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.

OBJECTIVE: To observe and analyse the hearing outcome in infants with mild-to-moderate sensorineural hearing loss (SNHL) who failed universal newborn hearing screening (UNHS).
METHODS: This retrospective cohort analysis included infants with mild-to-moderate SNHL and with complete etiological diagnosis and followed up over three years.
RESULTS: Out of 96 infants with mild-to-moderate SNHL 72 were stable (75%). Only one case was normal (1.04%), ten cases were improved (10.42%), and 13 were deteriorated (13.54%). The pathogenic mutation of GJB2 was the most common cause (50/96, 52.08%), and most of them were homozygous or complex heterozygous mutations of p.V37I (44/50, 88%). There were 11 cases (11.49%) with large vestibular aqueduct syndrome (LVAS) and nine cases (9.38%) with perinatal risk factors. Infants with GJB2 pathogenic mutation and those without certain etiology mostly had unchanged hearing levels, accounting for 84% (42/50) and 84.61% (22/26), respectively. Hearing deterioration in LVAS was associated with seven cases (63.64%). There was no difference in types of outcomes in perinatal risk factor infants, who were more likely to improve than the other groups, but there were three cases (33.3%) deteriorated to profound hearing loss. Comparison of outcomes of different etiologies showed statistically significant difference (Chi-square = 28.673, p = 0.000).
CONCLUSIONS: Normal and improved hearing in infants with mild-to-moderate SNHL was rare before the age of three, unlike in many previous studies, and appropriate intervention is recommended. However, intervention should be adjusted according to the hearing outcomes because of the possibility of improvement or deterioration. The etiological diagnosis of infants with mild-to-moderate SNHL would be helpful for predicting the outcome and managing intervention.

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.

BACKGROUND: National recommendations in the United States specify that all infants with hearing impairment should be identified by 3 months of age. Infants who fail universal newborn hearing screening (UNHS) require follow up testing after hospital discharge. Follow up testing may be difficult to obtain in some communities within the ideal time frame. A rapid access multidisciplinary clinic was established for failed UNHS. The objective of this study is to report outcomes and patient satisfaction from an early access hearing detection clinic.
METHODS: Infants that failed UNHS were seen in the multidisciplinary clinic between 1/1/19 and 2/28/22. Patients underwent automated auditory brainstem response (ABR) and distortion product otoacoustic emissions testing and consulted with an otolaryngology nurse practitioner. Failed results were followed by diagnostic ABR. Surveys were administered at the beginning and end of the appointment.
RESULTS: In total, 169 infants were seen at a mean age of 8.4 weeks (95%CI 7.5, 9.4). Repeat testing was abnormal in 38 (22.4%). Diagnostic ABR was performed at an average age of 13.7 weeks (n = 34, 95% CI: 10.8, 16.6) and led to a diagnosis of hearing loss in 18 infants. Twenty-seven parents completed surveys at the initial visit. Anxiety level among patients with normal repeat testing (n = 20) decreased from 1.9 to 1.2 (p = .002), while anxiety level among those with abnormal repeat testing (n = 7) was not statistically different before and after (2.1 vs 2.7, p = .2). Satisfaction level was 3.7 ± 0.7 (scored 1-4). All parents reported having a better understanding of their child\'s hearing problem after the visit.
CONCLUSIONS: This novel nurse practitioner-led early hearing detection clinic enabled timely diagnosis of hearing loss and reassurance to families without hearing loss. Age at hearing loss diagnosis compares favorably to published cohorts.

