Universal Neonatal Hearing Screening Program Research Articles

A cross-sectional study of neonatal hearing impairment: understanding its prevalence and risk factors in a tertiary care hospital in Kerala, India

Background: Congenital hearing loss is one of the most prevalent sensory impairments with severe consequences when undetected. The objective of neonatal hearing screening is to identify hearing impairments shortly after birth to initiate treatment as soon as possible and to allow affected children to enjoy normal development. The aim of the study was to determine the prevalence and identify the predisposing factors of HI among neonates in a tertiary care centre in Idukki district of Kerala using a universal neonatal hearing screening (UNHS) program, in an attempt to initiate early interventions in affected neonates. Methods: This was a cross-sectional study conducted in the department of ENT, Al Azhar Medical College and Super Specialty Hospital, Thodupuzha, Kerala over a period of 1 year involving 646 neonates. The study excluded any newborn whose parent/guardian did not give consent for the study, or neonates with microtia grade II and above. Results: The overall prevalence of HL in neonates in our study was 3.1 in 1000 live births out of which 61% were males and 39% females. The risk factors found in our study were preterm babies, maternal age >35, low birth weight, teenage pregnancy, primigravida, and neonatal jaundice requiring blood transfusion. Conclusions: In a standard neonatal population, both at-risk and non-at-risk neonates warrant the immediate adoption of a universal hearing screening program for all neonates in India. Hence, we would like to stress the importance of identifying infants with HL, administering early treatment and performing appropriate screening tests in infants at the right time period.

Summary of the Polish Universal Neonatal Hearing Screening Program 2021year review.

The Polish Universal Neonatal Hearing Screening Program (PUNHSP) has been carried out in Poland for 20 years. Its main goal is to screen newborns for hearing. This examination is performed on the 2-3 days of a child's life, and in addition, information is collected on the risk factors for the development of hearing loss. In total, 486 centers participate in PPPBSuN at three reference levels. By the end of 2021, 7 million children were registered in the PPPBSuN Central Database. The most common type of hearing loss in children was bilateral sensorineural hearing loss, diagnosed in 2021 in 160 children. The report below presents the most important results and conclusions from the functioning of PPPBSuN in 2021.

A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss.

Congenital unilateral sensorineural hearing loss (uSNHL) is associated with speech-language delays and academic difficulties. Yet, controversy exists in the choice of diagnosis and intervention methods. A cross-sectional prospective design was used to study hearing loss cause in twenty infants with congenital uSNHL consecutively recruited from a universal neonatal hearing-screening program. All normal-hearing ears showed ≤20 dB nHL auditory brainstem response (ABR) thresholds (ABRthrs). The impaired ear median ABRthr was 55 dB nHL, where 40% had no recordable ABRthr. None of the subjects tested positive for congenital cytomegalovirus (CMV) infection. Fourteen subjects agreed to participate in magnetic resonance imaging (MRI). Malformations were common for all degrees of uSNHL and found in 64% of all scans. Half of the MRIs demonstrated cochlear nerve aplasia or severe hypoplasia and 29% showed inner ear malformations. Impaired ear and normal-hearing ear ABR input/output functions on a group level for subjects with ABRthrs < 90 dB nHL were parallel shifted. A significant difference in interaural acoustic reflex thresholds (ARTs) existed. In congenital uSNHL, MRI is powerful in finding a possible hearing loss cause, while congenital CMV infection may be relatively uncommon. ABRs and ARTs indicated an absence of loudness recruitment, with implications for further research on hearing devices.

