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Neonatal hearing screening (NHS) is a critical public health measure for early identification of hearing loss, ensuring timely access to interventions that can dramatically improve a child's language development, cognitive abilities, and social inclusion. Beyond clinical benefits, NHS provides long-term advantages in education and quality of life. Given that congenital hearing loss affects approximately 1-2 in every 1000 newborns worldwide, the case for universal screening is clear. Countries like the United States and Australia have successfully implemented NHS, leading to earlier diagnoses, improved language development, and better educational outcomes. In Europe, while many nations have adopted NHS, consistency remains a challenge. Countries such as Norway and the United Kingdom stand out for their well-established systems, whereas others, like Greece, have made notable progress but have yet to mandate NHS nationwide. This highlights the need for cohesive national policies across Europe to ensure universal coverage. Screening methods such as Transient Evoked Otoacoustic Emissions (TEOAE) and Automated Auditory Brainstem Response (AABR) are established tools for detecting hearing impairments in neonates. Despite their demonstrated efficacy, NHS remains inconsistent globally, particularly in low- and middle-income regions that lack mandatory policies or access to reliable screening technologies. This perspective advocates for the urgent need to make NHS mandatory in all countries, emphasizing its societal benefits and cost-effectiveness. Early diagnosis supports prompt intervention, like hearing aids or cochlear implants, which are most effective when implemented before six months. It also empowers families to make informed decisions, fosters educational inclusion, and mitigates the social and emotional challenges of undiagnosed hearing loss. Policymakers, healthcare providers, and international organizations must prioritize universal NHS to ensure no child is left behind due to unaddressed hearing loss.

Background: In recent years, neonatal hearing screening (NHS) has gained rapid traction in both developed and developing nations. However, the efficacy of these efforts depends on comprehensive standardization across all screening facets. This study aimed to assess the status and quality of NHS by investigating the knowledge, attitudes, beliefs, and practices of hearing screening practitioners regarding NHS., Methods: A cross-sectional survey was conducted, and an online questionnaire based on the knowledge-attitude/belief ( A / B )-practice model was distributed to all NHS practitioners in Luzhou, western China. Valid questionnaires were examined and uniformly graded., Results: A total of 63 valid questionnaires were collected. The practitioners were mainly female (96.83%), with nursing backgrounds (63.49%), and undergraduate degrees (66.67%). Most had ≤5 years of experience (74.60%) and had junior/intermediate titles (93.65%). The NHS within the Luzhou area started in 2006 with provincial institutions, expanding to 42 institutions by 2022. Statistically significant correlations were observed between the A / B score and the conducting years of each NHS institution ( p  < .05) as well as between the Knowledge (K) and Practice (P) scores ( p  < .01). No significant correlation was found between the K score, P score, A / B score, and working years of practitioners ( p  > .05), or in the total score of NHS institutions at different levels or in different counties by one-way ANOVA ( p  > .05)., Conclusions: It has been 17 years since the first medical institution in Luzhou launched NHS, and the overall performance of practitioners from different institutions has been consistent in terms of their knowledge, attitudes, or level of practice. However, there is room for further improvement in both the professional development of individuals and aspects related to work, such as health education and long-term follow-up.

Introduction: Syndromic hearing loss (SHL) is characterized by distinctive clinical phenotypes as well as genetic and phenotypic heterogeneity. More than 400 species of SHL have been described, the majority of which are autosomal dominant., Methods: 11 forms of SHL were obtained from 14 unrelated families with probands ranging in age from 5 to 78 months. The results of whole exome sequencing(WES), audiological characteristics, middle and inner ear radiological findings, and additional clinical phenotype characteristics were retrospectively analyzed., Results: Fourteen people with SHL were found. Two of them had Waardenburg syndrome, two had Branchio-Oto-Renal syndrome, two had CHARGE syndrome, and one had Treacher Collins syndrome, Kleefstra syndrome, Muenke syndrome, Osteopathia Striata with Cranial Sclerosis, Ayme-Gripp syndrome, Tatton-Brown-Rahman syndrome, Stickler syndrome, or Stapes Ankylosis with Broad Thumbs and Toes. In this investigation, ten variants were first reported., Discussion: The combination of a neonatal hearing screening and WES can diagnose syndrome-type hearing loss in infancy and childhood, according to our findings, expansion of the gene variant spectrum and phenotype for various age groups of SHL is essential and can provide valuable guidelines for clinical intervention decisions. It is imperative for medical practitioners to conduct diligent and prolonged patient monitoring due to the inherent variability in both the auditory impairment and the comprehensive clinical manifestation of SHL., Competing Interests: The authors declare no conflict of interest., (© 2024 Zheng et al.)

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

Background:  Childhood hearing loss is a global health concern. Despite the proven benefits of neonatal hearing screening (NHS), it is not yet mandated in South Africa. The lack of awareness of hearing loss and absence of NHS leads to delayed diagnosis and adverse developmental outcomes for affected children., Aim:  The study aimed to assess the availability of NHS services across primary healthcare (PHC) facilities in the City of Cape Town (CCT)., Setting:  Surveys were conducted with 26 PHC facilities in the CCT metropolitan areas that offer mother and child healthcare services., Methods:  Surveys gathered data through online and telephone methods. The surveys aimed to assess the availability and nature of NHS services, care pathways and training of healthcare professionals regarding NHS., Results:  None of the facilities used objective screening methods to screen hearing or have standardised care pathways for at-risk babies. Instead, they relied on parental concerns, with the use of the Road to Health book. None of the respondents reported having received hearing screening training, and the majority of participants (62%) lacked confidence in their knowledge of ear and hearing care., Conclusion:  The absence of NHS services highlights the need for standardised protocols and increased awareness among healthcare workers and caregivers. Implementing NHS services could facilitate earlier diagnosis and intervention of hearing loss for infants in the Western Cape.Contribution: This study's findings could guide efforts to improving access to NHS access at PHC level in Cape Town, ultimately providing early hearing screening services to infants.

Objectives: Increased neonatal referral rate of conductive hearing loss (CHL) related to otitis media with effusion (OME) following universal neonatal hearing screening (UNHS) may cause an unnecessary clinical, emotional, and financial burden. This study analyzes the long-term, audiological, and medical characteristics of CHL associated with OME in neonates in order to establish a standardized protocol following technology-driven improvements in detection and referral rates in UNHS., Methods: A retrospective study of all neonates with OME-related CHL referred to the University Hospital of Leuven (Belgium) after failing UNHS with the MAICO devices between January 1, 2013 and December 31, 2021 was performed. Follow-up consultations, auditory tests, referral side, birth month, hearing loss degree, underlying pathologies and risk factors, time to normalization, and need for ventilation tubes were assessed., Results: The incidence of CHL related to OME was stable between 2013 and 2021. Of all referred infants with OME, 52.3 % demonstrated spontaneous recovery. The average time to hearing normalization was significantly longer in children with underlying congenital pathologies compared to those without. Moreover, 74.4 % of these children received ventilation tubes compared to 32.0 % of children without underlying pathologies. No correlation was found between the incidence of OME-related CHL with either a hearing loss degree, admission to neonatal intensive care, or history of a nasogastric feeding tube., Conclusions: In children who failed UNHS due to OME, hearing recovers spontaneously without surgical intervention in 2/3 of the infants without underlying conditions within one year. In children with underlying congenital disorders, the time to hearing recovery is longer and the risk for surgical intervention is higher, underlining the need for implementing a UNHS standardized protocol., 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 © 2024 Elsevier B.V. All rights reserved.)

