A qualitative review of parents' perspectives on the value of CAEP recording in influencing their acceptance of hearing devices for their child.

Objective: To obtain a parental perspective on how audiological tests, including recording cortical auditory-evoked potentials (CAEP) to speech sounds, influenced their uptake of hearing devices for their infant. Design: A focus group was established by parents of hearing-impaired children. A facilitator explored how audiology tests influenced their understanding and management of hearing loss in their child and their acceptance of hearing aids or cochlear implant referral. The views were transcribed and thematic analysis was used to understand key topics. Study samples: Eight sets of parents participated. Their children had been enrolled in an audiology pathway that included CAEP testing. The sample included six children who were aided, one child who was going through the implant assessment and one child who was implanted. Results: Parents reported that it was important for them to understand the test results because this influenced acceptance of hearing aids and cochlear implant assessments. Seven sets of parents had not understood ABR results, while six reported that CAEPs had helped them to understand their child's hearing and need for intervention. Conclusion: Compliance with early hearing aid use and referral for cochlear implant depends upon parents' understanding of their infant's hearing loss by including CAEPs in the audiology pathway.

Role of Cortical Auditory Evoked Potentials in Reducing the Age at Hearing Aid Fitting in Children With Hearing Loss Identified by Newborn Hearing Screening.

Recording of free-field cortical auditory evoked potential (CAEP) responses to speech tokens was introduced into the audiology management for infants with a permanent childhood hearing impairment (PCHI) during 2011-2015 at a U.K. service. Children with bilateral PCHI were studied from two sequential cohorts. Thirty-four children had followed an audiology pathway prior to CAEP introduction, and 44 children followed a pathway after the introduction of CAEP and were tested with unaided and aided CAEP responses. Data analysis explored the age of diagnosis, hearing aid fitting, and referral for cochlear implant (CI) assessment for each of these groups. CAEP offered a novel educative process for the parents and audiologists supporting decision-making for hearing aid fitting and CI referral. Delays in hearing aid fitting and CI referral were categorized as being due to the audiologist's recommendation or parental choice. Results showed that the median age of hearing aid fitting prior to CAEP introduction was 9.2 months. After the inclusion of CAEP recording in the infant pathways, it was 3.9 months. This reduction was attributable to earlier fitting of hearing aids for children with mild and moderate hearing losses, for which the median age fell from 19 to 5 months. Children with profound hearing loss were referred for CI assessment at a significantly earlier age following the introduction of CAEP. Although there has also been a national trend for earlier hearing aid fitting in children, the current study demonstrates that the inclusion of CAEP recording in the pathway facilitated earlier hearing aid fitting for milder impairments.

Predicting the type of hearing loss using click auditory brainstem response in babies referred from newborn hearing screening.

OBJECTIVES: This study examined the ability of click auditory brainstem response (ABR) undertaken below the age of 6 months (from expected date of delivery) to differentiate between conductive and sensorineural hearing loss (SNHL), using the latency of wave V measured 20 dB above threshold. DESIGN: Subjects were recruited if they had an ABR threshold of ≥ 40 dB nHL and ≤ 70 dB nHL in one or both ears measured below the age of 6 months and they had also attended follow-up appointments for behavioral assessment of their hearing in which the type of hearing loss had been confirmed. Forty-five children (84 ears) with SNHL, 82 children (141 ears) with temporary conductive hearing loss (TCHL), and 5 children (10 ears) with permanent conductive hearing loss (PCHL) were recruited. The differences between mean wave V latencies measured 20 dB above ABR threshold were examined using the independent t-test for the groups of cases with SNHL, TCHL, and PCHL. Signal-detection theory was used to examine the relationship between sensitivity and specificity when the latency of wave V 20 dB above threshold was used to identify the presence of SNHL. Receiver operating characteristics were generated and the coordinates of the curve examined for the best compromise between sensitivity and false-alarm rate. The specificity, positive predictive value, and probability of missing a true case were determined for the most promising criteria. RESULTS: There were significant differences between the two groups with SNHL and TCHL. The mean latency of wave V 20 dB above threshold was 1 msec shorter in those with SNHL compared with those with TCHL. There were significant differences between children with PCHL and SNHL but no difference between those with PCHL and TCHL. When a criterion of < 7.6 msec was chosen to predict the presence of SNHL the test sensitivity was 0.98, test specificity 0.71, and positive predictive value was 0.66. Nine out of 10 of those with a latency 20 dB above threshold of < 7.0 msec had an SNHL. CONCLUSIONS: The latency of wave V 20 dB above threshold measured using click ABR is a useful indicator of the type of hearing loss in babies referred from newborn hearing screening.

