Stapes surgery in osteogenesis imperfecta: a clinical study of 16 patients.

Osteogenesis Imperfecta (OI), is a genetic disease of connective tissue, with the main feature of bone fractures, accompanied by blue sclerae and hearing loss. Hearing loss affects about 50% of the patients, beginning in the second and third decade of life. The hearing loss is progressive, starting with a conductive loss, then a mixed and later on a sensorineural loss. There are similarities between otosclerosis, although OI is a distinct entity. The diseases are treated in the same surgical way. The result is, however, not as good in OI as in otosclerosis according to different reports. A screening study of 15 patients with OI at the Karolinska Hospital, Sweden, has been performed, differing in age from 25-60. Audiometric examinations have been measured of both air and bone conduction. About 50% of the included patients had hearing loss. Three of the patients wore hearing aids. Of these 15 patients, only 4 had been operated on with stapes surgery. One of these patients needs a new operation. An indication for stapes surgery was found in about 50% of the investigated OI patients.