Influence of screening devices and protocol on audiological outcome of 25 years of universal neonatal hearing and vestibular screening in Flanders

Background: Congenital hearing loss has been linked with life-long deficits in speech and language abilities and children with vestibular deficits show delayed development of gross motor milestones. Aim and scope of the research: To analyze if the universal neonatal hearing screening (UNHS) and vestibular screening program in Flanders meet the current guidelines and benchmarks of the position statements from the Joint Committee on Infant Hearing and how big the impact of screening devices and strategy is. Methods: The UNHS is organized since 1997 by a Flemish public child care organization and performed by trained nurses. It is based on a two-step screening with Automated Auditory Brainstem Responses and in case of absent responses (named ‘refer’), audiological and medical diagnostic are done in one of 21 certified centers. Vestibular screening started in 2018 around the age of 6 months with cervical Vestibular Evoked Myogenic Potentials (cVEMP) for all children with confirmed permanent hearing loss. Results: The coverage of the UNHS from 1998 till 2020 was 96,3 % (range 91,1 – 98%). There was a bilateral refer for 0,27 % of the infants and unilateral refer for 0,37 %. A sensorineural or mixed hearing loss &gt; 40 dB was diagnosed in 32 % of the referred infants, a conductive hearing loss which often resolved over time in 39 %, a normal hearing in 25% and 4,7 % were lost in follow-up for the diagnostic evaluation. There was an effect of the screening device on the detection of conductive hearing losses and false-positive cases. The incidence was higher with the MAICO MB Classic® device compared to the ALGO® devices. The incidences of sensorineural or mixed hearing loss were independent of the screening device and remained relatively stable over the years. A vestibular deficit was found in 9,5 % of all children with sensorineural hearing loss. The incidence was significantly higher in infants with severe or profound hearing loss (p=0,003) Conclusion: The Flemish UNHS has a high coverage, low referral rate and low loss of follow-up rate. Strict monitoring and follow-up by a central organization are essential for optimal results. Long-term follow-up remains a challenge.

Prevalence of hearing impairment and outcome of universal neonatal hearing screening program in a tertiary care hospital – in UAE

Background and objective: Hearing loss is one of the most common congenital anomalies, occurring in 1 to 3 per 1000 newborn infants in the well-baby nursery population, and 2 to 4 per 100 infants in the Neonatal intensive care unit graduates. The aim of the study is to identify babies with potential hearing loss and direct them to early intervention and rehabilitation. The program also initiated to create awareness about the need for detecting childhood deafness among parents and general population. An estimate of coverage, prevalence and proportion of babies defaulting newborn hearing screening program and diagnostic evaluation monitored closely to find the effectiveness of the program. Study design: This is a retrospective study and was conducted at Latifa women and children’s hospital (LWCH), a tertiary care referral hospital at Dubai, United Arab Emirates, over a period of two years from January 2018 to December 2019. Neonates admitted in Neonatal intensive care unit (NICU) and Post Natal wards (PNW) were screened. We followed three tier approach of hearing screening. All eligible neonates were subjected to Transient Evoked Oto-acoustic emission (TEOAE) prior to discharge. Automated auditory brainstem response (AABR) hearing screening was conducted in neonates who did not pass TEOAE test. Infant who failed second screening (AABR) were subjected to comprehensive hearing evaluation prior to three months of age. Results: Out of the total 7923 neonates, 7895(99.65%) were screened and 118 (1.49%) newborns referred for comprehensive audiological evaluation. Among them, 27(0.34%) were identified with various degrees of hearing loss. The prevalence of hearing loss was found to be 0.16% and 0.92% among neonates from PNW and NICU respectively. Conclusion: Implementation of Universal newborn hearing screening program is the need of the hour, as early detection of Hearing loss will aid early rehabilitation and better outcomes.

