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IntroductionChildren with early-identified unilateral hearing loss (UHL) might be at risk for delays in early speech and language, functional communication, psychosocial skills, and quality of life (QOL). However, a paucity of relevant research prohibits strong conclusions. This study aimed to provide new evidence relevant to this issue.MethodsParticipants were 34 children, ages 9;0 to 12;7 (years;months), who were identified with UHL via newborn hearing screening. Nineteen children had been fitted with hearing devices, whereas 15 had not. Assessments included measures of speech perception and intelligibility; language and cognition; functional communication; psychosocial abilities; and QOL.Results and discussionAs a group, the children scored significantly below the normative mean and more than one standard deviation below the typical range on speech perception in spatially separated noise, and significantly below the normative mean on written passage comprehension. Outcomes in other aspects appear typical. There was however considerable within participant variation in the children's degree of hearing loss over time, raising the possibility that this pattern of results might change as children get older. The current study also revealed that participants with higher levels of nonverbal ability demonstrated better general language skills and better ability to comprehend written passages. By contrast, neither perception of speech in collocated noise nor fitting with a hearing device accounted for unique variance in outcome measures. Future research should, however, evaluate the fitting of hearing devices using random assignment of participants to groups in order to avoid any confounding influence of degree of hearing loss or children's past/current level of progress.

ObjectivesThe aims of this study were to report the audiological characteristics of children with congenital unilateral hearing loss (UHL), examine the age at which the first reliable behavioural audiograms can be obtained, and investigate hearing changes from diagnosis at birth to the first reliable behavioural audiogram.MethodThis study included a sample of 91 children who were diagnosed with UHL via newborn hearing screening and had reliable behavioural audiograms before 7 years of age. Information about diagnosis, audiological characteristics and etiology were extracted from clinical reports. Regression analysis was used to explore the potential reasons influencing the age at which first reliable behavioural audiograms were obtained. Correlation and ANOVA analyses were conducted to examine changes in hearing at octave frequencies between 0.5 and 4 kHz. The proportions of hearing loss change, as well as the clinical characteristics of children with and without progressive hearing loss, were described according to two adopted definitions: Definition 1: criterion (1): a decrease in 10 dB or greater at two or more adjacent frequencies between 0.5 and 4 kHz, or criterion (2): a decrease in 15 dB or greater at one octave frequency in the same frequency range. Definition 2: a change of ≥20 dB in the average of pure-tone thresholds at 0.5, 1, and 2 kHz.ResultsThe study revealed that 48 children (52.7% of the sample of 91 children) had their first reliable behavioural audiogram by 3 years of age. The mean age at the first reliable behavioural audiogram was 3.0 years (SD 1.4; IQR: 1.8, 4.1). We found a significant association between children's behaviour and the presence or absence of ongoing middle ear issues in relation to the delay in obtaining a reliable behavioural audiogram. When comparing the hearing thresholds at diagnosis with the first reliable behavioural audiogram across different frequencies, it was observed that the majority of children experienced deterioration rather than improvement in the initial impaired ear at each frequency. Notably, there were more instances of hearing changes (either deterioration or improvement), in the 500 Hz and 1,000 Hz frequency ranges compared to the 2,000 Hz and 4,000 Hz ranges. Seventy-eight percent (n = 71) of children had hearing deterioration between the diagnosis and the first behavioural audiogram at one or more frequencies between 0.5 and 4 kHz, with a high proportion of them (52 out of the 71, 73.2%) developing severe to profound hearing loss. When using the averaged three frequency thresholds (i.e., definition 2), only 26.4% of children (n = 24) in the sample were identified as having hearing deterioration. Applying definition 2 therefore underestimates the proportion of children that experienced hearing changes. The study also reported diverse characteristics of children with or without hearing deterioration.ConclusionThe finding that 78% of children diagnosed with UHL at birth had a decrease in hearing loss between the hearing levels at first diagnosis and their first behavioural audiogram highlights the importance of monitoring hearing threshold levels after diagnosis, so that appropriate intervention can be implemented in a timely manner. For clinical management, deterioration of 15 dB at one or more frequencies that does not recover warrants action.

IntroductionEarly identification of mild hearing loss has resulted in early hearing amplification without adequate evidence of effectiveness. This paper describes learnings from a pilot trial, combined with a qualitative study, to highlight the importance of community engagement in designing research studies to determine whether early amplification benefits young children with bilateral mild hearing loss.MethodsPART 1 of the study is a proof-of-concept non-blinded multi-centre randomised controlled trial (RCT) of hearing device fitting vs. no fitting aimed to gather preliminary data and determine its acceptability/feasibility in children

Abstract Some people using hearing aids have difficulty discriminating between sounds even though the sounds are audible. As such, cochlear implants may provide greater benefits for speech perception. One method to identify people with auditory discrimination deficits is to measure discrimination thresholds using spectral ripple noise (SRN). Previous studies have shown that behavioral discrimination of SRN was associated with speech perception, and behavioral discrimination was also related to cortical responses to acoustic change or ACCs. We hypothesized that cortical ACCs could be directly related to speech perception. In this study, we investigated the relationship between subjective speech perception and objective ACC responses measured using SRNs. We tested 13 normal-hearing and 10 hearing-impaired adults using hearing aids. Our results showed that behavioral SRN discrimination was correlated with speech perception in quiet and in noise. Furthermore, cortical ACC responses to phase changes in the SRN were significantly correlated with speech perception. Audibility was a major predictor of discrimination and speech perception, but direct measures of auditory discrimination could contribute information about a listener’s sensitivity to acoustic cues that underpin speech perception. The findings lend support for potential application of measuring ACC responses to SRNs for identifying people who may benefit from cochlear implants.

