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. Coined the term Otosclerosis
Adam Politzer
Coined the term Otosclerosis
in 1893

5. Coined the term Otospongiosis
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 years
Hormonal influence: es in pregnancy, menopause, stress (trauma, surgery)

7. Otosclerosis + osteogenesis imperfecta + blue sclera
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 ( )

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

15. Tuning Fork Tests Rinne Weber A.B.C. Stapedial Negative (BC > AC)
Lateralizes to
Deaf ear
Normal
Cochlear
Positive (AC > BC)
Better ear
Decreased
Mixed

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
Bing
Gelle
Otosclerosis
No change
Normal / SNHL
Intensity es
Intensity es

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 + decreased middle ear compliance.
Others have A curve.

22. Stapedial reflex present

23. Stapedial reflex absent

24. 200 coronal oblique cuts are taken
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 canal fenestration: 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 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

51. Fenestration with burr

52. Posterior crus fractured

53. 1 cm
Teflon piston
Length of piston = medial surface of incus to stapes footplate mm
Range = 3.75 – 4.25 mm

54. Piston placed in perforation

55. Stapedius tendon cut

56. Stapedius tendon cut

57. Piston crimped around incus

58. Anterior crus fractured

59. Stapes superstructure removed

60. Footplate perforation sealed

61. Tympanomeatal flap put back

62. Laser Stapedotomy

63. Stapedius vaporization

64. Footplate fenestration

65. Rossette formation

66. Stapedotomy Piston

67. Stapedectomy

68. Footplate Fenestration

69. Stapes superstructure removed

70. Footplate removal

71. Prosthesis placed over vein graft

72. Complications of stapes surgery

73. Intra-operative Post-operative  Floating footplate  Otitis media  Submerged footplate  Oval window granuloma  Dislocated incus  Perilymph fistula  Perforated TM  Sensori-neural HL  Damage to facial nerve  Persistent air-bone gap or chorda tympani  Vestibular dysfunction  Persistent stapedial artery  Delayed facial palsy in  Perilymph flooding laser surgery

74. Sodium Fluoride
Given in cochlear otosclerosis & active focus of otospongiosis (Schwartze sign).
Acts by:
a. Decreases bone resorption
b. Increases bone formation
c. Prevents enzymatic damage to cochlea
20 mg orally, thrice daily for months

75. Hearing Aid
For patients who:
Are unfit for surgery
Refuse surgery

76. Thank You