65. International Classification of Headache
doi:10.1007/s00415-009-0038-8 Disorders, 3rd edition (beta version). Cephalalgia
2013;33(9):629-808. doi:10.1177/0333102413485658
40 Best C, Tschan R, Eckhardt-Henn A, Dieterich M.
Who is at risk for ongoing dizziness and 54 Lempert T, Olesen J, Furman J, et al. Vestibular
psychological strain after a vestibular disorder? migraine: diagnostic criteria. J Vestib Res 2012;
Neuroscience 2009;164(4):1579-1587. 22(4):167-172. doi:10.3233/VES-2012-0453
doi:10.1016/j.neuroscience.2009.09.034
55 Radtke A, Neuhauser H, von Brevern M, et al.
41 Boldingh MI, Ljostad U, Mygland A, Monstad P. Vestibular migraine–validity of clinical diagnostic
Comparison of interictal vestibular function in criteria. Cephalalgia 2011;31(8):906-913.
vestibular migraine vs migraine without vertigo. doi:10.1177/0333102411405228
Headache 2013;53(7):1123-1133. doi:10.1111/head.12129
56 Neuhauser H, Radtke A, von Brevern M, Lempert
42 Casani AP, Sellari-Franceschini S, Napolitano A, T. Zolmitriptan for treatment of migrainous
et al. Otoneurologic dysfunctions in migraine vertigo: a pilot randomized placebo-controlled
patients with or without vertigo. Otol Neurotol 2009; trial. Neurology 2003;60(5):882-883. doi:10.1212/
30(7):961-967. doi:10.1097/MAO.0b013e3181b4e780 01.wnl.0000049476.40047.a3
43 Cass SP, Furman JM, Ankerstjerne K, et al. 57 Furman JM, Marcus DA, Balaban CD. Rizatriptan
Migraine-related vestibulopathy. Ann Otol reduces vestibular-induced motion sickness in
Rhinol Laryngol 1997;106(3):182-189. migraineurs. J Headache Pain 2011;12(1):81-88.
doi:10.1177/000348949710600302 doi:10.1007/s10194-010-0250-z
44 Radtke A, von Brevern M, Neuhauser H, et al. 58 Beh SC. External trigeminal nerve stimulation:
Vestibular migraine: long-term follow-up of potential rescue treatment for acute vestibular
clinical symptoms and vestibulo-cochlear migraine. J Neurol Sci 2019;408:116550.
findings. Neurology 2012;79(15):1607-1614. doi:10.1016/j.jns.2019.116550
doi:10.1212/WNL.0b013e31826e264f
59 Beh SC, Friedman DI. Acute vestibular migraine
45 Teggi R, Colombo B, Bernasconi L, et al. treatment with noninvasive vagus nerve
Migrainous vertigo: results of caloric testing and stimulation. Neurology 2019;93(18):e1715-e1719.
stabilometric findings. Headache 2009;49(3): doi:10.1212/WNL.0000000000008388
435-444. doi:10.1111/j.1526-4610.2009.01338.x

398 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

60 Lepcha A, Amalanathan S, Augustine AM, et al. 74 Thompson KJ, Goetting JC, Staab JP, Shepard NT.
Flunarizine in the prophylaxis of migrainous Retrospective review and telephone follow-up
vertigo: a randomized controlled trial. Eur Arch to evaluate a physical therapy protocol for
Otorhinolaryngol 2014;271(11):2931-2936. treating persistent postural-perceptual
doi:10.1007/s00405-013-2786-4 dizziness: a pilot study. J Vestib Res 2015;25(2):
97-103; quiz 103-104. doi:10.3233/VES-150551
61 Salviz M, Yuce T, Acar H, et al. Propranolol and
venlafaxine for vestibular migraine prophylaxis: a 75 Rauch SD, Merchant SN, Thedinger BA. Ménière's
randomized controlled trial. Laryngoscope 2016; syndrome and endolymphatic hydrops.
126(1):169-174. doi:10.1002/lary.25445 Double-blind temporal bone study. Ann Otol
Rhinol Laryngol 1989;98(11):873-883.
62 Liu F, Ma T, Che X, et al. The efficacy of
doi:10.1177/000348948909801108
venlafaxine, flunarizine, and valproic acid in the
prophylaxis of vestibular migraine. Front Neurol 76 Ishiyama G. Selected otologic disorders causing
2017;8:524. doi:10.3389/fneur.2017.00524 dizziness. Continuum (Minneap Minn) 2021;
27(2, Neuro-otology):468–490.
63 Bayer O, Adrion C, Al Tawil A, et al. Results and
lessons learnt from a randomized controlled trial: 77 Fife TD, Tourkevich R. Tinnitus, hyperacusis,
prophylactic treatment of vestibular migraine otalgia, and hearing loss. Continuum (Minneap
with metoprolol (PROVEMIG). Trials 2019;20(1):813. Minn) 2021;27(2, Neuro-otology):491–525.
doi:10.1186/s13063-019-3903-5
78 Harris JP, Alexander TH. Current-day prevalence
64 Bikhazi P, Jackson C, Ruckenstein MJ. Efficacy of of Ménière's syndrome. Audiol Neurootol 2010;
antimigrainous therapy in the treatment of 15(5):318-322. doi:10.1159/000286213
migraine-associated dizziness. Am J Otol 1997;
79 Perez-Garrigues H, Lopez-Escamez JA, Perez P,
18(3):350-354.
et al. Time course of episodes of definitive
65 Bisdorff AR. Treatment of migraine related vertigo in Ménière's disease. Arch Otolaryngol
vertigo with lamotrigine an observational study. Head Neck Surg 2008;134(11):1149-1154.
Bull Soc Sci Med Grand Duche Luxemb 2004; doi:10.1001/archotol.134.11.1149
2(2):103-108.
80 Eggers SDZ, Bisdorff A, von Brevern M, et al.
66 Carmona S, Settecase N. Use of topiramate Classification of vestibular signs and examination
(topamax) in a subgroup of migraine-vertigo techniques: nystagmus and nystagmus-like
patients with auditory symptoms. Ann N Y Acad movements. J Vestib Res 2019;29(2-3):57-87.
Sci 2005;1039:517-520. doi:10.1196/annals.1325.057 doi:10.3233/VES-190658
67 Iwasaki S, Ushio M, Chihara Y, et al. Migraine- 81 Huppert D, Strupp M, Brandt T. Long-term
associated vertigo: clinical characteristics of course of Ménière's disease revisited. Acta
Japanese patients and effect of lomerizine, a Otolaryngol 2010;130(6):644-651.
calcium channel antagonist. Acta Otolaryngol doi:10.3109/00016480903382808
Suppl 2007(559):45-49. doi:10.1080/
82 Belinchon A, Perez-Garrigues H, Tenias JM,
03655230701596491
Lopez A. Hearing assessment in Ménière's
68 Maione A. Migraine-related vertigo: diagnostic disease. Laryngoscope 2011;121(3):622-626.
criteria and prophylactic treatment. doi:10.1002/lary.21335
Laryngoscope 2006;116(10):1782-1786.
83 Baloh RW, Jacobson K, Winder T. Drop attacks
doi:10.1097/01.mlg.0000231302.77922.c5
with Ménière's syndrome. Ann Neurol 1990;
69 Waterston J. Chronic migrainous vertigo. 28(3):384-387. doi:10.1002/ana.410280314
J Clin Neurosci 2004;11(4):384-388.
84 Ishiyama G, Ishiyama A, Baloh RW. Drop attacks
doi:10.1016/j.jocn.2003.08.008
and vertigo secondary to a non-Ménière otologic
70 Brodsky JR, Cusick BA, Zhou G. Evaluation and cause. Arch Neurol 2003;60(1):71-75.
management of vestibular migraine in children: doi:10.1001/archneur.60.1.71
experience from a pediatric vestibular clinic.
85 Goebel JA. 2015 Equilibrium Committee
Eur J Paediatr Neurol 2016;20(1):85-92.
Amendment to the 1995 AAO-HNS Guidelines for
doi:10.1016/j.ejpn.2015.09.011
the Definition of Ménière's Disease. Otolaryngol
71 Gode S, Celebisoy N, Kirazli T, et al. Clinical Head Neck Surg 2016;154(3):403-404.
assessment of topiramate therapy in patients doi:10.1177/0194599816628524
with migrainous vertigo. Headache 2010;50(1):
86 Lopez-Escamez JA, Attyé A. Systematic review
77-84. doi:10.1111/j.1526-4610.2009.01496.x
of magnetic resonance imaging for diagnosis of
72 Taghdiri F, Togha M, Razeghi Jahromi S, Refaeian Ménière disease. J Vestib Res 2019;29(2-3):
F. Cinnarizine for the prophylaxis of migraine 121-129. doi:10.3233/VES-180646
associated vertigo: a retrospective study.
87 McCaslin DL, Rivas A, Jacobson GP, Bennett ML.
Springerplus 2014;3:231. doi:10.1186/2193-1801-3-231
The dissociation of video head impulse test
73 Alghadir AH, Anwer S. Effects of vestibular (vHIT) and bithermal caloric test results provide
rehabilitation in the management of a vestibular topological localization of vestibular system
migraine: a review. Front Neurol 2018;9:440. impairment in patients with "definite" Ménière's
doi:10.3389/fneur.2018.00440 disease. Am J Audiol 2015;24(1):1-10.
doi:10.1044/2014_AJA-14-0040

CONTINUUMJOURNAL.COM 399

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EPISODIC SPONTANEOUS DIZZINESS

88 McGarvie LA, Curthoys IS, MacDougall HG, 101 Lee H, Cho YW. Auditory disturbance as a
Halmagyi GM. What does the dissociation prodrome of anterior inferior cerebellar artery
between the results of video head impulse infarction. J Neurol Neurosurg Psychiatry 2003;
versus caloric testing reveal about the vestibular 74(12):1644-1648. doi:10.1136/jnnp.74.12.1644
dysfunction in Ménière's disease? Acta
102 Park JH, Kim H, Han HJ. Recurrent
Otolaryngol 2015;135(9):859-865.
audiovestibular disturbance initially mimicking
doi:10.3109/00016489.2015.1015606
Ménière's disease in a patient with anterior
89 Hannigan IP, Welgampola MS, Watson SRD. inferior cerebellar infarction. Neurol Sci 2008;
Dissociation of caloric and head impulse tests: 29(5):359-362. doi:10.1007/s10072-008-0996-0
a marker of Ménière's disease. J Neurol 2019.
103 Newman-Toker DE, Kerber KA, Hsieh YH, et al.
doi:10.1007/s00415-019-09431-9
HINTS outperforms ABCD2 to screen for stroke
90 Taylor RL, Wijewardene AA, Gibson WPR, et al. in acute continuous vertigo and dizziness.
The vestibular evoked-potential profile of Acad Emerg Med 2013;20(10):986-996.
Ménière's disease. Clin Neurophysiol 2011;122(6): doi:10.1111/acem.12223
1256-1263. doi:10.1016/j.clinph.2010.11.009
104 Gottesman RF, Sharma P, Robinson KA, et al.
91 Noij KS, Herrmann BS, Guinan JJ Jr, Rauch SD. Clinical characteristics of symptomatic
Cervical vestibular evoked myogenic potentials vertebral artery dissection: a systematic review.
in Ménière's disease: a comparison of response Neurologist 2012;18(5):245-254.
metrics. Otol Neurotol 2019;40(3):e215-e224. doi:10.1097/NRL.0b013e31826754e1
doi:10.1097/MAO.0000000000002092
105 Choi KD, Kim JS. Vascular vertigo: updates.
92 Rosengren SM, Colebatch JG, Young AS, et al. J Neurol 2019;266(8):1835-1843.
Vestibular evoked myogenic potentials in doi:10.1007/s00415-018-9040-3
practice: methods, pitfalls and clinical
106 Jannetta PJ. Neurovascular cross-compression
applications. Clin Neurophysiol Pract 2019;
in patients with hyperactive dysfunction
4:47-68. doi:10.1016/j.cnp.2019.01.005
symptoms of the eighth cranial nerve. Surg
93 Saber Tehrani AS, Kattah JC, Kerber KA, et al. Forum 1975;26:467-469.
Diagnosing stroke in acute dizziness and vertigo:
107 Ward BK, Gold DR. Tinnitus, oscillopsia, and
pitfalls and pearls. Stroke 2018;49(3):788-795.
hyperventilation-induced nystagmus: vestibular
doi:10.1161/STROKEAHA.117.016979
paroxysmia. Open J Clin Med Case Rep 2016;
94 Choi KD, Choi JH, Kim JS, et al. Rotational 2(7):1100.
vertebral artery occlusion: mechanisms and
108 Best C, Gawehn J, Krämer HH, et al. MRI and
long-term outcome. Stroke 2013;44(7):1817-1824.
neurophysiology in vestibular paroxysmia:
doi:10.1161/STROKEAHA.113.001219
contradiction and correlation. J Neurol
95 Paul NL, Simoni M, Rothwell PM, Oxford Vascular Neurosurg Psychiatry 2013;84(12):1349-1356. doi:
Study. Transient isolated brainstem symptoms 10.1136/jnnp-2013-305513
preceding posterior circulation stroke: a
109 Hüfner K, Barresi D, Glaser M, et al. Vestibular
population-based study. Lancet Neurol 2013;
paroxysmia: diagnostic features and medical
12(1):65-71. doi:10.1016/S1474-4422(12)70299-5
treatment. Neurology 2008;71(13):1006-1014. doi:
96 Choi JH, Park MG, Choi SY, et al. Acute transient 10.1212/01.wnl.0000326594.91291.f8
vestibular syndrome: prevalence of stroke and
110 Bayer O, Brémová T, Strupp M, Hüfner K. A
efficacy of bedside evaluation. Stroke 2017;48(3):
randomized double-blind, placebo-controlled,
556-562. doi:10.1161/STROKEAHA.116.015507
cross-over trial (Vestparoxy) of the treatment of
97 Atzema CL, Grewal K, Lu H, et al. Outcomes vestibular paroxysmia with oxcarbazepine.
among patients discharged from the emergency J Neurol 2018;265(2):291-298. doi:10.1007/
department with a diagnosis of peripheral vertigo. s00415-017-8682-x
Ann Neurol 2016;79(1):32-41. doi:10.1002/ana.24521
111 Strupp M, Elger C, Goldschagg N. Treatment of
98 Lee CC, Su YC, Ho HC, et al. Risk of stroke in vestibular paroxysmia with lacosamide. Neurol
patients hospitalized for isolated vertigo: a Clin Pract 2019;9(6):539-541. doi:10.1212/
four-year follow-up study. Stroke 2011;42(1): CPJ.0000000000000610
48-52. doi:10.1161/STROKEAHA.110.597070
112 Slater R. Benign recurrent vertigo. J Neurol
99 Gomez CR, Cruz-Flores S, Malkoff MD, et al. Neurosurg Psychiatry 1979;42(4):363-367.
Isolated vertigo as a manifestation of doi:10.1136/jnnp.42.4.363
vertebrobasilar ischemia. Neurology 1996;47(1):
113 Pan Q, Zhang Y, Zhang S, et al. Clinical features
94-97. doi:10.1212/wnl.47.1.94
and outcomes of benign recurrent vertigo: a
100 Chang TP, Wang Z, Winnick AA, et al. Sudden longitudinal study. Acta Neurol Scand 2020;
hearing loss with vertigo portends greater stroke 141(5):374-379. doi:10.1111/ane.13214
risk than sudden hearing loss or vertigo alone.
114 Lee SU, Choi JY, Kim HJ, Kim JS. Recurrent
J Stroke Cerebrovasc Dis 2018;27(2):472-478.
spontaneous vertigo with interictal headshaking
doi:10.1016/j.jstrokecerebrovasdis.2017.09.033
nystagmus. Neurology 2018;90(24):e2135-e2145.
doi:10.1212/WNL.0000000000005689

400 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

115 American Psychiatric Association. Diagnostic 122 Staab JP, Ruckenstein MJ. Which comes first?
and statistical manual of mental disorders, fifth Psychogenic dizziness versus otogenic anxiety.
edition (DSM-5). Washington, DC: American Laryngoscope 2003;113(10):1714-1718.
Psychiatric Association, 2013:947. doi:10.1097/00005537-200310000-00010
116 Jacob RG, Redfern MS, Furman JM. Space and 123 Gibbons CH, Freeman R. Delayed orthostatic
motion discomfort and abnormal balance hypotension: a frequent cause of orthostatic
control in patients with anxiety disorders. intolerance. Neurology 2006;67(1):28-32.
J Neurol Neurosurg Psychiatry 2009;80(1):74-78. doi:10.1212/01.wnl.0000223828.28215.0b
doi:10.1136/jnnp.2007.136432
124 Kim HA, Yi HA, Lee H. Recent advances in
117 Tecer A, Tukel R, Erdamar B, Sunay T. orthostatic hypotension presenting orthostatic
Audiovestibular functioning in patients with dizziness or vertigo. Neurol Sci 2015;36(11):
panic disorder. J Psychosom Res 2004;57(2): 1995-2002. doi:10.1007/s10072-015-2363-2
177-182. doi:10.1016/s0022-3999(03)00568-3
125 Low PA, Opfer-Gehrking TL, McPhee BR, et al.
118 Perna G, Dario A, Caldirola D, et al. Panic Prospective evaluation of clinical characteristics
disorder: the role of the balance system. of orthostatic hypotension. Mayo Clin Proc 1995;
J Psychiatr Res 2001;35(5):279-286. 70(7):617-622. doi:10.4065/70.7.617
doi:10.1016/s0022-3956(01)00031-0
126 Ward C, Kenny RA. Reproducibility of orthostatic
119 Redfern MS, Furman JM, Jacob RG. Visually hypotension in symptomatic elderly. Am J Med
induced postural sway in anxiety disorders. 1996;100(4):418-422. doi:10.1016/S0002-9343(97)
J Anxiety Disord 2007;21(5):704-716. 89517-4
doi:10.1016/j.janxdis.2006.09.002
127 Culić V, Mirić D, Eterović D. Correlation between
120 Staab JP, Ruckenstein MJ. Expanding the symptomatology and site of acute myocardial
differential diagnosis of chronic dizziness. Arch infarction. Int J Cardiol 2001;77(2-3):163-168.
Otolaryngol Head Neck Surg 2007;133(2):170-176. doi:10.1016/s0167-5273(00)00414-9
doi:10.1001/archotol.133.2.170
128 Newman-Toker DE, Dy FJ, Stanton VA, et al. How
121 Godemann F, Siefert K, Hantschke-Brüggemann often is dizziness from primary cardiovascular
M, et al. What accounts for vertigo one year disease true vertigo? A systematic review.
after neuritis vestibularis - anxiety or a J Gen Intern Med 2008;23(12):2087-2094.
dysfunctional vestibular organ? J Psychiatr Res doi:10.1007/s11606-008-0801-z
2005;39(5):529-234. doi:10.1016/j.
jpsychires.2004.12.006

CONTINUUMJOURNAL.COM 401

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 REVIEW ARTICLE


Selected Otologic
CONTINUUM AUDIO
INTERVIEW AVAILABLE
ONLINE
Disorders Causing
Dizziness
Downloaded from http://journals.lww.com/continuum by wqLe/H3/oM2vx1Qs5A5qMemKKHLPqE4qkmGNpdheG3ikf1o85ttJSkFL2oTkgOkhMEmawHvsuTTE5xJG2ZjqccytIAhVn2qegy060KLcKJuPZsx5htaYiopRjp2zor2egUzWJAEWYNrnNdb1tgu/p0M6EMGzm2Aj on 04/28/2022

By Gail Ishiyama, MD

ABSTRACT
PURPOSE OF REVIEW: This article details updated clinical presentations and
current treatment paradigms of the common otologic disorders that may
present to the neurologist for vertigo, including Ménière disease, superior
semicircular canal dehiscence syndrome, perilymphatic fistula, barotrauma,
cholesteatoma, Ramsay Hunt syndrome, enlarged vestibular aqueduct
syndrome, and autoimmune inner ear disease including Cogan syndrome.

RECENT FINDINGS: The recent data on modern imaging techniques with

three-dimensional delayed IV contrast in Ménière disease, findings on the
clinical and testing parameters to diagnose semicircular canal dehiscence
and barotrauma, and clinical findings in Ramsay Hunt syndrome,
cholesteatoma, and enlarged vestibular aqueduct syndrome are discussed
in the article. The most recent findings on the treatment and evaluation of
CITE AS:
autoimmune inner ear disease and Cogan syndrome are also covered.
CONTINUUM (MINNEAP MINN)
2021;27(2, NEURO-OTOLOGY): SUMMARY: This article discusses the common clinical otologic entities in
468–490.
patients who may present to the neurologist for vertigo, and it can be used
Address correspondence to as a guide in the diagnosis of these conditions with the use of auditory,
Dr Gail Ishiyama, Reed vestibular, and imaging results.
Neurological Research Center,
UCLA Department of Neurology,
Division of Neurotology,
710 Westwood Blvd, Box 951769, INTRODUCTION
Los Angeles, CA 90095,

T
[email protected]. his article presents several otologic diseases in patients who may
present to the neurologist for vertigo and dizziness. Within each
RELATIONSHIP DISCLOSURE:
Dr Ishiyama has received
diagnosis, key clinical issues may serve as red flags that should alert
research/grant support from the neurologist to which diagnoses require immediate treatment, as
the National Institutes of well as the clinical presentation and current diagnostic criteria.
Health/National Institute on
Deafness and Other
Communication Disorders PATHOPHYSIOLOGY OF MÉNIÈRE DISEASE
(10-001449). This section focuses on the pathophysiology of Ménière disease; for more
UNLABELED USE OF information about its clinical features, refer to the article “Episodic Spontaneous
PRODUCTS/INVESTIGATIONAL Dizziness” by Scott D. Z. Eggers, MD,1 and for more information about the
USE DISCLOSURE:
treatment of Ménière disease, refer to the article “Tinnitus, Hyperacusis, Otalgia,
Dr Ishiyama reports no
disclosure. and Hearing Loss” by Terry D. Fife, MD, FAAN, FANS, and Roksolyana
Tourkevich, MD,2 both of which are in this issue of Continuum.
© 2021 American Academy
Evidence of the pathophysiology of Ménière disease was first noted in archival
of Neurology. human temporal bones, which nearly universally revealed endolymphatic

468 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

hydrops, a ballooning of the endolymphatic space. The ballooning of the KEY POINT
endolymphatic space of the saccule in an archival human temporal bone of a
● Ménière disease may be
patient with a history of Ménière disease can be seen in FIGURE 8-1; the cochlear caused by oxidative damage
duct and utricle were also dilated. Of note, isolated saccular hydrops has been of the microvasculature
recently demonstrated to be associated with the full spectrum of Ménière disease.3 resulting in degeneration of
Clues to the pathologic mechanisms of Ménière disease can be found in MRI the blood-labyrinthine
barrier.
studies developed since 2007. High-resolution three-dimensional 4-hour delayed
IV contrast MRI has been developed to visualize the histopathologic hallmark of
Ménière disease: endolymphatic hydrops; the evaluation of hydrops is based on the
fact that gadolinium is taken up in the perilymph but not in the endolymph.
An incidental finding first noted by Tagaya and colleagues4 was that, in a
patient with Ménière disease, the ipsilateral affected ear compared with the
contralateral unaffected ear has an increased uptake of gadolinium, indicative of
a breach of the blood-labyrinthine barrier in Ménière disease. It was unclear
whether this was a finding specific to Ménière disease because this was noted in
several different otologic diseases. However, comparisons between patients with
unilateral Ménière disease and patients with unilateral sudden sensorineural
hearing loss show that a significantly greater degree of gadolinium uptake occurs
in the ipsilateral Ménière disease–affected inner ear.5 The ipsilateral increased
gadolinium uptake in the left inner ear in Ménière disease can be seen in
FIGURE 8-2. Histopathologic studies confirmed that on electron microscopy the
ultrastructural pathology of the vestibular end organ from patients with
intractable Ménière disease displays severe degenerative changes in the
microvasculature: vacuolization in the endothelial cells, excessive transcytosis
and vesicles, pericyte migration, and thickening and disorganization of the
overlying basement membrane.6 In 2018, Ishiyama and colleagues7
demonstrated the overexpression of inducible nitric oxide synthase and the
presence of nitrotyrosine in the neuroepithelium and within the
microvasculature, indicating that oxidative stress likely mediates the damage to
the vascular endothelium, as well as the sensory neuroepithelium. Further
studies reveal widespread structural damage to the microvasculature, indicating
that a possible pathophysiology of Ménière disease is oxidative damage to the
microvasculature of the blood-labyrinthine barrier causing neurodegenerative
changes.8

FIGURE 8-1
Archival celloidin-embedded human temporal bone demonstrating saccular hydrops in
unilateral Ménière disease. A, Normal endolymphatic space in the saccule. B, “Ballooning
out” of the endolymphatic space, causing distension of the membranous labyrinth.

CONTINUUMJOURNAL.COM 469

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

KEY POINTS On a clinical note, some

patients with Ménière disease
● Tumarkin attacks occur in
some patients with Ménière
experience sudden falls, called
disease and are important to Tumarkin falls (also known as
recognize because the falls vestibular drop attacks or otolithic
are unpredictable and may crises) (CASE 8-1). These drop
lead to serious injury and are
attacks are sudden violent falls
nearly always an indication
for ablative treatment. without loss of consciousness
described subjectively as if being
● The normal acoustic pushed by an external force.9
reflex helps distinguish the A video (edhub.ama-assn.org/
FIGURE 8-2
conductive hearing loss of
superior semicircular canal
High-resolution delayed IV contrast axial MRI jn-learning/video-player/
through the labyrinth in a case of left-sided 18471263) of a Tumarkin fall
dehiscence from that of
Ménière disease (endolymphatic hydrops). The
otosclerosis, which is
delayed contrast T2 fluid-attenuated inversion captured at a patient’s workplace
associated with an absent security camera can be seen in an
recovery (FLAIR) demonstrates significantly
acoustic reflex.
greater contrast enhancement within the article by Chen and colleagues.10
membranous labyrinth on the left side (arrow) It is important to recognize the
● The demonstration of a
compared with the right. This indicates
thinning or dehiscence of
breakdown of the blood-labyrinthine barrier.
cause of these falls because of the
the superior semicircular unpredictable and forceful
This patient had unilateral Ménière disease on the
canal on CT of the temporal
left associated with spells of vertigo, left-sided nature and the fact that many
bone does not necessarily
aural fullness, and fluctuating hearing loss. Tumarkin falls are associated
indicate the presence of
superior semicircular canal with trauma. Thus, these sudden
dehiscence syndrome. falls are nearly always an
indication for ablative treatment if localized to the inner ear vestibular system.
● All cases of superior
semicircular canal These spells are also called otolithic attacks, and they are believed to be caused by a
dehiscence should have sudden stimulation of the vestibulospinal reflex generated from the otolithic
radiographic evidence, but organs: the utricle and saccule. Indeed, pathologic evidence exists for disruption
CT alone overestimates the of the otolithic membrane in Tumarkin falls (FIGURE 8-3).11 Ablative surgery is
diagnosis by 6-fold to
20-fold. Patients diagnosed
curative of these dangerous falls and the vertigo spells. In a study of patients
with superior semicircular older than 65 years of age who underwent vestibular ablation for dangerous
canal dehiscence should Tumarkin falls, these patients derived benefit and were able to compensate well
meet criteria based on after vestibular ablation.12
clinical presentation and
audiologic and vestibular
A second clinical point is the common dissociation of the head impulse test13
testing. (normal in Ménière disease) and the vestibular caloric response (often
abnormally diminished on the ipsilateral side).
● The absence of a fistula
sign at bedside testing does
SUPERIOR SEMICIRCULAR CANAL DEHISCENCE
not rule out the diagnosis of
a perilymphatic fistula. Superior semicircular canal dehiscence was first described by Minor and
colleagues14 in 1998. Patients with superior semicircular canal dehiscence report
vestibular hypersensitivity, including vertiginous symptoms in the setting of a
loud noise (Tullio phenomenon) and pressure sensitivity with Valsalva
maneuvers (eg, coughing, sneezing, straining, lifting heavy objects) or with
positive pressure placed over the external ear (the Hennebert sign). Conductive
hyperacusis can manifest with hearing one’s voice in the affected ear (autophony),
hearing eye movements or stomach sounds, or pulsatile tinnitus. Careful neuro-
otologic assessment may reveal the slow phase of the nystagmus directed upward
and away from the affected ear when stimulated by pneumatic otoscopy, a
Valsalva maneuver against the pinched nostrils, or loud sound to the affected ear.
However, this sign is not noted in all patients, and the induced nystagmus is not
always in the plane of the superior semicircular canal or the affected canal.

470 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 A 57-year-old woman CASE 8-1
presented with recurrent
spells of rotational vertigo
with intractable nausea and
vomiting, associated with
tinnitus on the right side,
and fluctuating hearing loss.
Her most recent audiogram
demonstrated nonserviceable
hearing with 75-dB hearing
loss on the right. However,
she still had spells with
increased tinnitus to a loud
roar, followed by spinning
vertigo and vomiting. Recently,
she began having sudden
violent falls, always to the
right side, and the last fall
caused a dislocated shoulder;
thus, she was referred to a FIGURE 8-3
neurologist for urgent Representative intraoperative images in three
evaluation. different patients with Tumarkin crises and
intractable unilateral Ménière disease about to
Videonystagmography undergo labyrinthectomy that demonstrate
demonstrated 43% reduced disintegration of the utricle. A, Right ear of patient
vestibular response on the right 1 showing disintegration (small arrow) of the
and a normal head impulse test utricular membrane (large arrow). B, High-
magnification view of panel A, also showing freely
bilaterally. High-resolution
mobile otoconia (arrows). C, Left ear of patient 2
three-dimensional 4-hour demonstrating more severe disintegration (small
delayed IV contrast MRI arrow) of the otolithic membrane (large arrow). D,
demonstrated endolymphatic High-magnification view of panel C, also showing
aggregates of displaced otoconia (arrows). E,
hydrops of the utricle, saccule,
Right ear of patient 3 showing atrophy of the
and cochlear duct and utricular membrane (arrow). F, High-magnification
hyperintensity on gadolinium view of panel E revealing completely absent
uptake on the right side. She otoconia and otoconial membrane (arrow points
underwent labyrinthectomy, to the area of the utricle). The asterisk in panels A
and E indicates the superior semicircular canal.
and otolithic debris was noted A = anterior; I = inferior; P = posterior; S = superior.
within the vestibule (FIGURE 8-3). Reprinted with permission from Calzada AP, et al, Otol
On follow-up 5 years after Neurotol.11 © 2012 Otology & Neurotology, Inc.

surgery, she had no further

spells of drop attacks or
vertigo.

This is a common clinical picture of Ménière disease affecting the right ear COMMENT
and points out how specialized MRI procedures are emerging as helpful
adjuncts to diagnosis and to identifying the side that is causing the vertigo
and vestibular attacks.

