Outcomes and Time to Emergence of Auditory Skills After Cochlear Implantation of Children With Charge Syndrome

Young, Nancy M.; Tournis, Elizabeth; Sandy, Jenelle; Hoff, Stephen R.; Ryan, Maura E.

doi:10.1097/mao.0000000000001488

Cited by 13 publications

(12 citation statements)

References 30 publications

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“…Peters et al (2010) found 56% of surgeons believed additional disability to be a contraindication to bilateral implantation in children. More recent studies neither support nor refute this hypothesis: some show fewer children with additional needs receiving bilateral devices relative to unilateral devices (Eshraghi et al 2015; Young et al 2017; Datta et al 2020) and others show no difference (Nasralla et al 2018; Mesallam et al 2019). Nevertheless, data from the present study suggest children with an additional disability had an increased likelihood of having one CI, which may account for lower daily device use in unilateral users.…”

Section: Discussionmentioning

confidence: 92%

Relationships Between Daily Device Use and Early Communication Outcomes in Young Children With Cochlear Implants

et al. 2021

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Objectives: Children with significant hearing loss can gain access to sound via a cochlear implant (CI), but they must wear the device to reap the communication benefits of the device. That is, poor daily device use may result in underdeveloped perceptual and language skills in children and adolescents using CIs. This retrospective study focuses on the relationship between daily CI use and communication performance (auditory skills, speech recognition, expressive and receptive language) in young children, with the hypothesis that greater daily device use coincides with better communication outcomes. Design: The authors conducted a clinical chart review of patients with CIs younger than 5 years old who used at least 1 CI speech processor with datalogging technology. Participants (n = 65) had a mean chronologic age of 3.5 years, mean implantation age of 1.9 years, and mean device experience of 1.6 years. Approximately one quarter of participants had additional disabilities. Daily device use (i.e., datalogging information), child characteristics (e.g., age at CI), and assessments of communication skills (i.e., parent questionnaires, speech recognition tests, standardized language assessments) were obtained from each child’s records. The investigators performed correlational analyses to examine relationships between communication outcomes and daily device use, and they employed group comparisons and correlations to identify child characteristics that were significantly associated with daily device use (p < 0.05, corrections for family-wise error). Results: Young children with CIs used their device, on average, 6.7 hr/d, with 63% below full-time use (<8 hr/d). Children without additional disabilities who wore their CI more hours per day had significantly better auditory, speech recognition, and language skills. A significant correlation also emerged between daily device use and early auditory skills in young CI users with additional disabilities, though relationships were more complicated for this subsample. Longer daily device use significantly correlated with younger age at CI and longer device experience. Differences in device use occurred in regards to absence versus presence of additional disabilities, bilateral versus unilateral device configuration, sign versus spoken language, and private versus government-assisted insurance. Conclusions: The strong relationship between daily device use and early communication suggests clinicians and parents should focus on increasing the number of hours per day young children wear their CIs to enhance auditory and language outcomes. However, intervention strategies must consider barriers to consistent device use and goals of the family to efficiently and effectively support families of young children with CIs who struggle with inconsistent device use.

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Section: Discussionmentioning

confidence: 92%

Relationships Between Daily Device Use and Early Communication Outcomes in Young Children With Cochlear Implants

et al. 2021

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“…However, emergence of measurable open-set speech perception occurred on average 4.5 years post-CI, far later than usual. Delayed emergence of skills has been reported in implanted children with other complicating conditions (18)(19)(20). In addition, the majority of nonmedically complex children who receive a CI before 3 years of age develop measurable open-set speech perception, varying from 93.5% for those implanted under 1 year of age to 84.7% for those implanted after 2 years (21).…”

Section: Discussionmentioning

confidence: 99%

Cochlear Implantation Can Improve Auditory Skills, Language and Social Engagement of Children With Autism Spectrum Disorder

