Detecting Hearing Loss Through Targeted Surveillance: Risk Registry and Surveillance Timeframe Recommendations.

PURPOSE: The purpose of this study was to inform the revision of a targeted surveillance risk registry by identifying which risk factors predict postnatally identified hearing loss (PNIHL) in children who pass newborn hearing screening and to determine whether hearing surveillance beyond the age of 1 year is warranted. METHOD: We used retrospective analysis of the audiological outcomes of children born in the state of Queensland, Australia, between January 1, 2010, and December 31, 2019, who passed the newborn hearing screen with risk factors. RESULTS: Approximately one third of children were lost to follow-up and could not be included in the analysis. Risk factors that predicted PNIHL in the analyzed cohort were as follows: syndromes associated with hearing loss, craniofacial anomalies, perinatal infections, and family history of permanent childhood hearing loss. Severe asphyxia did not predict PNIHL but yielded some cases of significant bilateral hearing loss. Hearing loss in children with a history of prolonged ventilation was mild and/or unilateral in nature (except in cases where the hearing loss was due to an unrelated etiology). There were no cases of PNIHL in children with hyperbilirubinemia or neonatal bacterial meningitis. For the risk factors that predicted PNIHL, nearly all hearing losses were detected by 1 year of age, except for children with family history where one quarter of hearing losses had a later onset. CONCLUSIONS: The four risk factors recommended for efficient postnatal identification of hearing loss are as follows: syndromes associated with hearing loss, craniofacial anomalies, perinatal infection, and family history of permanent childhood hearing loss. Hearing surveillance through to 1 year old is sufficient except for children with a family history, where a second phase assessment is indicated. Alternative targeted surveillance protocols and models of care are required to minimize loss to follow-up.

Exploring the Relationship between Persons with Hearing Loss/Deafness and Their Hearing Dogs.

The reported positive outcomes of animal-assisted services have led to an emerging interest in many different aspects of human-animal interactions. The influence of an assistance animal is thought to encompass several psychosocial domains in the life of a person with a significant health impairment. However, little is known about the mechanisms underlying the relationship between Hearing Dogs and their owners. A prospective study design using a written questionnaire method was utilized to survey 58 current and 23 prospective Australian Lions Hearing Dogs owners. The Pet Expectations Inventory (PEI) was used to investigate the anticipated role of Hearing Dogs in waitlisted persons with hearing loss/Deafness, whereas the Lexington Attachment to Pets Scale (LAPS) was completed by current owners to assess emotional attachment. The results revealed a high mean PEI score (M = 73.1, SD = 10.9, Mdn = 73.0, range: 55-91), with prospective owners strongly expecting the role of Hearing Dogs to include companionship/love and security. Furthermore, strong attachment features were evident in the owners' relationships with Hearing Dogs, as demonstrated by a high total LAPS score (M = 81.2, SD = 7.5, range: 63-91). Mean scores for statements within the "people substitution" category were highest (range = 3.6/4.00-3.9/4.00). In this demographically homogenous study cohort, it appeared that the high expectations of potential Hearing Dog owners for their animals to serve supportive roles beyond hearing assistance should be achievable, as evidenced by the strong attachment relationships displayed between Hearing Dogs and their owners.

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.

Understanding the experience of assistance dog providers supporting people with disability: an exploratory study.

PURPOSE: Assistance dogs can provide many health and wellbeing benefits to people with disability, however challenges remain. Assistance dog providers provide crucial support to assistance dog handlers, however limited literature exists exploring their experience working with people with disability. This study aimed to understand the experiences of assistance dog providers working in Australia. MATERIALS AND METHODS: Twelve assistance dog providers participated in semi-structured interviews. Inductive thematic analysis was used to analyse the transcripts. RESULTS: Five themes emerged describing the participant experience. Theme one discussed instances of discrimination experienced by assistance dog handlers, stemming from the lack of public awareness. Theme two captured the inconsistencies regarding the training of assistance dog providers with a specific focus on the gaps in the disability specific knowledge. Theme three emphasised the lack of clarity regarding legislation outlining the training requirements of assistance dog providers and the public access rights of the handlers. Theme four emphasised funding barriers, and theme five discussed barriers to interprofessional collaboration between assistance dog providers and allied health professionals. CONCLUSION: These findings contribute to understanding the current barriers experienced by assistance dog providers and assistance dog handlers, as well as the legislative changes required to better support people with disability.

