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Background:  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 by low-middle income countries remains relatively unknown. Risk factors consistent with the epidemiological profile, evolution of risks and disease burden in these countries need to be considered., Objectives:  This study aimed to profile the frequency of risk factors and their manifestation in hearing outcomes of young children in the KwaZulu-Natal province of South Africa., Method:  A chart review of N = 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)., Results:  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., Conclusion:  Understanding the profile of high risk factors in a given context has implications for prevention, 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.

Objective: To analyze the mutation spectrum of 23-site chip newborn deafness genetic screening in Beijing, and to provide basis for genetic counseling and clinical diagnosis and treatment. Methods: The study included 21 006 babies born in Beijing from December 2022 to June 2023. All subjects underwent newborn deafness genetic screening in Beijing Tongren Hospital, covering 23 variants in 4 genes, the GJB2 gene（c.35delG, c.176_191del16, c.235delC, c.299_300delAT, c.109G>A, c.257C>G, c.512insAACG, c.427C>T, c.35insG）, SLC26A4 gene（c.919-2A>G, c.2168A>G, c.1174A>T, c.1226G>A, c.1229C>T, c.1975G>C, c.2027T>A, c.589G>A, c.1707+5G>A, c.917insG, c.281C>T）, Mt12SrRNA （m.1555A>G, m.1494C>T） and GJB3 gene（c.538C>T）. The mutation detection rate and allele frequency were analyzed. Results: The overall mutation detection rate was 11.516%（2 419/21 006）, with the GJB2 gene being the most frequently involved at 9.097%（1 911/21 006）, followed by the SLC26A4 gene at 2.123%（446/21 006）, the GJB3 gene at 0.362%（76/21 006） and Mt12SrRNA at 0.176%（37/21 006）. Among the GJB2 genes, c.109G>A and c.235delC mutation detection rates were the highest, with 6.579%（1 382/21 006） and 1.795%（377/21 006）, respectively. Of the SLC26A4 genes, c.919-2A>G and c.2168A>G had the highest mutation rates of 1.423%（299/21 006） and 0.233%（49/21 106）, respectively. Regarding the allele frequency, GJB2 c.109G>A was the most common variant with an allele frequency of 3.359%（1 411/42 012）, followed by the GJB2 c.235delC at 0.897%（377/42 012） and the SLC26A4 c.919-2A>G at 0.719%（302/42 012）. Conclusion: 23-site chip newborn deafness genetic screening in Beijing showed that GJB2 c.109G>A mutation detection rate and allele frequency were the highest. This study has enriched the epidemiological data of 23-site chip genetic screening mutation profiles for neonatal deafness, which can provide evidence for clinical practice., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Objective: The EUSCREEN project concerns the study of European vision and hearing screening programmes. Part of the project was the development of a cost-effectiveness model to analyse such programmes. We describe the development and usability of an online tool to enable stakeholders to design, analyse or modify a newborn hearing screening (NHS) programme., Design: Data from literature, from existing NHS programmes, and observations by users were used to develop and refine the tool. Required inputs include prevalence of the hearing impairment, test sequence and its timing, attendance, sensitivity, and specificity of each screening step. Outputs include the number of cases detected and the costs of screening and diagnostics., Study Sample: Eleven NHS programmes with reliable data., Results: Three analyses are presented, exploring the effect of low attendance, number of screening steps, testing in the maternity ward, or screening at a later age, on the benefits and costs of the programme. Knowledge of the epidemiology of a staged screening programme is crucial when using the tool., Conclusions: This study presents a tool intended to aid stakeholders to design a new or analyse an existing hearing screening programme in terms of benefits and costs.

Background:  Unavailability of healthcare resources can lead to poor patient outcomes. The latter is true for infants with hearing loss and require early hearing detection and intervention (EHDI)., Aim:  To determine the availability and distribution of resources for EHDI in state hospitals in the Eastern Cape (EC) province, South Africa., Setting:  Sixteen state hospitals (nine district, four regional and three tertiary hospitals)., Methods:  Descriptive cross-sectional survey completed between July 2022 and October 2022., Results:  Thirteen hospitals had audiologists (n = 4) or speech therapists and audiologists (n = 9). Specific to equipment, 10 hospitals had a screening otoacoustic emissions or automated auditory brainstem response, 8 hospitals had diagnostic middle ear analysers and only 3 hospitals had diagnostic auditory brainstem response and/or auditory steady state response. Twelve hospitals did not have visual response audiometry (VRA) and 94% had no hearing aid verification systems. Budget allocations were uneven, with only 10 hospitals, i.e., 4 districts, all regional and 2 tertiary hospitals being allocated varying amounts. Subsequently, only 50% provided newborn hearing screening, 56% provided diagnostic evaluations and 14 hospitals fitted hearing aids., Conclusion:  Results revealed a limited and uneven distribution of resources, which negatively impacted the provision of EHDI. Even distribution of healthcare resources and further research aimed at strengthening hearing health services is recommended as these could potentially improve equitable access to EHDI and the overall quality of healthcare provided.Contribution: This study highlights the need for even distribution of resources and strengthening of health systems, especially in the dawn of the National Health Insurance.

Successful intervention to support a child with congenital hearing loss requires early identification and consistent access to frequent professional services. In the early 2000s, the United States implemented an initiative, Early Hearing Detection and Intervention (EHDI), to provide timely identification and treatment of congenital hearing loss. This national program aims to screen hearing by 1 month of age, diagnose hearing loss by 3 months of age, and provide intervention to infants with hearing loss by 6 months of age. To date, the United States is successfully implementing hearing screening by 1 month of age but continually struggling to diagnose and treat congenital hearing loss promptly for many infants. This article begins by exploring the current state of American children and families, focusing on social determinants of health, specifically race and poverty. The objective is to understand how race affects social determinants of health, and ultimately hearing healthcare access for children. A narrative literature review spanning public health, sociology, and hearing research was completed to inform this work. The current body of literature supports the conclusion that race and racism, separate from poverty, lead to decreased access to pediatric hearing healthcare. Interventions targeting these issues are necessary to improve timely access to hearing loss diagnosis and treatment for American children., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Universal newborn hearing screening (UNHS) is effective in identifying newborns with possible hearing loss (HL). Outpatient follow-up for newborns referred after hospital-based screening remains a potential area of improvement. In this study, we evaluate the efficacy of a community health worker (CHW) intervention in promoting adherence to outpatient rescreening for newborns referred after initial UNHS., Methods: A mixed prospective-retrospective cohort study was performed to evaluate a CHW intervention at an academic medical center. Caregivers of referred newborns were contacted by CHWs prior to discharge and educated about HL and the importance of follow-up screening. The CHW outreach intervention was performed for 297 referred newborns between May 2020 and June 2021 and compared to a cohort of 238 newborns without the CHW intervention between March 2019 and June 2021. Statistical analyses were conducted using 2 × 2 Chi-square tests, two-tailed unpaired t -tests, multinomial logistic regression, and multiple linear regression., Results: In the intervention group, 236 of 297 newborns (79.5%) completed their outpatient follow-up rescreening; in the comparison group, 170 of 238 newborns (71.4%) completed their follow-up rescreening ( P  = .031, OR = 1.55 with regression P  = .04). In the intervention group, the average time to follow-up was 13.4 days versus 12.5 days for the comparison group ( P  = .449, multiple R 2  = .02 with P  = .78)., Conclusions: CHW outreach intervention may increase adherence to outpatient follow-up rescreening for newborns referred after initial, hospital-based UNHS. Expansion of nursery teams to include CHWs may thus improve completion of recommended follow-up hearing screens., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Stephen Leong, BA: None. Kristy Medina, MPH: None. Patricia J. Peretz, MPH: None. Maria I. Olmeda-Jenkins, MA, CCC-A: None. Melissa A. Oliver, MED: None. Megan Kuhlmey, AuD, CCC-A: None. Stacey A. Bernstein, MS, CCC-SLP: None. Fajah Ferrer: None. Luz Adriana Matiz, MD: None. Anil K. Lalwani, MD: Medical Advisory Board of Spiral Therapeutics.

