Otolaryngology outcomes of infants with conductive hearing loss identified through universal newborn hearing screening.

OBJECTIVE: Infants and children diagnosed with a conductive hearing loss (CHL) are often referred for otolaryngology assessment. Although this is also a regular occurrence for infants diagnosed with a CHL through Universal Newborn Hearing Screening (UNHS), less is known about these infants and their outcomes. Using a cohort of infants diagnosed with CHL through UNHS and referred to otolaryngology, this study aimed to investigate the relationship between specific demographic or clinical characteristics and 1) triage category 2) middle ear diagnosis and intervention and, 3) service-related factors at otolaryngology. METHODS: Retrospective analysis through clinical chart review was performed on all infants born between January 2014 and December 2017 who referred on UNHS, diagnosed with a CHL and referred to the Queensland Children's Hospital. Descriptive analysis and Chi squared analysis was conducted on data from 95 records. RESULTS: Analysis between all infants referred from UNHS and those who referred, diagnosed with CHL and then referred to otolaryngology suggest that bilateral referrals/medical exclusion, preterm and infants with ≥1 risk factors are more readily associated with referral to otolaryngology for CHL. Nearly all (92.86 %) infants who were referred to otolaryngology had a primary diagnosis of OM and most infants (89.66 %) received grommets as an intervention. The average age of first appointment at otolaryngology was 427 days, the average age of intervention was 579 days and the average occasions of service at otolaryngology was 6.72. CONCLUSION: This paper provides a snapshot into the journey and outcomes of infants referred from UNHS, diagnosed with CHL, and referred to otolaryngology. Further investigation in both general and UNHS populations is needed to better understand and apply these findings.

The congenital hearing phenotype in GJB2 in Queensland, Australia: V37I and mild hearing loss predominates.

GJB2 was originally identified in severe, non-syndromic sensorineural hearing loss (SNHL), but was subsequently associated with mild and moderate SNHL. Given the increasing utilisation of genetic testing pre-conceptually, prenatally, and neonatally, it is crucial to understand genotype-phenotype correlations. This study evaluated the nature and frequency of GJB2 variants in an Australian paediatric population with varying degrees of SNHL ascertained through newborn hearing screening. Audiograms from individuals with GJB2 variants and/or a GJB6 deletion (GJB6-D13S11830) were retrospectively reviewed (n = 127). Two-thirds were biallelic (homozygous/compound heterozygous) for pathogenic/likely pathogenic variants of GJB2 and/or GJB6 (n = 80). The most frequent variant was c.109 G > A, followed by c.35delG and c.101 T > C. Compared to biallelic carriage of other GJB2 variants, c.109 G > A positive individuals (homozygous/compound heterozygous) were more likely to have mild HL at their initial and latest audiograms (p = 0.0004). Biallelic carriage of c.35delG was associated with moderately-severe or greater SNHL at both initial and latest audiograms (p = 0.007). The c.101 T > C variant presented with milder SNHL and U-shaped audiograms (p = 0.02). In this agnostically identified cohort, mild SNHL predominated in GJB2/GJB6 carriers in contrast to previous studies targeting individuals with significant loss. Consequently, c.109 G > A, associated with milder phenotypes, was the most frequent. This study provides valuable data to support prognostic confidence in genetic counselling.

Mild matters: trial learnings and importance of community engagement in research for early identified bilateral mild hearing loss.

Introduction: Early identification of mild hearing loss has resulted in early hearing amplification without adequate evidence of effectiveness. This paper describes learnings from a pilot trial, combined with a qualitative study, to highlight the importance of community engagement in designing research studies to determine whether early amplification benefits young children with bilateral mild hearing loss. Methods: PART 1 of the study is a proof-of-concept non-blinded multi-centre randomised controlled trial (RCT) of hearing device fitting vs. no fitting aimed to gather preliminary data and determine its acceptability/feasibility in children <2 years old with bilateral mild hearing loss. Results: PART 2 is a qualitative study to understand the barriers/enablers to RCT participation. Of 40 potentially eligible families, nine (23%) declined, three were uncontactable (7%), 26 (65%) ineligible: of these, nine (35%) did not meet hearing threshold inclusion criteria, 11 (42%) were already fitted or had made decisions on fitting hearing device, two (7%) had conductive loss and four (16%) were ineligible for other reasons. Two of 11 (18%) eligible families were randomised. With the limited sample size, outcome measures were not compared between groups. Both participants completed the trial, reported the RCT to be acceptable, and neither changed group post-enrolment. Discussion: Whilst recruitment uptake could potentially be increased by altering the eligibility criteria, better communication with and reimbursement of clinicians as recruiters, and improving awareness of the study amongst external stakeholders, the RCT methodology does not conform to family-centred practice, and potentially raises ethical concerns regarding potential adverse consequences of not offering early amplification. Parental perception of losing control over choice of management due to randomisation is not an easily modifiable factor. Alternative methodological approaches without randomisation are required to determine whether hearing amplification benefits infants with mild hearing loss.Clinical Trial Registration: identifier [ACTRN12618001608257].

