Universal Newborn Hearing Screening Research Articles

Community-based infant hearing screening: Outcomes of a rural pilot programme.

Community-based universal newborn hearing screening (UNHS) has not been fully realised in South Africa despite the availability of contextually relevant early hearing detection and intervention guidelines. Research has confirmed the feasibility of implementing UNHS programmes in urban contexts; however, limited information exists for rural contexts. The aim of the study was to describe the outcomes in terms of coverage rate, referral rate and follow-up rate of a 1-year UNHS pilot programme implemented at three primary health care (PHC) clinics in the Limpopo province. A descriptive retrospective review of 2 302 audiological records of infants who underwent NHS between July 2014 to June 2015 was conducted. The mean age at first-stage screen was 112 days (16 weeks). The coverage rate was 87% for the infants screened at 3- and 10- days clinic visits and 27% for infants screened at the 6-week immunisation visit. The first-stage referral rate was 33.9% and 8.3% for the overall second stage referral for diagnostic audiology services. The follow-up rate for rescreens at the clinical level was 77%, while for initial diagnostic assessments, it was 26%. Although not all benchmarks were met within the first year of implementation, the high coverage- and low referral rates, especially in the last 6 months, are the first steps in improving the outcomes of the screening programme.Contribution:The findings confirm the feasibility of implementing community-based UNHS programmes in rural areas in South Africa. Regular monitoring and evaluation contribute to the success of screening programmes.

Follow-Up of Neonatal Hearing Screening in the Risk Factor Group for Hearing Loss: Results from a Tertiary Medical Center

Introduction: Universal newborn hearing screening has been successfully implemented in many places around the world, and it is recommended that cases with risk factors for hearing loss be followed-up regardless of hearing screening results. However, there is a need for clarity regarding the recommended rate of follow-up and which tests should be performed. The aim of this study was to assess the audiologic follow-up program for the group with risk factors. Method: Our retrospective study involved children of various ages with a risk factor for hearing loss who passed the initial neonatal hearing test but were later diagnosed with hearing loss. Out of 113,708 children born at Hadassah University Medical Center during the years 2013–2021, 6763 were at risk of hearing loss, and their follow-up audiologic test results were studied. Results: Audiologic testing including ABR, OAE, tympanometry and behavioral audiometry was performed in 1534 of 6763 (23%) of the risk factor group that returned to the hospital. In total, 73 children (4.7%) were diagnosed with hearing loss, 54 of whom failed the initial screening and 19 who had passed it. Further examination of the children that passed the initial screening and were later diagnosed with a hearing loss revealed that four cases had been missed in screening (one familial mild hearing loss, one familial progressive loss, one premature infant with a high tone loss, and one NICU graduate with CNS involvement). Another nine cases had late-onset hearing loss (three meningitis, five CMV, and one with a mitochondrial disease). An additional six cases were diagnosed late, and the age of onset of the hearing loss was unknown (two intubated, two with hydrocephalus, one with Cerebral Palsy, and one with general developmental delay). Conclusions: These results reveal the importance of implementing a refined protocol for monitoring hearing in the high-risk group of children that pass neonatal hearing screening with respect to which hearing tests should be conducted, at what age, and the duration of follow-up. Also, barriers to follow-up must be dealt with, and parents should be more involved in the monitoring process.

Targeted gene sequencing and hearing follow-up in 7501 newborns reveals an improved strategy for newborn hearing screening.

