1. بسم الله الرحمن الرحيم

2. OTOSCLEROSIS

3. DEFINITION
A primary disease of the otic capsule characterized pathologically by abnormal resorption and deposition of bone

4. HISTOPATHOLOGY Resorption of bone by osteocytes
Formation of new vascular spongy bone
Formation of dense sclerotic bone

5. Fissula ante fenestram (80% to 90%)
AREAS OF PREDILECTION
Fissula ante fenestram (80% to 90%)

6. OTHER AREAS
Round window, the apex of the cochlea, the cochlear aqueduct, the semicircular canals, and the stapes footplate itself

7. COCHLEAR INVOLVEMENT

8. ETIOLOGY Unknown cause Positive family history in about 60%
Inherited by autosomal dominant transmission with incomplete penetration (60%)
Persistent measles virus infection
Detection of measles virus RNA in the affected bone
Detection of measles virus-specific antibodies in the perilymph

9. PHYSIOLOGY Conductive HL: due to fixation of the stapedial footplate
Mixed HL: due to
Liberation of toxic metabolites into the inner ear
Vascular compromise from sclerosis and narrowing of vascular channels
Direct extension of lesions into the inner ear
Cochlear otosclerosis

10. Involvement of footplate and cochlea

11. CLINICAL PRESENTATION
Hearing loss of gradual onset at years
Slowly progressive course
70% are bilateral
Accelerates with pregnancy (30-40%)
Tinnitus
Paracusis Willisii
Change of the speech pattern
Vestibular symptoms

12. PHYSICAL EXAMINATION Normal tympanic membrane
Schwartze sign (Flamingo flush)

13. PHYSICAL EXAMINATION Normal tympanic membrane
Schwartze sign (Flamingo flush)
Tuning fork tests

14. PURE TONE AUDIO

15. CARHART’S NOTCH Decrease in bone conduction thresholds
5 dB at 500 Hz
10 dB at 1000 Hz
15 dB at 2000 Hz
5 dB at 4000 Hz
Explanation is not known
Reverses following successful surgery

16. AUDIOMETRY
Pure tone audiogram
Speech discrimination

17. AUDIOMETRY Pure tone audiogram Speech discrimination
Impedence & tympanometry

18. CT SCAN
Double ring cochlea or Halo’s sign

19. COCHLEAR OTOSCLEROSIS
Isolated pure sensorineural hearing loss without a conductive component

20. CRITERIA FOR DIAGNOSIS OF COCHLEAR OTOSCLEROSIS
Progressive pure cochlear loss beginning at the usual age of onset for otosclerosis
Unilateral conductive hearing loss consistent with otosclerosis and bilateral symmetric SNHL
Positive Schwartze’s sign
Positive family history
Excellent discrimination
Stapedial reflex demonstrating the “on-off effect”
CT: demineralization of the cochlea

21. DIFFERENTIAL DIAGNOSIS
Congenital fixation of the stapes
Middle ear effusion
Chronic OM and ossicular discontinuity
Tympanosclerosis
Malleus head fixation
Systemic diseases

22. SYSTEMIC DISEASES Osteogenesis imperfecta Stapes fixation Blue sclera
Fractures

23. SYSTEMIC DISEASES Osteogenesis imperfecta Pagets disease
Stapes fixation
Blue sclera
Fractures
Pagets disease
Crowding in epitympanum
Elevated alkaline phosphatase
Skeletal bone involvement

24. TREATMENT
Observation
Hearing aid
Medical treatment
Surgical treatment

25. OBSERVATION

26. INDICATIONS OF OBSERVATION
Unilateral
Mild CHL
Young age

27. HEARING AID

28. INDICATIONS OF HEARING AID
Refuse surgery
Poor surgical candidate
Following improvement of CHL

29. MEDICAL TREATMENT

30. AIM OF MEDICAL TREATMENT
Stabilize the disease by reduction of the osteoclastic bone resorption and increase osteoblastic bone formation

31. MEDICAL MANAGEMENT
Sodium fluoride: mg /day/2years followed by 25 mg for life
Vitamin D
Calcium carbonate

32. INDICATIONS Cochlear otosclerosis
Patients with confirmed otosclerosis but having progressive SNHL disproportionate to age

33. CONTRAINDICATIONS Chronic nephritis Rheumatoid arthritis
Pregnancy and lactation
Children

34. SURGICAL TREATMENT

35. PATIENT SELECTION FOR SURGICAL TREATMENT
Socially unacceptable conductive or mixed hearing loss
Good speech discrimination
Age
Lifestyle and occupation