Conductive hearing loss in newborns: Hearing profile, risk factors, and occasions of service.

OBJECTIVE: Infants diagnosed with a conductive hearing loss (CHL) are at increased risk of developmental delays. Using a sample of infants diagnosed with CHL through UNHS, this study aimed to investigate the relationship between specific demographic or clinical characteristics and 1) occasions of service to reach a hearing diagnosis and 2) the profile of CHL. METHODS: Retrospective analysis was conducted for all infants with CHL born between 01/01/2007 and 31/12/2018 who had received UNHS. Chi squared analysis was conducted on data from 1208 records. RESULTS: Infants with ≥1 risk factor for hearing loss were more likely to attend more than three occasions of service. Infants who were bilateral refer/medical exclusion, Torres Strait Islander, had ≥1 risk factors for hearing loss or were born pre-term had greater proportions of bilateral CHL than unilateral CHL. Mild to moderate was the most frequent degree of CHL, although a unilateral or bilateral CHL did not have an association with the severity of CHL. Compared to other risk factors, infants with a syndrome had greater proportions of bilateral than unilateral CHL. Risk factors of craniofacial abnormality, prolonged ventilation, or syndrome had greater proportions of mild to moderate CHL than moderate or greater. On average, infants were diagnosed with a CHL at 37.29 weeks of age. CONCLUSION: These findings highlight the relationship between clinical/demographic characteristics and occasions of service to diagnose CHL in children, including the CHL profile. An understanding of this relationship may help clinicians to better plan, assess and manage infants diagnosed with a CHL through UNHS.

Neonatal bacterial meningitis: hearing screening and audiological monitoring outcomes.

OBJECTIVE: This study reviewed the outcomes of universal newborn hearing screening (UNHS) and ongoing hearing monitoring in children following recovery from neonatal bacterial meningitis to determine (a) whether screening may be a suitable alternative to diagnostic audiology for detecting permanent childhood hearing loss (PCHL) and (b) whether infants who pass UNHS should be monitored throughout childhood. DESIGN: Retrospective analysis of a UNHS database. STUDY SAMPLE: Data were extracted from the state-wide UNHS database for all children born in Queensland Australia between 1 September 2004 and 30 June 2020 with the risk factor of bacterial meningitis (in isolation or in combination with other risk factors) identified at the time of the UNHS. This cohort included 231 children. RESULTS: Results showed that all post-meningitic infants diagnosed with PCHL had a refer result on the UNHS or were medically excluded from screening. Additionally, no cases of PCHL were identified through the targeted surveillance program following a pass result on UNHS. CONCLUSIONS: UNHS may be sufficient to detect PCHL in post-meningitic neonates and routine audiological monitoring may not be required for children who pass the screen.

Positive predictive value of dual-modality vision screening in school children 4-7 years of age-a retrospective review in Queensland, Australia.

PURPOSE: To report the outcomes and positive predictive value (PPV) of vision screening in schoolchildren 4-7 years of age through the Primary School Nurse Health Readiness Program (PSNHRP) in Queensland, Australia. METHODS: A retrospective review of schoolchildren who underwent vision screening between January 2017 and December 2020 was conducted. Vision screening was performed through a dual-examination method, using the Parr 4m letter-matching vision test with crowding bars and the Spot photoscreener. Children were referred to an optometrist or ophthalmologist for review as required and if they failed either screening modality. PPVs were calculated based on whether a visual abnormality was confirmed by an ophthalmologist or optometrist. RESULTS: Of 185,685 eligible children, 176,164 (94.9%) consented to vision screening, 164,890 (93.6%) consented children underwent vision screening, and of those 12,148 (7.4%) were referred for an eye assessment. Of the 8,659 children with a known outcome (71.3% of referred), 6,011 (69.4% of known outcomes) had a confirmed visual abnormality and 2,648 (30.6%) children did not. The PPV was 0.73 when a referral was indicated by the photoscreener result, 0.76 when indicated by visual acuity testing, and 0.91 when indicated by both the photoscreener and visual acuity testing. CONCLUSIONS: The PSNHRP vision screening program showed a high uptake, and the dual screening method was effective in identifying visual abnormalities, with higher PPV when both visual acuity and photoscreener results indicated a need for referral.

