Cochlear implantation in a child with auditory neuropathy spectrum disorder

ObjectiveTo report outcomes of cochlear implantation (CI) in a child with auditory neuropathy spectrum disorder (ANSD) and to provide preliminary clinical evidence of the efficacy of CI in ANSD patients.MethodsA 4–year–old boy with diagnosed auditory neuropathy spectrum disorder (ANSD) received implantation of a Nucleus CI24R after an unsatisfactory trial of amplification. Post–implantation performance in both hearing sensitivity and speech recognition was assessed in different sessions. Aided hearing thresholds were tested by behavioral audiometry. Mandarin Early Speech Perception Test (MESP), Meaningful Auditory Integration Scale (MAIS), category of auditory performance (CAP) and Speech Intelligibility Rating (SIR) were used to assess the benefits in auditory skills or speech recognition the boy obtained from CI. The tests were administered before surgery and at 3 months and 7 months after opening.ResultsThe boy demonstrated improved auditory sensitivity by using CI. Concerning speech recognition and communication, both speech audiometry and questionnaires showed an obvious benefit from CI.ConclusionsCI has worked efficiently in this ANSD boy. But because of limited understanding of ANSD and rehabilitation effect by cochlear implantation in this condition, the clinical decision to implant should be cautious and only after a thorough evaluation. Meanwhile, well controlled and long term studies are needed to confirm the efficacy of cochlear implantation in patients with ANSD.