Objectives: Hearing loss (HL) constitutes an increasing worldwide health problem. Neonatal hearing screening improved early detection and management to alleviate HL detriments on the person and society. Still, HL in childhood, beyond infancy, is under-investigated, especially in developing countries. This study aimed to explore the prevalence of HL in childhood amongst Jordanian children with HL risk factors and investigate the associated risk factors., Methods: Retrospective cross-sectional review of audiological records in a tertiary public and teaching hospital. The data of 1307 children aged 0-15 years who underwent audiological assessment from 2000 to 2016 were included. A review of diagnostic audiological and medical records was conducted to investigate the prevalence of sensorineural HL in high-risk (HR) children and the most contributing risk factors., Results: Descriptive statistical analysis showed that the prevalence of sensorineural HL was 29.2% in the study sample. The HL was bilateral in 95% and mild to moderate HL in 73%. The mean age at the diagnosis was around 4.5 years. The most common risk factors were parental concern about their child's hearing, ototoxic drug use, and developmental and speech delay. The Chi-squared test showed that parental concern and ototoxic drug use were associated with an increased probability of having HL., Conclusion: The prevalence of HL amongst at-risk children in Jordan is relatively high, and the diagnosis is delayed. The results highlight the importance of implementing a hearing screening program in at-risk children. This needs to start from birth and include a serial follow-up to detect cases of delayed-onset HL., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objectives: Early hearing detection and intervention (EHDI) is a newborn hearing screening system created to detect infants with hearing loss (HL) and intervene to reduce language and communication impairment. Early hearing detection (EHD) consists of three sequential stages: identification, screening, and diagnostic testing. This study longitudinally reviews each stage of EHD in each state and proposes a framework to improve utilization of EHD data., Design: A retrospective public database review was conducted, accessing publicly available data from the Centers for Disease Control and Prevention. Summary descriptive statistics were utilized to generate a descriptive study of EHDI programs in each U.S. state from 2007 to 2016., Results: Data over 10 years from 50 states as well as Washington, DC were included in this analysis, creating up to 510 data points per analysis. Hundred percent (85 to 105) (median [min to max]) of newborns were identified by and entered EHDI programs. Ninety-eight percent (51 to 100) of identified infants completed screening. Of the infants who screened positive for HL, the proportion that received diagnostic testing was 55% (1 to 100). The overall proportion of infants who failed to complete EHD was 3% (1 to 51). Of the infants who fail to complete EHD 70% (0 to 100) are from missed screenings, 24% (0 to 95) are from missed diagnostic testing, and 0% (0 to 93) are from missed identification. Although there are more infants missed at screening, it was estimated, with limitations, that there is an order of magnitude more infants with HL among those who did not complete diagnostic testing compared with those who did not complete screening., Conclusions: Analysis demonstrates high completion rates at both identification and screening stages, whereas the diagnostic testing stage demonstrates low and highly variable completion rates. The low completion rates at diagnostic testing create a bottleneck in the EHD process and the large variability impedes the comparison of HL outcomes across states. Analysis also demonstrates that among all stages of EHD, whereas the largest number of infants are missed at screening, the largest number of children with HL are likely missed at diagnostic testing. Therefore, a focus by individual EHDI programs on addressing causes of low diagnostic testing completion rates would yield the greatest increase in the identification of children with HL. Potential causes of low diagnostic testing completion rates are further discussed. Finally, a new vocabulary framework is proposed to facilitate further study of EHD outcomes., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose of Review: Congenital cytomegalovirus infection (cCMV) is a major cause of childhood hearing loss and neurodevelopmental delay. Early identification of cCMV allows for interventions that improve outcomes, particularly for cCMV-related hearing loss that develops in early childhood. Most cCMV is asymptomatic at birth and is rarely diagnosed without newborn screening. Therefore, various approaches to cCMV screening are increasingly being adopted., Recent Findings: Both universal screening (testing all newborns) and targeted screening (testing triggered by failed hearing screening) for cCMV appear valuable, feasible and cost-effective, though universal screening is predicted to have greatest potential overall benefits. CMV PCR testing of newborn oral swabs is sensitive and practical and is therefore widely used in targeted screening programs. In contrast, PCR using dried-blood spots (DBS) is less sensitive but was adopted by current universal cCMV screening initiatives because DBS are already collected from all newborns in high-income countries, which circumvents large-scale oral swab collection., Summary: Targeted screening is widely recommended as standard of care, while universal screening is less common but is progressively considered as the optimal strategy for identification of children with cCMV. As with all screening programs, cCMV screening requires commitments to equitable and reliable testing, follow-up and services., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To verify the frequency of risk factors for hearing loss in newborns and their possible associations with universal neonatal hearing screening results before and during the COVID-19 pandemic., Methods: Historical cohort study with data analysis of newborns attended in a reference hearing health service of the Unified Health System (SUS) between January 2017 and December 2021., Results: Those born in 2020 and 2021 were 91% less likely to fail the screening than those born in 2017, 2018, and 2019; therefore, they had a lower percentage of referrals for a retest. There was a decrease in congenital syphilis (1.00%), decrease in HIV (0.95%), and an increase in toxoplasmosis (0.58%) and increase in rubella cases in 2021 in relation to 2017. Syphilis had lower frequency rates during the COVID-19 pandemic (2020-2021)., Conclusion: Newborns born in the pandemic year compared to those born pre-pandemic showed a reduction in the presence of two risk indicators for hearing loss and, consequently, a lower chance of failing the UNHS and a lower percentage of referral for retest., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objective: To screen new-borns to diagnose any hearing impairment early., Methods: The prospective, cross-sectionalstudy was conducted at the Department of Otorhinolaryngology, Head and Neck Surgery Liaquat National Hospital, Karachi, from November 1, 2020, to April 30, 2021, and new- borns of either gender aged >12h born via spontaneous vaginal delivery, induced labour, and Caesarean section. A predesigned questionnaire was used to collect detailed case history, including gestational age, duration of labour, and other prenatal, natal, and postnatal risk factors. Otoacoustic emission test was performed, and infants referred twice were scheduled for complete diagnostic evaluation and brainstem evoked response audiometry. Data was analysed using SPSS 23., Results: Of the 267 neonates, 249(93.3%) passed the first screening. Of the remaining 18(6.7%) neonates, 8(44.4%) passed the second screening, while 10(55.5%) were asked to come for a follow-up after three weeks. Of them, 3(30%) returned for check-up, while 7(70%) did not show up., Conclusions: Neonatal risk factors associated with hearing loss need to be identified, and a comprehensive hearing screening programme is required for neonates.

