BOR (Branchiootorenal) syndrome, which clinically overlaps with branchiootic syndrome, is characterized by multiple malformations clinically diagnosed by the following major criteria: second branchial arch anomalies, deafness, preauricular pits, auricular deformities and renal anomalies (ranging from mild to severe or complete absence of kidneys). Minor criteria include: external auditory canal anomalies, middle or inner ear anomalies, preauricular tags and others. To be diagnosed there must be 2 affected individuals in the family; or the individual must display 3 or more of the major criteria, or 2 major and 2 minor criteria. Both reduced penetrance and variable expressivity have been observed. The estimated prevalence of BOR syndrome is 1:40,000 in the general population and ~2% among profoundly deaf children. Hearing impairment can be mild to severe and can be conductive, sensorineural or mixed.