The main aim of this study was to explore the psycho-social impacts of Otoacoustic Emissions (OAE) screenings on families. This involved understanding the initial responses of parents to test results, identifying any resulting concerns, and acknowledging the coping mechanisms utilized to handle these unexpectedly encountered difficulties. This retrospective observational study was conducted from January 2021 to December 2022 at a tertiary care facility. An extensive assessment was carried out on 1100 newborns that had undergone the OAE screening. Parental emotional reactions, worries regarding their child's prospects, and employed coping mechanisms were determined using structured questionnaires and interviews. The study aimed to explore the link between socio-economic status and varying levels of post-test anxiety and to study the effect of immediate post-screening counselling. A notable emotional reaction was observed, with 85% of parents, whose neonates were advised for subsequent tests, showing signs of shock and denial. Apprehensions related to the child's growth and societal acceptance were dominant, with 70% parents concerned about potential hurdles. There was a marked correlation between a lower socio-economic status and elevated post-test anxiety. Prompt counselling post-screening resulted in a substantial reduction in parental stress and anxiety levels. While the relevance of OAE testing concerning neonatal health is unequivocal, the psycho-social repercussions it imposes on families are significant. The findings underscore the need for holistic healthcare approaches that not only focus on physiological outcomes but also prioritize the mental well-being of families., Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-024-04486-1., Competing Interests: Conflict of interestAll authors certify that they have no affiliations with or involvement in any organization or entity with any financial or non-financial interest in the subject matter or materials discussed in this manuscript., (© Association of Otolaryngologists of India 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Universal hearing screening offers unique possibilities for detection of congenital deafness as a consequence of congenital cytomegalovirus (CMVc) infection, so its selective study in the case of a failed test could be a non-negligible screening opportunity while other guidelines covering the possibility of universal screening are adopted. The aim of this study is to analyse the possibility of selective screening for CMVc after an altered hearing test in a regional hospital. During the period studied, the results obtained were unsatisfactory, especially in children born outside the hospital of residence, showing an excessive delay in hearing screening in many cases and in the few cases where CMVc screening could be performed, only 30% had the test ordered in a timely manner. The reasons for this are varied and the solution is to include selective screening for CMVc in the hearing screening programme. This implies shortening the timing of the hearing screening protocol to allow CMVc testing in saliva or urine (preferably) before 21 days of age and providing screening programmes with the necessary staff and time to perform it properly., (Copyright © 2023 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction: Gestational diabetes is a potential risk factor for neonatal hearing loss. Increased circulating sugars in mothers during pregnancy can impairs the micro circulation and can cause congenital anomalies of the inner ear resulting in congenital hearing loss. This study attempts to find the incidence of neonatal hearing loss among diabetic mothers., Methodology: This was a case control study with 86 neonates of diabetic mothers as cases and neonates of non diabetic mothers(n = 86) as controls. Antenatal diabetic history and sugar values of mothers were documented. Hearing status of the neonates were tested using DPOAE test and ABR test. DPOAE test was done on 3rd day and those who did not get a positive response underwent 2nd DPOAE and also ABR test if 2nd DPOAE was negative., Results: All neonates underwent DPOAE test and few were lost on follow up. First and second DPOAE showed a statistically significant difference between cases and controls. All babies who underwent ABR test had abnormal waveforms. 98% of cases showed moderate and severe bilateral hearing loss whereas all controls had only mild bilateral hearing loss., Discussion: This study showed a significantly higher percentage of abnormal hearing outcome among neonates of diabetic mothers than non diabetic mothers. This could be because of the toxic effects of maternal hyperglycemia on developing auditory system of the fetus. This study emphasis the need for better glycaemic control in diabetic pregnancy, the importance of early and mandatory hearing screening in newborns of diabetic mothers., Competing Interests: Conflict of InterestDr. Amrutha P, Dr. Nayana V G, Ms. Nimalka Maria Sequeria and Mr. Hemaraj Nayaka S declare they have no conflict of interest., (© Association of Otolaryngologists of India 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Early identification of hearing loss through newborn hearing screening followed by an early start of intervention has proven to be effective in promoting speech and language development in children with hearing loss. During the COVID-19 pandemic, newborn hearing screening was postponed for a group of newborns in the Netherlands. Therefore, meeting the guidelines for early identification was at risk. In this study, we examine parental attitudes, beliefs, and experiences concerning the hearing screening during the COVID-19 pandemic. Our results indicated that parents ( n = 1053) were very positive about newborn hearing screening and their experiences with the screening, even during the COVID-19 pandemic. Parents' beliefs on the information provision around newborn hearing screening were more inconsistent. The results showed that parents with a postponed hearing screening felt less informed about the hearing screening than parents without a postponed screening. Furthermore, child and family characteristics affected how parents experienced newborn hearing screening. Parents with a premature child were more worried about the hearing abilities of their child before the screening took place. The results also indicate that deafness in the family might lead to parental worries around newborn hearing screening.

The effect of hypoxia on the functioning of the outer hair cells of the cochlea, which are responsible for the response to otoemissions used in neonatal hearing screening, is well known. The aim of this study is to determine the influence of mild to moderate variations in umbilical cord pH at birth on the outcome of hearing screening with otoemissions in healthy newborns without hearing risk factors. The sample is composed of 4536 healthy infants. The results show no significant differences in the hearing screening outcome between the asphyctic (<7.20) and normal pH group. Nor is a figure below 7.20 detected in the sample that is related to an alteration in the screening. When broken down into subgroups with known factors of variation in the screening result, such as gender or lactation, no significant differences in response were detected. Apgar ≤7 is significantly related to pH<7.20. In conclusion, mild-moderate asphyxia associated with delivery of healthy newborns, without auditory risk factors, does not alter the outcome of otoemission screening., (Copyright © 2023 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Published by Elsevier España, S.L.U. All rights reserved.)

Recent studies showed that COVID-19 infection can affect cochleo-vestibular system. The possibility of a vertical transmission is controversial. Some studies suggested that it is possible but unlikely, others find no evidence of vertical transmission. The objective of this study was to investigate whether exposure to COVID-19 during pregnancy or at birth has an impact on the hearing of the offspring. As part of the national hearing screening program, we performed in all newborns between January 2022 and February 2023, TEOAEs (Transient Evoked Otoacoustic Emissions) at birth and at 3 months. For those "REFER" at the third month test, we performed aABR (Automatic Auditory Brainstem Response) at 6 months. We analysed separately result between infants born to COVID-positive mothers during pregnancy and those born to COVID-negative mothers. To statistical verify differences we performed "Chi-square test". We enrolled a total of 157 infants, of whom 16 were born to mothers who had a molecular PCR test positive for COVID-19. In the latter we tested a total of 32 ears and only 1 ear (3,1%) resulted "REFER". On the other hand, in the control group we tested a total of 282 ears and 22 (7,8%) were found to be "REFER". Our study showed no significant differences in audiological assessment between newborns exposed to COVID-19 infection during pregnancy or at birth compared to the unexposed group. However, further studies with a larger patient's sample will be necessary for a more comprehensive evaluation.

