MENIERE’S DISEASE

MENIERE’S DISEASE
Dr Harjitpal Singh
Assistant Professor(ENT),
Dr RKGMC, Hamirpur.

Meniere’s Disease
• Known after the name of a French physician Dr
Prosper Meniere who described it in 1861.
• Also called endolymphatic hydrops.
• Characterized by –
Vertigo.
Fluctuating SNHL.
Tinnitus.
Aural fullness.

ETIOLOGY
• Normally, endolymph moves from cochlea, (where
it is produced,) to endolymphatic sac, where it
is absorbed.
• Increased volume of endolymph.
• Due to –
Increased production by stria vascularis.
Decreased absorption by endolyphatic sac.
Both.

ETIOLOGY(cont.)
• Ischemia and vasospasm causing decreased blood supply of
labyrinth,
• Emotional factors contribute to the development of the
disease by causing sympathetic system overactivity
Increased sympathetic activity Arterial spasm 
Anoxia of stria vascularis Increased permeability
Increased transudation and endolymph production
 Meniere's disease.
• Hormonal disturbances by causing water and electrolyte
disturbances in endolymph, hypothyroidism (3% cases).
• Allergy and infection may also be responsible

ETIOLOGY(cont.)
• Autoimmune disorders.
• Viral infection.
• Metabolic disorders of carbohydrate metabolism.
• Syphilis and cochlear otosclerosis.
• Anatomical causes such as small vestibular
aqueduct
• Trauma, which may be physical or acoustic.
• Idiopathic

AETIOLOGIC FACTOR AND SYMPTOMATOLOGY

PATHOLOGY
• Distension of endolyphatic system – cochlear
aqueduct(scala media), saccule, utricle and semicircular
canals.
• Marked bulging of reissner’s membrane and herniation
into scala tympani.
• The distended saccule may come to lie against the stapes
footplate.
• The utricle and saccule may show outpouchings into the
semicircular canals.
• Distortion of cochlear duct produces hearing loss while
due to pressure changes, the labyrinth causes vertigo

(A) Normal cochlear duct. (B) Cochlear duct is distended with endolymph
pushing the Reissner’s membrane into scala vestibuli.

CLINICAL FEATURES
• Age group: 35-60 yrs.
• Males >? females. (hormonal :pregnant female
more prone)
• Usually unilateral.
• Incidence of 15 per 100,000.
• Prevalence of 218 per 100,000.
• 50% have positive family history

CLINICAL FEATURES(cont)
VERTIGO
• Due to periodic rupture of membrane.
• Attacks followed by periods of remission.
• Recurrent, well-defined episodes of spinning or
rotation with duration from 20min to 24 hr
• Nystagmus associated with attacks
• Nausea, vomiting, ataxia
• Bradycardia, pallor, cold sweats.
• Preceded by aural fullness in some cases.
• Tullio phenomenon : loud noise vertigo.

HEARING LOSS
• Characteristic fluctuating hearing loss.
• Precedes vertigo.
• Improves after the attack.
• Progressive loss with each attack.
• Diplacusis : A tone is normal in one ear and
higher pitch in the other.
• Recruitment : Slight increase in intensity is
perceived as abnormally loud.

TINNITUS
• Low pitched.
• Aggravated during attacks.
• May persist even after hearing improvment.
• Usually unilateral.

AURAL FULLNESS
• Fluctuating in nature.
• Precedes an attack of vertigo.

DIAGNOSIS
• Possible:
no definitive episode.
Episodic vertigo without hearing loss
• Probable:
One definitive episode
Hearing loss recorded in PTA at least once
Tinnitus or aural fullness
• Definite:
Two or more episodes of vertigo lasting 20 mins.
Hearing loss recorded in PTA at least once
Tinnitus or aural fullness
• Certain: definite plus HPE confirmation

MENIERE’S SYNDROME
• Also called secondary Meniere's disease.
• Symptoms similar to Meniere's disease.
• Causes:
Trauma.
Viral infections.
Syphilis.
Cogan’s syndrome.
Otosclerosis.
Autoimmune.

VARIANTS OF MENIERE’S
• Cochlear hydrops: only cochlear symptoms.
No vertigo.
• Vestibular hydrops: only episodic vertigo. No
cochlear symptoms.
• Drop attacks: Tumarkin’s otolithic crisis:
sudden drop attack. No vertigo. No hearing
loss.
• Lermoyez syndrome: symptoms in reverse
order. Hearing loss followed by vertigo.

EXAMINATION
• Ear examination is within normal limits.
• Tympanic membrane is intact.
• Tuning fork tests show sensorineural type of
hearing loss.
• In acute attacks, nystagmus positive. Towards
the normal side.

