Main outcomes from the first 2 years of France's screening programme for neonatal permanent hearing loss through a descriptive study.

Hearing loss in infants is often diagnosed late, despite universal screening programmes. Risk factors of hearing impairment in high-risk neonates, identified from population-based studies, can inform policy around targeted screening. Our aim was to determine the prevalence and the risk factors of hearing loss in a high-risk neonatal population. This was a retrospective cohort study of neonates hospitalised at the University Hospital Cologne, Germany from January 2009 to December 2014 and were part of the newborn hearing screening programme. Multivariable regression analyses using the lasso approach was performed. Data were available for 4512 (43% female) neonates with a mean gestational age at birth of 35.5weeks. The prevalence of hearing loss was 1.6%, and 42 (0.9%) neonates had permanent hearing loss. Craniofacial anomalies, hyperbilirubinaemia requiring exchange transfusion, oxygen supplementation after 36weeks of gestation and hydrops fetalis showed associations with permanent hearing loss. Our findings of risk factors for hearing loss were consistent with other studies. However, some commonly demonstrated risk factors such as perinatal infections, meningitis, sepsis and ototoxic drugs did not show significant associations in our cohort. Targeted screening based on risk factors may help early identification of hearing loss in neonates.

Associational study of neonatal hearing screening results and common metabolic disorders.

Background and aims Studies have shown an association between Neonatal hearing loss (NHL) and preterm birth and low birth weight, but the prevalence of NHL more specific within these categories is not known. Aim of this study is to present the risk of NHL by week of gestation and categories of birth weight in an nationwide cohort of neonates born Methods Results of the two-stage Automated Auditory Brainstem Response (AABR) Universal Newborn Hearing Screening Program in Dutch neonatal intensive care units and diagnostic examination were centrally registered between October 1998 and December 2012 and included in this study. NHL was defined as impaired when the neonates’ conventional Auditory Brainstem Response (ABR) level exceeded 35 dB in one (unilateral) or two (bilateral) ears at diagnostic examination. Birth weight was stratified into Results In total 18,564 neonates with a gestational age between 24.0 and 31.9 weeks were eligible for this study. Logistic regression analyses revealed significant associations between gestational age, birth weight and both unilateral and bilateral NHL (all p Conclusions Gestational age and birth weight are independently associated with NHL in a dose-response relationship. This information can be used to gain insight into health and related costs associated with very preterm births.

Despistaje de la hipoacusia neonatal: resultados después de 3 años de iniciar nuestro programa

The effects of hypoxia, premature birth, infection, ototoxic drugs, circulatory system and congenital disease on neonatal hearing loss

BackgroundTo date, data on the prevalence and age at first management of permanent childhood hearing loss in Germany are lacking.ObjectiveThis study aims to depict how often and at what age children and adolescents receive their (first) hearing technology.Materials and methodsIn this study, we analyzed claims data from a large German statutory health insurance company (BARMER). A cross-sectional study determined the provision of hearing devices and cochlear implants for children and adolescents aged under 18 years with permanent hearing loss from 2010 to 2020. A longitudinal analysis of a cohort of children born in 2010 was performed to gain insights about age at first management with hearing technology during the first 10 years of life.ResultsBetween 2010 and 2020, approximately 2800 to 3600 children and adolescents per year were provided with hearing devices and 10 to 30 with cochlear implants. In the 2010 birth cohort, 1.22% of children received their first prescription for hearing devices before the age of 10. The proportionately highest number of first prescriptions was found between 3 and 6 years. In 2020, particularly few children gained access to hearing technology.ConclusionThe analysis of the prescribed hearing systems reveals inaccuracies in documentation but also a possible care gap in hearing loss management. For a large percentage of children and adolescents, management of hearing loss took place after the age of 1. The frequent initial provision of hearing technology at preschool age indicates that the proportion of hearing loss that is acquired, detected late, or treated late remained quite high even after the introduction of newborn hearing screening. There is an obvious need for comprehensive tracking of children who fail newborn hearing screening and for other screening and hearing tests. The data for 2020 suggest that hearing loss was diagnosed and treated later due to the COVID-19 pandemic.

