How common is hearing impairment in osteogenesis imperfecta?

Hearing impairment has long been recognized as a common feature in osteogen esis imperfecta. The figures in some publications could be taken to imply t hat, with increasing age, the proportion of osteogenesis imperfecta patient s with hearing impairment approaches 100 per cent. The incidence of hearing loss in a large survey of 1394 patients with osteo genesis imperfecta was examined. It was found that the most common age of o nset was in the second, third and fourth decades of life. At the age of 50 approximately 50 per cent of the patients had symptoms of hearing impairmen t; over the next 20 years there was little further increase. Differences were shown between patients with different clinical types of os teogenesis imperfecta as delineated in the Sillence classification; bearing loss was significantly less common in the type TV disease than in the type I disorder. Among the 29 families with osteogenesis imperfecta type IA the re were distinct differences in the likelihood of hearing loss. These findi ngs provide insights which will be valuable in giving patients advice on th e likelihood of developing hearing loss in the future.