Increasing evidence suggests that assistance dogs may provide benefits for people with disability, however challenges remain.There is a need for national and international legislation outlining the definition of an assistance dog, the training requirements of assistance dog providers, as well as the public access rights of assistance dog handlers.Assistance dog providers require structured education opportunities supporting their knowledge of disability to support assistance dog handlers most effectively.Interprofessional practice between assistance dog providers and allied health professionals has the potential to result in greater client-centred care.

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.

Canine-Assisted Occupational Therapy for Children within a Student-Led University Clinic: The Influence on Child Engagement from the Perspectives of Student and Parent Participants.

AIMS: Animal-assisted therapy has gained increased attention in occupational therapy. Yet there remains minimal education opportunities available. In addition to supporting student learning, student-led clinics have the potential to provide a valuable, cost-effective health service to the community. This study aimed to explore the potential benefits to the children clients participating in a student-led, canine-assisted occupational therapy pediatric clinic from the perspectives of the students and parents. METHOD: Semi-structured interviews were conducted with nine occupational therapy students, and five parents to describe their experience. Transcripts were analyzed using inductive thematic analysis. RESULTS: Participants emphasized how the presence of the therapy dog supported child engagement within the therapy session. Ways in which participants believed the therapy dog influenced the children's engagement were described within three themes. The first highlighted the importance of the relationship between the children and the therapy dog. The second described how this relationship facilitated a sense of safety within the sessions. The third captured how this sense of safety supported the children's participation within their meaningful occupations. CONCLUSIONS: Parents and students involved in the canine-assisted occupational therapy clinic perceived that canine-assisted therapy was supportive of child engagement in therapy.

Awareness and Perceptions of University Nursing Students in Samoa Towards Childhood Hearing Impairment and Hearing Healthcare: A Cross-Sectional Study.

Introduction: Pacific Islands have among the highest prevalence of pediatric hearing impairment in the world. Given the limited availability of ear and hearing specialists in the Pacific region, the broader health professional community plays a vital role in the prevention of avoidable pediatric hearing impairment. Nurses emerge as one of the ideal candidates for task-sharing in the administration of ear and hearing care at the public and primary healthcare levels. Objectives: The aim of this study is to evaluate the awareness and perceptions of university nursing students in Samoa to pediatric hearing impairment and hearing healthcare. Methods: This study employed a cross-sectional study design. A 10-item questionnaire was administered to university nursing students in Samoa (N = 100). The statements aimed to evaluate perceptions towards childhood hearing impairment, as well as awareness of causal factors of permanent childhood hearing loss and available hearing healthcare. Participants were required to provide a response based on the five-point Likert scale "Strongly Agree," "Agree," "Neutral/Don't know," "Disagree," and "Strongly Disagree." Results: Overall, 79% of participants responded "Disagree/Strongly Disagree" to the item "Hearing loss is not that important because it does not kill." Overall, 52% of respondents were aware of hearing impairment as a public health concern in Samoa. Early identification and intervention services for newborns with hearing impairment are nonexistent in Samoa, and awareness was low for the importance of early detection (62%) and amplification suitability for babies (49%). Overall, awareness was also low for causal factors of permanent sensorineural hearing loss. Conclusions: A borderline neutral/positive perception was found among our future nursing professionals to engage with pediatric hearing impairment in Samoa. Awareness regarding the causes of permanent childhood hearing impairment, as well as early identification and intervention services, could be integrated in the university curriculum.

Knowledge and attitudes of school principals towards childhood hearing loss and hearing services: A cross-sectional survey to support the implementation of inclusive education in Samoa.

Objectives: To assess the knowledge and attitudes among school principals towards childhood hearing loss and hearing services to support the implementation of inclusive education in Samoa. Educators are uniquely positioned to advocate for inclusive education in their classrooms, and may be among the first professionals to suspect hearing loss in their young students. Given the well-known impacts of childhood hearing loss on learning and development, educators therefore play a vital role in referring students for an ear and hearing health assessment. Methods: A cross-sectional convenience sampling design and bilingual questionnaire were used (English/Samoan). School principals attending the annual conference for educators of Samoa over 4 days in June 2022 were invited to anonymously complete a 23-item questionnaire on childhood hearing loss and hearing services. Participants were required to respond to each statement with either 'yes', 'no' or 'unsure'. The questionnaire investigated general knowledge of healthy hearing and hearing loss, knowledge of causes and risk factors of hearing loss, knowledge of identification and intervention for hearing loss and the attitudes towards students with hearing loss. Results: 95.4% of the 109 participants agreed that healthy hearing is important, and 97.2% felt that childhood hearing loss is an important problem in Samoa. Participants agreed that hearing loss adversely impacts the interaction with peers (95.4%), listening in the classroom (94.5%), speech and language development (93.6%), classroom behaviour (88.1%) and reading skills (85.3%). 97.2% of participants felt that students with hearing loss should have the same opportunities as their normal-hearing counterparts, and 92.7% would include students with hearing loss in their classroom. 94.5% were keen to learn more about childhood hearing loss. There were no significant differences in response proportions between variable subgroups. Conclusion: Overall, the results displayed high levels of knowledge and very positive attitudes among school principals in Samoa towards their students with hearing loss.

