[Results of stapedectomy in osteogenesis imperfecta]

Osteogenesis imperfecta is a connective tissue disease connected with improper synthesis of collagen type I. Bone fragility, blue sclera and hearing loss are the main symptoms of classic type of the disease. Early (6 months) and late (10 years) results of stapedectomy in 3 patients with osteogenesis imperfecta syndrome are presented. Six months postoperatively air-bone gap was smaller than 10 dB in 2 patients and 17 dB in one patient. In all patients, bone conduction pure tone threshold improved after the operation. Audiometric tests performed 10 years after stapedectomy were similar to those obtained 6 months after the operation. Stapedectomy in patients with osteogenesis imperfecta syndrome provides good and stable hearing results.