Transcript Document
Meniere’s Disease
Dr. Vishal Sharma
Introduction
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Described by Prosper Meniere in 1861
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Vertigo + Deafness + Tinnitus + Aural fullness
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Etiology: endolymphatic hydrops ( Hallpike, 1938) due to
ed absorption of endolymph or
ed production of endolymph
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Especially involves cochlear duct & saccule
Prosper Meniere`
Normal membranous labyrinth
Endolymphatic Hydrops
Normal membranous labyrinth
Endolymphatic Hydrops
Pathogenesis
1. Endolymphatic hydrops
rupture of membranous labyrinth potassium rich endolymph mixes with perilymph sustained inactivation of hair cells & neurons of vestibulo-cochlear nerve bathed in perilymph deafness + vertigo + tinnitus
2.
ed Sympathetic activity
ischemia of cochlear & vestibular end organs deafness + vertigo
Etiology of Primary Meniere’s disease
A. Idiopathic B. Increased production of endolymph:
Allergy
Sodium & water retention
Autoimmune
Viral infection
sympathetic activity
ischemia of stria vascularis
fluid transudation
Endocrine
Hypo (thyroidism, pituitarism, adrenalism), Diabetes, Hyperlipoproteinemia C. Decreased absorption of endolymph:
Small size of endolymphatic sac / duct
Obstruction of endolymphatic sac / duct
Ischaemia of endolymphatic sac
Inner ear trauma
Secondary Meniere Syndrome Clinically resembles Meniere’s disease. Seen in:
Syphilis
Otosclerosis,
Cogan syndrome (interstitial keratitis)
Post-stapedectomy
Paget’s disease
Clinical Features
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30 - 60 years, more in males, unilateral 1. Vertigo: Sudden onset, episodic, rotatory, 30 min - 24 hr, along with nausea, vomiting & diaphoresis. 85 % pt have positional vertigo
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Vertigo caused by loud, low frequency sound
Tulio phenomenon
Clinical Features 2. Deafness: Accompanies vertigo, improves after vertigo attack, sensori-neural, fluctuant, progressive
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Intolerance to loud sound (due to recruitment )
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Distortion of sound frequency, called diplacusis binauralis dysharmonica
Clinical Features 3. Tinnitus: Low-pitch, roaring, non-pulsatile, continuous / intermittent. Increased during vertigo attacks 4. Aural fullness:
F
luctuating, not relieved by swallowing 5. Emotional upset, anxiety, agoraphobia
AAO-HNS Diagnosis Criteria (1995) A. Vertigo:
Spontaneous, > 2 episodes lasting > 20 min
B. Audiogram documented sensori-neural deafness C. Tinnitus or Aural fullness in diseased ear D. Other cases excluded E. Staging as per pure tone average (500 - 3000 Hz): 1 = < 25 dB 2 = 26 - 40 dB 3 = 41 - 70 dB 4 = > 70 dB
Meniere’s disease variants
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Lermoyez’s reverse Meniere syndrome: Deafness
vertigo
improvement in hearing
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Tumarkin’s sudden drop attack: Pt falls without vertigo / loss of consciousness
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Meyerhoff’s oculo-vestibular response: Vertigo due to opto-kinetic stimulus
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Cochlear hydrops: deafness & tinnitus only
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Vestibular hydrops: vertigo only
E.N.T. Examination
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Otoscopy: normal tympanic membrane
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Nystagmus: irritative
paralytic
recovery
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False +ve fistula sign ( Hennebert sign ): in 30% pt
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Rinne test:
p
ositive (A.C. > B.C.)
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Weber test: lateralizes towards better ear
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A.B.C. test: decreased in diseased ear
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Irritative nystagmus: occurs immediately with onset of an attack, for 20 seconds, toward diseased ear , due to initial excitation of action potential by increasing potassium in perilymph
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Paralytic nystagmus: occurs minutes into an attack , toward healthy ear , due to blockade of action potential by increased K + in perilymph
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Recovery nystagmus: occurs hours later, toward diseased ear , due to vestibular adaptation
Pure Tone Audiometry
Rising curve in early stage Low frequency SNHL due to more fluid accumulation in apical portion of scala media
Inverted curve Low + high frequency sensori-neural deafness
Flat curve Uniform sensori-neural deafness
Down sloping curve Further SNHL in high frequency
Other Audiological Tests
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Speech Audiometry: Score = 50 - 80 %
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A.B.L.B.: Recruitment present
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S.I.S.I.: positive (> 70 % score)
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Tone Decay Test: negative (decay < 20 dB)
Laddergram in A.B.L.B.
Electro-cochleography
Electro-cochleography findings in Meniere’s disease
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Summation potential : compound action potential ratio > 30 %
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Widened SP-AP waveform (> 2msec)
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Distorted cochlear micro-phonics
SP – AP Waveform
Cochlear Microphonics SP/AP > 30 % Normal Distorted CM
Bithermal Caloric Test I/L canal paresis in 75 % cases
Bithermal Caloric Test C/L directional preponderance
Glycerol Test (confirmatory)
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Do P.T.A. & speech audiogram. Glycerol (1.5 ml / Kg), mixed in lime juice given orally. Repeat audio tests after 2 hrs. Test is positive if:
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Pure Tone threshold improves > 10 dB
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Speech Discrimination Score increases > 15 %
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S.P. / A.P. ratio in E.Co.G. decreases > 15 %
Other Investigations
Full blood count + ESR
Urea, electrolytes
RBS, FBS
Fasting lipid profile
Thyroid function test
VDRL, TPHA
Immunological assay, antibody screening
Treatment of Acute attack
Reassurance
Bed rest + head support
Inj. Prochlorperazine (Stemetil): 12.5 mg I.V., T.I.D. – Q.I.D.