Hearing loss is a common congenital condition. Concurrent newborn hearing and limited genetic screening has been implemented in China for the last decade. However, the role of gene sequencing screening has not been evaluated. In this study, we enrolled 7501 newborns (52.7% male, 47.3% female) in our Newborn Screening with Targeted Sequencing (NESTS) program, and 90 common deafness genes were sequenced for them. Hearing status assessments were conducted via telephone from February 2021 to August 2022, for children aged 3 to 48 months. Of the universal newborn hearing screening, 126 (1.7%) newborns did not pass. Targeted sequencing identified 150 genetically positive newborns (2.0%), with 25 exhibiting dual-positive results in both screening. Following diagnostic audiometry revealed 18 hearing loss newborns and half of them had abnormal results in both screening. The positive predictive value for universal newborn hearing screening alone was merely 14.3% (18/126). However, when combined with targeted sequencing, this rate increased to 36.0% (9/25). Furthermore, limited genetic screening identified 316 carriers of hot-spot variants, but none exhibited biallelic variants. All 15 hot-spot carriers who failed physical screening demonstrated normal hearing during follow-up. In this cohort study of 7501 Newborns, Combining targeted sequencing with universal newborn hearing screening demonstrated technical feasibility and clinical utility of identifying individuals with hearing loss, especially when coupled with genetic counseling and closed-loop management. It is suggested to use this integrated method as an improved strategy instead of the current limited genetic screening program in some regions of China.

Situational assessment of age of suspicion, diagnosis, and intervention of hearing loss in pediatric population in India and factors that influence them

ObjectiveIn the absence of newborn hearing screening, identification of childhood hearing loss is primarily mediated by parents’ help-seeking behaviour. This study attempted to measure the variables age of suspicion, diagnosis, and intervention of congenital hearing loss in India in a large cohort to verify the efficiency of this approach. It aimed to study the influence of parental perceived awareness, accessibility, and affordability on these milestones. MethodData were collected through a survey method of research using a pre-developed questionnaire as the instrument. The study involved 384 parents of children with hearing loss receiving hearing healthcare services from various healthcare institutions and clinics in Karnataka, India. ResultsThe mean age of suspicion, diagnosis, and intervention of hearing loss after data analysis were 18.6, 25.0, and 30.6 months, respectively. Earlier parental suspicion of hearing loss was associated with earlier diagnosis and intervention in the child. While less than 4 % of the cohort was diagnosed before three months, less than 1 % received intervention within six months of age. Lack of awareness, accessibility, and affordability significantly delayed these milestones. Among the participants, 15.03 % had experienced at least one of the three barriers (lack of awareness, accessibility, and affordability), whereas 35.28 % had reported two barriers, and 41.71 % had reported all three barriers for their help-seeking behavior. ConclusionsRelying on parental help-seeking behaviour cannot result in early identification and intervention required for optimum pediatric hearing healthcare. India should implement comprehensive early hearing detection and intervention program that includes universal newborn hearing screening and addresses awareness, accessibility, and affordability of hearing healthcare services as critical components.

Risk factors and hearing outcomes in infants and young children in KwaZulu-Natal, South Africa.

Targeted new-born hearing screening, based on high risk factors is recommended in the absence of universal new-born hearing screening in resource-constrained settings. The relevance of risk factors listed in the guidelines of high-income countries and used bylow-middle income countriesremains relatively unknown. Risk factors consistent with the epidemiological profile, evolution of risks and disease burden in these countries need to be considered. This study aimed to profile the frequency of risk factors and their manifestation in hearing outcomesof youngchildren in the KwaZulu-Natal province of South Africa. A chart review ofN= 1433 patients' archival audiology records was conducted, conveniently sampled from a single tertiary hospital (n= 351), a provincial assessment and therapy centre (n= 649), a university clinic (n= 291), and two schools for the deaf (n= 142). Overall, 56% of the participants presented with either a conductive, sensorineural or a mixed hearing loss; 62% of the children had between 1 and 2 risk factors present (Mean [M] = 1.1; standard deviation [s.d.] = 0.98). Admission to neonatal intensive care unit, maternal infections, bacterial and viral infections and chemotherapy, from the Joint Committee on Infant Hearing list of high risk factors were significantly associated with hearing loss (p 0.05). Known non-JCIH risks, emerging risks and other statistically significant contextually relevant risk factors were also noted. Understanding the profile of high risk factors in a given context has implications forprevention, early hearing identification and intervention services.Contribution:Targeted new-born hearing screening needs to be based on risk factors that are contextually relevant. This study is one of the first profiling high risk factors for hearing loss in children in KZN, the province with the second highest population in South Africa.