Childhood hearing loss detected beyond the newborn screen.

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

Watch me grow integrated (WMG-I): protocol for a cluster randomised controlled trial of a web-based surveillance approach for developmental screening in primary care settings.

INTRODUCTION: The increasing prevalence of developmental disorders in early childhood poses a significant global health burden. Early detection of developmental problems is vital to ensure timely access to early intervention, and universal developmental surveillance is recommended best practice for identifying issues. Despite this, there is currently considerable variation in developmental surveillance and screening between Australian states and territories and low rates of developmental screening uptake by parents. This study aims to evaluate an innovative web-based developmental surveillance programme and a sustainable approach to referral and care pathways, linking primary care general practice (GP) services that fall under federal policy responsibility and state government-funded child health services. METHODS AND ANALYSIS: The proposed study describes a longitudinal cluster randomised controlled trial (c-RCT) comparing a 'Watch Me Grow Integrated' (WMG-I) approach for developmental screening, to Surveillance as Usual (SaU) in GPs. Forty practices will be recruited across New South Wales and Queensland, and randomly allocated into either the (1) WMG-I or (2) SaU group. A cohort of 2000 children will be recruited during their 18-month vaccination visit or opportunistic visit to GP. At the end of the c-RCT, a qualitative study using focus groups/interviews will evaluate parent and practitioner views of the WMG-I programme and inform national and state policy recommendations. ETHICS AND DISSEMINATION: The South Western Sydney Local Health District (2020/ETH01625), UNSW Sydney (2020/ETH01625) and University of Queensland (2021/HE000667) Human Research Ethics Committees independently reviewed and approved this study. Findings will be reported to the funding bodies, study institutes and partners; families and peer-reviewed conferences/publications. TRIAL REGISTRATION NUMBER: ANZCTR12621000680864.

Clinical characteristics of infants identified with a conductive hearing loss through universal newborn hearing screening: A population-based sample.

BACKGROUND: Universal Newborn Hearing Screening (UNHS) aims to identify infants born with a permanent hearing loss. However, many are also diagnosed with a conductive hearing loss (CHL) and are at subsequent risk for developmental delays. The aim of this study was to investigate the prevalence of CHL and determine which clinical characteristics collected at birth, predict CHL within UNHS. MATERIALS AND METHODS: Retrospective analysis was conducted on all infants born between January 01, 2007 and December 31, 2018. During this period, 731,234 infants were screened, 9802 were direct referrals, and 1208 identified with a CHL. Chi squared analysis and logistic regression was conducted to determine CHL prevalence and identify which clinical characteristics predict CHL. RESULTS: The prevalence of CHL was 12.32%. Following adjustments for collinearity, clinical characteristics that could predict CHL were: bilateral referrals/medical exclusions to screen (Odds ratio, OR 1.89; 95% CI: 1.65-2.1), ≥1 risk factor for hearing loss (OR 2.03; 95% CI: 1.76-2.34), pre-term birth (OR 1.82; 95% CI: 1.57-2.10), male (OR 1.21; 95% CI: 1.07-1.37), and Indigenous status: 'Aboriginal (not Torres Strait Islander)' (OR 1.27; 95% CI:1.03-1.57 and 'not stated' (OR 2.95; 95% CI: 2.02-4.30). CONCLUSION: CHL within UNHS was highly prevalent, with six clinical characteristics that can predict that likelihood of an infant being diagnosed with a CHL. This data could be used to create alternative care pathways for infants with CHL, enabling early and targeted assessments, thereby reducing the risk of developmental delays for these infants.

Cochlear nerve deficiency is an important cause of auditory neuropathy spectrum disorder at a population level in children.

