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Editor's Note: The following article discusses the timely topic Clinical Guidance in the areas of Evidence-Based Early Hearing Detection and Intervention Programs. This article aims to discuss areas of services needed, guidance to countries/organizations attempting to initiate early hearing detection and intervention systems. Expert consensus and systematic/scoping reviews were combined to produce recommendations for evidence-based clinical practice. In Ear and Hearing, our long-term goal for the Point of View article is to stimulate the field's interest in and to enhance the appreciation of the author's area of expertise. Hearing is an important sense for children to develop cognitive, speech, language, and psychosocial skills. The goal of universal newborn hearing screening is to enable the detection of hearing loss in infants so that timely health and educational/therapeutic intervention can be provided as early as possible to improve outcomes. While many countries have implemented universal newborn hearing screening programs, many others are yet to start. As hearing screening is only the first step to identify children with hearing loss, many follow-up services are needed to help them thrive. However, not all of these services are universally available, even in high-income countries. The purposes of this article are (1) to discuss the areas of services needed in an integrated care system to support children with hearing loss and their families; (2) to provide guidance to countries/organizations attempting to initiate early hearing detection and intervention systems with the goal of meeting measurable benchmarks to assure quality; and (3) to help established programs expand and improve their services to support children with hearing loss to develop their full potential. Multiple databases were interrogated including PubMed, Medline (OVIDSP), Cochrane library, Google Scholar, Web of Science and One Search, ERIC, PsychInfo. Expert consensus and systematic/scoping reviews were combined to produce recommendations for evidence-based clinical practice. Eight essential areas were identified to be central to the integrated care: (1) hearing screening, (2) audiologic diagnosis and management, (3) amplification, (4) medical evaluation and management, (5) early intervention services, (6) family-to-family support, (7) D/deaf/hard of hearing leadership, and (8) data management. Checklists are provided to support the assessment of a country/organization's readiness and development in each area as well as to suggest alternative strategies for situations with limited resources. A three-tiered system (i.e., Basic, Intermediate, and Advanced) is proposed to help countries/organizations at all resource levels assess their readiness to provide the needed services and to improve their integrated care system. Future directions and policy implications are also discussed., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 The Authors. Ear & Hearing is published on behalf of the American Auditory Society, by Wolters Kluwer Health, Inc.)

Objectives: The purpose of this study was to (1) develop a Language-independent Test of Auditory Discrimination (LIT-AD) between speech sounds so that people with hearing loss who derive limited speech perception benefits from hearing aids (HAs) may be identified for consideration of cochlear implantation and (2) examine the relationship between the scores for the new discrimination test and those of a standard sentence test for adults wearing either HAs or cochlear implants (CIs)., Design: The test measures the ability of the listener to correctly discriminate pairs of nonsense syllables, presented as sequential triplets in an odd-one-out format, implemented as a game-based software tool for self-administration using a tablet computer. Stage 1 included first a review of phonemic inventories in the 40 most common languages in the world to select the consonants and vowels. Second, discrimination testing of 50 users of CIs at several signal to noise ratios (SNRs) was carried out to generate psychometric functions. These were used to calculate the corrections in SNR for each consonant-pair and vowel combination required to equalize difficulty across items. Third, all items were individually equalized in difficulty and the overall difficulty set. Stage 2 involved the validation of the LIT-AD in English-speaking listeners by comparing discrimination scores with performance in a standard sentence test. Forty-one users of HAs and 40 users of CIs were assessed. Correlation analyses were conducted to examine test-retest reliability and the relationship between performance in the two tests. Multiple regression analyses were used to examine the relationship between demographic characteristics and performance in the LIT-AD. The scores of the CI users were used to estimate the probability of superior performance with CIs for a non-CI user having a given LIT-AD score and duration of hearing loss., Results: The LIT-AD comprises 81 pairs of vowel-consonant-vowel syllables that were equalized in difficulty to discriminate. The test can be self-administered on a tablet computer, and it takes about 10 min to complete. The software automatically scores the responses and gives an overall score and a list of confusable items as output. There was good test-retest reliability. On average, higher LIT-AD discrimination scores were associated with better sentence perception for users of HAs (r = -0.54, p <0.001) and users of CIs (r = -0.73, p <0.001). The probability of superior performance with CIs for a certain LIT-AD score was estimated, after allowing for the effect of duration of hearing loss., Conclusions: The LIT-AD could increase access to CIs by screening for those who obtain limited benefits from HAs to facilitate timely referrals for CI candidacy evaluation. The test results can be used to provide patients and professionals with practical information about the probability of potential benefits for speech perception from cochlear implantation. The test will need to be evaluated for speakers of languages other than English to facilitate adoption in different countries., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 The Authors. Ear & Hearing is published on behalf of the American Auditory Society, by Wolters Kluwer Health, Inc.)

