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Meniere’s Disease

Meniere’s Disease. Dr. Vishal Sharma. Introduction. Described by Prosper Meniere in 1861 Vertigo + Deafness + Tinnitus + Aural fullness

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Meniere’s Disease

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  1. Meniere’s Disease Dr. Vishal Sharma

  2. Introduction • Described by Prosper Meniere in 1861 • Vertigo + Deafness + Tinnitus + Aural fullness • Etiology:endolymphatic hydrops(Hallpike, 1938) due to edabsorption of endolymphor ed production of endolymph • Especially involves cochlear duct & saccule

  3. Prosper Meniere`

  4. Normal membranous labyrinth

  5. Endolymphatic Hydrops

  6. Normal membranous labyrinth

  7. Endolymphatic Hydrops

  8. Pathogenesis

  9. 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

  10. Etiology of Primary Meniere’s disease

  11. A. Idiopathic B. Increased production of endolymph: Allergy Sodium & water retention Autoimmune Viral infection  sympathetic activity ischemia of stria vascularis  fluid transudation

  12. 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

  13. Secondary Meniere Syndrome Clinically resembles Meniere’s disease. Seen in: • Syphilis • Otosclerosis, • Cogan syndrome (interstitial keratitis) • Post-stapedectomy • Paget’s disease

  14. 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

  15. 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

  16. 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

  17. 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

  18. Meniere’s disease variants

  19. 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

  20. 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

  21. 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

  22. Pure Tone Audiometry

  23. Rising curve in early stage Low frequency SNHL due to more fluid accumulation in apical portion of scala media

  24. Inverted curve Low + high frequency sensori-neural deafness

  25. Flat curve Uniform sensori-neural deafness

  26. Down sloping curve Further SNHL in high frequency

  27. 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)

  28. Laddergram in A.B.L.B.

  29. Electro-cochleography

  30. Electro-cochleography findings in Meniere’s disease • Summation potential : compound action potential ratio > 30 % • Widened SP-AP waveform (> 2msec) • Distorted cochlear micro-phonics

  31. SP – AP Waveform

  32. Cochlear Microphonics SP/AP > 30 % Normal Distorted CM

  33. Bithermal Caloric Test I/L canal paresis in 75 % cases

  34. Bithermal Caloric Test C/L directional preponderance

  35. 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 %

  36. Other Investigations • Full blood count + ESR • Urea, electrolytes • RBS, FBS • Fasting lipid profile • Thyroid function test • VDRL, TPHA • Immunological assay, antibody screening

  37. 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

  38. 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

  39. 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

  40. Meniett Device Low pressure pulse generator. Pressure pulses transmitted to round window via grommet displace endolymph  relieve endolymph hydrops. Used for 5 min, TID.

  41. Meniett Device

  42. Surgical treatment of Meniere’s disease

  43. 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 labyrinthectomy2. Vestibular neurectomy 3. Vestibular end organ destruction by USG / cryoprobe C. Hearing ablation + Balance ablation: 1. Section of 8th nerve 2. Total labyrinthectomy

  44. 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

  45. Decompression Surgery

  46. Endolymphatic sac decompression

  47. Georges Portmann

  48. Sac shunting into mastoid

  49. Sac shunting into subarachnoid

  50. Fick’s needle puncture of footplate

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