Cochlear implant performance in children deafened by congenital cytomegalovirus—A systematic review

Kraaijenga, Véronique J. C.; Houwelingen, F. Van; Horst, S.F. Van der; Visscher, Jordi; Huisman, Jolien Marieke Lieselot; Hollman, Ernest J.; Stegeman, Inge; Smit, A.L.

doi:10.1111/coa.13142

Cited by 13 publications

(10 citation statements)

References 46 publications

(348 reference statements)

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“…After cochlear implantation, children deafened by congenital cytomegalovirus seem to show improved speech perception equivalent to non-cytomegalovirus congenitally deaf children, though speech production lagged when compared to the control group. It may be attributable to the other comorbidities related to congenital cytomegalovirus (Philips et al, 2014;Yoshida et al, 2017;Kraaijenga et al, 2018).…”

Section: Current Use Of CI In Congenital Hearing Lossmentioning

confidence: 99%

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: Current Use Of CI In Congenital Hearing Lossmentioning

confidence: 99%

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

et al. 2020

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“…38 Overall, they demonstrate that children with cCMV-related hearing loss do benefit from implantation with speech production and perception improving subsequent to implantation, however in general outcomes were not variable. 12,22,38 However, more than half of the studies reviewed (7 of 12) demonstrate poorer performance in children with cCMVrelated deafness. Given that in our study, electrophysiological measures suggest equivalent stimulability of the auditory nerve and brainstem with CI in children with cCMV and GJB2-related SNHL, when poorer outcomes are seen in children with cCMV-related SNHL 38 these may be due in part to delays in diagnosis and rehabilitation, older age of implantation in other studies, and combined cochleovestibular loss, which are more common in children with cCMV than other etiologies of deafness.…”

Section: Precipitous Decline To Profound Deafness In Young Children W...mentioning

confidence: 88%

“…The equivalency of these implant‐evoked electrophysiologic measures between children with cCMV and GJB2‐related SNHL also suggests that, from the perspective of the peripheral auditory system and the brainstem, cochlear implants are an equally effective means of rehabilitation in both clinical groups. A number of studies that look at implant performance in children with cCMV compared with other groups of children with deafness including those with GJB2‐related hearing loss and have undergone a systematic review 38 . Overall, they demonstrate that children with cCMV‐related hearing loss do benefit from implantation with speech production and perception improving subsequent to implantation, however in general outcomes were not variable 12,22,38 .…”

Section: Discussionmentioning

confidence: 99%

“…12,22,38 However, more than half of the studies reviewed (7 of 12) demonstrate poorer performance in children with cCMVrelated deafness. Given that in our study, electrophysiological measures suggest equivalent stimulability of the auditory nerve and brainstem with CI in children with cCMV and GJB2-related SNHL, when poorer outcomes are seen in children with cCMV-related SNHL 38 these may be due in part to delays in diagnosis and rehabilitation, older age of implantation in other studies, and combined cochleovestibular loss, which are more common in children with cCMV than other etiologies of deafness. Although our study only included children with cCMV who were otherwise considered asymptomatic, with the exception of their SNHL, neurological sequelae and additional disabilities are known to frequently occur in the setting of cCMV and may also impact outcome.…”

Section: Precipitous Decline To Profound Deafness In Young Children W...mentioning

confidence: 99%

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Hearing Instability in Children with Congenital Cytomegalovirus: Evidence and Neural Consequences

et al. 2022

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Objective/Hypothesis Sensorineural hearing loss (SNHL) is a common sequela of congenital cytomegalovirus (cCMV), potentially exacerbating neurocognitive delay. The objectives of this study were to assess: (1) age at which SNHL in children with cCMV; (2) stimulability of the auditory system in children with cCMV following cochlear implantation (CI); and (3) whether features of magnetic resonance imaging (MRI) potentially are predictive of hearing outcomes. Methods In this retrospective study of a prospectively acquired cohort, 123 children with cCMV who were referred for hearing loss at a single tertiary referral hospital over 20 years were compared with an unmatched comparative group of 90 children with GJB2‐related deafness. Outcome measures were results of newborn hearing screening (NHS), behavioral audiograms, and, in a subgroup of cochlear implant (CI) users, responses from the auditory nerve and brainstem evoked by CI at initial activation, as well as lesional volume of FLAIR‐hyperintense signal alterations on MRI. Results All but 3 of 123 children with cCMV had confirmed and persistent SNHL. At birth, 113 children with cCMV underwent NHS, 31 (27%) passed in both ears and 23 (20%) passed in one ear (no NHS data in 10 children). At the first audiologic assessment, 32 of 123 (26%) had normal hearing bilaterally; 35 of 123 (28%) had unilateral SNHL; and 57 of 123 (46%) had bilateral SNHL. More than half (67 of 123, 54%) experienced hearing deterioration in at least one ear. Survival analyses suggested that 60% of children who developed SNHL did so by 2.5 years and 80% by 5 years. In the children who passed NHS in one or both ears, 50% developed hearing loss by 3.5 years in the ear, which passed unilaterally (n = 23 ears), and 50% by 5 years in bilateral passes (n = 62 ears). Hearing loss was significant enough in all but one child with isolated high‐frequency loss for rehabilitation to be indicated. Hearing thresholds in individual ears were in the CI range in 83% (102 of 123), although duration of deafness was sufficient to preclude implantation at our center in 13 children with unilateral SNHL. Hearing aids were indicated in 16% (20 of 123). Responses from the auditory nerve and brainstem to initial CI stimulation were similar in children with cCMV‐related SNHL compared with GJB2‐related SNHL. Characteristic white matter changes on MRI were seen in all children with cCMV‐related SNHL (n = 91), but the lesion volume in each cortical hemisphere did not predict degree of SNHL. Conclusions cCMV‐related SNHL is often not detected by NHS but occurs with high prevalence in early childhood. Electrophysiological measures suggest equivalent stimulability of the auditory nerve and brainstem with CI in children with cCMV and GJB2‐related SNHL. Hyperintense white matter lesions on FLAIR MRI are consistently present in children with cCMV‐related SNHL but cannot be used to predict its time course or degree. Combined, the data show early and rapid deterioration of hearing in children with cCMV‐related SNHL with potential for...

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“…Studies examining anti-viral therapy are ongoing and may be beneficial in improving hearing (Bilavsky et al, 2016;Kimberlin et al, 2015;Pasternak et al, 2018). Cochlear implantation is also a very viable option, but outcomes may be limited by other factors, such as cognitive impairment (Kraaijenga et al, 2018).…”

Section: Disclaimer Statementsmentioning

confidence: 99%

Proceedings of the annual conference of the American cochlear implant alliance

Sorkin¹

2019

Cochlear Implants International

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Cochlear implant performance in children deafened by congenital cytomegalovirus—A systematic review

Cited by 13 publications

References 46 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

Hearing Instability in Children with Congenital Cytomegalovirus: Evidence and Neural Consequences

Proceedings of the annual conference of the American cochlear implant alliance

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