Objectives: Hearing loss is one of the most prevalent congenital disorders among children. Many countries have implemented universal newborn hearing screening (UNHS) for the early diagnosis and treatment of hearing loss. Despite widespread implementation, the value for money of UNHS is unclear due to lack of cost and outcomes data from rigorous study designs. The objective of this research is to conduct a within-study cost-effectiveness analysis of UNHS compared with targeted screening (targeting children with risk factors of hearing loss) from the Australian healthcare system perspective. This evaluation is the first economic evaluation to assess the cost-effectiveness of UNHS compared to targeted screening using real-world data from a natural experiment., Design: The evaluation assumed the Australian healthcare system perspective and considered a time horizon of 5 years. Utilities were estimated using responses to the Health Utilities Index Mark III. Screening costs were estimated based on the Victorian Infant Hearing Screening Program. Ongoing costs were estimated based on administrative data, while external data sources were used to estimate costs related to hearing services. Missing data were handled using the multiple imputation method. Outcome measures included quality-adjusted life years (QALYs) and four language and communication-related outcomes: Peabody Picture Vocabulary Test, Wechsler Nonverbal Scale of Ability, Progressive Achievement Test, and comprehensive, expressive, and total language scores based on the Preschool Language Scale., Results: On average, the UNHS cost an extra Australian dollar (A$)22,000 per diagnosed child and was associated with 0.45 more QALYs per diagnosed child compared with targeted screening to 5 years, resulting in an incremental cost-effectiveness ratio (ICER) of A$48,000 per QALY gained. The ICERs for language outcomes lay between A$3,900 (for expressive language score) and A$83,500 per one-point improvement in language score (for Wechsler Nonverbal Scale of Ability). UNHS had a 69% probability of being more cost-effective compared to targeted screening at a willingness to pay threshold of A$60,000 per QALY gained. ICERs were most sensitive to the screening costs., Conclusions: The evaluation demonstrated the usefulness of a within-study economic evaluation to understand the value for money of the UNHS program in the Australian context. Findings from this evaluation suggested that screening costs were the key driver of cost-effectiveness results. Most outcomes were not significantly different between UNHS and targeted screening groups. The ICER may be overestimated due to the short follow-up period. Further research is warranted to include long-term resource use and outcome data, late diagnosis, transition and remission between severity levels, and timing of diagnosis and treatment., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To address the question of whether, on a population level, early detection and amplification improve outcomes of children with hearing impairment., Design: All families of children who were born between 2002 and 2007, and who presented for hearing services below 3 years of age at Australian Hearing pediatric centers in New South Wales, Victoria, and Southern Queensland were invited to participate in a prospective study on outcomes. Children's speech, language, functional, and social outcomes were assessed at 3 years of age, using a battery of age-appropriate tests. Demographic information relating to the child, family, and educational intervention was solicited through the use of custom-designed questionnaires. Audiological data were collected from the national database of Australian Hearing and records held at educational intervention agencies for children. Regression analysis was used to investigate the effects of each of 15 predictor variables, including age of amplification, on outcomes., Results: Four hundred and fifty-one children enrolled in the study, 56% of whom received their first hearing aid fitting before 6 months of age. On the basis of clinical records, 44 children (10%) were diagnosed with auditory neuropathy spectrum disorder. There were 107 children (24%) reported to have additional disabilities. At 3 years of age, 317 children (70%) were hearing aid users and 134 children (30%) used cochlear implants. On the basis of parent reports, about 71% used an aural/oral mode of communication, and about 79% used English as the spoken language at home. Children's performance scores on standardized tests administered at 3 years of age were used in a factor analysis to derive a global development factor score. On average, the global score of hearing-impaired children was more than 1 SD below the mean of normal-hearing children at the same age. Regression analysis revealed that five factors, including female gender, absence of additional disabilities, less severe hearing loss, higher maternal education, and (for children with cochlear implants) earlier age of switch-on were associated with better outcomes at the 5% significance level. Whereas the effect of age of hearing aid fitting on child outcomes was weak, a younger age at cochlear implant switch-on was significantly associated with better outcomes for children with cochlear implants at 3 years of age., Conclusions: Fifty-six percent of the 451 children were fitted with hearing aids before 6 months of age. At 3 years of age, 134 children used cochlear implants and the remaining children used hearing aids. On average, outcomes were well below population norms. Significant predictors of child outcomes include: presence/absence of additional disabilities, severity of hearing loss, gender, maternal education, together with age of switch-on for children with cochlear implants.