Outcomes of a universal neonatal hearing screening program of 9941 newborns over a one-year period in Campinas, Brazil

ObjectivesTo characterize the outcomes of a universal neonatal hearing screening program in Brazil. MethodsRetrospective documentary analysis of the hearing screening of the neonates born in the hospital from August 2019 to July 2020. The universal neonatal hearing screening program performed the examination of TEOAE in newborn without risk factors for hearing loss and TEOAE and aABR in neonates with risk factors for hearing loss. Results9941 neonatal records were studied, 9088 newborns (91,42%) with no risk factors for hearing loss and 849 newborns (8.54%) with risk factors for hearing loss. 4 newborns (0.04%) had hearing loss in the group without risk factors and 16 newborns (1.88%) in the group with risk factors. In retesting those newborns who failed the UNHS test stage, we had 4 newborns (0.04%) without risk factors and 2 newborns (0,24%) with risk factors who evaded and did not return to service. ConclusionIt was possible to survey the outcomes of one year of the UNHS service and observe that the coverage rate of the service was 99.96%. The use of the combined methodology guaranteed a number of forwardings for less returns. Through this study, it was possible to observe that this service complies with the quality indicators recommended by the guidelines.

Outcome of Universal Neonatal Hearing Screening Programme at a Tertiary Care Centre: A Prospective Study.

Hearing is an important sense organ for human beings essential for speech, language and overall development. Universal neonatal hearing screening programmes are mandatory in many developed countries. In India out of 1000 neonates approximately 5-6 infants are diagnosed with hearing impairment but still universal neonatal hearing screening programme is in its infancy. To screen all neonates delivered at our centre in three years, with secondary objective of determining the significance of risk factors with neonatal hearing loss and to provide appropriate intervention following the detection of a permanent hearing impairment. It is a non-randomised prospective cohort study conducted at a tertiary care centre to screen all neonates born between Jan 2017 and Dec 2019 with 2 stage protocol using distortion product otoacoustic emission and auditory brainstem response. The study population was also divided into two groups, ''at risk'' and ''no risk'' groups to determine the significance of risk factors on neonatal hearing loss. Total 2676 neonates were screened, OAE referral rate was 1.53%. There was a 94.12% follow up. The incidence of hearing impairment was found to be 7 per 1000(In the "no risk" group, 2.9 per 1000 and in the "at risk" group, 41.38 per 1000). Statistically, a significant difference was found in the incidence of hearing impairment between the two groups (p < 0.05, chi-square Test). Hospital based universal hearing screening of new born before discharge is feasible at a tertiary care centre. A high incidence of 0.7% congenital hearing impairment warrants universal newborn hearing screening programme. Considering the limitations and infrastructure in India, we should at least employ screening for all high risk neonates.

UNHS: A Decade Long Feasibility and Sustenance Study from a Tertiary Care Hospital in India.

Objective The aim of this study is to estimate the incidence of hearing loss in neonates at a tertiary referral center, to assess the associated risk factors in those identified with hearing loss and to explore the challenges of starting and continuing a universal neonatal hearing screening programme in a tertiary care hospital over a decade. Materials and Methods It is a cohort prospective observational study conducted from January 2008 to January 2018. Five thousand five hundred and forty neonates underwent screening for hearing loss, of which 2688 were well babies and 2854 babies had one or more risk factors causing hearing loss. Otoacoustic emissions (OAE) is employed as the first level of screening within one month of birth. Brainstem evoked response audiometry (BERA) is done at 3months of gestational age for all the well babies who were referred on OAE testing and all the high risk babies irrespective of the result of the OAE screening. The High risk criteria are based on the set criteria by the Joint Committee on Infant Hearing (2007) and American Academy of Pediatrics (1994). Results In our study the incidence of hearing impairment is 5.41 per 1000 neonates screened. In well babies and at risk babies it is 1.49 and 9.11 per 1000 respectively.The common neonatal risk factors associated with hearing loss in our study are babies admitted to neonatal intensive care units (NICU), intrauterine growth retardation (IUGR), birth weight less than 1500g, respiratory distress syndrome (RDS) and hyperbilirubinemia. Conclusion The incidence of hearing loss in our study is comparable to that reported in literature (Bachmann KR, ArvedsonJC (1998) Early identification and intervention for children who are hearing impaired. Pediatr Rev. 1998 May; Vol. 19. No.5. pp. 155-165. http://www.ncbi.nlm.nih.gov/pubmed/9584525 Accessed from May 1998, Nagapoornima P, Ramesh A, Srilakshmi, Rao S, Patricia PL, Gore M, et al. Universal hearing screening. Indian J Pediatr. 2007 Jun 18; 74(6):545-9., Augustine AM, Jana AK, Kuruvilla KA, Danda S, Lepcha A, Ebenezer J, et al. (2014) Neonatal hearing screening-experience from a tertiary care hospital in southern India. Indian Pediatr. Vol. 51. No.3. pp 179-183. http://www.ncbi.nlm.nih.gov/pubmed/24277966 Accessed from Mar 2014). Hearing loss is more common in those babies with risk factors as reported in literature (Nagapoornima P, Ramesh A, Srilakshmi, Rao S, Patricia PL, Gore M, et al. Universal hearing screening. Indian J Pediatr. 2007 Jun 18; 74(6):545-9. http://www.ncbi.nlm.nih.gov/pubmed/21654001 Paul AK (2011) Early identification of hearing loss and centralized newborn hearing screening facility-the Cochin experience. Indian Pediatr. Vol. 48. No. 5. pp 355-359. Accessed from May 2011). Implementation of neonatal hearing screening program at a tertiary care hospital using a two tier system with OAE and BERA is a feasible but challenging service.

