Abstract
A 28-year-old man presented with progressive right hearing loss from a year previously. He had suffered from left hearing loss since his junior high school days. He received medication from other clinic but his symptom was not improved. He visited Kyoto University hospital when he was 27 years old. He had right mixed hearing impairment with 51.7 dB of air conduction hearing level, 20 dB of air-bone gap, and no stapedial muscle reflex. Blue sclera was noted and the patient had a history of osteogenesis imperfecta with several instances of bone fractures. CT scan showed demineralization in the bilateral cochleae and vestibules. We made the diagnosis of van der Hoeve Syndrome based on these clinical symptoms, signs, and examinations. A small fenestration stapedectomy was attempted in his right ear but the footplate was fractured. A partial stapedectomy was performed with removal of the posterior half of the footplate. The averaged improvement for air conduction hearing level was 31.7 dB and the air-bone gap was 10 dB even two years after the surgery. This case suggests that stapedectomy for van der Hoeve syndrome is useful if it is carefully performed.
