Permanent Childhood Hearing Impairment Research Articles

Prevalence of moderate or greater permanent childhood hearing impairment and effectiveness of targeted surveillance for babies who pass newborn hearing screening

Objective To assess the utility of targeted surveillance for the identification of moderate to profound PCHI in babies who pass newborn hearing screening in England and have risk factors. Design Retrospective analysis. Study sample A total of 3,957,891 children born 01/04/2012–31/03/2018 in England. Results A total of 7148 PCHI cases were identified (1.81 per 1,000 babies). 6,707 followed an immediate referral from the screen (1 per 16 referrals), 51 followed targeted surveillance referral (1 per 540 referrals) and 390 without a referral. Audiology uptake was higher following an immediate referral (96.7% overall, 77.2% within NHSP-defined timescales) than following targeted surveillance (63.8% overall, 51.1% within 52 weeks of birth). The screening was 94.5% sensitive overall, with similar sensitivities for each of the risk factors. General linearised logistic regression models identified syndrome as the risk factor with the highest odds ratio (14.08 for all babies, 22.19 for babies without immediate referral). Close family history of hearing loss was the next highest (10.93 for all babies, 12.29 for babies without immediate referral). Conclusion The evidence for a targeted surveillance programme, based on risk factors, for babies in England who pass the newborn screen is not strong.

Parenting Stress in Mothers of Children with Permanent Hearing Impairment

Permanent childhood hearing impairment (PCHI) represents the most frequent sensory pathology at birth. PCHI has a relevant psychological impact on the life of both the affected children and their families. Thus, the aim of this work is to explore the degree of parental distress felt by mothers of a deaf or hard-of-hearing child, to determine if this stress is associated with variables related to the children's health (e.g., the severity of hearing loss, presence of other conditions, difficulty with treatment options, difficulty with rehabilitation) or family characteristics such as socio-economic and educational status. The study used the Parenting Stress Index-Short Form (PSI-SF) questionnaire administered to mothers. The results were analyzed in relation to variables such as parents' education level, number of children, severity of hearing loss, presence of other chronic conditions, presence of cognitive delay, familiarity with hearing loss, time of diagnosis, use of prosthetics, and start in a rehabilitation program. The data indicate a correlation between maternal stress levels and low-educational levels, as well as the presence of congenital infections and cognitive delay. These results highlight the need for a comprehensive physical and psychological approach for hearing-impaired children, as stress factors can affect the adherence to effective rehabilitation.

Newborn hearing screening in the UK

The newborn hearing screening programme (NHSP) exists to identify permanent childhood hearing impairment (PCHI) in babies born in the UK. Early identification of hearing loss facilitates the provision of hearing aids or a prompt referral for cochlear implantation to ensure children optimise speech, language and communication skills, with the hope that this translates to the best possible educational, mental health, social and quality of life outcomes. Diagnosis of hearing impairment allows aetiological investigations to be offered to look for a potential cause of the PCHI, and given the recent advances in genomic testing, this can facilitate testing for additional family members and future children if a genetic cause is identified. In this article, the authors describe the structure of the NHSP and illustrate referral pathways into the programme from primary care for the few babies that may have been missed or fallen outside the remit of the programme.

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

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. 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. 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." 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. 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.

Permanent childhood hearing impairment in infants admitted to the neonatal intensive care unit: nested case-control study.

The prevalence of permanent childhood hearing impairment (PCHI) in infants admitted to a neonatal intensive care unit (NICU) is higher than that in the general population. Our study objective was to identify risk factors associated with PCHI in infants who required admission to the NICU for more than 48 h. We performed a case-control study, including infants of all gestational ages who were admitted to NICU for more than 48 h and who underwent newborn hearing screening between 2005 and 2019. Infants admitted to NICU and diagnosed with PCHI by formal audiology were classified as "cases". The "controls" were infants who were admitted to NICU and did not have PCHI. Cases and controls (1:4) were matched based on their birth gestation, birthing place, and treating NICU. The prevalence of PCHI in infants admitted to NICU was 6.3% as compared with our general population prevalence of 0.25%. There were 77 cases and 269 controls during the study period. The median age at diagnosis of PCHI in these infants was 132 days (interquartile range 75.5-518.5). Using regression analysis, "any ventilation episodes", presence of seizures, and major congenital anomalies were significantly associated with PCHI in infants of all gestational ages. There were higher prevalence of PCHI in preterms (<32 weeks) who received furosemide and lower prevalence with antenatal use of magnesium sulphate.Conclusions: In our study, the prevalence of hearing loss was high in infants admitted to NICU. Gestation-specific risk factors identified in this case-control study would help in counselling of parents. What is Known: •In the UK, 1-2/1000 infants are born with hearing loss and infants admitted to the neonatal unit for 48 h or more have increased prevalence of hearing loss(1 in 100 live births). •Identification of risk factors in infants admitted to neonatal unit would help with risk stratification and further management. What is New: •In our study, infants admitted to the neonatal unit had higher prevalence of hearing loss (6.3 in 100 live births). •In infants across all gestational age "any ventilation episodes", presence of seizures, and severe congenital anomalies were associated with a statistically significant increase in prevalence of hearing loss. Higher prevalence of hearing loss was noted in preterm infants (<32 weeks) who received furosemide treatment and lower prevalence was noted with antenatal use of magnesium sulphate.