Objective: Infants diagnosed with a conductive hearing loss (CHL) are at increased risk of developmental delays. Using a sample of infants diagnosed with CHL through UNHS, this study aimed to investigate the relationship between specific demographic or clinical characteristics and 1) occasions of service to reach a hearing diagnosis and 2) the profile of CHL., Methods: Retrospective analysis was conducted for all infants with CHL born between 01/01/2007 and 31/12/2018 who had received UNHS. Chi squared analysis was conducted on data from 1208 records., Results: Infants with ≥1 risk factor for hearing loss were more likely to attend more than three occasions of service. Infants who were bilateral refer/medical exclusion, Torres Strait Islander, had ≥1 risk factors for hearing loss or were born pre-term had greater proportions of bilateral CHL than unilateral CHL. Mild to moderate was the most frequent degree of CHL, although a unilateral or bilateral CHL did not have an association with the severity of CHL. Compared to other risk factors, infants with a syndrome had greater proportions of bilateral than unilateral CHL. Risk factors of craniofacial abnormality, prolonged ventilation, or syndrome had greater proportions of mild to moderate CHL than moderate or greater. On average, infants were diagnosed with a CHL at 37.29 weeks of age., Conclusion: These findings highlight the relationship between clinical/demographic characteristics and occasions of service to diagnose CHL in children, including the CHL profile. An understanding of this relationship may help clinicians to better plan, assess and manage infants diagnosed with a CHL through UNHS., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Introduction: Genetic causes are responsible for half of the cases of hearing loss, most of them being the result of non-syndromic genetic changes resulting from autosomal recessive inheritance. Parental consanguinity might be an indicator to consider in the diagnosis of these cases. The aim of this study was to assess its importance as a risk factor for childhood hearing loss., Material and Methods: A retrospective cohort study conducted in a district hospital, between 2014 and 2018. We included all live births born during this period and excluded those with risk factors for childhood hearing loss other than parental consanguinity and those without hearing screening. We formed two study groups: newborns with parental consanguinity and newborns without risk factors. All the participants underwent hearing screening with the primary outcome of this study being the result of the screening. Those with a not normal result or with parental consanguinity also underwent diagnostic audiological evaluation., Results: Among 8513 live births, we studied 96 newborns with first-degree parental consanguinity and 96 newborns without risk factors. We found a statistically significant difference (p = 0.007) between the groups, with a 'refer' screening result rate of 24% in the group with parental consanguinity and 9.4% in the group without risk factors. We diagnosed one case of sensorineural hearing loss and another of mixed hearing loss in the first group and none of these cases in the second., Conclusion: Parental consanguinity was associated with a higher risk of a refer screening result in newborns, which suggests the need to consider this as a risk factor for childhood hearing loss.

Objective: To investigate the perceptions of parents towards universal new-born screening for hearing loss in Nigeria., Methods: A systematic review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) system. A comprehensive search was undertaken using multiple databases (National Library of Medicine, PubMed, Cochrane Library, Scopus, Google Scholar, and MEDLINE) published in English between 2009 and 2018. The sample size of 10 studies were retrieved by using a predefined inclusion and exclusion criteria, keywords, and Boolean operator search patterns. Data extraction and analysis and quality appraisal of the selected articles was carried out., Results: 80% of the identified studies indicated negative perceptions of Nigerian parents towards new-born hearing loss screening programmes. Possible reasons behind such negative perceptions were categorised into demographic factors, psychological factors, behavioural factors, and service-related factors. Specifically, barriers included low income, misconceptions, mode of delivery, fear of associated risk with screening procedure, preference for traditional medicines, inappropriately designed screening programmes, cost of screening programmes, resource-limited settings, lack of awareness about risk factors and their effects., Conclusion: The research concluded that it is necessary to focus on the range of underlying factors (including awareness and education) contributing in shaping the above mentioned negative perceptions among Nigerian parents. The findings have great implications for the theoretical researchers for developing future research path as well as practical suggestion for authorities in policymaking in the healthcare sector in Nigeria., Competing Interests: Declaration of competing interest Authors have declared that no competing interests exist in connection with this study., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objective: To understand the characteristics of postnatal hearing loss (PNHL) identified via different referral pathways, to inform childhood hearing screening and referral practices., Design: Retrospective analysis of screening and audiology records., Study Sample: A cohort of 385 children who passed newborn hearing screening in Queensland, Australia between September 2004 and December 2017 and were later diagnosed with permanent hearing loss., Results: Neonatally identified risk factors facilitated detection for half the cohort, with PNHL detected earlier (average age of 31 months) and at a milder degree. PNHL was detected at an average age of 49 months via other pathways. Proportions of bilateral moderate or greater PNHL were greatest in children with significant medical circumstances (60.7%) and those with noted delays or concerns (39.2%), whereas childhood hearing screening programs detected greater proportions of unilateral moderate or greater PNHL (47.4%)., Conclusions: Risk-factor-based surveillance detects PNHL early but does not detect all cases. Screening children with speech and language delays, parental or professional concern, or with relevant medical circumstances (eg meningitis or chemotherapy) is warranted based on the types of PNHL detected. Further evidence may be required to justify the rollout of postnatal population childhood screening programs.

Objective: To investigate the audiological characteristics and possible causes of unilateral hearing loss in infants and young children. Methods: 105 infants from Beijing Maternal and Child Health Care Institution who failed the newborn hearing screening and were referred to the Children's Hearing Diagnosis Center of PLA General Hospital for hearing diagnosis. They were diagnosed with unilateral hearing loss and underwent clinical data collection. A full set of audiological examinations included ABR, 40 Hz auditory event related potential, ASSR, DPOAE, tympanometry. Results: ①In initial diagnosis, 45 cases（42.86%） had mild hearing loss, 19 cases（18.10%） had moderate hearing loss, 14 cases（13.33%） had severe hearing loss, and 27 cases（25.71%） had severe hearing loss; Among them, 65 cases（61.90%） were conductive hearing loss or mixed hearing loss, and 40 cases（38.10%） were sensorineural hearing loss. ②83 of 105 cases had follow-up visits: 24 cases were normal, 15 cases with mild hearing loss, 4 cases with moderate hearing loss, 12 cases with severe hearing loss, and 26 cases with extremely severe hearing loss, 2 cases of hearing loss in both ears. ③From the initial diagnosis to the follow-up diagnosis, the change of mild hearing loss was the largest, followed by moderate hearing loss, severe and extremely severe hearing loss basically did not change; the number of mild and severe conductive hearing loss which recovered to normal hearing was most, the number of sensorineural hearing loss changed little. Conclusion: The infants who failed the newborn hearing screening and were diagnosed with unilateral hearing loss were mainly mild to moderate conductive hearing loss and severe to extremely severe sensorineural hearing loss. The hearing of children with hearing loss gradually improved, and severe and extremely severe sensorineural hearing loss remained unchanged., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Purpose: In 2015 a new regulation and guidelines for the universal newborn hearing screening by AABR measurement have been implemented in Hungary. The aim of our study was to analyse (1) the past 5 years of data from our diagnostic centre about the incidence and types of congenital hearing losses, and (2) the first experiences with the National Newborn Hearing Screening Registry, started in 2019, and (3) the influence of the screening on the pediatric cochlear implant program., Methods: 1269 children referred to our diagnostic centre between 2017 and 2021 were investigated. A third AABR measurement and full audiological evaluation were performed. Furthermore, one-year period data of the screening registry, and the number of implanted children at or under the age of 3 were analysed using the national databases., Results: Altogether 276 newborns (22% of the referred cases after the two-stage screening) had hearing loss, 134 (49%) out of them was conductive origin, almost twice frequent in male as in female. Permanent sensorineural hearing impairment was found in 142 (51%), 58 (40%) of them had bilateral, severe to profound hearing loss, occurring more frequently in male as in female. The national digital registration of the screening data within 12 months concerned 68%. The number of early cochlear implantation in one year increased from 1 to 23 children in the past 15 years., Conclusion: A third AABR after the two-stage screening increased the efficiency and filtered the 78% false-positive cases. The audiological diagnostics verified and typed the hearing losses ensuring the early intervention., (© 2022. The Author(s).)

Objective: This study aimed to research risk factors of hearing loss among neonates in the neonatal intensive care unit., Method: Hearing screening tests were performed on 572 neonates in the neonatal intensive care unit. Those who failed screening tests were referred for diagnostic tests., Results: The pass rates for automated auditory brainstem response, distortion product otoacoustic emission and acoustic impedance tests at first hearing screening were 69.93 per cent, 70.02 per cent and 92.92 per cent for 1144 ears. Failure in the first screening correlated with preterm birth, very low birth weight, revised advanced maternal age, neonatal hyperbilirubinaemia and Activity, Pulse, Grimace, Appearance, Respiration score less than 8. Thirty cases failed in diagnostic hearing tests for brainstem auditory evoked potentials, 28 failed in otoacoustic emissions and 33 failed in acoustic impedance, which correlated with preterm birth, very low birth weight, twins, advanced maternal age and revised advanced maternal age., Conclusion: Abnormalities in the hearing levels of most neonates who needed hearing retests were completely or partially reversible. Preterm birth, very low birth weight, twins and advanced maternal age are potential risk factors for hearing impairment.

