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

The purpose of the present systematic review was to synthesize evidence on associated risk factors of hearing loss (HL) in children. Evidence-based research articles on HL published between January 2013 and December 2022 using PubMed, Cochrane, and Scopus databases were searched. The study included children between zero and three years of age who have permanent bilateral/unilateral HL (BHL/UHL) by employing case-control studies, randomized controlled trials, nonrandomized studies, prospective or retrospective cohort studies, and studies with or without comparison groups. The Newcastle-Ottawa Scale (NOS) and the Joanna Briggs Institute (JBI) critical appraisal checklist for longitudinal and cross-sectional studies were used to rate the quality of the chosen studies. The studies that would be considered were reviewed by two independent authors, and a third author was contacted if there was a dispute. A preliminary literature search uncovered 505 articles from the electronic search and 41 studies by hand searching. Duplicate records were eliminated, leaving 432 records. The abstract and title were read, and 340 studies were eliminated. There were 92 articles in total that qualified for full-text screening. Among these, 75 articles were disregarded since they lacked information or failed to assess the risk factors for HL. The qualitative synthesis, therefore, included 17 articles. Most often, cross-sectional study designs were used in the studies that were reviewed, which were then followed by longitudinal studies. Three of the studies that were reviewed used a prospective cohort design. The quality of all the included studies was rated to be good. The current review revealed that the primary statistically significant risk factors for HL included ventilator support; craniofacial anomalies; low birth weight (LBW); forceps delivery; loop diuretics; meningitis; asphyxia; intensive care; consanguinity; sepsis; Apgar scores between 0 and 4 at one minute; toxoplasmosis, other agents, rubella, cytomegalovirus, and herpes (TORCH) infections; and hyperbilirubinemia.

Newborn hearing screening (NHS) was implemented in Albania in four maternity hospitals in 2018 and 2019. Implementation outcome, screening outcome, and screening quality measures were evaluated. Infants were first screened by midwives and nurses before discharge from the maternity hospital and returned for follow-up screening. Acceptability, appropriateness, feasibility, adoption, fidelity, coverage, attendance, and stepwise and final-referral rates were assessed by onsite observations, interviews, questionnaires, and a screening database. A post hoc analysis was performed to identify reasons for loss to follow up (LTFU) in a multivariate logistic regression. In total, 22,818 infants were born, of which 96.6% were screened. For the second screening step, 33.6% of infants were LTFU, 40.4% for the third, and 35.8% for diagnostic assessment. Twenty-two (0.1%) were diagnosed with hearing loss of ≥40 dB, six unilateral. NHS was appropriate and feasible: most infants are born in maternity hospitals, hence nurses and midwives could perform screening, and screening rooms and logistic support were supplied. Adoption among screeners was good. Referral rates decreased steadily, reflecting increasing skill. Occasionally, screening was repeated during a screening step, contrary to the protocol. NHS in Albania was implemented successfully, though LTFU was high. It is important to have effective data tracking and supervision throughout the screening.

The aim of the study to screen the neonates for earlier diagnosis of sensorineural hearing loss and to assess the relationship between the hearing loss in newborns along with the high risk factors. A prospective, cohort, observational analytical study done at department of ENT,MGMMC & MYH INDORE (M.P.) carried out during 2018-2019 over 200 neonates who were randomly selected screened by OAE & BERA before their discharge from the hospital and after stabilizing high risk neonates. Out of 200 neonates, sensorineural hearing loss found to be in 4 neonates (2%) and hearing impairment seen 13.8 times more in high risk neonates as compare to the low risk neonates. The core goal of the study was to emphasize the importance of universal newborn hearing screening for early diagnosis & intervention in newborns & Neonates in terms of auditory rehabilitation as every child is precious and hearing is their fundamental right.

With widespread agreement on the importance of early identification of hearing loss, universal newborn hearing screening (UNHS) has become the standard of care in several countries. Despite advancements in screening technology, UNHS and early hearing detection and intervention programs continue to be burdened by high referral rates of false-positive cases due to temporary obstruction of sound in the outer/middle ear at birth. A sensitive adjunct test of middle ear at the time of screening would aid in the interpretation of screening outcomes, minimize unnecessary rescreens, and prioritize referral to diagnostic assessment for infants with permanent congenital hearing loss. Determination of middle ear status is also an important aspect of diagnostic assessment in infants. Standard single-frequency tympanometry used to determine middle ear status in infants is neither efficient nor accurate in newborns and young infants. A growing body of research has demonstrated the utility of wideband acoustic immittance (WAI) testing in both screening and diagnostic settings. Wideband power absorbance (WBA), a WAI measure, has been shown to be more sensitive than tympanometry in the assessment of outer/middle ear function in newborns. Furthermore, age-graded norms also support successful application of WBA in young infants. Despite its merits, uptake of this technology is low among pediatric audiologists and hearing screening health workers. This report describes normative data, methods for assessment and interpretation of WBA, test-retest variations, and other factors pertinent to clinical use of WAI in newborns and infants. Clinical cases illustrate the use of WAI testing in newborn and infant hearing assessment.

