Objective/Hypothesis: The aim of this study was to investigate cochlear implantation (CI) outcome in children with nerve deficiency. Study Design: Retrospective chart review. Methods: A total of seven children with prelingual profound deficiency (hypoplasia or aplasia) were included. A control group of 10 CI children with no cochlear nerve anomalies was also included. In addition to implant stimulation levels, children's performance on pure-tone audiometry, speech reception measure, and auditory and speech skills ratings were compared across groups. Additionally, pre- and postoperative audiologic results were evaluated for the group with nerve deficiency. Results: In general, children with nerve deficiency performed poorer than those without nerve deficiency on all tested measures. Stimulation levels were considerably higher and more variable than the control group. Results further showed that performance was dependent on the diameter of the internal auditory canal. Conclusion: Overall, cochlear implantation outcome in children with auditory nerve deficiency is poorer and extremely more variable than those without nerve deficiency. However, three of the patients had a noticeable improvement in auditory performance postimplantation suggesting that CI is a viable option in this population but expected benefit can be dependent on the status of the cochlear nerve.
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