Systematic review of cochlear implantation in CHARGE syndrome

Amin, Nikul; Sethukumar, Priya; Pai, Irumee; Rajput, Kaukab; Nash, Robert

doi:10.1080/14670100.2019.1634857

Cited by 10 publications

(16 citation statements)

References 59 publications

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“…Regarding hearing loss specifically, nearly all patients have ear malformations, which lead to a mixed conductive and sensory neural hearing loss. About 40% of CHARGE patients have profound hearing loss, while 80% have a less severe hearing impairment (Amin et al, 2019). Overall, there seems to be an improvement in audiological outcomes post CI, however, they were inferior to outcomes of patients without CHARGE syndrome.…”

Section: Sgnmentioning

confidence: 90%

“…Two patients did not benefit from CI and underwent auditory brainstem implantation (Song et al, 2011;Eppsteiner et al, 2012). Recent systematic review indicates that CI is beneficial for patients with CHARGE syndrome (Amin et al, 2019) CI is strongly recommended in favorable cases, auditory brainstem implantation may be an alternative for patients with CHARGE syndrome who fail to benefit from CI (Song et al, 2011).…”

Section: Chd7mentioning

confidence: 99%

“…Overall, there seems to be an improvement in audiological outcomes post CI, however, they were inferior to outcomes of patients without CHARGE syndrome. The consensus is that CI is strongly recommended in favorable cases, i.e., those with large cochleovestibular nerve diameter and absence of severe mental retardation (Lanson et al, 2007;Song et al, 2011;Ricci et al, 2014;Amin et al, 2019). In unsuccessful CI cases, auditory brain stem implantation was suggested as an alternative (Song et al, 2011;Eppsteiner et al, 2012).…”

Section: Sgnmentioning

confidence: 99%

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Genotype-Phenotype Correlation for Predicting Cochlear Implant Outcome: Current Challenges and Opportunities

et al. 2020

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The use and utility of cochlear implantation has rapidly increased in recent years as technological advances in the field have expanded both the efficacy and eligible patient population for implantation. This review aims to serve as a general overview of the most common hearing disorders that have favorable auditory outcomes with cochlear implants (CI). Hearing loss in children caused by congenital cytomegalovirus infection, syndromic conditions including Pendred Syndrome, and non-syndromic genetic conditions such as hearing impairment associated with GJB2 mutations have shown to be successfully managed by CI. Furthermore, cochlear implantation provides the auditory rehabilitation for the most common etiology of hearing loss in adults and age-related hearing loss (ARHL) or presbycusis. However, in some cases, cochlear implantation have been associated with some challenges. Regarding implantation in children, studies have shown that sometimes parents seem to have unrealistic expectations regarding the ability of CI to provide auditory rehabilitation and speech improvement. Given the evidence revealing the beneficial effects of early intervention via CI in individuals with hearing disorders especially hearing loss due to genetic etiology, early auditory and genetic screening efforts may yield better clinical outcomes. There is a need to better understand genotype-phenotype correlations and CI outcome, so that effective genetic counseling and successful treatment strategies can be developed at the appropriate time for hearing impaired individuals.

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Section: Sgnmentioning

confidence: 90%

Section: Chd7mentioning

confidence: 99%

Section: Sgnmentioning

confidence: 99%

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Genotype-Phenotype Correlation for Predicting Cochlear Implant Outcome: Current Challenges and Opportunities

et al. 2020

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“…Six children in this cohort underwent bilateral cochlear implantation while no patients in a 2007 study were implanted bilaterally. In a systematic review of 156 children with CHARGE syndrome who underwent 165 implants, there were only four cases of simultaneous bilateral implantation, as well as five cases of bilateral sequential implants (20), suggesting that bilateral implantation in CHARGE patients is not common practice. Bilateral implantation has the potential to provide some benefits of binaural hearing, but also allows for certain assurances, for example in the case of device failure.…”

Section: Discussionmentioning

confidence: 99%

Cochlear Implant Outcomes in CHARGE Syndrome: Updated Perspectives

Kay‐Rivest

McMenomey²,

Jethanamest³

et al. 2022

Otology &Amp; Neurotology

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ObjectiveTo evaluate outcomes of auditory implants in children with CHARGE syndrome and describe the evolution in management of hearing loss in this complex population.MethodsA retrospective case review was performed at a tertiary referral center. Children with CHARGE syndrome who received either a cochlear implant (CI) or auditory brainstem implant (ABI) were included. Clinical records, demographic information, CHARGE features, neuroimaging, audiology, hearing rehabilitation interventions, operative notes, and outcomes were reviewed.ResultsThirteen children with CHARGE syndrome underwent a total of 19 cochlear implants between 2008 and 2020. Among the congenitally deafened children (n = 9), six underwent bilateral implantation (five simultaneous and one sequential). Bilateral implantation was performed even in the presence of diminutive-appearing cochlear nerves. The average age of implantation was 1.1 years, and the mean device use time was 9.4 hours per day. Patients showed improvements in subjective family assessment related to hearing. In this group, two patients use oral communication, five use total communication, and two use sign language exclusively. Among the children with progressive hearing loss, the mean age of hearing deterioration was 4.4 years of age, and the device use time on average was 9.8 hours per day. The highest performer in the cohort was a child who lost hearing in their only hearing ear at age 4 and had normal cochleovestibular anatomy on that side. One child received an auditory brainstem implant at age two after deriving no benefit from a CI and can detect environmental sounds but is currently a nonuser. Over time, we noted that implantation occurred earlier in life and that practice has shifted toward bilateral implantation.ConclusionsCompared to a previous institutional cohort, children evaluated in this study were often implanted at a younger age and bilaterally with significantly improved outcomes. A CI evaluation should be considered in children with CHARGE syndrome to maximize sensory input and auditory ability.

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“…Patients are less likely to benefit, but meaningful hearing can be achieved in selected cases [ 3 , 4 ]. In children with syndromic disorders such as CHARGE (congenital features of coloboma of the eye, heart defect, atresia of the nasal choanae, retardation of growth and/or development, genital and/or urinary abnormalities, and ear abnormalities and deafness), CI implant surgery is very challenging due to abnormal anatomy and comorbidity [ 5 , 6 ]. Disabilities including developmental delay, cerebral palsy, visual impairment, autism, and attention deficit disorder significantly affect the outcomes [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

Pre- and post-operative imaging of cochlear implants: a pictorial review

et al. 2020

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Cochlear implants are increasingly used to treat sensorineural hearing disorders in both children and adults. Pre-operative computed tomography and magnetic resonance imaging play a pivotal role in patient selection, to rule out findings that preclude surgery or identify conditions which may have an impact on the surgical procedure. The post-operative position of the electrode array within the cochlea can be reliably identified using cone-beam computed tomography. Recognition of scalar dislocation, cochlear dislocation, electrode fold, and malposition of the electrode array may have important consequences for the patient such as revision surgery or adapted fitting.

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Systematic review of cochlear implantation in CHARGE syndrome

Cited by 10 publications

References 59 publications

Genotype-Phenotype Correlation for Predicting Cochlear Implant Outcome: Current Challenges and Opportunities

Genotype-Phenotype Correlation for Predicting Cochlear Implant Outcome: Current Challenges and Opportunities

Cochlear Implant Outcomes in CHARGE Syndrome: Updated Perspectives

Pre- and post-operative imaging of cochlear implants: a pictorial review

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