Hearing Implants in Pediatrics With Cochlear Nerve Deficiency: An Updated Systematic Review

European Archives of Oto-Rhino-Laryngology

Alahmadi, A., Abdelsamad, Y., et al. (2024).

European Archives of Oto-Rhino-Laryngology, Advance online publication. https://doi.org/10.1007/s00405-024-09087-w.

<div>This systematic review investigates the effects of cochlear implantation (CI) or auditory brainstem implant (ABI) in children, less than 18 years old, with cochlear nerve deficiency (CND).</div>

No funding received



January 2000 to September 2022

<div>English-language studies. Excludes case reports, reviews,&nbsp;preclinical and invitro studies,&nbsp;and conference proceedings</div>

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<div>Overall, CI demonstrated feasibility and improved Categories of Auditory Performance (CAP) scores for children with CND; however, other measures (e.g., Speech Intelligibility Rating [SIR], Meaningful Auditory Integration Scale [MAIS]) found limited effects. The authors conclude that the effects of CI can be limited, especially in children with an absent or abnormal cochlear nerve.</div> <div>&nbsp;</div> <div>Subgroup analysis by CN classification showed significant outcome differences between CN hypoplasia and CN aplasia. Children with CN hypoplasia demonstrated higher CAP, MAIS, and Meaningful Use of Speech Scale (MUSS) scores and a greater likelihood of using spoken language compared to children with CN aplasia.&nbsp;</div> <div>&nbsp;</div> <div>Limitations of this review include small sample sizes, low evidence quality, and a lack of sufficient data to conduct a meta-analysis. Additional research is needed to investigate the effects of simultaneous CI and ABI in children with CND.</div>

<div>ABI improved environmental sound awareness, speech detection, and speech recognition in children with CND.&nbsp; ABI showed associations with higher CAP and SIR scores as well as better behavioral responses and speech perception compared to CI. Subgroup analysis by CN classification showed no significant differences between CN hypoplasia and CN aplasia.</div> <div>&nbsp;</div> <div>In studies reporting adverse effects, ABI demonstrated associations with higher risks for neurological complications and programming difficulties.</div> <div>&nbsp;</div> <div>Limitations of this review include small sample sizes, low evidence quality, and a lack of sufficient data to conduct a meta-analysis. Additional research is needed to investigate the effects of simultaneous CI and ABI in children with CND.</div>