Auditory neuropathy in siblings with Waardenburg's syndrome

We report two siblings with a family history of Waardenburg's syndrome (WS) for whom the audiological profile corresponds to auditory neuropathy (AN). They have; (1) bilateral severe to profound hearing loss, (2) robust oto-acoustic emissions (OAEs) in both ears, and (3) no auditory evoked responses at 95 dBnHL bilaterally. Electrocochleography (ECochG) and auditory middle and late latency potentials were performed in one of the children. Results showed cochlear and neural activities in both ears. Central auditory responses were not conclusive. These children did not have any history of neonatal illness and one child was diagnosed with AN at the age of 3 weeks and the other at the age of 11 months.