Predicting the degree of hearing loss using click auditory brainstem response in babies referred from newborn hearing screening.

OBJECTIVES: The predictive ability of the auditory brainstem response (ABR) is an important factor governing the program sensitivity of neonatal hearing screens. The study examined the accuracy of the click-evoked auditory brainstem response (ck-ABR) when undertaken below the age of 6 months (from expected date of delivery) in predicting the pure-tone thresholds subsequently found to be present in children with a congenital permanent childhood hearing impairment. DESIGN: Children with permanent childhood hearing impairment were ascertained from neonatal screening programs that have been subject to longitudinal evaluation. Ninety-two children who had ck-ABR recorded when below 6 months of age and repeatable ear specific pure-tone audiometry were recruited. Those with recognized temporary middle ear effusions at either test were excluded. The relationship between ABR and pure-tone thresholds was tested using the Pearson correlation coefficient with a linear regression model used to estimate pure-tone threshold (dependent variable) from ABR (independent variable). Correlation coefficients were obtained for pure-tone frequencies at octave intervals between 0.25 kHz and 4 kHz and at various frequency combinations. The difference between ABR and pure-tone threshold was analyzed. Those with a difference of greater than 20 dB were further examined. The ABR and pure-tone differences were also compared in babies born at term and prematurely. RESULTS: Of the 92 children recruited to the study two children had a confirmed auditory neuropathy spectrum disorder (2%) and 10 (11%) had an audiometrically confirmed progressive hearing impairment. When these children were excluded, there was a high linear positive correlation (r = 0.90, SE = 14.3 dB) between the ABR and pure-tone thresholds averaged at 2 to 4 kHz. Although the correlation varied for different audiometric configurations, in all cases with a sloping hearing loss the correlation with their best frequency was weaker than the correlation at 2 to 4 kHz. For the total cohort the mean difference between ABR and pure-tone thresholds averaged at 2 to 4 kHz was 4.4 dB (SD = 19.29). The modal difference was 0 dB (58%) and 76 % had a difference of 20 dB or less. ABR underestimated the subsequently recorded pure-tone thresholds by more than 20 dB in 11 children and 10 of these children showed progression of their hearing loss measured by serial pure-tone audiometry. ABR overestimated the pure-tone thresholds by more than 20 dB in 15 children. Nine of these children (60%) had suffered a perinatal illness and the mean difference between the pure-tone and ABR thresholds was significantly greater in those born at <35 weeks of gestation (p < 0.001). CONCLUSIONS: There is a high positive linear correlation between ck-ABR and pure-tone average thresholds at 2 to 4 kHz. However, the predictive value of ABR is reduced in certain neonatal groups. The cause for this is discussed as are the implications for undertaking a test battery at this age to improve the predictive accuracy.

The longitudinal follow up of a universal neonatal hearing screen: the implications for confirming deafness in childhood.

OBJECTIVE: To examine the implications of universal neonatal screening for confirming deafness in early childhood. DESIGN: A cohort that had received a universal neonatal hearing screen was followed-up until school entry, and confirmation of deafness compared with that from parallel cohorts that had not received the screen. STUDY: Three 10-year cohorts were compared: a cohort of 35,668 enrolled for universal neonatal hearing screening, a historical cohort of 31,538 when neonatal screening was unavailable, and a cohort of 32,890 when targeted screening was in place. RESULTS: Prevalence of deafness by school age was 3.65/1000 with a neonatal yield of 1.79/1000. The screen had been 89% sensitive to moderate and worse deafness, 79% to unilateral deafness, and 49% to mild impairments. The median age of confirmation had been reduced from 218 to 16 weeks, but for those not identified neonatally there had been no significant improvement in the age of confirmation over the previous 25 years. The yield from post-neonatal screens had been reduced from 1.8/1000 to 0.2/1000, and with a yield of 0.8/1000, reactive referral remained a more effective route to identification. CONCLUSIONS: Even with UNHS in place post-neonatal routes to identification need to be maintained and improvements investigated.

