Surgical Findings in van der Hoeve's Syndrome

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Abstract
Many otologists seem to be reluctant to perform middle ear surgery on patients with van der Hoeve's syndrome due to a dearth of information on surgical experiences in the literature. We review our surgical experience with eight ears in five patients. Surgery revealed soft brittle bone in the external canal. The foot plate in each case had a heavy growth of white chalky mounded bone, but had discrete margins and was only slightly fixed, a combination that predisposes to a floating foot plate. Certain guidelines have evolved from our observations. Special care is necessary in curretting the canal wall, fracturing the foot plate to avoid a "floating foot plate," removing the fragments, and finally in crimping the prosthesis. The generally satisfactory results tend to substantiate the hypothesis that stapedectomy is the treatment of choice for stapes fixation in van der Hoeve's syndrome.