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How common is hearing impairment in osteogenesis imperfecta?

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How common is hearing impairment in osteogenesis imperfecta?

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Paterson CR; Monk EA; McAllion SJ Department of Medicine, University of Dundee, Dundee, Scotland. c.r.paterson@dundee.ac.uk. Hearing impairment has long been recognized as a common feature in osteogenesis imperfecta. The figures in some publications could be taken to imply that, with increasing age, the proportion of osteogenesis imperfecta patients with hearing impairment approaches 100 per cent. The incidence of hearing loss in a large survey of 1394 patients with osteogenesis imperfecta was examined. It was found that the most common age of onset 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 impairment; over the next 20 years there was little further increase. Differences were shown between patients with different clinical types of osteogenesis imperfecta as delineated in the Sillence classification; hearing loss was significantly less common in the type IV disease than in the type I disorder.

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