Second-level hospitals face peculiarities that make it difficult to implement hearing rescreening protocols, which is also common in other settings. This study analyzes the hearing rescreening process in these kinds of hospitals. A total of 1130 individuals were included; in this cohort, 61.07% were hospital newborns who failed their first otoacoustic emission test after birth (n = 679) or were unable to perform the test (n = 11), and who were then referred to an outpatient clinic. The remaining 38.93% were individuals born in another hospital with their first test conducted in the outpatient clinic (n = 440). A high number of rescreenings were made outside of the recommended time frame, mainly in children referred from another hospital. There was a high lost-to-follow-up rate, especially regarding otolaryngologist referrals. Neonatal hearing screening at second-level hospitals is difficult because of staffing and time constraints. This results in turnaround times that are longer than recommended, interfering with the timely detection of hearing loss. This is particularly serious in outpatient children with impaired screening. Referral to out-of-town centers leads to unacceptable follow-up loss. Legislative support for all these rescreening issues is necessary. In this article, these findings are discussed and some solutions are proposed.

Introduction: Neonates undergo neonatal hearing screening to detect congenital hearing loss at an early stage. Once confirmed, it is necessary to perform an etiological workup to start appropriate treatment. The study objective was to assess the different etiologies, risk factors, and hearing results of infants with permanent hearing loss and to evaluate the efficacy and consequences of the different screening devices over the last 21 years., Methods: We conducted a single-center retrospective cohort analysis for all neonatal hearing screening program referrals and performed an etiological workup in case of confirmed hearing loss. We analyzed the evolution of the etiological protocols based on these results., Results: The governmental neonatal hearing screening program referred 545 infants to our center. Hearing loss was confirmed in 362 (66.4%) infants and an audiological workup was performed in 458 (84%) cases. 133 (24.4%) infants were diagnosed with permanent hearing loss. Ninety infants (56 bilateral and 34 unilateral) had sensorineural hearing loss, and the degree was predominantly moderate or profound. The most common etiology in bilateral sensorineural hearing loss was a genetic etiology (32.1%), and in unilateral sensorineural hearing loss, an anatomical abnormality (26.5%). Familial history of hearing loss was the most frequently encountered risk factor., Conclusion: There is a significant number of false positives after the neonatal hearing screening. Permanent hearing loss is found only in a limited number of infants. During the 21 years of this study, we noticed an increase in etiological diagnoses, especially genetic causes, due to more advanced techniques. Genetic causes and anatomical abnormalities are the most common etiology of bilateral and unilateral sensorineural hearing loss, respectively, but a portion remains unknown after extensive examinations., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Verstappen, Foulon, Van den Houte, Heuninck, Van Overmeire, Gordts and Topsakal.)

An early diagnosis and intervention for congenital cytomegalovirus infection can reduce long-term disability; however, the introduction of universal neonatal screening has been controversial worldwide. The present study clarified the outcome of a targeted screening protocol for detecting congenital cytomegalovirus infection based on suggestive perinatal conditions. In addition, the positive rate was compared to those from the reported studies and the validity of the targeted screening criteria was discussed. A total of 2121 newborn infants were admitted to our hospital between October 2018 and October 2021. Cytomegalovirus DNA was examined by the isothermal nucleic acid amplification method for urine samples from newborns with any of the following: microcephaly, abnormal ultrasound findings in the brain and visceral organs, repeated failure in neonatal hearing screening, suspicious maternal cytomegalovirus infection during pregnancy, and other abnormal findings suggestive of congenital cytomegalovirus infection. Among 2121 newborns, 102 (4.8%) were subject to the urine cytomegalovirus DNA test based on the abovementioned criteria. Of them, three were cytomegalovirus DNA-positive. According to the protocol, the cytomegalovirus DNA-positive rates were 0.14% among the total enrollment of 2121 newborns and 2.9% (3/102) among the targeted newborns. This protocol may overlook congenital cytomegalovirus infection that is asymptomatic or exhibits inapparent clinical manifestations only at birth; however, it is feasible and helps lead to the diagnosis of congenital cytomegalovirus infection that may otherwise be overlooked., (© 2023 Japanese Teratology Society.)

Congenital hearing loss is hearing loss present in a child at birth or soon after birth. It is a debilitating condition with the potential for lifelong disability. It is thought to be multifactorial in aetiology with both genetic (autosomal and X-linked) and acquired causes (such as maternal infections, drug intake, trauma). Gestational Diabetes Mellitus (GDM) is a relatively common condition found in pregnant females but is a rather understudied risk factor in terms of congenital hearing loss. GDM is easily treatable which makes the hearing loss due to it easily preventable. (1) Determine correlation between Gestational Diabetes Mellitus and congenital hearing loss in neonates. (2) Calculate the prevalence of Gestational Diabetes Mellitus related congenital hearing loss. A two-step screening process was used for hearing evaluation of neonates with normal mothers (non-exposed) and neonates with mothers suffering from GDM (exposed) through Otoacoustic emission (OAE) and Brainstem Evoked Response Audiometry (BERA). (1) The difference of neonates diagnosed with hearing impairment in exposed and non-exposed group was statistically significant with a p -value of 0.024. OR 2.1538 95% CI 0.6120-7.5796, p  < 0.05. (2) Prevalence of hearing loss in neonates of GDM mothers: 13.3%. Through rigorous exclusion of the already known risk factors for congenital hearing loss, Gestational Diabetes Mellitus has been isolated as an independent risk factor for neonatal hearing impairment. We hope to identify additional cases of congenital hearing loss early leading to a decrease in disease burden., Competing Interests: Conflict of interestThe authors declare no conflict of interest during the making of this manuscript. Ethical Approval. Ethical clearance was taken prior to conducting the study from the institutional ethics committee of Srimanta Sankaradeva University of Health Sciences (No. MC/190/2007/Pt.II/April-2021/TH-1) and the study was performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards., (© Association of Otolaryngologists of India 2023, Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Background: Hearing loss is one of the most common congenital disorders. The Early Diagnosis and Intervention Process is designed for the early diagnosis and intervention of hearing loss in infants. The present study aimed to examine the results of Early Hearing Detection and Intervention (EHDI) in northeastern Iran from 2005 to 2019., Setting: Northeastern Iran., Methods: In most cases, the two-stage protocol (otoacoustic emissions [OAE] and automated auditory brainstem response [AABR]) has been used. Infant assessment methods included the use of OAE, ABR, auditory steady-state response, high-frequency tympanometry, and behavioral audiometry. Interventions included medical interventions, hearing rehabilitation, hearing aids, and cochlear implants., Results: 1,162,821 infants were screened. The screening coverage increased from less than 1% in 2005 to about 99% in 2018. The referral rate has been about 1%. 2.17 out of every 1000 infants are hearing impaired, and the most common cases are bilateral hearing loss and mild to moderate hearing loss., Conclusions: During 2005 to 2019 the coverage rate reached to more than 95% of live births. To improve the EHDI process in this population, better follow-up of diagnosed neonates and expansion of diagnostic and intervention services are needed., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 International Journal of Preventive Medicine.)