Hearing Screening for Congenital CytoMegaloVirus-Exploring Parents' Experiences of Completing Targeted Congenital Cytomegalovirus Screening at the Time of Their Infants' Newborn Hearing Screening.

Background/Objectives: Congenital cytomegalovirus (cCMV) is the leading infectious cause of sensorineural hearing loss and neurodevelopmental disabilities, with prompt detection (<21 days of life) required to enable accurate diagnosis and anti-viral treatment where clinically appropriate. International guidelines recommend cCMV screening for infants who do not pass their Universal Newborn Hearing Screening (UNHS). This study aimed to explore parental experiences of targeted cCMV screening through the UNHS in Victoria, Australia between 2019 and 2020 (HearS-cCMV study). Methods: A qualitative study comprising 18 semi-structured interviews with parents who took saliva swabs from their infants who did not pass their UNHS. A maximum variation sampling strategy was used with data analysed using thematic analysis. Results: Four themes described 18 parents' experiences of cCMV screening: (1) parents' lack of CMV awareness prior to cCMV screening; (2) overall positive experience; (3) varied understanding of CMV post screening; and (4) parents were glad to screen their infant for cCMV. Enablers of targeted cCMV screening included the swab being simple and non-invasive, being easier to complete in the hospital than at home, and the screening being well delivered by the staff. Barriers included a potential increase in anxiety, especially with false positives, and the timing of cCMV screening coinciding with their infant not passing UNHS being difficult for some parents. Conclusions: Parent experiences of targeted cCMV screening were positive. Increasing public knowledge of cCMV and training staff members to complete the CMV swab would reduce the risk of false positives and associated parental anxiety. This would facilitate successful routine targeted cCMV screening.

Newborn concurrent hearing and genetic screening for hearing impairment: A systematic review and meta‑analysis.

Hearing loss is the most prevalent neurosensory disorder in humans, with significant implications for language, social and cognitive development if not diagnosed and treated early. The present systematic review and meta-analysis aimed to determine the rate of hearing screening pass and genetic screening failure [universal newborn hearing screening (UNHS) pass/genetic failure] and to investigate the advantages of combining newborn hearing and genetic screening for newborn hearing impairment. The PubMed, Embase and Cochrane databases were searched from inception to September 2023 to identify studies reporting the combination of neonatal hearing screening with genetic screening. Duplicate literature, unpublished literature, studies with incomplete data, animal experiments, literature reviews and systematic studies were excluded. All the data were processed by STATA15.1 statistical software. A total of nine cross-sectional studies were included in this meta-analysis. The sample sizes ranged from 1,716 to 180,469, and there were a total of 377,688 participants. The pooled results revealed that the prevalence of passing the UNHS while failing genetic screening was 0.31% (95% CI, 0.22-0.41%). The prevalence of UNHS pass and gap junction protein beta 2 and solute carrier family 26 member 4 variant screen failure was 0.01% (95% CI, 0.00-0.02%) and 0.00% (95% CI, 0.00%), respectively, while the prevalence of mitochondrially encoded 12S RRNA variant screening failure and UNHS pass was 0.21% (95% CI, 0.18-0.26%). Combined screening has a significant advantage over pure hearing screening, especially in terms of identifying newborns with mitochondrial gene mutations that render them sensitive to certain medications. In clinical practice, decision-makers can consider practical circumstances and leverage the benefits of combined newborn hearing and genetic screening for early diagnosis, early counseling, and early intervention in patients with hearing loss.

Coalition for Global Hearing Health Hearing Care Pathways Working Group: Guidelines for Clinical Guidance for Readiness and Development of Evidence-Based Early Hearing Detection and Intervention Programs.

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.