This study examined the extent to which cognitive ability at 5 years of age predicted language development from 5 to 9 years of age in a population-based sample of children with hearing loss who participated in the Longitudinal Outcomes of Children with Hearing Impairment (LOCHI) study. The developmental outcomes of 81 children with hearing loss were evaluated at 5 and 9 years of age. Hearing loss ranged from mild to severe degrees, and all participants used hearing aids. They all used spoken language as the primary mode of communication and education. Nine-year-old language was assessed using the Clinical Evaluation of Language Fundamentals – 4th edition (CELF-4), the Peabody Picture Vocabulary Test – 4th edition (PPVT-4), and the Expressive Vocabulary Test – 2nd edition (EVT-2). Multiple regression analyses were conducted to examine the extent to which children’s scores on these standardized assessments were predicted by their cognitive ability (non-verbal IQ and verbal working memory) measured at 5 years of age. The influence of early language scores at 5 years and a range of demographic characteristics on language scores at 9 years of age was evaluated. We found that 5-year-old digit span score was a significant predictor of receptive and expressive language, but not receptive or expressive vocabulary, at 9 years of age. Also, 5-year-old non-word repetition test score was a significant predictor of only expressive language and vocabulary, but not receptive language or vocabulary at 9 years of age. After allowing for the effects of non-verbal IQ and 5-year-old receptive vocabulary, early digit span score (but not non-word repetition score) was a significant predictor of expressive and receptive language scores at 9 years of age. The findings shed light on the unique role of early verbal working memory in predicting the development of receptive and expressive language skills and vocabulary skills in children who use hearing aids.

This article reports on the psychosocial development and factors influencing outcomes of 5-year-old children with cochlear implants (CIs) or hearing aids (HAs). It further examines differences between children with CIs and HAs with similar levels of hearing loss. Data were collected as part of the Longitudinal Outcomes of Children with Hearing Impairment study—a prospective, population-based study. Parents/caregivers of children completed the Strengths and Difficulties Questionnaire ( n = 333), the Social Skills subscale from the Child Development Inventory ( n = 317), and questionnaires on functional auditory behavior (Parents’ Evaluation of Aural/oral performance of Children), and demographics. Children completed assessments of nonverbal cognitive ability (Wechsler Non-verbal Scale of Ability) and language (Preschool Language Scale - fourth edition). On average, parent-rated Strengths and Difficulties Questionnaire scores on emotional or behavioral difficulties were within 1 SD of the normative mean; however, Child Development Inventory scores on social skills were more than 1 SD below the norm. Children with severe-to-profound hearing losses using HAs had significantly more behavioral problems than children with CIs. Regression analyses showed that non-verbal cognitive ability, language, and functional auditory behavior were significantly associated with psychosocial outcomes for children with HAs, whereas outcomes for children with CIs were associated with functional auditory behavior and the presence of additional disabilities. Age at hearing intervention, severity of hearing loss, and communication mode were not associated with outcomes. The results suggest that even children who develop good language ability with the help of a HA or CI may have psychosocial problems if they exhibit difficulties with listening and communicating in everyday environments. The findings have implications for developing interventions for young children with hearing loss.

Hearing loss is an etiologically heterogeneous trait with differences in the age of onset, severity and site of lesion. It is caused by a combination of genetic and/or environmental factors. A longitudinal study to examine the efficacy of early intervention for improving child outcomes is ongoing in Australia. To determine the cause of hearing loss in these children we undertook molecular testing of perinatal "Guthrie" blood spots of children whose hearing loss was either detected via newborn hearing screening or detected later in infancy. We analyzed the GJB2 and SLC26A4 genes for the presence of mutations, screened for the mitochondrial DNA (mtDNA) A1555G mutation, and screened for congenital CMV infection in DNA isolated from dried newborn blood spots. Results were obtained from 364 children. We established etiology for 60% of children. One or two known GJB2 mutations were present in 82 children. Twenty-four children had one or two known SLC26A4 mutations. GJB2 or SLC26A4 changes with unknown consequences on hearing were found in 32 children. The A1555G mutation was found in one child, and CMV infection was detected in 28 children. Auditory neuropathy spectrum disorder was confirmed in 26 children whose DNA evaluations were negative. A secondary objective was to investigate the relationship between etiology and audiological outcomes over the first 3 years of life. Regression analysis was used to investigate the relationship between hearing levels and etiology. Data analysis does not support the existence of differential effects of etiology on degree of hearing loss or on progressiveness of hearing loss.