Abstract
Osteogenesis imperfecta (OI) is a heterogeneous group of connective tissue disorders. The classic triad of blue sclerae, spontaneous fractures and hearing loss is known as the Van der Hoeve and De Kleyn syndrome. Between 1989 and 2000, six patients with OI presented with conductive hearing loss. Five of them proceeded to stapedotomy. All the patients who had surgery had significant hearing gain. None of the patients had any complications. This study presents a higher incidence of spontaneous fractured crura as the cause of the conductive hearing loss than previously reported, and that the presence of a fractured crura with mobile footplate can be anticipated by the presence of a large conductive hearing loss. The pre- and postoperative results are presented and support the view that stapes surgery in OI can have encouraging results, provided the operator anticipates the possibility of a fractured crura and a mobile footplate.
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