機能性難聴との鑑別を要した遠視および手指骨異常を伴う耳小骨奇形症例

抄録

We report a case of hereditary conductive hearing impairment, which was diagnosed by subsequent examinations, although it was considered as pseudohypacusis at the first visit. Since the patient had hyperopia and broad thumbs, Teunissen-Cremers syndrome was suspected. Since her hearing was fluctuated, determining of a true threshold of her hearing was difficult. CT, tympanometry, stapedials muscle reflex, and other tests were performed. Finally, we diagnosed of conductive hearing impairment based on auditory brainstem response (ABR). Exploratory tympanotomy was performed and ossicular anomaly and stapes fixation were detected. Her hearing was improved by stapedotomy. When pseudohypacusis was suspected, a careful examination is necessary. It was considered that the diagnosis of conductive hearing impairment is very important in this case, because hearing improvement can be expected following an appropriate surgical procedure.