Towards Comprehensive Newborn Hearing and Genetic Screening in Russia: Perspectives of Implementation

The universal newborn hearing screening (NHS) program was implemented in Russia in 2008 to replace the high-risk newborn hearing screening. More than 95% coverage and significant improvement in early detection and intervention is achieved. Meanwhile, it was shown that current OAE-based hearing screening missed 13% of newborns with genetically ascertained hereditary sensorineural hearing loss (SNHL). The aim of the study is to assess the results of genetic investigation and NHS in a large cohort of Russian children with bilateral SNHL and to study the feasibility of implementation of combined hearing and genetic screening in Russia. Genetic, audiological and NHS data of 1292 pediatric patients with bilateral SNHL born in 2008–2021 were analyzed. GJB2 sequencing was performed for all subjects, 644 patients had pathological GJB2 genotype, 406 of them were homozygous for c.35delG variant. The group of 155 GJB2-negative patients were searched for other SNHL genes, The pathological genotypes were identified at 87 patients. The most frequent genes were STRC (21.8%), USH2A (16.1%), OTOF (8%) and SLC26A4 (6.9%). Children with confirmed genetic etiology passed NHS in 21% of cases. The perspectives of implementation of national comprehensive newborn hearing and genetic screening including whole exome sequencing technologies are discussed.

Otolaryngology outcomes of infants with conductive hearing loss identified through universal newborn hearing screening

ObjectiveInfants and children diagnosed with a conductive hearing loss (CHL) are often referred for otolaryngology assessment. Although this is also a regular occurrence for infants diagnosed with a CHL through Universal Newborn Hearing Screening (UNHS), less is known about these infants and their outcomes. Using a cohort of infants diagnosed with CHL through UNHS and referred to otolaryngology, this study aimed to investigate the relationship between specific demographic or clinical characteristics and 1) triage category 2) middle ear diagnosis and intervention and, 3) service-related factors at otolaryngology. MethodsRetrospective analysis through clinical chart review was performed on all infants born between January 2014 and December 2017 who referred on UNHS, diagnosed with a CHL and referred to the Queensland Children's Hospital. Descriptive analysis and Chi squared analysis was conducted on data from 95 records. ResultsAnalysis between all infants referred from UNHS and those who referred, diagnosed with CHL and then referred to otolaryngology suggest that bilateral referrals/medical exclusion, preterm and infants with ≥1 risk factors are more readily associated with referral to otolaryngology for CHL. Nearly all (92.86 %) infants who were referred to otolaryngology had a primary diagnosis of OM and most infants (89.66 %) received grommets as an intervention. The average age of first appointment at otolaryngology was 427 days, the average age of intervention was 579 days and the average occasions of service at otolaryngology was 6.72. ConclusionThis paper provides a snapshot into the journey and outcomes of infants referred from UNHS, diagnosed with CHL, and referred to otolaryngology. Further investigation in both general and UNHS populations is needed to better understand and apply these findings.

Cost-effectiveness of Neonatal Hearing Screening Programs: Systematic Review.

Introduction Universal newborn hearing screening (UNHS) has been widely and strongly advocated as an early detection strategy for hearing loss in children. This intervention aims to prevent delays in speech and language development, which, in turn, has long-term beneficial effects on the social and emotional development and quality of life of individuals. However, the implementation of UNHS programs is circumstantial in different settings, for different reasons. Objectives The present systematic review aimed to identify whether the implementation of UNHS programs are cost-effective, as well as their variations by localities. Data Synthesis A search was conducted in seven databases: PubMed (Medline), Scopus, Web of Science, Embase, CINAHL, Lilacs, and Cochrane Library. Studies that included a cost analysis of UNHS programs were eligible for inclusion. Studies on evaluations of preschool or school-based programs only were excluded, among others. A total of 1,291 records were found. Of these, 23 articles were analyzed in full. All articles identified the cost-effectiveness of the UNHS programs implemented. Regarding the UNHS protocols, a wide variation was observed in all aspects: tests used, period established between tests and retests, professionals responsible for screening, environment, and criteria for defining hearing loss, limiting the generalization of this information. All studies presented values related to the expenses with the program, but none of them showed statistical elements for the described analyzes or any theoretical basis for such. Conclusion It is necessary to estimate local specific issues, as well as the accuracy of the chosen tests and the NHS protocols used, so that more accurate analyzes on cost-effectiveness are possible.

Newborn and infant hearing screening for early detection of hearing loss in Nairobi, Kenya.

