DR.KAUSHIK SUTRADHAR

PGT , DEPTT. OF ENT

is a disorder of the inner ear characterized by

Acute rotatory vertigo which is unpredictible, precipitous in nature associated with nausea and/or vomiting.

Hearing loss which is unilateral low-tone fluctuant sensorineural

Tinnitus

Aural fullness

1747 – Antonio Scarpa described anatomy of membranous labyrinth

1861 – Prosper Meniere described the classic features of Meniere’s disease & attributed it to labyrinthine causes

1871 – Knappin theorized that dilated membranous labyrinth to be the cause of this disorder

1927 – Guild described endolymphatic ciruclation

1938 – Hallpike and Portmann described pathology of Meniere’s disease by studying temporal bones

Type I (aka inner)

Type II(aka outer)

With Kinocilium

VESTIBULAR HAIR CELLS

• Perilymph is similar in composition to CSF (Containing high Na and low K ions)

• Endolymph similar in composition to intracellular fluid (Containing low Na and high K concentration). It is secreted by stria vascularis

1.Secretory function2.Absorptive function3.Immune / defense function

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 4th and 5th

decades of life

Bilateral Meniere’s syndrome is seen in 5% of these patients

Idiopathic

Anatomical (small vestibular aqueduct)

Viral infection (HSV type 1)

Traumatic (physical, acoustic)

Allergy

Autoimmunity

CSOM

Syphilis

Otosclerosis

Longitudinal flow

Radial flow

Dynamic flow

Was first proposed by Guild

Striavascularis is the principal source

This is a slow process

Elimination occurs at the endolymphatic sac level

This is active process (energy consuming)

Production occurs from dark vestibular cells & planum semilunatum

Absorption occurs at the stria vestibularis

First proposed by Lawrence

This is a combination of both longitudinal and radial flow patterns

Endolymphatic hydrops causes distortion of membranous labyrinth

Pressure building up in the scala media may cause mirco ruptures of membranous labyrinth

This would account for the episodic nature of the attacks

Healing of these ruptures causes resolution of the disorder

Small amounts of excess endolymph can be cleared by radial flow

Larger volumes need longitudinal flow for their clearance

Endolymphatic sinus temporarily accommodates excess endolymph till the sac is ready for it

Endolymphatic valve of Bast isolates pars superior and prevents endolymph from draining out of the utricle

The excess volume tends to accumulate in the apical end of the cochlea, where the membranes are more lax than elsewhere, even though the endolymph pressure would be similar elsewhere in the cochlea.

1.Stage I – Patient has solely cochlear symptoms

2. Stages II – IV – Patients have progressively more cochlear and vestibular symptoms

3. Stage V – End stage Meniere’s disease (dead ear)

Classical Meniere’s disease

Vestibular Meniere’s disease – vestibular symptoms and aural pressure

Cochlear Meniere’s disease – cochlear symptoms and aural pressure

Lermoyez syndrome – Reverse Meniere’s

Tumarkin’s crisis – Utricular Meniere’s

variant of Meniere’s disease

sudden sensorineural hearing loss, which improves during or immediately after the attack of vertigo.

Cause is sudden spasm of the labyrinthine artery followed by immediate dilatation

AKA Tumarkin’s drop attacks

abrupt falling attacks of brief duration without loss of consciousness.

due to an enlarging utricle due to excess endolymphatic volume

Sensori neural in nature

Fluctuating and progressive

Affects low frequencies

Mild low frequency conductive hearing loss (rare)

Profound sensori neural hearing loss (End stage)

Roaring in nature

Could be continuous / intermittent

Non pulsatile in nature

Frequency of tinnitus corresponds to the region of cochlea which has suffered the maximum damage

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 spontaneous vertigo Audiometrically documented hearing loss at least during one

attack Tinnitus and aural fullness in the affected ear

Definitive Meniere’s disease

Two or more definitive episodes of spontaneous vertigo one atleast lasting for 20 mins.Audiometrically documented hearing loss at least on one occasion.

Tinnitus and aural fullness in the affected ear

History1. Nature of the sensation2. Timing of the initial spell3. Frequency and duration of the symptoms4. Precipitating factors

Vestibular tests Complete Haemogram Audiometry Loudness recruiment VEMP Dehydration tests Posturography Electronystagmography

This is abnormal growth in the perceived intensity of sound

This is usually positive in patients with Meniere’s disease

ABLB is the test used to look for the presence of recruitment

This test is really time consuming

Alternate Binaural loudness balance test

Tone is presented alternately between the two ears. The level of the tone stays the same in one ear (i.e. fixed ear) and is varied up / down in the other ear (i.e. variable ear). The patient is asked to report when the sound is louder in the right ear, louder in the left ear, and when it sounds equal in both ears.