Objectives: To investigate whether Gestational diabetes mellitus (GDM) is related to presumptive hearing impairment in newborns., Study Design: The newborns were divided into two groups. The first group included the children of gestational diabetic pregnancies (DPs), and the second group, the children of non-gestational diabetic pregnancies (NDPs). Transient evoked otoacoustic emissions (TEOAE) and automated Auditory Brainstem Response (aABR) tests were applied as hearing screening tests., Results: Twenty (40.8%) newborns in the DPs group and 5 (7.7%) newborns in the NDPs group failed the first hearing screening test ( p  = .001). The number of newborns with bilateral failed hearing screening tests was higher in the DPs group at the first screening (75% vs. 20%, p  = .04). Fifteen (75.0%) of 20 newborns in the DPs group and 1 (20.0%) of 5 newborns in the NDPs group failed the second TEOAE hearing screening test ( p  = .04)., Conclusion: The possibility of presumptive hearing impairment is higher in the newborns of mothers with gestational diabetes compared to the newborns of non-diabetic mothers.

Aim: This study aimed to evaluate the implementation of France's neonatal hearing loss screening programme  years after its launch, and to estimate permanent bilateral neonatal hearing loss (PBNHL) prevalence and distribution by severity., Methods: This descriptive study used aggregated regional data on all births in France in 2015-2016. Screening coverage, refusal rate, positive predictive value (PPV), proportion of children with suspected PBNHL, PBNHL prevalence and distribution by severity were calculated., Results: Eight hundred thousand neonates were eligible for the screening programme per year. Between 2015 and 2016, screening coverage increased (83.3% vs. 93.8%; p < 0.001), and the refusal rate remained stable (0.1%). In 2016, when considering the additional tests performed several weeks after birth, the proportion of suspected PBNHL neonates decreased (1.4% vs. 0.9%) while the PPV increased (4.7% vs. 7.6%). In 2015, the estimated prevalence of PBNHL (moderate to profound) was 0.09% (95% CI 0.08-0.10). Among neonates with >= 41 decibels deficit, 56.8%, 16.6%, and 26.6% had moderate, severe and profound hearing loss, respectively., Conclusion: The national target of 90% screening coverage was exceeded. The additional test could be useful to avoid overcrowding in diagnostic structures. Diagnostic data quality must be improved to confirm PBNHL prevalence and distribution by severity., (© 2022 Foundation Acta Paediatrica.)

Introduction: Early detection of hearing loss is important to the management of infants and young children with hearing loss. Pediatricians are often the first to encounter a child suspected to have hearing loss. The objective of this study is to evaluate the knowledge and attitude of newborn hearing screening and management of hearing in among pediatricians., Methods: A cross-sectional survey-based study utilizing a validated questionnaire that was distributed to pediatricians in single tertiary academic health care institution that aims to evaluate the knowledge and attitudes related to children with hearing loss and newborn hearing screening., Results: A total of 67 pediatricians agreed to participate and were involved in our study. Mean age was 35 years and 44.1% were males. The majority (79.4%) thought it was very important to have a newborn hearing screening program. However, two-thirds (64.7%) were unsure that there is a universal hearing screening program in the country. Never the less, the majority of the respondents (75%) were at least somewhat confident in explaining the hearing screening program process in our institution. There was a gap in the knowledge of our pediatricians with regard to the process of dealing with a newborn who failed screening program and the candidacy for cochlear implants. Also, some participants were unsure whether to refer a child with hearing impairment to an otolaryngologist or not. Most of the participants thought that physicians need more information related to permanent hearing loss., Conclusion: There are gaps in Pediatricians knowledge and awareness towards hearing loss assessment and management in newborns. This likely requires further academic collaboration between specialties to improve the care of newborns. Future research should focus on the auditory and speech outcome and rehabilitation awareness., Competing Interests: Declaration of competing interest The authors have no conflict of interest to declare., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Background: Universal Newborn Hearing Screening (UNHS) aims to identify infants born with a permanent hearing loss. However, many are also diagnosed with a conductive hearing loss (CHL) and are at subsequent risk for developmental delays. The aim of this study was to investigate the prevalence of CHL and determine which clinical characteristics collected at birth, predict CHL within UNHS., Materials and Methods: Retrospective analysis was conducted on all infants born between January 01, 2007 and December 31, 2018. During this period, 731,234 infants were screened, 9802 were direct referrals, and 1208 identified with a CHL. Chi squared analysis and logistic regression was conducted to determine CHL prevalence and identify which clinical characteristics predict CHL., Results: The prevalence of CHL was 12.32%. Following adjustments for collinearity, clinical characteristics that could predict CHL were: bilateral referrals/medical exclusions to screen (Odds ratio, OR 1.89; 95% CI: 1.65-2.1), ≥1 risk factor for hearing loss (OR 2.03; 95% CI: 1.76-2.34), pre-term birth (OR 1.82; 95% CI: 1.57-2.10), male (OR 1.21; 95% CI: 1.07-1.37), and Indigenous status: 'Aboriginal (not Torres Strait Islander)' (OR 1.27; 95% CI:1.03-1.57 and 'not stated' (OR 2.95; 95% CI: 2.02-4.30)., Conclusion: CHL within UNHS was highly prevalent, with six clinical characteristics that can predict that likelihood of an infant being diagnosed with a CHL. This data could be used to create alternative care pathways for infants with CHL, enabling early and targeted assessments, thereby reducing the risk of developmental delays for these infants., Competing Interests: Declaration of interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Purpose: The purpose of this study was to (a) provide introductory literature regarding cultural constructs, health disparities, and social determinants of health (SDoH); (b) summarize the literature regarding the Centers for Disease Control and Prevention (CDC) Early Hearing Detection and Intervention (EHDI) Hearing Screening Follow-Up Survey (HSFS) data; (c) explore the CDC EHDI HSFS data regarding the contribution of maternal demographics to loss-to-follow-up/loss-to-documentation (LTF/D) between hearing screening and audiologic diagnosis for 2016, 2017, and 2018; and (d) examine these health disparities within the context of potential ethnoracial biases., Method: This is a comprehensive narrative literature review of cultural constructs, hearing health disparities, and SDoH as they relate to the CDC EHDI HSFS data. We explore the maternal demographic data reported on the CDC EHDI website and report disparities for maternal age, education, ethnicity, and race for 2016, 2017, and 2018. We focus on LTF/D for screening and diagnosis within the context of racial and cultural bias., Results: A literature review demonstrates the increase in quality of the CDC EHDI HSFS data over the past 2 decades. LTF/D rates for hearing screening and audiologic diagnostic testing have improved from higher than 60% to current rates of less than 30%. Comparisons of diagnostic completion rates reported on the CDC website for the EHDI HSFS 2016, 2017, and 2018 data show trends for maternal age, education, and race, but not for ethnicity. Trends were defined as changes more than 10% for variables averaged over a 3-year period (2016-2018)., Conclusions: Although there have been significant improvements in LTF/D over the past 2 decades, there continue to be opportunities for further improvement. Beyond neonatal screening, delays continue to be reported in the diagnosis of young children with hearing loss. Notwithstanding the extraordinarily diverse families within the United States, the imperative is to minimize such delays so that all children with hearing loss can, at the very least, have auditory accessibility to spoken language by 3 months of age. Conscious awareness is essential before developing a potentially effective plan of action that might remediate the problem.