OBJECTIVE: We have clarified the role of Universal Neonatal Hearing Screening (UNHS) for both early diagnosis and rapid treatment in order to improve the prognosis of the deaf child and reduce patient management costs. Although in Sicily UNHS has been progressively implemented, there is scarce data in the literature on this matter. Therefore, the main objective was to collect in the year 2018 the following data: number of newborns screened for hearing loss, number of infants \"referred\" to transiently evoked otoacoustic emissions (TEOAE), number of infants with pathologic auditory brainstem response (ABR) and number of infants affected by permanent hearing loss.
METHODS: UNHS monitoring was conducted through the collection of data through a questionnaire, which was analysed evaluating the effectiveness and adherence to the screening program prepared by the Department for Health Activities and the Epidemiological Observatory (DASOE).
RESULTS: In 2018, there were 40,243 newborns in Sicily. A total of 37,562 newborns were screened (93.3%). There were 1,328 \"referred\" infants with TEOAE (3.5%). On the 2nd level, \"referred\" newborns examined were 1,080 of 1,328 expected (missing 248 \"refer\" newborns, equal to 18.6%). The number of \"referred\" infants confirmed with TEOAE was 113 of 1,080, while \"referred\" infants confirmed with ABR were 71. On the 3rd level, 67 of 71 were infants examined: 28 infants were suffering from monolateral hearing loss (13 slight/mild, 13 moderate, 1 severe and 1 profound) and 39 from bilateral hearing loss (1slight/mild, 19 moderate, 13 severe and 7 profound). Excluding 7 infants from the NICU, 60 of 37,562 infants had hearing loss (1.5%).
CONCLUSIONS: The monitoring of the UNHS in Sicily has allowed obtaining the data of individual centres, absent in the literature to date, to verify the effectiveness of the screening, according to JCIH criteria, to highlight some criticalities and, finally, to propose possible solutions.
Screening uditivo neonatale universale nella regione Sicilia nel 2018.
UNASSIGNED: Abbiamo chiarito il ruolo dello screening uditivo neonatale universale (UNHS) sia come diagnosi precoce che come trattamento rapido al fine di migliorare la prognosi del bambino sordo e ridurre i costi di gestione del paziente. Anche se in Sicilia l’UNHS è stato progressivamente attuato, vi sono scarsi dati nella letteratura in materia. Pertanto, l’obiettivo principale è stato quello di raccogliere nell’anno 2018 i seguenti dati: numero di neonati sottoposti a screening per la perdita dell’udito, numero di neonati risultati “refer” alle TEOAE, numero di lattanti con ABR patologico e numero di lattanti affetti da perdita permanente dell’udito.
METHODS: Il monitoraggio del UNHS è stato condotto attraverso la raccolta dei dati forniti dal questionario, che valuta l’efficacia dello screening e l’aderenza alle fasi successive.
UNASSIGNED: Nel 2018, i nati in Sicilia sono stati 40.243. Sono stati sottoposti a screening 37.562 neonati, pari al 93,3%. Sono risultati “refer” con le TEOAE 1.328 neonati (pari al 3,5%). Al 2° livello, i neonati “refer” esaminati sono stati 1.080 su 1.328 attesi (dispersi 248 neonati refer, pari al 18,6%). I neonati “refer” confermati con le TEOAE sono stati 113 su 1.080, mentre i neonati “refer” confermati con ABR sono stati 71. Al 3° livello, i bambini esaminati sono stati 67 su 71 previsti: ventotto bambini soffrivano di perdita monolaterale dell’udito (13 lieve, 13 moderata, 1 grave e 1 profonda) e trentanove soffrivano di perdita bilaterale dell’udito (1 lieve, 19 moderata, 13 grave e 7 profonda). Escludendo 7 bambini provenienti dalla NICU, i bambini con perdita dell’udito sono stati 60 su 37.562 (pari all’1,5‰).
CONCLUSIONS: Il monitoraggio dell’UNHS in Sicilia ha permesso di ottenere i dati dei singoli centri, dati assenti nella letteratura, di verificare l’efficacia dello screening, secondo criteri JCIH, di evidenziare alcune criticità e, infine, di proporre possibili soluzioni.

OBJECTIVE: Most developed countries have implemented some form of universal newborn hearing screening program. Early identification and rehabilitation of congenital hearing loss is important in functional outcome, and the need to identify the cause of hearing impairment has become clear. We aimed to evaluate audiological and etiological outcomes in a large group of patients with failed neonatal hearing screening.
METHODS: We performed a retrospective chart analysis of patients who were referred to our tertiary referral center after failing neonatal hearing screening during a 12-year period (2007-2019). Screening was based on automated auditory brainstem response (AABR) or a combined approach of AABR and auditory steady-state response (ASSR) with chirp stimulus. Extensive audiometric testing was performed to confirm and determine the type and degree of hearing loss. In case of permanent hearing loss, a standardized etiological protocol was followed to determine the cause.
RESULTS: Of the 802 referred newborns, hearing loss was confirmed by diagnostic ABR in 78%. Main causes of hearing loss included otitis media with effusion (56%, higher in patients screened by AABR/ASSR compared to AABR), a genetic disorder (12%), congenital cytomegalovirus infection (cCMV, 5%) and atresia/stenosis of the external ear canal (5%). Of the patients with permanent hearing loss, 15% showed changes in hearing loss severity over time.
CONCLUSIONS: In the majority of newborns referred after failing universal neonatal hearing screening, hearing loss could be confirmed. The leading cause was reversible hearing loss due to otitis media with effusion, but hearing loss proved permanent in about 35% of referred newborns, with genetics as predominant cause. Follow-up of congenital hearing loss patients is important as deterioration as well as improvement was observed over time.