Clinicians’ views of using cortical auditory evoked potentials (CAEP) in the permanent childhood hearing impairment patient pathway

Objective: To obtain clinicians’ views on the use of cortical auditory evoked potentials (CAEP) in the clinical pathway.Design: A questionnaire aimed at clinicians who use the HEARLab system with the Aided Cortical Assessment (ACA) Module. Results compared for Australians (where HEARLab produced) to other countries.Sample: The questionnaire was completed by 49 clinicians; 33 from Australia and 13 clinicians outside of Australia and 3 clinicians, destination unknown.Results: The findings of this research demonstrated that clinicians using CAEPs found them valuable for clinical practice. CAEPs were used to verify or modify hearing aid fittings and were used for counselling parents to reinforce the need for hearing aids. With the use of speech token as the stimulus clinicians had more relevant information to increase confidence in decision-making on paediatric hearing management.Conclusions: The main benefit from the use of CAEPs (using speech token stimuli) was for infant hearing aid fitting programmes, to facilitate earlier decisions relating to hearing aid fitting, for fine-tuning the aids and as an additional measure for cochlear implant referrals.

Prevalence of permanent childhood hearing impairment identified by universal newborn hearing screening: A systematic review and meta-analysis

Introduction Universal newborn hearing screening (UNHS) programmes have been implemented in many very highly-developed (UN Development Programme, 2015) countries over the last 20 years. The prevalence of permanent childhood hearing impairment (PCHI) detected by these programmes has not been systematically evaluated to assess the expected burden on audiological and other clinical services. In addition, the PCHI risk associated with Neonatal Intensive Care Unit (NICU) admission, a known PCHI risk factor, has not been estimated. We addressed these research gaps using systematic review and meta-analytical techniques to calculate the population-based prevalence of PCHI (defined as more than or equal to 26 decibels of hearing loss in both ears [≥ 26 dB HL]) identified through UNHS (defined as universal screening using otoacoustic emissions and/or auditory brainstem response testing within the first 6 months of life, followed by referral for diagnostic investigations). Our secondary objective was to compare PCHI prevalence in infants who were and were not admitted to NICU. Methods In accordance with the registered protocol (PROSPERO: CRD42016051267), six electronic databases (including Embase and Medline) were interrogated in January 2017 to identify eligible studies in very highly-developed countries to calculate prevalence of PCHI ascertained through UNHS. We identified further reports through searching citations of included papers and unpublished literature (November 2017). Eligible papers could be in any language and published on any date. Papers reporting outcomes for at-risk populations only, with no English abstract (unless unpublished) or of ineligible study type (e.g. case report or letter) were excluded. One reviewer extracted data and quality assessed all papers while two further reviewers undertook independent data extraction and quality assessment of a random sample of papers to ensure consistency; differences were resolved by consensus. Quality was assessed using criteria adapted from the Newcastle-Ottawa scale, STARD and QUADAS-2 tools. Pooled prevalence was calculated using random effects modelling, Freeman–Tukey double arcsine transformation of prevalences and Wilson (Score) method for calculating 95% confidence intervals (CIs) (Stata: Release 15; StataCorp LP). Results Literature searches identified 6195 non-duplicate references, from which 41 were eligible for inclusion (including 5 reports identified from the unpublished literature and citation searching). These reports contained data on 32 separate study populations (median size = 25,945 infants, interquartile range = 11,198, 83,691 infants). Studies took place between 1990 and 2014, with the majority in Europe (n = 23). Pooled prevalence of bilateral PCHI ≥ 26 dB HL identified through UNHS in the screened population was 1.08 (95% CI: 0.90–1.28) per 1000 infants. Infants admitted to NICU had 6.9 times (95% CI: 3.8–12.5) higher prevalence of PCHI than those not admitted, based on 3 studies reporting this information. Smaller studies were associated with significantly larger prevalences (Egger's test: P = 0.017). Key strengths of this review include use of a systematic search strategy and robust statistical methods. The findings are limited to very highly-developed countries. Conclusions In very highly-developed countries, around 1 in every 1000 infants will need referral to clinical services for investigation and management of PCHI. Infants admitted to NICU have a significantly increased prevalence of PCHI. Further analyses will evaluate screen test performance and other risk factors for PCHI. ESRC-funded PhD: ES/J500185/1.

