In Japan, otosclerosis is a relatively rare disease compared to the incidence in Caucasians. In this paper we report two sisters,53 and 49 years old, who have otosclerosis.
These patients consulted Aichi Medical University complaining of bilateral tinnitus and hearing loss, and were diagnosed as otosclerosis. The older sister had a hearing level of 56 dBHL in the right ear, and 49 dBHL in the left with combined hearing impairment in the low frequency range. The younger sister had a hearing level of 96 dBHL in the right, and 73 dBHL in the left with combined hearing impairment in the high frequency range. Radiographic examination showed no specific findings.
Bilateral stapedotomy was performed in the ears of both patients. Intraoperative findings revealed otosclerosis with sclerotic changes of the stapes. The superstructure of stapes in the left ear of the younger sister showed sclerotic changes histopathologically. Abnormal vascularity, thought to be the stapedial artery, was revealed through the foramen obliteration in the older sister. Both patients had unilateral hearing recovery postoperatively.