Hearing loss has long been associated with congenital hypothyroidism and, if not noticed and treated early, may result in delayed language acquisition and difficulties in comprehension. In light of the implications of congenital hypothyroidism and its associated hearing loss, we decided to conduct this study. The aim of this study was to identify the prevalence of hearing loss in congenital hypothyroidism and its relation to the severity of the disease and age of initiation of treatment. This study was conducted from July 2016 to June 2021 at a tertiary care hospital in Bhopal. The hearing status of congenital hypothyroid children and matched controls was assessed by pure tone audiometry or free field audiometry and, in selected cases, with brainstem evoked response audiometry. Eight children (25%) with congenital hypothyroidism had hearing loss, compared to one (3.12%) in the control group ( p  < 0.05). No statistically significant difference has been found in the gender, mean age of diagnosis, mean age of initiation of levothyroxine treatment, mean screening FT4 levels, and mean screening TSH levels between congenital hypothyroid children with and without hearing loss. Due to the high prevalence of hearing loss in congenital hypothyroidism and its impact on language and cognitive development, it is critical to create awareness among healthcare professionals that children diagnosed with congenital hypothyroidism must undergo audiological evaluation at the time of diagnosis and periodically thereafter. Alternatively, children with unexplained hearing loss must be screened for congenital hypothyroidism., Competing Interests: Conflict of interestThe authors declare that they have no conflict of interest., (© Association of Otolaryngologists of India 2022.)

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).)

This study aims to find out the prevalence of hearing impairment in neonates of mothers with diabetes mellitus. The objective is to assess any correlation between the glycemic control and the development of neonatal hearing impairment. A total of 120 neonates of diabetic mothers were included in the study. Data was collected from hospital medical records, direct interview of parent or care taker and clinical examination of child done whenever possible. 120 Neonates were evaluated by OAE soon after birth. Those who failed the test are evaluated with BERA and results were statistically analysed. In our study prevalence of deafness in neonates of mothers with diabetes mellitus was 4.16%. Prevalence of hearing impairment was higher in neonates of mothers with pre gestational diabetes (9.09%) than gestational diabetes mellitus (3.06%). Mean of HbA1c was higher in mothers of neonates with hearing impairment than those without hearing impairment. Prevalence of deafness in neonates of mothers with diabetes mellitus was 4.16%, which is about sevena times higher than the prevalence of deafness in neonates without any risk factors. Prevalence was higher in neonates of mothers with pre gestational diabetes than neonates of mothers with gestational diabetes mellitus., Competing Interests: Conflict of interestThere are no conflict of interest., (© Association of Otolaryngologists of India 2021.)

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.)

Congenital deafness is a major pediatric problem, affecting about 1.5-3 per 1000 newborns. The early treatment through cochlear implantation and auditory rehabilitation has been a historic milestone. Early diagnosis of congenital deafness is an essential requirement to obtain the best results, which is achieved through neonatal screening, a diagnostic practice that we began systematically at the Hospital Clínico in Valencia (Spain) 30 years ago. Neonatal hearing screening is successful in most developed countries. Its implementation has been slow due to the multiple difficulties that its universal application entails since it involves several health professionals and must be carried out, in a short time interval after birth. In addition, it must have a good performance that prevents the overload of other services and that requires experience and continuous adjustments in search of proper protocols. The aim of this review is to shed some light on some key points of neonatal hearing screening, highlighting our experience in the solutions to common problems. We will discuss about techniques, protocols and neonatal or nutritional factors that can influence the screening results. To a summary of our work, an update on the subject is provided with the intention of sharing experiences and facilitating the start-up of the new units.

Background: Very preterm infants are at a greater risk of developing neurodevelopmental impairments such as neuro-motor delays, vision and hearing deficits (Roze and Breart, 2004; Saigal and Doyle, 2008) [1,2]. The hearing difficulties in preterm infants vary depending on the co-morbid conditions. However, prematurity itself is considered as a risk factor that influence the functioning of auditory system., Aim: The current study aims to compare the DPOAEs in very preterm infants and term infants at 1 month, 3 months and 6 months of age (corrected age in preterm infants)., Method: DPOAEs were recorded in 72 very preterm infants and 30 term infants at 1 month, 3 months and 6 months of age. All these infants had obtained 'pass' results in newborn hearing screening using ABR. DPOAE f2 test frequency was measured at six frequencies (1500 Hz, 2000 Hz, 3000 Hz, 4500 Hz, 6000 Hz and 8000 Hz) with primary tone stimulus intensity L1 equal to 65 dBSPL and L2 equal to 55 dBSPL with primary tone f2/f1 frequency ratio of 1.2. Otoscopic examination and tympanometry was performed prior to DPOAE testing, to ascertain normal middle ear status., Results: DPOAE amplitude did not change significantly between two groups from 1 month till 6 months of age (p > 0.05). DPOAE amplitude and noise floor in very preterm infants were not different from term infants and DPOAE amplitude did not vary significantly across f2 frequencies at various time periods., Conclusion: The current study findings provided evidence that prematurity did not constitute as a factor to influence the results of DPOAE in very preterm infants who passed newborn hearing screening test. Any significant reduction in DPOAE amplitude or absence of DPOAE in very preterm infants has to be considered and monitored effectively, as it may not reflect a developmental process of cochlear function; instead it could indicate the presence of inner ear or middle ear pathology., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Objective: To compare the hearing screening results of two-step transient evoked otoacoustic emissions (TEOAE) and one-step automatic auditory brainstem response (AABR) in non-risk newborns, and to explore a more suitable hearing screening protocol for infants discharged within 48 h after birth in remote areas of China. Methods: To analyze the age effect on pass rate for hearing screening, 2005 newborns were divided into three groups according to screening time after birth: <24, 24-48, and 48-72 h. All subjects received TEOAE + AABR test as first hearing screen, and those who failed in any test were rescreened with TEOAE + AABR at 6 weeks after birth. The first screening results of AABR and TEOAE were compared among the three groups. The results of two-step TEOAE screening and one-step AABR screening were compared for newborns who were discharged within 48 h. The time spent on screening was recorded for TEOAE and AABR. Results: The pass rate of TEOAE and AABR increased significantly with the increase of first screening time ( P < 0.05), and the false positive rate decreased significantly with the increase of first screening time ( P < 0.05). The failure rate of first screening of AABR within 48 h was 7.31%, which was significantly lower than that of TEOAE (9.93%) ( P < 0.05). The average time spent on AABR was 12.51 ± 6.36 min, which was significantly higher than that of TEOAE (4.05 ± 1.56 min, P < 0.05). The failure rate of TEOAE two-step screening was 1.59%, which was significantly lower than one-step AABR. Conclusions: Compared with TEOAE, AABR screening within 48 h after birth can reduce the failure rate and false positive rate of first screening. However, compared with TEOAE two-step screening, one-step AABR screening has higher referral rate for audiological diagnosis. In remote areas of China, especially in hospitals with high delivery rate, one-step AABR screening is not feasible, and two-step TEOAE screening protocol is still applicable to UNHS screening as more and more infants discharged within 48 h after birth., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Sheng, Zhou, Wang, Yu, Liu, Liang, Zhou, Wu, Tang and Huang.)