Identification of risk factors for hearing impairment in newborns: a hospital based study.

Aim To identify risk factors for hearing impairment presented in neonates born in Cantonal Hospital Zenica (CHZ) and to estimate their influence on outcome of hearing tests in Newborn Hearing Screening (NHS). Methods Retrospective-prospective study was done at the Department of Gynaecology and Maternity. The NHS was performed with transitory evoked otoacoustic emissions (TEOAE) during a six-month period using "Titan" device (Interacoustics, Denmark). The questionnaire was written for the purpose of getting more structured basic information about every newborn and to identify risk factors for hearing impairment. Chi-square test was used to investigate the difference between experimental and control group refer incidence. Results A total of 1217 newborns was screened for hearing impairment of which 259 (21.28%) with one or more known risk factors for hearing impairment. The following risk factors for hearing impairment were identified during the study period: family history of permanent childhood hearing impairment in 42 (3.45%) newborns, prematurity in 39 (3.21%), low APGAR scores in 29 (2.40%), asphyxia in 31 (2.55%), hyperbilirubinemia in 41 (3.37%), admission of ototoxic medication (aminoglycosides) after birth in 155 (12.74%). Conclusion There were many serious risk factors for hearing loss identified in this study. Identification of risk factors for hearing impairment in neonates is necessary because a follow up of the children with risk factors is very important.

Role of Cortical Auditory Evoked Potentials in Reducing the Age at Hearing Aid Fitting in Children With Hearing Loss Identified by Newborn Hearing Screening.

Recording of free-field cortical auditory evoked potential (CAEP) responses to speech tokens was introduced into the audiology management for infants with a permanent childhood hearing impairment (PCHI) during 2011–2015 at a U.K. service. Children with bilateral PCHI were studied from two sequential cohorts. Thirty-four children had followed an audiology pathway prior to CAEP introduction, and 44 children followed a pathway after the introduction of CAEP and were tested with unaided and aided CAEP responses. Data analysis explored the age of diagnosis, hearing aid fitting, and referral for cochlear implant (CI) assessment for each of these groups. CAEP offered a novel educative process for the parents and audiologists supporting decision-making for hearing aid fitting and CI referral. Delays in hearing aid fitting and CI referral were categorized as being due to the audiologist’s recommendation or parental choice. Results showed that the median age of hearing aid fitting prior to CAEP introduction was 9.2 months. After the inclusion of CAEP recording in the infant pathways, it was 3.9 months. This reduction was attributable to earlier fitting of hearing aids for children with mild and moderate hearing losses, for which the median age fell from 19 to 5 months. Children with profound hearing loss were referred for CI assessment at a significantly earlier age following the introduction of CAEP. Although there has also been a national trend for earlier hearing aid fitting in children, the current study demonstrates that the inclusion of CAEP recording in the pathway facilitated earlier hearing aid fitting for milder impairments.

Implementation of Universal Newborn Hearing Screening (UNHS)