CONTINUUMJOURNAL.COM 471

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

The sensitivity of the auditory and vestibular system to loud noise and pressure
is hypothesized to occur due to the presence of a “third window.” In normal
inner ear anatomy, two windows allow for the sound pressure waves: the oval
window and the round window. The osseous covering of the superior
semicircular canal is most often the area of bony defect, which allows for a “third
window.” In the normal inner ear, sound waves from the ossicular movement on
the oval window pass through the incompressible perilymph within the scala
vestibuli and scala tympani and produce an outward movement of the round
window. In superior semicircular canal dehiscence, the sound waves create
pressure that is shunted to the vestibular system, resulting in hypersensitivity
and vertigo. This shunting effect is often associated with bone conduction
hyperacusis confirmed by bone conduction hearing thresholds that are less than
0 dB, autophony, and pulsatile tinnitus.15
Workup should include vestibular-evoked myogenic potentials and temporal
bone CT with a slice thickness of less than 1 mm (ideally 0.625 mm or less)
reformatted in the planes of the superior semicircular canal (Pöschl view) and
orthogonal to it (Stenvers view).
A special note regarding cervical-vestibular evoked myogenic potentials:
threshold values distinguish patients with superior semicircular canal dehiscence
from controls with a sensitivity of 85% to 91% and a specificity of 90% to 96%. In
a retrospective cohort of 65 patients, at 500-Hz tone bursts, vestibular-evoked
myogenic potential thresholds were 66 dB for ears with superior semicircular
canal dehiscence and 85 dB for ears without superior semicircular canal
dehiscence. Thus, using a threshold of 65 dB or less had 91.4% sensitivity and
95.8% specificity.16 Using the corrected cervical vestibular-evoked myogenic
potential amplitudes with a cutoff at greater than 2.5 standard deviations

CASE 8-2 A 43-year-old woman presented with pulsatile tinnitus, vertigo induced
with loud sounds in the right ear, and the perception of hearing her voice
in her right ear. Coughing, sneezing, and straining were associated with a
brief sense of tilting and vertiginous sensation. She also reported a
sensation of right aural fullness but did not note drops in hearing and did
not describe hearing loss.
At bedside testing, the struck 512-Hz tuning fork on the forehead (or
even on the ankle) was heard in her right ear. On audiologic testing, she
had low-frequency conductive hearing loss on the right side, and
vestibular-evoked myogenic potentials revealed a threshold for
activation on the right of 65 dB compared with 95 dB on the left and an
amplitude twice that of the left. The acoustic reflexes were normal
bilaterally. CT of the temporal bone revealed thinning of the bone
overlying the superior semicircular canal on the right. After undergoing
the transmastoid approach to patch the dehiscence, she no longer
experienced the vertigo attacks with a Valsalva maneuver.

COMMENT This case demonstrates the key clinical findings in superior semicircular
canal dehiscence and meets the diagnostic criteria.15,20

472 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

from normal distinguished patients with superior semicircular canal KEY POINTS
dehiscence from controls with a sensitivity of 100% and specificity of 93%.17
● In trauma associated with
Fife and colleagues18 concluded that cervical vestibular-evoked myogenic hearing loss and/or vertigo,
potential threshold values and cervical vestibular-evoked myogenic potential the CT should be evaluated
amplitudes may be used to distinguish patients with superior semicircular canal in the coronal view for air
dehiscence. bubbles (pneumolabyrinth),
which is evidence of a
Workup should include pure tone audiometry, with the audiometer calibrated
traumatic perilymphatic
to test for bone conduction below the 0-dB hearing level. Bone conduction fistula. Pneumolabyrinth,
thresholds with negative decibel values are indicative of a conductive ossicular fracture or
hyperacusis, consistent with semicircular canal dehiscence. On pure tone dislocation, or a temporal
bone fracture through the
audiometry, an air-bone gap with bone hearing better than air hearing may be
otic capsule may be
seen at 250 Hz, 500 Hz, and 1000 Hz in superior semicircular canal dehiscence. indications for urgent
Acoustic reflexes should be tested because an absent acoustic reflex would be surgical exploration to
consistent with otosclerosis as the cause of a conductive hearing loss. preserve inner ear function.
Most cases of canal dehiscence or “third window phenomena” are due to
● Clinicians should have a
dehiscence affecting the superior semicircular canal, but other locations of high level of suspicion in
dehiscence may also occur and present with similar clinical findings except that children presenting with
the temporal bone CT shows a different location of the dehiscence. The posterior hearing loss and should rule
canal may also exhibit bony dehiscence and is associated with a high-riding out perilymphatic fistula in
the setting of inner ear
jugular bulb and fibrous dysplasia.19 Fife and colleagues20 proposed an algorithm anomaly as etiology.
that can be applied for the evaluation of patients presenting with signs or
symptoms of superior semicircular canal dehiscence syndrome, such as ● Mild symptoms
autophony, Tullio phenomenon, Valsalva maneuver–induced vertigo, or consistent with
perilymphatic fistula may be
conductive hyperacusis (CASE 8-2).
treated conservatively with
A review of the literature demonstrates four surgical approaches: middle fossa avoidance of a Valsalva
craniotomy, transmastoid, endoscopic approach, and transcanal with reinforcement maneuver or with rest.
of the round or oval window21; the 2019 multicenter study demonstrated that However, conservative
the transmastoid approach for correction of superior semicircular canal dehiscence treatment is not
recommended for traumatic
was associated with a significantly shorter duration of hospitalization and lower perilymphatic fistula
recurrence rate compared with a middle fossa craniotomy. Patients in both groups secondary to penetrating
had improvement in the Tullio phenomenon, autophony, and pulsatile tinnitus, inner ear injury, temporal
and no difference was seen in audiometric outcomes between the transmastoid bone fracture, or ossicular
damage.
approach and the traditional middle fossa craniotomy approach.21
It is important to note that the demonstration of a thinning or dehiscence of ● Acoustic hyperacusis with
the superior semicircular canal on CT of the temporal bone does not necessarily bone conduction thresholds
indicate the presence of superior semicircular canal dehiscence syndrome. By less than 0 dB, autophony,
and abnormal cervical
using 0.5-mm-resolution CT, the incidence of radiographic evidence of thinning
vestibular-evoked myogenic
or dehiscence of the superior semicircular canal is reported to be from 3% to potentials can help
10%.22 However, true histopathologic dehiscence of the superior canal occurs in distinguish superior
only 0.5% of normative cases in studies of the human temporal bone, indicating semicircular canal
dehiscence from
that the CT diagnosis of dehiscence overestimates the diagnosis of superior
perilymphatic fistula.
semicircular canal dehiscence by 6-fold to 20-fold.23 Furthermore, even if
dehiscence of the temporal bone is present, it is likely that it is not always ● Barotrauma related to
symptomatic. The diagnosis of clinically significant superior semicircular canal scuba diving is often
dehiscence should be reserved for patients who meet certain criteria of associated with hearing loss
(90%) and variably
symptoms and signs on testing (TABLE 8-1).15,19 associated with vertigo
(averaging 50%).
PERILYMPHATIC FISTULA
A perilymphatic fistula is an abnormal communication between the fluid-filled
inner ear and the air-filled middle ear, causing a leakage of fluid at the oval or

CONTINUUMJOURNAL.COM 473

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

round window, with the etiology described as being congenital, acquired related
to closed head trauma, or related to a penetrating inner ear injury. Congenital
perilymphatic fistula is often related to middle ear defects, for example,
abnormal stapes or cochlea or vestibular dysplasia, and a dilated vestibular
aqueduct.24 The rupture in the round window was proposed to be caused by
either explosive (increased intracranial pressure) or implosive (Valsalva
maneuver–induced) mechanisms.25 Some of the more common causes of
perilymphatic fistula include barotrauma (discussed later), stapedectomy with
slippage of the stapes prosthesis, temporal bone fracture, and, less commonly, a
penetrating inner ear injury. In the case of a penetrating perilymphatic fistula
injury, immediate surgical exploration and intervention are indicated
(CASE 8-326). Other less dramatic causes can occur with relatively innocuous
“trauma,” such as blowing the nose hard, a slap to the ear, and other minor
Valsalva maneuvers.
Hennebert27 described a nystagmus triggered by positive pressure and negative
pressure into the ear canal. The presentation of vertigo triggered by pressure
changes is sometimes called the fistula sign. However, the fistula sign is reported
in only 29% to 71% of cases of perilymphatic fistula. Other causes of a positive
Hennebert sign include superior semicircular canal dehiscence, cholesteatoma
causing a destructive lesion of the labyrinthine capsule (discussed later),
barotrauma, and a phenomenon of a “slipped strut problem” in which the stapes
prosthesis used in stapedectomy surgery has entered into the inner ear, causing
a perilymphatic leak. The Hennebert sign can be elicited at the bedside by
pressing on the tragus or during vestibular testing by using a pneumatic otoscope
to elicit vertigo or nystagmus, indicative of a possible perilymphatic fistula.

TABLE 8-1 Diagnostic Criteria for Superior Semicircular Canal Dehiscencea

High-resolution CT imaging with ≤0.625-mm slice thickness reformatted in the plane of the
superior semicircular canal demonstrating dehiscence
At least one of the following symptoms
◆ Bone conduction hyperacusis (autophony, audible eye movements, audible footsteps, etc)
◆ Tullio phenomenon: sound-induced vertigo
◆ Pressure-induced vertigo (via nasal or glottic Valsalva maneuver or pressure applied to the
external auditory canal)
◆ Pulsatile tinnitus
At least one of the following diagnostic tests indicating a mobile third window
◆ Negative bone conduction thresholds on pure tone audiometry
◆ Enhanced vestibular-evoked myogenic potential responses (low cervical vestibular-evoked
myogenic potential thresholds or high ocular vestibular-evoked myogenic potential
amplitudes)
◆ Elevated summating potential to action potential on electrocochleography in the absence of
sensorineural hearing loss

CT = computed tomography.
a
Reprinted with permission from Ward BK, et al, Front Neurol.15 © 2017 Ward, Cary, and Minor.

474 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 Ikezono and colleagues28 developed a novel test for human cochlin-tomoprotein, KEY POINTS
which is present in perilymph but not in CSF or serum, yielding high
● High-resolution CT of the
specificity and sensitivity. However, cochlin-tomoprotein testing is not routinely temporal bone is always
available, so perilymphatic fistula remains predominantly a clinical diagnosis, indicated in audiovestibular
confirmed and treated in exploratory surgery. loss in the setting of diving
In cases of traumatic penetration into the inner ear, the CT may demonstrate to rule out anatomic risk
factors, ossicular disruption,
pneumolabyrinth (ie, tiny air bubbles in the labyrinthine fluid in the vestibule,
hemorrhage, or
semicircular canals, and scala vestibuli of the cochlea). It is believed that both the pneumolabyrinth.
perilymphatic fluid loss and the air bubbles within the labyrinth cause
audiovestibular dysfunction. It is important to be aware that children with ● Vestibular symptoms and
anomalies of the inner ear, such as malformation of the cochlea, may be vertigo in inner ear
barotrauma should be
susceptible to perilymphatic fistula–induced hearing loss, and, therefore, imaging referred to an otologic
is always indicated in unknown causes of sudden hearing loss in children. surgeon because surgical
For patients without severe hearing loss or severe vertiginous symptoms, correction results in a high
some neuro-otologists may recommend conservative treatment, which includes rate of symptom relief.
bed rest for 5 to 7 days, elevation of the head of the bed, and avoidance of ● The distinction between
Valsalva maneuvers or straining. barotrauma and
The hearing is followed up with serial audiograms, and if hearing loss is decompression inner ear
present, the patient should be counseled that earlier intervention is associated syndromes in diving is
critical, as inner ear
with a higher likelihood of hearing recovery. Friedland and Wackym29 reviewed
barotrauma can be managed
the surgical cases in a multi-institutional study and reported improvement in with an observational
vestibular symptoms in 90% of the patients presenting with disabling vertigo due period. In contrast,
to perilymphatic fistula. Perilymphatic fistula and superior semicircular canal decompression inner ear
disease, which presents
dehiscence can present similarly: pressure-induced vertigo or sound-induced
with a predominance of
vertigo, both of which represent a vestibular hypersensitivity. One distinction is vestibular symptoms,
that superior semicircular canal dehiscence is much more likely than should be treated with
perilymphatic fistula to present with acoustic hyperacusis, documented by a hyperbaric oxygen within
bone conduction threshold that is less than 0 dB and autophony. 5 hours of injury as any
further delay usually results
in permanent inner ear
INNER EAR DAMAGE RELATED TO SCUBA DIVING damage.
Two main types of inner ear damage may occur secondary to scuba diving. Inner
ear barotrauma is related to pressure changes being transmitted to the inner ear, ● Early recognition of
chronic otitis media and
such as when attempting to equalize the pressure with a forceful Valsalva invasive cholesteatoma is
maneuver. Inner ear decompression damage is related to air emboli or a critical. Because of the
supersaturation of inert gasses. It is important to distinguish between these two proximity of the middle ear
entities because the treatment differs greatly, and inner ear decompression is an canal to the facial nerve, the
horizontal semicircular
indication for immediate hyperbaric oxygen treatment (TABLE 8-230).
canal, and overlying dura,
invasive cholesteatoma can
Inner Ear Barotrauma cause hearing loss, vertigo,
Inner ear barotrauma generally refers to damage to the inner ear that causes facial paresis, meningitis,
and intracranial abscess.
auditory and vestibular symptoms related to underwater diving. Inner ear
barotrauma can occur when pressure changes within the middle ear are ● Surgical eradication of
transmitted to the cochlea. The function of the round window is to compensate cholesteatoma is indicated
for changes in pressure by flexing in and out to prevent damage to the inner ear and aims to prevent the
membranes. In deep-sea diving, especially during ascent or descent, or during extension through the dura
membrane and the
equalization attempts with a forced Valsalva maneuver, sudden changes in the associated intracranial
pressure can occur, causing damage to the inner ear. For barotrauma, complications.
mechanisms of damage can be both explosive and implosive. During descent, the
external pressure increases, and if the eustachian tube has “locked” and a forceful
Valsalva maneuver is attempted, the sudden perilymphatic fluid pressure

CONTINUUMJOURNAL.COM 475

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

increase can rupture the round window. A sudden opening of the eustachian tube can
cause an implosive injury, tearing the oval or round window, disrupting the Reissner
membrane and the basilar membrane, or causing inner ear hemorrhage, the last two
being examples of intralabyrinthine membrane rupture. Other than surgical exploration
and visualization, the diagnosis of inner ear barotrauma can be difficult to make.
For the workup and evaluation of patients with suspected inner ear
barotrauma, pure tone audiometry may reveal varied patterns of hearing loss,

CASE 8-3 A 45-year-old man fell from a bicycle while on a trail and had an
immediate onset of severe rotational vertigo with intractable vomiting,
headache, hearing loss, and tinnitus in his left ear. He was taken to the
local emergency department, and CT was conducted. The physician
noted the presence of a twig impaling the tympanic membrane. The
temporal bone CT revealed a twig through the tympanic membrane
(FIGURE 8-4A) and air bubbles in the vestibule in all three semicircular
canals and in the scala vestibule and basal turn of the cochlea
(FIGURE 8-4B). The patient was transferred to a university center hospital
for further treatment.
Examination revealed a spontaneous, continuous, right-beating
nystagmus in the primary position, and the bedside Weber test lateralized
to the right side indicative of a profound unilateral sensorineural hearing
loss on the left. The patient was immediately taken to the operating room
where it was discovered that one twig pierced the oval window and a
smaller twig penetrated the vestibule. The oval window was repaired,
and a stapes prosthesis was placed. Postoperatively, he had
improvement in the vertigo with only minimal spontaneous nystagmus in
the primary position, a speech reception threshold of 30 dB on the left,
and symmetrical responses to rotational testing on vestibular
nystagmogram (FIGURE 8-5).
Modified from Kita et al, Clin Pract Cases Emerg Med.26 © 2019 Kita et al.

COMMENT This case demonstrates a traumatic perilymphatic fistula with

pneumolabyrinth on CT, hearing loss, and vestibular loss with preservation
of audiovestibular function by rapid surgical intervention.

476 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

most often a flat or down-sloping hearing loss in patients presenting with hearing
loss.30 Electronystagmography is useful in quantifying vestibular damage and
has been reported to have a sensitivity and specificity of 77% in cases of inner ear
barotrauma.31 In studies of inner ear barotrauma, the incidence of vertigo ranged
from 28% to 77% and was overall found in 71 of 156 cases (46%). Hearing loss is
common for barotrauma with perilymphatic fistula in up to 228 of 253 cases
(90%).30 High-resolution CT of the temporal bone is critical to evaluate for

FIGURE 8-4
Penetrating ear trauma and perilymphatic fistula of the patient in CASE 8-3. A, A reconstructed
oblique coronal image shows a linear foreign body projecting from the external auditory
canal to the oval window, with a small projection into the vestibule (arrow). Extensive
intralabyrinthine air is present. B, Axial CT image through the left temporal bone shows air in
the vestibule (long arrow), the posterior semicircular canal (wide arrow), and the scala
vestibuli compartment of the cochlear basal turn (double arrows). Air was also seen in the
lateral and superior semicircular canals (not shown).
Reprinted from Kita AE, et al, Clin Pract Cases Emerg Med.26 © 2019 Kita et al.

FIGURE 8-5
Vestibular testing for the patient in CASE 8-3 conducted 1 month after the penetrating ear
trauma. The sinusoidal rotational vestibulo-ocular reflex at 0.05 Hz and a peak velocity of
60 degrees per second revealed normal and symmetrical gain and phase lead. This
demonstrates recovery of vestibular and auditory function with rapid intervention in the case
of penetrating inner ear trauma.

CONTINUUMJOURNAL.COM 477

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

anatomical risk factors, ossicular disruption, intralabyrinthine hemorrhage, or

other potential causes.
Conservative management of inner ear barotrauma includes initial
observation and medical therapy, including high-dose steroids with a taper for a
total duration of therapy of 18 days, and exploratory surgery with patching if
clinical deterioration with vertigo or a sudden drop in hearing occurs. Many
surgeons opt for exploration, especially in the case of bothersome vertigo. After
barotrauma diagnosis, the patient should consult with a specialist before
conducting more diving. It is important to note that surgical intervention in inner
ear barotrauma led to dramatic improvement in vertigo in 12 of 13 patients, and
thus many otologic surgeons intervene for vestibular symptoms.31

Inner Ear Decompressive Damage in Scuba Diving

Inner ear decompressive damage must be distinguished from inner ear barotrauma.
In the case of inner ear decompressive damage, the damage to the inner ear is
believed to be secondary to either of two mechanisms: (1) the local supersaturation
of inert gases, with the vestibular system more at risk than the cochlear system
because of its lower arterial perfusion and (2) slower inert gas washout or
right-to-left air emboli.32 Deep technical diving using helium and oxygen and trimix
breathing gases appears to confer a higher risk of inner ear decompression sickness.
As an aid in determining inner ear barotrauma from decompression inner ear
disease, key differences are delineated in TABLE 8-2.30 Decompression inner ear
damage, in contrast to barotrauma with perilymphatic fistula, presents with a
preponderance of vestibular symptoms soon after surfacing from the dive, after
an average of 36 minutes and ranging from 13 to 206 minutes after surfacing. Of
note, in 75% of cases, inner ear decompression presents with a pure vestibular
disorder and nearly 18% with a vestibular and cochlear disorder.33,34 In cases
of decompression inner ear syndrome, clinicians should arrange for rapid

TABLE 8-2 Distinguishing Inner Ear Barotrauma From Inner Ear Decompression Illness
With HOOYAH Criteriaa

Criterion Description Inner ear barotrauma Inner ear decompression

H Hard to clear Descent or ascent: difficulty with Not associated

Valsalva maneuver

O Onset Descent, ascent, or after diving Often soon after ascent, average 36 minutes

O Otoscopy Can be abnormal if the middle ear is Normal

involved

Y Your dive profile Fast ascent or descent Missed decompression stops, repetitive dives,
technical diving

A Additional Isolated to the inner ear Other decompression symptoms, “the bends” but only
symptoms in 17% in some studies

H Hearing Very common Vestibular only in 75%, vestibulocochlear in 18%,

cochlear in 6%

a
Reprinted with permission from Rozycki SW, et al, Diving Hyperb Med.30 © 2018 The authors.

478 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

transport to the nearest center for hyperbaric oxygen treatment, ideally within
5 hours, during which the patient should be given 100% oxygen. Steroids can
reduce edema, and low-molecular-weight dextran may improve
microcirculation.35

CHOLESTEATOMA AS A CAUSE OF VESTIBULAR SYMPTOMS

An acquired cholesteatoma is a mass of keratinizing epithelium that accumulates
in the middle ear, often a sequela of chronic otitis media beginning as an inward
growth often from the lateral epithelium of the tympanic membrane. This
area of the tympanic membrane has no annulus (the fibrous thickened edges
of the tympanic membrane that anchor it) and, thus, is susceptible to the
development of chronic otitis and cholesteatoma (CASE 8-4).36 This area of the
middle ear canal is adjacent to the horizontal semicircular canal, and an
acquired cholesteatoma can cause erosive changes invading into the labyrinth,
causing vertigo and hearing loss and eroding into the facial canal causing
facial paralysis.
Erosion into the fallopian canal of the facial nerve and facial nerve paralysis
associated with chronic otitis media are other red flags for impending intracranial
complications. Because of the bone-eroding properties of cholesteatoma, erosion
into the central nervous system can also occur and be associated with
life-threatening intracranial infections or complications, including lateral sinus
thrombosis, brain abscess, or cranial nerve damage.
In the case of recurrent vertigo spells in the setting of a history of otalgia and
partially treated persistent ear infections, a cholesteatoma eroding into the round
window, causing perilymphatic fistula, or eroding into the labyrinthine
structures, causing dehiscence of the horizontal semicircular canal, is most

A 23-year-old man presented with drainage from the right ear and ear CASE 8-4
pain, unilateral tinnitus on the right, and dizziness. He was seen by his
local otolaryngologist who prescribed a 10-day course of antibiotics.
However, 12 days later, he presented to the emergency department with
headache, vertigo, vomiting, and lethargy.
Examination in the emergency department revealed a pearly mass in
the right “attic space” behind the pars flaccida region of the tympanic
membrane. MRI revealed an abscess in the right temporal lobe, and the
mastoid space was filled with granulation tissue and a large
cholesteatoma. During surgery, a defect in the mastoid tegmen (the thin
plate of bone separating the mastoid and middle ear from the brain) and
invasion of the cholesteatoma through the dura were discovered.

Clues in this case include a patient presenting with new-onset vertigo COMMENT
spells, having a history of persistent otalgia or otorrhea with odor, and
otorrhea that persists beyond 3 weeks of antibiotic therapy or a recurrence
of infection within 2 weeks after treatment. This should trigger the clinician
to rule out bone-invading cholesteatoma and to refer the patient urgently
to an otologic surgeon.

CONTINUUMJOURNAL.COM 479

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

commonly the cause of vertigo. In one study of cholesteatoma-induced

labyrinthine fistula, the horizontal canal was involved in 87% of cases.37 Invasive
cholesteatoma is an indication for surgical resection to prevent complications and
intracranial spread. In the case of cholesteatoma, the debris from the keratinizing
mass may block the fistula site, and thus the fistula test may be negative.
A case report of a man presenting with hearing loss, tinnitus, and vertigo
reported a positive fistula test in a left-sided cholesteatoma producing a
left-beating nystagmus with positive pressure pushing on the tragus (a video of a
positive Hennebert test is available at cmaj.ca/lookup/suppl/doi:10.1503/
cmaj.180799/-/DC1)38; intraoperatively, a left lateral (horizontal) semicircular
canal dehiscence was noted in this patient. High-resolution CT of the temporal
bone is usually able to define the location and size of the fistula. However, in the
setting of asymmetry of hearing and vertigo, MRI with contrast of the internal
auditory canal is indicated to rule out other causes.
A patient with facial paralysis must not be given the diagnosis of Bell’s palsy
without a thorough evaluation of the clinical history and detailed examination. In
cases of true Bell’s palsy, the paralysis should occur over less than 24 to 48 hours,
often upon awakening, and should be complete. However, a gradual onset of
facial paralysis may occur in the setting of a tumor or an infection spreading from
otitis media or cholesteatoma. On a clinical note, in bacterial meningitis, the

CASE 8-5 A 47-year-old man presented with a history of right ear pain, fluid-filled
vesicular rash in the external auditory canal, hearing loss, vertigo, and
complete facial paralysis on the right.
On examination, a spontaneous left-beating nystagmus was present in
the primary position, with an increase in amplitude and frequency of
nystagmus with gaze to the left and a decrease with gaze to the right. The
head impulse test13 demonstrated catch-up saccades when the patient’s
head was turned quickly to the right. Test of hearing using a 512-Hz tuning
fork demonstrated that the Weber test lateralized to the left. MRI with
gadolinium revealed enhancement of the right facial nerve, and T2 fluid-
attenuated inversion recovery (FLAIR) images revealed swelling of the
facial nerve. The patient was treated with IV acyclovir and prednisolone.
Eight months later, the patient exhibited a House-Brackmann score of III,
partial facial palsy, and mild imbalance when walking, especially with his
eyes closed. Videonystagmography revealed a 70% caloric paresis.

COMMENT This patient presented with the classical triad of Ramsay Hunt syndrome of
vesicles, hearing loss, and facial paresis. Vestibular involvement is variable.
It is important to recognize and treat Ramsay Hunt immediately because
earlier intervention portends a better outcome. Several aspects of this
case highlight the importance of looking carefully for vesicles of
varicella-zoster virus: the complete facial paralysis associated with
ipsilateral otalgia and the associated audiovestibular disturbance. In this
case, only the external canal near the tympanic membrane exhibited
vesicles, requiring otoscopic examination to visualize.

480 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

clinician should rule out chronic otitis media or cholesteatoma as the source of KEY POINTS
infection. The signs of meningitis, including nuchal rigidity and fever in a patient
● Evaluation for
with cholesteatoma invading into the dura, may be masked by chronic antibiotic fistulization of the bony
use, which may have partially treated the infection. labyrinth may be tested at
The newest imaging application for cholesteatoma is the use of non–echo the bedside with the fistula
planar diffusion-weighted imaging (DWI) MRI to evaluate both primary and test using a pneumatic
otoscope (the Hennebert
postoperative recurrence of middle ear cholesteatoma. DWI can delineate the
test). However, the fistula
size, location, and extent of cholesteatoma because the keratin content of the test may be falsely negative
cholesteatoma demonstrates restricted diffusion on DWI (bright) and a lower in the case of a
value on the apparent diffusion coefficient map. One of the largest cholesteatoma abundantly
filled with keratin debris.
meta-analyses, which included 26 studies and 1152 patients in total, noted a
sensitivity of 91% and a specificity of 92% for middle ear cholesteatoma, and the ● The patient presenting
specialized DWI sequence can be used for both preoperative primary diagnosis with a gradual onset of
(usually combined with temporal bone CT) and for postoperative surveillance.39 facial weakness, often
A clinical note is that cholesteatoma in children is characterized by more severe incomplete in the setting of
otalgia and otorrhea, likely
recurrence, and thus, second-look surgery to evaluate for recurrence or residual has chronic otitis media
disease after the first surgery is often recommended. MRI can be used to follow with cholesteatoma causing
cholesteatoma by using the DWI hyperintensity property of cholesteatoma to dehiscence of the facial
evaluate for recurrence and severity. MRI was used to follow a teenage child with nerve canal.
recurrent ear infections after a resection at an outside institution, and the
● The classical signs of
imaging revealed a recurrence of cholesteatoma that was cleared meningitis may be masked
intraoperatively. Some of the congenital syndromes associated with a high in the patient with invasive
incidence of cholesteatoma potentially include Down syndrome, microtia cholesteatoma due to
antibiotics.
(congenital deformity of the outer ear), Treacher Collins syndrome, and Turner
syndrome.36 These patients may present initially with vertigo, nausea, and ● A diffusion-weighted
vomiting due to vestibular labyrinthine involvement (often the horizontal imaging sequence on MRI
semicircular canal), which portends an impending breach of the overlying dura can be used to follow a
membrane and possible intracranial complications with high morbidity and cholesteatoma, which will
be hyperintense on
mortality. The dictum is that any patient with chronic otitis media with an diffusion-weighted
exacerbation of otorrhea, ear pain, or pain in the temporal region, or headache imaging.
with high fever and vomiting, has an intracranial complication from invasive
cholesteatoma until proven otherwise. ● Chronic otitis media with
headache, nausea, and
vomiting should trigger a
RAMSAY HUNT SYNDROME WITH VERTIGO workup for meningitis.
Ramsay Hunt syndrome is theorized to be a reactivation of the varicella-zoster
virus in the geniculate ganglion or facial nerve presenting with a vesicular rash ● Areas of vesicular rash
and often involves the eighth cranial nerve, causing hearing loss, tinnitus, eruption in Ramsay Hunt
syndrome involve the
vertigo, and imbalance (CASE 8-5). Ramsay Hunt syndrome is the second most sensory distribution of the
common cause of atraumatic peripheral facial paralysis; a retrospective review facial nerve, which can
reported that 12% of 1507 consecutive patients with atraumatic facial palsy had include vesicles in the
Ramsay Hunt syndrome, and an incidence of approximately 5 per 100,000 per ipsilateral ear (concha and
antihelix, antitragus, and a
year, and an age older than 50 years renders a patient more susceptible.40 Ramsay portion of the lobule and
Hunt syndrome is a peripheral facial nerve palsy accompanied by an adjacent mastoid),
erythematous vesicular rash on the ear (zoster oticus) or in the mouth. ipsilateral hard palate, and
Ramsay Hunt syndrome with vertigo, hearing loss, and facial palsy without a anterior two-thirds of the
tongue, which has the
rash is known to occur, termed zoster sine herpete. In a case report, severe special taste sensory
prolonged vertigo lasting several days, with nausea and vomiting similar to that fibers.
seen in vestibular neuritis without facial palsy was the initial presentation of
atypical Ramsay Hunt syndrome, with zoster oticus appearing later; the diagnosis
was confirmed by varicella-zoster virus DNA extracted from the lesions.41

CONTINUUMJOURNAL.COM 481

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

A 2020 comprehensive study used a computer search of patient records for

120 patients with Ramsay Hunt syndrome and questionnaires with long-term
follow-up, ranging from 1 to 17 years (median of 6.6 years), in 81 patients.42
The vesicles could be present before, concomitant with, or after the facial palsy.
A clinically important note is the high frequency of a feeling of an ache around
the affected ear in 77% of the patients, which should alert the clinician to the
possibility of Ramsay Hunt syndrome.42 Ramsay Hunt syndrome (ie, varicella-
associated zoster virus causing facial nerve weakness) can present with vesicles
before and after the facial weakness. The ear canal and tympanic membrane
should be examined for vesicles because external ear vesicles may not be visible.
The outer ear was the primary and sole location of vesicles in 58%, but vesicles
were present solely in the ear canal or on the eardrum in some cases.
Regarding auditory and vestibular involvement in patients with Ramsay Hunt
syndrome, 52% had acute hearing loss on audiogram, but for those answering the
questionnaire, only 22% felt they had any hearing loss as a result of Ramsay Hunt
syndrome.42 In 31% of the records obtained from the hospitalization, patients
reported vertigo, nausea, and vomiting, likely indicative of vestibular nerve and
ganglion involvement. Of the patients who answered a questionnaire that had
been sent out, 51% mentioned vertigo and dizziness, and 32% reported persistent
vertigo or dizziness after a minimum of 1 year of recovery.42
Treatment for Ramsay Hunt syndrome generally consists of antiviral drugs
(acyclovir or famciclovir) combined with corticosteroid treatment. Murakami
and colleagues43 studied outcomes in 80 patients with Ramsay Hunt syndrome
treated with either IV or oral acyclovir and prednisone taper; they reported 75%
recovered if treated within 1 to 3 days of symptom onset, but only 30% recovered
if treated after more than 7 days. For this reason, it is imperative that the clinician
recognize the possibility of Ramsay Hunt syndrome to evaluate and start
treatment immediately. Of note, other cranial nerves have been reported to be
involved in Ramsay Hunt syndrome, but clearly cranial nerve VIII, associated
with vertigo and hearing loss, is the most frequently involved cranial nerve in
Ramsay Hunt syndrome other than the facial nerve; other nerves involved
include cranial nerves V and IX through XII and cervical nerves 2 through 4.

