Surgery for congenital absence of the oval window: report of two cases

Abstract

A 64-year-old man presented with bilateral hearing impairment and vertigo since he was 24 years of age. He was treated as Méniere's disease, but the treatment was not effective. Blue sclera was noted and the patient was also suffered from bone fracture three times in the past, and he was diagnosed as having van der Hoeve syndrome. CT scan and MR imaging showed progressive otic capsule demineralization. Small fenestra stapedectomy was performed in his right ear. Otic capsule was significantly demineralized and thin. Finally, he recovered his hearing using a hearing aid without any complication. Stapes surgery for van der Hoeve syndrome is safe and useful.