Permanent childhood hearing impairment (PCHI) is the commonest birth condition with a prevalence of 1-3/1000. This rises to 10/1000 for neonates who have been in Neonatal Intensive Care Unit. The early detection of PCHI, and the initiation of medical and educational interventions, has been demonstrated to significantly improve long-term outcomes for children with hearing loss and their families. Early identification, via universal new-born hearing screening gives babies a better ‘life chance' of developing speech and language skills and of making the most of social and emotional interaction from an early age. While the detection of early hearing is vitally important to a child’s development, it also means that support and information can be provided to parents at an early stage. Prior to the rollout of UNHS, the age of diagnosis of childhood hearing loss and habilitation in Ireland was difficult to ascertain with confidence due to the absence of National ICT system, but anecdotally was reported to be when children were aged over 2 years. The National Audiology Report1 recommended the introduction of UNHS, which commenced April 2011 and was rolled out on a phased basis, becoming a national programme in November 2013. Whilst this seems by inference to refer purely to hearing screening service for newborns; the programme actually spans a multitude of professionals (including ENT Consultants, Paediatricians, Audiologists, Visiting Teachers for the Deaf and Social Workers) across a range of services nationally (including Maternity Hospitals, Paediatric Hospitals, Cochlear Implant Team, Primary Care Services and Advocacy Services) who provide operational, clinical and managerial input to ensure the delivery of a high quality service. The implementation of UNHS required the development of new care pathways, up skilling of professionals, engagement with stakeholders, and procurement of a screening provider with a UNHS specific ICT system for the reporting of key performance indicators. To provide quality assurance and drive improvements local UNHS multidisciplinary governance groups were established to monitor services and provide input to the National Technical Group for Children’s Hearing Services (NTGCHS) who has overall responsibility for developing and managing the programme. Outcomes: To date the programme has been delivered nationally with over 250,000 children screened by 100 screeners based at 19 maternity sites. The programme outcomes will be reported, demonstrating that comprehensive national key performance indicators have been consistently met or exceeded, along with favourable benchmarking against international programmes. Next steps including ongoing team building and the development of a national audiology clinical management system to ensure continued integrated working will also be outlined. Key transferable learning points Necessity for quality ICT system for operational and service monitoring Establishing baseline KPIs and reviewing relevance Establishment of governance documentation Development of local and national groups to provide oversight Ensuring all stakeholders engage with governance groups Reference: 1. Health Services Executive. National Audiology Review. Dublin. 2011. Available from: http://www.hse.ie/eng/services/publications/corporate/AudiologyReview.pdf

Hearing impairment in children

Abstract Permanent childhood hearing impairment is best identified through newborn hearing screening programmes. Early identification and effective management of hearing loss optimizes outcomes. By making sound audible from the first months of life through hearing aids, it is possible to harness auditory plasticity and maximize lifetime auditory potential. For infants with profound hearing loss or auditory neuropathy the option of cochlear implantation may give improved hearing for speech, typically at around 12 months of age. The onus of responsibility for using hearing aids and providing a suitable sound environment for the child falls on the parents. This is best achieved using a family-centred model of audiology management rather than the traditional expert- or medical-models of intervention. For later-onset hearing loss, particularly conductive hearing loss secondary to otitis media or other middle-ear conditions, there is no longer a national screening programme in the UK with a greater reliance on surveillance. Many school-entry screens of hearing have been disbanded (with the joint understanding that community audiology services should be open to a wide range of referrals and easy to access) and there is risk of children with later-onset hearing loss being overlooked despite the recognised secondary effects of untreated hearing loss on speech development, social interaction and subsequent educational achievement. This article reviews the current knowledge base and discusses the appropriate methods of behavioural assessment techniques useful for healthcare professionals.

The impact of universal newborn hearing screening on long-term literacy outcomes: a prospective cohort study.

ObjectiveTo determine whether the benefits of universal newborn hearing screening (UNHS) seen at age 8 years persist through the second decade.DesignProspective cohort study of a population sample of children with permanent childhood hearing impairment (PCHI) followed up for 17 years since birth in periods with (or without) UNHS.SettingBirth cohort of 100 000 in southern England.Participants114 teenagers aged 13–19 years, 76 with PCHI and 38 with normal hearing. All had previously their reading assessed aged 6–10 years.InterventionsBirth in periods with and without UNHS; confirmation of PCHI before and after age 9 months.Main outcome measureReading comprehension ability. Regression modelling took account of severity of hearing loss, non-verbal ability, maternal education and main language.ResultsConfirmation of PCHI by age 9 months was associated with significantly higher mean z-scores for reading comprehension (adjusted mean difference 1.17, 95% CI 0.36 to 1.97) although birth during periods with UNHS was not (adjusted mean difference 0.15, 95% CI −0.75 to 1.06). The gap between the reading comprehension z-scores of teenagers with early compared with late confirmed PCHI had widened at an adjusted mean rate of 0.06 per year (95% CI −0.02 to 0.13) during the 9.2-year mean interval since the previous assessment.ConclusionsThe benefit to reading comprehension of confirmation of PCHI by age 9 months increases during the teenage years. This strengthens the case for UNHS programmes that lead to early confirmation of permanent hearing loss.Trial registration numberISRCTN03307358.