Infants admitted to neonatal intensive care units are at increased risk of hearing loss, yet early identification remains challenging. Understanding circulating biomarkers of hearing loss may improve risk stratification and inform targeted surveillance and intervention. To assess whether biological factors measured in bodily fluids predict hearing loss in neonates admitted to the neonatal intensive care. Systematic review and meta-analyses were conducted (registration ID: CRD42024531492). Comprehensive searches were undertaken to identify peer-reviewed studies published between 2000 and 2025 evaluating fluid biomarkers and hearing outcomes in neonates. Risk of bias was assessed across five domains. Biomarkers were grouped by biological domain and synthesised narratively. Where feasible, random-effects meta-analyses were performed using odds ratios and restricted maximum likelihood estimation for calculation of pooled effect sizes. Eighty-six studies were included, predominantly retrospective cohorts with substantial methodological heterogeneity. Elevated bilirubin exposure (n = 44 studies) was consistently associated with hearing loss, with unbound bilirubin demonstrating superior predictive performance. Infective conditions (n = 32 studies), particularly congenital cytomegalovirus, meningitis, and invasive fungal infections showed significant associations with hearing loss. Risk of bias was moderate to high across studies, driven by inconsistent hearing outcomes measures, poor repeatability of biomarker measures and limited inclusion of risk factors and confounders which affect biomarker concentrations. Systemic neonatal biomarkers, particularly unbound bilirubin and infective markers, show consistent associations with hearing loss, though high heterogeneity limits precision. Evidence was heterogeneous and largely exploratory. Future studies should utilise integrated databases where data have rigorous confounder adjustment, standardised biomarker assessments, and validated hearing outcome measures to identify clinically meaningful biomarkers of hearing loss. https://www.crd.york.ac.uk/PROSPERO/, identifier CRD42024531492.

Background - Cytomegalovirus (CMV) infection is the most common of congenital infections, with a prevalence of 0.3-1.2% in industrialized countries and is the most frequent non-genetic cause of permanent hearing loss in children, with an incidence ranging from 0.2 to 2%. Since there is no antenatal therapy or vaccine against CMV and since many patients with congenital CMV (cCMV) do not experience sequelae even in the absence of specific therapy, a universal screening programme has not been established. Current protocols provide test for the detection of cCMV in case of failure at newborn hearing screening. Aim - This article aims to analyze the pros and cons of the different screening approaches for cCMV, through an overview of the topic based on the most recent literature. Materials and Methods - The recent national and international literature on cCMV screening has been examined through a narrative review. Results - As part of the screening and early identification programmes for neonatal hearing loss, current procedures include a search for cCMV on urine or saliva within 3 weeks of life, following a positive result (Refer) at newborn audiological screening. This approach enables to recognize cCMV infection in newborns suffering from isolated hearing loss, which is already present at birth, however it does not allow cases of CMV-related hearing loss occurring in the postnatal period or late in childhood to be recognized. A universal newborn cCMV screening, in conjunction with audiological screening, would have the advantage of identifying all infants infected with cCMV, including 10%-15% of asymptomatic conditions at birth who may develop late hearing loss. One of the major obstacles to the establishment of universal cCMV screening is the unknown cost of its application and a clear understanding of the sensitivity and specificity of the cCMV test. Conclusions - Strategies are needed to identify children with congenital CMV infection at risk for developing late-onset hearing loss. A universal newborn screening to ensure timely recognition of children with cCMV, close audiological surveillance and possible early treatment at the onset of the first hearing symptoms should be provided.

Evaluation of Hearing Loss after Failed Neonatal Hearing Screening

Aim: The study aimed to assess the awareness and attitude of the mothers regarding neonatal hearing loss (NHL), its risk factors, and available detection and intervention choices.Material and Methods: A cross-sectional study design will be conducted among mothers in the Maternity and Children Hospital in Arar, northern Saudi Arabia.Results: A total of 200 mothers were included in the study, with a mean age 35.510.1 years.Most of the mothers were aware of risk factors for NHL such as middle ear infections (138, 69%) head trauma and/or ear slaps (134, 67%), family history (128, 64%).However, the participants were not aware of several other risk factors neonatal jaundice (32, 16%) and prematurity (49,24.5%).Discussion: The current study provides data on maternal knowledge and attitude of NHL in northern Saudi Arabia.There is insufficient knowledge regarding some risk factors such as maternal infection during pregnancy, neonatal jaundice, viral infection, head trauma and consanguineous marriages.The results show the maternal awareness gap that exists in the area.