Objective: The purpose of this study was to characterize the receptive language and speech production abilities of school-aged children with auditory neuropathy/dyssynchrony (AN/AD) and to compare those abilities to children with sensorineural (SN) hearing loss of similar age and degree of hearing loss., Design: Standardized speech and language tests were carried out on 12 AN/AD children, aged between 57 and 167 mo. Each of these subjects was a full-time hearing aid user or had been just before testing. Receptive language skills were assessed using the Peabody Picture Vocabulary Test (PPVT) and speech production ability was measured using the Diagnostic Evaluation of Articulation and Phonology (DEAP) and a Speech Intelligibility Rating Scale. Data from a matched cohort of children with sensorineural hearing loss were also obtained., Results: Receptive vocabulary and speech production were delayed (to varying degrees) in each of the AN/AD subjects (relative to normally hearing children). The group PPVT Language Quotient score was 0.65 +/- 0.19 and the average number of pronunciation errors was 11 +/- 8.4% higher than expected for age. Results for the AN/AD group were however similar to those obtained for a matched group of children with sensorineural hearing loss on both language and speech production measures., Conclusions: The findings of this study indicate that while AN/AD type hearing loss can pose a significant developmental risk, at least some children fit with conventional amplification can develop reasonable speech and language abilities.

Objective: This study aimed to investigate 1) how a hearing aid needs to be adjusted for an adult who uses a cochlear implant in the contralateral ear; 2) whether the use of a hearing aid with a cochlear implant leads to interference; and 3) whether adults derive binaural benefits from using a hearing aid with a cochlear implant for speech perception, localization, and functional performance in everyday life., Design: Twenty-one adults (11 female and 10 male) who used either a Nucleus CI-22 (N = 3) or a Nucleus CI-24 (N = 18) cochlear implant system in one ear participated in this study. Twelve of the adults were experienced hearing aid and implant users, whereas nine did not use a hearing aid after implantation. The hearing aids were fitted using the NAL-NL1 prescription, and fine-tuned for each individual by using a paired-comparisons test to identify the frequency response that was best for understanding speech, and a loudness balancing test to find the hearing aid gain that gave the same overall loudness as that perceived in the ear with a cochlear implant. Effects from using a hearing aid with a cochlear implant (bimodal hearing) were assessed by three measures. These included speech perception in noise in diotic and dichotic listening conditions, horizontal localization, and functional performance in everyday life. Performance with cochlear implant and hearing aid (CIHA) was compared with that with cochlear implant alone (CI) and hearing aid alone (HA)., Results: The NAL-NL1 prescribed appropriate frequency response slope on average, and the mean gain needed for binaural loudness balance was 4 dB lower than the prescribed gain. Individual variations in preferences suggest that it is desirable to fine-tune the hearing aid response slope and gain according to individual needs. The speech test results indicated that the CIHA scores were significantly better than the CI or the HA scores. Localization test results showed that the adults made significantly less error when locating a sound source using CIHA compared with CI or HA. Functional performance questionnaire scores for CIHA were significantly higher than CI scores or HA scores. All adults showed binaural benefits in at least one performance measure. On average, those who derived greater speech benefits also made less localization error and functioned more effectively in real life., Conclusions: The results clearly indicate that binaural advantages can be obtained from using a hearing aid with a cochlear implant in opposite ears. It is recommended that bimodal stimulation be standard practice for rehabilitation of adults who wear unilateral cochlear implants. A hearing aid should be fitted to the nonimplanted ear using the NAL-NL1 prescription as a starting point, and the frequency response slope and gain could be fine-tuned to suit individual needs.