MENIERE’S DISEASE

PRESENTER DR. DEEPA SHIVNANI

Where do we stand?

1.150 years have passed since this syndrome was described

2.Amount of literature has virtually doubled3.cause is multifactorial4.Not all individuals with histological features of

Meniere’s disease manifested the classic clinical features

Introduction : History

• First described by Prosper Meniere in 1861.

• In 1902, Parry performed a CN VIII division for vertigo in a patient with suspected Meniere’s disease.

• Portman did endolymphatic sac decompression via a transmastoid approach in 1926.

• In 1931,McKenzie performed a selective vestibular neurectomy.

Introduction

• Meniere's disease (idiopathic endolymphatic hydrops) is a disorder of the inner ear associated with a symptoms consisting of

• spontaneous, episodic attacks of vertigo;• sensori neural hearing loss which usually

fluctuates • tinnitus • sensation of aural fullness.

INCIDENCE

Roughly 1 in 1000 individuals are affected Constitutes 10% of all patients attending vertigo clinic Female preponderance Rare in children under the age of 10 Commonly begins between 3th to 6th decades of life Bilateral Meniere’s syndrome is seen in 5% of these patients

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TYPES OF MENIERE'S DISEASE

1. Classical Meniere’s disease2. Vestibular Meniere’s disease – vestibular symptoms and aural pressure3. Cochlear Meniere’s disease – cochlear symptoms and aural pressure4. Lermoyez syndrome – Reverse Meniere’s5. Tumarkin’s crisis – Utricular Meniere’s

LERMOYEZ SYNDROME

This is a variant of Meniere’s disease. It is characterized by sudden sensori neural hearing loss which improves during or immediately after the attack of vertigo.

TUMARKIN’S DROP ATTACKS

abrupt falling attacks of brief duration without loss of consciousness. due to excess endolymphatic volume. Utricular crisis is used to indicate this condition.In the later disease stages the valve of Bast remaining patent may cause sudden drainage of endolymph from the utricle due to longitudinal flow resulting in these drop attacks

• Several pathophysiological mechanisms are thought to be implicated in the otolithic catastrophe of Tumarkin:

• sudden shift of the utricular macula, sudden changes in the endolymphatic fluid pressure, and sudden electrolyte changes secondary to the rupture of the membrane labyrinth.

• Thus, the inappropriate stimulation of the otolithic organs might generate a failure of the vestibulospinal reflex with the loss of postural tonus and, consequently, the falling

Etiology

Pathophysiology

• Theories behind endolymphatic hydrops– Obstruction of endolymphatic duct/sac– Hypoplasia of endolymphatic duct/sac– Alteration of absorption of endolymph– Alteration in production of endolymph– Autoimmune insult – Vascular origin– Viral etiology

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Normal membranous labyrinth Dilated membranous labyrinth in Meniere's disease (Hydrops)

PATHOLOGY

A. Defective absorption by endolymphatic sac-• Poor vascularity of sac• Less absorptive tubular epithelium• increased peri saccular fibrosisB. Rupture of reissner’s membreane leading to

mixing of perilymph & endolymph- Schuknecht• allow leakage of the potassium-rich endolymph

into the perilymph, bathing the eighth cranial nerve and lateral sides of the hair cells

Histopathological changes

• Loss of shorter stereocilia of outer hair cell first occuring in the apical region

• Outer hair cell->inner hair cell->intercellular edema b/w marginal cell->vacuolization->atrophy of marginal and intermediate cells-> loss of spiral ganglion cell

• High concentrations of extracellular potassium depolarize the nerve cells, causing their acute inactivation.

• The result is a decrease in auditory and vestibular neuronal outflow consistent with the hearing loss and features of acute vestibular paralysis seen in a typical Meniere's attack

• The chronic deterioration in inner ear function presumably is the effect of repeated exposure to the effects of the potassium

MECHANISM OF MENIERE’S DISEASE

HEARING LOSS

1. Sensori neural in nature2. Fluctuating and progressive3. Affects low frequencies4. Mild low frequency conductive hearing loss (rare)5. Profound sensori neural hearing loss (End stage)