Importance: Hearing loss is a global social burden. Early identification of hearing loss missed by newborn hearing screening tests in the neonatal intensive care unit is crucial., Objective: To assess the association between expanded genomic sequencing combined with hearing screening and detection of hearing loss as well as improvement in the neonatal intensive care unit., Design, Setting, and Participants: This cohort study was performed between August 8, 2016, and December 31, 2020, among 8078 newborns admitted to the neonatal intensive care unit of the Children's Hospital of Fudan University in Shanghai, China. Follow-up for hearing status was performed via telephone interviews between September 1 and November 30, 2021., Exposures: A hearing screening test and the expanded genomic sequencing targeting 2742 genes were administered to each patient. Those who failed the hearing screening test or had positive genetic findings were referred for diagnostic audiometry at a median of 3 months of age., Main Outcomes and Measures: The primary outcome was hearing loss missed by hearing screening test. Secondary outcomes were genetic findings and benefits associated with the expanded genomic sequencing for clinical management of patients in the neonatal intensive care unit., Results: Of 8078 patients (4666 boys [57.8%]; median age, 6.3 days [IQR, 3.0-12.0 days]), 52 of 240 (21.7%) received a diagnosis of hearing loss. Expanded genomic sequencing combined with hearing screening was associated with a 15.6% increase (7 of 45 patients) in cases of diagnosed hearing loss that were missed by hearing screening. Of the 52 patients with hearing loss, genetic factors were identified for 39 patients (75.0%); GJB2 and SLC26A4 were the most common genes identified. Patients with genetic findings experienced a more severe degree of hearing loss than those without genetic findings (21 profound, 4 severe, 7 moderate, and 7 mild vs 2 severe, 4 moderate, and 7 mild; P = .005), with more bilateral hearing loss (39 of 39 [100%] vs 9 of 13 [69.2%]; P = .003). Clinical management strategies were changed for patients who underwent genomic sequencing combined with hearing screening., Conclusions and Relevance: This study suggests that expanded genomic sequencing combined with hearing screening may be effective at detecting hearing loss among patients in the neonatal intensive care unit.

Background: Cytomegalovirus (CMV) is the most frequent cause of congenital infection and ≈20% of all infected neonates present or will develop sensorineural hearing loss. Targeted congenital CMV (cCMV) screening in newborns who failed universal newborn hearing screening has been proposed as a strategy to identify neonates with both hearing loss and cCMV infection who could benefit from antiviral treatment implemented within the first month of life., Objectives: To evaluate the feasibility and performance of cCMV targeted screening in a French setting., Methods: Neonates were recruited in 5 maternity centers in greater Paris. A saliva sample for CMV polymerase chain reaction (PCR) testing was collected in neonates who failed newborn hearing screening. Outcomes including CMV PCR result and confirmation of hearing loss by an otorhinolaryngologist specialist were documented., Results: Two-hundred thirty-six newborns were included and a saliva sample was collected in 98% (231/236) of them. The result of CMV PCR was available at a median of 9 days (7-10 days) of life and in 96% of cases within the first month of life. Two neonates were infected with CMV. The result of the otorhinolaryngologist assessment was available in 75% (178/236) of cases at a median of 16 days (9-26 days). Hearing loss was confirmed in 2.8% (5/178). The 2 infected neonates had hearing loss confirmed at 5 and 8 days of life and were treated with valganciclovir at days 9 and 16, respectively., Conclusions: The result of this study confirms that targeted cCMV screening is feasible in these French settings., Competing Interests: Y.V. declares receiving fees for lectures from General Electric outside this work. M.L.-V. declares having received fees paid to her institution for expertise of diagnosis kits and for lectures from BioMérieux and Diasorin outside this work and for participating to conferences from Abbott and Roche. The other authors have no conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Hearing loss is one of the most common congenital defects in infancy; it increases speech and language delays and adversely affects academic achievement and socialemotional development. The risk of hearing loss in premature infants is higher than that in normal newborns, and because of the fragility of the auditory nervous system, it is more vulnerable to different risk factors. The hearing screening guidelines in current use were proposed by the American Academy of Pediatrics and updated in 2007, but there are no uniform guidelines for hearing screening in preterm infants. This review focuses on the risk factors related to hearing loss in premature infants, hearing screening strategies, and reasons for failure. The aim is to provide a more comprehensive understanding of hearing development in preterm infants to achieve early detection and early intervention. At the same time, attention should be paid to delayed auditory maturation in preterm infants to avoid excessive intervention. KEY POINTS: · Hearing loss is very common in infancy, especially in premature infants.. · Genetic factors, infection, hyperbilirubinemia, drugs, and noise are the main causes.. · We should pay attention to the delayed hearing maturity of premature infants and avoid excessive intervention.., Competing Interests: None declared., (Thieme. All rights reserved.)

Importance: Early identification of childhood hearing loss through newborn hearing screening mitigates permanent speech, language, and developmental delays, but many children are lost to follow-up or develop postnatal hearing loss. Early childhood hearing screening programs may help identify these children, but evidence on their outcomes is limited., Objective: To assess outcomes from a low-income, preschool-based hearing screening program and risk factors for hearing loss in this population., Design, Setting, and Participants: A retrospective cohort study of 6820 children aged 2 to 6 years from urban, low-income public preschools who received hearing screening from July 1, 2015, to June 30, 2019, was performed using San Francisco Department of Public Health records. A multivariate logistic regression analyzed risk factors for hearing loss. Data analysis was conducted from January 14, 2020, to April 20, 2021., Exposures: Annual single-visit, 2-tiered screening was implemented with conditioned play pure-tone audiometry (CPA) and distortion product otoacoustic emissions (OAEs)., Main Outcomes and Measures: Rates of successful screening, referred screening, loss to follow-up, and hearing loss., Results: Of 6820 children (age, 2-6 years) screened, 3425 (50.2%) were boys, 15% were White/non-Hispanic, and 48% had English as the primary home language. A total of 403 (5.9%) children were referred for full medical or audiologic evaluation after 2-tiered CPA/OAE screening. Only 24 children were unable to complete both CPA and OAE testing for a screening completion rate of 99.6%. After medical evaluation, 114 of 403 children (28.3%) passed hearing rescreening and 55 (13.6%) were lost to follow-up. The prevalence of conductive hearing loss was 2.9% (n = 195), and the prevalence of sensorineural hearing loss was 0.2% (n = 13). Primary language, race and ethnicity, and sex were not associated with rates of referral or hearing loss., Conclusions and Relevance: The findings of this cohort study suggest that preschool-based screening programs can be a useful method to identify early childhood hearing loss and that teacher concerns are associated with final diagnostic hearing status.

