1. 上海市第六人民医院 Shanghai Sixth People’s Hospital Shankai Yin Prof Dept of Otolaryngology Affiliated Sixth Hospital Otolaryngology Institute Shanghai Jiao Tong university Otosclerosis

2. 上海市第六人民医院 Shanghai Sixth People’s Hospital History  1704 Valsalva autopsy  1894 Politzer “ otosclerosis ”  1930s Lempert horizontal canal fenestration  1953 Rosen stapes mobilization  1955 Shea stapedectomy

3. 上海市第六人民医院 Shanghai Sixth People’s Hospital Histopathology Increased osteoclastic activity results in perivascular bony resorption forming fibrotic spaces (lytic phase), osteoblasts within the fibrotic spaces producing immature bone (bone production phase), cycling of resorption and bone formation results in otosclerotic bone (remodeling phase)

4. 上海市第六人民医院 Shanghai Sixth People’s Hospital

5. 上海市第六人民医院 Shanghai Sixth People’s Hospital

6. 上海市第六人民医院 Shanghai Sixth People’s Hospital

7. 上海市第六人民医院 Shanghai Sixth People’s Hospital Biscuit footplate

8. 上海市第六人民医院 Shanghai Sixth People’s Hospital Cochlear involvement

9. 上海市第六人民医院 Shanghai Sixth People’s Hospital Epidemiology  Hereditary in 70% of cases  Autosomal dominant  Incomplete penetrance  7-10% Caucasian temporal bones  1% Caucasians with symptoms  Other races lesser incidence

10. 上海市第六人民医院 Shanghai Sixth People’s Hospital Symptoms  Slowly progressive hearing loss ipsilateral or bilateral  Tinnitus in 75%  Vestibular complaints 25%

11. 上海市第六人民医院 Shanghai Sixth People’s Hospital Diagnosis  History  Physical examination  Audiogram  Imaging  Surgical exploration

12. 上海市第六人民医院 Shanghai Sixth People’s Hospital Physical examination  Otomicroscopy normal Schwartze ’ s sign  Tuning forks

13. 上海市第六人民医院 Shanghai Sixth People’s Hospital Differential diagnosis  Tympanosclerosis  MEE  incus/malleus fixation  ossicular discontinuity  congenital footplate fixation

14. 上海市第六人民医院 Shanghai Sixth People’s Hospital Systemic diseases  Osteogenesis imperfecta stapes fixation blue sclera fractures  Pagets disease crowding in epitympanum elevated alk phos skeletal bone involvement

15. 上海市第六人民医院 Shanghai Sixth People’s Hospital Imaging  Multidirectional tomography  CT  MRI

16. 上海市第六人民医院 Shanghai Sixth People’s Hospital Audiology  Tympanometry  Acoustic Reflexes  Pure tone audiogram  Speech discrimination

17. 上海市第六人民医院 Shanghai Sixth People’s Hospital Tympanogram  Normal type A  Shallow type As

18. 上海市第六人民医院 Shanghai Sixth People’s Hospital Acoustic Reflexes  Very sensitive  “ On-Off ” phenomenon  precedes hearing loss

19. 上海市第六人民医院 Shanghai Sixth People’s Hospital Pure tone audio

20. 上海市第六人民医院 Shanghai Sixth People’s Hospital Management  Amplification  Medical therapy  Stapes surgery

21. 上海市第六人民医院 Shanghai Sixth People’s Hospital Amplification  Refuse surgery  Poor surgical candidate  following improvement of CHL

22. 上海市第六人民医院 Shanghai Sixth People’s Hospital Medical management  Sodium fluoride  Vitamin D  Calcium carbonate

23. 上海市第六人民医院 Shanghai Sixth People’s Hospital Stapes surgery  Total Stapedectomy  Partial Stapedectomy  Anterior crurotomy  Stapedotomy

24. 上海市第六人民医院 Shanghai Sixth People’s Hospital Goals  Open the oval window for sound transmission  reconstruct sound conducting mechanism  without complication

25. 上海市第六人民医院 Shanghai Sixth People’s Hospital Variations  Procedure  Stapedotomy drill laser (CO2, KTP, argon)  Prosthesis  Oval window seal