A Systematic Review and Meta-analysis Examining Outcomes of Cochlear Implantation in Children With Bilateral Cochlear Nerve Deficiency
Otology & Neurotology
Maturi, J. R., Noij, K. S., et al. (2024).
Otology & Neurotology, 45(9), 971-984.
<div>This systematic review and meta-analysis investigates the effects of cochlear implants (CIs) on hearing and speech outcomes for children with bilateral cochlear nerve deficiency.</div>
Not stated
From database inception to July 14, 2023
<div>Qualitative or quantitative studies, excluding meta-analyses</div>
40
<div>Following cochlear implantation, 84% of children across studies showed some clinical improvement in auditory or speech testing. Meta-analysis of 36 studies showed 31% of children reached open-set speech perception, 22% achieved closed-set speech perception, 28% experienced improved detection and parent-perceived benefit, and 19% had no measurable stimulation.</div>
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<div>Limitations to this review included heterogeneity of reported outcomes and small sample sizes of the included studies.</div>
<div>Children with cochlear nerve aplasia had significantly worse auditory performance level (APL) scores compared to those with cochlear nerve hypoplasia. Children with syndromes also had significantly worse APL scores than their peers without syndromes. There were no significant differences for children with vs. without cochlear or vestibular malformations or for those with unilateral vs. bilateral CIs. For children with cochlear nerve aplasia, those implanted after 2 years of age had significantly higher APL scores than those implanted before 2 years; this age difference at implantation did not significantly impact outcomes for children with cochlear nerve hypoplasia. Male children with cochlear nerve hypoplasia showed significantly worse APL scores compared to their female peers; however, this gender difference was not significant for children with cochlear nerve aplasia. </div>
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<div>Limitations to this review included heterogeneity of reported outcomes and small sample sizes of the included studies.</div>
<div>Following cochlear implantation, children with cochlear nerve aplasia and hypoplasia experienced a 3.2 and 3.5 average point increase in Categories of Auditory Performance score, respectively. Limitations to this review included heterogeneity of reported outcomes and small sample sizes of the included studies.</div>
<div>On average, children with cochlear nerve aplasia and hypoplasia experienced a 0.9 and 1.3 increase in Speech Intelligibility Rating (SIR) score, respectively. Approximately 60% of children moved from a pre-speech SIR stage to a stage of intelligible speech following cochlear implantation. Limitations to this review included heterogeneity of reported outcomes and small sample sizes of the included studies.</div>
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