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To describe a group of children exhibiting electrophysiologic responses characteristic of auditory neuropathy (AN) who were subsequently identified as having absent or small cochlear nerves (i.e., cochlear nerve deficiency).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.Of the nine children with cochlear nerve deficiency, five (56%) were affected unilaterally and four (44%) bilaterally. Eight of nine presented after failing a newborn infant hearing screening, whereas one presented at 3 yr of age. On diagnostic ABR testing, all 9 children (9 of 13 affected ears; 69%) had evidence of a cochlear microphonic (CM) and absent neural responses in at least one ear. In the unilateral cases, AN characteristics were detected in all affected ears. In bilateral cases, at least one of the ears in each child demonstrated the AN phenotype, whereas the contralateral ear had no CM identified. Only one ear with cochlear nerve deficiency had present otoacoustic emissions as measured by distortion-product otoacoustic emissions. In children with appropriate available behavioral testing results, all ears without cochlear nerves were identified as having a profound hearing loss. Only 4 (31%) of the 13 ears with cochlear nerve deficiency had a small internal auditory canal on MRI.Children with cochlear nerve deficiency can present with electrophysiologic evidence of AN. These children frequently refer on newborn screening examinations that use ABR-based testing methods. Similar to other causes of AN, diagnostic ABR testing will show a CM with absent neural responses. Given that 9 (18%) of 51 children with available MRI and electrophysiologic characteristics of AN in our program have been identified as having cochlear nerve deficiency makes this a relatively common diagnosis. These findings suggest that MRI is indicated for all children diagnosed with AN. Moreover, electrophysiologic evidence of unilateral AN in association with a profound hearing loss should make the clinician highly suspicious for this problem. Although children with cochlear nerve deficiency who have a small nerve may benefit from cochlear implantation or amplification, these interventions are obviously contraindicated in children with completely absent cochlear nerves.