Abstract
Osteogenesis imperfecta is a connective tissue disorder caused by a genetic defect in the synthesis of type I collagen. The symptons of blue sclera, a tendency towards spontaneous fractures and conductive hearing loss due to osteogenesis imperfecta are often termed Van der Hoeve syndrome.A 28 year-old female with van der Hoeve Syndrome is reported here. At 20 years of age, she complained of bilateral tinnitus and hearing loss. Pure tone audiometry showed mixed hearing loss of 49 dB in the right ear, and 38 dB in the left ear. Stapes mobilization was then performed on her right ear. Surgical observation revealed that the stapedial footplates were fixed to the oval window but were otherwise normal in shape. She obtained an improvement in her hearing level in her immediate postoperative period.
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