Transcript Document

Meniere’s
Disease
Dr. Vishal Sharma
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
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
• 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
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
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
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
• 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
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
• 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
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
• 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)
Laddergram in A.B.L.B.
Electro-cochleography
Electro-cochleography findings
in Meniere’s disease
• Summation potential : compound action
potential ratio > 30 %
• Widened SP-AP waveform (> 2msec)
• 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)
• 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 %
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
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
Non-surgical treatment
Cochlear VasoDilators: Betahistine, Xanthinol
nicotinate, Carbogen (5 % CO2 + 95 % O2),
L.M.W. Dextran, Histamine drip.
Diuretics: Thiazide + Triamterene
Dexamethasone / Ig G: decreases auto-immunity
Dehydration by hyperosmolar fluids
Hormone replacement therapy
Meniett Device
Low pressure pulse
generator. Pressure
pulses transmitted to
round window via
grommet  displace
endolymph  relieve
endolymph hydrops.
Used for 5 min, TID.
Meniett Device
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
Decompression Surgery
1. Endolymphatic sac decompression (Portmann)
2. Endolymphatic sac shunting: into subarachnoid 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
Decompression 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
• 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%.
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
• 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
Vestibular Neurectomy
Vestibular Destruction
Ultrasound Probe
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
Total Destructive 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
• 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
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%
Thank You