Long term outcomes of endolymphatic sac shunting with local steroids for Meniere´s disease
Meniere’s Disease Dr. Vishal Sharma. Introduction Described by Prosper Meniere in 1861 Vertigo +...
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Transcript of Meniere’s Disease Dr. Vishal Sharma. Introduction Described by Prosper Meniere in 1861 Vertigo +...
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Meniere’s Disease
Dr. Vishal Sharma
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Introduction
• Described by Prosper Meniere in 1861
• Vertigo + Deafness + Tinnitus + Aural fullness
• Etiology: endolymphatic hydrops (Hallpike, 1938)
due to ed absorption of endolymph or
ed production of endolymph
• Especially involves cochlear duct & saccule
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Prosper Meniere`
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Normal membranous labyrinth
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Endolymphatic Hydrops
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Normal membranous labyrinth
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Endolymphatic Hydrops
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Pathogenesis
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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
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Etiology of Primary Meniere’s
disease
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A. Idiopathic
B. Increased production of endolymph:
Allergy
Sodium & water retention
Autoimmune
Viral infection
sympathetic activity ischemia of stria
vascularis fluid transudation
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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
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Secondary Meniere Syndrome
Clinically resembles Meniere’s disease. Seen in:
· Syphilis
· Otosclerosis,
· Cogan syndrome (interstitial keratitis)
· Post-stapedectomy
· Paget’s disease
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Clinical Features
• 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
• Vertigo caused by loud, low frequency sound
Tulio phenomenon
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Clinical Features
2. Deafness:
Accompanies vertigo, improves after vertigo
attack, sensori-neural, fluctuant, progressive
• Intolerance to loud sound (due to recruitment)
• Distortion of sound frequency, called diplacusis
binauralis dysharmonica
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Clinical Features
3. Tinnitus:
Low-pitch, roaring, non-pulsatile,
continuous / intermittent. Increased during
vertigo attacks
4. Aural fullness:
Fluctuating, not relieved by swallowing
5. Emotional upset, anxiety, agoraphobia
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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
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Meniere’s disease variants
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• Lermoyez’s reverse Meniere syndrome:
Deafness vertigo improvement in hearing
• Tumarkin’s sudden drop attack:
Pt falls without vertigo / loss of consciousness
• Meyerhoff’s oculo-vestibular response:
Vertigo due to opto-kinetic stimulus
• Cochlear hydrops: deafness & tinnitus only
• Vestibular hydrops: vertigo only
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E.N.T. Examination
• Otoscopy: normal tympanic membrane
• Nystagmus: irritative paralytic recovery
• False +ve fistula sign (Hennebert sign): in 30% pt
• Rinne test: positive (A.C. > B.C.)
• Weber test: lateralizes towards better ear
• 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
• Paralytic nystagmus: occurs minutes into an
attack, toward healthy ear, due to blockade of
action potential by increased K+ in perilymph
• Recovery nystagmus: occurs hours later, toward
diseased ear, due to vestibular adaptation
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Pure Tone Audiometry
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Rising curve in early stageLow frequency SNHL due to more fluid accumulation
in apical portion of scala media
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Inverted curve
Low + high frequency sensori-neural deafness
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Flat curveUniform sensori-neural deafness
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Down sloping curve
Further SNHL in high frequency
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Other Audiological Tests
• Speech Audiometry: Score = 50 - 80 %
• A.B.L.B.: Recruitment present
• S.I.S.I.: positive (> 70 % score)
• Tone Decay Test: negative (decay < 20 dB)
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Laddergram in A.B.L.B.
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Electro-cochleography
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Electro-cochleography findings in Meniere’s disease
• Summation potential : compound action
potential ratio > 30 %
• Widened SP-AP waveform (> 2msec)
• Distorted cochlear micro-phonics
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SP – AP Waveform
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Cochlear Microphonics
Normal
SP/AP > 30 %
Distorted CM
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Bithermal Caloric TestI/L canal paresis in 75 % cases
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Bithermal Caloric TestC/L directional preponderance
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Glycerol Test (confirmatory)
• 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:
• Pure Tone threshold improves > 10 dB
• Speech Discrimination Score increases > 15 %
• S.P. / A.P. ratio in E.Co.G. decreases > 15 %
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Other Investigations Full blood count + ESR
Urea, electrolytes
RBS, FBS
Fasting lipid profile
Thyroid function test
VDRL, TPHA
Immunological assay, antibody screening
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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
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Non-surgical treatment
Discussion: Reassurance. Avoid tea, coffee,
colas, chocolate, allergens, stress, smoking,
alcohol, flying, diving, heights.
Diet: Low salt (1.5 g/day), less fluids. Exercise.
Vestibular Depressants: Cinnarizine, Diazepam,
Prochlorperazine, Dimenhydrinate
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Non-surgical treatment
Cochlear VasoDilators: Betahistine, Xanthinol
nicotinate, Carbogen (5 % CO2 + 95 % O
2), L.M.W.
Dextran, Histamine drip.
Diuretics: Thiazide + Triamterene
Dexamethasone / Ig G: decreases auto-immunity
Dehydration by hyperosmolar fluids
Hormone replacement therapy
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Meniett Device
Low pressure pulse
generator. Pressure
pulses transmitted to
round window via
grommet displace
endolymph relieve
endolymph hydrops.
Used for 5 min, TID.
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Meniett Device
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Surgical treatment of Meniere’s disease
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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
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Decompression 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
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Decompression Surgery
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Endolymphatic sac decompression
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Georges Portmann
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Sac shunting into mastoid
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Sac shunting into subarachnoid
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Fick’s needle puncture of footplate
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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)
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Trans-tympanic injection
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Intra-tympanic drug instillation
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Grommet in P.I.Q.
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Trans-tympanic gentamicin
• 26.7 mg/ml solution used
• 0.75 ml solution instilled in affected ear (via
grommet) 3 times daily for 4 consecutive days
• After instillation, pt to lie supine with affected ear
up for 30 min & not swallow anything
• Vertigo control = 94%. Hearing unchanged or
improved = 74%. Hearing worsened = 26%.
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Silverstein micro wick
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Trans-tympanic drug perfusion
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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
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Vestibular Surgery
• Denervation of vestibule by vestibular
neurectomy via middle cranial fossa
• Destruction of vestibule (via round window or
lateral semicircular canal) by:
Cryo-probe
Ultrasound probe
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Vestibular Neurectomy
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Vestibular Destruction
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Ultrasound Probe
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Total Destructive Surgery
Destroys both cochlear & vestibular functions.
Done in pt with severe deafness.
Types of surgery are:
• Section of vestibular + cochlear nerves
• Trans-mastoid total labyrinthectomy
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Total Destructive Surgery
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Total Labyrinthectomy
Vestibule + semi-circular canals exposed
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Total Labyrinthectomy
Vestibule + ampullae opened to show neuro-epithelium
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Total Labyrinthectomy
Neuro-epithelium destroyed
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Treatment Ladder
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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
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Hearing level reporting
• Pure Tone Average taken for 0.5, 1, 2 & 3 KHz
• If multiple pre and post levels are available,
worst is always used
• PTA is considered improved / worse if a 10 dB
difference is noted
• Speech Discrimination Score is considered
improved / worse if a 15% difference is noted
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Prognosis• 60% have complete control of vertigo & 40%
have good hearing, without any treatment
• Medical & surgical therapies show high levels of
improvement with placebo
• Results vary greatly between different series
• Average result: Level A + B = 60 - 80%
Level C = 20 - 30%
Level D + E + F = 10 - 20%
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Thank You