ENLARGED VESTIBULAR AQUEDUCT SYNDROME

The vestibular aqueduct in normal human anatomy is a slitlike aperture forming
a bony canal running from the vestibule to the endolymphatic sac within the
osseous labyrinth. The opening on the cranial side is located close to the internal
auditory canal opening in the posterior surface of the petrous temporal bone. The
vestibular aqueduct contains the endolymphatic duct and the endolymphatic sac,
which are believed to be the areas of endolymph absorption. The enlarged
vestibular aqueduct was initially reported by Valvassori and Clemis44 in 1978 and
noted to be associated with bilateral sensorineural or mixed hearing loss, but the
pathophysiology of the hearing loss is unknown. Up to 10% to 15% of children
with sensorineural hearing loss have enlarged vestibular aqueduct syndrome.45
An enlarged vestibular aqueduct is the most common imaging finding in
children with a congenital inner ear anomaly, and the likely etiology is an arrest
in development in the fifth to eighth week of gestation when the endolymphatic
sac and duct are forming and the vestibular aqueduct develops. In at least half of
the cases of hearing loss associated with an enlarged vestibular aqueduct, a
sequence alteration of the SLC26A4 gene, which encodes for pendrin, an anion

482 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

exchanger, is present.46 Pathogenic sequence alterations in the SLC26A4 gene are KEY POINTS
the second most common cause of autosomal recessive nonsyndromic hearing
● In addition to evaluation
loss, and up to 71% of these patients have recurrent spells of episodic vertigo. of the external ear for
Mutations in the pendrin gene (SLC26A4) can be associated with nonsyndromic vesicles in Ramsay Hunt
deafness with an enlarged vestibular aqueduct and may be associated with goiter syndrome, the clinician
and vestibular symptoms. should evaluate the back of
the ear and conduct an
The enlarged vestibular aqueduct can serve as a third window mechanism,
otoscopic examination of
and the cervical vestibular-evoked myogenic potentials exhibit abnormally low the external canal and the
thresholds and high amplitude as is noted with superior semicircular canal tympanic membrane.
dehiscence syndrome.47 However, the vertigo associated with the Tullio
phenomenon (sound-induced) and Hennebert sign (pressure-induced) is less ● Facial paralysis occurs in
nearly all and hearing loss in
prominent than in the superior semicircular canal dehiscence syndrome, and, up to half of patients with
more commonly, recurrent spells of vertigo last hours or longer, similar to the Ramsay Hunt syndrome.
presentation of Ménière disease, with unknown pathophysiology. And although About 30% to 50% have
the hearing loss usually begins in early childhood, the vestibular symptoms can vestibular neuritis–like
vertigo and subsequent
begin in childhood or may be delayed until adulthood.48 Suzuki and colleagues49 imbalance.
studied the phenotype in 39 patients with hearing loss with SLC25A4 mutations
and reported that 24 (70.6%) of the patients with SLC25A4 mutations had ● A key factor in the
episodes of vertigo. A retrospective chart review of 22 patients with an enlarged recovery of cranial nerve
function after Ramsay Hunt
vestibular aqueduct revealed 14 (64%) had dizziness, with half of the patients
syndrome appears to be an
having recurrent spells of vertigo.50 An enlarged vestibular aqueduct is also earlier onset of treatment.
associated with other inner ear malformations including the cochlea.44,45 Treatment within 1 to 3 days
Workup and evaluation of a suspected enlarged vestibular aqueduct should of symptom onset ensures
75% of patients recover
include an audiogram, otoacoustic emissions, vestibular testing with cervical
function, but only 30% recover
vestibular-evoked myogenic potentials, and an imaging modality. Classically, if treated after 7 days.
previous studies relied on high-resolution CT of the temporal bone to evaluate
for an enlarged vestibular aqueduct. The CT image at the 45-degree oblique plane ● Up to 15% of children with
Pöschl view was used for calculation. Now, the axial image can be used to define sensorineural hearing loss
have an enlarged vestibular
an enlarged vestibular aqueduct if the width at the operculum is ≥2 mm or the aqueduct.
width at the midpoint is ≥1 mm.51 Because the most widely used modality for
imaging in the evaluation of hearing loss is MRI and because of the potential ● An enlarged vestibular
sensitivity of a pediatric patient to the effects of ionizing radiation, MRI is aqueduct and pathogenic
sequence alteration in the
considered the modality of choice to evaluate the inner ear. A large study
SLC26A4 gene are
comparing MRI and CT showed 93% agreement in the diagnosis of an enlarged associated with bilateral
vestibular aqueduct.52 In a study of 96 pediatric patients without sensorineural hearing loss and recurrent
hearing loss, the mean midpoint width was 0.527 mm ± 0.08 mm, ranging from spells of episodic vertigo.
0.353 to 0.887 mm.53 Similar findings, including a mean midpoint width of
● An enlarged vestibular
0.48 mm ± 0.17 mm, were noted in a study of 27 normal temporal bones of aqueduct, like superior
patients aged from 28 to 102 years.54 semicircular canal
Patients diagnosed with an enlarged vestibular aqueduct are often advised to dehiscence, can present
avoid sports or activities or to wear helmets because of the hypothesis that even with cervical vestibular-
evoked myogenic potentials
minor head impacts may be associated with drops in hearing; however, a more with low thresholds and high
recent meta-analysis reported that no strong data prove an association of hearing amplitudes, but the Tullio
loss with trauma.55 Brodsky and Choi56 recommend that providers should make phenomenon and the
families aware of the possible association between an enlarged vestibular Hennebert sign are generally
less prominent. A Ménière
aqueduct and sudden hearing drops due to head trauma, but the family should disease–like presentation
make their own decisions on whether contact sports should be permitted given with recurrent spells of
that head trauma may not significantly impact the risk of overall hearing loss vertigo can be associated
progression.56 They concluded that, currently, evidence is insufficient to support with an enlarged vestibular
aqueduct.
a recommendation that physicians should explicitly restrict patients from playing

CONTINUUMJOURNAL.COM 483

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

TABLE 8-3 Characteristic Auditory, Vestibular, and Imaging Findings in Selected

Otologic Disorders Causing Dizziness

Syndrome Hearing Vestibular Image

Ménière disease Usually low-frequency Often with unilateral reduced Three-dimensional delayed IV
sensorineural hearing loss caloric vestibular responses but gadolinium demonstrates
unilateral normal head impulse test endolymphatic hydrops and
response ipsilaterally bright perilymph

Superior Bony hyperacusis: bone Vestibular hypersensitivity: Tullio CT demonstrates thinning or

semicircular canal conduction <0 dB, pulsatile phenomenon and Hennebert sign; dehiscence over the superior
dehiscence tinnitus, autophony, cervical vestibular-evoked semicircular canal; CT should be
air-bone gap in low myogenic potential with high Pöschl view or Stenvers view, at
frequencies amplitude and low threshold slices ≤0.625 mm

Perilymphatic
fistula

Traumatic Can have sudden Can have severe vertigo with Pneumolabyrinth may indicate
sensorineural hearing loss complete vestibular loss penetrating injury; other potential
indications for surgical exploration
include ossicular fracture and
temporal bone fracture;
developmental abnormalities of
the cochlea are associated with a
higher risk of perilymphatic fistula

Idiopathic Can be normal Can be normal Only seen on surgical exploration

Diving and
barotrauma

Inner ear Often affected Less often affected Some are surgically correctable
barotrauma

Inner ear Rarely solely affected (6%) Often solely affected (75%) Needs immediate hyperbaric
decompression chamber

Cholesteatoma Often affected Often affected CT or MRI may demonstrate

with vertigo cholesteatoma invading the
horizontal semicircular canal,
diffusion-weighted imaging
hyperintensity on MRI

Ramsay Hunt About 50% of patients have About 30%-50% of patients have Look for vesicles on the external
syndrome hearing loss vertigo ear and otoscopic evaluation in the
setting of facial weakness

Enlarged vestibular 10% of all patients with 60%-79% have vestibular Both CT and MRI can demonstrate
aqueduct congenital hearing loss have symptoms, often recurrent a midpoint width of vestibular
an enlarged vestibular prolonged spontaneous vertigo aqueduct ≥1 mm or operculum
aqueduct ≥2 mm

Autoimmune inner Bilateral, often dropping About 50% have vertigo, dizziness, Normal
ear disease hearing loss over ≥3 and other vestibular symptoms
and ≤90 days

CT = computed tomography; MRI = magnetic resonance imaging.

484 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

contact sports because of an enlarged vestibular aqueduct alone. It is also KEY POINTS
important to note that cochlear implantation resulted in significant hearing
● An enlarged vestibular
improvement in patients with an enlarged vestibular aqueduct regardless of aqueduct can be diagnosed
morphology, including incomplete partition type II defects and enlarged by using either CT of the
vestibular aqueduct size. Cochlear implantation is safe in enlarged vestibular temporal bone or MRI of the
aqueduct syndrome and provides improvement of an average of 64 dB in pure internal auditory canal.
tone average and 58 dB in speech reception threshold and 35% improvement in
● Many patients with an
word score.57 enlarged vestibular
aqueduct have been advised
AUTOIMMUNE DISEASES AND INNER EAR SYNDROMES to avoid contact sports, but
A wide range of autoimmune disorders can involve the inner ear and are current data may not
support an association with
associated with hearing loss or vertigo. The proposed mechanisms of inner ear minor head trauma and
dysfunction include (1) antigen antibody–immune complex deposition, (2) drops in hearing. Also,
autoantibodies directed against inner ear antigens, and (3) specific cytotoxic T cochlear implantation in
cells causing inner ear damage. Following are specific examples of autoimmune these patients is safe and
effective.
inner ear disease that are associated with progressive hearing loss over weeks,
Cogan syndrome and Sjögren syndrome. ● Deafness associated with
autoimmune inner ear
Autoimmune Inner Ear Disease disease occurs over weeks
and not 1 or 2 days, as in
In one of the first case series of autoimmune inner ear disease, Lehnhardt58
sudden sensorineural
hypothesized an antigen-antibody reaction in 13 patients with progressive hearing loss. One-third of
bilateral sensorineural hearing loss. Nine of the patients had initial unilateral the patients with
hearing loss and then subsequent involvement of the contralateral ear, which autoimmune inner ear
disease have or will develop
Lehnhardt proposed was secondary to the first cochlear injury triggering
a systemic autoimmune
antibodies that then secondarily caused the involvement of the contralateral inner disease.
ear and hearing. In 1979, McCabe59 proposed the term autoimmune inner ear disease
in a series of patients with idiopathic bilateral hearing loss that was responsive to ● Up to 50% of patients with
steroids. The differential diagnosis includes syphilis, bilateral Ménière disease, autoimmune inner ear
disease report vertigo or
superficial siderosis, ototoxicity, neurofibromatosis type 2, and radiation injury. dizziness or have tinnitus
The timeframe of the development of hearing loss is key to the diagnosis of and aural fullness,
autoimmune inner ear disease: typically, the development of deafness occurs mimicking Ménière disease.
over weeks, and not quickly over a day or two, but it also does not progress over
● Corticosteroids are the
years as in age-associated presbycusis.
mainstay of treatment for
Autoimmune inner ear disease is defined as bilateral hearing loss with a decline autoimmune inner ear
in at least one ear that evolves in longer than 3 days but less than 90 days. It is disease, with serial
important to note that, in up to one-third of cases, autoimmune inner ear disease audiograms to evaluate
hearing with taper. Referral
occurs in the setting of a primary rheumatologic disorder, including systemic lupus
to rheumatology for
erythematosis, rheumatoid arthritis, Sjögren syndrome, Hashimoto thyroiditis, consideration of
granulomatosis with polyangiitis, Behçet disease, and primary neurologic steroid-sparing medications
immune-related entities such as multiple sclerosis and myasthenia gravis.60,61 and evaluation for systemic
Investigating the sera of patients with a history of autoimmune inner ear autoimmune disease should
be considered.
disease has identified antibodies to inner ear proteins such as cochlin, type II
collagen, peripheral myelin protein zero, and b-actin, and the most studied is the
antibody directed to 68-kDa inner ear antigen, characterized as heat shock
protein 70, which has a high specificity of 90% but low sensitivity of 42%.62 MRI
with and without gadolinium aids in ruling out retrocochlear pathologies:
vestibular schwannoma, intracranial metastases, demyelinating diseases, or
cerebrovascular ischemia.
Investigators have noted that vestibular symptoms often present as recurrent
vertigo spells during the course of autoimmune inner ear disease.63 A 2018 review

CONTINUUMJOURNAL.COM 485

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

of autoimmune inner ear disease reported vestibular symptoms, with imbalance

and motion intolerance, ataxia, and positional or episodic vertigo occurring in
50% of patients with autoimmune inner ear disease.64 Because 25% to 50% of
patients with autoimmune inner ear disease have tinnitus and aural fullness, it
can be difficult to distinguish from Ménière disease. Other considerations in
bilateral hearing loss with and without vertigo include otologic syphilis, Lyme
disease, and Cogan syndrome.
Corticosteroids remain the mainstay of treatment for autoimmune inner ear
disease, with the dosing relatively unchanged from the original recommendation:
untapered high-dose steroids for 4 weeks followed by attempted tapers
depending on clinical outcomes.61 Referral to rheumatology is nearly always
indicated to aid in steroid-sparing interventions and to investigate for primary
autoimmune diseases, which are a comorbidity in up to 30% of the cases of
autoimmune inner ear disease.60,65 Of note, autoimmune inner ear disease
preceded the development of autoimmune disease as long as months to years
later; thus, follow-up with these patients is strongly recommended.63

TABLE 8-4 “Red Flags” When a Patient Presents With Vertigo and These Symptoms

◆ Presence of sudden violent falls (ie, Tumarkin falls) with a sense of being pushed in a patient
with Ménière disease: indication for consideration of vestibular ablation due to the violent
nature of these falls, which can be associated with trauma
◆ Vertigo triggered by pressure on the tragus (Hennebert sign) or loud sounds (Tullio phenomenon):
superior semicircular canal dehiscence or perilymphatic fistula; both are surgically correctable
◆ Presence of fever and ear pain with draining ear (otorrhea) and vertigo: rule out
cholesteatoma invading into the horizontal semicircular canal causing dehiscence
syndrome, urgent surgical evaluation
◆ Severe otalgia and ipsilateral facial weakness: evaluate external canal, pinna, and tympanic
membrane for vesicles of Ramsay Hunt syndrome; immediate acyclovir or other antiviral,
with corticosteroids; rapid initiation of treatment is important because 75% recover cranial
nerve function if given within 1 to 3 days, but only 30% recover if given after 7 days
◆ Vertigo occurring soon after a complex dive, within 13 to 206 minutes after surfacing (average
of 36 minutes): consider inner ear decompression illness, which presents with a pure
vestibular disorder in 75% of cases; treatment is hyperbaric chamber as soon as possible
with supplemental oxygen while en route
◆ Presence of pneumolabyrinth on CT (best view to visualize the air bubbles is coronal cuts):
may indicate a penetrating trauma causing perilymphatic leak allowing air bubbles to enter
into the labyrinth; this requires urgent surgical exploration and reparative surgery
◆ History of congenital sensorineural hearing loss bilateral, fluctuating course: (1) enlarged
vestibular aqueduct is associated with up to 10%-15% of children with sensorineural hearing
loss, cochlear implant is restorative of hearing and safe in patients with enlarged vestibular
aqueduct; (2) children with congenital cochlear malformations have a much higher incidence
of perilymphatic fistula with even minor trauma, such as blowing the nose hard, and surgical
exploration may be indicated if cochlear malformation is identified on CT, about one-third of
patients with enlarged vestibular aqueduct had recurrent spells of vertigo in one study
◆ Fluctuating bilateral dropping hearing levels over from 3 days to 90 days: consider
autoimmune inner ear disease with institution of high-dose untapered corticosteroids for
4 weeks followed by attempted tapers (vertigo and vestibular symptoms in up to 50% of
patients with autoimmune inner ear disease)

CT = computed tomography.

486 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Cogan Syndrome
Cogan syndrome is a rare disease of people predominantly between the ages of 20
and 40 years (mean age of onset is 25 years old); it is characterized by
autoimmune nonsyphilitic keratitis of the eyes, vertigo, and tinnitus, but,
generally, patients maintain normal or near-normal vision, and up to 52%
develop profound hearing loss despite immunosuppressive treatment.66 In
atypical Cogan syndrome, progressive hearing loss occurs, and in 15% to 20% of
patients, systemic manifestations can occur, such as aortitis of the cardiovascular
system, or systemic vasculitis of the neurologic or gastrointestinal system.
Recurrent vertigo, similar to that which occurs in Ménière disease, is common,
but a distinction is that the hearing loss in Cogan syndrome is bilateral and often
progresses to complete deafness in 2 years. Systemic corticosteroids are the
mainstay for treatment of the audiovestibular symptoms of Cogan syndrome,
but if these are not effective, other immunosuppressants are tried, including
azathioprine, cyclosporin A, and methotrexate, among others.67
The typical audiovestibular presentation may be Ménière disease–like, with
fluctuating hearing loss and vertigo spells, and the audiometric profile is
consistent with cochlear, rather than retrocochlear, hearing loss. Of note,
deafness occurs in more than half of patients with Cogan syndrome. Temporal
bone studies of Cogan syndrome report degeneration of the structures of the
vestibular labyrinth and the cochlear duct, including the organ of Corti and stria
vascularis, and endolymphatic hydrops. A temporal bone of a patient with Cogan
syndrome after successful bilateral cochlear implantation revealed
endolymphatic hydrops of all cochlear turns and of the saccule and degenerative
and fibro-osseous changes throughout.68

Sjögren Syndrome
In a study using temporal bones from patients with a history of Sjögren
syndrome, an autoimmune illness associated with 22.5% to 46% occurrence of
sensorineural hearing loss, immunoglobulin deposition was identified in the stria

Special Imaging Considerations in Otologic Disorders Causing Dizziness TABLE 8-5

◆ MRI on 3-tesla delayed IV gadolinium evaluates for endolymphatic hydrops and permeability
within the blood-labyrinthine barrier in Ménière disease
◆ CT evaluates for superior semicircular canal dehiscence; use 0.5-mm CT reformatted in the
plane of the superior semicircular canal (Pöschl view) and orthogonal (Stenvers view)
◆ CT in the coronal view is best to visualize pneumolabyrinth, if present, other red flags in
trauma is the presence of ossicular fracture or dislocation: both indicate possible
penetrating trauma with damage to the inner ear
◆ CT is classically used to evaluate for cholesteatoma, and non–echo planar
diffusion-weighted imaging is increasingly being used to evaluate for restriction on
diffusion-weighted imaging indicative or cholesteatoma
◆ MRI with gadolinium reveals enhancement, and T2 fluid-attenuated inversion recovery
(FLAIR) reveals swelling of the facial nerve in Ramsay Hunt syndrome
◆ CT in the Pöschl plane, which runs parallel to the longitudinal axis of the vestibular aqueduct,
evaluates the diameter at the midpoint; if the vestibular aqueduct diameter is >1.0 mm, it is
consistent with an enlarged vestibular aqueduct

CT = computed tomography; IV = intravenous; MRI = magnetic resonance imaging.

CONTINUUMJOURNAL.COM 487

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

KEY POINTS vascularis of patients with a history of hearing loss but not in those without a
history of hearing loss.69 Sone and colleagues70 noted multiple studies related to
● The vasculitis and
audiovestibular dysfunction
temporal bone findings in patients with a history of systemic autoimmune
of Cogan syndrome usually disease, demonstrating stria vascularis and spiral ganglia neuronal atrophy. In
respond to high-dose addition, animal models of autoimmune inner ear disease also demonstrate the
corticosteroids, with the same histopathologic findings.68
expectation of a beneficial
response within 2 to
3 weeks. In intractable
Cogan syndrome, the CONCLUSION
progression to deafness This article summarizes the most recent clinical findings in the otologic diseases
occurs in more than half of
that often present with vertigo including Ménière disease, superior semicircular
patients.
canal dehiscence, perilymphatic fistula, barotrauma, cholesteatoma, Ramsay
● In both autoimmune inner Hunt syndrome, enlarged vestibular aqueduct, and autoimmune inner ear
ear disease– and Cogan disease. TABLE 8-3 is a summary of the auditory and vestibular testing results and
syndrome–associated imaging findings of these entities. Also, TABLE 8-4 presents a summary of red
deafness, cochlear
implantation is often
flags that the neurologist and neuro-otologist should always keep in mind when
restorative of hearing with evaluating a patient with vertigo of unknown etiology. TABLE 8-5 shows
good to excellent results. important new imaging considerations in these otologic entities. In most cases,
auditory and vestibular testing and an imaging study are indicated in the workup
● The finding of
of these patients.
immunoglobulin deposition
in the stria vascularis and
spiral ganglia in Sjögren
syndrome associated with REFERENCES
hearing loss indicates that
the hearing loss in
1 Eggers SDZ. Episodic spontaneous dizziness. 8 Ishiyama G, Lopez IA, Acuna D, Ishiyama A.
autoimmune inner ear
Continuum (Minneap Minn) 2021;27(2, Neuro- Investigations of the microvasculature of the human
disease and systemic otology:369-401. macula utricle in Meniere's disease. Front Cell
autoimmune disease may be Neurosci 2019;13:445. doi:10.3389/fncel.2019.00445
mediated in part by 2 Fife TD, Tourkevich R. Tinnitus, hyperacusis,
immunoglobulin deposition otalgia, and hearing loss. Continuum (Minneap 9 Baloh RW, Jacobson K, Winder T. Drop attacks
Minn) 2021;27(2, Neuro-otology):491-525. with Menière's syndrome. Ann Neurol 1990;28(3):
in the inner ears.
384-387. doi:10.1002/ana.410280314
3 Maxwell AK, Ishiyama G, Kaznezis S, Ishiyama A.
Isolated saccular hydrops on high-resolution MRI 10 Chen Z, Zhang Y, Zhang Q. Tumarkin drop attack
is associated with full spectrum Meniere’s recorded by video surveillance. JAMA Neurol 2020;
disease [published online January 19, 2021]. Otol 77(7):897-898. doi:10.1001/jamaneurol.2020.0884
Neurotol. doi:10.1097/MAO.0000000000003051
11 Calzada AP, Lopez IA, Ishiyama G, Ishiyama A.
4 Tagaya M, Yamazaki M, Teranishi M, et al. Otolithic membrane damage in patients with
Endolymphatic hydrops and blood-labyrinthine endolymphatic hydrops and drop attacks. Otol
barrier in Ménière's disease. Acta Otolaryngol Neurotol 2012;33(9):1593-1598. doi:10.1097/
2011;131(5):474-479. doi:10.3109/00016489. MAO.0b013e318271c48b
2010.534114
12 Ishiyama G, Ishiyama A, Jacobson K, Baloh RW.
5 Pakdaman MN, Ishiyama G, Ishiyama A, et al. Drop attacks in older patients secondary to an
Blood-labyrinth barrier permeability in Menière otologic cause. Neurology 2001;57(6):1103-1106.
disease and idiopathic sudden sensorineural doi:10.1212/wnl.57.6.1103
hearing loss: findings on delayed postcontrast
13 Halmagyi GM, Curthoys IS. A clinical sign of canal
3D-FLAIR MRI. AJNR Am J Neuroradiol 2016;37(10):
paresis. Arch Neurol 1988;45(7):737-739.
1903-1908. doi:10.3174/ajnr.A4822
doi:10.1001/archneur.1988.00520310043015
6 Ishiyama G, Lopez IA, Ishiyama P, et al. The blood
14 Minor LB, Solomon D, Zinreich JS, Zee DS. Sound-
labyrinthine barrier in the human normal and
and/or pressure-induced vertigo due to bone
Meniere's disease macula utricle. Sci Rep 2017;7:
dehiscence of the superior semicircular canal.
253. doi:10.1038/s41598-017-00330-5
Arch Otolaryngol Head Neck Surg 1998;124(3):
7 Ishiyama G, Wester J, Lopez IA, et al. Oxidative 249-258. doi:10.1001/archotol.124.3.249
stress in the blood labyrinthine barrier in the
15 Ward BK, Carey JP, Minor LB. Superior canal
macula utricle of Meniere's disease patients.
dehiscence syndrome: lessons from the first
Front Physiol 2018;9:1068. doi:10.3389/
20 years. Front Neurol 2017;8:177. doi:10.3389/
fphys.2018.01068
fneur.2017.00177

488 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

16 Zhou G, Gopen Q, Poe DS. Clinical and diagnostic 29 Friedland DR, Wackym PA. A critical appraisal of
characterization of canal dehiscence syndrome: spontaneous perilymphatic fistulas of the inner
a great otologic mimicker. Otol Neurotol 2007; ear. Am J Otol 1999;20(2):261-276.
28(7):920-926.
30 Rozycki SW, Brown MJ, Camacho M. Inner ear
17 Govender S, Fernando T, Dennis DL, et al. barotrauma in divers: an evidence-based tool for
Properties of 500Hz air- and bone-conducted evaluation and treatment. Diving Hyperb Med
vestibular evoked myogenic potentials (VEMPs) 2018;48(3):186-193. doi:10.28920/dhm48.3.186-193
in superior canal dehiscence. Clin Neurophysiol
31 Edmonds C. Inner ear barotrauma: a
2016;127(6):2522-2531. doi:10.1016/j.
retrospective clinical series of 50 cases. SPUMS
clinph.2016.02.019
Journal 2004;34:11-19.
18 Fife TD, Colebatch JG, Kerber KA, et al. Practice
32 Klingman C. Inner ear decompression sickness in
guideline: cervical and ocular vestibular evoked
compressed-air diving. Undersea Hyperbaric
myogenic potential testing: report of the
Med 2012;39(1):589-594.
Guideline Development, Dissemination, and
Implementation Subcommittee of the American 33 Shupak A, Gil A, Nachum Z, et al. Inner ear
Academy of Neurology. Neurology 2017;89(22): decompression sickness and inner ear
2288-2296. doi:10.1212/WNL.0000000000004690 barotrauma in recreational divers: a long-term
follow-up. Laryngoscope 2003;113:2141-2147.
19 Gopen Q, Zhou G, Poe D, et al. Posterior
semicircular canal dehiscence: first reported 34 Gempp E, Louge P. Inner ear decompression
case series. Otol Neurotol 2010;31(2):339-344. sickness in scuba divers: a review of 115 cases.
doi:10.1097/MAO.0b013e3181be65a4 Eur Arch Otorhinolaryngol 2013;270(6):1831-1837.
doi:10.1007/s00405-012-2233-y
20 Fife TD, Satya-Murti S, Burkard RF, Carey JP.
Vestibular evoked myogenic potential testing: 35 Livingstone DM, Smith KA, Lange B. Scuba diving
payment policy review for clinicians and payers. and otology: a systematic review with
Neurol Clin Pract 2018;8(2):129-134. doi:10.1212/ recommendations on diagnosis, treatment and
CPJ.0000000000000430 post-operative care. Diving Hyperb Med 2017;
47(2):97-109. doi:10.28920/dhm47.2.97-109
21 Schwartz SR, Almosnino G, Noonan KY, et al.
Comparison of transmastoid and middle fossa 36 Jennings BA, Prinsley P, Philpott C, et al. The
approaches for superior canal dehiscence genetics of cholesteatoma. A systematic review
repair: a multi-institutional study. Otolaryngol using narrative synthesis. Clin Otolaryngol 2018;
Head Neck Surg 2019;161(1):130-136. doi:10.1177/ 43(1):55-67. doi:10.1111/coa.12900
0194599819835173
37 Copeland BJ, Buchman CA. Management of
22 Linda W, Brewster R, Poe D, et al. Superior labyrinthine fistula in chronic ear surgery. Am J
semicircular canal dehiscence syndrome. Otolaryngol 2003;24(1):51-60. doi:10.1053/
J Neurosurg 2017;127(6):1268-1276. doi:10.3171/ ajot.2003.10
2016.9.JNS16503
38 Hsieh MC, Wu CC, Wang SH. Positive perilymph
23 Carey JP, Minor LB, Nager GT. Dehiscence or fistula test with semicircular canal dehiscence
thinning of bone overlying the superior from cholesteatoma. CMAJ 2019;191(4):E104.
semicircular canal in a temporal bone survey. doi:10.1503/cmaj.180799
Arch Otolaryngol Head Neck Surg 2000;126(2):
39 Lingham RK, Bassett P. A meta-analysis on the
137-147. doi:10.1001/archotol.126.2.137
diagnostic performance of non-echoplanar
24 Weissman JL, Weber PC, Bluestone CD. diffusion-weighted imaging in detecting middle
Congenital perilymphatic fistula: computed ear cholesteatoma: 10 years on. Otol Neurotol
tomography appearance of middle ear and inner 2017;38(4):521-528.
ear anomalies. Otolaryngology 1994;111(3 pt 1):
40 Robillard RB, Hilsinger RL, Adour KK. Ramsay Hunt
243-249. doi:10.1177/01945998941113P113
facial paralysis: clinical analyses of 185 patients.
25 Goodhill V, Brockman SJ, Harris I, Hantz O. Otolaryngol Head Neck Surg 1986;95(3 pt 1):
Sudden deafness and labyrinthine window 292-297. doi:10.1177/01945998860953P105
ruptures. Audio-vestibular observations. Ann
41 Nakamura Y, Matsumoto H. Case of atypical
Otol Rhinol Laryngol 1973;82(1):2-12.
Ramsay-Hunt syndrome who presented with
doi:10.1177/000348947308200103
severe vertigo and vomiting [in Japanese]. No To
26 Kita AE, Kim I, Ishiyama G, Ishiyama A. Hattatsu 2012;44(1):66-68.
Perilymphatic fistula after penetrating ear
42 Kanerva M, Jones S, Pitkaranta A. Ramsay Hunt
trauma. Clin Pract Cases Emerg Med 2019;3(2):
syndrome: characteristics and patient
115-118. doi:10.5811/cpcem.2019.1.37404
self-assessed long-term facial palsy outcome.
27 Hennebert C. Labyrinthite double reflexe moteur Eur Arch Otorhinolaryngol 2020;277(4):1235-1245.
otooculaire. Clinique 1905;19:214-215. doi:10.1007/s00405-020-05817-y
28 Ikezono T, Matsumura T, Matsuda H, et al. The 43 Murakami S, Honda N, Mizobuchi M, et al. Rapid
diagnostic performance of a novel ELISA for diagnosis of varicella zoster virus infection in
human CTP (cochlin-tomoprotein) to detect acute facial palsy. Neurology 1998;51(4):
perilymph leakage. PLoS One 2018;13(1):e0191498. 1202-1205. doi:10.1212/wnl.51.4.1202
doi:10.1371/journal.pone.0191498