Universal neonatal hearing screening program at a university hospital: an analysis using quality indicators

ABSTRACT Purpose: to assess the universal neonatal hearing screening program using quality indicators. Methods: the records of newborns who were submitted to the neonatal hearing screening in 2018 were analyzed, comparing the data with the first five quality indicators established by the national guideline for neonatal hearing screening attention: 1) rate of screening coverage (≥95%); 2) age at the screening in months (up to the first month of life); 3) rate of referrals for diagnosis (2% to 4%); 4) rate of attendance to diagnostic examination (≥90%); 5) age at confirmed diagnosis (up to the third month of life). The data were submitted to quantitative and descriptive statistical analysis. Results: the rate of coverage, age at screening, and the number of referrals for diagnosis met the indicators established by the national guideline. The rate of attendance to diagnostic examination fell short of the expected, and the age at confirmed diagnosis was verified in 70% of the cases. Conclusion: using the quality indicators furnished important data on the effectiveness of the neonatal hearing screening program and identified opportunities to improve the service, which can help identify hearing loss, early.

Hearing Loss in Low Birth Weight Neonates: A Comparative Study at Nepalgunj Medical College and Teaching Hospital

Introduction: Hearing impairment is the most common congenital abnormality that occurs in 1 to 4/1000 newborns. It has a profound effect on their optimal development of language, speech and cognitive skill. Early detection in order to achieve effective treatment is essential. An association between low birth weight and hearing loss is commonly associated with multiple risk factors that can alter hearing in a synergistic fashion. Universal neonatal hearing screening programs have become widely implemented aiming for the screening, confirmation of the diagnosis and intervention by 1, 3 and 6 months respectively. Transient Evoked Otoacoustic emissions is one of the test found to be a quick, objective, non-invasive, accurate and easy test for early detection of this problem. Aims: Early detection of hearing loss in neonates focusing on low birth weight for early optimum rehabilitation. Methods: A comparative case control study conducted in 100 neonates under 2 groups. 50 neonates with low birth weight and 50 with normal birth weight who were born at NGMCTH, Kohalpur. Their hearing evaluation was done with Transient Evoked Oto Acoustic Emission (TEOAE). Results: The total referral rate was 12 % and pass rate was 88 %. The referral rate in LBW group was 20 % and 4 % in normal weight neonates. The pass rate in low birth weight was 80 % and 96 % in normal weight babies. Conclusion: Hearing impairment is a severe consequence in neonates with low birth weight. To decrease the economic and social burden of effects of hearing loss, it is assumed that newborn screening can immeasurably improve the future of newborn with early rehabilitation.

