Download to read offline
More Related Content
Kmenieres
- 1. Menie`re’s disease Dr. KrupalA. Pardhi PG Resident II Year Dept. of E.N.T. & H.N.S SKNMC & GH,Pune
- 2. Introduction • It’s anidiopathic endolymphatic hydrops & a disorder of the inner ear. • It was first described by Prosper Meniere in 1861 • DEFINITION- Menie`re’s disease is a disorder characterized by spontaneous attacks of vertigo, with associated fluctuating sensorineural hearing loss, tinnitus, and aural fullness.
- 3. Etiology • Exact causeof the disease is not yet known. • Viral: The causative roles of viruses (HSV, cytomegalovirus or VZV) remain uncertain. • „Hereditary: Familial occurrence in 10–20% cases. There is an autosomal dominant mode of inheritance. • Autoimmune: Certain genetically acquired major histocompatibility complexes specifically human leukocyte antigens (HLA) B8/DR3 and Cw7 have been associated with Meniere’s disease.
- 4. Etiology & pathogenesis •overproduction or malabsorption of endolymph, result in endolymphatic hypertension. • Endolymphatic hypertension,in turn, results in gross enlargement of the membranous labyrinth – so-called endolymphatic hydrops. • Defective absorption by endolymphatic sac- -Poor vascularity of sac -Less absorptive tubular epithelium - increased perisaccular fibrosis
- 5. Overaccumulation of endolymph atthe expense of perilymphatic space results in the distortion of membranous labyrinth. Alterations in the size of endolymphatic duct and sac along with reductions in tubular specializations of the lining of these structures have been observed.
- 7. • Delayed endolymphatichydrops can occure after many years in - syphilis - mumps, - Cogan's syndrome, - trauma and even - chronic suppurative otitis media, • This produce the clinical picture of Meniere's disease - so-called Meniere's syndrome. • Sometimes the attacks of vertigo can occuer in the other, previously normal, ear this is known as contralateral delayed endolymphatic hydrops,
- 8. CLINICAL MANIFESTATIONS A typicalattack has three phases, each defined by the direction of the spontaneous nystagmus. • 1.Irritative phase- the nystagmus, usually horizontal or horizontal-torsional,beats towards the affected ear; this phase usually lasts less than an hour. • 2.Paretic phase- the nystagmus beats away from the affected ear; this phase lasts several hours, sometimes even a day or two. • 3.Recovery phase-the nystagmus again beats towards the affected side; this phase lasts about as long as the second phase.
- 9. The SNHL inMeniere’s disease typically is- • fluctuating and progressive. • It often occurs with the sensation of fullness or pressure in the ear. • A pattern of low-frequency fluctuating loss and a coincident nonchanging, highfrequency loss is described, • a “peaked” or “tent-like” tracing on the audiogram is obtained. • This peak classically occurs at 2 kHz. • Over time, the hearing loss normalizes across frequencies and becomes less variable. • In only 1% to 2% the hearing loss progress to profound deafness. • Additional features include Diplacusis (43.6%) , a difference in the perception of pitch between the ears recruitment (56%).
- 10. Tinnitus • tends tobe nonpulsatile • described as whistling or roaring. • It may be continuous or intermittent. • Tinnitus often begins, gets louder, or changes pitch as an attack approaches. • Frequently a period of improvement follows the attack.
- 11. Audiogram in Meniere'sdisease. This patient, a 14-year-old female, began to have attacks of acute spontaneous vertigo with right-sided aural fullness and then tinnitus. (a) Audiogram on presentation shows a 60 dB low-frequency Hearing loss with normal acoustic reflexes (Z), indicating a fully recruiting cochlear hearing loss. (b) Audiogram after four months of treatment with a rigorous low-sodium diet shows normal pure-tone thresholds.
- 12. • In theearly stages of the disease, most patients are well between attacks. • As the disease progresses, persistent hearing loss, tinnitus and postural imbalance persist. • Some patients, particularly those in the later stages of the disease, develop drop attacks (Tumarkin or otolithic crises), • recurrent vestibulopathy and atypical Meniere’s disease describe the condition on persons with less than the classic triad of hearing loss, vertigo, and sensation of aural fullness or tinnitus.
- 13. Tumarkin or otolithiccrises • Are thought to occur as a result of acute otolithic dysfunction. • The patient simply drops to the ground without warning and can sustain a fracture or other serious injury. • The patient may describe a sensation of being pushed to the ground by some external force during these attacks. • There is no associated vertigo or loss of consciousness. • Some patients may have only one or two of these attacks during the course of their illness, while others have repeated attacks.