TINNITUS

Roaring in nature subjective Could be continuous / intermittent Non pulsatile in nature Frequency of tinnitus corresponds to the region of cochlea which has

suffered the maximum damage

Diagnosis

Investigations

• Tuning forks tests : SNHL

• PTA • Speech audiometry• Recruitment test +ve• SISI >70%• Tone decay <20 dB

Investigations

• Caloric testing – canal paresis• ENG• Head Thurst test• ECoG – SP is larger & more negative• SP/AP ratio increases > 30%• Dehydration/Glycerol test• VEMP (Vestibular evoked myogenic potentials)

elevated threshold

Spontaneous nystagmus

• The direction of the observed spontaneous nystagmus varies; it can consistently beat toward the

• involved ear (irritative), • away from it (paralytic), or• change from an irritative to a paralytic pattern

over time, and thus cannot be used to lateralize the disease.

LOUDNESS RECRUITMENT

1. This is abnormal growth in the perceived intensity of sound2. This is usually positive in patients with Meniere’s disease3. ABLB (alternate binaural loudness balance test)is the test used to look

for the presence of recruitment4. This test is really time consuming

ELECTRO COCHLEOGRAPHY

1. Increased summating potential / action potential ratio. 1:3 is normal

2. Widened summating potential / action potential complex. A widening of greater than 2 ms is significant

VESTIBULAR TESTS

1. Not mandatory for diagnosis of Meniere’s disease2. Caloric test is still performed3. It is low frequency stimulation (0.003 Hz) of lateral canal4. Caloric asymmetry will point to the diseased ear5. 20% difference between the two ears (Jongkee’s formula) is significant

VEMP

1. Vestibular evoked myogenic potential2. Measures the relaxation of sternomastoid muscle in response to

ipsilateral click stimulus3. Brief high intensity ipsilateral clicks produce large short latency

inhibitory potentials (VEMP) in the toncially contracted Ipsilateral sternomastoid muscle

4. This test is due to the presence of vestibulo collic reflex5. Afferent arises from sound responsive cells in the saccule, conducted

via the inferior vestibular nerve.6. Efferent is via vestibulo spinal tract7. Normal responses are composed of biphasic (positive-negative) waves8. VEMP reveals saccular dysfunction

VEMPs

DEHYDRATION TESTS

1. Glycerol2. Frusemide3. Isosorbidethe test is considered positive if (1) there isa 10-dB or more improvement in pure tone thresholds at 2 or more frequencies (250 to 2000 Hz), or (2) there is a 12% or greater improvement in speech discrimination score.

GLYCEROL TEST

1. First introduced by Klockhoff and Lindblom – 19662. Glycerol is administered in doses of 1.5 mg/kg body wt in empty stomach3. Serum osmolality should increase at least by 10 mos/kg4. Side effects include Headache, Nausea, vomiting, drowsiness5. PTA is performed 2-3 hours after administration6. False positivity is rare7. Positivity depends on the phase of the disease

Management

• Conservative treatments include lifestyle and dietary adjustments,diuretics, and supplemental use of vestibular suppressants.

• Invasive or destructive procedures are indicated only in the 5% to 10% of patients with Meniere’s disease who fail conservative and medical measures.

• Overall, vertigo control is achieved in more than 99% of patients with Meniere’s disease.

MEDICAL MANAGEMENT

1. Dietary management2. Physiotherapy3. Psychological support4. Pharmacological intervention

Treatment

• Medical management –• ACUTE stage : labyrinth sedatives + anti-emetics• Carbogen, Histamine drip• Frustenberg Regimen -1. Low salt diet2. Diuretics + Pot. chlor3. High protein• Beta histine – to relieve vascular ischemia• Stop caffeine, nicotine, alcohol & tobacco

MEDICAL TREATMENTAcute Therapy

TREATMENT OF ACUTE EXACERBATION

1. Intravenous fluids – dehydration2. Vestibular suppressants – May delay recovery / rehabilitation process3. Corticosteroids – May help if tinnitus and deafness are debilitating