Postneonatal care pathways and the identification of deafness.

BACKGROUND: The need to maintain clinical pathways for the postneonatal identification of deafness was investigated following the successful implementation of universal newborn hearing screening. METHODS: Identification routes were compared for 378 hearing impaired children ascertained from three 10-year cohorts: one that had not received neonatal screening, one that had received targeted neonatal screening and one that was universally screened. The presence of risk factors was determined in those with a hearing impairment. RESULTS: The cohort comparison confirmed significant changes in the yield from all identification routes apart from referrals reactive to parental or professional concerns. In the cohort universally screened, this reactive yield was 1.23/1000 with 51% having risk factors for deafness. Universal newborn hearing screening did not reduce this postneonatal yield. CONCLUSIONS: Despite the successful implementation of newborn hearing screening, clinical pathways with access to audiological assessment need to be maintained within a universal Healthy Child Programme.

The relationship between language development and behaviour problems in children with hearing loss.

BACKGROUND: There are well-replicated findings that link poor development on a range of communication skills with increased behavioural problems. This paper examines this relationship in children with hearing loss. METHOD: One hundred and twenty children with hearing loss (67 boys, 53 girls) and 63 hearing children (37 boys, 26 girls) with a mean age of 8 years from eight districts in Southern England were assessed for receptive and expressive language skills. The relationships between these measures and an aggregate of parent- and teacher-reported behaviour problems in the children were investigated. RESULTS: Children with hearing loss had higher levels of behaviour problems compared to hearing children. Once the language abilities of children with hearing loss are taken into account, the negative effects of hearing loss on behaviour disappear. CONCLUSIONS: Behaviour problems are found more commonly in children with hearing loss and the level of behaviour problems is highest amongst those children with hearing loss with the least developed language capabilities.

Health status and health-related quality of life preference-based outcomes of children who are aged 7 to 9 years and have bilateral permanent childhood hearing impairment.

OBJECTIVE: The objective of this study was to describe the health status and health-related quality of life preference-based outcomes of children with diagnosed bilateral permanent childhood hearing impairment and a comparison group of English-speaking children with normal hearing. METHODS: We studied 120 children who were aged 7 to 9 years and had bilateral permanent childhood hearing impairment of moderate or greater severity, identified from a cohort of 156,733 children who were born in 8 districts of southern England, and 63 English-speaking children with normal hearing and the same place of birth and age at assessment. Principal caregivers were interviewed by using the Health Utilities Index Mark III questionnaire for proxy-assessed usual health status assessment. Levels of function within each of the 8 attributes of the Health Utilities Index Mark III (cognition, vision, hearing, speech, ambulation, dexterity, emotion, and pain) were recorded. RESULTS: Bilateral permanent childhood hearing impairment is associated with significantly increased proportions of suboptimal levels of function and significantly lower single-attribute utility scores in 6 of the 8 attributes of the Health Utilities Index Mark III: vision, hearing, speech, ambulation, dexterity, and cognition. Compared with the children with normal hearing, the mean multiattribute utility score for the children with hearing impairment was significantly lower for both the whole group and the moderate, severe, and profound severity subgroups. The differences in the distributions of the multiattribute utility scores between the children with hearing impairment as a group and the children with normal hearing and between each of the severity subgroups and the children with normal hearing all were statistically significant. CONCLUSIONS: This study provides rigorous evidence of an association between bilateral permanent childhood hearing impairment and diminished health status and health-related quality of life preference-based outcomes during midchildhood.

Language ability in children with permanent hearing impairment: the influence of early management and family participation.

