Hearing implants in pediatrics with cochlear nerve deficiency: an updated systematic review

Cochlear nerve deficiency (CND) accounts for about one-third of congenital hearing loss cases and presents several challenges during management. Currently, cochlear implant (CI) and auditory brainstem implants (ABI) represent the primary management options for CND. However, robust evidence on the outcomes of different CND management approaches remains limited. Therefore, this systematic review seeks to update and assess the effectiveness of various CND management approaches.

PubMed, Scopus, Web of Science, and CENTRAL databases.

We conducted an online bibliographic search across these databases. We included both interventional and observational studies evaluating CND management outcomes.

Of the 25 studies included, there were retrospective chart reviews, prospective observational studies, and one phase I clinical trial. Most studies focused on children with cochlear nerve aplasia or hypoplasia, with one study including patients with CHARGE syndrome. The mean age at the time of operation ranged from 0.5 to 4.5 years. Tools like the categories of auditory performance (CAP), speech intelligibility rating (SIR), and speech perception category (SPC) were commonly employed. CI outcomes were reported in 17 studies, while ABI outcomes were reported in 4 studies. The study found that the outcomes of both interventions were variable across the included studies.

While CI is feasible and an option in children with CND, its overall effectiveness is limited in some cases like cochlear nerve aplasia. Conversely, ABI appears to be a promising alternative with better auditory, speech, and learning outcomes.