Universal neonatal hearing screening in a public hospital in Brazil: parents participation

Abstract Background The National Policy for Hearing Health Care in Brazil proposes that all newborns in the country undergo hearing screening in the first month of life.The Universal Neonatal Hearing Screening Program being extremely important for the early intervention of deafness. However, many parents/guardians do not return to the auditory retest, interrupting the neonatal protocol and making early auditory diagnosis impossible. Objective:To analyze the reasons for the non-attendance to retest of the Hearing Screening of a hospital in Curitiba. Methods This study was carried out in a Public Hospital, with live births from August 2017-May 2018. All the born were considered for the variables gender, risk for hearing loss, age at birth, results of Hearing Screening. Failed babies were analyzed, including the active search of those who did not attend the retest, through contact with parents/guardians to collect the reasons for faults. Results 2,531 infants were born, male had a prevalence of 1,275 (50.38%), term born 1,734(68.51%) and ICU stay as the most common risk indicator - 118(79.73%). 2,261(89.33%) infant passed on both ear; among those who failed only 94(40.0%) attended the retest. An active search was performed by phone, and 78(55.31%) could not establish contact, totaling 47(54.02%) mothers contacting. These motherś age was 19 to 24 years, 56.92% of whom had high school. The majority of those in charge are aware of the result of the test of the ear of their son (82.97%), was directed to test again (87.23%), were not able to answer the reason for not attending (27.65%) or had forgotten about this commitment (19.14%). Conclusions The data analyzed showed the lack of information that parents / guardians of the newborns give to the Hearing Screening, which compromises the integrality of health actions in newborns, making it necessary to seek other ways to ensure family adherence to the Program. Key messages Babies at risk of deafness need constant monitoring. Without parental participation, there is no success in health programs.

Stability of ABR Wave V Threshold in Early Hearing Diagnostics in Children from Selected Groups at Risk of Congenital Hearing Loss.

&lt;b&gt;Introduction:&lt;/b&gt; Upon hearing that the "little" patient has trouble with hearing, we are mostly interested in the level of his hearing threshold. When the child is in the first year of life, results can only be achieved by means of ABR test. Subsequent control tests, especially in children from the hearing loss risk groups selected in this study, show that the obtained outcomes are subject to fluctuations. Their fluctuating nature is manifested by the instability of wave V threshold in subsequent diagnostic periods. Such variability often delays the implementation of the appropriate proceeding. Knowledge of the dissimilarity of behavior of the wave V threshold occurring in individual groups at risk of hearing loss allows for the correct interpretation of the obtained results, and thus, effective therapeutic measures. &lt;br&gt;&lt;b&gt;Aim:&lt;/b&gt; The aim of the paper is to analyze the stability of wave V threshold during the first year of life in children from selected risk groups for congenital hearing disorders. &lt;br&gt;&lt;b&gt;Material and methods:&lt;/b&gt; From the patient population of 2,114 individuals examined in 2015-2016 at a reference center participating in the Universal Neonatal Hearing Screening Program in 2015-2016, the results of 250 children were subjected to retrospective analysis. Furthermore, 4 groups of little patients were formed (children with Down syndrome; children with other diseases or damage to the nervous system; children with cleft palate or cleft lip and cleft palate; children with congenital cytomegaly) in whom diagnostic practice revealed variable results of the wave V threshold. We analyzed the results of tests obtained during the first year of the child's life divided into 4 diagnostic periods. &lt;br&gt;&lt;b&gt;Results:&lt;/b&gt; The highest percentage of instability in the established threshold of wave V between individual diagnostic periods occurred in the group of children with cleft palate or cleft lip and cleft palate. In the group of children with Down syndrome, it was observed that the instability of the ABR test results decreased over time. In the group of children with other diseases or damage of the nervous system, the highest percentage of the lack of stable ABR wave V thresholds was observed between the 1st and 2nd as well the 1st and 4th diagnostic periods. On the other hand, in the group of children with congenital CMV, there was a relatively low percentage of instability of results. &lt;br&gt;&lt;b&gt;Conclusions:&lt;/b&gt; (1) Although the ABR test is a diagnostic standard, in particular groups of patients the study is burdened with high variability of measurement results in subsequent diagnostic periods. Such a group of patients are children with cleft palate or cleft lip and cleft palate; therefore, it must receive particular attention in treatment planning; (2) in selected groups at risk of hearing loss, due to the high percentage of children with hearing impairment (70%), the validity of performing newborn hearing screening tests was confirmed.

