Indicators for Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder: A Systematic Review

Abstract

ANSD refers to a group of auditory diseases demonstrating intact outer hair cells and desynchronized neural firings of the auditory nerve.Cochlear implant has emerged as a promising intervention strategy for severe to profound sensorineaural hearing loss (SNHL).However,due to its variable outcomes in children with ANSD,a consensus has yet to be reached on its performance in them. This review aims to summarize and synthesize current evidence of the performance of CI in children with ANSD by identifying test tools that will predict post CI performance.A review of 17 articles was conducted in order to highlight these predictors.Most of the selected studies included case reports,case series,cohorts and comparison between children with ANSD and SNHL. Assessment of study quality reported overall low risk of bias.A set of pre operative and post operative indicators were identified that not only predicted speech and auditory performance but also gave some insight about site of lesion in ANSD individuals. This review also highlights the need to include more precise tools to describe the site of lesion in order to choose the most appropriate management strategy for children with ANSD.