Objective: The aim of the study was to evaluate the results of neonatal hearing screening in our hospital with the help of literature and to question and reveal the risk factors to gain healthy individuals and to raise awareness for all health workers and the public who are interested in this subject., Methods: A total of 16,388 newborn infants were evaluated between October 2009 and January 2018. All newborns were screened with transient evoked otoacoustic emissions (TEOAEs) test. Risk factors were investigated. The test repetition and auditory brainstem response (ABR) measurements were performed on newborns who could not pass the TEOAE test and the newborns in the risky group after 15 days., Results: A total of 116 newborns (0.7%) were suspected to have hearing loss. Twenty-seven newborns (0.16%) were found to be in intensive care unit. Twelve newborns (0.07%) had permanent hearing loss. Then, in order: 9 newborns (0.05%) had received phototherapy and 7 newborns (0.04%) were born to consanguineous marriages. In addition, 3 newborns (0.02%) had a low birth weight and 1 newborn (0.006%) had a history of fever., Conclusion: Screening tests should be performed in all newborns for early detection of hearing loss. Even though frequency of hearing loss is higher in newborns with risk factors, the treatment should be started within 6 months, the latest, and newborns should be referred for rehabilitation and training., Competing Interests: Conflict of Interest: No conflict of interest was declared by the author., (Copyright: © 2021 by Istanbul Northern Anatolian Association of Public Hospitals.)

Introduction: Recently, Interacoustics presented a new otoacoustic emission protocol where the probe pressurizes the ear cavity, thus eliminates the risk of non-assessment (REFER outcome) due to a negative middle ear pressure. This study evaluated the characteristics and the performance of this new protocol on a newborn well-baby population., Methods: One hundred sixty-three newborns (age 2.7 ± 1.1 days) for a total of 294 ears were assessed randomly. Transiently evoked otoacoustic responses were acquired by the Titan device (Interacoustics), using the default and a pressurized TEOAE protocol. The data were analyzed in terms of signal to noise ratios (S/Ns) at 5 frequencies, namely, 0.87, 1.94, 2.96, 3.97, and 4.97 kHz. To assess any possible gestational age (GE) effects on the TEOAE variables, the responses were subdivided in 4 different age subgroups., Results: There were no significant differences between the left and right ear TEOAE responses, for age (in days), GE (in weeks), weight (in grams), and S/N at all 5 frequencies. Considering the pooled 294 ears, paired t tests between the default and the pressurized TEOAE data showed significant differences across all 5 frequencies (p < 0.01). The pressurized protocol generated TEOAE responses presenting larger S/Ns, and a positive additive effect of approximately 2.31 dB was observed at all tested frequencies. There were no significant GE effects on the pressurized TEOAE responses. In terms of performance, both protocols performed equally (same number of PASSes)., Conclusion: The pressurized TEOAE protocol generates responses with higher S/Ns which might be useful in borderline cases where the middle ear status might cause a REFER screening outcome., (© 2021 S. Karger AG, Basel.)

Background: Literature shows that lower gestational age leads to greater delays in the auditory conduction, which suggests atypical maturation of the brainstem in normal-hearing premature newborns. Our aim is to investigate if there is a difference between the extrauterine and intrauterine maturation of the auditory system in normal-hearing newborns with a very premature (28-31 weeks) or extremely premature (<28 weeks) birth., Methods: Results of the Automated Auditory Brainstem Response Newborn Hearing Screening Program in Dutch Neonatal Intensive Care Units and diagnostic examinations were centrally registered from 1998 to 2016. Normal-hearing newborns with a gestational age of 25-31 weeks were included. Screening results at 32-45 weeks of postmenstrual age were compared between newborns born with different gestational ages. Multiple imputation was used to predict missing screening results. Small for gestational age was defined as birth weight corrected for gestational age < -1.6 standard deviation. Descriptive and (pooled) logistic regression analyses were performed., Results: 23,964 newborns with 28,754 screening results were eligible. At the same postmenstrual age, pass rates were lower when gestational age was lower in normal-hearing newborns with a very and extremely preterm birth. Pass rates of 80% could be obtained at 34-35, 32-33, and 30-32 weeks' postmenstrual age in newborns with 25, 26-27, 28-31 weeks gestational age, respectively. Small for gestational age had an additional negative effect on pass rates., Conclusion: Analysis of hearing screening data suggests that extrauterine maturation of the auditory system is delayed in normal-hearing newborns with a very or extremely premature birth., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest relevant to this article., (Copyright © 2020. Published by Elsevier B.V.)

Objectives: To investigate the Barriers and Priorities accorded by hospital and health administrators to neo-natal hearing screening., Methods: This qualitative exploratory descriptive study employing purposive sampling technique was conducted in Islamabad, over a period of 18 months from 1st August 2015 to 31st January 2017. Sample included the stakeholders i.e., Heads of public sector hospitals of Islamabad including Pakistan Institute of Medical Sciences, Capital Hospital, and Federal Government Services Hospital, Islamabad. Study included in depth interviews using a self-structured interview guide and audio recording. Recorded data was transcribed followed by thematic analyses which was manually drawn and verified., Results: The Outcomes from thematic analysis were drawn as Planning, Essential requirements for NNHS, High risk screening, education, Existence of skilled maternal & newborn health workers. Hearing screening equipment//facility instrumentation, Logistic support, Health ministry support and Financial cover are also significant outcomes. Lack of awareness in the public and professionals regarding the importance of early identification of HI, poor health infrastructure, burden on tertiary care and lack of referral top the list of barriers to initiation of NNHS program at hospital administrative level., Conclusion: The Barriers to NNHS identified at Hospital and Health care administrator level include lack of awareness, poor health infrastructure, burden on tertiary care and lack of referrals. Inherent barriers to NNHS cover the spectrum of lack of liaison/ linkages between obstetrics and other departments, deliveries at homes especially in rural areas, poor follow-up, scarcity of technical and adequately trained manpower. Intangible barriers to NNHS comprise lack of health care information system, attitudinal barriers, inadequate fiscal resources, and lack of integrated approach at intra departmental levels., Competing Interests: Conflict of Interest: None., (Copyright: © Pakistan Journal of Medical Sciences.)

Objective: To investigate the variance in reported prevalence rates of permanent neonatal hearing impairment (HI) worldwide. Design: A systematic review and meta-analysis was performed on reported prevalence rates of sensorineural and permanent conductive or mixed HI worse than 40 dB in neonates, detected as a result of a screening programme or audiometric study. Study sample: For meta-analysis, 35 articles were selected, 25 from high-income countries and 10 from middle-income countries according to the world bank classification system. Results: The prevalence rate of permanent uni- and bilateral HI worse than 40 dB in neonates varied from 1 to 6 per 1000, the overall prevalence was 2.21 per 1000 [1.71, 2.8]. In NICU populations the prevalence rate was higher with a larger fraction of bilateral cases. Although not significant, prevalence rates were slightly higher in Asia compared to Europe and the number of infants lost to follow-up appeared higher in countries with lower gross national income. Conclusion: Substantial variations exist in prevalence rates of neonatal permanent HI across countries and regions. There is a strong need for more data from low-income countries to identify demographic factors that account for this variability in reported prevalence rates. Reporting these data in a uniform way is advocated.

