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MENIERE'S DISEASE

MENIERE'S DISEASE
DHINGRA ENT BOOK

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MENIERE'S DISEASE

  1. 1. Meniere’s Disease Dr. Vishal Sharma
  2. 2. 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
  3. 3. Prosper Meniere`
  4. 4. Normal membranous labyrinth
  5. 5. Endolymphatic Hydrops
  6. 6. Normal membranous labyrinth
  7. 7. Endolymphatic Hydrops
  8. 8. Pathogenesis
  9. 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. 10. Etiology of Primary Meniere’s disease
  11. 11. A. Idiopathic B. Increased production of endolymph:  Allergy  Sodium & water retention  Autoimmune  Viral infection   sympathetic activity  ischemia of stria vascularis  fluid transudation
  12. 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. 13. Secondary Meniere Syndrome Clinically resembles Meniere’s disease. Seen in:  Syphilis  Otosclerosis,  Cogan syndrome (interstitial keratitis)  Post-stapedectomy  Paget’s disease
  14. 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. 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. 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. 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. 18. Meniere’s disease variants
  19. 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. 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. 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. 22. Pure Tone Audiometry
  23. 23. Rising curve in early stage Low frequency SNHL due to more fluid accumulation in apical portion of scala media
  24. 24. Inverted curve Low + high frequency sensori-neural deafness
  25. 25. Flat curve Uniform sensori-neural deafness
  26. 26. Down sloping curve Further SNHL in high frequency
  27. 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. 28. Laddergram in A.B.L.B.
  29. 29. Electro-cochleography
  30. 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. 31. SP – AP Waveform
  32. 32. Cochlear Microphonics Normal SP/AP > 30 % Distorted CM
  33. 33. Bithermal Caloric Test I/L canal paresis in 75 % cases
  34. 34. Bithermal Caloric Test C/L directional preponderance
  35. 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. 36. Other Investigations  Full blood count + ESR  Urea, electrolytes  RBS, FBS  Fasting lipid profile  Thyroid function test  VDRL, TPHA  Immunological assay, antibody screening
  37. 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. 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. 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. 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. 41. Meniett Device
  42. 42. Surgical treatment of Meniere’s disease
  43. 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 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
  44. 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. 45. Decompression Surgery
  46. 46. Endolymphatic sac decompression
  47. 47. Georges Portmann
  48. 48. Sac shunting into mastoid
  49. 49. Sac shunting into subarachnoid
  50. 50. Fick’s needle puncture of footplate
  51. 51. 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)
  52. 52. Trans-tympanic injection
  53. 53. Intra-tympanic drug instillation
  54. 54. Grommet in P.I.Q.
  55. 55. 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%.
  56. 56. Silverstein micro wick
  57. 57. Trans-tympanic drug perfusion
  58. 58. 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
  59. 59. 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
  60. 60. Vestibular Neurectomy
  61. 61. Vestibular Destruction
  62. 62. Ultrasound Probe
  63. 63. 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
  64. 64. Total Destructive Surgery
  65. 65. Total Labyrinthectomy Vestibule + semi-circular canals exposed
  66. 66. Total Labyrinthectomy Vestibule + ampullae opened to show neuro-epithelium
  67. 67. Total Labyrinthectomy Neuro-epithelium destroyed
  68. 68. Treatment Ladder
  69. 69. 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
  70. 70. 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
  71. 71. 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%
  72. 72. Thank You

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