- 14. Lermoyez syndrome • Describedan unusual clinical presentation in which tinnitus and hearing loss precede the onset of vertigo. • When the vertiginous episode occurs, the tinnitus and hearing loss dramatically resolve. • The temporal bone studies in a patient with such attacks noted hydrops and membrane ruptures isolated to the basal turns of the cochlea and the saccule.
- 15. AAO-HNS Criteria forMeniere’s Disease Diagnosis Major Symptoms Vertigo • Recurrent, well-defined episodes of spinning or rotation • Duration from 20 minutes to 24 hours. • Nystagmus associated with attacks • Nausea and vomiting during vertigo • No neurologic symptoms with vertigo Deafness • Hearing deficits fluctuate • Sensorineural hearing loss • Hearing loss progressive, usually unilateral Tinnitus • Variable, often low-pitched and louder during attacks • Usually unilateral • Subjective
- 16. Diagnosis Possible Meniere’s disease -Episodic vertigo without hearing loss or - Sensorineural hearing loss, fluctuating or fixed, with dysequilibrium, but without definite episodes - Other causes excluded Probable Meniere’s disease - One definitive episode of vertigo - Hearing loss documented by audiogram at least once - Tinnitus or sense of aural fullness in the presumed affected ear - Other causes excluded
- 17. • Definite Meniere’sdisease -Two or more definitive spontaneous episodes of vertigo lasting at least 20minutes - Audiometrically documented hearing loss on at least one occasion - Tinnitus or sense of aural fullness in the presumed affected ear - Other causes excluded • Certain Meniere’s disease - Definite Meniere’s disease, plus histopathologic confirmation
- 18. • Glycerol Dehydrationtest: Ingestion of glycerol 1.5 g/kg mixed with equal volume of water is followed by serial audiograms over 3 hours. - Positive test: 25 dB shift at three consecutive frequencies, or 16% improvement in speech discrimination. • Glycerol testing is not commonly used today because of headaches as a frequent side effect.
- 19. Investigations • The calorictest The caloric test often can localize the involved ear. • it reveals a canal paresis on the affected side (most common) but sometimes there is directional preponderance to healthy side or a combination of both.
- 20. Investigations Electrocochleography • The summatingpotential (SP) is larger and more negative due to the distension of basilar membrane into scala tympani. • Action potential (AP) of CN VIII: Reduction in amplitude. • The most commonly used value is the ratio of amplitudes of the SP and the eighth cranial nerve action potential (AP), the SP/AP ratio : Increases. • It is most sensitive and specific for Meniere's disease when tone-burst and click stimuli are used • Giving patients 4 g of oral sodium chloride for three days prior to the electrocochleogram may increase the sensitivity of the test.
- 22. Treatment • Therapy ofMeniere’s disease is aimed at the reduction of its associated symptoms. • Currently, almost all proven therapies are directed at relieving vertigo, which usually is the most distressing symptom.
- 23. Acute Attack • Reassuranceand bed rest • Vestibular sedatives: They are given parenteral if vomiting precludes oral use. They take care of vertigo and anxiety and include prochlorperazine (Stemetil), promethazine theoclate (Avomine), dimenhydrinate (Dramamine), and diazepam. Atropine can also be very effective. • Vasodilators: – Inhalation of carbogen (5% CO2 with 95% O2) causes cerebral vasodilation and improves labyrinthine circulation. – Histamine diphosphate 2.75 mg in 500 ml glucose slow IV drip helps in managing acute episodes but is contraindicated in asthmatics and should not be given empty stomach. The adverse side effects include tachycardia, disturbed cardiac rhythm, hypotension, hyperthermia and bronchospasm.
- 24. Chronic phase Dietary Modificationand Diuretics • Salt restriction and diuresis may be the best initial therapy for Meniere’s disease. • Carbonic anhydrase inhibitors such as acetazolamide were recommended on the basis of the localization of carbonic anhydrase to the dark cells and the stria vascularis. • However, these agents have not proved to be clinically more effective than other diuretics. • Despite the lack of hard evidence of their efficacy, a low-salt diet and diuresis constitute an appropriate and effective treatment for Meniere’s disease with a low risk of side effects • Frustenberg Regimen - 1. Low salt diet 2. Diuretics + Pot. chlor 3. High protein
- 25. Vasodilators • Betahistine isan oral preparation of histamine. • It has proved to be effective in treatment of Meniere’s disease in a placebo-controlled study. Symptomatic Treatment • Antivertiginous medications, antiemetics, sedatives, antidepressants, and psychiatric treatment have been reported to be beneficial in reducing the severity of the vertigo and vegetative symptoms and in improving tolerance of Meniere’s symptoms.