Objective: The objective of this study was to establish the local incidence of hearing screening failure rate in newborns with all three stages of hypoxic ischemic encephalopathy (HIE)., Methods: This retrospective cohort study was undertaken in a tertiary neonatal intensive care unit. Medical records and hearing secreening test results were collected for two years., Results: One hundred and ninety seven infants diagnosed with HIE, 20 of them died, 177 screened. Thirty five of 177 (19%) infants failed in screening test for hearing. Screening failure rate was 10/51 (19%), 20/105 (19%) and 5/21 (23%) in stage 1, 2 and 3, respectively and did not differ between HIE stages (p = 0.88). Furthermore failure rates were similar between infants who received therapeutic hypothermia or not (20% vs 19%, p = 0.84)., Conclusion: Hearing screening failure rate in HIE is quite high even in Stage 1 infants. Management and treatment of these infants should be made carefully concerning additional risks for hearing loss and long term follow-up even in Stage 1 HIE infants should be planned strictly., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Screening programs examining neonatal hearing serve to detect hearing defects, as a prerequisite for hearing rehabilitation, communication skills, and the enhancement of speech development. There are two methods through which neonatal hearing screening is carried out - the transiently evoked otoacoustic emissions (TEOAE) or the automatic BERA (AABR, automated auditory brainstem response). Positive screening means the discovery of a hearing defect (permanent hearing loss), and negative screening (normal TEOAE or the AABR results) means the absence of a hearing defect. The procedural aim is to update and adjust the neonatal hearing screening, which is determined by the Bulletin of the Ministry of Health of the Czech Republic No. 7/2012. Neonatal screening is performed at three levels: at neonatological site, at the ENT (phoniatric) rescreening site and at the ENT regional centre. The activities at each level are accurately and concretely identified including the issue of billing the performance to health insurance companies and informed consent to personal data protection (GDPR). The correct functioning of screening for hearing loss is based on the simple organization of the screening, patient examination comfort, medical recovery from it, and its economic viability. The schedule for neonatal hearing screening and rehabilitation recommends the following steps: 1. screening of a newborns hearing on the second or third day after delivery by a neonatological nurse using otoacoustic emissions, alternatively AABR for newborns at risk; 2. hearing rescreening in the third to sixth week of child`s age at the ENT rescreening site; 3. completion of hearing impairment diagnostics within three to sixth months of age at the ENT regional centre. The failure to follow the procedure above is a threat to the hearing and speech development of the child with severe permanent hearing impairment. The collaboration of ENT doctors with neonatologists and paediatricians allows for creating conditions under which the functional nationwide hearing screening of newborns can be established throughout the Czech Republic.

Objective: To analyze the hearing assessment characteristics and follow-up of some deafness gene screening homozygous infants in Zhuhai. Method: The clinical data of 28 newborns with homozygous mutations transferred to Zhuhai Maternal and Child Health Hospital from Feb. 1, 2015 to Oct. 25, 2018 in hospitals of Zhuhai City were retrospectively analyzed. All the children were screened for hearing. The hearing characteristics and long-term follow-up results of homozygous mutations at different gene sites were analyzed by auditory diagnosis and behavioral follow-up from 1 to 3 years. Result: Fourteen cases of GJB2 c.109G>A with a homozygous mutation, 11 cases passed the hearing screening, the audiological diagnosis was normal, and the behavior test and follow-up were normal from 1 to 3 years. Hearing screening was not passed in 3 newborns, mild to moderate abnormalities of single or bilateral ears were diagnosed by audiology, 1 000 Hz without positive, and middle ear lesions were diagnosed. Eight cases of GJB2 c.235del C homozygous mutation were followed up by behavioral audiometry and follow-up from 1 to 3 years after cure. Among them, 5 cases were diagnosed as severe hearing impairment of bilateral ears and 3 cases as mild and moderate hearing impairment. One case of GJB3 547G>A homozygous mutation was followed up for 1-3 years, and all of them failed to pass the follow-up of behavioral audiometry and follow-up. Four cases of SLC26A4 IVS7-2A>G, 1 case of SLC26A4 1229C>T homozygous mutation, all of them failed to pass the neonatal hearing screening. All the patients were diagnosed as severe hearing impairment of binaural hearing, and the follow-up of 1-3 years' follow-up did not pass the follow-up tests. Conclusion: GJB2 C.235del C, SLC26A4 IVS7-2A>G locus homozygous mutation infant hearing impairment was mainly severe hearing impairment in bilateral ears, and there was no change in 1-3 years follow-up. GJB2 C.109G A homozygous mutant infants had normal hearing, and it was suggested that they should be followed up closely. It is very important to give correct and reasonable genetic counseling to parents with GJB2 C.109G A homozygous mutation without unnecessary panic., 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.)

Recent prevalence estimates indicate that in 2015 almost half a billion people-about 6.8% of the world's population-had disabling hearing loss and that prevalence numbers will further increase. The World Health Organization (WHO) currently estimates that at least 34 million children under the age of 15 have disabling hearing loss. Based on a 2012 WHO report, approximately 7.5 million of these children were under the age of 5 years. This review article focuses on the importance of high-quality newborn and infant hearing screening (NIHS) programs as one strategy to ameliorate disabling hearing loss as a global health problem. Two WHO resolutions regarding the prevention of deafness and hearing loss have been adopted urging member states to implement screening programs for early identification of ear diseases and hearing loss in babies and young children. The effectiveness of these programs depends on factors such as governmental mandates and guidance; presence of a national committee with involvement of professionals, industries, and stakeholders; central oversight of hearing screening; clear definition of target parameters; presence of tracking systems with bi-directional data transfer from screening devices to screening centers; accessibility of pediatric audiological services and rehabilitation programs; using telemedicine where connectivity is available; and the opportunity for case discussions in professional excellence circles with boards of experts. There is a lack of such programs in middle- and low-income countries, but even in high-income countries there is potential for improvement. Facing the still growing burden of disabling hearing loss around the world, there is a need to invest in national, high-quality NIHS programs., Competing Interests: Conflicts of InterestThe authors declare no conflict of interest., (© 2019 by the authors.)

In the Netherlands, Youth Health Care services (YHC) have been carrying out neonatal hearing screening (NHS) in newborns since 2006. The aim of the NHS is to identify children with permanent hearing loss, so that intervention can be started before the age of 4 months. Early detection of hearing loss is important, as children who start intervention early have been shown to develop better. This article describes the structure and performance of the NHS carried out by the YHC, the quality of the program, and the timeliness of the start of intervention. Since its implementation, the NHS has been audited annually in order to monitor the program's quality. Monitoring reports and data from the Dutch Foundation for the Deaf and Hard of Hearing Child were used in this study. For many years, results have shown the NHS to be a stable screening program of high quality. The participation rate is high, refer percentage low, and the timeliness of the program is continually improving. Although the timeliness of post screening diagnostics and intervention need most improvement as they do not always meet the target times, this has improved over recent years., Competing Interests: Conflicts of InterestThe authors declare no conflicts of interest., (© 2018 by the authors.)

Summary The prevalence of hearing loss in newborns and infants is estimated between 1 to 3.47 cases per 1000 live births. Neonatal screening for hearing loss and audiological evaluation are becoming more extensively carried out. However, there is no consensus regarding the use of audiometry and other electrophysiological tests in current practices. This article is intended to provide professionals with recommendations about the "best practice" based on consensus opinion of the session's speakers, and a review of the literature on the efficacy of various assessment options for children with hearing loss., 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.)