Investigating Dog Welfare When Interacting with Autistic Children within Canine-Assisted Occupational Therapy Sessions: A Single Case Study.

Human-animal bond is defined as the mutually beneficial relationship between humans and animals. Recent years have seen increasing research regarding the benefits of interaction with animals for autistic children. However, there continue to be limited studies exploring the impact of this interaction on the welfare of therapy dogs. As part of a pilot randomised control trial assessing the efficacy of canine-assisted occupational therapy with autistic children, this project assessed welfare markers of the therapy dog involved. A total of twenty-one saliva samples were taken from the therapy dog to assess cortisol, alpha amylase, and oxytocin concentrations at home and throughout the treatment days. Additionally, six hours of therapy session videos were analysed for stress indicators of canine behaviour. No significant differences were found between days spent at home and treatment days for any of the biomarkers or stress indicators. Results suggest that the therapy dog involved did not experience increased stress resulting from interaction with the autistic children throughout the therapy sessions. This study supports the need for further research regarding therapy dog welfare when interacting with autistic children including an increased sample size of therapy dogs and therapists.

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.

Knowledge and attitudes of female caregivers in Samoa to childhood hearing loss and hearing services.

OBJECTIVES: To assess the knowledge and attitudes of 150 female caregivers in Samoa to childhood hearing loss and hearing services, and to compare findings between urban (n = 100) and rural-dwelling (n = 50) caregivers. METHODS: A semi-structured interview using a 26-item questionnaire was administered to participants in the Samoan language. Participants were required to respond "yes", "no", or "unsure". RESULTS: Highest awareness of aetiology of childhood hearing loss was found for otitis media (88.7%), followed by noise exposure (64.7%) and family history (38%). Highest awareness of public health measures that may prevent/reduce otitis media was found for routine childhood immunizations (74.7%) and breast-feeding for first 6 months of life (69.3%). Overall, 40% of Samoan caregivers agreed that curses may cause childhood hearing loss. There was overwhelming support for community-based hearing services for newborns/infants (98%) and school students (97.3%). CONCLUSIONS: There is positive support for community-based hearing health services for children among female caregivers of Samoa. There was high awareness of otitis media as a major cause of childhood hearing loss, as well as good knowledge of public health measures that reduce/minimise the risk of otitis media.

Stress among Parents of Children with Hearing Loss and How They Deal with It: A Systematic Review

Abstract Introduction Parenting a child with hearing loss may be accompanied by high stress levels and several challenges. Objectives The present systematic review was performed to compile the existing literature pertaining to parental stress in parents of children with hearing loss and coping strategies. Data synthesis The databases searched include PubMed/Medline, Scopus, Cochrane Library and Cumulative Index to Nursing, and Allied Health Literature. The systematic review was designed and performed using the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement guidelines. The screening for studies was performed independently by two researchers. Methodological quality appraisal was done using the Mixed methods appraisal tool. Twenty-eight studies were identified and included in the present review, which discussed the stress factors and coping mechanism associated with having child with hearing loss reported by parents. The factors leading to stress were thematically classified into parent/family-related factors, child-related factors, and professionals/service-related factors. The coping strategies were thematically classified into improved support systems and better professional resources. Conclusion The present systematic review was an attempt at exploring the various stress causing factors among parents of children with hearing loss. The various coping strategies would help in better planning services for these parents and helping to lessen the stress.

Stress among Parents of Children with Hearing Loss and How They Deal with It: A Systematic Review.

