1. Otosclerosis Department of Otorhinolaryngoglogy
the 2nd Hospital affliatted to Medical college
Zhejiang University
Xu Yaping

2. Introduction Otosclerosis
1. Primary metabolic bone disease of the otic capsule and ossicles
2. Results in fixation of the ossicles and conductive hearing loss
3. May have sensorineural component if the cochlea is involved
􀂄Genetically mediated via autosomal dominant transmission with incomplete penetrance (40%) and variable expressivity.

3. History of Otosclerosis and Stapes surgery
􀂄 1704 – Valsalva first described stapes fixation
􀂄 1857 – Toynbee linked stapes fixation to hearing loss
􀂄 1890 – Katz was first to find microscopic evidence of
otosclerosis
􀂄 1893 – Politzer described the clinical entity of
“otosclerosis”
􀂄 1890 – Bacon describes medical therapy for the
condition, and supports the common view that
“surgery should not be considered for a moment.“

4. 10% overall prevalence of histologic otosclerosis
Epidemiology
10% overall prevalence of histologic otosclerosis
1% overall prevalence of clinically significant otosclerosis
Clinical otosclerosis ––2:1 (W:M)
Possible progression during pregnancy (10%-17%)
Bilaterality more common (89% vs. 65%)
15-45 most common age range of presentation, increases with age

5. Pathophysiology Osseous dyscrasia Resorption and formation of new bone
Limited to the temporal bone and ossicles
Inciting event unknown,
many theories:
Hereditary, endocrine, metabolic, infectious, vascular, autoimmune,hormonal .

6. Most common sites of involvement
􀂄Fissula ante fenestrum
􀂄Round window niche (30%-50% of cases)
􀂄Anterior wall of the IAC

7. Histology otosclerosis has two main forms:
an early of spongiotic phase (otospongiosis)
multiple active cell groups including
osteocytes, osteoblasts, and histiocytes.
2. a late or sclerotic phase: dense sclerotic bone forms

8. Non-clinical foci of otosclerosis

10. Bipolar involvement of the footplate

11. Round Window

12. Diagnosis of Otosclerosis
􀂄1. Most common presentation
􀂄Women age
􀂄2. Conductive or Mixed hearing loss
slowly progressive,bilateral (80%),asymmetric
􀂄Tinnitus (75%)

13. a complete history:
Age of onset of hearing loss
Progression
Laterality
Associated symptoms
Dizziness
Otalgia
Otorrhea
Tinnitus

14. 􀂄2/3 have a significant family history
􀂄Particularly helpful in patients with severe or
profound mixed hearing loss
Prior otologic surgery
History of ear infections
Vestibular symptoms
􀂄25%
􀂄Most commonly dysequilibrium
􀂄Occasionally attacks of vertigo with rotatory nystagmus

15. Physical Exam Otomicroscopy Most helpful in ruling out other disorders
􀂄Middle ear effusions
􀂄Tympanosclerosis
􀂄Tympanic membrane perforations
􀂄Cholesteatoma or retraction pockets
􀂄Superior semicircular canal dehiscence
Schwartze’’s signs ( by Schwartze in 1873)
􀂄Red hue behind the tympanic membrane (in oval window niche area)
􀂄10% of cases
Pneumatic otoscopy
􀂄Distinguish from malleus fixation

16. 􀂄1. Hearing loss progresses form low frequencies to high
Tuning forks
􀂄1. Hearing loss progresses form low frequencies to high
frequencies
􀂄 , 512, and 1024 Hz TF should be used
3. Rinne
􀂄256 Hz ––negative test indicates at least a 20 dB ABG
􀂄512 Hz ––negative test indicates at least a 25 dB ABG
(air-bone gaps)

17. Differential Diagnosis
Ossicular discontinuity:A.conductive loss of 60 db
B. type Ad tympanogram
2.Congenital stapes fixation:A.25% incidence of other
congenital anomalies
B. non-progressive CHL
3.Malleus head fixation: when congenital, associated with other stigmata (aural atresia).
4.Paget’’s disease: diffuse involvement of the bony skeleton
5.Osteogenesis imperfecta: presence of blue sclera
6.Superior semicircular canal dehiscence: vertigo or eye movements with loud noise

18. Audiometry 􀂄Tympanometry 􀂄Impedance testing Acoustic reflexes
􀂄Pure tones

19. As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

20. Acoustic Reflexes
Otosclerosis has a predictable pattern of abnormal reflexes over time
Reduced reflex amplitude
Elevation of ipsilateral thresholds
Elevation of contralateral thresholds
Absence of reflexes

21. Pure Tone Audiometry Most useful audiometric test for otosclerosis
􀂄Characterizes the severity of disease
􀂄Frequency specific
Carhart’’s notch
􀂄Hallmark audiologic sign of otosclerosis
􀂄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

22. early stage
middle stage

23. late stage

24. Imaging Computed tomography (CT) of the temporal bone
􀂄Proponents of CT for evaluation of otosclerosis
Pre-op
Characterize the extent of otosclerosis
Severe or profound mixed hearing loss
Evaluate for enlarge cochlear aqueduct
Post-op
Recurrent CHL
Re-obliteration vs. prosthesis dislocation
Vertigo

26. Management options
Medical: Sodium Fluoride,Bisphosphonates,Vitamin D and Calcium
Amplification:Non-surgical candidates-wearing hearing aids.
Surgery: Stapedectomy vs. Stapedotomy
Combinations

27. Surgery Best surgical candidate Previously un-operated ear Good health
Unacceptable ABG
25 to 40 dB
Negative Rinne test
Excellent discrimination
Desire for surgery

28. Tympanosclerosis Definition: a whitish "plaque" of the TM.
Pathology: submucosal hyaline degeneration in the TM and middle ear mucosa.
extensive involvement of the TM and ossicle amy result in conductive hearing loss.(air-bone gap >40dB)
medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease.

29. The end, thank you!