meniere’s disease
TRANSCRIPT
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DR.KAUSHIK SUTRADHAR
PGT , DEPTT. OF ENT
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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
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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
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Type I (aka inner)
Type II(aka outer)
With Kinocilium
VESTIBULAR HAIR CELLS
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• 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
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1.Secretory function2.Absorptive function3.Immune / defense function
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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
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Idiopathic
Anatomical (small vestibular aqueduct)
Viral infection (HSV type 1)
Traumatic (physical, acoustic)
Allergy
Autoimmunity
CSOM
Syphilis
Otosclerosis
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Longitudinal flow
Radial flow
Dynamic flow
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Was first proposed by Guild
Striavascularis is the principal source
This is a slow process
Elimination occurs at the endolymphatic sac level
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This is active process (energy consuming)
Production occurs from dark vestibular cells & planum semilunatum
Absorption occurs at the stria vestibularis
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First proposed by Lawrence
This is a combination of both longitudinal and radial flow patterns
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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
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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
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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.
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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)
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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
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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
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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
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Sensori neural in nature
Fluctuating and progressive
Affects low frequencies
Mild low frequency conductive hearing loss (rare)
Profound sensori neural hearing loss (End stage)
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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
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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
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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
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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
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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.
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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
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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
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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
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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
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Antibodies to 68-kDa protein has been noted in many patients with meniere’s disease
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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
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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
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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
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Local overpressure therapy by means of Meniettdevice which applies intermittent micropressureto the inner ear via a tympanostomy tube
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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
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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
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1. Perilymph fistula
2. Acoustic neuroma / brain tumor
3. Retrocochlear damage
4. Low pressure hydrocephalus
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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)
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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
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Simple decompression
Cannulation of endolymphatic duct
Endolymphatic drainage to the subarachnoid space
Drainage to mastoid
Removal of extraosseous portion of the sac
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1. Labyrinthectomy
2. Translabyrinthine vestibular neurectomy
3. Retrolabyrinthine vestibular neurinectomy
4. Retrosigmoid vestibular neurinectomy
5. Middle cranial fossa vestibular neurinectomy
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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
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Vestibular rehabilatation
Cawthorne-Cooksey exercises
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