Audiologists’ perspectives on management of mild bilateral hearing loss in infants and young children

Ching, Teresa Y. C.; Saetre-Turner, Michelle; Marnane, Vivienne; Scarinci, Nerina; Choik, Chermaine; Tulloch, Kristen; Sung, Valerie

doi:10.1080/14992027.2021.1961170

Cited by 7 publications

(9 citation statements)

References 42 publications

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“…An artefact of newborn screening is the detection of mild hearing loss. As mild hearing-loss is being increasingly diagnosed at an earlier age [28], this presents prognostic and management uncertainty for both families [29] and clinicians [30]. This study provides evidence for a genetic basis for many mild hearing loss cases adding to the emerging body of literature describing genotypes in mild and moderate hearing loss cohorts [31,32].…”

Section: Discussionmentioning

confidence: 74%

The congenital hearing phenotype in GJB2 in Queensland, Australia: V37I and mild hearing loss predominates

Liddle,

Kriukelis,

Gabbett

et al. 2024

Preprint

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Guidelines recommend GJB2 (connexin 26) and GJB6 (connexin 30) testing for bilateral non-syndromic sensorineural hearing loss (SNHL). However, associated audiological phenotypes vary. There is limited Australian data on GJB2 variant frequency and associated phenotypes. Audiograms from a paediatric cohort with SNHL, predominantly identified through newborn hearing screening and carrying GJB2 variants and/or a GJB6 deletion (GJB6-D13S11830) were retrospectively reviewed (n = 127). Two thirds were homozygous or compound heterozygous for pathogenic or likely pathogenic variants of GJB2 and/or GJB6 (n = 80). The most frequent variant, c.109G > A, occurred in homozygous (n = 32), compound heterozygous (n = 8) and heterozygous (n = 5) states. Compared to homozygous/compound heterozygous carriage of other GJB2 variants, c.109G > A positive individuals (homozygous/compound heterozygous) were more likely to have mild HL at their initial (p = 0.00004) and latest audiograms (p = 0.0004). Homozygous/compound heterozygous carriage of c.35delG was associated with moderately-severe or greater HL at both initial (p = 0.007) and latest (p = 0.007) audiograms. The c.101T > C variant presented with milder HL and U-shaped audiograms (p = 0.02). In this agnostically identified cohort, mild HL predominated in GJB2/GJB6 carriers in contrast to previous studies targeting individuals with significant loss. Consequently, c.109G > A, associated with milder phenotypes, was the most frequent. This data provides valuable, balanced prognostic information for preconception, prenatal and paediatric counselling of couples and families carrying these variants.

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Section: Discussionmentioning

confidence: 74%

The congenital hearing phenotype in GJB2 in Queensland, Australia: V37I and mild hearing loss predominates

Liddle,

Kriukelis,

Gabbett

et al. 2024

Preprint

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“…At present, there is no consensus among professionals regarding the clinical need for amplification or intervention in children with MBHL ( Chen et al, 2022 ; Ching et al, 2022 ; Fitzpatrick et al, 2010 , 2016 ; Fitzpatrick, Cologrosso, et al, 2019 ). Given the absence of conclusive evidence and guidelines for audiologists, they encounter challenges in making decisions related to HA fitting and intervention for children with MBHL.…”

Section: Introductionmentioning

confidence: 99%

“…For instance, a study highlighted that pediatric audiologists in diagnostic and rehabilitation settings in Australia navigate their profession by recognizing the limitations of existing evidence, considering various child and family factors, and integrating the perspectives of parents and families. This approach involves adopting a flexible approach to deliver personalized services ( Ching et al, 2022 ).…”

Section: Introductionmentioning

confidence: 99%

Impact of Hearing Aids on Language Outcomes in Preschool Children With Mild Bilateral Hearing Loss