OBJECTIVE: The goal was to examine the relationships between management after confirmation, family participation, and speech and language outcomes in the same group of children with permanent childhood hearing impairment. METHODS: Speech, oral language, and nonverbal abilities, expressed as z scores and adjusted in a regression model, and Family Participation Rating Scale scores were assessed at a mean age of 7.9 years for 120 children with bilateral permanent childhood hearing impairment from a 1992-1997 United Kingdom birth cohort. Ages at institution of management and hearing aid fitting were obtained retrospectively from case notes. RESULTS: Compared with children managed later (> 9 months), those managed early (< or = 9 months) had higher adjusted mean z scores for both receptive and expressive language, relative to nonverbal ability, but not for speech. Compared with children aided later, a smaller group of more-impaired children aided early did not have significantly higher scores for these outcomes. Family Participation Rating Scale scores showed significant positive correlations with language and speech intelligibility scores only for those with confirmation after 9 months and were highest for those with late confirmed, severe/profound, permanent childhood hearing impairment. CONCLUSIONS: Early management of permanent childhood hearing impairment results in improved language. Family participation is also an important factor in cases that are confirmed late, especially for children with severe or profound permanent childhood hearing impairment.

Language ability after early detection of permanent childhood hearing impairment.

BACKGROUND: Children with bilateral permanent hearing impairment often have impaired language and speech abilities. However, the effects of universal newborn screening for permanent bilateral childhood hearing impairment and the effects of confirmation of hearing impairment by nine months of age on subsequent verbal abilities are uncertain. METHODS: We studied 120 children with bilateral permanent hearing impairment identified from a large birth cohort in southern England, at a mean of 7.9 years of age. Of the 120 children, 61 were born during periods with universal newborn screening and 57 had hearing impairment that was confirmed by nine months of age. The primary outcomes were language as compared with nonverbal ability and speech expressed as z scores (the number of standard deviations by which the score differed from the mean score among 63 age-matched children with normal hearing), adjusted for the severity of the hearing impairment and for maternal education. RESULTS: Confirmation of hearing impairment by nine months of age was associated with higher adjusted mean z scores for language as compared with nonverbal ability (adjusted mean difference for receptive language, 0.82; 95 percent confidence interval, 0.31 to 1.33; and adjusted mean difference for expressive language, 0.70; 95 percent confidence interval, 0.13 to 1.26). Birth during periods with universal newborn screening was also associated with higher adjusted z scores for receptive language as compared with nonverbal ability (adjusted mean difference, 0.60; 95 percent confidence interval, 0.07 to 1.13), although the z scores for expressive language as compared with nonverbal ability were not significantly higher. Speech scores did not differ significantly between those who were exposed to newborn screening or early confirmation and those who were not. CONCLUSIONS: Early detection of childhood hearing impairment was associated with higher scores for language but not for speech in midchildhood.

The economic costs of congenital bilateral permanent childhood hearing impairment.

OBJECTIVE: The objective of this study was to estimate the economic costs of bilateral permanent childhood hearing impairment (PCHI) in the preceding year of life for children aged 7 to 9 years. METHODS: A cost analysis was conducted by using a birth cohort of children born between 1992 and 1997 in 8 districts of Southern England, of which half had been born into populations exposed to universal newborn screening (UNS). Unit costs were applied to estimates of health, social, and broader resource use made by 120 hearing-impaired children and 63 children in a normally hearing comparison group. Associations between societal costs per child and severity of hearing impairment, language ability score, exposure to UNS, and age of confirmation were analyzed, including adjustment for potential confounders in a linear regression model. RESULTS: The mean societal cost in the preceding year of life at 7 to 9 years of age was 14092.5 pound sterling for children with PCHI, compared with 4206.8 pound sterling for the normally hearing children, a cost difference of 9885.7 pound sterling. After adjusting for severity and other potential confounders in a linear regression model, mean societal costs among children with PCHI were reduced by 2553 pound sterling for each unit increase in the z score for receptive language. Using similar regression models, exposure to a program of UNS was associated with a smaller cost reduction of 2213.2 pound sterling, whereas costs were similar between children whose PCHI was confirmed at <9 or >9 months. CONCLUSIONS. The study provides rigorous evidence of the annual health, social, and broader societal cost of bilateral PCHI in the preceding year of life at 7 to 9 years of age and shows that it is related to its severity and has an inverse relationship with language abilities after adjustment for severity.