OBJECTIVES: To determine the incidence, prevalence and describe risk factors and etiology for childhood Auditory Neuropathy Spectrum Disorder using population level data from a statewide universal newborn hearing program. METHODS: A retrospective statewide universal newborn hearing screening database review and descriptive analysis from 2012 to 2019 of demographic, risk factors and hearing loss etiology for babies with sensorineural hearing loss and ANSD was completed. A 2 stage aABR protocol was used and ANSD was classified when click evoked ABR were absent or grossly abnormal but otoacoustic emissions and or cochlear microphonics were present. Medical evaluation and investigation by a pediatrician or otolaryngologist was performed and etiology was assigned using a coding scheme. Next generation genetic sequencing was not available. RESULTS: From 2012 to 2019, 487 636 babies were screened for congenital hearing loss (99.1%) and 1150 were confirmed to have permanent SNHL, 80 of whom were diagnosed with ANSD (52 unilateral and 28 bilateral). The prevalence of ANSD was 7.0% and population prevalence was 0.16 per 1000 live births. The only demographic or risk factor significantly more likely to be associated with ANSD than SNHL was hyperbilirubinemia. The most common etiology for ANSD was hypoplasia or absence of the cochlear nerve with 37 cases (46.3%), and it was significantly more likely with unilateral than bilateral ANSD. CONCLUSION: At a population level, ANSD was more likely to be unilateral and the only perinatal risk factor significantly associated was hyperbilirubinemia. Cochlear nerve deficiency was the most common etiology. Given that this can occur in well babies, this provides further evidence for aABR as a preferred mode for newborn hearing screening.

Minimum Data Set for Families of Children With Hearing Loss: An eDelphi Study.

PURPOSE: Assessing the unique needs of each family following the diagnosis of a hearing loss is central to the delivery of family-centered hearing health care. Therefore, the aim of this study was to develop a Minimum Data Set (MDS) that could be used in the design of a needs assessment tool for families of children with hearing loss transitioning to early intervention. METHOD: A list of potential items for the MDS was prepared. In a two-round electronic Delphi study in Australia, hearing researchers (N = 15 in Round 1; N = 9 in Round 2), clinicians, and professionals working in early intervention for children with hearing loss (N = 85) were asked to review the potential items and to rate the importance of items using a Likert scale. RESULTS: Consensus was reached on 32 main items to be included in the MDS across six categories, including informational support (13 items), professional support (five items), peer support (one item), skills and knowledge (seven items), financial support (three items), and methods of information provision (three items). Eight optional items that could be considered for inclusion in the MDS were also identified. CONCLUSIONS: The proposed MDS could support hearing professionals in identifying families' needs in order to provide individualized information and support. Future research is needed to conduct a pilot study to evaluate the needs assessment tool in terms of usability, feasibility, and therapeutic effects.

Aetiology of permanent childhood hearing loss at a population level.

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

Detecting Hearing Loss in Infants With a Syndrome or Craniofacial Abnormalities Following the Newborn Hearing Screen.

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

Predicting hearing loss from 10 years of universal newborn hearing screening results and risk factors.

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

Integrating congenital cytomegalovirus screening within a newborn hearing screening program: Is it worthwhile?

OBJECTIVES: The aim of the present study was to review the potential impacts and barriers to upscaling a pilot congenital Cytomegalovirus (cCMV) screening program into a state-wide permanent universal newborn hearing screening (UNHS) program. DESIGN: This study reviewed the outcomes of the cCMV screening program pilot operating at three maternity hospitals to standard state-wide laboratory notifications in Queensland, Australia between August 2014 to April 2018. Stakeholder interviews were also conducted to inform state-wide program implementation. RESULTS: Of the 485 infants tested for CMV on a saliva swab at the pilot sites, 4 (0.8%) returned a positive result. Review of the state-wide laboratory infant CMV PCR notifications for the same time-period revealed more than half of infants with cCMV (63.7%) would not have been detected under a state-wide targeted screening program as they either passed newborn hearing screening, were deceased, symptomatic, or were born <34 weeks gestational age. Barriers to state-wide program implementation included program-level factors (timing of the cCMV screen, funding, cross-agency communication, workforce and training) and community-level factors (low public cCMV awareness and prevalence). CONCLUSIONS: Although cCMV screening alongside UNHS is achievable, a number of barriers need to be addressed prior to state-wide program implementation.

Family-Centered Care in the Transition to Early Hearing Intervention.

This study aimed to explore and compare families' and professionals' perspectives on the implementation of family-centered care (FCC) (Moeller, Carr, Seaver, Stredler-Brown, & Holzinger, 2013) during the period between diagnosis of hearing loss (HL) and enrollment in early intervention (EI). A convergent mixed-methods study incorporating self-report questionnaires and semistructured in-depth interviews was used. Seventeen family members of children with HL and the 11 professionals who support these families participated in this study. The results suggested that the services engaged during the transition period partially adhered to the principles of FCC, including the provision of timely access to EI services and provision of emotional and social support. However, areas for improvement identified include strengthening family/professional partnerships, shared decision-making processes, collaborative teamwork, program monitoring, and consistency in the provision of information and support. Qualitative and quantitative research findings also indicated a lack of consistency in service provision during the transition period.