Objective: To evaluate the impact of the Universal Neonatal Hearing Screening (UNHS) on the age at diagnosis, beginning of treatment, and first cochlear implant surgery., Methods: A retrospective cohort study with children up to 12 years old with bilateral hearing loss were divided into two groups: patients who underwent UNHS and the ones who didn't. The groups were compared according to their age at the beginning of the evaluation at a specialized center, at the beginning of the intervention, and, for the ones who had indication, at the cochlear implant surgery. The group who underwent UNHS was divided between the ones who passed the screening test and the ones who didn't. They were compared according to their ages at the same moments as the first two groups., Results: 135 patients were included. The median age at the first appointment in a specialized center was 1.42 (0.50 and 2.50) years, at the beginning of treatment 2.00 (1.00 and 3.52) years, and the cochlear implant surgery 2.83 (1.83 and 4.66) years. Children who underwent UNHS were younger than those who didn't, at the three evaluated moments (p < 0.001). In a subanalysis, children who passed the UNHS but were later diagnosed with hearing loss reached the first appointment with a specialist and started treatment older than those who failed the tests., Conclusion: Performing UNHS interfered with the timing of deafness diagnosis and treatment. However, children who passed the screening but were later diagnosed with hearing loss were the category with the most important delay., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (Copyright © 2021 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Aim: To evaluate and describe results of aetiological investigations offered to a population level cohort of babies who had confirmed permanent hearing loss after they either (i) failed universal neonatal hearing screening or (ii) passed newborn screening but were detected with a permanent hearing loss in early childhood., Methods: Descriptive analysis of results of investigations offered to neonates and young children in whom permanent hearing loss was detected as part of a statewide newborn hearing screening programme. A total of 306 285 newborns were screened between 2013 and 2017. The failed screening results were confirmed by a diagnostic audiological assessment battery. Medical evaluation for the identification of the cause of the hearing loss was performed by a paediatrician or otolaryngologist, investigations were ordered using a stepwise approach, and aetiology was assigned using a coding scheme., Results: Permanent hearing loss was confirmed in 967 children (0.3%). Data were available for 873. An aetiological factor was identified or presumed in 61.3% of cases. Genetic causes were present in 26.8% and structural causes were present in 24.9% of cases. Congenital cytomegalovirus was present in 4.4%., Conclusions: Use of a coding scheme is feasible at a population level and allows collation of data from multiple sites and will allow outcome mapping and service planning., (© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Aim: To assess the degree to which timely audiological assessment of congenital hearing loss is achieved at our institution - Perth Children's Hospital, Western Australia, and to review cases which breached this timeframe in order to address barriers to timely assessment. The benchmark used to determine timely assessment is that set out by The Joint Committee on Infant Hearing (JCIH) in which diagnostic audiological testing occurs by three months of age for those who do not pass newborn hearing screening., Methods: A retrospective chart review of infants who underwent diagnostic auditory assessment at Perth Children's Hospital between 2016-2019. A total of 151 children were identified as meeting the inclusion criteria and their medical files were reviewed. Time to first dABR was the time point for whether testing was achieved within the 3 month timeframe., Results: Of the 151 children who underwent dABR assessments, 1 was identified as having breached the 90 day time limit (tested on day 91) for which no valid reason for delay could be identified. The timely delivery of dABR assessments in 99.3% of cases within this cohort compares favourably with the literature., Conclusions: Conclusion Timely diagnostic audiological assessment is achievable for children with congenital hearing loss. The reasons for patients breaching this timeframe are explored in the paper along with factors which may help avoid delays., (© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Objectives: Neonatal intensive care unit (NICU) patients are at high risk for congenital hearing loss. Previous studies have found sociodemographic factors associated with loss to follow-up for newborn hearing screening, but none have specifically studied the NICU population. Our objective is to determine if demographics and socioeconomic status is associated with loss to follow-up in a newborn population with extended NICU stay., Design: A retrospective cohort study was conducted on 443 NICU infants with extended NICU stay utilizing data extracted from infant and maternal medical records at an urban safety-net hospital., Results: Younger maternal age (adjusted odds ratio [OR] 0.95, confidence interval [CI] 0.91 to 0.99), higher gravidity (adjusted OR 1.39, CI 1.12 to 1.72), and former smoking status (adjusted OR 2.57, CI 1.07-6.18) were identified as independent predictors of loss to follow-up for NHS after conducting a multivariable logistic regression. Demographic and socioeconomic variables, such as sex, parity, birth weight, mode of birth, highest level of maternal education, maternal race/ethnicity, zip code metrics, and maternal language were not found to be associated with loss to follow-up., Conclusions: Maternal age, gravidity, and smoking status are risk factors for loss to follow-up for NHS in newborns with extended NICU stay, a group at high risk for hearing loss. Our findings demonstrate that socioeconomic and demographic factors for loss to follow-up in the extended-stay NICU population are distinct from the well-baby population. Further investigation of these patients will allow prioritization of limited resources to subgroups within the extended-stay NICU population at risk for loss to follow-up for newborn hearing screening., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To detect common pathogenic variants associated with congenital deafness among neonates from Huizhou and surrounding areas and discuss its implications., Methods: Thirteen hot-spot mutations in four most common pathogenic genes were screened among 20 934 neonates from March 2017 to December 2019., Results: In total 760 neonates were found to carry common pathogenic variants (3.63%). Sixty two neonates have carried homozygous/compound heterozygous variants or homoplasmy/heteroplasmy mutations of mtDNA (0.29%). Further analysis of five abnormal cases revealed that 3 of them have carried compound heterozygous mutations of GJB2 gene, and 2 were due to compound heterozygous variants of the CDH23 gene., Conclusion: Genetic testing has a great clinical significance for the prevention and reduction of congenital hearing loss, but the scope needs to be updated and redefined by removing mutation sites with a very low rate, adding new significant sites, and improvement of the technical strategies.

Compelling evidence indicates that some newborns harboring genetic variants associated with hearing loss might not be identified by current physiologic newborn hearing screening (NBHS) rendering current NBHS protocols suboptimal. Incorporating genomic sequencing into NBHS would improve clinical diagnosis and decrease time to early intervention efforts., Competing Interests: Disclosure The authors have no disclosures to report., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: Newborn hearing screening can identify congenital deafness and hearing loss. The current status of newborn hearing screening in the south of China is unclear. We aimed to assess the hearing loss of newborns in Dongguan, China., Methods: A total of 62,545 newborns were enrolled in this retrospective, cross-sectional study between September 2015 and August 2020. The screening procedure was carried out using a two-step hearing screening. The trends were examined by the Cochran-Armitage trend test., Results: From 2015 to 2020, the total initial newborn hearing screening rate was 98.16%, and it significantly increased over time (Z = 2.488). The initial screening pass rate of newborns was 90.08%, and no significant difference was observed in the initial screening pass rate between different years (Z = 0.845). After two-step hearing screening, the overall hearing screening pass rate of newborns was 94.65%. The overall hearing screening pass rate in normal newborns was higher than that in high-risk newborns (95.70% vs. 93.59%)., Conclusion: The initial newborn hearing screening rate increased yearly in the study period, but there was still an approximately 10% referral rate. The initial screening pass rate in China needs to be further improved.

Congenital cytomegalovirus (cCMV) is the most common nongenetic cause of sensorineural hearing loss. Despite its prevalence, universal screening for cCMV is not currently performed. Hearing loss caused by cCMV is most often severe to profound, often bilateral, and may be fluctuating or progressive. Infants with hearing loss at birth and confirmed cCMV might benefit from antiviral therapy. Roughly half of hearing loss cases owing to cCMV are delayed in onset, and consequently, these children pass newborn hearing screening. Children with cCMV require close audiologic monitoring, require appropriate management with hearing aids, and should be monitored for cochlear implant candidacy., Competing Interests: Disclosure Dr L.B. Mithal and Dr S.R. Hoff are site investigators in the ValEAR Trial, NIHU01 DC014706 (PI: Park)., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: This study investigated whether demographic variables, risk factor presence or absence and universal newborn hearing screening (UNHS) results can be used to predict permanent childhood hearing loss (PCHL) in infants referred from screening., Design: Retrospective analysis of a UNHS database., Study Sample: Data were extracted from the state-wide UNHS database storing details of the 613,027 infants who were born in Queensland, Australia between 1 January 2007 and 31 December 2016 and participated in UNHS. This study included the 6735 children who were referred from the UNHS program for diagnostic audiology due to failing the screen in one or both ears or bypassing screening., Results: Factors with a significant positive association with PCHL that were incorporated into a logistic regression model were: female gender, non-indigenous status, family history of PCHL, craniofacial anomalies and syndromes associated with PCHL, and a bilateral refer result on screening., Conclusions: Odds of PCHL vary among infants referred for diagnostic assessment from UNHS programs. When an infant refers on the newborn hearing screen, information about their gender, indigenous status, identified risk factors and specific screening outcome can be used to predict the likelihood of a congenital PCHL diagnosis.

