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Otosclerosis

Otosclerosis. Dr. Vishal Sharma. Definition. Hereditary disease of bony labyrinth showing replacement of lamellar enchondral bone by irregularly laid new bone . New bone is spongy + more vascular in active Otospongiosis but thicker & more cellular in inactive Otosclerosis.

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Otosclerosis

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  1. Otosclerosis Dr. Vishal Sharma

  2. Definition • Hereditary disease of bony labyrinth showing replacement of lamellar enchondral bone by irregularly laid new bone. • New bone is spongy + more vascular in active Otospongiosis but thicker & more cellular in inactive Otosclerosis.

  3. Antonio Valsalva First described ankylosis of stapes in 1741

  4. Adam Politzer Coined the term Otosclerosis in 1893

  5. Friedrich Siebenmann Coined the term Otospongiosis in 1912

  6. Epidemiology • Exact etiology is unknown (? Viral) • Autosomal dominant:variable penetrance • Race: common in white races & Indians • Female : Male = 2 : 1 • Age: Common in 20 - 40 years • Hormonal influence:es in pregnancy, menopause, stress (trauma, surgery)

  7. Van der Hoeve syndrome Otosclerosis + osteogenesis imperfecta + blue sclera

  8. Types of Otosclerosis A. Stapedial B. Cochlear: otosclerotic focus is seen over   Round window  Promontory C. Stapedial + cochlear:mixed type D. Malignant: rapidly progressing cochlear lesion with severe sensori-neural deafness.

  9. Types of Stapedial Otosclerosis 1. Anterior focus (commonest): 2 mm anterior to oval window. 2. Posterior focus: 2 mm behind oval window. 3. Circumferential: involves footplate margin only.

  10. Types of Stapedial Otosclerosis 4. Biscuit type: footplate involved, margin is free. 5. Obliterative: obliterates oval window completely.

  11. Symptoms of Otosclerosis 1. Deafness: Bilateral, slowly progressive  Conductive: stapedial otosclerosis  Sensori-neural: cochlear otosclerosis  Mixed: stapedial + cochlear otosclerosis 2. Soft, modulated, monotonous voice 3. Tinnitus & vertigo: in cochlear lesion

  12. Symptoms of Otosclerosis 4. Paracusis Willisii: Pt has better hearing in noisy surroundings (people increase their voice intensity & pt’s speech discrimination becomes better).

  13. Thomas Willis (1621-1675)

  14. Otoscopy Normal T.M. is seen in most cases. Pinkish colour over promontory seen in otospongiosis (2 - 10 % cases)  Schwartze sign / Flamingo pink blush.

  15. Tuning Fork Tests

  16. Gelle & Bing Tests Vibrating tuning fork placed over mastoid & then: External auditory canal is blocked in Bing test or E.A.C. pressure ed by Siegalization in Gelle test

  17. Pure Tone Audiometry • Low frequency conductive deafness • Carhart’s notch in bone conduction at 2 KHz

  18. Carhart’s notch • Proposed theories 1. Stapes fixation disrupts normal ossicular resonance (2000 Hz) 2. Normal compression mode of bone conduction is disturbed because of relative perilymph immobility 3. Mechanical artefact • Carhart’s notch reverses with stapes surgery

  19. Speech Audiometry Speech Discrimination Score (maximum score achieved) is almost 100 %. Speech Reception Threshold(intensity at which 50 % words are heard) is increased by the amount of conductive hearing loss.

  20. Speech Audiometry

  21. Impedance Audiometry • As curve seen in 40 % cases of otosclerosis. • Normal middle ear pressure + decreasedmiddle ear compliance. • Others have A curve.

  22. Stapedial reflex present

  23. Stapedial reflex absent

  24. C.T. scan temporal bone 200 coronal oblique cuts are taken

  25. Stapedial otosclerosis (coronal)

  26. Cochlear otospongiosis (axial)

  27. Differential Diagnosis • Otitis Media with Effusion: type B tympanogram • Adhesive Otitis Media: absence of T.M. mobility • Tympanosclerosis: white patch on T.M. • Ossicular discontinuity: type Ad tympanogram • Congenital ossicular chain fixation:tympanotomy • Malleus head fixation: tympanotomy

  28. History of development of stapes surgery

  29. Stapes mobilization: Kessel (1880), Rosen (1952) Lateral semicircular canalfenestration: Holmgren (1923), Sourdille (1932), Lempert (1938) Complete Stapedectomy: Jack (1893), Shea (1956) Partial Stapedectomy (posterior 1/3):Plester (1960) Stapedotomy: Shea (1962), Marquet (1965) Laser Stapedotomy:Perkins & Di Bartolomeo (1980)

  30. Johannes Kessel

  31. Samuel Rosen

  32. Gunnar Holmgren

  33. Maurice Sourdille

  34. Julius Lempert

  35. John J. Shea Jr.

  36. Inclusion criteria for surgery • Pure Tone Average between 30 - 60 dB • Air bone gap > 15 dB • Speech discrimination score > 60 % • Absence of sensorineural deafness

  37. Contraindications for surgery  Only hearing ear  Meniere’s disease  Otitis media  Otitis externa  Extremes of age  Pregnancy  Professions: divers, high construction workers, frequent travelers, noisy surroundings

  38. Surgical steps for Stapedotomy

  39. Right T.M. (upright)

  40. Right T.M. (supine)

  41. Per-meatal Incision

  42. Tympanomeatal flap raised

  43. Bony overhang curetted

  44. Bony overhang curetted

  45. Chorda tympani preserved

  46. Chorda tympani separated

  47. Confirm footplate fixation Checking for absence of round window reflex

  48. Depth measurement prosthesis

  49. Incudo-stapedial joint broken

  50. Footplate perforation made

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