- 26. Local Overpressure Therapy •A relatively recent approach to decrease hydrops by pulsing pressure in the middle ear. • overpressure in the middle ear was reported to decrease Meniere’s symptoms during acute vertigo attacks. • Since 2000, the Meniett device has been approved for use by the U.S. Food and Drug Administration (FDA). • The pressure is delivered in complex pulses of up to 20 cm of water, over a 5 minute period. • A randomized controlled trial demonstrated that patients using the Meniett device experienced a significant decrease in vertigo symptoms for the first 3 months of therapy • Long-term treatment with the Meniett device has shown results similar to the natural course of Meniere’s disease. • simple placement of a ventilation tube with no additional therapy also has been reported to control vertigo symptoms
- 27. Intratympanic Injection • Theterm chemical labyrinthectomy often is applied to intratympanic gentamicin treatment, • Gentamicin has a vestibulotoxicity that is high relative to its cochleotoxicity; • The gentamicin can be administered through either a tympanostomy tube or directly injected through the tympanic membrane. • The risk of hearing loss with gentamicin using many current protocols is similar to that with the natural history of Meniere’s disease. • Intratympanic injection of dexamethasone is a reasonable procedure to offer when vertigo is intractable • Dexamethasone injections may need to be repeated every 3 months to maintain the patient free of vertigo symptoms.
- 28. Surgical treatments Endolymphatic sacsurgery • may result in a reduction in the frequency, duration and intensity of vertigo attacks. • it is not always effective in stopping the vertigo attacks • no benefits for the auditory symptoms. Selective vestibular neurectomy • effective in stopping vertigo attacks in most patients • Vestibular nerve section by Middle Fossa Approach or Retrolabyrinthine Approach • hearing loss and facial paralysis is potential complications. surgical labyrinthectomy • can stop the vertigo attacks at the expense of losing any • remaining hearing on that side.
- 29. • Sacculotomy -puncture ofthe stapes footplate with a sharp needle to rupture the underlying saccule.
- 30. • Endolymphatic sacsurgery The endolymphatic sac is located by tracing an imaginary (Donaldson’s) line through the horizontal semicircular canal, perpendicular to and bisecting the posterior semicircular canal. • Endolymphatic sac procedure. A, A standard simple mastoidectomy is performed. The middle and posterior fossa dura plates, sinodural angle, sigmoid sinus, and antrum are identified. The horizontal canal and incus are then identified as well as the digastric ridge. The facial nerve is skeletonized from the horizontal canal to the stylomastoid foramen
- 31. B, The posteriorsemicircular canal is identified and the posterior fossa dura plate is removed between the sigmoid sinus and the posterior canal. C,The upper edge of the endolymphatic sac is identified; it generally lies at or below Donaldson’s line
- 32. Endolymphatic subarachnoid shunt. A,After opening the lateral wall of the endolymphatic sac, an incision is made into the medial wall and the posterior fossa to open into the lateral prolongation of the basal cistern.
- 33. B, A silicone(Silastic) shunt tube is inserted to maintain a drainage route between the endolymphatic sac and the basal cistern. A muscle plug is usually sutured over the lateral aspect of the endolymphatic sac to facilitate closure and prevent cerebrospinal fluid leak.
- 34. Paparella’s technique ofendolymphatic mastoid shunting. A “T”-shaped piece of silicone (Silastic) is inserted into the open endolymphatic sac to form a permanent fistula between the lumen of the sac and the mastoid cavity.
- 35. Surgical labyrinthectomy • TranscanalLabyrinthectomy • Transmastoid Labyrinthectomy
- 36. Schuknecht’s transtympanic labyrinthotomyfor Meniere’s disease.
- 37. Transmastoid labyrinthectomy. A, Apostauricular incision is made. B, A complete mastoidectomy is performed. The fossa incudis, digastric ridge, and vertical portion of the facial nerve are identified. The facial recess is opened. C, The incudostapedial joint is disarticulated and the incus removed. The semicircular canals are then opened, beginning with the horizontal canal and continuing to the posterior and finally the superior canals.
- 38. A, The ampullaeand neuroepithelium of the three semicircular canals are exposed, as are the saccule and utricle. B, The neuroepithelium is removed under direct visualization.
- 39.