Auditory neuropathy characteristics in children with cochlear nerve deficiency

Objective: To describe a group of children exhibit-ing electrophysiologic responses characteristic of auditory neuropathy (AN) who were subsequently identified as having absent or small cochlear nerves (i.e., cochlear nerve deficiency). Design: A retrospective review of the clinical records, audiological testing results, and magnetic resonance imaging (MRI) studies. Fifty-one of 65 children with AN characteristics on auditory brain stem response (ABR) testing had MRI available for review. Nine (18%) of these 51 children with ABR characteristic of AN have been identified as having small (N 2; 4%) or absent (N 7; 14%) cochlear nerves on MRI. Results: Of the nine children with cochlear nerv