Loudness balance is said to have been obtained when the patient indicates that the sound is heard equally in both ears. The tester then records the two levels in dB hearing level where the balance

has occurred.

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

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

Small distorted cochlear microphonics

Vestibular evoked myogenic potential Measures the relaxation of sternomastoid muscle in

response to ipsilateral click stimulus Brief high intensity ipsilateral clicks produce large

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

This test is due to the presence of vestibulo collicreflex

Afferent arises from sound responsive cells in the saccule, conducted via the inferior vestibular nerve.

Efferent is via vestibulo spinal tract Normal responses are composed of biphasic

(positive-negative) waves VEMP reveals saccular dysfunction

Glycerol Mannitol Frusemide Isosorbide These tests involve the subject ingesting

glycerol or mannitol and observing for a change in symptoms and a measurable improvement in hearingTests are positive if there is pure tone improvement of 10dB or more at two / more frequencies between 200-2000Hz

First introduced by Klockhoff and Lindblom –1966

Glycerol is administered in doses of 1.5 mg/kg body wt in empty stomach

Serum osmolality should increase at least by 10 mos/kg

Side effects include Headache, Nausea, vomiting, drowsiness

PTA is performed 2-3 hours after administration

False positivity is rare

Positivity depends on the phase of the disease

Antibodies to 68-kDa protein has been noted in many patients with meniere’s disease

the aim is to decrease the production or accumulation of the endolymph

CONSERATIVE Dietary sodium restriction (1mg/day) Restriction of caffeine and nicotine like substances Diuretics like bendroflurazide,dyazide, chlorthalidone Betahistine

histamine analogue with weak H1, H2 agonistic and moderate H3 antagonistic action

causes improved microvascular circulation in striaevascularis

inhibition of vestibular nuclei activity Calcium agonists

Steroids

1. Topical application via tympanostomy tubes

2. Shea et al reported 35.4% hearing improvement and complete vertigo control in 63.4% cases treated with 16 mg intratympanic and 16 mg i.v. dexamethasone for three consecutive days followed by oral dexamethasone

3. Silverstein microwick can be used for intratympanic drug administration

Intratympanic injection of aminoglycosides

a form of chemical labyrinthectomy, gentamycin therapy ablates the vestibular “dark cells” of the secretory epithelium thus decreasing endolymph production

response to this is measured by in response to rapid, rotatory head thrusts

Alternobaric oxygen therapy

Local overpressure therapy by means of Meniettdevice which applies intermittent micropressureto the inner ear via a tympanostomy tube

1. Diagnosis should be confirmed

2. Ventilation tube should be inserted

3. Patient should be trained for self administration of the treatment

4. Usually administered thrice a day about 5 mins each time

5. Treatment lasts for 5 weeks

1. Classic unilateral Meniere’s disease

2. Intense vestibular / cochlear symptoms

3. Failed medical therapy

4. Over 65 years of age

5. Imbalance / aural fullness / tinnitus after gentamycin treatment

1. Perilymph fistula

2. Acoustic neuroma / brain tumor

3. Retrocochlear damage

4. Low pressure hydrocephalus

1. Isordil2. ϒ – globulin3. Urea4. Glycerol5. Lithium6. Anticholinergics – Glycopyrrolate 1-2 mg /day7. Antidopaminergics – Droperidol 2.5 – 10 mg orally /

day8. Leuprolide acetate – Blocks normal sex hormone

production9. Innovar – A combination of droperidol and fentanyl

can be used to suppress vestibular symptoms (can replace endolymphatic sac surgery)

Endolymph decompression First described by portmann 1926 Via the round window by otic-periotic shunt that

perforates the basilar membrane

Cochleosacculotomy creates a fracture dislocation of osseous spiral laminaboth these procedures have highdegree of hearing loss

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

Simple decompression

Cannulation of endolymphatic duct

Endolymphatic drainage to the subarachnoid space

Drainage to mastoid

Removal of extraosseous portion of the sac

1. Labyrinthectomy

2. Translabyrinthine vestibular neurectomy

3. Retrolabyrinthine vestibular neurinectomy

4. Retrosigmoid vestibular neurinectomy

5. Middle cranial fossa vestibular neurinectomy

Vestibular neurectomy1. more complete vertigo control than shunt

procedures2. Lower risk of hearing loss than gentamicin

therapy3. Middle fossa approach, risk of facial nerve

injury is higher4. Suboccipital approach Labyrinthectomy1. Transcanal approach2. Transmastoid approach, more common

Vestibular rehabilatation

Cawthorne-Cooksey exercises