Purpose The current Joint Committee on Infant Hearing guidelines recommend that infants with syndromes or craniofacial abnormalities (CFAs) who pass the universal newborn hearing screening (UNHS) undergo audiological assessment by 9 months of age. However, emerging research suggests that children with these risk factors are at increased risk of early hearing loss despite passing UNHS. To establish whether earlier diagnostic audiological assessment is warranted for all infants with a syndrome or CFA, regardless of screening outcome, this study compared audiological outcomes of those who passed UNHS and those who referred. Method A retrospective analysis was performed on infants with a syndrome or CFA born between July 1, 2012, and June 30, 2017 who participated in Queensland, Australia's state-wide UNHS program. Results Permanent childhood hearing loss (PCHL) yield was higher among infants who referred on newborn hearing screening (51.20%) than in those who passed. Nonetheless, 27.47% of infants who passed were subsequently diagnosed with hearing loss (4.45% PCHL, 23.02% transient conductive), but PCHL was generally milder in this cohort. After microtia/atresia, the most common PCHL etiologies were Trisomy 21, other syndromes, and cleft palate. Of the other syndromes, Pierre Robin sequence featured prominently among infants who passed the hearing screen and were subsequently diagnosed with PCHL, whereas there was a broader mix of other syndromes that caused PCHL in infants who referred on screening. Conclusion Children identified with a syndrome or CFA benefit from early diagnostic audiological assessment, regardless of their newborn hearing screening outcome.

Objectives: We identified the neonatal and maternal risk factors for hearing loss (HL) in children using National Health Insurance Service data of Korea., Methods: We retrospectively analyzed data from the National Health Insurance Service. Infants born from 2007 to 2013 were tracked to 2015. Those diagnosed with hearing disabilities or who underwent cochlear implant surgery were included in the hearing disability group. We compared the incidence of any diagnosed disability other than a hearing disability; any maternal disability at delivery; maternal age at delivery; prenatal and neonatal Toxoplasma, syphilis, rubella, cytomegalovirus, and herpes simplex infections; craniofacial anomaly; low birth weight, hyperbilirubinemia, and bacterial meningitis; neonatal intensive care unit (NICU) admission for > 5 days; exchange transfusion; and ototoxic drug use (aminoglycosides or loop diuretics), between the hearing disability and control groups., Results: The total number of newborns came to 3,164,825. Risk factors were sought in a hearing disability group (n = 847) compared to a control group (n = 2508). A diagnosed disability other than a hearing disability, which was commonly a brain lesion, the use of ototoxic drugs, NICU admission for >5 days, and a maternal disability at delivery, which was commonly a hearing disability, were significant neonatal and maternal risk factors for HL in children., Conclusions: Accompanying brain lesions, maternal hearing disabilities at delivery, use of ototoxic drugs during the neonatal period, and hospitalization in NICU for >5 days were significant risk factors for HL in children, as revealed by analysis of population-based data., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Objectives: We have clarified the role of Universal Neonatal Hearing Screening (UNHS) for both early diagnosis and rapid treatment in order to improve the prognosis of the deaf child and reduce patient management costs. Although in Sicily UNHS has been progressively implemented, there is scarce data in the literature on this matter. Therefore, the main objective was to collect in the year 2018 the following data: number of newborns screened for hearing loss, number of infants "referred" to transiently evoked otoacoustic emissions (TEOAE), number of infants with pathologic auditory brainstem response (ABR) and number of infants affected by permanent hearing loss., Methods: UNHS monitoring was conducted through the collection of data through a questionnaire, which was analysed evaluating the effectiveness and adherence to the screening program prepared by the Department for Health Activities and the Epidemiological Observatory (DASOE)., Results: In 2018, there were 40,243 newborns in Sicily. A total of 37,562 newborns were screened (93.3%). There were 1,328 "referred" infants with TEOAE (3.5%). On the 2 nd level, "referred" newborns examined were 1,080 of 1,328 expected (missing 248 "refer" newborns, equal to 18.6%). The number of "referred" infants confirmed with TEOAE was 113 of 1,080, while "referred" infants confirmed with ABR were 71. On the 3 rd level, 67 of 71 were infants examined: 28 infants were suffering from monolateral hearing loss (13 slight/mild, 13 moderate, 1 severe and 1 profound) and 39 from bilateral hearing loss (1slight/mild, 19 moderate, 13 severe and 7 profound). Excluding 7 infants from the NICU, 60 of 37,562 infants had hearing loss (1.5%)., Conclusions: The monitoring of the UNHS in Sicily has allowed obtaining the data of individual centres, absent in the literature to date, to verify the effectiveness of the screening, according to JCIH criteria, to highlight some criticalities and, finally, to propose possible solutions., (Copyright © 2021 Società Italiana di Otorinolaringoiatria e Chirurgia Cervico-Facciale, Rome, Italy.)

Background: The aim of the present study was to determine the possible relationship between cultured microorganisms and hearing loss in infants admitted to the neonatal intensive care unit (NICU) who could not pass a standard hearing test., Method: The medical records of infants treated at the NICU were retrospectively evaluated. The patients were first divided into two groups, and group 1 was divided into two subgroups: Group 1 included patients with hearing loss accompanied by proven sepsis caused by either gram-negative (group 1A) or gram-positive (group 1B) bacteria, and group 2 included patients with clinical sepsis. The groups were compared with potential risk factors related to hearing loss., Results: Between January 2014 and January 2019, the cases of 3,800 infants admitted to the NICU were reviewed. Of 3,548 living babies, the Auditory Brainstem Response (ABR) test showed that 35 infants (0.98%) were diagnosed with hearing loss. In 12 infants with hearing loss, microbial growth in the blood cultures was detected, whereas in the remaining 23, the blood cultures were negative. Of the cases with microbial growth, five were gram negative and seven were gram positive. In the comparison of groups 1A, 1B, and 2, there were statistically significant differences in terms of risk factors such as low birth weight (p = .048), neonatal hospitalization time (p = .001), free oxygen support (p = .001), intraventricular bleeding (p = .001), loop diuretic use (p = .001), and blood transfusion (p = .048)., Conclusion: The relationship between hearing loss and microorganisms causing sepsis could not be determined in this research., (© 2021 Wiley Periodicals LLC.)

Objective: To explore the trends in actions and factors influencing families of children with hearing loss, regarding early treatment following the implementation of a newborn hearing screening (NHS) in Taiwan., Design: A retrospective study was conducted by extracting data from the treatment histories of families with children who had hearing loss and who were contacted and assessed by the non-profit organisation (NPO). Children born between 2012 and 2018 were included. The time lapse between initial NHS and early treatment sought by each family was documented and the factors predicting the timing of those actions were identified., Study Sample: Data of 2095 families of children with hearing loss and who received treatment from an NPO for the annual birth cohort of 2012 through 2018 were included., Results: The median age at treatments initiation improved through the years. Parental educational level and the child's degree of hearing loss were significant indicators of the child's initial age at treatment intervention, age when hearing aids were fitted, and days taken to contact the organisation., Conclusion: The NHS in Taiwan promoted earlier treatment for children with hearing loss. Furthermore, patient instructions and education may be important to enable parents to take actions for early intervention.

The prevalence of permanent childhood hearing impairment (PCHI) in infants admitted to a neonatal intensive care unit (NICU) is higher than that in the general population. Our study objective was to identify risk factors associated with PCHI in infants who required admission to the NICU for more than 48 h. We performed a case-control study, including infants of all gestational ages who were admitted to NICU for more than 48 h and who underwent newborn hearing screening between 2005 and 2019. Infants admitted to NICU and diagnosed with PCHI by formal audiology were classified as "cases". The "controls" were infants who were admitted to NICU and did not have PCHI. Cases and controls (1:4) were matched based on their birth gestation, birthing place, and treating NICU. The prevalence of PCHI in infants admitted to NICU was 6.3% as compared with our general population prevalence of 0.25%. There were 77 cases and 269 controls during the study period. The median age at diagnosis of PCHI in these infants was 132 days (interquartile range 75.5-518.5). Using regression analysis, "any ventilation episodes", presence of seizures, and major congenital anomalies were significantly associated with PCHI in infants of all gestational ages. There were higher prevalence of PCHI in preterms (<32 weeks) who received furosemide and lower prevalence with antenatal use of magnesium sulphate.Conclusions: In our study, the prevalence of hearing loss was high in infants admitted to NICU. Gestation-specific risk factors identified in this case-control study would help in counselling of parents. What is Known: • In the UK, 1-2/1000 infants are born with hearing loss and infants admitted to the neonatal unit for 48 h or more have increased prevalence of hearing loss (1 in 100 live births). • Identification of risk factors in infants admitted to neonatal unit would help with risk stratification and further management. What is New: • In our study, infants admitted to the neonatal unit had higher prevalence of hearing loss (6.3 in 100 live births). • In infants across all gestational age "any ventilation episodes", presence of seizures, and severe congenital anomalies were associated with a statistically significant increase in prevalence of hearing loss. Higher prevalence of hearing loss was noted in preterm infants (<32 weeks) who received furosemide treatment and lower prevalence was noted with antenatal use of magnesium sulphate.