Limitations and drawbacks of the hospital-based universal neonatal hearing screening program: First report from the Arabian Peninsula and insights

ObjectivesTo assess the efficacy of the current universal neonatal hearing screening program in a tertiary medical institution in Oman, identify its limitations and drawbacks, and explore their causative factors. MethodsA retrospective review was carried out to analyse the hearing screening of 12,743 live babies born between January 2016 and December 2018. Screen coverage, drop outs, follow up rate, and age at completion of screening, diagnosis, and intervention were analysed. The results were compared with the Joint Committee on Infant Hearing (JCIH) performance quality indices. Prospective questionnaire-based telephonic interviews were then conducted with the parents or caregivers of neonates with hearing loss. Finally, the causes of loss to follow up or delays in hearing screenings, diagnosis, and/or early intervention were studied. ResultsThe true prevalence of hearing loss was 4.0 in 1000. The coverage of first-stage screening was 90% whereas the compliance with the second stage was 88.04%. 22.8% of the patients eventually obtained final diagnostic confirmation. The overall compliance with amplification was 30.2%. The completion ages of primary screening and final confirmation were 7.98 and 17.3 weeks respectively. The importance of hearing screening is well received by parents, but problems related to communication, delays in the appointment system, and inefficient follow up tracking were identified as the main limitations and drawbacks of the program. ConclusionThe coverage of the neonatal hearing screening program had not yet reached the required goal of 95%. The performance indicators also fell below the international benchmark. There is a need to address the identified causative factors. Effective communication and well-maintained tracking systems need to be implemented.

Follow-up on the diagnostic level of children covered by the Universal Neonatal Hearing Screening Program in Poland, divided into voivodships.

Thanks to the Polish Universal Neonatal Hearing Screening Program (PUNHSP), all newborns in Poland undergo a free, screening hearing examination. Between 2006 and 2015, the average number of tested children per year was 373,477. According to the analysis of The Central Database (CDB), only 55.8% of the children attended the detailed hearing examinations at the second level of the Program. The aim of this study is to analyse the dates concerning the attendance of the children at the diagnostic level of PUNHSP in different regions of Poland. To conduct an analysis of this fact and find out the reasons for low attendance at the second level in 2015, a telephone survey questionnaire was developed for parents who had not registered their babies for further consultation - 3,239 randomly selected parents. The analysis revealed that the number of children examined at the second diagnostic level of the program is in fact much higher than the results of The Central Database show. The actual number is 83.6% as opposed to 55.8%. As a result of the telephone questionnaire some inaccuracies in the input data to the CDB were detected. The main errors in gathering the information for the CDB were incorrect OAE test result and no examination performed. C onclusion: In Poland the worst results (i.e. questionnaire results compared to CDB) for the attendance at the diagnostic level were shown in Pomorskie, Lubelskie, Mazowieckie and Podlaskie regions. In many cases there was a large discrepancy between the reality and the information in the CDB. The improvement of clarity concerning the CDB application is important in order to minimise the possibility of malformation in the CDB.

