Abstract
Osteogenesis imperfecta is a connective tissue disorder characterized by osseous fragility, blue sclerae and hearing loss. Pre-operative and post-operative audiograms were used to assess the impact of stapedotomy on improving hearing on an osteogenesis imperfecta patient for the conductive hearing loss. Operative findings were fixation of the stapes footplate with normal superstructure configuration and hypervascularization of the promontory’s mucosa. Immediate post-operative results showed a significant hearing improvement with an important closure of the airbone gap. The long-term results were not yet established. These satisfactory results and the lack of complications make stapedotomy an appealing method for the management of osteogenesis imperfecta associated hearing loss.
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