ObjectiveTo describe the frequency and types of hearing loss in children with syndromic and non-syndromic craniosynostosis.DesignRetrospective cohort study.SettingLarge tertiary pediatric hospital.PatientsChildren with craniosynostosis that underwent at least one audiological evaluation between the years of 2010 and 2021 at a single institution. Hearing loss was defined as conductive or permanent (sensorineural or mixed).ResultsOf 130 total children examined, 107 (82.3%) had non-syndromic craniosynostosis and 23 (17.7%) had syndromic craniosynostosis. Within the non-syndromic cohort, 77 (72%) had normal hearing and 30 (28%) had hearing loss, of which 21/30 (70%) had conductive hearing loss and 9/30 (30%) had permanent hearing loss. Within the syndromic cohort, two (9.5%) had normal hearing and 21 (90.5%) had hearing loss, of which 16/21 (76.2%) had conductive hearing loss and 5/21 (23.8%) had permanent hearing loss. Multivariable analysis involving syndromic status, anatomical type of craniosynostosis, and medical complexity revealed that patients with syndromic craniosynostosis had higher odds for conductive and permanent hearing loss (49.80 OR, P = .002 and 34.91 OR, P < .05, respectively). Patients with unicoronal craniosynostosis and those with significant medical comorbidities had higher odds for permanent hearing loss (19.50 OR, P = .03 and 23.36 OR, P < .05, respectively).ConclusionsChildren with craniosynostosis had high rates of hearing loss, especially those with syndromic or unicoronal craniosynostosis and those with significant medical comorbidities. Twenty-eight percent of patients with non-syndromic craniosynostosis also had hearing loss. Larger prospective studies are necessary to more precisely estimate hearing loss associated with non-syndromic craniosynostosis.

Permanent congenital hearing loss in children does not only influence speech acquisition but also has a severe impact on the entire intellectual and psychological development, and thus social integration is handicapped. However, at least partial compensation can be achieved by complex rehabilitative measures. One of the first medical therapies in children with congenital hearing loss is to equip them with hearing aids. As part of quality management the purpose of this study is to discuss pedaudiological aspects and results of hearing - impaired children equipped with hearing aids against the background of guidelines from Germany and the US. Data of 107 children equipped with hearing aids from 1997 to 1999 were analysed referring to the relevant German and US guideline. In 53 girls and 54 boys, 45% were suffering from at least severe, 45% from moderate and 10% from mild hearing loss. 93 children presented sensory hearing loss, 8 combined and 2 conductive hearing loss. 2 children had unilateral hearing loss, one a combined on one ear and a sensory hearing loss on the other and one child had a central auditory processing disorder. The age when the hearing loss was diagnosed depended on its grade and correlated with adverse environmental aspects. 34 children suffered from a syndrome or multiple handicaps, in these cases, the hearing loss was diagnosed at a later date than in children suffering from hearing loss only. In cases with near deafness audiological results with hearing aids were not sufficient; in these cases, cochlea implantation had to be discussed as an alternative measure. The data obtained from this study underline the need for universal hearing screening. Discussing the data against the relevant German and US guidelines clearly shows that German benchmarks are not as strict as the American ones. This has to be taken into account for further guideline development in Germany. As permanent hearing loss in children with syndromes and multiple handicaps and children with adverse environment is diagnosed at a later date than in other children public, measures have to be taken to improve this situation.