CONTINUUMJOURNAL.COM 489

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

SELECTED OTOLOGIC DISORDERS CAUSING DIZZINESS

44 Valvassori GE, Clemis JD. The large vestibular 57 Patel ND, Ascha MS, Manzoon NF, et al.
aqueduct syndrome. Laryngoscope 1978;88(5): Morphology and cochlear implantation in
723-728. doi:10.1002/lary.1978.88.5.723 enlarged vestibular aqueduct. Am J Otolaryngol
2018;39(6):657-663. doi:10.1016/j.
45 Steinbach S, Brockmeier S, Kiefer J. The large
amjoto.2018.06.006
vestibular aqueduct—case report and review of
the literature. Acta Otolaryngol 2005;126(8): 58 Lehnhardt E. Sudden hearing disorders occurring
788-795. doi:10.1080/00016480500527276 simultaneously or successively on both sides [in
German]. Z Laryngol Rhinol Otol 1958;37(1):1-16.
46 Miyagawa M, Nishio SY, Usami S. Mutation
spectrum and genotype-phenotype correlation 59 McCabe BF. Autoimmune sensorineural hearing
of hearing loss patients caused by SLC26A4 loss. Ann Otol Rhinol Laryngol 1979;88(5 pt 1):
mutations in the Japanese: a large cohort study. 585-589. doi:10.1177/000348947908800501
J Human Genet 2014;59(5):262-268. doi:10.1038/
60 Bovo R, Almoni C, Martini A. Immune-mediated
jhg.2014.12
inner ear disease. Acta Otolaryngol 2006;126(10):
47 Sheykholeslami K, Schmerber S, Habiby Kermany 1012-1021. doi:10.1080/00016480600606723
M, Kaga K. Vestibular-evoked myogenic
61 Rauch SD. Clinical management of
potentials in three patients with large vestibular
immune-mediated inner-ear disease. Ann N Y
aqueduct. Hear Res 2004;190(1-2):161-168.
Acad Sci 1997;830:203-210. doi:10.1111/j.1749-
doi:10.1016/S0378-5955(04)00018-8
6632.1997.tb51891.x
48 Oh AK, Ishiyama A, Baloh RW. Vertigo and the
62 Hirose K, Wener MH, Duckert LG. Utility of
enlarged vestibular aqueduct syndrome. J Neurol
laboratory testing in autoimmune inner ear
2001;248(11):971-974. doi:10.1007/s004150170050
disease. Laryngoscope 1999;109(11):1749-1754.
49 Suzuki H, Oshima A, Tsukamoto K, et al. Clinical doi:10.1097/00005537-199911000-00005
characteristics and genotype-phenotype
63 Broughton SS, Meyerhoff WE, Cohen SB.
correlation of hearing loss patients with SLC26A4
Immune-mediated inner ear disease: 10-year
mutations. Acta Otolaryngol 2007;127(12):
experience. Semin Arthritis Rheum 2004;34(2):
1292-1297. doi:10.1080/00016480701258739
544-548. doi:10.1016/j.semarthrit.2004.07.001
50 Song JJ, Hong SK, Lee SY, et al. Vestibular
64 Ciorba A, Corazzi V, Bianchini C, et al.
manifestations in subjects with enlarged
Autoimmune inner ear disease (AIED): a
vestibular aqueduct. Otol Neurotol 2018;39(6):
diagnostic challenge. Int J Immunopathol
e461-e467. doi:10.1097/MAO.0000000000001817
Pharmacol 2018;32:2058738418808680. doi:
51 Boston M, Halsted M, Meinzen-Derr J, et al. The 10.1177/2058738418808680
large vestibular aqueduct: a new definition
65 Vambutas A, Pathak S. AAO: autoimmune and
based on audiologic and computed tomography
autoinflammatory (disease) in otology: what is
correlation. Otolaryngol Head Neck Surg 2007;
new in immune-mediated hearing loss.
136(6):972-977. doi:10.1016/j.otohns.2006.12.011
Laryngoscope Investig Otolaryngol 2016;1(5):
52 Connor SEJ, Dudau C, Pai I, Gaganasiou M. Is CT 110-115. doi:10.1002/lio2.28
or MRI the optimal imaging investigation for the
66 Gluth MB, Baratz KH, Matteson EL, Driscoll CLW.
diagnosis of large vestibular aqueduct syndrome
Cogan syndrome: a retrospective review of 60
and large endolymphatic sac anomaly? Eur Arch
patients throughout a half century. Mayo Clin
Otorhinolaryngol 2019;276(3):593-702. doi:
Proc 2006;81(4):483-488. doi:10.4065/81.4.483
10.1007/s00405-019-05279-x
67 Illiescu DA, Timaru CM, Batras M, De Simone A,
53 Juliano AF, Ting EY, Mingkwansook V, et al.
Stefan C. Cogan’s syndrome. Rom J Ophthalmol
Vestibular aqueduct measurements in the 45°
2015;59(1):6-13.
oblique (Pöschl) plane. AJNR Am J Neuroradiol
2016;37(7):1331-1337. doi:10.3174/ajnr.A4735 68 Kamakura T, Lee DJ, Herrmann BS, Nadol JB Jr.
Histopathology of the human inner ear in Cogan's
54 Sando I, Ikeda M. The vestibular aqueduct in
syndrome with cochlear implantation. Audiol
patients with Meniere's disease. A temporal
Neurotol 2017;22(2):116-123. doi:10.1159/
bone histopathological investigation. Acta
000477534
Otolaryngol 1984;97(5-6):558-570. doi:
10.3109/00016488409132934 69 Calzada AP, Balaker AE, Ishiyama G, et al.
Temporal bone histopathology and
55 Alemi AS, Chan DK. Progressive hearing loss and
immunoglobulin deposition in Sjogren's
head trauma in enlarged vestibular aqueduct: a
syndrome. Otol Neurotol 2012;33(2):258-266.
systematic review and meta-analysis.
doi:10.1097/MAO.0b013e318241b548
Otolaryngol Head Neck Surg 2015;153(4):512-517.
doi:10.1177/0194599815596343 70 Sone M, Schachern PA, Paparella MM,
Morizonon N. Study of systemic lupus
56 Brodsky JR, Choi SS. Should children with an
erythematosus in temporal bones. Ann Otol
enlarged vestibular aqueduct be restricted from
Rhinol Laryngol 1999;108(4):338-344.
playing contact sports? Laryngoscope 2018;
doi:10.1177/000348949910800404
128(10):2219-2220. doi:10.1002/lary.27119

490 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 Tinnitus, Hyperacusis, REVIEW ARTICLE


Otalgia, and Hearing Loss C O N T I N U UM A U D I O
I NT E R V I E W A V A I L AB L E
ONLINE
By Terry D. Fife, MD, FAAN, FANS; Roksolyana Tourkevich, MD
Downloaded from http://journals.lww.com/continuum by wqLe/H3/oM2vx1Qs5A5qMemKKHLPqE4qkmGNpdheG3ikf1o85ttJSkFL2oTkgOkhMEmawHvsuTTE5xJG2ZjqccytIAhVn2qegy060KLcKJuPZsx5htaYiopRjp2zor2egUzWJAEWYNo65TEub0G9tZtPaMachiqn on 04/28/2022

ABSTRACT
PURPOSE OF REVIEW: This article reviews the causes of tinnitus, hyperacusis,
and otalgia, as well as hearing loss relevant for clinicians in the field of
neurology.

RECENT FINDINGS: Important causes of unilateral and bilateral tinnitus are

discussed, including those that are treatable or caused by serious
structural or vascular causes. Concepts of hyperacusis and misophonia
are covered, along with various types of neurologic disorders that can
lead to pain in the ear. Hearing loss is common but not always purely
otologic.

SUMMARY: Tinnitus and hearing loss are common symptoms that are
sometimes related to a primary neurologic disorder. This review, tailored
to neurologists who care for patients who may be referred to or CITE AS:
encountered in neurology practice, provides information on hearing CONTINUUM (MINNEAP MINN)
2021;27(2, NEURO-OTOLOGY):
disorders, how to recognize when a neurologic process may be involved, 491–525.
and when to refer to otolaryngology or other specialists.
Address correspondence to
Dr Terry D. Fife, Barrow
Neurological Institute, 240 W
INTRODUCTION Thomas Rd, Ste 301, Phoenix,

H
earing loss and tinnitus are exceptionally common symptoms AZ 85013, [email protected].
encountered in nearly all areas of medicine and may affect patients
RELATIONSHIP DISCLOSURE:
of all ages. Hearing loss severe enough to impair daily communication Drs Fife and Tourkevich report
is seen in more than half of individuals aged 60 to 70 years and no disclosures.
affects 80% of those 85 years and older.1,2 Hearing loss as a measure UNLABELED USE OF
of years of disability is the fourth leading cause of disability globally.1 Similarly, PRODUCTS/INVESTIGATIONAL
clinically significant chronic tinnitus affects about 10% to 15% of people, and USE DISCLOSURE:
Drs Fife and Tourkevich discuss
the incidence, like that of hearing loss, increases with age. Tinnitus is frequently the unlabeled/investigational
also accompanied by hearing loss.3 Hyperacusis, an abnormally low tolerance use of carbamazepine,
for ordinary sounds, is far less common but may be related to tinnitus or may gabapentin, oxcarbazepine, or
pregabalin for the treatment of
occur as an isolated condition that can affect both adults and children.4 certain types of pulsatile
Neurologists may be asked to consult on sudden hearing loss or acute tinnitus, pulse-asynchronous tinnitus;
baclofen, carbamazepine,
especially when it is either unilateral or occurs in association with neurologic
clonazepam, onabotulinumtoxinA,
symptoms. Neurologists should be especially aware of neurologic conditions or oxcarbazepine for the
that may include tinnitus, hyperacusis, and hearing loss. It is valuable to have treatment of palatal or middle ear
myoclonus; and betahistine for
some familiarity with hearing and other auditory symptoms and to be able to the treatment of Ménière
recognize when to refer to the otolaryngologist. This article begins by focusing disease.
on tinnitus, then on hyperacusis and a brief section on unexplained unilateral
otalgia, followed by a discussion of hearing loss. The final two sections © 2021 American Academy
include a brief overview of audiometry and current hearing augmentation. of Neurology.

CONTINUUMJOURNAL.COM 491

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

TINNITUS
Tinnitus is the perception of sound such as ringing, crickets, buzzing, or hissing
that occurs in the absence of an external sound stimulus. The word comes from
the Latin tinnire meaning to ring. Tinnitus is considered a symptom rather than a
discrete disease entity, although, in many cases, the specific cause remains
undetermined. Many people (12% to 30% of the general population) experience
tinnitus and are aware of it but are not particularly bothered by it. About 0.5%
to 3% of those with tinnitus find it intrusive and very distressing. The prevalence
of tinnitus increases with age. Risk factors include hearing loss, increased age,
male sex, noise exposure, family history of hearing loss or tinnitus,
temporomandibular joint (TMJ) syndrome, eustachian tube dysfunction,
ceruminous impaction of the external ear, hypertension, diabetes, and exposure
to ototoxic drugs such as aminoglycosides, salicylates, loop diuretics, and
quinine. TABLE 9-1 outlines some conditions associated with tinnitus.
Most tinnitus is subjective, meaning no source of the perceived sound can
be identified and it is evident only to the individual. Objective tinnitus, which is
much less common, is generated by vascular or other anatomic mechanisms that

TABLE 9-1 Conditions and Medications Associated With Tinnitus

Inner ear
◆ Bilateral sensorineural hearing loss
◆ Loud noise exposure (bilateral)
◆ Ménière disease (unilateral)
◆ Labyrinthitis (unilateral)
◆ Sudden sensorineural hearing loss (unilateral)
◆ Labyrinthine barotrauma (unilateral or bilateral)
◆ Autoimmune inner ear disease (bilateral)
◆ Superior canal dehiscence syndrome (unilateral or bilateral)a
◆ Pregnancy (bilateral)
◆ Hyperviscosity states (often bilateral)
◆ Sudden brief unilateral tapering tinnitus (unilateral)
Middle ear or external ear
◆ Otosclerosis (unilateral or bilateral)
◆ Ceruminous impaction (unilateral or bilateral)
◆ Cholesteatoma (unilateral)
◆ Eustachian tube dysfunction (unilateral or bilateral)a
◆ Stapedius or tensor tympani spasms (unilateral)
Nonotologic causes
◆ Stress, generalized anxiety (bilateral)
◆ Temporomandibular joint syndrome (unilateral or bilateral)

CONTINUED ON PAGE 493

492 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

occur in or near the ear and with proper methods may be audible to an external
observer.

Pathophysiology of Tinnitus
The precise mechanism of chronic subjective tinnitus is still not well understood.
In most cases, dysfunction affecting the peripheral auditory system with
associated loss of cochlear hair cell function underlies tinnitus. The loss of the usual
cochlear input that results from the lesion causes a frequency-specific increase in
output to the central auditory pathways. The reduction of sound that results from
reduced hearing causes the plastic changes in the brain to replace the missing
auditory stimulation. In people with persisting tinnitus, impairment of the limbic
system and central auditory processes seems to lead to reduced ability to suppress
phantom sounds.5 By at least one theory, tinnitus may be akin to denervation
hypersensitivity or phantom limb pain, which occurs in the somatosensory
system. At least the denervation hypersensitivity may lead to the initial tinnitus,
whereas long-term persistence may be related more to the limbic system and
central auditory processing.5

CONTINUED FROM PAGE 492

◆ Palatal myoclonus (bilateral)

◆ Jugular vein venous hum (unilateral or bilateral)
◆ Idiopathic intracranial hypertension (bilateral)
◆ Vascular (arteriovenous malformation, turbulent flow) (unilateral or bilateral)
◆ Glomus jugulare paraganglioma (unilateral or bilateral)
Medications
◆ Loop diuretics (furosemide, ethacrynic acid) (bilateral)
◆ Aspirin, nonsteroidal anti-inflammatory drugs (bilateral)
◆ Hydroxychloroquine (bilateral)
◆ Quinine (cinchonism) (bilateral)
◆ Quinidine (bilateral)
◆ Cisplatin (bilateral)
◆ Methotrexate (bilateral)
◆ Gentamicin (bilateral)
◆ Azithromycin, clarithromycin (bilateral)
◆ Angiotensin-converting enzyme inhibitors (bilateral)
Metabolic
◆ Diabetes mellitus (bilateral)
◆ Hyperinsulinemia (bilateral)
◆ Hyperviscosity syndromes (bilateral)
◆ Pregnancy (bilateral)

a
This condition is associated with autophony (ie, the increased hearing of one’s own voice or internal bodily
sounds).

CONTINUUMJOURNAL.COM 493

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

TABLE 9-2 Causes and Common Descriptive Features of Tinnitus

Descriptive feature Common patient descriptions Causes/disorders

Unilateral tinnitus

Constant continuous Ringing, crickets, chirping, hissing, machine Ceruminous impaction

sound, cicadalike for most conditions;
Unilateral sensorineural hearing loss
continuous roaring or humming for venous hum
Unilateral noise exposure
Ménière disease
Barotrauma
Vestibular schwannoma
Venous hum

Pulse synchronous Throbbing, whooshing, thumping Unilateral conductive hearing loss

Idiopathic intracranial hypertension
Carotid artery sounds transmitted (bruit,
turbulence, dissection)
Cardiac sounds transmitted such as valvular
sound
High-riding jugular bulb
Superior canal dehiscence syndrome
Glomus tumors
Dural arteriovenous fistula, other arteriovenous
malformation
High-blood-flow states (pregnancy,
hypertension, hyperthyroidism, anemia)

Pulse asynchronous Sound of ocean waves, echoey, reverberating for Patulous eustachian tube
patulous eustachian tube; manual typewriter,
Typewriter tinnitus
Morse code, machine gun for typewriter tinnitus;
clicking, tapping, fluttering, or drumlike thumping Middle ear myoclonus
for middle ear myoclonus

Bilateral tinnitus

Constant continuous Ringing, crickets, chirping, hissing, machine Idiopathic tinnitus

(even if intermittent sound, cicadalike
Bilateral sensorineural hearing loss
initially)

Pulse synchronous Throbbing, whooshing, thumping Bilateral conductive hearing loss

Many of the causes of pulse synchronous
unilateral tinnitus may be reported by patients
as being present bilaterally

Pulse asynchronous Rhythmic clicking, tapping, or thumping Palatal myoclonus/oculopalatal myoclonus

494 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 That is, in some individuals, the thalamic-paralimbic inhibitory feedback loop KEY POINTS
is insufficient to suppress the tinnitus. Over a period usually of days to weeks, the
● Among the most serious
loss of thalamic inhibitory gating permits the auditory signals to cause causes of unilateral
synchronization and reorganization of cortical networks in the frontal, parietal, pulse-synchronous tinnitus
and limbic regions which may lead to perpetuation of chronic tinnitus based on are a dural arteriovenous
central generators.6,7 Consequently, chronic tinnitus cannot be eliminated even fistula, arteriovenous
malformation, or a glomus
by severing the auditory nerve(s).8 In addition, other medical and psychological
tumor arising from the
factors influence how an individual may react to and cope with tinnitus. jugular foramen or middle
Functional MRI (fMRI) in 11 patients with tinnitus and 11 control subjects ear.
showed hyperactivity especially in the nucleus accumbens, a part of the
corticostriatal pathways involved in reward and aversion responses and emotion. ● Many forms of unilateral
and bilateral tinnitus are
This system influences which sounds are important and which may be more bothersome to
suppressed from consciousness. Tinnitus may in some ways mirror what patients with coexistence of
happens with certain mood disorders and chronic pain.5 depression, insomnia,
The mechanisms for objective forms of tinnitus are often easier to understand stress, anxiety, and
excessive caffeine use.
because mechanical processes generate the sound. For example, turbulent flow in
a vessel or arteriovenous malformation (AVM) is transmitted to the cochlea via
bone conduction. Although this type of tinnitus is the least common, it is often
the most concerning because it is sometimes due to an underlying serious
structural lesion such as a dural arteriovenous fistula (AVF), AVM, glomus
tumor, or carotid artery dissection.

History and Examination of Tinnitus

In taking the history of a patient with tinnitus, the neurologist should ascertain
the character and duration, whether the tinnitus is unilateral or bilateral, whether
it is constant or intermittent, and whether it is triggered or modulated by
any circumstances. It is also potentially helpful to have a description of the
character of the tinnitus such as ringing, crickets, chirping, hissing, cicadalike,
or low pitched (TABLE 9-2). The character description may have some use but
does not often distinguish the mechanism. The clinician should inquire whether
the tinnitus is caused or modulated in pitch or loudness by jaw clenching,
neck movements, eye movements, or pressure on certain areas of the head
or neck. When tinnitus is strongly linked to somatic triggers, such as jaw
clenching, muscular contraction, or mechanical activities, it is referred to as
somatic tinnitus.
Finally, determining the perceived severity and life impact of the tinnitus
may aid in gauging psychosocial stressors and the severity of the symptom.
Patient-rated scales, such as the Tinnitus Handicap Inventory that is scored on
a scale of 0 to 100, help rate the impact on the patient’s life as the patient
perceives it. Nearly all forms of tinnitus are made more noticeable by
comorbidities such as depression, insomnia, stress, anxiety, and excess caffeine
use. In musical ear syndrome, a patient with hearing loss hears music or
melodies. Although the precise cause is not known, it may be that sound
deprivation results in a substitution with musical sounds from auditory
association areas of the brain. This may be analogous to Charles Bonnet
syndrome in which visual deprivation results in visual hallucinations. Hearing
well-formed words and voices suggests auditory hallucinations due to psychosis
rather than tinnitus.
Examination for tinnitus should include otoscopy to assure that the ear canal
is not impacted with cerumen, which can be a cause of tinnitus and reduced

CONTINUUMJOURNAL.COM 495

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

hearing. Limitation of jaw opening to less than 4 cm with clicking or popping or

TMJ joint crepitus may suggest TMJ syndrome, which is associated with tinnitus.
Funduscopic examination for papilledema can be a clue for idiopathic
intracranial hypertension which may cause pulsatile tinnitus.
Pulsatile tinnitus may be pulse-synchronous or pulse-asynchronous.
Whether the tinnitus is subjective (the examiner does not hear it) or objective
(audible to the examiner) can depend on the examiner’s own hearing, the location
(s) auscultated, the environmental quietness, and the quality of the listening tools.
For example, cursory auscultation with a poor-quality stethoscope in a noisy clinic
setting may miss what is evident when using an electronic noise-canceling
stethoscope in a quiet room. Particularly for pulse-synchronous tinnitus,
auscultation should include the skull, temples, periauricular regions, carotid
arteries, and heart because a dural AVF or other AVM may produce an audible
bruit. The distinction between subjective and objective tinnitus seems prone to
misclassification depending on the quality of the auscultation effort.

Classification of Tinnitus
Widely accepted classifications of tinnitus are few, but TABLE 9-3 offers one
simple classification, and TABLE 9-2 outlines some of the common descriptions
and related causes of tinnitus. In addition, as mentioned earlier, tinnitus can be
pulse-synchronous or pulse-asynchronous; constant or intermittent; acute,
subacute, or chronic; and unilateral or bilateral.
Other categorizations are divided among primary (idiopathic) and secondary
(due to an identified condition); based on the grade of psychological or functional
severity; or peripheral (originating in the cochlea) versus central (dysfunction
within the central nervous system auditory processing pathways) causes. By far,
the most common form of tinnitus is bilateral high-pitched ringing, crickets, or

TABLE 9-3 Classification of Tinnitusa

Unilateral
◆ Constant or intermittent nonrhythmic (most common type)
◆ Pulse-synchronous (pulsatile)
◆ Pulse-asynchronous (rhythmic but not tied to heartbeat)
◇ Clicking/staccato
◇ Hum/low-pitched
Bilateral
◆ Constant or intermittent nonrhythmic (most common type)
◆ Pulse-synchronous (pulsatile)
◆ Pulse-asynchronous (rhythmic but not tied to heartbeat)
◇ Clicking/staccato
◇ Hum/low-pitched

a
Within this scheme, tinnitus may be intermittent or constant. Duration may be further characterized as
acute (less than 3 months), subacute (3 to 6 months), or chronic (longer than 6 months).

496 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

cicada-sounding tinnitus. From the neurologist’s point of view, the unilateral and KEY POINT
pulse-synchronous forms are of greatest concern for a structural or neurologic origin.
● Otologic causes of
unilateral pulse-
Constant or Intermittent Nonrhythmic Tinnitus synchronous tinnitus include
Most tinnitus is a steady, sustained sound that has no rhythmic or staccato ceruminous impaction and
features. It may come and go and be more noticeable in quiet environments but middle ear disorders leading
to conductive hearing loss
does not pulsate or have interruptions in the sound. This category accounts for such that bone-conducted
the large majority of tinnitus experienced by humans. It is often associated with sounds from vascular and
some degree of high-frequency hearing loss but sometimes occurs in patients other internal structures are
with normal hearing or precedes hearing loss by several years. heard more loudly.

Pulsatile Tinnitus
Pulsatile tinnitus refers to tinnitus that has a rhythmic, periodic sound that
alternates between louder and softer or that has discrete interruptions with
periods of silence between the sounds. Pulse-synchronous means the pattern
of pulsation occurs with the same timing and tempo as the individual’s
pulse and heartbeat. Pulse-asynchronous refers to a less common category in
which tinnitus has a rhythmic recurring quality but does not correlate with
heart rate or pulse. Many of the causes of pulse-synchronous tinnitus are
benign and simply related to turbulent flow in arteries or transmitted heart
sounds. Sometimes, however, serious conditions may present in this manner,
and it is important not to overlook these cases. Pulsatile tinnitus can be
unilateral or bilateral.

UNILATERAL CONSTANT OR INTERMITTENT NONRHYTHMIC TINNITUS. The most

common cause of unilateral tinnitus is ceruminous impaction of the external ear
canal. Once this is excluded by otoscopy, other common causes include prior
unilateral excessive noise exposure, barotrauma, unilateral hearing loss, sudden
sensorineural hearing loss, or viral labyrinthitis as examples. Structural lesions
such as vestibular schwannoma may present with unilateral tinnitus, but it is
most commonly also associated with slowly progressive ipsilateral hearing loss,
as well. Ménière disease may sometimes present with fluctuating hearing or ear
fullness and unilateral tinnitus that may vary in pitch and loudness (CASE 9-1).
Unilateral tinnitus not infrequently is simply the asymmetrical onset of what
will eventually be bilateral tinnitus after several years. This same pattern may
occur or develop in conjunction with unilateral hearing loss that later becomes
bilateral.
A venous hum may cause unilateral tinnitus that may be high or low pitched
and sometimes roaring or rough tinnitus that is often unilateral but occasionally
described as bilateral. It may occur in adults or children and results from
turbulent flow in the internal jugular vein. The hum is usually present
throughout the cardiac cycle but may fluctuate and can be made to abate by
compression of the internal jugular vein, the Valsalva maneuver, turning the
head, or lying flat. It may be audible with auscultation of the neck just above the
clavicle, more often on the right than on the left. A venous hum may be annoying
to the patient, but it is not a harmful condition and needs no treatment.

UNILATERAL PULSE-SYNCHRONOUS TINNITUS. Unilateral conductive hearing loss

sometimes results in pulsatile tinnitus due to the lack of masking effect from
air-conducted sound in the ear affected with hearing loss. The absence of

CONTINUUMJOURNAL.COM 497

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

external sounds heard by that ear may make one’s own voice and internal heart
and vascular sounds more apparent. Idiopathic intracranial hypertension and
obstructive hydrocephalus (eg, due to aqueductal stenosis) are commonly
accompanied by unilateral and, less often, bilateral pulsatile tinnitus.9,10 It is
caused by elevated intracranial pressure creating changes in arterial flow and
venous draining leading to turbulence audible to the patient.
Carotid artery blood flow is another fairly common cause of pulse-synchronous
tinnitus as irregular flow creates sound that is transmitted to the ear so that
the patient perceives the sound. Atherosclerosis-related carotid bruits, carotid
artery dissections, tortuous carotid siphon, and, less commonly, fibromuscular
dysplasia may all be causes. Occasionally, conditions that cause high-flow states
such as pregnancy, hypertension, anemia, or hyperthyroidism can cause

CASE 9-1 A 37-year-old man was referred by another neurologist because of

3 years of constant tinnitus that periodically fluctuates in pitch and
loudness along with reduced hearing in the left ear. Although he seemed
most focused on the tinnitus, when queried about other symptoms, he
mentioned about eight episodes of vertigo over the previous 3 years
usually lasting several hours.
He had a history of migraine
managed adequately with
onabotulinumtoxinA,
verapamil, and nortriptyline.
Brain MRI of the internal
auditory canal with and
without contrast performed
3 years ago because of
migraine headaches and
the left-sided tinnitus with
hearing loss was normal.
Examination was normal
except for reduced hearing
in the left ear during
bedside screening tests.
His audiogram (FIGURE 9-1) FIGURE 9-1
The audiogram for the patient in CASE 9-1
showed low-frequency
demonstrates low-frequency left-sided hearing
left-sided hearing loss loss characteristic of Ménière disease.
characteristic of left ANSI = American National Standards Institute, which sets
Ménière disease. specifications for audiometers.

COMMENT This patient has left-sided Ménière disease and was referred primarily for the
left-sided tinnitus. Most patients with Ménière disease are mainly concerned
with the vertigo attacks if they have had them, so it is unusual that the patient
did not mention it until he was asked if he had ever had vertigo. This patient also
had unilateral constant left-sided tinnitus, but it would periodically fluctuate in
pitch and loudness, which is a clue for Ménière disease.

498 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

increased flow through the heart or carotid arteries. Less often, heart sounds such
as murmurs are loud enough to be perceived by patients. In superior canal
dehiscence, patients have a heightened sense of hearing of internal bodily
sounds (autophony), which may cause pulse-synchronous tinnitus. Jugular bulb
abnormalities, such as a high-riding jugular bulb due to the proximity between
the jugular bulb and the cochlea, may also cause unilateral pulse-synchronous
tinnitus.11
Glomus tumors are rare paragangliomas that are derived from embryonic
neural crest cells that make up chemoreceptors such as the carotid bodies. These
tumors most commonly affect women in their forties and fifties; they are
vascular and usually benign but may be locally invasive and may erode through
bone. Those that present with hearing loss and pulsatile tinnitus usually originate
in the jugular foramen (ie, glomus jugulare) or the middle ear (ie, glomus
tympanicum). Sensorineural or conductive hearing loss and pulsatile tinnitus are
common presenting symptoms.
Dural AVFs and, to a far lesser extent, other AVMs may cause and even
present solely with unilateral pulse-synchronous tinnitus.12 Dural AVFs are
abnormal connections between branches of dural arteries and dural veins or
venous sinuses. The most common locations of dural AVFs that result in pulsatile
tinnitus are in the transverse, sigmoid, and cavernous sinuses. Most of the time,
the tinnitus is unilateral (CASE 9-2), but if the dural AVF is near the midline,
tinnitus may be reported as seeming to be bilateral. The most common cause in
adults with an acquired dural AVF is venous sinus thrombosis and venous
hypertension.13 Treatment is neurosurgical, often consisting of endovascular
coiling or embolization.14

A 57-year-old woman presented with 1 year of left-sided pulsatile tinnitus CASE 9-2
that correlated with her heartbeat along with reduced hearing in the left
ear, all with gradual onset and progression. Auscultation of the left ear
and periauricular region was unrevealing, and the otologic and neurologic
examinations were normal except for left-sided reduced hearing. Digital
compression of the left internal jugular vein just above the clavicle and
just lateral to the carotid artery pulsation diminished the tinnitus.
An audiogram that had been conducted before the visit showed
low-frequency conductive hearing loss on the left. Brain MRI showed a
left occipitotemporal dural arteriovenous fistula (AVF) in which the
middle meningeal artery and transosseous branches of the left occipital
artery drained into the sigmoid sinus and petrous sinuses. She was
referred to neurosurgery, and endovascular embolization occluded the
dural AVF with resolution of the tinnitus.

This case illustrates how a dural AVF may present with unilateral pulsatile COMMENT
tinnitus. Dural AVFs commonly present with pulsatile tinnitus and are
important to recognize because they confer an annual mortality rate from
hemorrhage approaching 10%.

CONTINUUMJOURNAL.COM 499

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

UNILATERAL PULSE-ASYNCHRONOUS TINNITUS. A patulous (distended or wide-open)

eustachian tube can lead to rhythmic roaring tinnitus that is usually synchronous
with respiration. The eustachian tube is a small mucosa-lined fibrocartilaginous
tube connecting the nasopharynx to the middle ear that serves to equilibrate
pressure between the middle ear and the outside world. It is approximately 3.5 cm
long and with a lumen of approximately 1.5 mm to 2.5 mm. The eustachian tube is
usually closed but opens with swallowing and yawning by contraction of the
tensor veli palatini muscle. Failure of this tube to open leads to increased or
decreased middle ear pressure resulting in muffled hearing and often a feeling of
fullness or pressure. A patulous or persistently open eustachian tube leads to a
hollow roaring kind of sound that modulates with respiration and swallowing.
Sometimes, when the eustachian tube fails to open regularly or completely (the
opposite of a patulous eustachian tube), cracking sounds or the sound of fluid can
be heard in the ear on the affected side.
So-called typewriter tinnitus is a form of unilateral random and intermittent
tinnitus that produces a sharp distinct series of staccato sounds likened by
patients to a “machine gun,” “Morse code,” or a “tapping” sound.15 Possible
causes include microvascular loop compression, Ehlers-Danlos syndrome, and
basilar invagination due to conditions such as osteogenesis imperfecta or Paget
disease. This type of unilateral rhythmic tinnitus may respond to carbamazepine,
oxcarbazepine, gabapentin, or pregabalin.16
Middle ear myoclonus is an uncommon cause of unilateral pulse-
asynchronous tinnitus, often described by patients as intermittent salvos of
unilateral fluttering, thumping, or buzzing (CASE 9-3). It is thought that the
stapedius and tensor tympani muscles rhythmically contract, causing these
sounds typically at a rate of 1 to 2 per second. That can cause visible movement of
the tympanic membrane.17 The stapedius muscle has its origin on the posterior
wall of the temporal bone in the middle ear and attaches to the neck of the stapes;
it dampens sounds transmitted through the ossicular chain and is innervated by
the facial nerve. The tensor tympani muscle has its origin in the cartilaginous and
bony edges of the auditory tube and inserts on the handle of the malleus and, like

CASE 9-3 A 38-year-old woman developed bothersome clicking/thumping tinnitus

in her right ear over the past 2 years; it was initially intermittent but was
daily over the last year. The clicking would momentarily diminish with
mouth-opening or swallowing, but it was otherwise not influenced by
activity; however, sometimes when lying with her right ear down, the
clicking was bilateral. Brain MRI was normal, she had received several
normal otolaryngology evaluations, and several audiograms were normal.
Neurologic examination was normal and synchronous movements of
the tympanic membrane were not seen. Nevertheless, clicking near the
right ear could be heard by the examiner in a quiet room.

COMMENT This case illustrates a patient with palatal or middle ear myoclonus, but it is
indeterminate whether it was due to myoclonus of the stapedius, tensor
tympani, or tensor veli palatini muscle.

500 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

the stapedius muscle, dampens sounds through the ossicular chain and moves the KEY POINTS
tympanic membrane during yawning and swallowing. It is innervated by the
● Unilateral pulse-
medial pterygoid nerve of the trigeminal nerve. Treatment regimens for these synchronous tinnitus may
conditions are poorly delineated in the literature but might include trials of be caused by sounds
clonazepam, baclofen, carbamazepine, oxcarbazepine, or onabotulinumtoxinA transmitted from the carotid
administration into the muscle affected assuming it can be ascertained by artery (bruit) or the heart
(murmur), increased
visual observation.
intracranial pressure, or
A similar phenomenon may also occur with the tensor veli palatini and is conditions that cause
referred to as palatal myoclonus, which can be unilateral or bilateral. Palatal high-flow states such as
myoclonus is discussed in more detail later in this article. pregnancy, anemia, and
hyperthyroidism.