Hearing impairment is the most frequent of the sensorial defects in humans, and if not treated promptly, can severely impair cognitive and spoken language skills. For this reason, a universal newborn hearing screening (UNHS) has been established. The purpose of our study is to examine, by means of a retrospective analysis, the results of the UNHS program in the Umbria region during the spread of COVID-19 (2020-2021), comparing the same data from the years 2011-2012, to understand if the program has improved. Our study has shown how the coverage rate of well born babies\' (WB) screening has significantly increased to currently meet the JCIH benchmark. The percentage of WB referrals significantly decreased in 2020-2021, another indicator of the screening program\'s greater efficiency in Umbria. However, a critical issue has emerged: the percentage of those lost to follow-up is greater than 30%, well above the benchmark. As far as the COVID-19 pandemic has certainly had a significant impact, it is necessary to carefully monitor those who do not access the diagnostic level. To emphasize the importance of a proper screening program, it will be helpful to strengthen the computerized data collection system and create an information network between audiologists, pediatricians and families.

OBJECTIVE: This investigation was to ascertain the performance of the UNHS in Taiwan.
METHODS: The predefined questionnaire was delivered on the phone in 2016. The descriptive analysis was applied to the research data. 941 neonates in birth cohorts 2013-2014 who were documented as a bilateral referral in the national UNHS tracking system were targeted. The respondents were either caregivers or family members.
RESULTS: 40.3% of 941 children were lost to follow-up, and 66.24% of 363 children were diagnosed with SNHL. 45.15% of 163 children used hearing amplification device(s). 77.46% of hearing amplification device users and 7.51% of non-users participated in the auditory training courses. By six months of age, 38.51% and 22.58% of children diagnosed with bilateral SNHL commenced the hearing amplification device fitting and the auditory training courses, respectively.
CONCLUSIONS: More efforts are needed to enhance the performance of the UNHS to achieve national goals stated in the 2014 Taiwan UNHS Revised Guidelines and the well-known benchmarks set by the JCIH in 2007. The development of an electronic tracking system for storing and sharing information on the follow-up on children with congenital hearing loss was as significant as the improvements in the understanding of early hearing detection and intervention of the public and stakeholders.

BACKGROUND: Nowadays, due to universal newborn hearing screening (UNHS) the number of children with mild-to-moderate hearing loss diagnosed in the first year of life has increased significantly. Aside from that, identification of the genetic cause improves the genetic counselling of the families and allows to reveal possible comorbidities which may need a special approach.
OBJECTIVE: To present the characteristics of the early audiologic phenotype in hearing impaired patients with biallelic mutations in the USH2A gene based on systematic analysis of the audiological data.
METHODS: 13 patients with mutations in the USH2A gene underwent audiological examination. Most of them were found among a large group of infants with bilateral nonsyndromic sensorineural hearing loss (SNHL) examined under 12 months.
RESULTS: Eight out of eleven children failed UNHS and were initially diagnosed as having bilateral nonsyndromic SNHL. Seven children underwent an audiological assessment before the age of 9 months. The earliest audiological examination was carried out at 1 and 3 months. The children with pathogenic variants in the USH2A gene in our examined group were identified in the first year of life via UNHS. The hearing threshold levels (HTL) for the USH2A group are compactly distributed between 51.25 dB and 66.25 dB, quartiles are 54 dB and 63.4 dB, with a median of 60 dB. The audiological profile of patients with biallelic USH2A mutations differs from audiograms of patients who had STRC-related hearing loss. We have not found any significant elevation in hearing thresholds in the first decade of life. We also estimated the prevalence of the USH2A and STRC mutations among GJB2-negative infants with bilateral nonsyndromic SNHL examined under 12 months, and it was 7.5% and 16.1%, respectively.
CONCLUSIONS: According to our results, the early hearing phenotype in pediatric patients with biallelic mutations in the USH2A- gene is characterized by nonsyndromic mild-to-moderate SNHL in the first decade of life. Our results indicate that the presence of mutations in the USH2A or STRC genes can be expected in a child with congenital mild-to-moderate nonsyndromic SNHL. This information is of practical importance for parents, as they have to know the prognosis of hearing loss for their child from the very beginning. Post-screening follow-up should include adequate clinical, genetic, and social support for children and their parents.