Cochlear Implantation in Pediatric Cochlear Nerve Deficiency: A Systematic Review and Meta-Analysis

Cochlear nerve deficiency (CND) is commonly implicated in moderate-to-profound pediatric sensorineural hearing loss (SNHL). Although cochlear implantation (CI) was previously contraindicated in CND patients, recent studies have demonstrated the potential for auditory response to CI in a subset of CND patients, though clinical outcomes remain variable. This study aims to evaluate pre- and postoperative speech and auditory outcomes of CI in pediatric patients with bilateral SNHL and radiographically confirmed CND.

Embase and Ovid MEDLINE.

A systematic review was conducted to identify studies reporting pre- and postoperative outcomes of children with CND confirmed by magnetic resonance imaging who underwent CI. A random-effects model was used to account for within- and between-study variance in speech and auditory outcomes.

After abstract screening of 818 distinct articles, 16 studies were selected for final inclusion, consisting of 248 patients with cochlear nerve hypoplasia or aplasia who underwent CI. Various speech perception and language development tests were investigated across studies (Speech Intelligibility Rating, Speech Perception Category, Speech Awareness Thresholds, Meaningful Auditory Integration Scale, Meaningful Use of Speech Scale, Categories of Auditory Performance, and Auditory Level). Pooled outcomes demonstrated improvement in speech perception after CI in pediatric patients with CND (SMD 2.18, 95% CI 1.68-2.69).

Indications for CI are expanding as research demonstrates benefit in populations previously thought inappropriate. Our study demonstrates global postoperative improvement in speech and auditory outcomes in children with bilateral SNHL and CND after CI.

NA Laryngoscope, 135:1259-1266, 2025.