BILATERAL CONTINUOUS OR INTERMITTENT, NONRHYTHMIC. This category accounts ● “Typewriter tinnitus” is a

for the tinnitus that most people experience. It may begin as intermittent term for a pulse-
bilateral tinnitus and, over time, become constant. It is always subjective asynchronous tapping or
tinnitus, meaning it is audible only to the patient. The tinnitus may consist of Morse code–like staccato
tinnitus that may respond
high-pitched ringing, crickets chirping, hissing, or a cicadalike or machinelike to treatment with
sound or, less commonly, a low-pitched or musical sound. This type of tinnitus is carbamazepine,
very commonly associated with sensorineural hearing loss but may precede oxcarbazepine, gabapentin,
hearing loss yet to come by 1 or 2 years. or pregabalin.

● Myoclonus of the
MANAGEMENT. Although this is a very common problem, evidence-based stapedius or tensor tympani
effective treatments are limited, and many remedies offered are not supported may cause a benign type of
by evidence, despite claims to the contrary. Of course, the first step in treatment unilateral fluttering or
thumping tinnitus that is
is to minimize aggravating factors such as medications that cause tinnitus,
rhythmic but not
including those listed in TABLE 9-1. Patients should also reduce exposure to loud synchronous with the heart
noises, and insomnia and stress should be managed through counseling, lifestyle rate.
changes, and possibly pharmacotherapy. Medications that are sometimes used
with anecdotal success include antidepressant medications and anxiolytic ● Bilateral high-pitched
subjective tinnitus that is
medications such as benzodiazepines, which probably work mostly because they constant but varies with
control anxiety or mood disorders. Pentoxifylline, gabapentin, pregabalin, ambient noise is the most
papaverine, and clopidogrel have also been used anecdotally but have not been common form of tinnitus and
found effective by controlled trials. If somatic tinnitus contributions such as neck is often associated with
some degree of
pain are present, these can be managed, sometimes indirectly improving sensorineural hearing loss.
tinnitus. TMJ syndrome may be treated if present, and massage relaxation
techniques or acupuncture may be tried. Counseling, tinnitus retraining therapy,
and cognitive-behavioral therapy are supported by weak evidence that they can
reduce the negative impact on quality of life for patients with tinnitus.18,19
Combined hearing aids and sound generators are commonly used but appear to
offer little or no difference in tinnitus symptom severity by evidence-based
standards.20 Masking devices may result in some temporary improvement, but
studies published to date have not found a significant change in the severity of
tinnitus compared with the use of relaxation and other coping strategies.21 Deep
brain stimulation or neuromodulation has been preliminarily explored to treat
tinnitus but further study is needed.22

BILATERAL PULSE-SYNCHRONOUS. Several of the conditions that may cause

pulse-synchronous unilateral tinnitus may sometimes present as bilateral
tinnitus, including intracranial hypertension, glomus tumors, and conditions that
result in increased blood flow states. Superior canal dehiscence can also cause
patients to perceive bilateral pulsatile tinnitus. Bilateral conductive hearing loss

CONTINUUMJOURNAL.COM 501

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

KEY POINT may also result in pulsatile tinnitus because air-conducted sound no longer masks
internal heart sounds.
● Palatal myoclonus and
oculopalatal myoclonus
cause an objective clicking BILATERAL PULSE-ASYNCHRONOUS. Some of the causes for unilateral pulse-
sound audible to the patient asynchronous tinnitus may rarely be perceived by the patient as bilateral and
and others and that persists simultaneous, but this is quite uncommon.
during sleep.
Palatal myoclonus and oculopalatal myoclonus comprise a rhythmic
involuntary movement of the uvula and soft palate, and often a clicking sound
is heard both by patient and examiner, usually at a frequency of 0.5 Hz to 2 Hz.
Because it is midline, it is often perceived by the patient as bilateral. In the case of
oculopalatal myoclonus, the soft palate movements are synchronous with
pendular torsional or vertical bobbing eye movements in one or both eyes and
may be seen after stroke or with degenerative disorders, encephalitis, neoplasm,
or other conditions that disturb the Guillain-Mollaret triangle (FIGURE 9-2).
Oculopalatal myoclonus persists during sleep. Isolated palatal myoclonus in
the absence of structural causes and eye movement findings is referred to as
essential palatal myoclonus. Some individuals with essential palatal myoclonus
can exert some volitional control over the movements.23 Treatment for
oculopalatal myoclonus and essential myoclonus has variable success using
benzodiazepines, valproic acid, baclofen, or onabotulinumtoxinA injected into
portions of the soft palate and in the tensor veli palatini.

Diagnostic Evaluation of Tinnitus

A workup for tinnitus should include an audiogram. Matching tinnitus pitch and
loudness can be achieved by having the patient choose from among various
sound frequencies that most resemble the tinnitus in pitch and loudness. Patients
can also match the tinnitus through a variety of online tinnitus-matching
websites. Although this is of interest, it rarely helps management strategies
except perhaps for some sound-based interventions. Serum laboratory tests may
include thyroid function,
antinuclear antibody, fluorescent
treponemal antibody absorption,
complete blood cell count, fasting
glucose or hemoglobin A1c, and
lipid panel and an assessment for
serum zinc deficiency.
Guidelines on imaging studies
are outlined in TABLE 9-4.24 As
noted in TABLE 9-4, the workup
for pulse-synchronous tinnitus
should be directed at excluding
dural AVF, AVM, and glomus
jugulare and glomus tympanicum
usually by CT angiography of the
head and neck or even
cervicocerebral digital
FIGURE 9-2 subtraction angiography. This is
Guillain-Mollaret triangle structures and
in contrast to constant bilateral
pathways.
D = dentate nucleus of the cerebellum; O = olivary nucleus tinnitus for which imaging has
of the medulla; RN = red nucleus. low diagnostic value.24

502 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 In the absence of vestibular symptoms or imbalance, vestibular testing
such as videonystagmography is rarely helpful. Vestibular-evoked myogenic
potential (VEMP) testing (cervical or ocular) may be helpful if pulse-synchronous
tinnitus is associated with other forms of autophony and superior semicircular
canal dehiscence syndrome is suspected. For more information about superior
semicircular canal dehiscence syndrome, refer to the article “Selected Otologic
Disorders Causing Dizziness” by Gail Ishiyama, MD,25 in this issue of Continuum.

HYPERACUSIS
Hyperacusis is an abnormally low tolerance for ordinary environmental sounds.
A small subset of patients who develop hyperacusis have no known underlying

Imaging Studies for Tinnitusa TABLE 9-4

Subjective or objective pulsatile tinnitus (not due to myoclonus)

◆ Usually indicated (favorable risk-benefit ratio for patients)
◇ CT angiography (CTA) head and neck with contrast
◇ Cervicocerebral CT and head venogram with contrast
◆ Sometimes indicated (equivocal risk-benefit ratio for patients)
◇ Cervicocerebral angiography
◇ Temporal bone CT without contrast
◆ Usually not indicated (risk-benefit ratio for patients is likely to be unfavorable)
◇ Temporal bone CT with and without contrast
Asymmetric or unilateral subjective nonpulsatile tinnitusb
◆ Sometimes indicated (equivocal risk-benefit ratio for patients)
◇ Temporal bone CT with or without contrast
◇ Head and neck CTA with contrast
◆ Usually not indicated (risk-benefit ratio for patients is likely to be unfavorable)
◇ Temporal bone CT with and without contrast
◇ Cervicocerebral angiography
Symmetric bilateral subjective nonpulsatile tinnitusc
◆ Usually not indicated (risk-benefit ratio for patients is likely to be unfavorable)
◇ Temporal bone CT with and without contrast
◇ Head CT venogram with contrast
◇ Head and neck CTA with contrast
◇ Head CTA with contrast
◇ Cervicocerebral angiography
◇ Temporal bone CT without contrast

CT = computed tomography.
a
Data from Expert Panel on Neurologic Imaging, J Am Coll Radiol.24
b
Normal otologic examination, no hearing asymmetry, no neurologic deficits, no history of trauma.
c
No hearing loss, no neurologic deficit, no history of trauma.

CONTINUUMJOURNAL.COM 503

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

cause but nevertheless find ordinary sound excessively loud and bothersome,
depending on pitch and loudness. For patients with hyperacusis, any sound
beyond some threshold evokes negative reactions. Reactions to sound may
include discomfort, dislike, irritation, anxiety, panic, fear, and ear pain and
may result in depression and anxiety. Sometimes, this sound threshold is so
low that it approaches the hearing threshold. A hallmark feature of hyperacusis
is that the loudness of sounds that bother these patients does not bother most
other people (CASE 9-4). Its cause is not clear but is believed to be due to a
disorder of the central auditory pathways with effects on and reinforcement
by the limbic and autonomic systems and not due to any primary inner ear
process.26 The best treatment strategies have not been established but have
included sound avoidance, cognitive-behavioral therapy and desensitization, and
cognitive-behavioral therapy combined with management of any comorbid
anxiety and depression.
Not all aversion to loud sounds is hyperacusis. Many people with bilateral
hearing loss, and particularly those who wear hearing aids, may find that
some sounds seem excessively loud whereas others are not heard well, but this is
not hyperacusis in the strict sense. Medical disorders have been linked to
increased sound sensitivity including Williams syndrome, Bell’s palsy

CASE 9-4 A 34-year-old woman was seen in consultation for 4 years of bilateral
severe hypersensitivity to sound. She had a history of falling on the ice
while figure skating near the time of the onset of symptoms but landed on
her buttocks and did not strike her head. She recalled a reverberating
sensation go through her ears, but she seemed to recover uneventfully.
The sound sensitivity started gradually and had gotten much worse,
causing her to seek accommodation from her employer to work from
home. She had seen four otolaryngologists, an otologist, and three
neurologists. She wore noise-canceling headphones most of the time
after trying sound desensitization and finding it intolerable. Sounds such
as people talking, background voices, clanging dishes and silverware,
traffic, and doors opening and closing, as well as some air conditioner
sounds and electrical hums, all bothered her, causing ear pain, anxiety,
and depression.
Her examination was normal except for some erosion of the auricle and
bulbous deformity of the ear cartilage on both sides (cauliflower ears)
from the continued headphone use. MRI, multiple audiograms, temporal
bone CT, vestibular-evoked myogenic potential testing, otoacoustic
emissions, auditory brainstem responses, and routine laboratory tests
including thyroid values had all been normal.

COMMENT This case illustrates the extreme disability encountered by some patients
with hyperacusis. This patient was referred to both a psychiatrist and a
psychologist for a combined treatment approach to improve her function
and to help her discontinue her constant headphone use.

504 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

(unilateral, due to stapedius paresis), and Lyme disease. Sound sensitivity is
found sometimes in the aftermath of concussion, during active migraine events,
during benzodiazepine withdrawal, and in some patients with depression,
but these are more in keeping with phonophobia, the fear of or aversion to
loud sounds.
Misophonia is a form of selective dislike of a specific type of sound to the
point that a negative emotional reaction to it occurs. Examples can be anxiety
from the sound of balloons, clicking of a pen, foot-tapping, whistling, chewing,
or swallowing. Unlike hyperacusis, which is sensitivity to sounds of certain
frequencies and loudness, misophonia is usually the dislike of a specific type
of sound and evokes emotional responses such as disgust, irritation, feeling
trapped, rage, panic, anxiety, or fear. Most patients with misophonia learn to
function, but a smaller number develop severe avoidance behaviors and
functional limitations. The cause is not known, but one theory is that patients
may become sensitized from previous experiences, and repeated exposure
creates enhancement of synaptic connections to the limbic system and
emotional memory.27 Proneness to misophonia has been linked to higher
personality traits for anxiety.28 Treatment consists of cognitive-behavioral
therapy (CASE 9-5).

IDIOPATHIC UNILATERAL OTALGIA

Occasionally, patients are referred to a neurologist due to idiopathic otalgia (ear
pain), most commonly affecting one side. Otalgia may be primary, meaning it
arises from ear structures, or secondary, meaning it is either referred from or
generated elsewhere. Most such referrals initially go to primary care doctors and
otolaryngologists, so referral to neurology usually means no explanation such as

A 24-year-old university graduate student returned home to live with his CASE 9-5
parents so he could take his classes online due to the COVID-19
pandemic. He was seen yearly by his neurologist for migraine headache
and migrainous dizziness managed adequately with diet, exercise, and
infrequent use of low-dose diazepam but mentioned another complaint.
He noted that he was exceptionally bothered by the sound of other
people swallowing. He did not seem affected by the sound of his own
swallowing but was particularly bothered by hearing either of his parents
swallow. He was sometimes annoyed by the sounds of his friends
swallowing but felt his parents swallowed very loudly to the point that he
had to leave the room because it made him feel nauseated. He
periodically saw a psychologist for stress management who diagnosed
this as misophonia.

This case illustrates a common form of misophonia in a young adult. This COMMENT
patient was provided with stress-reduction techniques and education, but
no specific therapy was directed at the misophonia itself. The patient
eventually returned to his university and took his classes online from a
residence near the campus.

CONTINUUMJOURNAL.COM 505

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

otitis or other evident structural cause can be found on examination or by

temporal bone MRI or CT or both.

Anatomic Background
A variety of cranial nerves converges to supply sensation and function to the ear and
periauricular region: (1) the auricle receives innervation from cranial nerves V, VII,
X, and the second and third cervical nerve roots; and (2) the external ear canal,
tympanic membrane, and middle ear are supplied from cranial nerves V, VII, and X.
After adequately excluding the common otologic causes such as eustachian
tube dysfunction or inner, middle, or external ear disorders, causes of secondary
or referred pain should be considered. Such causes may include oromandibular
disorders including TMJ syndrome, hemicranial headaches, herpes zoster oticus,
postherpetic neuralgia, giant cell arteritis, and neuralgia affecting trigeminal,
geniculate, glossopharyngeal, sphenopalatine, occipital, and vagus nerves.
Glossopharyngeal (vagoglossopharyngeal) neuralgia is a rare cause of severe pain
in the back of the throat or side of the tongue or ear on one side, often in salvos of
sharp pains similar to those experienced by patients with the far more common

TABLE 9-5 Selected Bedside Tests of Hearing and Signs or Phenomena

Test, sign, or
phenomenon How it is performed Outcomes Implication

Conversational Note whether patient has Normal: no evident hearing loss; Screening observation for
hearing, finger rub trouble hearing abnormal: patient cannot hear hearing loss
examiner

Weber testa Vibrating tuning fork is placed Normal: patient hears the tuning Sound is louder on the side of
in the middle of the top of the fork equally on each side; conductive hearing loss and
patient’s forehead abnormal: patient hears the tuning away from the side of
fork louder on one side sensorineural hearing loss

Rinne testa Vibrating tuning fork is placed Normal: patient still hears the If sound is heard better by bone
on the mastoid until no longer tuning fork when it is placed next to than by air conduction, it
heard, then placed next to the the ear (air conduction is better); indicates conductive hearing
ear on the same side abnormal: patient cannot hear the loss on that side
tuning fork when it is placed next to
the ear (air conduction is worse
than bone conduction)

Tullio A loud sound or sustained Normal: no response; abnormal: Seen in canal dehiscence,
phenomenon sound is applied in the ear vertigo/dizziness, possibly Ménière disease, perilymphatic
nystagmus fistula, luetic otitis

Hennebert sign Application of positive or Normal: no response; abnormal: Seen in canal dehiscence,
negative pressure to the several beats of nystagmus Ménière disease, perilymphatic
sealed external ear canal fistula, luetic otitis

Autophony Not elicited by the examiner Patient reports hearing internal Increased hearing of one’s own
but asked about in the history bodily sounds voice or internal bodily sounds
(heartbeat, eye movements,
chewing, joint sounds)

a
A 512-Hz tuning fork, if available, is preferred over a 125-Hz or 256-Hz fork.

506 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

trigeminal neuralgia, but the pain is evoked by swallowing, talking, or coughing KEY POINTS
and sometimes associated with syncopal events when severe. Possible causes
● While phonophobia is an
include microvascular compression, multiple sclerosis plaque at the root entry aversion to loud sounds as
zone, tumors or nearby infections, local squamous cell carcinoma, or, less often, may occur during migraine
Eagle syndrome (stylalgia) in which the stylohyoid ligament stretches the headaches, hyperacusis is a
glossopharyngeal nerve. Treatment is similar to that for trigeminal neuralgia rare disorder with constant
intolerance to sounds of
with carbamazepine topping the list of medications and microvascular
ordinary loudness that do
decompression or stereotactic radiosurgery being reserved for severe symptoms not bother most people.
not responding to medications.
● Unilateral continuing
HEARING LOSS otalgia without an evident
structural cause can
When evaluating hearing loss, it is important to distinguish conductive hearing sometimes be attributable
loss from sensorineural hearing loss.29 Hearing loss can also be mixed (conductive to hemicranial headache
and sensorineural). In conductive hearing loss, amplification and transmission of disorders, herpes zoster
sound to the cochlea (external ear to the middle ear) are impeded. In sensorineural oticus, or postherpetic
neuralgia, giant cell arteritis,
hearing loss, processing of neural impulses and their transmission centrally (inner or neuralgia of cranial nerves
ear, cranial nerve VIII, or rarely central pathways) are affected. V, VII, IX, and X or of the
This section begins with otologic history and examination. Because vestibular sphenopalatine or greater
and auditory pathways are anatomically closely located, vestibular examination occipital nerves.
is essential. Vestibular examination is discussed further in “Approach to the
● In a patient presenting
History and Evaluation of Vertigo and Dizziness” by Terry D. Fife, MD, FAAN, with hearing impairment, the
FANS,30 and “Vestibular Testing” by Timothy C. Hain, MD, and Marcello first step is to distinguish
Cherchi, MD, PhD, FAAN,31 in this issue of Continuum. Conductive hearing loss conductive from
sensorineural hearing loss.
and its common etiologies are briefly reviewed here. Sensorineural hearing loss is
discussed in more detail, specifically how a neurologist should approach a patient
with acute or chronic, unilateral or bilateral sensorineural hearing loss. These
patients often present with dizziness or imbalance or both.

History and Examination of Hearing Loss

The clinician should start with a thorough history that includes the date of symptom
onset and symptom description. Was the hearing loss gradual or sudden in onset;
unilateral or bilateral; progressively worsening, stable, or fluctuating? Has dizziness
been an associated symptom at any time? Does the patient have an associated
history of ear pain, discharge, fullness, popping, autophony, hyperacusis, distortion
of sound, or tinnitus? If tinnitus is present, is it bilateral or unilateral, high pitched or
low pitched, pulsatile or nonpulsatile? If pulsatile, is it synchronous with the
heartbeat or not? Past medical history should include prenatal, perinatal, and
postnatal events; early childhood diseases; infections; head and acoustic
trauma/surgery; noise exposure; and the use of ototoxic medications. Family history
may be helpful, such as if a history of hearing loss or neurofibromatosis is noted.
TABLE 9-5 outlines some of the methods for bedside testing of hearing. Otologic
examination starts with noting if the patient can hear the examiner’s conversational
voice in a quiet room and if any loss of hearing of a finger rub is present.
Weber and Rinne testing can then help with localizing unilateral hearing
loss (conductive versus sensorineural). Weber testing is performed by placing
a 512-Hz vibrating tuning fork in the middle of the patient’s forehead. The
stimulus travels through the bone to bilateral acoustic nerves. With normal
hearing, the sound will not lateralize to either ear. The sound will radiate to the
impaired ear with conductive hearing loss and away from the impaired ear
with sensorineural hearing loss. Rinne testing is performed by placing a 512-Hz

CONTINUUMJOURNAL.COM 507

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

vibrating tuning fork on the mastoid process. When the sound is no longer
heard by the patient, the tuning fork is placed in front of the auditory canal. If
the sound is not heard in this position, bone conduction is better than air
conduction, indicating conductive hearing loss. Air conduction would be expected
to be stronger than bone conduction in an individual with normal hearing. It is
recommended to mask the sound input to the ear that is not being tested by
crinkling paper in front of that ear.
Otoscopic inspection of the auditory canal and tympanic membrane is
essential. The examiner should assess for obstruction, otitis media,
cholesteatoma, and vesicular eruptions suggestive of herpes zoster. If on
otoscopy anything looks other than normal, a consultation with an
otolaryngologist should be considered. Images of normal and pathologic
tympanic membranes can be found at diagnosis101.welchallyn.com/otoscopy/
educational-topics/ear-pathologies/.
Otologic examination is then followed by detailed vestibular examination. For
more information about the vestibular examination, refer to “Approach to the
History and Evaluation of Vertigo and Dizziness” by Terry D. Fife, MD, FAAN,
FANS,30 in this issue of Continuum.

TESTING. Audiometry, vestibular and laboratory testing, and imaging are used to
confirm and further investigate the potential diagnosis.

AUDIOMETRY. Although bedside hearing tests have good specificity for detecting
hearing loss, the sensitivity is poor when compared with an audiogram, so an
audiogram is the optimal test for the assessment of hearing loss.32 When ordering
an audiogram, the suspected diagnosis should be mentioned, and air-bone gap
testing should be requested because it is not always automatically included.

VESTIBULAR TESTING. Depending on the working diagnosis, videonystagmography

with caloric testing and ocular and cervical VEMPs can reveal vestibular
function information not detected during the bedside examination. The video
head impulse test is a quantitative functional assessment of the function of each
of the three semicircular canals on each side. The bedside head impulse test can
help in detecting vestibular hypofunction related to the horizontal semicircular

TABLE 9-6 Recommendations and Appropriateness of Initial Imaging for Acquired

Sensorineural Hearing Lossa

Appropriateness
Procedure category

Head and internal auditory canals MRI with and without contrast Usually appropriate

Temporal bone CT with or without contrast May be appropriate

Head CT with or without contrast, temporal bone CT with and without contrast, head CT Usually not appropriate
angiography, magnetic resonance venography, head magnetic resonance angiography

CT = computed tomography; MRI = magnetic resonance imaging.

a
Data from Expert Panel on Neurologic Imaging, J Am Coll Radiol.34

508 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

canal on one or both sides. Vestibular testing is discussed in detail in the article KEY POINT
“Vestibular Testing” by Timothy C. Hain, MD, and Marcello Cherchi, MD, PhD,
● Vestibular schwannoma
FAAN,31 in this issue of Continuum. can occasionally present
with sudden unilateral
LABORATORY TESTING. Most recent clinical practice guidelines for sudden hearing hearing loss, although it is
loss from the American Academy of Otolaryngology–Head and Neck Surgery do usually more gradual and
not recommend routine laboratory testing.29 History and physical examination progressive.
findings should guide a clinician to targeted laboratory testing, such as serologic
testing when considering autoimmune and infectious diseases, and gap junction
beta-2 protein for connexin 26 mutation, a common cause of autosomal
recessive sensorineural hearing loss. Several other genes have been found to be
associated with hearing loss.33 For specific genes, refer to Human Phenotype
Ontology Sensorineural Hearing Impairment at hpo.jax.org/app/browse/term/
HP:0000407 and consult with a geneticist.

IMAGING. TABLE 9-634 outlines initial imaging studies for acquired sensorineural
hearing loss as studies that are rated for appropriateness. Imaging should be
performed to confirm or narrow the list of suspected diagnoses based on history
and examination. Therefore, specific recommendations are listed in the
discussion of different conductive and sensorineural hearing loss etiologies.

Conductive Hearing Loss

In conductive hearing loss, air transmission of sound waves to the inner ear
endolymph is impeded. This results in an air-bone gap on the audiogram. With a
gap of 15 dB or more, conductive hearing loss is suspected. Common etiologies of
conductive hearing loss are listed in TABLE 9-7.35-39

Sensorineural Hearing Loss

In patients with sensorineural hearing loss, one should determine whether it is
unilateral or bilateral, constant or episodic, or fluctuating. In patients with
sudden sensorineural hearing loss, audiometry should be obtained as soon as
possible (within 14 days of symptom onset). Retrocochlear pathology is best
evaluated with brain MRI with attention to the internal auditory canal.29 It is not
recommended to order routine CT of the head in the initial evaluation or to
obtain routine laboratory tests.29,34

SUDDEN UNILATERAL SENSORINEURAL HEARING LOSS. Most commonly, sudden

unilateral sensorineural hearing loss is idiopathic. The most common etiologies of
an abrupt-onset audiovestibular syndrome include labyrinthitis, Ménière
disease, temporal bone fracture, and labyrinthine infarction. Hearing and
vestibular loss are usually very gradual and insidious in cases of vestibular
schwannoma but may be acute in approximately 1% of cases.29

IDIOPATHIC SUDDEN SENSORINEURAL HEARING LOSS. Isolated sudden sensorineural

hearing loss without vestibular symptoms is referred to as idiopathic sudden
sensorineural hearing loss and is most commonly unilateral. The cause is often
undetermined, but most cases are believed to be due to viral infection. Despite
the presumed viral cause, the majority of patients do not report preceding viral
upper respiratory infection symptoms. Other less common causes include
immune-mediated mechanisms; cochlear membrane rupture, as may occur with
barotrauma; cochlear infarction; and possibly iron deficiency anemia. Treatment

CONTINUUMJOURNAL.COM 509

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

with pulse methylprednisolone (1000 mg/d IV for 3 days) or intratympanic

corticosteroids is recommended within the first 7 days of onset, but the earlier
administration begins, the better.

LABYRINTHITIS. Labyrinthitis (vestibular neuritis plus unilateral sensorineural

hearing loss) is typically of viral etiology and usually unilateral. Less often,
bacterial, spirochetal, and autoimmune mechanisms may be involved. Systemic
infections have greater likelihood of bilateral involvement. Bacterial labyrinthitis
can be a complication of meningitis and otitis media. On examination, usually
unilateral sensorineural hearing loss is present; nystagmus is unidirectional with
the fast phase of nystagmus beating away from the impaired ear. The nystagmus
adheres to the Alexander law, beating more intensely in the direction of the fast

TABLE 9-7 Etiology of Conductive Hearing Loss

Location Etiology Comments and/or typical presentations

External ear Impacted cerumen Common

ear

Foreign body Immense ear discomfort and itchiness; on examination, a live spider lodged in
the ear canal

Auricular malformations Congenital or posttrauma

Infections Long-standing left ear pain, itching, and poor hearing; on examination,
edematous, erythematous, foul-smelling discharge; edema partially occluding
ear canal and discharge occluding tympanic membrane

Neoplasms Poor hearing bilaterally; on examination, multiple sessile protrusions on the bony
canal near the drum consistent with bony exostoses35

Middle ear Effusion Common; tympanic membrane often appears dull and yellow, with air fluid level
ear (air bubbles) behind the drum seen during otoscopy

Barotrauma Can result in tympanic membrane perforation, hemotympanum

Otitis media with or without Chronic otitis media associated with eustachian tube dysfunction can result in
cholesteatoma36 acquired cholesteatoma; a cholesteatoma is an abnormal noncancerous but
locally invasive collection of squamous cells behind the eardrum; these growths
can extend into the middle ear and erode ossicles resulting in conductive
hearing impairment or erode into the middle ear damaging the cochlea; on
otoscopy, expect gray-white keratin mass on or behind the tympanic
membrane; otorrhea is commonly purulent and can be foul smelling

Neoplasm (rare) Progressive left hearing loss and left pulsatile tinnitus; on examination, red
pulsating mass behind the left tympanic membrane, a systolic bruit over the left
mastoid, and left facial nerve involvement; diagnosis is glomus tympanicum
(rarely metastasize; can also involve cranial nerves IX to XII); differential for red
pulsating mass in the middle ear includes high-riding jugular bulb (conductive
hearing loss from sound transmission through the middle ear), dehiscent jugular
bulb, aberrant intratympanic carotid artery, and persistent stapedial artery37,38

Otosclerosis Abnormal bone remodeling of the otic capsule resulting in progressive fixation
of stapes footplate in the oval window, with extension to the cochlea, where
spiral ligament atrophies and hyalinizes with subsequent bilateral mixed hearing
loss39; autosomal dominant with incomplete penetrance; vestibular symptoms
sometimes occur after stapes surgery

510 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

phase and less during gaze away from the fast phase. Head impulse shows KEY POINTS
catch-up saccades ipsilesionally. No skew deviation, which is more suggestive of
● When acute unilateral
a central mechanism, should be observed. hearing loss is present,
labyrinthine ischemia should
LABYRINTHINE INFARCTION. The labyrinthine artery usually arises from the anterior be considered, but a
inferior cerebellar artery (AICA) (rarely from the basilar artery) (FIGURE 9-3). viral/inflammatory cause is
The AICA also provides vascular supply to some vestibular nuclei, the lateral considered to be more
common.
pons, middle cerebellar peduncle, flocculus, inferior lateral cerebellum, and
facial nerve. The labyrinthine artery branches off at the labyrinth to supply the ● Occlusion of the
vestibulocochlear nerve, semicircular canals, utricle, and saccule. Depending on posterior inferior
where in its course the AICA is occluded, it may cause a combination of cerebellar artery can
symptoms, such as hearing loss, dizziness (central or peripheral vestibular), rarely cause acute
audiovestibular symptoms.
ipsilateral lower motor neuron facial palsy, ipsilateral limb ataxia, and postural
imbalance, among other symptoms. Isolated labyrinthine ischemia is thought to ● If any part of HINTS (Head
be rare and may be difficult to distinguish from viral labyrinthitis (CASE 9-6). Impulse, Nystagmus, Test of
Sudden unilateral hearing loss thus could precede the impending infarction Skew) suggests central
localization, the etiology
of the cerebellum or ipsilateral pontomedullary region. Occlusion of the
must be assumed to be
posterior inferior cerebellar artery (PICA) can rarely cause similar audiovestibular central until proven
symptoms. HINTS (Head Impulse, Nystagmus, Test of Skew) is sensitive and otherwise.
specific. When performed by an experienced clinician, it is superior to early MRI;
early MRI may miss a small infarction because of slice thickness or because the ● Isolated labyrinthine
stroke is unlikely to be
MRI, if obtained early enough, may not yet show diffusion restriction.40 visible on standard MRI.

CHRONIC UNILATERAL SENSORINEURAL HEARING LOSS. This section focuses on

adult-onset chronic unilateral sensorineural hearing loss. Ménière disease

FIGURE 9-3
Vascular supply to the membranous labyrinth.
Reprinted with permission from Barrow Neurological Institute. © 2007 Barrow Neurological Institute,
Phoenix, Arizona.

CONTINUUMJOURNAL.COM 511

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

(endolymphatic hydrops) and vestibular paroxysmia typically present with

fluctuating auditory symptoms and episodic vertigo. Although Ménière disease
symptoms typically last a few hours; in vestibular paroxysmia, the episodes are
much shorter, lasting seconds to minutes at a time. Cochlear Ménière disease
lacks vestibular symptoms, is rare, and is more difficult to diagnose. Systemic
processes, such as autoimmune disease, may rarely cause endolymphatic
hydrops, but it is more likely to be bilateral. In sarcoidosis, fluctuating
sensorineural hearing loss and vertigo can occasionally be the only presenting
symptoms. A chest radiograph to look for hilar adenopathy and referral to
ophthalmology to look for uveitis should be considered. Hearing usually
improves with steroid therapy. Acquired and congenital syphilis can present in
adults as fluctuating or persistent unilateral or bilateral sensorineural hearing
loss. Patients should be referred to ophthalmology where they can be evaluated
for interstitial keratitis typically seen in congenital syphilis; patients should be
evaluated for Hutchinson teeth, as well. Fluorescent treponemal antibody testing
should be considered because venereal disease research laboratory testing is
often normal. Testing and management of acquired syphilitic otitis are similar to
that of neurosyphilis. Vestibular schwannoma, meningioma, ependymoma,
cerebellar medulloblastoma, perineural spread of parotid gland adenoid cystic
carcinoma and salivary duct carcinoma or cutaneous squamous cell carcinoma
(in patients with a history of facial/scalp malignancy), and other
cerebellopontine angle mass lesions more commonly present with slow
progression of auditory symptoms and vertigo. Noise-induced hearing loss is not
rare; middle and high frequencies are usually affected first, followed by
sensorineural hearing loss across all frequencies with continued exposure.
Although it is often bilateral, unilateral cases are possible.

