Objective
The aim of this mini-systematic review was to evaluate the evidence reporting speech, language, and auditory behavioral outcome measures for children with a diagnosis of auditory neuropathy spectrum disorder (ANSD) who received cochlear implants (CIs) prior to 3 years of age.
Method
A mini-systematic review of the literature supporting evidence-based practices was performed. Two databases were searched utilizing a search strategy derived from the PICO (patient, intervention, comparison, outcome) framework. Peer-reviewed articles published between 2009 and 2019 evaluating children with a diagnosis of ANSD who were implanted prior to 3 years of age with a range of speech, language, and auditory behavioral outcomes were included. Four articles meeting inclusion criteria were critically appraised for reputable research design and risks of bias. Each of the four studies was assigned a level of evidence for effectiveness and quality assessment rating.
Results
Evidence supports cochlear implantation as an appropriate intervention for children with ANSD. Improvements in outcome performance were observed in all the included studies. Children with ANSD fit with CIs can achieve outcomes similar to children with sensorineural hearing loss and CIs, despite the heterogeneity of ANSD.
Conclusion
These findings have implications for clinical practice and for future research with current CI technology for facilitating parent education, counseling, and realistic expectations for children with ANSD and CIs.
Understanding auditory neuropathy spectrum disorder: a systematic review in transgenic mouse models