Kindliche Hör- und Sprachentwicklungsstörungen, Diagnostik und Genetik

This article gives an overview on important aspects of hearing and language development of infants and toddlers. Newborn hearing screening is implemented in Germany for all infants since January 2009. The realization of early postnatal hearing screening is regulated by a publication of the "Gemeinsamer Bundesausschuss". It regulates the measurement method (TEOAE or AABR screening) and differs between healthy and sick newborns. It also rules the timetable of rescreening and if it still fails of paedaudiological follow up. The second part refers to objective and subjective assessment of hearing loss and different important issues of permanent childhood hearing impairment are discussed. Especially CMV infection by materno-fetal transmission and hereditary hearing loss is addressed in comparison to otitis media with effusion. Speech and language development in the first three years of live and the corresponding nomenclature is introduced in the last part. Different parent questionnaires are well established in evaluation of early language acquisition. An early diagnosis of language delay is possible and therapeutic measures can be established to prevent further social and psychological problems.

Neonatal hearing screening of high-risk infants using automated auditory brainstem response: a retrospective analysis of referral rates

The past decade has seen the widespread introduction of universal neonatal hearing screening (UNHS) programmes worldwide. Regrettably, such a programme is only now in the process of nationwide implementation in the Republic of Ireland and has been largely restricted to one screening modality for initial testing; namely transient evoked otoacoustic emissions (TEOAE). The aim of this study is to analyse the effects of employing a different screening protocol which utilises an alternative initial test, automated auditory brainstem response (AABR), on referral rates to specialist audiology services. A retrospective analysis was performed of all neonatal hearing screening in Letterkenny General Hospital over 5 years, from January 2008 to December 2012, which was confined to infants possessing one or more known risk factors for permanent childhood hearing impairment. 1,163 infants were screened using AABR during this period and subsequent re-test and referral rates to specialist audiology services were examined. Of the infants screened, 50 (4.3%) failed the initial AABR. A further 16 of these (32%) failed the repeat AABR, resulting in an overall referral rate to specialist audiology services of 1.38%. Our results demonstrate strikingly low overall referral rates in this cohort, falling well below both the international benchmark and the initial results of the Irish UNHS programme. This provides a convincing argument for examining AABR as a potential alternative to TEOAE in the nationwide UNHS.

O74 – 2025 Benefits of universal newborn screening for permanent childhood hearing impairment to reading comprehension in adolescence: early confirmation of deafness matters

O74 – 2025 Benefits of universal newborn screening for permanent childhood hearing impairment to reading comprehension in adolescence: early confirmation of deafness matters

Predicting the Degree of Hearing Loss Using Click Auditory Brainstem Response in Babies Referred From Newborn Hearing Screening

The predictive ability of the auditory brainstem response (ABR) is an important factor governing the program sensitivity of neonatal hearing screens. The study examined the accuracy of the click-evoked auditory brainstem response (ck-ABR) when undertaken below the age of 6 months (from expected date of delivery) in predicting the pure-tone thresholds subsequently found to be present in children with a congenital permanent childhood hearing impairment. Children with permanent childhood hearing impairment were ascertained from neonatal screening programs that have been subject to longitudinal evaluation. Ninety-two children who had ck-ABR recorded when below 6 months of age and repeatable ear specific pure-tone audiometry were recruited. Those with recognized temporary middle ear effusions at either test were excluded. The relationship between ABR and pure-tone thresholds was tested using the Pearson correlation coefficient with a linear regression model used to estimate pure-tone threshold (dependent variable) from ABR (independent variable). Correlation coefficients were obtained for pure-tone frequencies at octave intervals between 0.25 kHz and 4 kHz and at various frequency combinations. The difference between ABR and pure-tone threshold was analyzed. Those with a difference of greater than 20 dB were further examined. The ABR and pure-tone differences were also compared in babies born at term and prematurely. Of the 92 children recruited to the study two children had a confirmed auditory neuropathy spectrum disorder (2%) and 10 (11%) had an audiometrically confirmed progressive hearing impairment. When these children were excluded, there was a high linear positive correlation (r = 0.90, SE = 14.3 dB) between the ABR and pure-tone thresholds averaged at 2 to 4 kHz. Although the correlation varied for different audiometric configurations, in all cases with a sloping hearing loss the correlation with their best frequency was weaker than the correlation at 2 to 4 kHz. For the total cohort the mean difference between ABR and pure-tone thresholds averaged at 2 to 4 kHz was 4.4 dB (SD = 19.29). The modal difference was 0 dB (58%) and 76 % had a difference of 20 dB or less. ABR underestimated the subsequently recorded pure-tone thresholds by more than 20 dB in 11 children and 10 of these children showed progression of their hearing loss measured by serial pure-tone audiometry. ABR overestimated the pure-tone thresholds by more than 20 dB in 15 children. Nine of these children (60%) had suffered a perinatal illness and the mean difference between the pure-tone and ABR thresholds was significantly greater in those born at <35 weeks of gestation (p < 0.001). There is a high positive linear correlation between ck-ABR and pure-tone average thresholds at 2 to 4 kHz. However, the predictive value of ABR is reduced in certain neonatal groups. The cause for this is discussed as are the implications for undertaking a test battery at this age to improve the predictive accuracy.