Introduction Parenting a child with hearing loss may be accompanied by high stress levels and several challenges. Objectives The present systematic review was performed to compile the existing literature pertaining to parental stress in parents of children with hearing loss and coping strategies. Data synthesis The databases searched include PubMed/Medline, Scopus, Cochrane Library and Cumulative Index to Nursing, and Allied Health Literature. The systematic review was designed and performed using the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement guidelines. The screening for studies was performed independently by two researchers. Methodological quality appraisal was done using the Mixed methods appraisal tool. Twenty-eight studies were identified and included in the present review, which discussed the stress factors and coping mechanism associated with having child with hearing loss reported by parents. The factors leading to stress were thematically classified into parent/family-related factors, child-related factors, and professionals/service-related factors. The coping strategies were thematically classified into improved support systems and better professional resources. Conclusion The present systematic review was an attempt at exploring the various stress causing factors among parents of children with hearing loss. The various coping strategies would help in better planning services for these parents and helping to lessen the stress.

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.

Measuring resonance frequency of the middle ear in school-aged children: potential applications for detecting middle ear dysfunction.

OBJECTIVE: This study established a normative range of resonance frequency (RF) values as estimated using Wideband Tympanometry (WBT) and determined the ability of WBT-estimated RF to predict the presence of middle ear dysfunction in school-aged children. DESIGN: Cross-sectional data were collected using a hearing screening test battery consisting of WBT, Pure Tone Screening (PTS), 226- kHz tympanometry, and ipsilateral Acoustic Stapedial Reflexes (ASR). STUDY SAMPLE: About 1590 children aged 4-13 years. RESULTS: RF significantly decreased from 4 to 13 years of age (4-6 years, 928.95 kHz; 7-9 years, 872.80 kHz; 10-13 years, 863.68 kHz). RF had area under the receiver operating characteristic curve (AROC) values between 0.589 and 0.626 to predict ears that failed PTS or 226- kHz tympanometry. RF below 627 kHz accurately predicted the presence of a Type B tympanogram (AROC 0.945). RF had high test-retest reliability with Intra-Class Coefficient value of 0.817 and good agreement according to Bland-Altman plot analysis. CONCLUSIONS: WBT-estimated RF had fair diagnostic accuracy for predicting PTS and tympanometry results, but had excellent accuracy for predicting the presence of middle ear dysfunction, indicated by a Type B tympanogram. WBT-estimated RF does not require age-, gender-, ear- or ethnicity-specific normative data for clinical use with children.

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.

Issues Regarding the Welfare of Assistance Dogs.

While the roles and efficacy of assistance animals have received attention in the literature, there has been less research focused on animal welfare issues regarding assistance animals. This is a pertinent area, given the burgeoning of types of assistance animals, situations in which they are employed, and access issues arising from increased assistance animal engagement. Animal welfare as pertains to assistance animals is discussed in this paper with respect to overall research on animal welfare concerns in a variety of contexts, training and access issues, and legal and regulatory concerns. Relevant examples from global contexts, as well as the specific Australian context, are offered. Conclusions include that while human quality of life is often considered and protected in laws and policies, this is much less true for assistance animals. Additional attention is required to ensure that the quality of life of both persons with disabilities and their assistance animals can be ensured. Support for a person to meet the needs of an assistance animal, as well as considerations for keeping such working dyads together in changed circumstances (e.g., following a move to an assisted living mode of accommodation), are recommended.

Knowledge and Attitudes of Pacific Islander Doctors and Medical Students to Childhood Hearing Loss and Hearing Services: Results of a Structured Questionnaire Survey in SAMOA.

The Pacific Islands have among the highest global rates of childhood hearing loss in the world. Given the scarcity of ENT Specialists and audiologists in the region, the role of the wider health professional community in addressing preventable childhood hearing loss is crucial. A 10-item 5-point Likert scale questionnaire was administered to medical officers and medical students in Samoa. The statements aimed to investigate knowledge and attitudes to childhood hearing loss and hearing services. Overall, 95.8% of participants selected "Disagree/Strongly Disagree" in response to the statement "Hearing loss is not that important because it does not kill." There was good knowledge regarding early detection and intervention services for infant hearing assessments (73.6%) and amplification suitability for babies (69.4%-72.2%). Highest knowledge of causes of childhood sensorineural hearing loss was measured for neonatal meningitis/seizures (55.6%), followed by intra-uterine infections (52.8%), and aminoglycosides (52.8%). Knowledge was lowest for jaundice (33.3%) and birth asphyxia (38.9%).

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.