Jenks

Hoff

Haney

et al. 2021

Otology &Amp; Neurotology

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Objective: To review outcomes of cochlear implantation (CI) in children diagnosed with autism spectrum disorder (ASD). Study Design: Retrospective case review and parent survey. Setting: Tertiary care children's hospital. Patients: Thirty children with ASD who underwent CI between 1991 and 2018. Mean age at CI ¼ 3.5 years (0.8-11.8), mean age at diagnosis of ASD ¼ 5.1 years (2.0-15.0) (22/30 diagnosed after CI), mean follow-up ¼ 10.5 years (1.4-21.6). Parents of 7 children returned a survey. Intervention: Unilateral or bilateral cochlear implantation. Main Outcome Measures: Speech perception; expressive communication mode; educational placement; social engagement; consistency of CI use; parent survey of child behavior change. Results: Thirty-three percent of all and 45% of the 22 consistent device users developed measurable open-set speech perception by an average of 4.5 years of device use.Educational placement at last follow-up included 13% mainstreamed without interpreter, 50% Special Education programs, 10% therapeutic residential or day programs, 23% total communication programs, and one home schooled. Spoken language alone was used by 31% and spoken plus sign by 14%, with the remainder using sign alone, augmentative communication devices or no mode of communication. By parent report, 86% showed improvement in social engagement compared to pre-CI. Survey results showed the behaviors most frequently ranked as most affected by CI were communication and attention, while awareness of environment had the lowest (most affected) mean ranking. Conclusions: Findings support a growing body of literature that cochlear implantation has the potential to improve auditory skills, language, and enhance social engagement in some deaf children with autism spectrum disorder.

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“…Despite the variety of techniques proposed for cochlear implantation in CHARGE syndrome, considerable variability in outcomes exists. One recent study found that after cochlear implantation in 12 children with CHARGE, two saw minimal benefit, four demonstrated improved sound detection, three developed closed set speech perception, and three had open set speech perception . This variability likely reflects the large range of otologic abnormalities that can exist in CHARGE syndrome, such as the degree of cochlear hypoplasia, the diameter of the internal auditory canal to accommodate varying numbers of cochlear neurons, and presence of SGNs.…”

Section: Discussionmentioning

confidence: 99%

Otopathology in CHARGE syndrome

Chen

Nourmahnad

O’Malley

et al. 2020

Laryngoscope Investig Oto

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Postmortem temporal bone computed tomography (CT) and histopathologic findings in an infant with CHARGE syndrome revealed bilateral cochleovestibular hypoplasia, including cochlear pathology relevant to cochlear implant candidacy. Both ears had absence of the superior semicircular canals (SCCs), severely hypoplastic posterior SCCs, and hypoplastic (right ear) or absent (left ear) lateral SCCs seen on CT and histopathology. Histopathology further revealed the absence of all SCC ampullae except the right lateral SCC ampulla and atrophic vestibular neuroepithelium in the saccule and utricle bilaterally. The right cochlea consisted of a basal turn with patent round window, and malformed middle turn (type IV cochlear hypoplasia), with a small internal auditory canal (IAC) but near normal cochlear nerve aperture (fossette). Quantification of spiral ganglion neurons (SGNs) on histologic sections revealed a reduced SGN population (35% of normal for age), but this ear would still have likely achieved benefit from a cochlear implant based on this population. The left cochlea consisted of only a basal turn with patent round window (type III cochlear hypoplasia) with a small IAC and very small cochlear nerve aperture. Notably, histology revealed that there were no SGNs in the cochlea, and therefore, this ear would not have been a good candidate for cochlear implantation. Level of evidence: IV.

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Outcomes and Time to Emergence of Auditory Skills After Cochlear Implantation of Children With Charge Syndrome

Abstract: Auditory skills may develop slowly in children with CHARGE syndrome who receive a CI but most can achieve at least improved detection. In our series, half acquired some speech perception ability. Cochlear nerve deficiency is frequent, but should not be a contraindication to implantation.

Cited by 13 publications

References 30 publications

Relationships Between Daily Device Use and Early Communication Outcomes in Young Children With Cochlear Implants

Relationships Between Daily Device Use and Early Communication Outcomes in Young Children With Cochlear Implants

Cochlear Implantation Can Improve Auditory Skills, Language and Social Engagement of Children With Autism Spectrum Disorder

Otopathology in CHARGE syndrome

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