2000 年 10 巻 1 号 p. 32-37
A 8-year-old girl of CHARGE association with a moderate mixed hearing loss was reported. Her temporal bone CT and MRI showed ossicular chain deformity, Mondini type malfomation of the cochlea, and the absent three semicircular canals with dilatated vestible. The hearing loss showed mixed type due to congenital middle ear anomaly. The patient underwent middle ear surgery in the left ear. A monopodal stapes attached the fallopian canal, the footplate was missing, and the oval window was covered by loose connevtive tissue. Post operative hearing improved by 27. 5dB. However, her hearing gain decreased by about 10dB one year after surgery.