Objectives Universal neonatal hearing screening (UNHS) started late in some underdeveloped areas in China, with relatively scarce screening resources and a wide regional distribution. This study aimed to compare the screening performance between rural and urban populations, and to examine the characteristics and problems of UNHS in underdeveloped regions in China. Methods A two-step hearing screening program was used in neonates born in Liuzhou Maternal and Child Health Hospital and in patients who were born in other hospitals, but admitted to the neonatal intensive care unit. This program involved distortion product otoacoustic emission and automated auditory brainstem response. Characteristics of each newborn, as well as the screening outcomes and performance were compared between rural and urban populations. Results A total of 19,098 newborns were screened with a referral rate of 17.9% at the first step. Sixty-three (0.33%) newborns had hearing loss. The prevalence of permanent hearing loss was 2.25‰. The average screening age was significantly older in the rural population than in the urban population in the first ( P < 0.01) and second steps of screening ( P < 0.05). The rural population had a higher referral rate in both steps than the urban population ( P < 0.01). The follow-up rate was much lower in the rural population than in the urban population ( P < 0.05), but dramatically increased in 2014 compared with the previous 2 years. Conclusions A low follow-up rate is a critical issue when carrying out UNHS in developing countries, such as China, especially for rural populations. The government should establish more hearing referral centres to increase service coverage and supply financial assistance for low-income populations.

Aim: Very preterm infants are at risk of neonatal hearing loss. However, it is unknown whether infants with a normal neonatal hearing screening result risk sensorineural hearing loss (SNHL) at a later age., Methods: This cohort study was conducted at the Erasmus Medical University Center Rotterdam, the Netherlands, on 77 very preterm infants born between October 2005 and September 2008. All infants underwent auditory brainstem response audiometry during neonatal hearing screening and at two years of corrected age. The frequency of SNHL in infants with a normal neonatal hearing screening was analysed and the risk factors associated with newly diagnosed SNHL in these infants were examined., Results: We found that 3.9% (3/77) of the very preterm infants showed permanent hearing loss during their neonatal hearing screening. In addition, a relatively high prevalence of newly diagnosed SNHL (4.3%) was found in three of the 70 infants followed up at the age of two. The total prevalence rate of permanent hearing loss in the cohort was approximately 8%., Conclusion: A normal outcome of neonatal hearing screening did not guarantee normal hearing at two years of age in this very preterm cohort and paediatricians should be alert to the possibility of late-onset SNHL., (©2017 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Introduction: The success of a newborn hearing screening program depends on successful tracking and follow-up to ensure that children who have had positive screening results in the first few days of life receive appropriate and timely diagnostic and intervention services. The easy availability, through a suitable infrastructure, of the data necessary for the tracking, diagnosis, and care of children concerned is a major key to enhancing the quality and efficiency of newborn hearing screening programs., Materials and Methods: Two systems for the automated two-way transmission of newborn hearing screening and configuration data, based on mobile communication technology, for the screening devices MADSEN AccuScreen ® and Natus Echo-Screen ® were developed and tested in a field study. Radio modem connections were compared with conventional analogue modem transmissions from Natus Echo-Screen devices for duration, transmission rate, number of lost connections, and frequency of use., Results: The average session duration was significantly lower with the MADSEN AccuScreen (12 s) and Natus Echo-Screen both with radio modem (15 s) than the Natus Echo-Screen with analogue modem (108 s). The transmission rate was significantly higher (898 and 1,758 vs. 181 bytes/s) for the devices with radio modems. Both radio modem devices had significantly lower rates of broken connections after initial connection (2.1 and 0.9 vs. 5.5%). An increase in the frequency of data transmission from the clinics with mobile radio devices was found., Conclusions: The use of mobile communication technology in newborn hearing screening devices offers improvements in the average session duration, transmission rate, and reliability of the connection over analogue solutions. We observed a behavioral change in clinical staff using the new technology: the data exchange with the tracking center is more often used. The requirements for on-site support were reduced. These savings outweigh the small increase in costs for the Internet service provider.

Objective: By comparing the Universal Neonatal Hearing Screening (UNHS) program as implemented in Shanghai and other regions in China and countries around the world, this study makes an assessment of the Shanghai model and summarizes the experiences implementing the UNHS program, so as to provide a valuable reference for other countries or regions to carry out UNHS more effectively. Since Shanghai is one of the most developed regions in China, we also examined the relationship between economic development and the UNHS starting year and coverage rate., Methods: The study conducted a systematic review of published studies in Chinese and English on the program status of neonatal hearing screening to compare and analyze the implementation of the UNHS program in 20 cities or provinces in China and 24 regions or countries around the world. The literature search in Chinese was conducted in the three most authoritative publication databases, CNKI (China National Knowledge Infrastructure), WANFANGDATA, and CQVIP (http://www.cqvip.com/). We searched all publications in those databases with the keywords "neonatal hearing screening" (in Chinese) between 2005 and 2014. English literature was searched using the same keywords (in English). The publication database included Medline and Web of Science, and the search time period was 2000-2014., Results: Shanghai was one of the first regions in China to implement UNHS, and its coverage rate was among the top regions by international comparison. The starting time of the UNHS program had no relationship with the Gross Domestic Product (GDP) per capita in the same year. Economic level serves as a threshold for carrying out UNHS but is not a linear contributor to the exact starting time of such a program. The screening coverage rate generally showed a rising trend with the increasing GDP per capita in China, but it had no relationship with the area's GDP per capita in selected regions and countries around the world. The system design of UNHS is the key factor influencing screening coverage. Policy makers, program administrators, and cost-sharing structures are important factors that influence the coverage rates of UNHS., Conclusion: When to carry out a UNHS program is determined by the willingness and preference of the local government, which is influenced by the area's social, political and cultural conditions. Mandatory hearing screening and minimal-cost to no-cost intervention are two pillars for a good coverage rate of UNHS. In terms of system design, decision-making, implementation, funding and the concrete implementation plan are all important factors affecting the implementation of the UNHS., (Copyright © 2016 The Author(s). Published by Elsevier Ireland Ltd.. All rights reserved.)

Unlabelled: Auditory neuropathy spectrum disorder (ANSD) is a particular kind of hearing disorder characterised by normal outer hair cell function and abnormal or absent auditory brain stem responses. Little data are available regarding the prevalence of this condition in healthy newborns. We performed a retrospective medical records review of 791 referrals from universal neonatal hearing screening (UNHS) at a well-baby clinic to investigate the prevalence of ANSD. Hearing screening was performed by automated auditory brain stem response (ABR) testing. A diagnosis of ANSD was established when ABR tracings were absent in the presence of otoacoustic emissions and/or a cochlear microphonic. Amongst 201 infants with confirmed congenital hearing loss, 13 infants were diagnosed with ANSD. The condition was unilateral in six and bilateral in seven infants. A risk factor for hearing loss could be identified in three infants. Abnormalities on magnetic resonance imaging were found in six infants; five of them had cochlear nerve deficiency., Conclusion: The prevalence of ANSD was 6.5 % amongst well babies with confirmed congenital hearing loss identified through UNHS. The estimated incidence of ANSD in our population of newborns at the well-baby clinic was 0.09/1000 live births. Magnetic resonance revealed an underlying anatomical abnormality in about half of the patients., What Is Known: • Auditory neuropathy dyssynchrony spectrum disorder (ANSD) is a particular form of hearing loss, mostly encountered in neonatal intensive care unit (NICU) graduates. • Little data are available on the prevalence and risk factors for ANSD in healthy newborns. What is new: • The estimated prevalence of ANSD in healthy newborns is 0.09/1000 live births. • In about half of the healthy newborns with ANSD, a structural abnormality was detected on magnetic resonance imaging of the posterior fossa/brain.

