A Case of Branchio-oto-renal Syndrome

This is the authors' abstract. We don't add key points for this paper.

Branchio-oto-renal (BOR) syndrome is an autosomal dominant disorder with branchial anomalies: ear malformation, hearing loss, cervical fistula, and renal anomalies.We experienced a case of BOR syndrome. A 16-year-old female had bilateral preauricular pits, bilateral hearing loss, left cervical fistula, and right renal aplasia. Computed tomography revealed inner ear anomalies with cochlear hypoplasia and an enlarged internal auditory meatus. We treated left cervical fistula only in this case. As the rate of improvement in hearing loss by surgical treatment such as tympanoplasty is not necessarily high, we should decide the indication for sur gery carefully.