Non-compliance With Neonatal Hearing Screening Follow-up in Rural Western India

The reasons of failure to follow-up for the Universal Neonatal Hearing Screening (UNHS) program were delineated. Review of case records for data related to follow-up of neonates who underwent the UNHS between February 2012 - January 2015. 2534 neonates underwent primary screening with Distortion Product Oto-acoustic Emission (DPOAE). 14 (26.9%) were lost to follow-up between the first and second DPOAE screenings. 275 neonates (including high-risk cases) were to undergo confirmatory Brain Evoked Response Audiometry testing out of which 201 (73.4%) came for follow-up. Out of 74 who failed to follow-up (including those lost between first and second DOPAE screenings), unwillingness and non-compliance was the commonest reason. Increasing awareness and counseling of the caretaker are important interventions for ensuring good follow-up in hearing screening programs.

Analysis of the changes in the Polish Universal Neonatal Hearing Screening Program over 15 years of activity.

One of the examples of a nationwide pro-health program in Poland is the Polish Universal Neonatal Hearing Screening Program (PUNHSP). The Program is aimed at early diagnosis and intervention in children with hearing impairments and it is an example of a well-managed program. Presenting the results of the PUNHSP activity as well as organizational aspects and own experience can significantly help institutions managing other programs to achieve high efficiency in their functioning. The aim of this work is a detailed analysis of the Program evaluation during the 15 years of its activity, i.e. the identification of changes and the consequences of their introduction in the perspective of improving quality and efficiency. The material is PUNHSP data, registered in the Central Database of the Program and organizational information related to management, IT support and PUNHSP infrastructure. The analysis was based on quality assessment parameters (identification of changes and the purpose of their introduction) and effectiveness (consequences of introducing changes). The analysis concerns the whole period of PUNHSP activity - from 2002 to 2017. As a result of the analysis, 13 main modifications of the Program were identified as the "change" criteria and 11 smaller ones - side ones resulting from the necessity to adapt the PUNHSP functionality to current needs. The changes were grouped into five categories: legal, administrative, management, audit and control, as well as IT, changes. When analyzing the PUNHSP evaluation, it can be assumed that the changes introduced positively influenced the various aspects of the PUNHSP activity, but do not exhaust the possibilities of further optimization of its activity. The Program requires constant development in order to increase the efficiency and effectiveness of its operation, and the solutions used in it could be a stimulus to improve and create other pro-health programs.

Neonates with congenital Cytomegalovirus and hearing loss identified via the universal newborn hearing screening program

BackgroundCongenital cytomegalovirus (CMV) is the most common non-genetic cause of sensorineural hearing loss. Currently, there are no universal CMV screening programs for newborns or routine CMV testing of neonates with hearing loss in Australia, or elsewhere. ObjectivesThis study was undertaken to determine the prevalence of congenital CMV infection in infants with hearing loss identified using routine resources via the Australian universal neonatal hearing screening (UNHS) program. Study designInfants who failed UNHS, referred for audiological testing and found to have permanent hearing loss were screened for CMV via PCR of urine and saliva. Congenital CMV was diagnosed if CMV was detected in infants ≤30 days of age, or using retrospective testing on stored new born screening cards, retrospective testing, or using clinical criteria if >30 days of age. The cohort was analyzed for time of testing and prevalence of congenital CMV determined. ResultsThe Audiology Department reviewed 1669 infants who failed UNHS between 2009 and 2016. Thirty percent (502/1669) had permanent hearing loss confirmed, of whom 336/502 were offered CMV testing. A definite (n = 11) or probable (n = 8) diagnosis of congenital CMV occurred in 19/323 (5.9%), of whom definite diagnoses were made in 4/19 on tests positive prior to 21 days of life, in 5/19 who were positive on neonatal blood screening card (NBSC) testing, in 2/19 who were positive on placental testing. In 8/19 probable diagnoses were made based on positive testing between ages 23–42 days and a consistent clinical syndrome in the absence of another cause for hearing loss after genetic and other testing. CMV testing mirrored the timing of audiological testing, with ∼40% completing audiology and CMV testing by 21 days, and 64% by 30 days. ConclusionThis program, utilizing existing clinical services identified probable congenital CMV in ∼6% of a large cohort failing UNHS with permanent hearing loss, of whom more than half were definite diagnoses. No additional assets were required to those already existing in this tertiary referral pediatric centre, whilst providing useful and timely data for clinical and audiological management.

