A case of van der Hoeve syndrome treated with stapes surgery in both ears

Abstract

A 40-year-old woman had bilateral mixed hearing loss with air-bone gap and no stapedial muscle reflex. She had blue sclera and a history of bone fracture with osteogenesis imperfecta. We made the diagnosis of van der Hoeve syndrome based on these clinical symptoms, signs, and examination findings. In the left ear, stapedotomy was attempted, but the footplate was fractured. Partial stapedectomy was thus performed with removal of the posterior half of the footplate. In the right ear, stapedotomy was performed successfully. After the surgery, her bilateral hearing loss improved. Hearing loss in cases of van der Hoeve syndrome is conductive loss and resembles that found in cases of otosclerosis. The present findings suggest that stapes surgery for van der Hoeve syndrome is valid, and stapedectomy is useful if carefully performed.