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Cochlear Nerve Aplasia and Hypoplasia: Predictors of Cochlear Implant Success

Objective To identify factors predicting performance outcomes following cochlear implantation in patients with cochlear nerve aplasia or hypoplasia. Data Sources Individual patient data extracted from published case series and reports. Review Methods The MEDLINE database, Cochrane Library, Embase, Web of Science, and Google Scholar were queried for “cochlear implant” in conjunction with “aplasia” or “hypoplasia” between 1985 and 2015. Eighteen studies were included describing 97 subjects with individual postimplant auditory data. Postimplant performance was categorized as follows: level 1, nonstimulation/minimal detection; level 2, improved detection; level 3, closed-set speech perception; or level 4, open-set speech perception. The subjects achieving speech perception (levels 3 and 4) were descriptively compared with those who did not. Results Subjects with a hypoplastic cochlear nerve on magnetic resonance imaging had higher reported rates of achieving speech perception than those with an aplastic nerve. Subjects with syndromic medical comorbidities had higher reported rates of nonstimulation than nonsyndromic subjects. The data showed that some children with an aplastic cochlear nerve or those with partial electrode insertion could obtain levels of speech discrimination. Reporting of patient characteristics and auditory outcomes was extremely variable across studies. Conclusion As previously shown, cochlear implant in patients with cochlear nerve aplasia or hypoplasia can provide meaningful hearing for select patients. The current study suggests that presence of a cochlear nerve on magnetic resonance imaging and lack of comorbid medical syndrome are associated with better auditory outcomes in such patients. Future efforts to report individual data in a consistent manner may allow better determination of predictive factors.