Pediatric Cochlear Implant Outcomes in Auditory Neuropathy, Cochlear Nerve Deficiency, and Sensorineural Hearing Loss: An 8-Year Longitudinal Study

OBJECTIVES: Cochlear implants (CIs) have transformed the management of children with significant hearing loss; however, outcomes vary considerably across different etiologies. This longitudinal study aimed to compare CI outcomes in children with sensorineural hearing loss (SNHL), auditory neuropathy spectrum disorder (ANSD), and cochlear nerve deficiency (CND). DESIGN: The scores of five CI outcome measures: Categories of Auditory Performance, Speech Intelligibility Rating, Open Set Words, Closed Set Words, and Meaningful Auditory Integration Scale, were compared among 47 children with SNHL, 35 with ANSD, and 13 with CND, for a total of 95. Data were collected as part of routine clinical follow-ups at Necker Hospital in France at baseline and five post-CI intervals: 1, 2, 3, 5, and 8 years. RESULTS: While children with ANSD and SNHL achieved comparable outcomes, those with CND consistently showed lower performance across follow-up visits (p < 0.001). All three groups demonstrated significant improvements in spoken language; yet, the performance gap between CND and the other groups persisted. Findings also suggested a hierarchy of outcome measure difficulty, progressing from Meaningful Auditory Integration Scale and Closed Set Words to Open Set Words, and then to Speech Intelligibility Rating and categories of auditory performance, with the latter two requiring longer-term CI use to reach optimal outcomes. CONCLUSIONS: Our findings highlight that lesion site, particularly cochlear nerve integrity, is a strong determinant of long-term CI outcomes. Persistent deficits in CND underscore the need for tailored habilitation and realistic expectations. The observed hierarchy of outcome measures supports the use of comprehensive batteries that combine basic and advanced assessments to capture both immediate and long-term impacts.