Objective(s)
To describe cochlear implantation (CI) outcomes, with speech perception, auditory, language, and parent-reported auditory and speech behaviors, in children with an enlarged vestibular aqueduct (EVA) and incomplete partition type 2 (IP-II) and compare to control children without inner ear malformations (IEMs) and to determine cerebrospinal fluid gusher rates and effect on outcomes.
Study Design
Systematic review and meta-analysis.
Methods
MEDLINE, Embase, Cochrane, and CINAHL databases were searched from inception to February 2020. Studies reporting relevant outcomes in children with EVA or EVA + IP-II and controls without IEMs undergoing CI were included. Mean differences in speech perception, auditory, and language scores between cases and controls were meta-analyzed. Gusher rates were determined by proportion meta-analyses.
Results
Of 214 identified articles, 42 met inclusion criteria, evaluating 775 cases and 2,191 controls. Of –cases, 578 (74.6%) had EVA and 197 (25.4%) had EVA + IP-II. Cases showed a significant improvement in speech perception, auditory and language performance, comparable to controls. Parent-reported auditory and speech production behaviors outcomes were positive among cases and comparable to controls. Pooled gusher proportions in EVA and EVA + IP-II cases were 27.7% (95% CI: 17.6–39.1) and 48.6% (95% CI: 28.6–69.0), respectively, with a proportion difference of 20.9% (95% CI: 11.0–30.1). Gusher occurrence did not impact speech perception or language outcomes.
Conclusion
Outcomes in children with EVA or EVA + IP-II undergoing CI are favorable and largely comparable to outcomes in children with hearing loss undergoing CI without IEMs. Intraoperative gusher is more prevalent among children with EVA + IP-II as compared to iEVA. Gusher does not influence speech perception and language development outcomes.
Level of Evidence
NA Laryngoscope, 2021
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