36. ABSOLUTE CONTRAINDICATION OF SURGERY
The better or the only functioning ear

37. OTHER CONTRAINDICATIONS
? Patients experience frequent changes in barometric pressure
“Malignant” otosclerosis
Endolymphatic hydrops
TM perforation
Infections

38. STAPES SURGERY STAMP (STApedotomy Minus Prosthesis) or Stapedioplasty
Stapedectomy
Stapedotomy

39. STAPEDECTOMY Results probably are the best
More traumatic to the inner ear
Increased post-op vestibular symptoms
Higher incidence of postoperative SNHL
The operation is unavoidable in:
Comminuted fracture of the footplate
Revision surgery

40. STAPEDOTOMY
Equal or better results with less vestibulocochlear side effects

41. COMPARISON

42. STAMP Preservation of the stapedius tendon No prosthesis complications
Reduction in hyperacusis
Reduction in risk for long-term postoperative inner ear injuries
No prosthesis complications
Very difficult technique

43. SURGICAL PROCEDURE

44. The Incision
Permeatal (Transcanal)
Endaural

50. STAPEDOTOMY

51. LASER STAPEDOTMY

52. STAMP

53. OPERATIVE PROBLEMS & COMPLICATIONS

54. TM PERFORATION
Proceed and then repair

55. CHORDA TYMPANI INJURY 30% of cases Metallic taste
Symptoms usually resolves in 3-4 months
More symptoms if bilateral

56. OBTRUSIVE FACIAL NERVE
0.5 %
Stapedotomy is usually possible

58. BLEEDING
Mucosal trauma
Active phase
Persistent stapedial artery

59. Persistent stapedial artery

62. ROUND WINDOW OTOSCLEROSIS
About 1% complete (Shuknecht)
If complete:
Abandon surgery
If incomplete or not sure:
Do not remove bone and proceed

63. OBLITERATIVE OTOSCLEROSIS OF THE OVAL WINDOW
A total stapedectomy is contraindicated because of high risk of surgically induced SNHL

64. INCUS PROBLEMS Subluxation: Proceed Dislocation:
Remove incus & use a malleus-grip prosthesis

65. FLOATING FOOTPLATE
May be avoided if control holes are used or by using laser fenestration

66. FLOATING FOOTPLATE
May be extracted by needles/hooks with hole inferior to the oval window

67. FLOATING FOOTPLATE
In many cases should be left and surgery is completed with unpredictable results or use laser fenestration

68. MALLEUS ANKYLOSIS About 0.5% May be congenital or acquired
Causes about dB CHL
Remove malleus head and the incus and use malleus grip prosthesis

69. CSF GUSHER Introduce spinal catheter and proceed Or
Due to fundal defect of IAM or widened cochlear aqueduct
Introduce spinal catheter and proceed Or
Pack with fascia and gauze for 4-5 days with delayed reconstruction that avoid reopening the fenestra

70. PERILYMPH FISTULA
Primary or secondary

71. PREVENTION OF PERILYMPH FISTULA
Stapedectomy < stapedotomy
Oval window seal
No fat or gel-foam for seal
Prohibit nose blowing, flying, diving, & lifting heavy objects postoperatively

72. DIAGNOSIS OF PERILYMPH FISTULA
Drop or fluctuation in hearing
Vertigo & tinnitus
Audiometry
ENG
Fistula test
Radiology

75. TREATMENT
Surgical closure

76. REPARATIVE GRANULOMA
Granuloma formation around the prosthesis and incus
1-5%
Gradual deterioration days postoperativly
Vertigo, tinnitus and deafness
Otoscopy: reddish discoloration of the posterior TM

77. REPARATIVE GRANULOMA
Treatment is by emergency tympanotomy and excision

78. SNHL
0.2-10%
Serous labyrinthitis - high frequencies
Surgical trauma

79. PERSISTENCE OR RECURRENCE OF CHL
Prosthesis malfunction
Fibrous adhesion
Incus erosion

83. PERSISTENCE OR RECURRENCE OF CHL
Prosthesis malfunction
Fibrous adhesion
Incus erosion
Missed pathology: e.g. malleus fixation, round window otosclerosis
Otosclerosis regrowth

84. RARE COMPLICATIONS
Facial paralysis
Acute otitis media
Cholesteatoma

85. THANK YOU