Stapedectomy in osteogenesis imperfecta.

Operative findings and results of stapedectomy in 32 patients (43 ears) with osteogenesis imperfecta are presented. The stapes footplates were in many ears thick and of a very soft consistency. In all the operated ears we found the stapes footplates fixated in the oval window. Brittle and atrophic stapes crura were a common finding, but in only 5 ears fractures of the crura were observed. A thick and vascular middle ear mucosa was seen in several ears. Although the middle ear proved quite different from that of otosclerosis, no major difficulties and no serious complications occurred during stapedectomy. Both the immediate postoperative hearing gain and the long-term hearing results were, in general, satisfactory. Our study supports the statement of others, that stapedectomy in osteogenesis imperfecta is a safe and reasonable alternative to hearing aids.