Hung,

Ho,

Chen

et al. 2024

Trends in Hearing

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This study aimed to investigate the role of hearing aid (HA) usage in language outcomes among preschool children aged 3–5 years with mild bilateral hearing loss (MBHL). The data were retrieved from a total of 52 children with MBHL and 30 children with normal hearing (NH). The association between demographical, audiological factors and language outcomes was examined. Analyses of variance were conducted to compare the language abilities of HA users, non-HA users, and their NH peers. Furthermore, regression analyses were performed to identify significant predictors of language outcomes. Aided better ear pure-tone average (BEPTA) was significantly correlated with language comprehension scores. Among children with MBHL, those who used HA outperformed the ones who did not use HA across all linguistic domains. The language skills of children with MBHL were comparable to those of their peers with NH. The degree of improvement in audibility in terms of aided BEPTA was a significant predictor of language comprehension. It is noteworthy that 50% of the parents expressed reluctance regarding HA use for their children with MBHL. The findings highlight the positive impact of HA usage on language development in this population. Professionals may therefore consider HAs as a viable treatment option for children with MBHL, especially when there is a potential risk of language delay due to hearing loss. It was observed that 25% of the children with MBHL had late-onset hearing loss. Consequently, the implementation of preschool screening or a listening performance checklist is recommended to facilitate early detection.

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“…The number of appointments required to reach a diagnosis can be much more than for children with larger degrees of loss ( 16 ). It is likely that this leads to some stress for those involved, particularly families but also professionals ( 16 , 17 ). With limited evidence for the outcomes of early-detected children with these types of loss, clinical management of these children is challenging ( 16 , 17 ).…”

Section: Introductionmentioning

confidence: 99%

“…It is likely that this leads to some stress for those involved, particularly families but also professionals ( 16 , 17 ). With limited evidence for the outcomes of early-detected children with these types of loss, clinical management of these children is challenging ( 16 , 17 ). Clinical practice guidelines reflect the uncertainty in outcomes for children with mild and unilateral hearing loss, with references to individual observations, watchful waiting, behavioral verification of hearing levels and needs-based approaches to the decision of if and when to provide amplification (e.g., King ( 18 ), Fitzpatrick et al ( 19 )).…”

Section: Introductionmentioning

confidence: 99%

Language and health-related quality of life outcomes of children early-detected with unilateral and mild bilateral hearing loss

Carew,

Shepherd,

Smith

et al. 2023

Front. Pediatr.

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IntroductionWe aimed to describe the language and health-related quality of life (HRQoL) outcomes of children early-identified with unilateral or mild bilateral permanent hearing loss. This was a cross-sectional community-based study of children with mild bilateral or unilateral permanent hearing loss (including unilateral auditory neuropathy spectrum disorder (ANSD)), drawn from a population-based databank in Victoria, Australia.MethodsEnrolment in this databank is independent of early intervention and amplification approaches. Language and caregiver-reported HRQoL outcomes are described by type and degree of loss at three timepoints across child development: at age 2 years (n = 255), 5–7 years (n = 173) and 9–12 years (n = 45).ResultsAcross all age groups, average language outcomes were poorer than population normative scores by between a half to two thirds of a standard deviation. Children with mild bilateral hearing loss demonstrated poorer average language outcomes than children with unilateral hearing loss, particularly at younger ages. Children with unilateral ANSD showed language outcomes comparable to their peers with unilateral profound hearing loss. Children had poorer HRQoL psychosocial scores compared to physical scores, without obvious patterns of outcomes linked to degree or type of hearing loss.DiscussionThis study demonstrates children with early-identified unilateral or mild bilateral hearing loss have average language and HRQoL outcomes poorer than population normative expectations from an early age. These outcomes are observed at later ages across childhood. These findings provide a contemporary description of language and quality of life outcomes for children identified but not targeted by universal newborn hearing screening and raise questions of how to provide better support for these populations of children and their families.

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Audiologists’ perspectives on management of mild bilateral hearing loss in infants and young children

Cited by 7 publications

References 42 publications

The congenital hearing phenotype in GJB2 in Queensland, Australia: V37I and mild hearing loss predominates

The congenital hearing phenotype in GJB2 in Queensland, Australia: V37I and mild hearing loss predominates

Impact of Hearing Aids on Language Outcomes in Preschool Children With Mild Bilateral Hearing Loss

Language and health-related quality of life outcomes of children early-detected with unilateral and mild bilateral hearing loss

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