Abstract
Van der Hoeve syndrome is a hereditary disease which has three symptoms: blue sclera, hearing loss and osteogenesis imperfecta. The symptoms are caused by the obstruction of collagen synthesis, and there are many possible complications in surgery for this disease. We report a case of van der Hoeve syndrome treated by partial stapedectomy of unilateral ear.
In operative findings, it showed an otosclerotic lesion along with the stiffening and fixation of the stapes footplate on the oval window. In postoperative audiogram, the air-bone gap in the main speech frequency range was down to 20-30dB, however there were no improvements in the bone conductive thresholds as compared to preoperation.
This result indicates that stapes surgery in van der Hoeve syndrome is useful for hearing enhancement.