Purpose: The current study aimed to describe the medical case history factors in a group of neonates admitted to high care and Kangaroo Mother Care wards at two hospitals in Gauteng, South Africa and to explore the relationship between specific case history factors and audiological outcomes., Methods: This study was part of a bigger study titled "Early detection of hearing loss: exploring risk-based hearing screening within a developing country context" where all case history data were recorded from participant medical files at the time of an initial hearing screening, through the use of a case history form that was developed for this study. Results were analyzed using descriptive statistics. The relationship between case history factors and audiological outcomes was analyzed using Fisher's exact test., Results: Findings revealed that, of all the case history data, preterm birth (95.7%), exposure to ototoxic medication (87.7%), neonatal jaundice (NNJ) (80.6%), and birthweight below 1500 g (66.7%) were the most frequently occurring case history factors in this South African sample. No statistically significant association was found between these frequently occurring case history factors and the repeat hearing screening outcomes in this sample., Conclusions: Risk factors for hearing impairment cannot be viewed in isolation but should be considered in relation to their interaction with multiple other risk factors. Contextually understanding the profile of high risk has implications for medical intervention, as well as for the field of Audiology where planning for early identification and intervention services for hearing impairment is vital. Such contextually relevant evidence allows for a snap shot of how this population presents, what their needs and requirements are, and their possible future requirements; hence, raising important planning implications for the department of health and for targeted newborn hearing screening as well as early childhood intervention within the South African context.

Introduction: Despite the high burden of hearing loss (HL) globaly, most countries in resource limited settings lack infant hearing screening programs(IHS) for early HL detection. We examined the feasibility of establishing an IHS program in this setting, and in this pilot program measured the prevalence of infant hearing loss (IHL) and described the characteristics of the infants with HL., Methods: We assessed feasibility of establishing an IHS program at a regional referral hospital in south-western Uganda. We recruited infants aged 1 day to 3 months and performed a three-staged screening. At stage 1, we used Transient Evoked Oto-acoustic Emissions (TEOAEs), at stage 2 we repeated TEOAEs for infants who failed TEOAEs at stage 1 and at stage 3, we conducted Automated brainstem responses(ABRs) for those who failed stage 2. IHL was present if they failed an ABR at 35dBHL., Results: We screened 401 infants, mean age was 7.2 days (SD = 7.1). 74.6% (299 of 401) passed stage 1, the rest (25.4% or 102 of 401) were referred for stage 2. Of those referred (n = 102), only 34.3% (35 of 102) returned for stage 2 screening. About 14.3% (5/35) failed the repeat TEOAEs in at least one ear. At stage 3, 80% (4 of 5) failed the ABR screening in at least one ear, while 25% (n = 1) failed the test bilaterally. Among the 334 infants that completed the staged screening, the prevalence of IHL was 4/334 or 12 per 1000. Risk factors to IHL were Newborn Special Care Unit (NSCU) admission, gentamycin or oxygen therapy and prematurity., Conclusions: IHS program establishment in a resource limited setting is feasible. Preliminary data indicate a high prevalence of IHL. Targeted screening of infants at high risk may be a more realistic and sustainable initial step towards establishing IHS program s in a developing country like Uganda., Competing Interests: The authors have declared no competing interests.

Objective: To identify children with postnatal hearing loss, a structured monitoring system is needed. The goal of this study was to describe a targeted surveillance program in Italy to identify children with postnatal hearing loss., Methods: Between January, 2013, and December, 2016, all children who received bilateral 'pass' result at the newborn hearing screening, and who were identified as having at least one risk factor, were referred for targeted surveillance. The hospital records of these children were retrieved., Results: Among children enrolled, 66 were identified with permanent hearing loss. The most frequent risk factors were family history (35%), prematurity (25.5%), low birthweight (19.2%), severe hyperbilirubinemia (19%), prolonged ventilation (15%) and congenital infection (12.5%)., Conclusions: An audiological surveillance program in newborns who 'pass' in neonatal screening, but have risk factors, is effective in identifying permanent postnatal hearing disorders.

Neonatal hearing screening has been developped in a large number of countries. The rational to build such nationwide programs is robust. The prevalence of hearing impairment of various etiologies is high (1/1,000), diagnosis of hearing impairment in infants is uneasy and is made most of the time after the age of 18 months when treatment is less efficient and, last, appropriate test to screen for hearing impairment are available: Otoacoustic Emission and Auditory Evoked Potential. In France the screening is organised at the regional level. The organization of such a program is complexe. Midwifes and nurses should be trained to informed the parents and to perform the test. If the test is abnormal the infant will be oriented to a specialzed department of pediatrics for appropriate diagnosis and treatment., (© 2021 médecine/sciences – Inserm.)

Introduction: Neonatal hearing screening in France involves confirmation by a childhood hearing expert centre in case of suspected hearing loss. Although click-evoked air-conduction auditory brainstem responses (AC-ABR) are the gold standard in France, there are no guidelines for bone-conduction ABRs (BC-ABR). The present study assessed the interest of associating click-evoked BC- and AC-ABRs for diagnostic confirmation in neonatal hearing screening., Materials and Methods: A retrospective study included 59 infant ears with conductive hearing loss referred to the centre of Lyon, France. Objective hearing thresholds were compared between click-evoked BC- and AC-ABRs on a method previously validated in a normal-hearing population., Results: There was a significant difference in mean threshold between AC-ABR (53.27±1.189 dBnHL) and BC-ABR (28.1±0.935 dBnHL) (P<0.001). AC thresholds ranged from 40 to 60 dBnHL while BC thresholds exceeded 40 dBnHL in only 9 ears., Conclusion: Using BC-ABRs could reduce the false-positive rate in neonatal bilateral permanent hearing loss screening, in complement to AC-ABRs using the same stimulus. Click-evoked BC-ABR could be contributive whenever conductive hearing loss is suspected, in complement to AC-ABR, without unreasonably increasing examination time., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objective: To characterize the association between hyperbilirubinemia and a failed newborn hearing screen in infants born at 22-32 weeks of gestation., Study Design: We included infants with gestational ages of 22-32 weeks who were discharged from neonatal intensive care units in the US from 2002 to 2017 with available newborn hearing screen results obtained after 34 weeks postmenstrual age. We excluded infants with severe birth asphyxia or craniofacial abnormalities. We identified 95 672 infants from 313 neonatal intensive care units. We used multivariable logistic regression to examine the association between maximum total bilirubin at <21 days postnatal age with failed hearing screen, adjusting for important demographic and clinical risk factors., Results: The median gestational age and birth weight were 30 weeks (IQR, 28-32 weeks) and 1330 g (IQR, 1010-1630 g), respectively. The median maximum total bilirubin was 8.3 mg/dL (IQR, 6.7-10.0 mg/dL), and 5275 infants (6%) failed their newborn hearing screen. On adjusted analysis, each 1 mg/dL increase in maximum total bilirubin was associated with a small, but significant, increase in odds of a failed hearing screen (OR, 1.03; 95% CI, 1.02-1.04)., Conclusions: An increased maximum total bilirubin level was independently associated with hearing screen failure. Further prospective studies are needed to understand whether this increased risk of hearing screen failure translates to increased risk of hearing loss., (Copyright © 2020 Elsevier Inc. All rights reserved.)