INVESTIGATIONS
PURE TONE AUDIOMETRY
• Sensorineural type of graph.
• No air – bone gap.
• Initially, only lower freq affected  rising
graph.
• As higher freq are affected, flat graph
downward sloping graph.

INVESTIGATIONS(cont)
SPEECH AUDIOMETRY
• Speech discrimination score is between 55 -85 %.
• Even lower during acute attacks.
• Showing cochlear pathology.
• Normal - >90%
• Cochlear – below 90%.

SPECIAL AUDIOMETRY TESTS
• Recruitment test positive.
• SISI score of over 70%.
• Tone decay test shows decay of about 20 dB.
• These indicate a cochlear pathology.

CALORIC TESTS
• Cold caloric test shows reduced or absent
response on the affected side.
• Often, it reveals a canal paresis on the
affected side (most common).

ELECTROCOCHLEOGRAPHY
• Measures the electrical activity of cochlea and
the auditory nerve.
• Measures :
• CM: cochlear microphonics.
• SP: summating potential.
• AP: action potential.

• Active electrode: Transtympanically over the
promontory.
• Reference electrode: mastoid.
• Ground electrode: forehead.
• Graph with latency on x – axis and amplitude on
y – axis.

• In Meniere's disease, basilar membrane
becomes taut causing increase in summating
potential.
• SP:AP ratio increases.
• Normal: 30%.
• Meniere’s: > 30%.
• Best obtained in acute phase.

GLYCEROL DEHYDRATION TEST
• Originally introduced by Klockhoff and
Lindblom in 1966.
• Initially performed using K+ sparing diuretics.
• Glycerol acts as dehydrating agent and
reduces the endolymph pressure and
improves symptoms.
• Tinnitus and aural fullness also improves.

GLYCEROL DEHYDRATION TEST
• 10dB improvement in 2 or more adjecent
octaves or 10% improvement in SDS after 1-2
hours is considered test positive.
• Test can be combined with transtympanic
electro – cochleography.
• Mannitol and ethanol easily penetrate the
cochlear membranes – not effective.
• Isosorbide – under trial.

ACETAZOLAMIDE TEST
• Intravenous acetazolamide considerably
reduces the volumes and pressures of the
perilymph and endolymph by inhibiting
carbonic anhydrase.
• Acetazolamide 500 mg in aqueous solution is
injected intravenously over one minute.

ACETAZOLAMIDE TEST
• Electrocochleogram monitored continuously
for 45 minutes.
• Improvement is seen within 10 -15 minutes.
• Effective even in patients in remission.
• Faster than glycerol test.

STAGING OF MENIERE’S DISEASE
• It is based on pure tone average in dB in
previous 6 months.
• Stage I—less than 25 dB
• Stage II—26–40 dB
• Stage III—41–70 dB
• Stage IV—more than 70 dB.

TREATMENT OF MENIERE’S DISEASE

TREATMENT – CHRONIC PHASE
• Vestibular sedatives, Vasodilators, Diuretics
• Probanthine 15 mg thrice a day is effective
• Hormones after finding out any endocrinal disorder such as
thyroid profile
• Chemical labyrinthectomy with injection Gentamicin, which is
vestibulotoxic and is given in middle ear daily or weekly.
Gentamycin profusion of round window may give 80 to 90
percent control.
• In bilateral disease injection streptomycin intramuscular is given.
• Microwick:It is a small wick made of polyvinyl acetate It is meant
to deliver drugs from external canal to the inner ear and
thus avoid repeated intratympanic injections. It requires a
grommet to be inserted into the tympanic membrane.

INTRATYMPANIC DELIVERY TECHNIQUES
• Direct injection.
• Through tympanostomy tube.
• Indwelling catheter.
• Sponge placed through TM.
• Injection into round window niche.
• Mini pumps

CRITERIA FOR ABLATIVE PROCEDURES
• Only in definite Meniere’s disease.
• Ablative procedure done in long standing,
unilateral Meniere's disease with no
evidence of disease in contralateral ear at
time of procedure.
• Advanced age is a relative contraindication.

CHEMICAL LABYRINTHECTOMY
• Intra – tympanic gentamicin injection.
• Vestibulotoxic > cochleotoxic.
• Less effect on hearing.
• Methods:
5 times a day via tympanostomy tube.
3 times a day via microcatheter (4 days).
Weekly once injection for 4 weeks.
Single titrated dose injection + repeat dose if
needed.

CHEMICAL LABYRINTHECTOMY(cont)
• Gets concentrated in hair cells.
• Destroys type I hair cells more than type II.
• Recurrence is common.
• Hearing loss is inevitable.
• Less effective than surgical labyrinthectomy or
vestibular neurectomy.

AIMS OF SURGERY
• Abolish /alter the function of diseased labyrinth.
• Modification of underlying pathology.
• Altering production or distribution of endolymph.

