What is it about?

This systematic review of current systematic reviews indicates that, in the long term, children with auditory neuropathy spectrum disorder (ANSD), especially those with pre-synaptic ANSD, who receive cochlear implants (CIs), can hear, and understand spoken language just as well as children with sensorineural hearing loss (SNHL). For children with post-synaptic ANSD (or cochlear nerve deficiency), poor speech perception outcomes are more likely in those with an absent auditory nerve (aplasia) compared to those with an impaired auditory nerve (hypoplasia) on MRI.

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Why is it important?

The findings could be used for clinical decision-making and to assist in guiding parents of children who are candidates for CIs. However, caution should be exercised in the interpretation of these findings due to the low quality of evidence used to draw conclusions.


Current evidence supports the benefits of CIs for children with ANSD. To enhance the quality of this evidence, it is essential to conduct well-designed, prospective studies with appropriate sample sizes, employ valid outcome measures, clarify matching criteria, and consider the influence of confounding factors.

Zahra Jafari
Dalhousie University

Read the Original

This page is a summary of: An Umbrella Review of Cochlear Implant Outcomes in Children With Auditory Neuropathy, Journal of Speech Language and Hearing Research, August 2023, American Speech-Language-Hearing Association (ASHA),
DOI: 10.1044/2023_jslhr-23-00128.
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