Genetic hearing loss with preauricular sinus and branchiogenic fistula

A mother and her two children had hearing loss associated with bilateral preauricular sinus and branchiogenic fistula. All six cochleas studied showed two turns rather than 2 1/2 turns. Complete studies including audiometry, tympanotomy findings, and temporal bone polytomography of these anomalies are reported. Similar cases reported in the English literature are reviewed. It seems that all previous cases may have had an abnormal cochlea as was seen in these cases.