Exploring factors influencing the use of an eHealth intervention for families of children with hearing loss: An application of the COM-B model.

BACKGROUND: Prior to developing a successful eHealth intervention, it is important that we explore stakeholders' capacity to adapt to eHealth. OBJECTIVE: To explore what factors influence the use eHealth services from the perspectives of families of children with hearing loss and professionals who support families as they transition into early intervention. METHODS: A qualitative study incorporating semi-structured in-depth interviews was conducted with families (n = 17) and professionals (n = 11). Interview topic guides were developed based on the COM-B model of behaviour change to explore barriers and facilitators related to capability, opportunity, and motivation. RESULTS: The COM-B model captured several factors that may influence the use eHealth interventions for families of children with hearing loss. The capability factors included computer literacy and familiarity with social media. The opportunity factors were access to online resources, reliable Internet, and affordable equipment. Professionals' and families' preferences and a culture of face-to-face services were also identified as barriers for using eHealth. The motivation factors included families' and professionals' confidence in using technology and beliefs that there were benefits (e.g., saving travel) associated with using eHealth services. In contrast, beliefs that eHealth may be difficult to set up and not able to replace in-person communication identified as barriers to families and professionals adopting eHealth interventions. CONCLUSION: Findings of this study indicated that implementation of an eHealth intervention could be facilitated by addressing the barriers in stakeholders' capabilities, opportunities (e.g., equipment and social support), and motivation (e.g., negative beliefs about eHealth) before developing eHealth services.

A qualitative investigation of families' needs in the transition to early intervention after diagnosis of hearing loss.

BACKGROUND: Families of children with hearing loss must make a number of decisions during the transition from diagnosis of hearing loss to enrolment in early intervention and thus require a wealth of information and support. This study aimed to investigate families' needs during this period and explore how these needs might differ for families of children with hearing loss who have additional disabilities. METHODS: An exploratory qualitative study incorporating semistructured in-depth interviews and thematic analysis was used. A total of 28 participants from two groups were involved: (a) family members of children with hearing loss (n = 17) and (b) professionals who support these families during the transition period from diagnosis of hearing loss to enrolment in early intervention (n = 11). RESULTS: Analysis of qualitative data revealed four major themes: (a) families require information that meet their specific needs; (b) families require supportive professionals to "walk the journey" with them; (c) some families want to connect with other families who "are in the same boat"; and (d) professional support needs differ for children with hearing loss who have additional disabilities. CONCLUSIONS: Families and professionals in this study identified a wide range of family needs during the transition to early intervention. The results highlighted the importance of providing individualized services and considering families' needs when providing family-centred services.

Integration of congenital cytomegalovirus screening within a newborn hearing screening programme.

AIM: Targeted screening by a salivary cytomegalovirus (CMV) polymerase chain reaction (PCR) of infants who 'refer' on their newborn hearing screen has been suggested as an easy, reliable and cost-effective approach to identify and treat babies with congenital CMV (cCMV) to improve hearing outcomes. This study aimed to investigate the feasibility and cost-effectiveness of introducing targeted salivary cCMV testing into a newborn hearing screening programme. METHODS: The study included three tertiary maternity hospitals in Queensland, Australia between August 2014 and April 2016. Infants who 'referred' on the newborn hearing screen were offered a salivary swab for CMV PCR at the point of referral to audiology. Swabs were routinely processed and tested for CMV DNA by real-time quantitative PCR. Parents of babies with a positive CMV PCR were notified, and the babies were medically assessed and, where appropriate, were offered treatment (oral valganciclovir). RESULTS: Of eligible infants, the parents of 83.0% (234/283) consented to the cCMV screen. Of these, 96.6% returned a negative result (226/234), and 3.4% (8/234) returned a positive result (three true positive; five false positive). The prevalence of cCMV for infants with confirmed hearing loss was 3.64% (P = 2/55; confidence interval = 0.44-12.53%). The cost comparison suggests the cost implementation of cCMV screening (and subsequent potential treatment benefits and management over time), compared to non-screening (and subsequent management), to be negligible. CONCLUSION: Incorporating cCMV testing into Universal Newborn Hearing Screening within Queensland is realistic and achievable, both practically and financially.