A 44-year-old female with van der Hoeve syndrome is reported. She showed symptoms of blue sclera and hearing loss, but had no tendency towards spontaneous fractures. Her father also had blue sclera and hearing loss. Her daughter and son had blue sclera and many spontaneous fractures, but had normal hearing. Pure tone audiometry showed a mixed hearing loss of 73. 3 dB in the left ear, and 53. 3 dB in the right ear. The operation findings of both ears revealed the pathophysiology of deafness to

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... fixation of the stapedial footplate but the shapes of the stapes were normal. A small fenestra stapedotomy was perfomed in both ears. She obtained improvement in both hearing levels. The diagnosis and therapeutic problems of this syndrome are discussed with a review of the literature.