Early detection of hearing loss and subsequent intervention leads to better speech, language and educational outcomes giving way to improved social economic prospects in adult life. This can be achieved through establishing newborn and infant hearing screening programs. To determine the prevalence of hearing loss in newborns and infants in Nairobi, Kenya. A cross-sectional pilot study was conducted at the National hospital and at a sub county hospital immunization clinic. A total of 9,963 babies aged 0-3 years, were enrolled in the hearing screening program through convenient sampling over a period of nine months. A case history was administered followed by Distortion Product Oto-acoustic emissions (DPOAEs) and automated auditory brainstem response (AABR) hearing screening. The screening coverage rate was 98.6% (9963/10,104). The referral rate for the initial screen was 3.6% (356/ 9,963), the return rate for follow-up rescreening was 72% (258 babies out of 356) with a lost to follow-up rate of 28% (98/356). The referral rate of the second screen was 10% (26/258). All the 26 babies referred from the second screen returned for diagnostic hearing evaluation and were confirmed with hearing loss, yielding a prevalence of 3/1000. Establishing universal newborn and infant hearing screening programs is essential for early detection and intervention for hearing loss. Data management and efficient follow-up systems are an integral part of achieving diagnostic confirmation of hearing loss and early intervention.

Family-Perceived Barriers and Facilitators Toward a Tele-Audiology Infant Diagnostic Testing Approach in Victoria, Australia.

Background: Universal newborn hearing screening programs allow for early identification of congenital hearing loss. However, some families experience difficulties accessing diagnostic audiology services following a refer screen result. Methods: This study aimed to assess the opinions of families who had experienced infant diagnostic audiology assessments regarding a telehealth option for these appointments within Victoria, Australia. Families who attended in-person infant diagnostic audiology appointments were sent a questionnaire exploring their experiences of the service and their opinion regarding a proposed telehealth option for infant diagnostic audiology (50 responses received). These results were also compared to those of families who were surveyed following testing in 2020, where the audiologist conducted the appointment remotely to comply with COVID-19-related social distancing recommendations at the time (10 responses received). Results: There were not significant differences between the duration or number of appointments, perceived understanding of results, or concerns regarding a tele-audiology model between families who experienced face-to-face and tele-audiology infant diagnostic audiology appointments. Opinions of infant diagnostic audiology appointments utilizing telehealth technology were largely positive, and minimal technological difficulties were identified. Conclusion: Overall positive attitudes of many families with infant diagnostic appointment experiences toward a tele-audiology option of this service suggest that offering a telehealth model of appointments may be an appropriate model to improve service access for families requiring infant diagnostic audiology in Victoria.

Retesting of Neonatal Hearing Screening Before and During the COVID-19 Pandemic: A Longitudinal Study

Introduction: Universal Newborn Hearing Screening (UNHS) plays an essential role in the early identification of hearing loss in neonates. Risk factors for hearing impairment may include family history, prematurity, and exposure to ototoxic substances. Coronavirus Disease 2019 (COVID-19) might be a significant contributing factor affecting the structures of the inner ear. Objective: To assess the auditory follow-up process of retesting for UNHS before and during the COVID-19 pandemic among neonates from an Outpatient Auditory Health Service (SASA) in the state of Santa Catarina with SUS (Unified Health System) assistance. Methods: A retrospective longitudinal study analyzed data from neonates attended at a SUS Auditory Health Service (SASA) from January 2018 to December 2022. Information related to UNHS and retest outcomes was assessed. Data were analyzed using Microsoft Excel® and MedCalc® Statistical Software version 22.006, utilizing statistical measures and regression analyses to identify factors associated with UNHS failures and retesting. Results: A failure to retest rate of 2.6% in the right ear and 2.2% in the left ear was observed among evaluated neonates. The average age of mothers of neonates who did not pass the test was 33 years, while the overall average was 27 years. Failure to pass the retest and a longer interval between UNHS and retesting were associated with UNHS Initial Retest Default (IRD). There was an increase in dropout rates for UNHS retesting, and the time interval between UNHS and retesting was extended during the pandemic. Conclusion: Several factors, including the interval between tests, mothers' age, and medical conditions, influenced the retest outcomes. The pandemic led to a significant increase in dropout rates and extended time for retesting.