CASE 9-6 A 68-year-old woman presented to the emergency department with an

acute onset of vertigo, vomiting, imbalance, and muffled hearing on the
left. Examination showed mild right-beating spontaneous nystagmus. Her
head impulse test was without catch-up saccades (ie, normal). No skew
deviation was present. The rest of the vestibular examination was normal.
Tuning-fork testing was consistent with sensorineural hearing loss on the
left. Her gait was ataxic. Brain MRI was normal. Her symptoms continued
to persist, and, on reexamination the next day, she was found to have left
upper extremity dysmetria, gaze-evoked nystagmus, and saccadic
pursuit. Repeat MRI showed an acute cerebellar infarct consistent with
an anterior inferior cerebellar artery (AICA) territory stroke.

COMMENT This is a case of labyrinthine stroke (from the AICA occlusion) with
additional posterior fossa ischemia that progressed beyond just the
labyrinth by the next day to include the cerebellum. Completely isolated
labyrinthine ischemia can occur, but depending on the location of the
thrombosis, it may also involve the AICA distribution of the cerebellum
and/or lateral pons.

512 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

MÉNIÈRE DISEASE (ENDOLYMPHATIC HYDROPS). For more information, refer to the KEY POINT
article “Episodic Spontaneous Vertigo” by Scott D. Z. Eggers, MD,41 and for the
● Ménière disease presents
putative causes of Ménière disease, refer to the article “Selected Otologic
with an abrupt onset of
Disorders Causing Dizziness” by Gail Ishiyama, MD,25 in this issue of recurrent vertiginous
Continuum. attacks with associated
The typical presentation of Ménière disease includes recurrent spontaneous fluctuating unilateral
low-frequency
episodes of unilateral auditory symptoms such as hearing loss, ear fullness,
sensorineural hearing loss.
and nonpulsatile tinnitus, with associated vertigo. Auditory symptoms may
precede vertigo, occur during it, or follow it. Vertigo lasts at least 20 minutes
and no longer than 24 hours. Although vertigo typically resolves within 2 to
6 hours, hearing loss may take longer to resolve, sometimes with incomplete
recovery. In the early stages of disease, low- to midfrequency sensorineural
hearing loss is more common. Hearing loss is usually progressive and
permanent and eventually involves all frequencies. As auditory symptoms
tend to fluctuate in the early stages of the disease, an audiogram should be
done soon after the attack. The lack of sensorineural hearing loss on
audiogram does not rule out Ménière disease, although, after a couple of years
of recurrent symptoms, permanent sensorineural hearing loss on audiogram is
expected. Delayed or absent cervical VEMPs can be seen in Ménière disease.
Ordering antinuclear antibody, erythrocyte sedimentation rate, thyroid
function, testing for Lyme disease, and fluorescent treponemal antibody tests
should be considered because systemic processes may rarely cause
endolymphatic hydrops.
In the majority of patients, vertiginous attacks will spontaneously remit.
Preventive treatment is stepwise starting with a low-sodium diet, ideally less
than 1500 mg/d, and a diuretic such as hydrochlorothiazide-triamterene
25 mg/37.5 mg. If vertigo attacks continue, consider high-dose betahistine
(eg, 16 mg 3 times a day to 32 mg 3 times a day) therapy. Betahistine is used in
Europe but is not approved in the United States; it can be ordered through a
compounding pharmacy. At the onset of a typical Ménière attack, the patient
can use promethazine, prochlorperazine, or low-dose benzodiazepines.
Meclizine is less effective with severe symptoms. To allow central
compensation, long-term use of vestibular suppressants is not recommended.
Intratympanic dexamethasone shortly after the attack may reduce the
frequency of the vertiginous attacks, although supportive evidence for this
approach is weak. As the disease progresses, patients can experience abrupt
drop attacks without loss of consciousness, as if someone pushed them to the
ground (otolithic crises of Tumarkin). At this point of disease progression,
intratympanic gentamicin injections may be considered; hearing loss is less
likely with lower doses. In a minority of cases, the contralateral ear can
become involved to cause bilateral Ménière disease, especially if caused
by an autoimmune mechanism (TABLE 9-8). Most cases of Ménière disease
remain unilateral. Of note, patients with Ménière disease are more likely to
have coexistent vestibular migraine, especially with bilateral Ménière disease.

VESTIBULAR SCHWANNOMA. Vestibular schwannoma is a benign neoplasm derived

from Schwann cells of the vestibular portion of cranial nerve VIII. Most
vestibular schwannomas are sporadic and incidentally found on imaging. A few
are associated with neurofibromatosis type 2, especially if bilateral vestibular
schwannomas are present. In the internal auditory canal, a compressed cochlear

CONTINUUMJOURNAL.COM 513

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

TABLE 9-8 Sensorineural Hearing Loss Etiologies

Toxic/metabolic
◆ Acetyl salicylic acid >2.5 g/d
◆ Alcohol
◆ Alkalizing agents
◆ Aminoglycosides (eg, gentamicin)
◆ Antivirals
◆ Benzodiazepines
◆ Bisphosphonates
◆ Chemotherapeutic agents
◆ Cocaine
◆ Ecstasy
◆ General anesthetics
◆ Heroin
◆ Immunosuppressive drugs
◆ Insecticides
◆ Loop diuretics
◆ Nonsteroidal anti-inflammatory drugs
◆ Organic mercury
◆ Pegylated interferon
◆ Retinoid
◆ Skeletal muscle relaxants
◆ Synthetic prostacyclin
◆ Uremia
Genetic (nonsyndromal are more prevalent)
◆ Alport syndrome
◆ Autosomal dominant cerebellar ataxia type 1
◆ Bone dysplasias
◆ Branchio-oto-renal syndrome
◆ Charcot-Marie-Tooth disease
◆ Chiari malformation
◆ Friedreich ataxia
◆ Gaucher disease
◆ GJB2 mutation: the most common cause of nonsyndromic autosomal recessive hereditary
hearing loss; GJB2 encodes for the gap junction protein beta 2 connexin 26
◆ Hurler syndrome
◆ Jervell and Lange-Nielsen syndrome

CONTINUED ON PAGE 515

514 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CONTINUED FROM PAGE 514

◆ Mitochondriopathies:
◇ Mitochondrial encephalomyopathy with lactic acidosis and stroke like episodes
◇ Progressive external ophthalmoplegia
◇ Mitochondrial neurogastrointestinal encephalomyopathy
◇ Myoclonic epilepsy with ragged red fibers
◆ Neurofibromatosis type 2
◆ Pendred syndrome
◆ Perrault syndrome
◆ Refsum syndrome: hearing loss can present in adulthood
◆ Spinocerebellar ataxia 7
◆ Spinocerebellar ataxia 31
◆ Spinocerebellar ataxia 36
◆ Stickler syndrome
◆ Treacher Collins syndrome (sometimes hearing loss is due to microtia, atresia of the
external ear)
◆ Usher syndrome
◆ Waardenburg syndrome
Immune
◆ Behçet disease: bilateral sensorineural hearing loss (rarely unilateral); vestibular
symptoms can be the initial manifestation of the disease; mimics vestibular neuritis
◆ Cogan syndrome: recurrent episodic sensorineural hearing loss (unilateral or bilateral,
fluctuating or progressively worsening over time, profound in half of the patients)
◆ Giant cell arteritis: unilateral sensorineural hearing loss can be an initial manifestation of
the disease; it can progress in severity and can involve the other ear
◆ Granulomatosis with polyangiitis conductive hearing loss from obstruction of the eustachian
tube by granulomas in the nasopharynx and/or sudden irreversible low-frequency
sensorineural hearing loss, which can be an initial manifestation of the disease
◆ Guillain-Barré syndrome (rarely with hearing loss)
◆ Multiple sclerosis (rarely with hearing loss)
◆ Relapsing polychondritis: sudden unilateral or bilateral sensorineural hearing loss
◆ Rheumatoid arthritis: mostly sensorineural hearing loss
◆ Sarcoidosis: common sensorineural hearing loss (unilateral or bilateral), rare conductive
hearing loss
◆ Sjögren syndrome: sensorineural hearing loss, rarely is an initial manifestation of the disease
◆ Systemic lupus erythematosus: sensorineural hearing loss, occasionally sudden in onset in
patients with high titer of anticardiolipin antibodies
◆ Systemic sclerosis (scleroderma): sensorineural hearing loss, conductive hearing loss or mixed
◆ Vogt-Koyanagi-Harada disease: bilateral rapidly progressive sensorineural hearing loss

CONTINUED ON PAGE 516

CONTINUUMJOURNAL.COM 515

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

CONTINUED FROM PAGE 515

Infectious
◆ Bacterial meningitis (Neisseria, Pneumococcus, Hemophilus, Mycobacterium tuberculosis)
◆ Fungal (eg, cryptococcosis, coccidiomycosis)
◆ Spirochetal (eg, syphilis, Borrelia)
◆ Viral (eg, human immunodeficiency virus [HIV], herpes simplex virus, herpes zoster,
cytomegalovirus), paramyxovirus (mumps)
Neoplastic
◆ Acoustic neurofibroma
◆ Cerebellopontine angle and petrous meningioma and metastasis
◆ Leptomeningeal carcinomatosis or lymphomatosis
◆ Meningeal carcinoma
◆ Myelodysplastic syndrome–associated hypercoagulability
◆ Neurolymphomatosis
◆ Vestibular schwannoma
Vascular
◆ Sickle cell crisis
◆ Stroke
Neurodegenerative
◆ Multiple system atrophy: unilateral or bilateral higher-frequency sensorineural hearing loss
◆ Parkinson disease: unilateral or bilateral higher-frequency sensorineural hearing loss
Iatrogenic
◆ Aminoglycoside or other ototoxic medication administration
◆ General anesthetic hemodynamic complication
◆ Microembolism from surgical complications
◆ Radiation
Other
◆ Bilateral Ménière disease
◆ Histiocytosis X: chronic bilateral
◆ Intracranial hypotension
◆ Superficial siderosis: chronic bilateral

516 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

portion of cranial nerve VIII results in progressive hearing loss and nonpulsatile KEY POINTS
tinnitus.42 Facial paresthesia and weakness can be seen when larger tumors
● Intracranial hypotension
compress the nearby facial nerve. With cerebellopontine angle involvement, can present with tinnitus,
vestibular schwannomas may also compress the brainstem and cerebellum. altered hearing, dizziness,
Diagnosis is made through brain MRI with contrast with attention to the or vertigo.
internal auditory canal, audiometry, and videonystagmography. Vestibular
● Among the more
schwannomas are discussed further in the article “Vertigo Related to
prevalent etiologies of
Central Nervous System Disorders” by Kamala Saha, MD,43 in this issue chronic bilateral
of Continuum. sensorineural hearing loss
are age-related hearing loss,
SUDDEN BILATERAL SENSORINEURAL HEARING LOSS (ONSET LESS THAN 1 MONTH). heritable factors, and noise
exposure.
Bilateral sensorineural hearing loss is less often associated with vestibular
symptoms and is less likely to recover compared with unilateral sudden
sensorineural hearing loss.44 Systemic processes, such as toxic, metabolic,
immune-mediated, vascular, neoplastic, and infectious disease should be
considered as a possible cause (TABLE 9-8).
Intracranial hypotension, whether spontaneous or from overshunting from a
ventriculoperitoneal shunt, may be associated with audiovestibular symptoms.
The sensorineural hearing loss can be unilateral or bilateral, or hearing may be
distorted, but it is not usually a complete hearing loss.45,46 Sensorineural hearing
loss can involve all frequencies and is generally thought to be due to mild sagging
of the brain and traction on the vestibulocochlear nerve(s) and disturbed
perilymphatic-CSF dynamics within the labyrinth.

CHRONIC BILATERAL SENSORINEURAL HEARING LOSS. This is the most common

category of bilateral sensorineural hearing loss. The three main causes of
chronic sensorineural hearing loss in adults are (1) age-related hearing loss
(FIGURE 9-447), (2) heritable factors, and (3) noise exposure, which is the most
common preventable cause. These three contributing causes are not mutually
exclusive. In other words, as one ages, hearing diminishes in many people,
possibly on a heritable basis, but noise exposure can accelerate the progression
and severity of hearing loss. Nevertheless, some older people maintain excellent
hearing, so it does not affect everyone equally as they age.
As mentioned earlier, sensorineural hearing loss is often associated with
tinnitus. Hearing loss tends to make it more difficult to distinguish voices
in the foreground from ambient background noise. Thus, it may be more
difficult to hear on the telephone, in a moving car, in restaurants, and when
many people are talking in a room, and certain sounds seem tinny and less
rich and full. Certain parts of speech are harder to hear, such as consonant
sounds including F, S, V, S, SH, and ZH (eg, the sound of the S in measure).
Meanwhile, some sounds may seem especially loud or distracting. Finally, the
direction from which a high-pitched sound emanates may be more difficult to
ascertain.
The number of differential diagnoses for chronic bilateral sensorineural
hearing loss is large (some are included in TABLE 9-8). Hereditary causes are
many and beyond the scope of this article48; the Hereditary Hearing Loss website
is a good source of information about nonsyndromic and less common syndromic
etiologies of hereditary hearing loss (hereditaryhearingloss.org). The Human
Phenotype Ontology bilateral sensorineural hearing impairment website is
another helpful resource (hpo.jax.org/app/browse/term/HP:0008619).

CONTINUUMJOURNAL.COM 517

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

FIGURE 9-4
Relationship between age and hearing loss in the United States from 2001 to 2008. Hearing
loss is defined as greater than 25-dB thresholds from 500 Hz to 4000 Hz in the better-hearing
ear. Note that the prevalence of hearing loss approximately doubles every decade of life
between the 12- to 17-year and 70- to 79-year age groups.
Modified with permission from Yamasoba T, et al, Hear Res.47 © 2013 Elsevier B.V.

MANAGEMENT OF SENSORINEURAL HEARING LOSS. The management of disorders

29
that cause hearing loss varies depending on its etiology. Regarding the hearing
loss itself, intratympanic dexamethasone injections by an otolaryngologist or
high-dose IV corticosteroid therapy in patients with incomplete recovery from
sudden sensorineural hearing loss should be considered as soon as possible after the
onset of symptoms. No medications, diet, nutritional supplements, exercises, or
therapies have been established to help chronic sensorineural hearing loss. Known or
suspected ototoxic medications should be stopped. If an underlying autoimmune
cause is identified, then immunotherapy can be instituted, although it is rarely
successful if hearing loss has been present and is nonfluctuating for longer than
1 year.
Determining the frequency, severity, and evolution of sensorineural hearing
loss is best done by obtaining an audiogram and monitoring with serial
audiograms when indicated. The following sections are a neurologist’s primer for
interpreting an audiogram and include a simplified overview of options for
hearing augmentation from hearing aids to cochlear implants. Patient education
is valuable to help set expectations and advise on treatment risks and benefits
and to allay any patient anxiety.

NEUROLOGIST’S PRIMER FOR INTERPRETING AN AUDIOGRAM

An audiogram is a graphical representation of human hearing (FIGURE 9-5).
Across the top are the pure tone frequencies. Frequency is the pitch, measured in
Hertz (Hz), that is, cycles per second of the sound vibration. For human hearing,
the pure tones range from 250 Hz (low pitched sounds) to 8000 Hz
(higher-pitched sounds) is assessed. Of course, sound frequencies are much
broader, but this is what is used in standard hearing tests.

518 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

FIGURE 9-5
Blank audiogram form. Pure tone frequencies in hertz (Hz) are aligned across horizontally
(x-axis), and decibels (dB hearing level) of hearing normalized to humans are on the vertical
(y-axis). Masked refers to application of some noise to the ear opposite the one being tested
to avoid the confounding effect of hearing from the ear not being tested.
AC = air-conducted hearing; BC = bone-conducted hearing; Discrim = speech discrimination; SRT = speech
reception threshold.

Down the vertical axis of FIGURE 9-5 is the intensity, which refers to the
loudness and is measured in decibels hearing level (dB HL) and often ranges
from -10 dB to 120 dB in gradations of 10 dB. A decibel is 1/10 of a bel, a unit of
intensity of sound. The dB HL measurements on the y-axis are normalized to
human hearing, so 0 dB does not mean no sound is present; it simply means no
amplification of sound above idealized human hearing.
The audiologist uses an audiometer to present the pure tones via headphones
or inserted earphones in a sound-proof booth to test pure tone thresholds, that is,
the quietest sound at a given frequency that the person can hear at least half the
time. This is done sequentially in each ear at each of the specified frequencies and
plotted as pure tone threshold values. By convention, X represents the left, and
O represents the right ear for air-conducted sounds. To bypass the natural pathway
of sound and test the hearing directly at the cochlea, the audiologist presents
tones via bone conduction by placing a bone oscillator on the mastoid bone of
each ear sequentially. A differential of 25 dB or more between the threshold for
bone- and air-conducted sound thresholds is referred to as the air-bone gap and may
suggest conductive hearing loss (as opposed to sensorineural hearing loss).
Threshold values of 0 dB to 20 dB are considered normal, 25 dB to 40 dB mild, 41 dB
to 55 dB moderate, 56 dB to 70 dB moderately severe, and 71 dB to 90 dB severe
hearing loss; threshold values greater than 90 dB indicate hearing loss is profound at
that frequency.
Masking is applying some narrow-band noise in the ear not being tested to
prevent that ear from hearing sound applied to the ear being tested. Masking

CONTINUUMJOURNAL.COM 519

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

usually is done when greater than 10 dB or more air-bone gap is present or when
one ear hears much better than the other (eg, a difference of 40 dB or more). By
convention [ denotes the right ear (AD) and ] denotes the left ear (AS) for
bone-conducted thresholds.
Other tests used include speech reception threshold and speech discrimination
score. With the speech reception threshold, a list of two-syllable words is given
at the lowest reception threshold at which the individual can recognize speech
and represents the threshold at which at least half the words are identified
correctly in a quiet environment. The speech discrimination score measures how
well the patient can hear speech that is loud enough to hear comfortably. A
speech discrimination score of 100% means the individual understands all the
words spoken.

HEARING AUGMENTATION
Hearing augmentation generally either amplifies the sound or provides some
way to circumvent the dysfunctional part of the peripheral auditory system in
those with hearing loss. TABLE 9-949,50 outlines some of the ever-growing list
of ways to improve hearing in patients with hearing loss. A few consistent
guidelines are available for objective measures of the degree of hearing loss
that warrant a hearing aid, but for most people, it is tied to the ability to
understand speech.

Cochlear Implants
A cochlear implant is a surgically implanted device that converts sound into
electrical impulses that are delivered to the auditory nerve by electrodes
implanted within the lumen of the cochlea. This bypasses the cochlear hair cells.
The components include an external microphone, sound processor and
transmitter, and an internal receiver and electrode array extending from the
receiver through the round window into the cochlea.

Indications
For children with severe or profound hearing loss from birth, hearing
augmentation is ideally initiated before language develops. If cochlear implants
are placed sequentially, it is best to keep the time between placements to less than
18 months.51 Because bilateral hearing improves overall hearing and spatial
localization through binaural summation within central auditory processing
pathways, it is often advised that cochlear implants and other hearing
augmentation be applied in both ears even if bilateral hearing loss is
asymmetrical. For adults, cochlear implant surgery has no upper age limit
provided the patient does not have dementia. Adult candidates for cochlear
implantation should have severe to profound bilateral sensorineural hearing
loss that negatively impacts communication and cannot be sufficiently improved
by hearing aids. Some insurance coverage policies add that hearing must be at
least 70 dB or worse at 500 Hz, 1000 Hz, and 2000 Hz to qualify for payment
coverage.
Cochlear implants allow patients with severe hearing loss to hear sound,
which is different from hearing aids that just amplify sound. Because the sounds
are translated by a sound processor and direct nerve stimulation, differences
in sound quality occur, and it requires some practice hearing while seeing text
to improve familiarity with how these sounds correlate to previously heard

520 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

language. Cochlear implants made by all manufacturers are approved as “MRI
conditional,” meaning that certain measures must be followed to assure the
magnetic resonance study is done safely. In addition, the electrode leads do
not become hot in ordinary magnetic resonance units because of the short length
of electrode leads.
Two 2019 studies explored the use of a vestibular prosthesis either alone52 or
combined with a cochlear implant53 with multichannel vestibular electrodes

Various Hearing Augmentation Options TABLE 9-9

Type of hearing aid Description Indication

Receiver-in-canal A small receiver in the ear canal is connected by a thin Mild to severe hearing loss
wire to the electronic device that is located behind the adversely affecting quality of life;
ear; the receiver is in the ear canal but does not usually at least 40-dB hearing loss
completely seal the canal; sound detected by the at three frequencies, especially in
receiver in the ear canal is amplified the 2000-Hz to 4000-Hz range

Behind-the-ear Custom molds in the ear canal deliver sound Mild to severe hearing loss
transmitted by a tube from the electronic device that is adversely affecting quality of life;
located behind the ear usually at least 40-dB hearing loss
at three frequencies, especially in
the 2000-Hz to 4000-Hz range

In-the-ear Hearing devices that fill the external ear canal and are Mild to severe hearing loss
larger and more visible than invisible-in-canal and adversely affecting quality of life;
completely-in-the-canal types usually at least 40-dB hearing loss
at three frequencies, especially in
the 2000-Hz to 4000-Hz range

Invisible-in-canal and Invisible-in-canal hearing devices placed deep in the Mild to severe hearing loss
completely-in-canal ear canal and removed by tugging on a small plastic adversely affecting quality of life;
string; completely-in-canal devices are very similar usually at least 40-dB hearing loss
at three frequencies, especially in
the 2000-Hz to 4000-Hz range

Direct-to-consumer Mostly receiver-in-canal and behind-the-ear devices Perceived mild to moderate hearing
that may be purchased by consulting a doctor or loss adversely affecting quality of
audiologist; variability in quality; the US Food and Drug life50
Administration (FDA) Reauthorization Act of 2017
developed FDA standards and package labels for over-
the-counter hearing aids that as of the time of
publication of this article are still pending49

Contralateral routing of Contralateral routing of signals is a system with a Severe hearing loss in one ear and
signals, bilateral routing of microphone in the deaf ear that wirelessly transmits little or no hearing loss in the other
signals sound from the deaf side to the normal-hearing ear; ear (single-sided deafness)
bilateral routing of signals is the same but with some
amplification also in the better-hearing ear

Bone-anchored A titanium screw is anchored into the bone behind the Single-sided deafness or chronic
poor-hearing ear, and a titanium abutment attaches conductive hearing loss as long as
from the bone-anchored screw so it protrudes outside the cochlea on one side functions
of the skin or via a subdermal magnet it attaches to a at least at a moderate hearing level
sound processor that transmits sound through the skull
bone to the cochlea on both sides

CONTINUUMJOURNAL.COM 521

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

(FIGURE 9-6) for patients with severe bilateral peripheral vestibular

loss without or with profound bilateral hearing loss. Preliminary results
suggest that sustained canal-ocular vestibulo-ocular function can be
demonstrated,52,54 although it remains to be determined if this translates to
meaningful clinical improvement for patients. Vestibular prosthesis, either
alone or added to a cochlear implant, is still investigational at this time.

CONCLUSION
Tinnitus and hearing loss are common conditions affecting people of all ages,
although more commonly older people. This article is intended to be a guide to
approaching tinnitus, hyperacusis, idiopathic otalgia, and hearing loss and
introduce neurologists to the basics of audiometry and hearing augmentation. It
is hoped that this serves as a primer for organizing and understanding these
conditions and for recognizing important neurologic disorders that might be
associated with these symptoms.

FIGURE 9-6
Depiction of how a combined cochlear implant and vestibular prosthesis system would work.
Systems in development include a vestibular implant without or with a cochlear implant. Not
all necessary ground and reference electrodes are shown. The electrode in red is for a
cochlear implant, which is an established device; the electrode in blue for a hypothetical
vestibular prosthesis is still investigational.
Reprinted with permission from Barrow Neurological Institute. © 2020 Barrow Neurological Institute, Phoenix,
Arizona.

522 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

USEFUL WEBSITES
BILATERAL SENSORINEURAL HEARING IMPAIRMENT ONTOLOGY SENSORINEURAL HEARING IMPAIRMENT
This Human Phenotype webpage provides a list This Human Phenotype webpage provides a list of
of diseases and genes associated specifically diseases and genes associated with hearing loss.
with bilateral sensorineural hearing loss. hpo.jax.org/app/browse/term/HP:0000407
hpo.jax.org/app/browse/term/
HP:0008619
OTOSCOPIC PATHOLOGIES
This webpage briefly illustrates some important
HEREDITARY HEARING LOSS HOME PAGE common pathologies and compares them with what
This site lists data and links for all known gene will be observed in a normal ear canal and tympanic
localizations and identifications for monogenic membrane.
nonsyndromic hearing impairment. diagnosis101.welchallyn.com/otoscopy/
hereditaryhearingloss.org educational-topics/ear-pathologies/

REFERENCES

1 Mahboubi H, Lin HW, Bhattacharyya N. 12 In 't Veld M, Fronczek R, de Laat JA, et al. The
Prevalence, characteristics, and treatment incidence of cranial arteriovenous shunts in
patterns of hearing difficulty in the United States. patients with pulsatile tinnitus: a prospective
JAMA Otolaryngol Head Neck Surg 2018;144(1): observational study. Otol Neurotol 2018;39(5):
65-70. doi:10.1001/jamaoto.2017.2223 648-653. doi:10.1097/MAO.0000000000001767
2 Cunningham LL, Tucci DL. Hearing loss in adults. 13 Gandi D, Chen J, Pearl M, et al. Intracranial dural
N Engl J Med 2017;377(25):2465-2473. doi:10.1056/ arteriovenous fistulas: classification, imaging
NEJMra1616601 findings, and treatment. AJNR Am J Neuroradiol
2012;33(6):1007-1013. doi:10.3174/ajnr.A2798
3 Shargorodsky J, Curhan GC, Farwell WR, et al.
Prevalence and characteristics of tinnitus among 14 Gross BA, Albuquerque FC, Moon K, McDougall
US adults. Am J Med 2010;123(8):711-718. CG. Evolution of treatment and a detailed
doi:10.1016/j.amjmed.2010.02.015 analysis of occlusion, recurrence, and clinical
outcomes in an endovascular library of 260 dural
4 Nemholt S, Schmidt JH, Wedderkopp N, Baguley
arteriovenous fistulas. J Neurosurg 2017;126(6):
DM. A cross-sectional study of the prevalence
1184-1893. doi:10.3171/2016.5.JNS16331
and factors associated with tinnitus and/or
hyperacusis in children. Ear Hearing 2020;41(2): 15 Levine RA. Typewriter tinnitus: a
344-355. doi:10.1097/AUD.0000000000000759 carbamazepine-responsive syndrome related to
auditory nerve vascular compression. ORL J
5 Leaver AM, Renier L, Chevillet MA, et al.
Otorhinolaryngol Relat Spec 2006;68(1):43-46.
Dysregulation of limbic and auditory networks in
discussion 46-47. doi:10.1159/000090490
tinnitus. Neuron 2011;69(1):33-43. doi:10.1016/
j.neuron.2010.12.002 16 Levine RA. Tinnitus: diagnostic approach leading
to treatment. Semin Neurol 2013;33(3):256-269.
6 Rauschecker JP, Leaver AM, Mühlau M. Tuning
doi:10.1055/s-0033-1354603
out the noise: limbic-auditory interactions in
tinnitus. Neuron 2010;66(6):819-826. doi:10.1016/ 17 Keidar E, Kwartowitz G. Tensor tympani
j.neuron.2010.04.032 syndrome. Updated August 24, 2020. Accessed
December 20, 2020. www.ncbi.nlm.nih.gov/
7 Haider HF, Bojić T, Ribeiro SF, et al.
books/NBK519055
Pathophysiology of subjective tinnitus: triggers
and maintenance. Front Neurosci 2018;12:866. 18 Phillips JS, McFerran D. Tinnitus retraining
doi:10.3389/fnins.2018.00866 therapy (TRT) for tinnitus. Cochrane Database
Syst Rev 2010;2010(3):CD007330. doi:10.1002/
8 Pulec JL. Tinnitus: surgical therapy. Am J Otol
14651858.CD007330.pub2
1984;5(6):479-480.
19 Fuller T, Cima R, Langguth R, et al. Cognitive
9 Sismanis A. Pulsatile tinnitus: contemporary
behavioural therapy for tinnitus. Cochrane
assessment and management. Curr Opin
Database Syst Rev 2020;1(1):CD012614.
Otolaryngol Head Neck Surg 2011;19(5):348-357.
doi:10.1002/14651858.CD012614.pub2
doi:10.1097/MOO.0b013e3283493fd8
20 Sereda M, Xia J, El Refaie A, et al. Sound therapy
10 Guo P, Sun W, Shi S, Wang W. Patients with
(using amplification devices and/or sound
pulse-synchronous tinnitus should be suspected
generators) for tinnitus in adults. Cochrane
to have elevated cerebrospinal fluid pressure.
Database Syst Rev 2018;2018(8):CD013094.
J Int Med Res 2019;47(9):4104-4113. doi:10.1177/
doi:10.1002/14651858.CD013094
0300060519857846
21 Hobson J, Chisholm E, El Refaie A. Sound therapy
11 Kao E, Kefayati S, Amans MR, et al. Flow patterns
(masking) in the management of tinnitus in adults.
in the jugular veins of pulsatile tinnitus patients.
Cochrane Database Syst Rev 2012;11(11):CD006371.
J Biomech 2017;52:61-67. doi:10.1016/j.jbiomech.
doi:10.1002/14651858.CD006371.pub3
2016.12.008

