Impact of cochlear abnormalities on hearing outcomes for children with cochlear implants

Ronner, Evette A.; Basonbul, Razan A.; Bhakta, Rupal T.; Mankarious, Leila A.; Lee, Dong Hoon; Cohen, Michael S.

doi:10.1016/j.amjoto.2019.102372

Cited by 5 publications

(5 citation statements)

References 12 publications

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“…20,73,74 It can be theorized that patients with anomalies met criteria for implantation at later average time due to the progression of HL especially in the setting of EVA in comparison with control groups with genetic or congenital derived HL. This phenomena of the older age of CI in children who have anomalies resulting in poorer outcomes have been shown in a previous study carried out by Ronner et al 75 Despite this finding, our review did not reveal a significant difference between patients with and without these anomalies.…”

Section: Discussioncontrasting

confidence: 49%

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

et al. 2021

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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.

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Section: Discussioncontrasting

confidence: 49%

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

et al. 2021

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“…Studies suggest that patients with IEMs take longer to develop speech perception skills than those without IEMs [14,15,[17][18][19]. However, as time of post-implantation progresses, patients with IEMs show no statistically significant difference in speech perception on openset tests compared to children with normal cochlear anatomy.…”

Section: Speech Perception Resultsmentioning

confidence: 99%

“…Ronner et al discussed the preoperative and postoperative early speech perception word intelligibility scores for cochlear implantation patients. Patients were stratified into normal anatomy, low-risk anatomy, and high-risk anatomy (Table 4) [18].…”

Section: Closed-set Testingmentioning

confidence: 99%

Systematic review of cochlear implantation in patients with inner ear malformations

Shah

Walters²,

Langlie³

et al. 2022

PLoS ONE

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Objectives To evaluate the outcomes of cochlear implantation in patients with severe to profound sensorineural hearing loss due to inner ear malformations (IEMs) when compared to patients without IEMs. We discussed audiological outcomes such as open-set testing, closed-set testing, CAP score, and SIR score as well as postoperative outcomes such as cerebrospinal fluid gusher and incomplete insertion rate associated with cochlear implantation in individuals with IEMs. Data sources PubMed, Science Direct, Web of Science, Scopus, and EMBASE databases. Review methods After screening a total of 222 studies, twelve eligible original articles were included in the review to analyze the speech and hearing outcomes of implanted patients with IEMs. Five reviewers independently screened, selected, and extracted data. The “Tool to Assess Risk of Bias in Cohort Studies” published by the CLARITY group was used to perform quality assessment on eligible studies. Systematic review registration number: CRD42021237489. Results IEMs are more likely to be associated with abnormal position of the facial nerve, raising the risk of intraoperative complications. These patients may benefit from cochlear implantation, but audiological outcomes may also be less favorable than in individuals without IEMs. Furthermore, due to the risk of cerebrospinal fluid gusher, incomplete insertion of electrodes, and postoperative facial nerve stimulation, surgeons can employ precautionary measures such as preoperative imaging and proper counseling. Postoperative imaging is suggested to be beneficial in ensuring proper electrode placement. Conclusions Cochlear implants (CIs) have the potential to provide auditory rehabilitation to individuals with IEMs. Precise classification of the malformation, preoperative imaging and anatomical mapping, appropriate electrode selection, intra-operative techniques, and postoperative imaging are recommended in this population.

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“…There is a theory that patients with inner ear malformations, especially with EVA, may meet the criteria for implantation later (compared to controls with congenital HL) due to the progression of HL [54]. Due to the instability of patients with fluctuating HL, some parents hesitate to proceed with surgery when spontaneous improvement is possible.…”

Section: Discussionmentioning

confidence: 99%

Cochlear implantation in children with enlarged vestibular aqueduct (EVA): relationship to Pendred syndrome diagnosis, surgical outcomes, and radiological findings

Remjasz-Jurek¹,

Clarós²,

Clarós-Pujol³

et al. 2023

J Hear Sci

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IntroductionThe cochlear implant (CI) procedure in patients with inner ear malformations is challenging. The aim of this study was to evaluate auditory perception and speech development in children with enlarged vestibular aqueduct (EVA) and to relate the results to the diagnosis of Pendred syndrome (PS), imaging findings, surgical course, and postoperative period.Material and methodsThe study group consisted of 49 children with EVA, aged 11 months to 15 years, with severe to profound hearing loss. The EVA patients included 22 with PS and 27 with nonsyndromic EVA (NSEVA). The control group consisted of 46 children with nonsyndromic deafness. Outcomes after cochlear implantation were evaluated annually for at least 10 years. Auditory performance was assessed by categories of auditory performance (CAP) and Meaningful Auditory Integration Scale (MAIS). Speech outcomes were evaluated by Speech Intelligibility Rating Scale (SIR) and Meaning Use of Speech Scale (MUSS). Genetic counselling, imaging studies, and vestibular testing were also evaluated when available.ResultsAll patients included in the study benefited from cochlear implants, especially when implantation was performed before the age of 3 years. After CI, EVA patients (PS and NSEVA) achieved a steeper rate of increase in auditory perception and speech intelligibility, demonstrating higher scores at each follow-up point compared to nonsyndromic patients (NS). There were no differences in auditory and speech perception between NSEVA and PS patients. In addition to EVA, the most commonly diagnosed malformation was incomplete partition type 2 (IP-2), the presence of which negatively affected postoperative outcomes. During cochleostomy, cerebrospinal fluid (CSF)/perilymph leakage was observed in 50% of implanted ears, but its presence did not affect the final outcomes.ConclusionsEarly cochlear implantation is associated with satisfactory speech and auditory development in children with EVA. Due to the presence of inner ear malformations in patients with Pendred syndrome, detailed imaging of the temporal bone is indicated. Despite the frequent occurrence of CSF/perilymph leakage during cochleostomy, patients with EVA benefit satisfactorily from cochlear implantation.

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Impact of cochlear abnormalities on hearing outcomes for children with cochlear implants

Cited by 5 publications

References 12 publications

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

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

Systematic review of cochlear implantation in patients with inner ear malformations

Cochlear implantation in children with enlarged vestibular aqueduct (EVA): relationship to Pendred syndrome diagnosis, surgical outcomes, and radiological findings

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