BRANCHIOOTORENAL SYNDROME

Branchio-oto-renal (BOR) syndrome is a rare, autosomal dominant geneti c disorder involving branchial cleft-and renal anomalies, hearing loss , and other otologic manifestations. We report a case of a family with three generations of branchial cleft anomalies and otologic anomalies with hearing loss. A review of the literature, classic clinical prese ntations, associated findings, and the differential diagnosis of BOR s yndrome is presented. Due to BOR syndrome's variability of both penetr ance and expression and a high incidence of renal anomalies, the pract icing otolaryngologist should consider BOR syndrome when evaluating he aring loss and branchial cleft remnants in any child.