SWOT analysis of Russian universal newborn hearing screening program

Introduction. The negative impact of congenital hearing loss on child’s development, education and socialization can be reduced by early identification and intervention as a result of universal newborn hearing screening. The screening program needs continuous improvement by monitoring of outcomes and correction of algorithms. The SWOT analysis can be applied to make effective decisions. &#x0D; The purpose of the study is to identify strategies for the improvement of the Russian newborn hearing screening program based on SWOT analysis.&#x0D; Materials and methods. The study was based on the experience of two pediatric audiological centres in Novosibirsk and St. Petersburg. &#x0D; At the first stage, in accordance with the SWOT methodology, internal and external factors, the strengths and weaknesses of the Russian hearing screening program were identified, as well as the opportunities and threats that can affect the audiological care for children. At the second stage, pairwise analysis of strengths, weaknesses, opportunities and threats was carried out.&#x0D; Results. The strengths of the Russian hearing screening program are national level implementation, high coverage of newborns in maternity hospitals, the use of modern objective audiological methods. Weaknesses are associated with a lack of medical personnel, need to equipment replacement, number of cases lost to follow-up. Opportunities are the availability of high-quality domestic diagnostic equipment, possibility for active interaction of specialists and families. The threats are caused by an imperfect legal framework and funding mechanisms, low awareness of parents, related professionals and society about the problem of congenital hearing loss. &#x0D; Limitations. The study results are limited by the expertise of the authors without involving the wide range of stakeholders.&#x0D; Conclusion. The developed strategies can increase timely audiological service to hearing impaired children and their families and to plan the necessary resources in the face of modern challenges.

Newborn and Infant Hearing Screening for Early Detection of Hearing Loss in Nairobi, Kenya

Background: Early detection of hearing loss and subsequent intervention leads to better speech, language and educational outcomes giving way to improved socioeconomic prospects in adult life. This can be achieved through establishing universal newborn hearing screening (UNHS) programs. The objectives of this study were to assess the feasibility of implementing a UNHS program and to also determine the prevalence of hearing loss in newborns in Nairobi, Kenya.&#x0D; Materials And Methods: A cross-sectional pilot study was conducted at the National Hospital and a sub-county hospital immunization clinic. A total of 9,963 babies aged 0-3 years, were enrolled in the hearing screening program through convenient sampling over nine months. A case history was administered followed by Distortion Product Oto-acoustic emissions (DPOAEs) and automated auditory brainstem response (AABR) hearing screening.&#x0D; Results: The screening coverage rate was 98.6% (9963/10,104). The referral rate for the initial screen was 3.6% (356/ 9,963), and the return rate for follow-up rescreening was 72% (258 babies out of 356) with a loss to follow-up rate of 28% (98/356). The referral rate of the second screen was 10% (26/258). All 26 babies referred from the second screen returned for diagnostic hearing evaluation and were confirmed to have hearing loss, yielding a prevalence of 3/1000.&#x0D; Conclusions: Establishing universal newborn and infant hearing screening programs in Kenya is feasible and essential for early detection and intervention for hearing loss. Data management and efficient follow-up systems are an integral part of achieving diagnostic confirmation of hearing loss and early intervention.

Pediatric hearing screening: Exploring the need for a three-tier screening strategy

Abstract Pediatric hearing impairment is a global health problem and has implications on the individual as well as the society. Undiagnosed hearing impairment has immense economic burden on the family, society, and nation. The challenge lies in diagnosing pediatric hearing impairment early so that effective treatment or rehabilitation can be initiated. Even though universal newborn hearing screening has been implemented in many countries, yet the coverage of the program has not been uniform throughout. Further, many cases of childhood deafness are known to manifest later in the school going age. As such, reliance on hearing screening in the newborn period alone will lead to many undiagnosed cases of hearing impairment. Therefore, a viewpoint is provided here for a three-tiered screening strategy so that maximum cases of hearing impairment can be diagnosed and effective treatment can be initiated, thereby reducing the global economic burden of pediatric hearing impairment.

