Cochlear implantation in children with auditory neuropathy spectrum disorder: an updated systematic review

Background: The goal of managing auditory neuropathy spectrum disorder (ANSD) is to restore the children's ability to discriminate auditory information. Children who are not making sufficient progress in speech comprehension, and speech and language development after receiving adequate auditory re/habilitation and/or acoustic amplification may be candidates for cochlear implantation (CI). Despite the growing number of published literature on CI outcomes in children with ANSD, the current evidence is primarily based on case reports or retrospective chart reviews some of which had a limited number of children. In addition, the outcomes of CI seem to vary between children with ANSD. Thus, compelling evidence is lacking. This updated systematic review evaluated the speech perception, language, and speech intelligibility outcomes of children with ANSD post-CI.

Methods: An online bibliographic search was conducted in PubMed, Scopus, Web of Science, and CENTRAL databases. We included both interventional and observational studies that assessed the outcomes of the CI in children with ANSD.

Results: Thirty-three studies were included in this systematic review. Several tests were used to assess speech perception following CI in children with ANSD. The findings of this study revealed that children with ANSD had mean Categories of Auditory Performance scores ranging from 4.3 to 7 post-operatively, this result was better than the pre-operative scores which ranged between 0.4 to 2.5. Likewise, the Infant-Toddler Meaningful Auditory Integration Scale, Phonetically Balanced Kindergarten, and multisyllabic lexical neighborhood test showed clinically relevant improvement after CI. The same findings were reported for language and speech intelligibility scores. One study investigated the quality of life/children satisfaction after CI and showed overall good satisfaction with the outcomes.

Conclusions: The present systematic review suggests that CI is a feasible and effective hearing rehabilitation modality for children with ANSD.

Registration and protocol: PROSPERO ID: CRD42021279140.