Aim: To detect any abnormality in the maturational process of hearing threshold during the early life in at-risk preterm infants., Study Design: The threshold of brainstem auditory evoked response was recorded and analyzed longitudinally from 30 to 42weeks of postconceptional age in 357 at-risk infants born at 23-36weeks of gestation. The results were compared with those in 82 low-risk infants born at 30-42weeks at various postconceptional ages., Results: From 31 to 42weeks, the response threshold in the at-risk infants was consistently slightly higher than that in the low-risk infants. No statistically significant difference was found between the two groups of infants at any designated postconceptional ages. The threshold in the at-risk infants born at 23-29weeks of gestation tended to be higher than those born at 30-36weeks at various postconceptional ages, but the difference did not reach statistical significance. There was also no significant difference in the slope of BAER threshold-age function between the at-risk infants, irrespective of gestational ages, and the low-risk infants., Conclusion: During the early life, hearing threshold in at-risk preterm, mainly very preterm, infants is marginally elevated, but the maturational process of the threshold is generally similar to that in low-risk infants, without notable abnormality., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

We discuss issues related to cochlear implantation in children with auditory neuropathy spectrum disorder (ANSD). We describe the varied nature of this disease category including the numerous potential causes of auditory neuropathy. The most prevalent etiology for infants with ANSD is associated with prolonged neonatal intensive care unit (NICU) stay. We discuss the potential contribution of cochlear hypoxia to this etiology. The second part of this review describes in detail our own experience at the Hospital for Sick Children in Toronto, with cochlear implantation of children diagnosed with ANSD. We outline the detection, diagnosis, and referral routes for our patients. We provide an overview of our "standard operation procedures" regarding candidacy, and discuss some of the special considerations that need to be applied to children with ANSD. This includes decisions to implant children with better audiometric thresholds that are standard in non-ANSD patients, concerns about the possibility of spontaneous remission and the appropriate timing of implantation. Finally we review an extensive published literature in outcomes after cochlear implantation (CI) in ANSD. This is not a systematic review but rather an exercise to distill out some important reoccurring themes and the general consensus of opinion to date. Our conclusion is that the hearing loss category ANSD, together with its numerous co-morbidities, is far too heterogeneous to make definitive statements about prognosis with CI., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Aim: This study aimed to describe changes in hearing thresholds in babies born below 30 weeks of gestation at 28-42 weeks of postconceptional age, detect differences between gestational ages and explore the prevalence of hearing impairment., Methods: The threshold of brainstem auditory evoked response (BAER) was obtained longitudinally at 28-42 weeks of postconceptional age in 71 babies born at 23-29 weeks of gestation., Results: The BAER threshold was 28 decibels above normal hearing level (dB nHL) at 28 weeks of postconceptional age and decreased to 13 dB nHL at 39-42 weeks. The threshold was slightly higher in babies born at 23-26 weeks of gestation than in those born at 27-29 weeks at most postconceptional ages studied. From 28 to 36 weeks, the threshold decreased at 0.98 dB/week. At term, BAER threshold elevation (>20 dB nHL) occurred in 12 (16.9%) of the 71 babies., Conclusion: The hearing threshold of babies born at 23-29 weeks of gestation decreased from 28 dB at 28 weeks of postconceptional age to 13 dB at 42 weeks. There were no major differences between gestational ages. During the preterm period, the threshold changed at around 1 dB/week. At term, one in six babies had hearing impairment., (©2015 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

In 2009 many countries of the world met to discuss newborn and infant hearing screening current issues and guiding principles for action under World Health Organization (WHO) banner, though most of the countries who had begun this work as universal program or high risk screen do not have exact data and protocol. The developing countries also decided to become part of it and common guideline was proposed. India being part of it included hearing screening as one of the 30 diseases to be screened under Rashtriya Bal Swasthya Karyakram (RBSK). This article discusses all these issues of newborn hearing screening in the world and India.

Introduction: The implementation of Neonatal Hearing Screening (NHS) program is still at the preliminary stage particularly in developing countries despite the burden of permanent congenital and early-onset hearing impairment. There has been an earlier report of NHS in a city in Nigeria, however, this is a report of a preliminary NHS carried in a rural/sub-urban area in Nigeria., Method: This prospective study, which took place between October 2009 and April 2010, involved all newborns delivered at the University College Hospital, Ibadan and the Bilal Missionary Maternity, Agodi, Ibadan, a small maternity service located in Agodi community serving predominantly low socioeconomic class people. All the neonates delivered during the study period were included in the screening. The screening was performed within 72 h of delivery using automated auditory brainstem response (AABR) and repeated after 6 weeks among those with referral result. Subsequently the neonates were referred to diagnostic audiology., Result: Among the 453 newborns (231 males and 222 female), AABR screening showed referral, in 43.7% of neonates. At first screening, 224 (49.4%) were referred while 229 (50.6%) passed, however, during the post-natal period 40/229 (17.5%) reported for second screening, out of these 26 showed pass to the screening. This gave a total pass of 255/453 (56.3%). The presence of maternal pre-ecclampsia (P = 0.05) was found to be a significant morbidity factor associated with referral in the screening, while parental socioepidemiological variables; and the neonates' birthweight, gestational age and APGAR score were not., Conclusion: The proportion of referral on hearing screening encountered was far higher than previously reported, however, continuation of infant screening in future should be comprehensive with viral and genetic analysis in order to address the issue of aetiologic diagnosis; in addition, the implementation should factor the high drop out from the first stage screening in order to substantiate the findings in our region., (Copyright © 2014. Published by Elsevier Ireland Ltd.)

Objectives: Measuring parent satisfaction with neonatal hearing screening programs is one of the most important indicators for assessing the quality and effectiveness and a useful instrument to identify service shortfalls. The aims of this study were to translate and adapt Parent Satisfaction Questionnaire with Neonatal Hearing Screening Programs (PSQ-NHSPs) to Persian language, determine the validity and reliability of this translation, and determine the level of parent satisfaction with neonatal hearing screening programs in Iran., Methods: Translated Persian version of the original English PSQ-HNSPs was presented to 138 parents whose children had received hearing screening before discharge from hospital., Results: The majority of parents responded strongly agree or agree to all dimensions of the questionnaire: overall satisfaction (90.6%), personnel in charge of hearing test (60.1%), and appointment activities (58%). Good internal consistency reliability (a=0.89) and moderate test-retest reliability (r=0.61) of the Persian version of PSQ-NHSPs were indicated. Construct validity was demonstrated by a significant positive relationship between overall satisfaction and specific dimensions in the questionnaire., Conclusion: The Persian version of PSQ-NHSPs is a valid and reliable tool to assess the level of parent satisfaction with neonatal hearing screening programs., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Unlabelled: Deafness is commonest curable childhood handicap. Most remedies and programmes don't address this issue at childhood level leading to detrimental impact on development of newborns. Aims and objectives are (A) screen all newborns for deafness and detect prevalence of deafness in children less than 2 years of age. and (B) assess efficacy of multi-staged OAE/ABR protocol for hearing screening., Methodology: Non-randomized, prospective study from August 2008 to August 2011. All infants underwent a series of oto-acoustic emission (OAE) and final confirmatory auditory brainstem evoked response (ABR) audiometry. Finally, out of 1,101 children, 1,069 children passed the test while 12 children had impaired hearing after final testing, confirmed by ABR. Positive predictive value of OAE after multiple test increased to 100 %. OAE-ABR test series is effective in screening neonates and multiple tests reduce economic burden. High risk screening will miss nearly 50 % deaf children, thus universal screening is indispensable in picking early deafness.