1992 年 2 巻 2 号 p. 251-254
Two cases, a female and her mother, of van der Hoeve syndrome are reported. They showed a conductive or mixed hearing loss. A stapedotomy was performed on each case. The findings at surgery were similar in both cases. The stapedial footplates in both cases were fixed firmly, and were normal in shape. The histopathological findings of stapes indicated similar type to that of otosclerosis in one patient, and those were almost normal in the other patient. After the stapedotomy, ecxellent hearing level was obtained in both patients.