Objective:Viral infections are known to be a risk factor for neonatal hearing loss. COVID-19 infection has been reported to affect hearing test results in one small sample sized study. We aimed to investigate the incidence the risk of neonatal hearing loss in infants of mothers who had COVID-19 infection during pregnancy, regarding their trimesters, by evaluating the neonatal hearing screening results.Design:In this retrospective case-control study, neonatal hearing test results of 458 women with a history of COVID-19 infection in pregnancy were compared with 339 women who gave birth before the pandemic. Data of pregnant women who attended the COVID-19 outpatient clinic of the emergency service of a tertiary pandemic hospital and who had confirmed infection with a reverse transcriptase–polymerase chain reaction (RT-PCR) test were determined from the hospital’s records and their neonatal hearing screening results were analyzed from the national database. Neonates born before <34 weeks, and with reported risk factors in the database such as congenital anomaly or known TORCH infection during pregnancy were excluded. The screening tests, Automated Auditory Brainstem Response or Transient Evoked Otoacoustic Emission (TEOAE), were used for screening, and patients who failed the first screening were reevaluated at least 2 weeks apart with a second screening.Results:The incidence of failed second screening was 1.3% in the COVID-19 group and 2.9% in controls, and no significant difference was observed between the two groups according to the final screening results on the second test. Among the 458 mothers, 8 were infected in first trimester, 126 in second trimester, 127 in third trimester but did not deliver within 15 days after infection and 197 were positive at birth. Six neonates in the infected group failed the second screening (3 [2.4%] in the second trimester, 1 [0.8%] third trimester, and 2 [1.0%] positive at birth).Conclusions:COVID-19 infection during pregnancy was not found to be a risk factor for hearing loss, according to the newborn hearing screening results.

BackgroundTo evaluate the incidence of hearing loss in neonates in our secondary care hospital under pilot UNHS programme. To assess association between various risk factors and neonatal hearing loss.MethodsProspective, observational cohort study was done in a secondary level hospital in North India after ethical approval, for 1 year. Inclusion criteria are as follows: neonates born in hospital during study period, consenting to testing. Exclusion criteria are as follows: sick neonates, non-consenting parents. Neonates underwent TEOAE at 48 h of birth; those failing retested at 1 month. Neonates failing 2nd stage are tested after 3 months using BERA. Neonates were evaluated for the presence of maternal/neonatal high-risk factors.ResultsOut of 506 neonates, 143 passed 1st OAE screening, 363 were refer, and referral rate is 71.7%. A total of 341/345 neonates passed 2nd stage; 4 were diagnosed with hearing loss on BERA at 3 months. (18 neonates lost to follow-up, excluded from final cohort.) Overall incidence of hearing loss was 0.82%, 1.08% for males and 0.44% for females (p = 0.87, NS). One-hundred nine neonates were high risk (prematurity, 36; consanguinity, 4; caesarean section for relevant indications, 68; craniofacial abnormalities, 1). Incidence of hearing loss for high-risk group was 1.83% and 0.53% for well-born neonates (p = 0.19, NS).ConclusionIncidence of hearing loss in our district in North India is as follows: 8.2 per 1000 live births for well neonates, 18.3 per 1000 live births for high-risk neonates, and respective overall national incidence rates were 1.59 to 8.8 per 1000 and 7 to 49 per 1000. UNHS programmes must be implemented in all hospitals; protocol may be varied according to local population profile and resources available.

Congenital hearing loss is the total or partial inability to hear sounds through the ears. It is the most common disability in newborns in Chile and worldwide, and is a permanent condition. The direct impact on children who are not adequately diagnosed is the alteration in acquisition of language and cognitive skills and a decline in their social and school insertion, jeopardizing their professional and potentially productive life. Universal screening programs for hearing loss are essential for the diagnosis, since 50% of infants with hearing loss have no known risk factor. Screening before one month of age, confirmation before 3 months, and effective intervention before 6 months, allows the development of these children as if they had normal hearing. In Chile there is a selective program of screening for infants aged less than 32 weeks or 1,500 grams, as part of Explicit Health Guarantees, but it covers only 0.9% of newborns per year. Therefore, a large majority of children remain without diagnosis. The aim of this review is to compare the situation in Chile with other countries, raising the need to move towards a universal neonatal hearing loss screening program, and propose necessary conditions in terms of justification and implementation of a universal screening public policy.