Predictors of Cochlear Implant Outcomes in Paediatric Auditory Neuropathy Spectrum Disorder (ANSD): A Systematic Review

Abstract

Abstract: Objective: This review identifies prognostic factors influencing cochlear implant (CI) outcomes in paediatric auditory neuropathy spectrum disorder (ANSD). Design: A systematic search across six databases identified studies on CI outcomes in children with ANSD based on predefined eligibility criteria. Prognostic indicators were extracted, findings synthesised narratively, and quality assessed using the Joanna Briggs Institute (JBI) checklists. Sample: Six observational studies met inclusion criteria, including 204 children with ANSD. Cohorts varied in diagnostic methods, comparators, analytic variables, and outcome measures. Results: Evidence indicates that CI outcomes in ANSD are influenced by multiple prognostic factors. Children with favourable profiles, such as presynaptic aetiologies (e.g., OTOF mutations), intact cochlear nerve anatomy, and early implantation (<2 years), achieved outcomes comparable to peers with typical sensorineural hearing loss (SNHL). Conversely, cochlear nerve deficiency and delayed implantation were associated with poorer outcomes. No eligible evidence was identified regarding comorbidities as a prognostic factor. Additionally, preoperative residual hearing and cortical auditory evoked potential (P1) emerged as beneficial supplementary markers. The study quality was moderate in five studies and moderate to high in one. Conclusions: Accurate CI outcome prediction in ANSD requires a multidimensional approach, integrating anatomic, aetiological, and neurophysiological factors to guide decision-making and optimise rehabilitation.