Stapes surgery in osteogenesis imperfecta: retrospective analysis of 18 operated ears

Purpose To evaluate short and long term results of stapes surgery in patients with osteogenesis imperfecta (OI), Methods Retrospective case series of 18 primary stapes surgeries performed on 11 hearing-impaired OI patients with evidence of stapes fixation, in a Tertiary referral center. We analysed pre-operative and post-operative hearing results at 1 month and at least 1 year Results The main operative findings were stapes fixation, thickened footplate and fragile or fractured stapes crura. No revision surgery was necessary. Hearing improvement was achieved in 94% of the cases. We obtained an air-bone gap closure to within 10 dB in 46% of the cases and to within 15 dB in 92% of the cases at 1-year follow-up. The mean hearing gain in air conduction (at 0.5, 1, 2 and 4 kHz) was 18.4 dB at 1 month and 22.4 dB at 1 year. Conclusion Stapes surgery in OI gives good results with few complications in our series. A hearing gain is often obtained in spite of the sensorineural hearing loss caused by the natural progression of the disease