The report of the Polish Universal Neonatal Hearing Screening Program in 2016.

The Polish Universal Neonatal Hearing Screening Program (PUNHSP) has been carried out in Poland for 14 years. The main aim of this Program is to organize hearing screening tests and to gather the information about risk factors of hearing loss in almost all newborns in Poland. It consists of 496 centers at 3 referral levels. A total of 5 458 114 children had been registered in the Central Database (CDB) of PUNHSP by the 22nd of August 2017. Bilateral sensorineural hearing loss was the most frequently appearing hearing impairment in children. It was diagnosed in 260 cases in 2016. This report presents the most important results and conclusions concerning the running of the PUNHSP in 2016.

International consensus (ICON) on audiological assessment of hearing loss in children

The prevalence of hearing loss in newborns and infants is estimated between 1 to 3.47 cases per 1000 live births. Early diagnosis and rehabilitation of congenital hearing loss are mandatory in order to achieve a satisfactory linguistic and cognitive development. Without appropriate opportunities to learn language, these children will fall behind their normal hearing peers in communication, cognition, reading and socio-emotional development. After promising results, neonatal screening for hearing loss and audiological evaluation are becoming more extensively carried out. In planning universal neonatal hearing screening programs, transient evoked otoacoustic emissions and auditory brainstem responses are the gold standard for the screening and diagnosis program. However, there is no consensus regarding the use of audiometry and other electrophysiological tests (such as auditory steady-state responses) in current practices. Several screening and audiological assessment procedures have been described and advocated all around the world. But, a systematic scheme of performing diagnosis in the pediatric audiology population is lacking. A consensus conference was held at the International Federation of Oto-rhino-laryngological Societies Congress, in June 2017, to discuss the different current practices and to identify the best neonatal hearing screening and audiological assessment management. This article is intended to provide professionals with recommendations about the “best practice” based on consensus opinion of the session's speakers, and a review of the literature on the efficacy of various assessment options for children with hearing loss.

Role of Otoacoustic Emissions in Hearing Assessment of Neonates: A Prospective Observational Study.

Hearing plays an important role for children in learning speech and language, socialization and cognitive development. The child learns to speak based on what is heard (Rundjan et al. in Sari Pediatr 6(4):149-154, 2005). The aim of the study was to find out the percentage of hearing loss by OAE among low risk and high risk neonates. All the neonates (Low and High risk) born in a tertiary care center were screened by OAE before their discharge from the hospital and after stabilizing high risk neonates. The referred neonates were followed after two weeks. Total 722 neonates were screened of which 130 were high risk and 592 were low risk. Neonates with Serum bilirubin >20mg/dl or requiring exchange transfusion were excluded as OAE will be unreliable in them and they should be subjected to BERA directly. Percentage of bilateral hearing loss came to be 4.2%, 4 participants were lost to follow up and percentage of hearing loss on subsequent OAE came to be 11.6%. Low birth weight was an important risk factor for hearing loss (p value significant on initial and follow-up). Percentage of hearing loss with low birth weight, hyperbilirubenemia, low apgar score and prematurity to be 16%. Mechanical ventilation contributed 13% of total hearing loss followed by ototoxicity. Neonates with family history of childhood SNHL, in utero infection, craniofacial anomaly and bacterial meningitis contributed 3% of total hearing loss. On subsequent follow-up, one neonate was low-risk and the other 25 were high risk, of which 3 high risks showed sustained OAE refer. However the low risk neonate had normal outer hair cell function i.e. OAE pass. This study clearly demonstrates importance of Universal Neonatal Hearing Screening Programme so that hearing loss can be detected as early as possible and possible intervention can be taken at the earliest.