Inj. Promethazine (Phenergan): 25 mg I.V., T.I.D. – Q.I.D.
Inj. Diazepam (Calmpose): 5 mg I.V. stat
Non-surgical treatment D iscussion: Reassurance. Avoid tea, coffee, colas, chocolate, allergens, stress, smoking, alcohol, flying, diving, heights. D iet: Low salt (1.5 g/day), less fluids. Exercise.
Vestibular D epressants: Cinnarizine, Diazepam, Prochlorperazine, Dimenhydrinate
Non-surgical treatment Cochlear Vaso D ilators: Betahistine, Xanthinol nicotinate, Carbogen (5 % CO 2 + 95 % O 2 ), L.M.W. Dextran, Histamine drip.
D iuretics: Thiazide + Triamterene D examethasone / Ig G: decreases auto-immunity D ehydration by hyperosmolar fluids Hormone replacement therapy
Meniett D evice Low pressure pulse generator. Pressure pulses transmitted to round window via grommet
displace endolymph
relieve endolymph hydrops.
Used for 5 min, TID.
Meniett D evice
Surgical treatment of Meniere’s disease
A. Hearing preservation + Balance preservation:
1. Endolymphatic sac decompression / shunting 2. Sacculotomy by puncture of footplate 3. Cochlear duct piercing via round window
B. Hearing preservation + Balance ablation:
1. Chemical labyrinthectomy 2 . Vestibular neurectomy 3. Vestibular end organ destruction by USG / cryoprobe
C. Hearing ablation + Balance ablation:
1. Section of 8th nerve 2. Total labyrinthectomy
D ecompression Surgery 1. Endolymphatic sac decompression (Portmann) 2. Endolymphatic sac shunting: into sub arachnoid space or mastoid cavity 3. Sacculotomy:
Fick’s needle puncture of footplate
Cody’s tack puncture of footplate 4. Cochlear duct piercing via round window
D ecompression Surgery
Endolymphatic sac decompression
Georges Portmann
Sac shunting into mastoid
Sac shunting into subarachnoid
Fick’s needle puncture of footplate
Chemical Labyrinthectomy
Trans-tympanic drug injection
Intra-tympanic drug instillation via grommet
Intra-tympanic drug instillation via Silverstein micro wick
Trans-tympanic drug perfusion Drug used: Gentamicin (vestibulo-toxic)
Trans-tympanic injection
Intra-tympanic drug instillation
Grommet in P.I.Q.
Trans-tympanic gentamicin
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26.7 mg/ml solution used
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0.75 ml solution instilled in affected ear (via grommet) 3 times daily for 4 consecutive days
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After instillation, pt to lie supine with affected ear up for 30 min & not swallow anything
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Vertigo control = 94%.
Hearing unchanged or improved = 74%. Hearing worsened = 26%.
Silverstein micro wick
Trans-tympanic drug perfusion
Trans-tympanic Dexamethasone Mechanism of action:
reducing inflammation
control of auto-immune injury Solution strength: 0.25 mg/ml Dose: 5 drops every alternate day for 3 months
Vestibular Surgery
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D enervation of vestibule by vestibular neurectomy via middle cranial fossa
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D estruction of vestibule (via round window or lateral semicircular canal) by:
Cryo-probe
Ultrasound probe
Vestibular Neurectomy
Vestibular Destruction
Ultrasound Probe
Total D estructive Surgery Destroys both cochlear & vestibular functions. Done in pt with severe deafness.
Types of surgery are:
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Section of vestibular + cochlear nerves
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Trans-mastoid total labyrinthectomy
Total D estructive Surgery
Total Labyrinthectomy
Vestibule + semi-circular canals exposed
Total Labyrinthectomy
Vestibule + ampullae opened to show neuro-epithelium
Total Labyrinthectomy
Neuro-epithelium destroyed
Treatment Ladder
Vertigo Control Level Score
Average vertigo spells per month post-treatment (24 mth) = ------------------------------------------------------------------------- X 100 Average vertigo spells per month pre-treatment (6 mth) Score 0 = Complete control = Level A Score 1 - 40 = Substantial control = Level B Score 41 - 80 = Limited control = Level C Score 81 - 120 = Insignificant control = Level D Score > 120 = Worse = Level E Severe vertigo requiring other treatment = Level F
Hearing level reporting
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Pure Tone Average taken for 0.5, 1, 2 & 3 KHz
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If multiple pre and post levels are available, worst is always used
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PTA is considered improved / worse if a 10 dB difference is noted
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Speech Discrimination Score is considered improved / worse if a 15% difference is noted
Prognosis
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60% have complete control of vertigo & 40% have good hearing, without any treatment
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Medical & surgical therapies show high levels of improvement with placebo
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Results vary greatly between different series
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Average result: Level A + B = 60 - 80% Level C = 20 - 30% Level D + E + F = 10 - 20%
Thank You