LOW SALT DIET

1. Frustenberg diet2. 2 grams / 24 hours (restricted salt

intake)3. Life style modification

ROLE OF DIURETICS

1. Diuretics play a vital role in alleviating acute symptoms2. This has been in use since 1930’s3. Thiazide group of drugs are commonly used4. Frusemide may be used to alleviate acute symptoms5. Clear scientific evidence is lacking regarding the usefulness of

diuretics

BETAHISTINE

1. Cochlear vascular insufficiency has been proposed as one of the mechanism of Meniere's disease

2. Betahistine is supposed to cause vasodilatation of cochlear blood vessels3. Betahistine has weak H1 agonistic property and considerable H3

antagonist properties4. It reduces the frequency & intensity of vertigo. Has minimal effect on

tinnitus5. Doesn’t help much with hearing loss

INTRATYMPANIC STEROIDS

1. Immune modulating effects2. Improves fluid dynamics of inner ear due to mineralocorticoid effects3. Vertigo was controlled on an immediate basis4. Methylprednisolone has the best effect as it penetrates the round

window better5. Silverstein microwick can be used for intratympanic drug

administration

OTHER TREATMENT MODALITIES (ANCILLARY)

1. Stress reduction2. Patient education3. Hearing aids – can be used to suppress troublesome tinnitus4. Tinnitus retraining

VIBRATOR THERAPY

1. Meniett Device 2. Low pressure pulse generator3. Vibrations are transmitted via external auditory

canal4. Vibrations alter inner ear fluid dynamics by their

effects on the oval and round windows5. Exact mechanism of action is not known6. It is totally non invasive7. This device is portable

VIBRATOR THERAPY STEPS

1. Diagnosis should be confirmed2. Ventilation tube should be inserted3. Patient should be trained for self administration of the treatment4. Usually administered thrice a day about 5 mins each time5. Treatment lasts for 5 weeks

INDICATIONS FOR VIBRATOR THERAPY

1. Classic unilateral Meniere’s disease2. Intense vestibular / cochlear symptoms3. Failed medical therapy4. Over 65 years of age5. Imbalance / aural fullness / tinnitus after gentamycin treatment

CONTRAINDICATIONS FOR VIBRATOR THERAPY

1. Perilymph fistula2. Acoustic neuroma / brain tumor3. Retrocochlear damage4. Low pressure hydrocephalus

ROLE OF AMINOGLYCOSIDES

1. Vestibulotoxic effects are put to therapeutic use.2. Sensation of vertigo reduced while hearing is preserved3. Streptomycin / gentamycin are predominantly Vestibulotoxic4. Intratympanic administration is preferred

INTRATYMPANIC GENTAMYCIN

1. Fixed dose protocol is used2. 40 mg/ml gentamycin is buffered with soda bicarb (pH6.4) final

concentration 26.7mg/ml.3. T tube grommet inserted into the postero inferior quadrant of ear drum.

A mcirocatheter is inserted through the grommet4. 1ml of gentamycin solution is injected into the middle ear cavity via the

microcatheter5. Three injections are given per day in outpatient setting6. Injections are given for 4 days7. After injection patient should lie supine with the infiltrated ear up for 30

mins8. Vertigo usually develops between 2-4 days after cessation of treatment

SURGICAL MANAGEMENT

1. Sac enhancement procedure2. Sac decompression procedure3. Labyrinthine ablative procedures

Endolymphatic Sac Surgery

– Decompression:Removal of bone overlying the sac

– Shunting:Placement of synthetic shunt to drain endolymph into mastoid

– Drainage:Incision of the sac to allow drainage

– Removal of sac:Excision of the sac

ABLATIVE PROCEDURES

1. Labyrinthectomy2. Translabyrinthine vestibular neurectomy3. Retrolabyrinthine vestibular neurinectomy4. Retrosigmoid vestibular neurinectomy5. Middle cranial fossa vestibular neurinectomy

SHUNT PROCEDURE

1. External shunts – Drains the sac into mastoid cavity / subarachnoid space2. Internal shunts – Drains excessive endolymph into the perilymphatic

space (cochleosacculotomy / labyrinthotomy)

COCHLEOSACCULOTOMY / LABYRINTHOTOMY

1. Helpful in treating debilitated patients2. Involves disruption of osseous spiral lamina3. Angular pick introduced via round window towards oval window. It

will accommodate 3 mm long pick4. After perforation the pick is withdrawn and the round window is

sealed by fat

Endolymphatic Sac Surgery

SAC IDENTIFICATION

THANK YOU……

ANY QUESTION?????