Abstract
Branchio-Oto (BO) syndrome is an autosomal dominant genetic disorder with branchial anomalies involving preauricular pits, cervical fistulas or cysts and hearing loss. We present the case of a 31-year-old female with BO syndrome who had complained of bilateral hearing loss, various ear anomalies and right cervical fistula. The cervical fistula was totally resected after staining with Pioctanin under general anesthesia. Pathological examinations showed that not only the sebaceous gland but also salivary gland tissue, which was apart from the parotid or submandibular gland, was present around the fistula. The present case was diagnosed as BO syndrome because of the presence of typical ear anomaly and functional disorder and cervical fistula without anomaly. The cervical fistula of this case was connected with ectopic salivary gland tissue and was considered to be a salivary duct fistula.