Effectiveness of targeted surveillance to identify moderate to profound permanent childhood hearing impairment in babies with risk factors who pass newborn screening

Objective: To examine the effectiveness of targeted surveillance for the identification of moderate–profound PCHI in babies who pass the newborn hearing screen in England and have risk factors. Design: Retrospective analysis. Study sample: 2 307 880 children born 01/04/06–30/09/09 in England. Results: Overall the prevalence for all PCHI in children with risk factors who pass newborn hearing screening is 1.49/1000. The risk factors with the highest prevalence are (1) Syndrome (other than Down's) associated with a hearing loss; (2) NICU with refer in both ears at OAE and pass in both ears at AABR; (3) Craniofacial anomaly; (4) Down's syndrome; (5) Congenital infection. Conclusion: Targeted surveillance for children who pass the screen and have the risk factors 1–5 listed above will be retained within the English NHSP; targeted surveillance for children who pass the screen and have other risk factors is not effective and has been discontinued.

Initial results from the newborn hearing screening programme in Ireland

Hearing screening programmes aim to detect hearing loss in the neonate. The Health Service Executive (HSE) South was the first phase of a national roll-out of a neonatal hearing screening programme in Ireland, going live on 28 April 2011. Over 11,738 babies have been screened for permanent childhood hearing impairment (PCHI) during the first 12 months. The percentage of eligible babies offered hearing screening was 99.2 %. Only 0.2 % (n = 25) of those offered screening declined. 493 (4 %) were referred for immediate diagnostic audiological assessment. The average time between screen and diagnostic audiology appointment was 2 weeks. 15 (1.3/1,000) babies have been identified with a PCHI over the 12-month period. 946 (4 %) babies screened were admitted to the neonatal intensive care unit (NICU) for >48 h. The prevalance of PCHI is 7.3/1,000 in the NICU population compared to 0.6/1000 in the well baby population. 214 (1.8 % of total babies screened) had a clear response in the screening programmes, but were deemed to be at risk of an acquired childhood hearing impairment. These babies will be reassessed with a diagnostic audiology appointment at 8-9 months of age. To date, there is one case of acquired hearing impairment through this targeted follow-up screen. Of the 15 cases of PCHI identified, 8 (53 %) of these had one or more risk factors for hearing loss and 7 (37 %) were admitted to the NICU for >48 h. Four babies were referred for assessment at the National Cochlear Implant Centre.

1237 Development and Quality of Life in NICU Graduates and Healthy Newborns with PCHI at 3–5 Years of Age After NHS

BackgroundNICU graduates have both an increased risk of permanent childhood hearing impairment (PCHI) and delayed psychomotor development. Little follow up is available after introduction of neonatal hearing screening (NHS).AimComparison of...

The p.V37I Exclusive Genotype Of GJB2: A Genetic Risk-Indicator of Postnatal Permanent Childhood Hearing Impairment

Postnatal permanent childhood hearing impairment (PCHI) is frequent (0.25%–0.99%) and difficult to detect in the early stage, which may impede the speech, language and cognitive development of affected children. Genetic tests of common variants associated with postnatal PCHI in newborns may provide an efficient way to identify those at risk. In this study, we detected a strong association of the p.V37I exclusive genotype of GJB2 with postnatal PCHI in Chinese Hans (P = 1.4×10−10; OR 62.92, 95% CI 21.27–186.12). This common genotype in Eastern Asians was present in a substantial percentage (20%) of postnatal PCHI subjects, and its prevalence was significantly increased in normal-hearing newborns who failed at least one newborn hearing screen. Our results indicated that the p.V37I exclusive genotype of GJB2 may cause subclinical hearing impairment at birth and increases risk for postnatal PCHI. Genetic testing of GJB2 in East Asian newborns will facilitate prompt detection and intervention of postnatal PCHI.