A Case of CHARGE Association.

A 7-year-old girl with CHARGE association is reported. This patient exhibited coloboma, tetralogy of Fallot, retarded development, ear anomaly and deafness, which fulfiled the criteria of CHARGE association. Pure tone audiography showed bilateral moderate conductive hearing loss. CT of her temporal bone revealed normal cochlea and hypoplastic bilateral semicircular canals. She was unresponsive to cold water stimuli in the caloric test. As an ossicular chain anomaly was suspected, an exploratory operation was carried out on her right ear. The stapes lacked its anterior crus and footplate, which was considered to be the cause of her conductive hearing loss. This disease is not rare and otorhinolaryngologists should consider this condition when examining young patients with deafness and ear anomalies.