CONTINUUMJOURNAL.COM 523

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

TINNITUS, HYPERACUSIS, OTALGIA, AND HEARING LOSS

22 Deklerck AN, Marechal C, Pérez Fernández AM, 37 Sullivan AM, Curtin HD, Moonis G. Arterial
et al. Invasive neuromodulation as a treatment anomalies of the middle ear: a pictorial review
for tinnitus: a systematic review. with clinical-embryologic and imaging
Neuromodulation 2020;23(4):451-462. doi:10.1111/ correlation. Neuroimaging Clin N Am 2019;29(1):
ner.13042 93-102. doi:10.1016/j.nic.2018.09.010
23 Salehi PP, Kasle D, Torabi SJ, et al. The etiology, 38 Weiss RL, Zahtz G, Goldofsky E, et al. High jugular
pathogeneses, and treatment of objective bulb and conductive hearing loss. Laryngoscope
tinnitus: unique case series and literature review. 1997;107(3):321-327. doi:10.1097/00005537-
Am J Otolaryngol 2019;40(4):594-597. doi:10.1016/ 199703000-00008
j.amjoto.2019.03.017
39 Cureoglu S, Baylan MY, Paparella MM. Cochlear
24 Expert Panel on Neurologic Imaging; Kessler MM, otosclerosis. Curr Opin Otolaryngol Head Neck
Moussa M, Bykowski J, et al. ACR Appropriateness Surg 2010;18(5):357-362. doi:10.1097/MOO.
Criteria® Tinnitus. J Am Coll Radiol 2017;14(11S): 0b013e32833d11d9
S584-S591. doi:10.1016/j.jacr.2017.08.052
40 Kim HA, Lee H, Ji-Soo Kim. Vertigo due to
25 Ishiyama G. Selected otologic disorders causing vascular mechanisms. Semin Neurol 2020;40(1):
dizziness. Continuum (Minneap Minn) 2021; 67-75.
27(2, Neuro-otology):468-490.
41 Eggers SDZ. Episodic spontaneous vertigo.
26 Jastreboff PJ, Jastreboff MM. Decreased sound Continuum (Minneap Minn) 2021;
tolerance: hyperacusis, misophonia, diplacousis, 27(2, Neuro-otology):369–401.
and polyacousis. Handb Clin Neurol 2015;129:
42 Moffat DA, Baguley DM, von Blumenthal H, et al.
375-387. doi:10.1016/B978-0-444-62630-1.00021-4
Sudden deafness in vestibular schwannoma.
27 Palumbo DB, Alsalman O, De Ridder D, et al. J Laryngol Otol 1994;108(2):116-119. doi:10.1017/
Misophonia and potential underlying s0022215100126052
mechanisms: a perspective. Front Psychol 2018;
43 Saha K. Vertigo related to central nervous system
9:953. doi:10.3389/fpsyg.2018.00953
disorders. Continuum (Minneap Minn) 2021;
28 Daniels EC, Rodriguez A, Zabelina DL. Severity of 27(2, Neuro-otology):447-467.
misophonia symptoms is associated with worse
44 Sara SA, Teh BM, Friedland P.Bilateral sudden
cognitive control when exposed to misophonia
sensorineural hearing loss: review. J Laryngol
trigger sounds. PLoS One 2020;15(1):e0227118.
Otol 2014;128:S8-S15. doi:10.1017/
doi:10.1371/journal.pone.0227118
S002221511300306X
29 Chandrasekhar SS, Tsai Do BS, Schwartz SR, et al.
45 Choi JH, Cho KY, Cha SY, et al. Audiovestibular
Clinical practice guideline: sudden hearing loss
impairments associated with intracranial
(update). Otolaryngol Head Neck Surg 2019;
hypotension. J Neurol Sci 2015;357(1–2):96-100.
161(1 suppl):S1-S45. doi: 10.1177/0194599819859885
doi:10.1016/j.jns.2015.07.002
30 Fife TD. Approach to the history and evaluation
46 Satzer D, Guillaume DJ. Hearing loss in
of vertigo and dizziness. Continuum (Minneap
hydrocephalus: a review, with focus on
Minn) 2021;27(2, Neuro-otology):306-329.
mechanisms. Neurosurg Rev 2016;39(1):13-24;
31 Hain TC, Cherchi M. Vestibular testing. discussion 25. doi:10.1007/s10143-015-0650-2
Continuum (Minneap Minn) 2021;27(2, Neuro-
47 Yamasoba T, Lin FR, Someya S, et al. Current
otology):330-347.
concepts in age-related hearing loss:
32 Boatman DF, Miglioretti DL, Eberwein C, et al. epidemiology and mechanistic pathways. Hear
How accurate are bedside hearing tests? Res 2013;303:30-38. doi:10.1016/j.heares.
Neurology 2007;68(16):1311-1314. doi:10.1212/ 2013.01.021
01.wnl.0000259524.08148.16
48 Van Camp G, Willems PJ, Smith RJ. Nonsyndromic
33 Willems PJ. Genetic causes of hearing loss. hearing impairment: unparalleled heterogeneity.
N Engl J Med 2000;342(15):1101-1109. doi:10.1056/ Am J Hum Genet 1997;60(4):758-764.
NEJM200004133421506
49 National Institute on Deafness and Other
34 Expert Panel on Neurologic Imaging; Sharma A, Communication Disorders. Over-the-counter
Kirsch CFE, Aulino JM, et al. ACR Appropriateness hearing aids. March 23, 2020. Accessed
Criteria® hearing loss and/or vertigo. J Am Coll December 20, 2020. www.nidcd.nih.gov/health/
Radiol 2018;15(11S):S321-S331. doi:10.1016/ over-counter-hearing-aids
j.jacr.2018.09.020
50 Reed NS, Betz J, Lin FR, Mamo SK. Pilot
35 Kozin ED, Remenschneider AK, Shah PV, et al. electroacoustic analyses of a sample of direct-
Endoscopic transcanal removal of symptomatic to-consumer amplification products. Otol
external auditory canal exostoses. Am J Neurotol 2017;38(6):804-808. doi:10.1097/
Otolaryngol 2015;36(2):283-286. doi:10.1016/ MAO.0000000000001414
j.amjoto.2014.10.018
36 Castle JT. Cholesteatoma pearls: practical points
and update. Head Neck Pathol 2018;12(3):419-429.
doi:10.1007/s12105-018-0915-5

524 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

51 Simon F, Roman S, Truy E, et al. Guidelines 53 Guyot JP, Fornos AP. Milestones in the
(short version) of the French Society of development of a vestibular implant. Curr Opin
Otorhinolaryngology (SFORL) on pediatric Neurol 2019;32(1):145-153. doi:10.1097/
cochlear implant indications. Eur Ann WCO.0000000000000639
Otorhinolaryngol Head Neck Dis 2019;136(5):
54 Perez Fornos A, Guinand N, van de Berg R, et al.
385-391. doi:10.1016/j.anorl.2019.05.018
Artificial balance: restoration of the
52 Boutros PJ, Schoo DP, Rahman M, et al. vestibulo-ocular reflex in humans with a
Continuous vestibular implant stimulation prototype vestibular neuroprosthesis. Front
partially restores eye-stabilizing reflexes. Neurol 2014;5:66. doi:10.3389/fneur.2014.00066
JCI Insight 2019;4(22):e128397. doi:10.1172/
jci.insight.128397

CONTINUUMJOURNAL.COM 525

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 Vertigo Related to REVIEW ARTICLE


Central Nervous System CONTINUUM AUDIO
INTERVIEW AVAILABLE
ONLINE

Disorders
Downloaded from http://journals.lww.com/continuum by wqLe/H3/oM2vx1Qs5A5qMemKKHLPqE4qkmGNpdheG3ikf1o85ttJSkFL2oTkgOkhMEmawHvsuTTE5xJG2ZjqccytIAhVn2qegy060KLcKJuPZsx5htaYiopRjp2zor2egUzWJAEWYNo65TEub0G9tZtPaMachiqn on 04/28/2022

By Kamala Saha, MD

ABSTRACT
PURPOSE OF REVIEW: This article provides an overview of the numerous causes
of vertigo and dizziness that are due to central nervous system (CNS)
pathology and guides clinicians in formulating a differential diagnosis and
treating patients with CNS causes of vertigo.

Specific autoimmune vestibulocerebellar syndromes

RECENT FINDINGS:
may now be tested for, and this article discusses the antibodies
known to cause such syndromes. Superficial siderosis can be more
accurately diagnosed with imaging studies, and treatment using iron
chelation has recently been studied but has not yet been established
as an effective treatment. Central autonomic network damage in
the brain can cause central orthostatic hypotension in some
neurodegenerative diseases, and medication has been approved for
CITE AS:
treatment. CONTINUUM (MINNEAP MINN)
2021;27(2, NEURO-OTOLOGY):
SUMMARY: CNS causes of vertigo are numerous and important for clinicians 447–467.

to recognize. Examination findings are still an extremely valuable way to

Address correspondence to
diagnose central vertigo; therefore, learning how to differentiate central Dr Kamala Saha, 240 W Thomas
from peripheral vertigo based on examination is an important skill. CNS Rd, Ste 301, Phoenix, AZ 85013,
Kamala.Saha@DignityHealth.
causes of vertigo often have available treatments.
org.

RELATIONSHIP DISCLOSURE:
Dr Saha reports no disclosure.
INTRODUCTION

V
ertigo can be a challenging symptom for clinicians to treat. UNLABELED USE OF
PRODUCTS/INVESTIGATIONAL
Taking a detailed history is the first step in trying to tease out USE DISCLOSURE:
whether the vertigo may be central, meaning caused by central Dr Saha discusses the
nervous system (CNS) pathology, rather than peripheral in origin. unlabeled/investigational use
of acetazolamide and
Following the history, neurologic examination is crucial. Having 4-aminopyridine for the
a strong understanding of the eye movements that can suggest central treatment of episodic ataxia
type 2, deferiprone as an iron
vertigo is extremely beneficial when trying to care for patients. Radiographic
chelator in the treatment of
studies and vestibular testing can aid in making a diagnosis. For more superficial siderosis, and
information on vestibular testing, refer to the article “Vestibular Testing” 4-aminopyridine for the
treatment of vertical
by Timothy C. Hain, MD, and Marcello Cherchi, MD, PhD, FAAN,1 in this nystagmus and central
issue of Continuum. Some types of central vertigo and dizziness, such as positional nystagmus.
superficial siderosis and Chiari malformations, are purely radiographic
diagnoses, whereas vestibular migraine is entirely a clinical diagnosis. In © 2021 American Academy
patients with multiple sclerosis (MS), eye movements often are the key to of Neurology.

CONTINUUMJOURNAL.COM 447

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

determining whether the origin of the vertigo is the nervous system or the
inner ear. Structural lesions, such as tumors, vascular lesions, and strokes, can
be seen on imaging. Autoimmune vestibulocerebellar disorders causing
vertigo are relatively rare, but because of advances in antibody testing,
recognition of these disorders is increasing. This article focuses on a variety of
CNS causes of vertigo important for neurologists to recognize.

FIGURE 7-1
Structures of the central nervous system vestibular system.
MLF = medial longitudinal fasciculus.
Reprinted with permission from Barrow Neurological Institute. © 2020 Barrow Neurological Institute,
Phoenix, Arizona.

448 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CENTRAL NERVOUS SYSTEM CAUSES OF VERTIGO KEY POINT
Central vertigo is a false sensation of motion caused by a lesion in the CNS that
● Multiple sclerosis lesions
results in dysfunction of the vestibular nuclei or their projections to the causing vertigo occur most
cerebellum (FIGURE 7-1). The vestibular nuclei are located in the caudal pontine frequently in the root entry
tegmentum and dorsolateral medulla and can be subdivided into four separate zone of cranial nerve VIII and
subnuclei: the superior, lateral (Deiters), medial, and inferior vestibular. These the medial vestibular
nucleus.
nuclei receive afferents from the peripheral vestibular system by way of the
vestibular division of cranial nerve VIII. They also receive afferents from the
cerebellum, the reticular formation in the pons, the spinal cord, and the
vestibular nuclei on the opposite side. Projections from the vestibular nuclei
reach the cerebellum, extraocular nuclei, and spinal cord.2 Lesions in any of these
areas can result in the symptom of vertigo. The following sections discuss the
CNS disorders capable of producing lesions that result in central vertigo.

Vestibular Migraine
Vestibular migraine is a common cause of vertigo seen in patients with a history of
migraine. Vestibular migraine causes episodic vertigo that can appear positional,
spontaneous, or visually induced. Vestibular symptoms may occur during headaches
but also commonly occur without headache.3 Between episodes, some patients may
experience chronic dizziness and imbalance. Vestibular migraine remains a clinical
diagnosis, and diagnostic criteria have been developed by the Bárány Society and the
International Headache Society.4 Although a clear understanding of the
pathophysiology is lacking, it may be related to the presumed pathology of migraine.
Pathologic nystagmus and central vestibular dysfunction have been seen in the majority
of patients with vestibular migraine studied, although they are often nonspecific.5

Multiple Sclerosis
MS causes inflammatory demyelinating lesions throughout the CNS and is
known to cause lesions specifically in areas that result in vertigo (ie, the brainstem
and cerebellum). It has been estimated that 20% of patients with MS will
experience true vertigo during their lifetime, and in about 5% of patients with MS,
it is the presenting symptom of the disease.6 The most common CNS sites known
to cause vertigo in MS are lesions at the root entry zone of cranial nerve VIII
(the lateral pontomedullary junction) and the medial vestibular nucleus.6-8
Additionally, patients can have symptoms from lesions scattered throughout the
cerebellum. In one retrospective analysis of a university-based population of
patients with MS presenting with acute vertigo due to demyelinating plaques,
three-fourths of the patients had a lesion in the root entry zone of cranial nerve
VIII and one-fourth had a lesion in the medial vestibular nucleus.6 It should be
noted that a root entry zone lesion may cause vertigo that behaves as if caused by a
peripheral vestibular lesion although the lesion may be in the CNS.
Vertigo due to MS may present acutely during an exacerbation, or it can
persist in a chronic form as a result of disease burden. During an exacerbation,
vertigo typically begins acutely and can be accompanied by nausea and vomiting.
Patients may be ataxic and may also report diplopia. Symptoms can be explained
by active (enhancing) lesions in the areas described above. The vertigo usually
improves or remits as the exacerbation resolves. Treatment is usually indicated
and consists of corticosteroids for most patients with MS who are able to tolerate
them. Vertigo can be managed symptomatically with antiemetics or vestibular
suppressants such as benzodiazepines (which are not recommended for

CONTINUUMJOURNAL.COM 449

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

long-term use). Vestibular therapy is not usually of strong benefit for central
vertigo in MS; however, it has been shown to improve balance and disability due
to dizziness or general disequilibrium in patients with MS.9
Central vertigo from MS is usually seen along with various focal findings on
neurologic examination. Abnormal saccades with reduced velocities, nystagmus
(potentially in multiple directions), impaired suppression of the vestibulo-ocular
reflex, and internuclear ophthalmoplegia (INO) are the prominent features that
can be observed.10 INO is the most common eye movement disorder seen in MS and
is caused by demyelination of the medial longitudinal fasciculus in the pons or
midbrain. It is a disorder of impaired conjugate lateral gaze, resulting in slowing
adduction or even paralysis of the adducting eye if severe enough. The abducting
eye exhibits nystagmus, and patients report diplopia. An INO can be unilateral
or bilateral in patients with MS, and variants exist. It is worth noting that the
presence of an INO does not necessarily mean a patient will have vertigo.
Similarly, not all patients with MS with vertigo have an INO on their examination.
Patients with MS may have saccadic dysmetria from cerebellar involvement,
particularly when the cerebellar peduncle is affected.11 Cerebellar lesions can also
cause impaired smooth pursuit, and gaze-evoked, downbeat, or acquired
pendular nystagmus that may be associated with oculopalatal tremor and often
with dizziness, imbalance, and oscillopsia (a perception of objects bouncing or
oscillating). Acquired pendular nystagmus likely results from damage to the
neural integrator network in the brainstem and cerebellum.12
Treatment of the eye movement dysfunction is both pharmacologic and
nonpharmacologic. For acquired pendular nystagmus, gabapentin and
memantine can be trialed. Downbeat nystagmus, typically from a lesion in the
flocculus, can improve with clonazepam, baclofen, or gabapentin in some
patients with MS. Recently, 4-aminopyridine (dalfampridine) has been studied
and deemed effective for vertical nystagmus and central positional nystagmus.11
Prism lenses can be helpful for some types of nystagmus and diplopia.
Improvement in the nystagmus does not always result in improvement in the
symptoms of dizziness or unsteadiness, but it can lessen oscillopsia for some.
Another important type of vertigo that patients with MS may experience is
central positional vertigo. This can be more challenging to diagnose and may be
confused with benign paroxysmal positional vertigo (BPPV). A central positional
vertigo is much rarer than BPPV. BPPV is more prevalent in patients with MS
than in the general population. A retrospective analysis of 1153 patients with MS
with acute vertigo found that more than 50% of the patients had BPPV, and all
were treated successfully with canalith repositioning maneuvers.13 Central
positional vertigo is similar to BPPV in that it is triggered by position change.
However, several pearls can help differentiate it from the more common BPPV:

u Patients with BPPV typically have a brief period of latency during a provocative maneuver
such as the Dix-Hallpike test. Central positional vertigo often has no latency, so
nystagmus commences immediately upon positioning.
u The nystagmus in BPPV fatigues after some time in the head-hanging position, whereas
central positional vertigo may exhibit nystagmus that persists and is prominent even after
repeat positioning.
u The pattern of nystagmus is perhaps the most important difference. Classic posterior
canal BPPV presents with both upbeat and torsional components, whereas central
positional vertigo is more likely to present without both components simultaneously.

450 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 Therefore, since the clinical presentation of both types of vertigo can look KEY POINTS
identical, it is important that neurologists pay close attention to the directional
● Treatment of vertigo as
features of nystagmus to better differentiate them and treat accordingly. Several part of a multiple sclerosis
case studies have shown the most common lesion responsible for central positional exacerbation is usually with
vertigo in patients with MS to be in the superior cerebellar peduncle (brachium steroids, plus a very short
conjunctivum)10; however, other lesions in the cerebellum are also known to cause course of a vestibular
suppressant.
central positional vertigo. Small lesions in this region may be missed if thin MRI
slices are not obtained when imaging the posterior fossa (CASE 7-1). ● Although known to
experience central
Stroke and Transient Ischemic Attack positional vertigo, patients
Strokes and transient ischemic attacks (TIAs) are known causes of central with multiple sclerosis are
much more likely to be
vertigo, dizziness, and imbalance when the posterior circulation is affected. A experiencing benign
cerebellar ischemic stroke in the posterior inferior cerebellar artery (PICA) paroxysmal positional
territory, the anterior inferior cerebellar artery (AICA) territory, or the superior vertigo if positional vertigo is
cerebellar artery territory may be associated with vertigo or nystagmus, or both. the presenting symptom.
Vertigo and nystagmus more commonly occur when the area of infarction affects ● Anterior inferior
the cerebellar peduncles, flocculus, nodulus, vermis, and paravermian regions cerebellar artery territory
and are less common with small lesions that are far lateral in the cerebellar infarcts can cause vertigo
hemisphere. Vertigo may also manifest from a brainstem infarction, which can due to a peripheral lesion or
central lesion, or both.
affect the lateral medulla, medial medulla, pons, or midbrain.
Vertigo with unilateral hearing loss can be caused by a labyrinthine infarction.
The labyrinth is supplied by the internal auditory artery, usually a branch of the
AICA. This can be missed on brain imaging,14,15 so clinical suspicion is
imperative. In fact, AICA territory infarct can present with both peripheral and
central findings. Involvement of the root entry zone of the facial nerve or the
labyrinth could lead to peripheral findings such as facial paresis, hearing loss, and
vertigo. At the same time, expansion of stroke can lead to involvement of the
pons or cerebellum, or both, which can cause central vertigo and ataxia. Recent
literature points out that audiovestibular loss in isolation can be an impending
sign of AICA territory infarction, with initial symptoms of only vertigo and
hearing loss occurring days or weeks before the presentation of a posterior fossa
stroke.16 Most patients with this presentation seem to have evidence of reduced
basilar artery flow near the AICA origin. This type of infarct should be
considered in patients with vascular risk factors who experience audiovestibular
loss even if MRI is unrevealing (CASE 7-2).
Chronic vertigo due to the late effects of stroke is not an uncommon symptom;
it is often a residual symptom that can persist long after the infarct occurs. It may
be caused by central or peripheral damage or a combination of the two, as
discussed above. Initial management usually is a short course of a vestibular
suppressant followed by physical therapy. Central lesions may not respond as
quickly or as successfully to therapy as peripheral insults; however, therapy can
be helpful in improving balance overall after a stroke. Visually induced vertigo is
common in these patients, and they report symptoms that are exacerbated or
triggered by complex visual surroundings. Therapy programs with visual-vestibular
stimulation during therapy can result in greater improvements in such patients.17

Tumors and Other Structural Lesions in the Central Nervous System

Neoplasms and vascular lesions can cause central vertigo and other neurologic
symptoms based on their location in the CNS, including vestibular schwannoma,
cavernous malformation, hemangioblastoma, and medulloblastoma.

CONTINUUMJOURNAL.COM 451

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

CASE 7-1 A 36-year-old woman with multiple sclerosis presented to the

emergency department with vertigo that had started that morning when
she rolled over in bed to her right side to get up. She had nausea and had
vomited once because of the vertigo. She denied headache, vision loss,
or diplopia and had not noticed any change in hearing.
On examination, she had full extraocular movements without
spontaneous or gaze-evoked nystagmus. She had a right afferent
pupillary defect that had been documented on examination during a prior
emergency department visit and had no skew deviation. The head
impulse test was normal to the right and left sides. She had normal
coordination and gait. A Dix-Hallpike test to the right was performed; she
had prominent upbeating and torsional nystagmus in a counterclockwise
direction after a latency of 10 seconds and felt extremely nauseated
during testing. A canalith repositioning procedure was then performed,
and she felt better and was discharged.
The next morning, she returned to the emergency department after
experiencing vertigo with vomiting. Although her vertigo had improved
after the canalith repositioning procedure was done, it had not gone away
entirely and she continued to experience a spinning sensation every time
she got out of bed or stood up after bending over. Neurologic
examination was repeated, and her extraocular movements were intact.
A Dix-Hallpike test to the right side was normal this time, and no
nystagmus was observed. A Dix-Hallpike test to the left side was then
performed and revealed downbeat nystagmus with immediate onset that
did not fatigue. An MRI with thin slices in the posterior fossa revealed an
enhancing lesion in the right middle cerebellar peduncle adjacent to the
cerebellar nodulus (FIGURE 7-2) as well as scattered nonenhancing
hemispheric white matter abnormalities. The patient was treated with
steroids and vestibular suppressants for 3 days, and her symptoms and
positional nystagmus completely resolved.

452 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

FIGURE 7-2
Axial postcontrast T1-weighted MRI shows an
acute demyelinating lesion (circle) in the right
middle cerebellar peduncle adjacent to the
cerebellar nodulus.
Reprinted with permission from Barrow Neurological Institute.
© 2020 Barrow Neurological Institute, Phoenix, Arizona.

This patient had multiple sclerosis, which may lead neurologists to attribute COMMENT
new neurologic symptoms to her known disease. However, the most
common cause of positional vertigo is still benign paroxysmal positional
vertigo (BPPV); therefore, the Dix-Hallpike test was an essential part of her
examination. BPPV was localized to the right posterior semicircular canal
based on her initial examination findings. Successful treatment of BPPV
is with a canalith repositioning procedure, not medications. A canalith
repositioning procedure should be performed immediately after
confirming the diagnosis. Although this patient improved somewhat after
the canalith repositioning procedure, her symptoms had not completely
resolved; thus, another cause of her vertigo was explored. This case
reminds us that even if positional vertigo is found and treated on one side,
the other side must also be examined as symptoms and examination
findings can sometimes be bilateral. This patient had two different types of
vertigo: BPPV in the right ear and central positional vertigo as evidenced by
the downbeat nystagmus during positional testing on the left. It is also
imperative to remember that not all positional vertigo or positional
nystagmus equates to a diagnosis of BPPV. Multiple sclerosis can cause
central positional vertigo with only positional nystagmus, and the treatment
is different from that of BPPV.

CONTINUUMJOURNAL.COM 453

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

Vestibular schwannoma, also known as acoustic neuroma, is a tumor that

arises from the Schwann cells around cranial nerve VIII. Although the tumor
forms from the vestibular portion of the nerve, it most commonly presents with
hearing loss as it can compress the cochlear division of the nerve. Vertigo is an
infrequent presentation of vestibular schwannoma, estimated to be seen initially
in less than 15% of patients. Slowly progressive unilateral hearing loss and
tinnitus are more common initial symptoms, although in a small percentage of
patients hearing loss may occur suddenly. When patients present with dizziness,
they may describe spinning vertigo, lightheadedness, or gait imbalance. As
vestibular schwannomas enlarge, they may lead to peripheral vestibular loss that
contributes to the feeling of loss of balance. However, these tumors can also grow
to compress the brainstem and thereby cause central vertigo.
An audiogram showing significantly asymmetric hearing loss may lead to
suspicion of vestibular schwannoma. Asymmetry of 15 dB at 3 kHz on
audiometry is associated with increased positive yield of finding an abnormality
on MRI that explains the patient’s hearing loss.18 If a vestibular schwannoma is
suspected, an MRI of the brain with and without gadolinium can be used to
evaluate for an enhancing lesion in the internal auditory canal or
cerebellopontine angle (FIGURE 7-3). A cerebellopontine angle meningioma can
present similarly in many respects. Observation and sequential imaging,
radiosurgery, and microsurgery are all management options for vestibular
schwannoma. Decisions for treatment are often based on the patient’s age and

CASE 7-2 A 72-year-old man with a history of hypertension and hyperlipidemia

presented to his primary care physician for his annual physical
examination. While there, he mentioned that just a few days earlier he
had experienced sudden vertigo and hearing loss in his left ear. He was
still feeling a bit dizzy, but his hearing seemed to have improved
somewhat. His doctor observed some mild horizontal end-gaze
nystagmus to the right. No other abnormalities were found on
examination, and the patient reported that he otherwise felt well. His
examination suggested a peripheral vestibulopathy, and he was
counseled that his symptoms should continue to improve.
The following week, the vertigo returned and he was unsteady walking.
He presented to the emergency department and was notably ataxic on
examination, had left arm dysmetria, and now had bilateral end-gaze
horizontal direction-changing nystagmus. Imaging revealed an anterior
inferior cerebellar artery (AICA) territory infarction, and vessel studies
showed a narrow basilar artery near the origin of the AICA on the left side.

COMMENT Vertigo and fluctuating hearing loss can be a harbinger of impending AICA
territory infarct. AICA territory infarcts can present with both peripheral
and central findings simultaneously. In this case, the initial findings
appeared peripheral in nature because of likely labyrinth involvement.
However, in a patient with vascular risk factors, stroke should be suspected
as an etiology early before a complete territory infarction ensues.

454 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 surgical risk factors, quality-of- KEY POINTS
life considerations, the size of the
● Central vertigo in
tumor and its rate of growth, the vestibular schwannoma
status of vestibular and hearing often results from brainstem
function, and the desire or need compression.
to preserve hearing function.
● Cavernous malformations
Cavernous malformations are
are seen in the posterior
either sporadic or inherited fossa 25% of the time, and
vascular malformations. When posterior fossa cavernous
sporadic, they are usually single malformations have higher
lesions that may be associated rates of hemorrhage than
supratentorial cavernous
with a developmental venous malformations.
anomaly. Cavernous
malformations are made up of
FIGURE 7-3 dilated capillaries with thin walls
Vestibular schwannoma. Axial postcontrast
T1-weighted MRI shows an enhancing left
and are often surrounded by
cerebellopontine angle extraaxial mass (arrow), hemosiderin from recurrent
consistent with vestibular schwannoma. hemorrhage. They are described
on MRI as having a popcorn ball
appearance (FIGURE 7-4). About
one-fourth of cavernous malformations are found in the posterior fossa, usually
in the pons or the cerebellum; these tend to have higher annual bleeding rates
than supratentorial cavernous malformations. Vertigo can be a presenting
symptom of a cavernous malformation, especially if active hemorrhage is
present. Nausea, vomiting, and diplopia can also accompany the acute vertigo in
these cases. Cavernous malformations may be managed with observation if
lesions are asymptomatic. Brainstem lesions are often initially managed

FIGURE 7-4
Cavernous malformation. Axial T2-weighted (A) and postcontrast T1-weighted (B) images
of a left cerebellar cavernous malformation. A T2-hyperintense cystic component and
T2-hypointense rim of hemosiderin surround the lesion (A, arrow). An adjacent
developmental venous anomaly, a common association, is seen on the postcontrast image
(B, arrow).

CONTINUUMJOURNAL.COM 455

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

conservatively, but repeat hemorrhage may prompt surgical resection. A

systematic review of patients with brainstem cavernous malformations who
underwent surgical resection showed that 58% of patients had eventual
neurologic improvement, 26% remained stable, and 12% worsened. Mortality
was 2%.19
Hemangioblastomas are tumors that grow slowly in the spinal cord,
cerebellum, or brainstem. They can be sporadic but are often seen in patients
with von Hippel-Lindau disease, along with various other tumors. Because
of the strong association with von Hippel-Lindau disease, finding a CNS
hemangioblastoma often prompts genetic evaluation for the disease in patients
who have not yet been diagnosed.20 Hemangioblastoma can cause vertigo and
ataxia because of compression of structures in the brainstem or cerebellum or
because of hemorrhage in those areas. In the cerebellum, where they usually
present, hemangioblastomas often appear as enhancing nodules within a cyst.
Rarely, hemangioblastomas can occur in the cerebellopontine angle and may be
misdiagnosed as vestibular schwannoma since the symptoms and MRI findings
can be similar.20 Hemangioblastomas are very vascular and may require
embolization of feeding arteries before surgical resection.
Medulloblastoma is the most common malignant brain tumor diagnosed
in children. It presents with headache, nausea, and vomiting, often because
of fourth ventricle involvement causing increased intracranial pressure. In
addition, patients may have dizziness or vertigo due to brainstem compression
and cerebellar involvement. Midline cerebellar lesions may cause more gait or
truncal ataxia than lateral cerebellar tumors, which can cause more limb
dysmetria. Medulloblastoma is usually seen on MRI in the cerebellum, with
some areas of enhancement and possible obstruction of the fourth ventricle.
Central patterns of nystagmus can be seen in primary gaze or with end gaze
on examination; however, medulloblastoma is also known to cause central
positional vertigo only. Therefore, BPPV is sometimes suspected but should
be considered unlikely when patients are young and the symptoms do not
improve with repositioning maneuvers or when the nystagmus is sustained.21

Meningitis and Encephalitis

Acute bacterial meningitis may cause bilateral hearing and vestibular loss,
especially in children. The most common organisms known to cause this include
Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae type
b. Cases attributed to H. influenzae type b have decreased since a vaccine was
introduced. The causative lesion may be in the inner ear end organs, the
vestibulocochlear nerve, brainstem, or auditory and vestibular pathways.
Infection may spread to the inner ear from the subarachnoid space via the
cochlear aqueduct or the cochlear modiolus.22
Dizziness and bilateral vestibulopathy may sometimes occur with chronic
meningitis. Inflammation within the central audiovestibular pathways and
cranial nerve nuclei may be responsible for these symptoms. The list of
specific causes of chronic infectious meningitis is long and includes tuberculosis,
fungal infections such as coccidioidomycosis and cryptococcosis, and Lyme
disease.
Occasionally, certain types of localized brainstem encephalitis may cause
dizziness. The term rhombencephalitis refers to inflammation affecting the
brainstem or cerebellum, or both; it may be associated with dizziness,

456 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

unsteadiness, nausea, diplopia, headache, and altered awareness. Listeria KEY POINTS
monocytogenes is the most common infectious cause of rhombencephalitis.23
● Hemangioblastoma is
Sarcoidosis, a noninfectious disorder of unknown etiology, is a granulomatous typically associated with
process that can affect multiple body systems. Less commonly, it can affect the von Hippel-Lindau disease.
nervous system exclusively, presenting as neurosarcoidosis. Sarcoidosis has a
predilection for the basal meninges and can affect the vestibulocochlear nerve ● Medulloblastoma causes
vertigo and increased
exit or, rarely, can manifest with granulomas in the cerebellopontine angle.
intracranial pressure from
Combined evidence from retrospective review has shown that audiovestibular fourth ventricle
manifestations of sarcoidosis are primarily caused by cranial nerve VIII involvement.
neuropathy.24
Carcinomatous or lymphomatous meningitis may also cause multiple evolving ● Listeria monocytogenes is
the most common infectious
cranial neuropathies and brainstem symptoms. This involves seeding of cause of rhombencephalitis.
malignant cells to the leptomeninges. It can be seen in solid cancers, such as
breast or lung, and with hematologic malignancies. Primary brain tumors can ● Chiari malformation type 1
also spread to the meninges. Headache, cranial neuropathies, nausea, and is a radiographic diagnosis
usually made by measuring
dizziness are common at presentation. Imaging reveals diffuse leptomeningeal
cerebellar tonsil herniation
enhancement, often in the cerebellar folia and ventral surface of the brainstem, greater than or equal to
when patients have dizziness or unsteadiness. 5 mm below the foramen
magnum.
Chiari Malformation
Chiari malformations can be classified as types I through IV based on the
anatomic structures involved in the malformation. Chiari malformation type I,
the most common type, is a congenital lesion that may not manifest with
symptoms until adulthood. In Chiari malformation type I, the cerebellar tonsils
extend below the foramen magnum. Diagnosis is radiographic, and most sources
agree that it is defined as tonsillar
herniation of greater than or
equal to 5 mm below the foramen
magnum (FIGURE 7-5). Of note,
the degree of herniation does not
necessarily correlate with the
extent of symptoms experienced
by patients. Chiari malformation
type I can be asymptomatic but
when symptomatic often
presents with some combination
of posterior headache, neck pain,
weakness, dysphagia, or vertigo
and gait imbalance.
The vertigo in patients with
Chiari malformations is usually
induced by position change such
as neck extension. This may be
FIGURE 7-5
caused by pressure being applied
Sagittal T1-weighted MRI of a patient with
to the brainstem or cerebellum or Chiari malformation type I. Cerebellar tonsillar
their blood supply.25 Vertigo is herniation of 9 mm through the foramen magnum
usually episodic and brief, often is noted. This is measured by first drawing a line
from the opisthion to the basion (which forms a
relieved by changing position.
line across the foramen magnum) and then
Nausea and vomiting may also measuring the distance from that line to the most
accompany the vertigo. inferior margin of the cerebellar tonsils.

