Cochlear Implantation in Pediatric Patients With Enlarged Vestibular Aqueduct: A Systematic Review

Liliya Benchetrit; Nicolette Jabbour; Swathi Appachi; Yi-Chun Liu; Michael S Cohen; Samantha Anne

doi:10.1002/lary.29742

Cochlear Implantation in Pediatric Patients With Enlarged Vestibular Aqueduct: A Systematic Review

Laryngoscope. 2022 Jul;132(7):1459-1472. doi: 10.1002/lary.29742. Epub 2021 Jul 7.

Authors

Liliya Benchetrit¹, Nicolette Jabbour², Swathi Appachi³, Yi-Chun Liu⁴, Michael S Cohen⁵, Samantha Anne³

Affiliations

¹ Department of Otolaryngology-Head and Neck Surgery, Boston University Medical Center, Boston, Massachusetts, U.S.A.
² Boston University School of Medicine, Boston, Massachusetts, U.S.A.
³ Cleveland Clinic, Head and Neck Institute, Cleveland, Ohio, U.S.A.
⁴ Texas Children's Hospital, Division of Pediatric Otolaryngology, Houston, Texas, U.S.A.
⁵ Department of Otolaryngology-Head and Neck Surgery, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A.

PMID: 34233033
DOI: 10.1002/lary.29742

Abstract

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, 132:1459-1472, 2022.

Keywords: Cochlear implantation; auditory outcomes; enlarged vestibular aqueduct; language development; speech perception.

Publication types

Meta-Analysis
Review
Systematic Review
Research Support, N.I.H., Extramural

MeSH terms

Child
Cochlear Implantation*
Cochlear Implants*
Hearing Loss, Sensorineural* / surgery
Humans
Retrospective Studies
Vestibular Aqueduct* / abnormalities
Vestibular Aqueduct* / surgery

Supplementary concepts

Deafness, Autosomal Recessive 4

Grants and funding

UL1 TR001430/TR/NCATS NIH HHS/United States