OBJECTIVE: Pediatric deafness is an important consideration in neurodevelopment. Early identification and intervention are major factors in seeing that deaf children reach their full potential. Often, it is the medical professionals who themselves have limited knowledge about hearing loss or the consequences of delayed language acquisition. These knowledge gaps can negatively influence the timeous and holistic care that children with hearing loss require. With a dual experiential expertise gained through both parenting children with disabling hearing loss and being medical doctors, the purpose of this study was to better understand the field of pediatric hearing loss through doctors\' insights gained as parents.
METHODS: Interpretative Phenomenological Analysis was the approach used for this qualitative enquiry. Five South African participants with children between the ages of two and ten years, were selected using purposive sampling and an in-depth semi-structured interview used as the data instrument. Thereafter, three levels of thematic analysis were conducted.
RESULTS: Generally, doctors have limited knowledge of pediatric deafness. Perceptions towards deafness are typically through a medical-model lens, while appreciation of Universal Newborn Hearing Screening is lacking. Through exploring the various gains of parenting a deaf child, participants acknowledged the power of the lived experience in changing their own practice as both parents and practitioners.
CONCLUSIONS: Doctors need to know more about pediatric deafness. The pathway of care from identification to intervention in South Africa needs revision. Exposure to the lived experience is a powerful means of enabling expert insights to influence such change in a practical and meaningful way.

To evaluate and discuss the outcomes of the universal newborn hearing screening program conducted at four public hospitals in Malaysia.
A retrospective analysis of the universal newborn hearing screening database from each hospital was performed. The database consisted of 28,432 and 30,340 screening results of babies born in 2015 and 2016, respectively. Quality indicators (coverage rate, referral rate, return for follow-up rate, and ages at screening and diagnosis) were calculated.
Overall coverage rate across the four hospitals was 75% in 2015 and 87.4% in 2016. Over the two years, the referral rates for the first screening ranged from 2.7% to 33.93% with only one hospital achieving the recommended benchmark of <4% in both years. The return for follow-up rates for each participating hospital was generally below the recommended benchmark of ≥95%. The mean age at screening was 3.9 ± 1.2 days and 3.3 ± 0.4 days, respectively. The mean age at diagnosis for 70 infants diagnosed with permanent hearing loss was 4.7 ± 0.7 months in 2015 and 3.6 ± 0.9 months in 2016.
Quality measures for the universal newborn hearing screening program in four public hospitals in Malaysia were lower than the required standards. Nevertheless, some quality indicators showed statistically significant improvements over the two years. Next steps involve identifying and implementing the best practice strategies to improve the outcome measures and thus the quality of the program.

Newborn hearing screening (NHS) can reduce the economic and social burden of hearing impairment. To track the progress of the goal set by the National Program of Action for Child Development (NPACD) and to estimate the detection rates of hearing impairment, the present study examined NHS coverage in 2008-2010 and 2016 and the detection of hearing impairment across China in 2016.
Licensed medical institutions across China were surveyed in 2012 and 2018 by the National Center for Birth Defects Monitoring of China to collect data for the 2008-2010 period and for 2016 on live births, initial screening rates (total and referral), secondary screening rates (total and referral), and rates of hearing impairment diagnosis among infants who were referred in the secondary screening. To calculate universal newborn hearing screening (UNHS) coverage, the number of newborns who received NHS within 4 weeks after birth was divided by the number of live births. The detection rate of hearing impairment was calculated by combining referral rates on primary and secondary screening with the rate of diagnosis.
National UNHS coverage increased from 29.9% in 2008 to 86.5% in 2016, with different regions showing different increases. During this period, the number of provinces with UNHS coverage over 90.0% increased from 2 to 17, with UNHS coverage in 2016 being substantially higher in eastern provinces (93.1%) than in western provinces (79.4%). In 2016, the detection rate of hearing impairment across the country was 0.23% (95% CI 0.15-0.25%), and it varied from 0.17% in western provinces to 0.22% in central provinces and 0.28% in eastern provinces. The lowest rate was 0.02% in Heilongjiang Province and the highest rate was 0.63% in Hainan Province.
National UNHS coverage increased substantially from 2008 to 2016, although provinces and regions still showed differences. The detection rate of infant hearing impairment in China is comparable to that in other countries. A national individual-level information system is urgently needed in China to facilitate the integration of screening, diagnosis and treatment of infant hearing impairment, which may also lead to a more accurate estimate of the detection rate.