Establishment of the Norwegian hearing register for children.

The Norwegian Directorate of Health approved the Norwegian Hearing Register for Children in 2022. The main objective of the register is to improve the quality of treatment for children with permanent hearing loss, by measures, follow-ups and monitoring the quality and results of the health care system. Inclusion criteria are children who do not pass universal newborn hearing screening and/or children with permanent hearing loss <18 years of age. Hearing loss is defined as pure-tone audiometry threshold of (PTA4) > 20 dB in at least one ear. Data are registered at the Ear, Nose and Throat departments at inclusion and at follow-ups at the age of 3, 6, 10, and 15 years. The register collects information about the child within a holistic perspective. The key elements of the register are (a) data concerning newborn hearing screening; (b) data concerning hearing, medical information, hearing amplification and intervention (c) patient reported outcome measures registered by caregivers using three questionnaires; Pediatric Quality of Life Inventory, Strengths and Difficulties Questionnaire and Parents' Evaluation of Aural/Oral Performance of Children. The register has established four quality indicators regarding newborn hearing screening and early intervention (a) the rate of false positive neonatal screens; (b) testing for congenital cytomegalovirus within 3 weeks of age for children who do not pass newborn hearing screening; (c) audiological evaluation to confirm the hearing status no later than 3 months of age and (d) initiated intervention within 3 months after confirmation of hearing status. The register will include the total population of hearing impaired children over long time periods. Thus, the register enables each hospital to monitor their quality indicator scores continuously and compare them with national levels in real time. This facilitates and accelerates identification of improvement areas in the hospitals and will be an important contributor for quality improvement in NHS, diagnostics and hearing intervention for children in Norway. In addition, data from the register will be a unique source for research, and study designs with a long follow-up time can be applied.

Universal newborn hearing screening program in Saudi Arabia: Current insight

Newborn hearing screening (NHS) programs are essential to identify hearing loss early in life and to improve outcomes in children. In Saudi Arabia, the national NHS program has been operational since 2016; however, few studies have evaluated its status, and none have covered all provinces across the country. This cross-sectional retrospective study provides an overview of the program's status across all provinces, focusing on screening coverage rates, referral/fail rates, and follow-up procedures. In 2021, 199,034 newborns were screened, with a coverage rate of 92.6% and an overall referral/fail rate of 1.87%. These performance measures provide a foundation for future progress and improvements. This study highlights the importance of ongoing efforts to enhance the program's effectiveness and sustainability.

Congenital Deafness and Deaf-Mutism: A Historical Perspective.

Hearing loss is the most common sensory deficit and one of the most common congenital abnormalities. The estimated prevalence of moderate and severe hearing loss in a normal newborn is 0.1-0.3%, while the prevalence is 2-4% in newborns admitted to the newborn intensive care unit. Therefore, early detection and prompt treatment are of utmost importance in preventing the unwanted sequel of hearing loss on normal language development. The problem of congenital deafness is today addressed on the one hand with hearing screening at birth, on the other with the early (at around 3 months of age) application of hearing aids or, in case of lack of benefit, by the cochlear implant. Molecular genetics, antibody tests for some viruses, and diagnostic imaging have largely contributed to an effective etiological classification. A correct diagnosis and timely fitting of hearing aids or cochlear implants is useful for deaf children. The association between congenital deafness and "mutism", with all the consequences on/the consideration that deaf mutes have had since ancient times, not only from a social point of view but also from a legislative point of view, continued until the end of the nineteenth century, with the development on one side of new methods for the rehabilitation of language and on the other of sign language. But we need to get to the last decades of the last century to have, on the one hand, the diffusion of "universal newborn hearing screening", the discovery of the genetic causes of over half of congenital deafness, and on the other hand the cochlear implants that have allowed thousands of children born deaf the development of normal speech. Below, we will analyze the evolution of the problem between deafness and deaf-mutism over the centuries, with particular attention to the nineteenth century.