SURGICAL TREATMENT
• Conservative Procedures.
 Decompression of endolymphatic sac,
 Endolymphatic shunt operation.
 Sacculotomy (Fick’s operation).
 Section of vestibular nerve.
 Ultrasonic destruction of vestibular labyrinth
• Destructive Procedures.
 Labyrinthectomy
• Intermittent Low-Pressure Pulse Therapy
[Meniett Device Therapy]

SURGICAL TREATMENT(cont)
ENDOLYMPHATIC SAC DECOMPRESSION
• By this operation pressure in the sac is relieved and patient
becomes free of symptoms.
• The procedure is done under local or general anesthesia.
• A simple mastoidectomy is done and three SCC are
delineated.
• Endolymphatic sac lies close to posterior SCC keeping in
mind the Donaldson’s line.
• Bone in this area is drilled with a diamond burr to expose
the sac, which gets decompressed thus relieving the
symptoms without affecting the hearing.

ENDOLYMPHATIC SAC DECOMPRESSION
• Rationale:
Release of pressure in the sac.
Neovascularisation of saccule.
Passive diffusion of endolymph.
Creation of osmotic gradient out of the sac.

ENDOLYMPHATIC SHUNT OPERATION: A tube is put
between subarachnoid space and endolymphatic
sac for drainage of excessive fluid.
SACCULOTOMY (Fick procedure): Saccule is
punctured with a needle through stapes
footplate. Cody’s tack procedure, places a
stainless steel tack to periodically decompress the
sac.

VESTIBULAR NERVE SECTION: This involves selective
section of the vestibular division of the eighth nerve,
particularly in cases with intractable vertigo but with
a good hearing. The middle cranial fossa approach to
the eighth nerve is chosen. 85 - 95 % improvement in
vertigo & 80 – 90 % preservation of hearing.
ULTRASONIC DESTRUCTION OF VESTIBULAR LABYRINTH:
Ultrasonic vibrations are passed to the semicircular
canal by an applicator through the mastoid route.
Cochlear function is preserved.

SURGICAL LABYRINTHECTOMY:
• Most destructive procedure. Used only when
cochlear function is not serviceable.
• Complete destruction of vestibular and auditory
function.
• Ideal for patients with complete hearing loss.
• Transcanal and transmastoid approach.

SURGICAL LABYRINTHECTOMY: Transcanal approach
• Exposing middle ear through tympanomeatal flap.
• Removal of incus and stapes to expose oval
window.
• Hook is introduced into vestibule to remove entire
neuroepithelium.
• Drilling over promontory to join oval and round
window can be done.
• Limited exposure.

SURGICAL LABYRINTHECTOMY: Transmastoid approach
• More common and better exposure.
• Mastoidectomy to expose the HSCC.
• Superior and posterior canals identified by drilling
superiorly and posteriorly resp.
• Canals followed to identify vestibule and
neuroepithelium is removed.
• Bonewax can be used to preserve some amount
of hearing.

MENIETT DEVICE THERAPY
• Increasing the middle ear pressure decreases
the vertigo.
• Meniett device: hand held air pressure
generator. Can be self administered 3 times a
day.
• Needs tympanostomy tube placement prior to
start of therapy.
• Significant effect for first 3 months of use.

MENIETT DEVICE THERAPY(cont)
Pressure waves pass through ventilation tube (1) to round window membrane (2)
and transmitted to perilymph (yellow) and compress endolymphatic labyrinth
(blue) to redistribute endolymph pressure to sac (3) and blood vessels (4).

VESTIBULAR NEURECTOMY
• Involves transection on the vestibular nerve.
• 85 - 95 % improvement in vertigo.
• 80 – 90 % preservation of hearing.
• Retrosigmoid approach: most common.
• Middle cranial fossa approach.
• Retro labyrinthine and transmeatal approach:
abandoned due to less exposure.

Meniere’s Disease
SUMMARY
• Tuning fork tests show fluctuating SNHL.
• Pure tone audiometry shows low-tone SNHL.
• Impaired speech discrimination during the attack. •
Caloric test shows canal paresis.
• Recruitment test positve.
• Short increment sensitivity index (SISI) test score is
better than 70 percent in most of the cases.
• Tone decay of less than 20 percent.
• Glycerol test 1 to 2 mL/kg in lemon water is given and
improvement in hear

FLUCTUATING HEARING LOSS
Feature of:
• Meniere’s disease
• Lermoyez syndrome
• Labyrinthine fistula
• Metabolic diseases like diabetes, hypothyroidism, hyperlipidemia
• Glomus jugulare
• Glue ear
• Eustachian tube dysfunction

MENIERE’S DISEASE

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MENIERE’S DISEASE