CONTINUUMJOURNAL.COM 457

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

Although several patterns of nystagmus have been reported, most commonly a

downbeat nystagmus is observed when examining a patient in primary gaze,
especially when the patient is supine with the head slightly tilted back. Downbeat
nystagmus localizes to the cervicomedullary junction. Vertigo can improve in
some patients who are candidates for surgical decompression to treat Chiari
malformation type I; however, if nystagmus was present before surgery, it
may not entirely resolve following decompression surgery. Since surgical
decompression is a major surgery, attempts to exclude significant contributing
migraine or greater occipital neuralgia alone or in combination should be made
before surgical decompression.

Superficial Siderosis
Superficial siderosis is an uncommon disorder that can affect various areas in the
CNS, including the brainstem, spinal cord, cerebellum, supratentorial brain,
nerve roots, and cranial nerves. It frequently leads to progressive symptoms of
vertigo, ataxia, and hearing loss. The two types of superficial siderosis are cortical
superficial siderosis and infratentorial superficial siderosis. Cortical superficial
siderosis affects the supratentorial brain and can be seen in cerebral amyloid
angiopathy.26 Infratentorial superficial siderosis more commonly affects the
vestibular end organs, cranial nerve VIII, the brainstem, the cerebellum, and the
spinal cord.
Patients with superficial siderosis develop neuronal damage over time from
hemosiderin deposition on the leptomeningeal surfaces of the nervous system.
The hemosiderin is a product of blood breakdown and deposits in areas adjacent
to the CSF.27 Superficial siderosis develops from small amounts of bleeding in
the brain or spinal cord and may be caused by repeat episodes of bleeding or a
onetime event of bleeding, such as a traumatic or aneurysmal subarachnoid
hemorrhage. If the bleeds are chronic and recurrent, the source is usually
from disruption of dural integrity caused by various etiologies, such as a

FIGURE 7-6
Myxopapillary ependymoma causing superficial siderosis. A, Sagittal postcontrast
T1-weighted MRI shows an intradural mass at L1-L2 that was confirmed to be a myxopapillary
ependymoma. This is a well-defined intradural tumor that enhances homogenously. B,
Axial susceptibility-weighted imaging (SWI) shows superficial siderosis supratentorially,
with pial surfaces coated with low signal hemosiderin. C, Axial fluid-attenuated inversion
recovery (FLAIR) MRI shows some mild cerebellar atrophy. D, Axial SWI shows a significant
degree of superficial siderosis infratentorially from prior hemorrhage related to the
myxopapillary ependymoma.

458 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

meningocele, pseudomeningocele, nerve root avulsion, or tumor.28 Myxopapillary KEY POINTS
ependymoma is the most common tumor seen to cause infratentorial superficial
● Downbeat nystagmus in
siderosis. patients with Chiari
Symptoms of superficial siderosis can vary, but the most commonly seen is malformations localizes to
hearing loss that is progressive and leads to deafness. Cerebellar ataxia is also the cervicomedullary
common and progressive, often with associated gaze-evoked nystagmus and junction.
saccadic dysmetria. Gait imbalance can result from a combination of cerebellar
● Infratentorial superficial
damage and vestibulopathy since damage to the vestibular end organs and nerve siderosis most commonly
is possible. Less common symptoms and signs of superficial siderosis include causes hearing loss, but
myelopathy, cognitive deficits, and seizures. Rarely, patients may report ataxia and vertigo are often
anosmia, which is caused by damage to cranial nerve I,27 the second most also present.

frequently affected cranial nerve in superficial siderosis. ● Imaging, usually MRI with
The hearing loss seen in superficial siderosis usually affects high frequencies gradient recalled echo and
early on. It can be asymmetric at first but will progress and cause profound susceptibility-weighted
damage bilaterally with time. It is typically more severe than what would be imaging sequences, shows
the findings of hemosiderin
expected for hearing loss due to presbycusis (hearing loss associated with aging).29 damage in superficial
Hearing aids can be used in earlier stages, and cochlear implantation has been siderosis but does not
shown to have some benefit based on systematic review of available studies.30 necessarily correlate with
When vestibular damage is present, patients may report dizziness or vertigo clinical symptoms in a
patient.
and exhibit gait instability. Since some gait instability in superficial siderosis is
usually because of cerebellar damage, the vestibular system is often forgotten as a
potential site of damage. However, cranial nerve VIII has a long course from the
end organs through the internal auditory canal, making it vulnerable to
damage.28 Damage to cranial nerve VIII can be assessed using various vestibular
tests, such as videonystagmography, rotary chair testing, vestibular evoked
myogenic potentials, and video head impulse testing. Overall, most patients with
superficial siderosis appear to have both peripheral and central vestibulopathy.31
Before imaging was available, the diagnosis of superficial siderosis was made
postmortem. Today, however, the diagnosis is made by MRI. Hemosiderin is
seen easily on MRI sequences, including gradient recalled echo (GRE),
T2-weighted, and susceptibility-weighted imaging (SWI). Superficial siderosis
appears as rims of hypointensity (FIGURE 7-6). Although imaging remains the
gold standard for diagnosis of superficial siderosis, it does not help in determining
whether a patient is symptomatic from the superficial siderosis seen on the scans.
A study of patients with superficial siderosis confirmed with MRI showed that
only 15% of them actually exhibited symptoms of superficial siderosis.32
Treatment of superficial siderosis can be symptomatic depending on the
particular symptoms of the individual patient. However, it also must focus on
identifying any underlying structural lesion that may be the etiology of the
patient’s superficial siderosis. Imaging of various types can be used. The entire
neuraxis should be evaluated,33 as spinal lesions can be the culprit when no
obvious source is seen in the brain. The highest rate of success in finding an
underlying etiology of superficial siderosis has been when either spinal MRI or
CT myelography was used.28 Surgical treatment may commence when a source
of chronic persistent CSF leakage of blood is identified, if amenable to
intervention. This may or may not result in improvement of patient symptoms;
however, it can halt progression of symptoms for some. Iron chelators have been
studied as potential treatments in superficial siderosis. A long-term open-label
observational study suggested that the iron chelator deferiprone can be used
safely in patients with superficial siderosis and is well tolerated. All patients had a

CONTINUUMJOURNAL.COM 459

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

reduction in iron seen in the brain on MRI after treatment with deferiprone, and
half the patients had clinical improvement in symptoms in a study of four
patients without controls.34 It should also be noted that deferiprone has a US
Food and Drug Administration (FDA) boxed warning for the possibility of
agranulocytosis/neutropenia, thus patients must be monitored while taking it.
Larger randomized trials are needed to determine whether iron chelators are an
effective treatment for superficial siderosis.

Neurodegenerative Disease
Vertigo and dizziness are commonly seen in patients with Parkinson disease,
multiple system atrophy, progressive supranuclear palsy, and cerebellar ataxia.35
Cerebellar ataxia has numerous potential etiologies, such as genetic disease,
vitamin deficiencies, paraneoplastic disease, environmental/toxin exposures,
and as a result of adverse effects of medications. Although the possible causes are
myriad, the manifestations can be similar. Vertigo is often paroxysmal, and
bedside examination usually reveals central nystagmus. Typical patterns
include spontaneous downbeat nystagmus and direction-changing horizontal
end-gaze nystagmus. Downbeat nystagmus results from degeneration of the
cerebellum, leading to floccular hypofunction.36 Prism glasses and medications
can be trialed to help alleviate symptoms. Some evidence supports the use of
aminopyridines for downbeat nystagmus and gait ataxia in these patients.37
Currently, no medications are approved by the FDA for the treatment of
cerebellar ataxia.
In Parkinson disease and the atypical conditions that cause parkinsonism,
such as multiple system atrophy, central orthostatic hypotension may be a
cause of presyncopal dizziness. This is because of involvement of the central
autonomic network that helps to regulate visceromotor, neuroendocrine, and
pain responses (FIGURE 7-7). The central autonomic network is made up of
multiple brain regions, including the amygdala, hypothalamus, nucleus of
the tractus solitarius, and ventrolateral medulla.38 Specific groups of neurons in
the medulla have been found to be affected in patients with Parkinson disease
and, to a greater extent, patients with multiple system atrophy, leading to
impaired sympathetic vasomotor outflow and impaired release of vasopressin.39
The damage to these neurons is proposed to explain the orthostatic
hypotension and autonomic reflex impairment that affect patients. Beyond
nonpharmacologic treatments, medications such as the α-adrenergic agonist
midodrine and droxidopa, a norepinephrine precursor, are FDA approved for the
treatment of symptomatic neurogenic orthostatic hypotension.

Episodic Ataxias
Seven autosomal dominant episodic ataxias have been identified, aptly named
episodic ataxia type 1 through episodic ataxia type 7. Of these types, most cases
encountered are usually episodic ataxia type 1 or episodic ataxia type 2. In
patients with episodic ataxia type 2, vertigo is severe and episodic, often
accompanied by nausea and vomiting as well as unsteadiness. Patients with
episodic ataxia type 2 usually start having episodes during adolescence, and each
episode can last hours. Stress is a common trigger, as are heat, exertion, alcohol,
and caffeine.40 Genetic testing usually reveals mutations in the CACNA1A gene,
specifically in the P/Q-type calcium channel α1A subunit. Episodic ataxia type 2
is felt to be caused by a loss of P/Q-type calcium channel function in the

460 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 KEY POINTS

● Treatment of superficial
siderosis is symptomatic,
but identifying any possible
underlying structural lesion
causing the superficial
siderosis is imperative.
Surgery and iron chelators
are being investigated but
have not yet been
established as effective
treatments.

● Patients with cerebellar

ataxia often have
paroxysmal vertigo along
with central nystagmus
findings on examination.

● The central autonomic

network is damaged in some
neurodegenerative diseases
and can lead to central
orthostatic hypotension.

FIGURE 7-7
The central autonomic network with its multiple involved brain regions and connection to
the peripheral autonomic nervous system.
Reprinted with permission from Barrow Neurological Institute. © 2020 Barrow Neurological Institute,
Phoenix, Arizona.

cerebellum, in both Purkinje cells and granule layer neurons.41 Neurologic

examination can be helpful, as these patients often have central pattern
nystagmus not only during their episodes but even between episodes (CASE 7-3).
Cerebellar damage accrues over time.
Several medications may be used to treat episodes of vertigo in patients with
episodic ataxia type 2. Both acetazolamide and 4-aminopyridine have
demonstrated efficacy. A randomized controlled trial in patients with episodic
ataxia showed 4-aminopyridine 5 mg 3 times a day to be effective in reducing
episodes, possibly by increasing excitability of Purkinje cells and increasing
levels of γ-aminobutyric acid (GABA).42,43 One recently published trial, the

CONTINUUMJOURNAL.COM 461

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

CASE 7-3 A 23-year-old man presented for neurologic consultation because of

episodic vertigo. He had started having the episodes of vertigo around
age 18. Episodes were monthly on average, lasting 3 to 4 hours each time.
He was a college athlete and noticed that episodes often occurred after
tough workouts with his team. He always had nausea with episodes and
sometimes vomited; his vision would blur, and he would have to sit down
because he was so unsteady. No one else in his family experienced similar
episodes. Neurologic examination showed normal coordination and gait;
however, he was noted to have downbeat nystagmus in primary gaze.
MRI of his brain was normal. Videonystagmography was ordered, and
the tracing confirmed downbeat nystagmus in primary gaze (FIGURE 7-8).
Genetic testing using sequence analysis was ordered and revealed a
heterozygous pathogenic variant in CACNA1A. He was started on
acetazolamide 250 mg once daily and titrated to 250 mg 2 times a day,
which resulted in complete cessation of his episodic vertigo.

FIGURE 7-8
Videonystagmography of the patient in CASE 7-3 showing downbeat nystagmus in primary
gaze. This was also present in supine, head-hanging right, and head-hanging left positions.

COMMENT Episodic ataxia type 2 is confirmed with genetic testing. In this patient
without a family history, the disease may have been caused by a de novo
pathogenic variant. Whereas downbeat nystagmus is common during
episodes, it is often observed in patients even between episodes. Most
patients find benefit with relatively low doses of acetazolamide, as did this
patient.

462 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

EAT2TREAT (Fampridine and Acetazolamide for the Treatment of Episodic KEY POINTS
Ataxia Type 2) study, found that both acetazolamide as well as 4-aminopyridine
● Diagnosis of autoimmune
significantly reduced the number of attacks in patients with episodic ataxia vestibulocerebellar
type 2 compared to placebo.44 disorders depends on both a
It is worth noting that overlap exists between other neurologic disorders and clinical syndrome that is
episodic ataxia type 2. For example, other CACNA1A gene mutations in the same characteristic and a positive
antibody result.
subunit are seen in patients with spinocerebellar ataxia type 6 and in patients
with familial hemiplegic migraine type 1. Cerebellar atrophy can be seen in all of ● To improve test yield,
these conditions. Some patients with spinocerebellar ataxia (particularly both serum and CSF samples
spinocerebellar ataxia type 6) may experience episodic vertigo similar to patients should be obtained for
with episodic ataxia type 2; however, they do not find benefit with the antibody testing for
autoimmune
medications discussed above. vestibulocerebellar
disorders.
Autoimmune Vestibulocerebellar Disorders
The literature on autoimmune vestibulocerebellar disorders is actively expanding
as more is learned about the autoantibodies that are biomarkers for these
disorders. Typically, the antibodies target antigens in the vestibulocerebellar
pathways, vestibular nuclei, or vestibular end organs. This results in autoimmune
syndromes characterized by symptoms such as dizziness and ataxia that can
progress rather quickly. Clinicians must be proficient not only in diagnosis but
also in initiating treatment in hopes of halting this progression.
The presentation of patients with these disorders is subacute, meaning that
patients usually have an onset of cerebellar symptoms over weeks to months.
Symptoms may begin first with a prodrome of nausea and vomiting.45
Truncal and appendicular ataxia begin after this, along with vertigo, dysarthria,
diplopia, and dysphagia.46 Extraocular movements are abnormal and may
include any the following: positional vertical nystagmus (upbeat or downbeat),
spontaneous downbeat nystagmus, spontaneous or gaze-evoked horizontal
nystagmus, opsoclonus, periodic alternating nystagmus, or internuclear
ophthalmoplegia.47
The diagnostic workup centers on testing for specific autoantibodies after a
clinical syndrome is suspected. In general, antibody testing should be done on
both serum and CSF samples. Diagnostic certainty results from the combination
of a characteristic clinical syndrome and positive result of the accompanying
antibody in either serum or CSF, or both. CSF is often abnormal and a mild
pleocytosis may be seen.46 Mildly elevated protein and IgG index are also
sometimes seen. In some instances, antibody testing may be negative and
repeat testing may be considered. Diagnosis is still sometimes made despite
negative antibody testing if the clinical syndrome is characteristic and
patients have supportive findings from CSF studies and a positive response to
immunotherapy. It is possible that numerous additional autoantibodies remain to be
discovered, and not all known antibodies are included in every commercial panel.
Neurologists should carefully ensure that the panel they order includes the antibody
or antibodies they suspect in a particular syndrome. Brain imaging, such as MRI, is
often normal but can show cerebellar atrophy in some patients. It is still usually a part
of the workup, especially to exclude other potential causes.
These antibodies are often classified as either intraneuronal/cytoplasmic or
cell surface antibodies. Intraneuronal/cytoplasmic antibodies are usually seen in
paraneoplastic syndromes, and the underlying process is thought to be cytotoxic
T-cell–mediated neuronal disruption.48 The antibodies seen include anti-Yo

CONTINUUMJOURNAL.COM 463

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

CASE 7-4 A 62-year-old woman initially presented to the emergency department

with a flulike illness, with nausea and vomiting for several days. She was
afebrile, and basic laboratory tests suggested dehydration. She was
given IV fluids and discharged in stable condition.
She continued to feel unwell over the next 3 months. She reported
being unsteady on her feet and was seeing double, so her family brought
her back to the emergency department. She denied a history of alcohol
overuse. The emergency department physician consulted neurology, and
the neurologist examining her saw that she had horizontal gaze-evoked
nystagmus. She was noted to be ataxic when walking. Her brain MRI
showed mild and diffuse cerebellar atrophy (FIGURE 7-9). Laboratory
studies, including thyroid-stimulating hormone (TSH); vitamins B1, B12, and E;
and a celiac panel, were normal. CSF studies were similarly benign, with
normal cell count, protein, and glucose and negative Gram stain and
culture. Serum and CSF paraneoplastic and autoimmune antibodies
were ordered.
She was admitted to the hospital,
where CT of the chest/abdomen/pelvis
was obtained and revealed an ovarian
mass that was confirmed with
follow-up MRI. She underwent total
abdominal hysterectomy and bilateral
salpingo-oophorectomy and was then
given an empiric trial of IV
immunoglobulin (IVIg) over 5 days.
Her gait improved modestly, but the
nystagmus, although less prominent,
still persisted at the time of discharge
to rehabilitation. Follow-up on
FIGURE 7-9
antibody panels after discharge Imaging of the patient in CASE 7-4.
showed a positive anti-Yo titer in Sagittal postcontrast T1-weighted MRI
CSF, but it was negative in serum. shows mild and diffuse cerebellar
atrophy.

COMMENT This case illustrates a typical time course for an autoimmune

vestibulocerebellar disorder, as it began with a prodrome and was
followed by a subacute worsening of symptoms before diagnosis. This
case highlights the necessity to sometimes begin treatment before
obtaining all test results, as it can take time before antibody panel test
results are available.

464 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

(Purkinje cell cytoplasmic antibody 1 [PCA-1]), anti-Hu antibody (antineuronal KEY POINT
nuclear antibody type 1 [ANNA-1]), anti-Ri antibody (antineuronal nuclear
● Identifying the specific
antibody type 2 [ANNA-2]), Ma1 and Ma2 antibodies, amphiphysin antibody, antibody causing an
CV2 antibody (collapsin response mediator protein-5 [CRMP5]), and glutamic autoimmune
acid decarboxylase 65 (GAD65) antibody. Several additional antibodies have been vestibulocerebellar disorder
described in smaller numbers of patients thus far.47 In general, these antibodies are can help prognosticate and
determine the likelihood of a
part of paraneoplastic syndromes, and thus a search for an underlying malignancy
malignancy eventually being
followed by any treatment indicated is imperative. The most common antibody found.
causing paraneoplastic cerebellar degeneration is the anti-Yo antibody, usually
associated with gynecologic and breast malignancies in women older than age
60.49 Overall prognosis has generally been poor for paraneoplastic syndromes,
but a trial of immunotherapy is still usually warranted (CASE 7-4).
The antibodies against extracellular cell surface/synaptic antigens are often
nonparaneoplastic and may cause autoimmune vestibulocerebellar syndromes.
Antibodies include contactin-associated proteinlike 2 (CASPR2) antibody,
voltage-gated calcium channel antibodies (both P/Q type and N type), anti-Tr
(delta/notchlike epidermal growth factor-related [DNER]) antibody, and
metabotropic glutamate receptor 1 (mGLuR1) antibody. These antibodies exert
direct effects after binding, and functional modifications of ion channels and
neurotransmitter receptors lead to impairments in vestibulocerebellar
pathways.47 They are overall less likely to be associated with malignancies and
generally respond more positively to immunotherapy, leading to better
long-term prognoses.
Immunotherapy is the first treatment for an autoimmune vestibulocerebellar
syndrome, either at the time of diagnosis or when the diagnosis is strongly
suspected based on clinical observations and data. Corticosteroids, plasma
exchange, and IV immunoglobulin (IVIg) are all potential treatment options. If
these treatments fail, the second line of treatment usually consists of either
rituximab or cyclophosphamide.47 A maintenance form of immunotherapy is
chosen depending on factors such as how the patients respond to initial
treatment, how their disease progresses, and their antibody type. A variety of
options for maintenance treatment exist, including rituximab, cyclophosphamide,
azathioprine, methotrexate, and IVIg. The specific course of treatment, including
dosing and duration of treatment, must be tailored to the individual patient.
Additionally, if an underlying tumor is found, tumor therapy must commence as
quickly as possible and can often be done in parallel to immunotherapy. A team of
specialists that includes an oncologist is usually necessary.

CONCLUSION
Vertigo and dizziness can be challenging symptoms to address, in part because a
description of these symptoms is often difficult for patients to formulate.
Whereas some etiologies are peripheral, others localize to the CNS. The process
of determining whether vertigo has a central etiology begins with meticulous
history taking followed by a detailed examination with particular attention to eye
movements, coordination, gait, and speech. Careful examination skills are
paramount in diagnosing central vertigo, as brain imaging has limitations in
certain etiologies. A variety of treatments ranging from medications
and therapies to even surgical interventions may be employed to treat
central vertigo.

CONTINUUMJOURNAL.COM 465

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

VERTIGO RELATED TO CNS DISORDERS

REFERENCES

1 Hain TC, Cherchi M. Vestibular testing. 15 Kim HA, Lee H, Kim JS. Vertigo due to vascular
Continuum (Minneap Minn) 2021; mechanisms. Semin Neurol 2020;40(1):067-075.
27(2, Neuro-otology):330-347. doi:10.1055/s-0039-3402737
2 Barmack NH. Central vestibular system: 16 Kim HA, Lee H. Recent advances in
vestibular nuclei and posterior cerebellum. Brain understanding audiovestibular loss of a vascular
Res Bull 2003;60(5-6):511-541. doi:10.1016/s0361- cause. J Stroke 2017;19(1):61-66. doi:10.5853/
9230(03)00055-8 jos.2016.00857
3 Baloh RW. Vestibular migraine I: mechanisms, 17 Pavlou M, Lingeswaran A, Davies RA, et al.
diagnosis, and clinical features. Semin Neurol Simulator based rehabilitation in refractory
2020;40(1):76-82. doi:10.1055/s-0039-3402735 dizziness. J Neurol 2004;251(8):983-995.
doi:10.1007/s00415-004-0476-2
4 Lempert T, Olesen J, Furman J, et al. Vestibular
migraine: diagnostic criteria. J Vestib Res 2012; 18 Ahsan SF, Standring R, Osborn DA, et al. Clinical
22(4):167-172. doi:10.3233/VES-2012-0453 predictors of abnormal magnetic resonance
imaging findings in patients with asymmetric
5 von Brevern M, Lempert T. Vestibular migraine.
sensorineural hearing loss. JAMA Otolaryngol
Handb Clin Neurol 2016;137:301-316. doi:10.1016/
Head Neck Surg 2015;141(5):451-456. doi:10.1001/
B978-0-444-63437-5.00022-4
jamaoto.2015.142
6 Frohman EM, Kramer PD, Dewey RB, et al. Benign
19 Kearns KN, Chen CJ, Tvrdik P, et al. Outcomes of
paroxysmal positioning vertigo in multiple
surgery for brainstem cavernous malformations:
sclerosis: diagnosis, pathophysiology and
a systematic review. Stroke 2019;50(10):
therapeutic techniques. Mult Scler 2003;9(3):
2964-2966. doi:10.1161/STROKEAHA.119.026120
250-255. doi:10.1191/1352458503ms901oa
20 Persad AR, Khormi YH, van Landeghem F, Chow
7 Gass A, Steinke W, Schwartz A, Hennerici MG.
MM. Unusual case of hemangioblastoma of the
High resolution magnetic resonance imaging in
cerebellopontine angle. Surg Neurol Int 2017;
peripheral vestibular dysfunction in multiple
8:264. doi:10.4103/sni.sni_310_17
sclerosis. J Neurol Neurosurg Psychiatry 1998;
65(6):945. doi:10.1136/jnnp.65.6.945 21 Kronenbuerger M, Olivi A, Zee D. Pearls &
Oy-sters: positional vertigo and vertical
8 Thomke F, Hopf HC. Pontine lesions mimicking
nystagmus in medulloblastoma: a picture is
acute peripheral vestibulopathy. J Neurol
worth a thousand words. Neurology 2018;90(4):
Neurosurg Psychiatry 1999;66:340-349.
e352-e354. doi:10.1212/WNL.0000000000004866
doi:10.1136/jnnp.66.3.340
22 Dichgans M, Jäger L, Mayer T, et al. Bacterial
9 Hebert J, Corboy J, Manago M, Schenkman M.
meningitis in adults: demonstration of inner ear
Effects of vestibular rehabilitation on multiple
involvement using high-resolution MRI.
sclerosis–related fatigue and upright postural
Neurology 1999;52(5):1003. doi:10.1212/
control: a randomized controlled trial. Phys Ther
WNL.52.5.1003
2011;91(8):1166-1183. doi:10.2522/ptj.20100399
23 Campos LG, Trindade RA, Faistauer Â, et al.
10 Anagnostou E, Mandellos D, Limbitaki G, et al.
Rhombencephalitis: pictorial essay. Radiol Bras
Positional nystagmus and vertigo due to a solitary
2016;49(5):329-336. doi:10.1590/0100-3984.
brachium conjunctivum plaque. J Neurol
2015.0189
Neurosurg Psychiatry 2006;77(6):790-792.
doi:10.1136/jnnp.2005.084624 24 Colvin IB. Audiovestibular manifestations of
sarcoidosis: a review of the literature.
11 Serra A, Chisari CG, Matta M. Eye movement
Laryngoscope 2009;116(1):75-82. doi:10.1097/
abnormalities in multiple sclerosis: pathogenesis,
01.mlg.0000184580.52723.9f
modeling, and treatment. Front Neurol 2018;9:31.
doi:10.3389/fneur.2018.00031 25 Solomon D. Distinguishing and treating causes of
central vertigo. Otolaryngol Clin North Am 2000;
12 Kang S, Shaikh AG. Acquired pendular
33(3):579-601. doi:10.1016/s0030-6665(05)70228-0
nystagmus. J Neurol Sci 2017;375:8-17.
doi:10.1016/j.jns.2017.01.033 26 Charidimou A, Linn J, Vernooij MW, et al. Cortical
superficial siderosis: detection and clinical
13 Frohman EM, Zhang H, Dewey KS, et al. Vertigo in
significance in cerebral amyloid angiopathy and
MS: utility of positional and particle repositioning
related conditions. Brain 2015;138(pt 8):2126-2139.
maneuvers. Neurology 2000;55(10):1566-1569.
doi:10.1093/brain/awv162
doi:10.1212/wnl.55.10.1566
27 Ramadass T, Narayanan N, Dhanraj M,
14 Kim HA, Lee SR, Lee H. Acute peripheral
Chidambarnathan N. Superficial siderosis of the
vestibular syndrome of a vascular cause. J Neurol
CNS and otoneurological manifestations. Indian J
Sci 2007;254(1-2):99-101. doi:10.1016/j.
Otolaryngol Head Neck Surg 2011;63(4):380-383.
jns.2006.12.015
doi:10.1007/s12070-011-0152-7

466 APRIL 2021

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

28 Yoo A, Jou J, Klopfenstein JD, Kattah JC. Focused 39 Benarroch EE. New findings on the
neuro-otological review of superficial siderosis neuropathology of multiple system atrophy.
of the central nervous system. Front Neurol 2018; Auton Neurosci 2002;96(1):59-62. doi:10.1016/
9:358. doi:10.3389/fneur.2018.00358 s1566-0702(01)00374-5
29 Sydlowski SA, Cevette MJ, Shallop J. Superficial 40 Spacey S. Episodic ataxia type 2. In: Adam MP,
siderosis of the central nervous system: Ardinger HH, Pagon RA, et al, editors.
phenotype and implications for audiology and GeneReviews® [Internet]. Seattle, WA: University
otology. Otol Neurotol 2011;32(6):900-908. of Washington, Seattle, 1993–2020.
doi:10.1097/MAO.0b013e31822558a9
41 Guterman EL, Yurgionas B, Nelson AB. Pearls &
30 Tyler G, Martin T, Baguley D. Systematic review of Oy-sters: episodic ataxia type 2: case report and
outcome of cochlear implantation in superficial review of the literature. Neurology 2016;86(23):
siderosis. Otol Neurotol 2012;33(6):976-982. e239-e241. doi:10.1212/WNL.0000000000002743
doi:10.1097/MAO.0b013e3182565a46
42 Strupp M, Kalla R, Claassen J, et al. A randomized
31 Lee SY, Lee DH, Bae YJ, et al. Bilateral trial of 4-aminopyridine in EA2 and related
vestibulopathy in superficial siderosis. Front familial episodic ataxias. Neurology 2011;77(3):
Neurol 2018;9:422. doi:10.3389/fneur.2018.00422 269-275. doi:10.1212/WNL.0b013e318225ab07
32 Offenbacher H, Fazekas F, Schmidt R, et al. 43 Strupp M, Kalla R, Dichgans M, et al. Treatment of
Superficial siderosis of the central nervous episodic ataxia type 2 with the potassium
system: MRI findings and clinical significance. channel blocker 4-aminopyridine. Neurology
Neuroradiology 1996;38(suppl 1):S51-S56. 2004;62(9):1623-1625. doi:10.1212/01.
doi:10.1007/BF02278119 wnl.0000125691.74109.53
33 Rivas Rodriguez F, Srinivasan A. Superficial 44 Strupp M, Muth C, Mansmann U. Fampridine and
siderosis of the CNS. Am J Roentgenol 2011;197: acetazolamide for the treatment of episodic
W149-W152. doi:10.2214/AJR.09.7215 ataxia type 2 (EAT2TREAT): a randomised,
double-blind, placebo-controlled, three-
34 Cossu G, Abbruzzese G, Forni GL, et al. Efficacy
period crossover trial (2331). Neurology 2020;
and safety of deferiprone for the treatment of
94(15 suppl):2331.
superficial siderosis: results from a long-term
observational study. Neurol Sci 2019;40(7): 45 Afzal S, Recio M, Shamim S. Paraneoplastic
1357-1361. doi:10.1007/s10072-019-03847-x cerebellar ataxia and the paraneoplastic
syndromes. Proc (Bayl Univ Med Cent) 2015;28(2):
35 Zwergal A, Feil K, Schniepp R, Strupp M.
217-220. doi:10.1080/08998280.2015.11929234
Cerebellar dizziness and vertigo: etiologies,
diagnostic assessment, and treatment. Semin 46 Hasadsri L, Lee J, Wang BH, et al. Anti-Yo
Neurol 2020;40(1):87-96. doi:10.1055/s-0039- associated paraneoplastic cerebellar
3400315 degeneration in a man with large cell cancer of
the lung. Case Rep Neurol Med 2013;2013:725936.
36 Kalla R, Deutschlander A, Hufner K, et al.
doi:10.1155/2013/725936
Detection of floccular hypometabolism in
downbeat nystagmus by fMRI. Neurology 2006; 47 Narayan RN, McKeon A, Fife TD. Autoimmune
66(2):281-283. doi:10.1212/01.wnl.0000194242. vestibulocerebellar syndromes. Semin Neurol
28018.d9 2020;40(1):97-115. doi:10.1055/s-0039-3402061
37 Strupp M, Teufel J, Zwergal A, et al. 48 Darnell RB, Posner JB. Paraneoplastic syndromes
Aminopyridines for the treatment of neurologic involving the nervous system. N Engl J Med 2003;
disorders. Neurol Clin Pract 2017;7(1):65-76. 349(16):1543-1554. doi:10.1056/NEJMra023009
doi:10.1212/CPJ.0000000000000321
49 Dalmau J, Rosenfeld MR. Paraneoplastic
38 Benarroch E. The central autonomic network: syndromes of the CNS. Lancet Neurol 2008;7(4):
functional organization, dysfunction, and 327-340. doi:10.1016/S1474-4422(08)70060-7
perspective. Mayo Clin Proc 1993;68(10):988-1001.
doi:10.1016/s0025-6196(12)62272-1

CONTINUUMJOURNAL.COM 467

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.