Newborn Hearing Screening Program Research Articles

Parental/Caregiver Satisfaction and Anxiety with Newborn Hearing Screening Program in Dakshina Karnataka, India – A Questionnaire Based Study

Parental/Caregiver Satisfaction and Anxiety with Newborn Hearing Screening Program in Dakshina Karnataka, India – A Questionnaire Based Study

Newborn Hearing Screening Success… Patient Care Failure.

To assess the efficacy of a newborn hearing screening program in an urban hospital 24 years after a longitudinal study identified failings in its program and recommended solutions. A hospital-specific, Pennsylvania database was queried to determine rates of in-hospital testing, referral, and diagnosis for Temple University Hospital (TUH), and to compare these with reporting sites statewide. At TUH, interviews with newborn hearing screeners and audiologists were conducted to identify barriers to care. In the year 2022, 2,006/2,069 newborns (97%) were successfully screened prior to hospital discharge at TUH. (63 babies were never tested). There were 73 who did not pass automated auditory brainstem response testing (ABR) in at least one ear. All of these 73 infants returned for repeat automated ABR testing and 50/73 passed in both ears. 23/2006 (1%) were referred to diagnostic testing. 13/23 were successfully scheduled. 11/23 passed in both ears. 2/13 were found to have hearing losses. 10 infants (43%) were lost to follow-up and were never diagnosed. Examining lost-to-follow-up rates statewide, we identified a relationship between successful follow-up and local median family income. Legislation on the state level and improvements in audiometric technology have led to highly successful in-hospital screening with relatively low false-positive rates. Despite this, inadequate outpatient follow-up testing and inequities in the American health care system continue to delay audiometric diagnosis and habilitation of congenital hearing loss. Lack of transportation, childcare for siblings, and newborn insurance status impede access to diagnostic testing. Level 3 evidence-retrospective review comparing cases and controls. Laryngoscope, 2024.

Revisiting the transient-evoked otoacoustic emissions passing criteria used for newborn hearing screening

Objective To assess transient-evoked otoacoustic emissions (TEOAE) data from 15 years of a newborn hearing screening program and evaluate how well various criteria separate ears with and without hearing loss. Design Retrospective review of TEOAE data using logistic regression, receiver operating characteristic curves, and cumulative percentage graphs. Study sample: Children with hearing loss who passed TEOAE screening as a newborn were compared to children who failed TEOAE screening and normal hearing children who either passed or failed. Exclusions were applied for acquired hearing loss or auditory neuropathy. Results Ears with hearing loss that passed screening had significantly lower TEOAE response levels compared to ears with normal hearing. Noise levels, test times, and number of sweeps were also lower. Most of these ears had mild hearing loss. Logistic regression results showed that high-frequency TEOAE response level is the best predictor of hearing loss. A multivariate “logit” score calculated from the regression was the best indicator for separating ears with hearing loss from ears with normal hearing. Conclusions TEOAE response levels or an algorithm which incorporates logit scores should be considered as a minimum passing criterion to increase the sensitivity of the TEOAE screening.

Coalition for Global Hearing Health Hearing Care Pathways Working Group: Guidelines for Clinical Guidance for Readiness and Development of Evidence-Based Early Hearing Detection and Intervention Programs.

Editor's Note: The following article discusses the timely topic Clinical Guidance in the areas of Evidence-Based Early Hearing Detection and Intervention Programs. This article aims to discuss areas of services needed, guidance to countries/organizations attempting to initiate early hearing detection and intervention systems. Expert consensus and systematic/scoping reviews were combined to produce recommendations for evidence-based clinical practice. In Ear and Hearing, our long-term goal for the Point of View article is to stimulate the field's interest in and to enhance the appreciation of the author's area of expertise. Hearing is an important sense for children to develop cognitive, speech, language, and psychosocial skills. The goal of universal newborn hearing screening is to enable the detection of hearing loss in infants so that timely health and educational/therapeutic intervention can be provided as early as possible to improve outcomes. While many countries have implemented universal newborn hearing screening programs, many others are yet to start. As hearing screening is only the first step to identify children with hearing loss, many follow-up services are needed to help them thrive. However, not all of these services are universally available, even in high-income countries. The purposes of this article are (1) to discuss the areas of services needed in an integrated care system to support children with hearing loss and their families; (2) to provide guidance to countries/organizations attempting to initiate early hearing detection and intervention systems with the goal of meeting measurable benchmarks to assure quality; and (3) to help established programs expand and improve their services to support children with hearing loss to develop their full potential. Multiple databases were interrogated including PubMed, Medline (OVIDSP), Cochrane library, Google Scholar, Web of Science and One Search, ERIC, PsychInfo. Expert consensus and systematic/scoping reviews were combined to produce recommendations for evidence-based clinical practice. Eight essential areas were identified to be central to the integrated care: (1) hearing screening, (2) audiologic diagnosis and management, (3) amplification, (4) medical evaluation and management, (5) early intervention services, (6) family-to-family support, (7) D/deaf/hard of hearing leadership, and (8) data management. Checklists are provided to support the assessment of a country/organization's readiness and development in each area as well as to suggest alternative strategies for situations with limited resources. A three-tiered system (i.e., Basic, Intermediate, and Advanced) is proposed to help countries/organizations at all resource levels assess their readiness to provide the needed services and to improve their integrated care system. Future directions and policy implications are also discussed.

Cochlear implantation in pediatrics: Impact of newborn hearing screening on intervention time

ObjectivesThe newborn hearing screening (NHS) program was globally established for early hearing loss (HL) identification and intervention. Early intervention is essential to minimize or prevent the negative consequences of HL. In Saudi Arabia, the NHS was officially implemented in 2016. Currently, its impact on the timing of cochlear implantations (CIs) in Saudi Arabia remains unclear, and information on potential hospital-related delays affecting early implantation is lacking. Thus, this study aimed to evaluate the effect of implementing the NHS on age at CI in children with prelingual deafness in a CI center in Saudi Arabia, and to evaluate the hospital timing in the CI process. MethodsAll pediatric CI users who presented for the first time to the CI committee (CIC) at a tertiary center and received their implants between 2015 and 2022 were enrolled in this study. Date of birth (DOB), date of presentation to the CI committee (DOCIC), and date of CI surgery (DOCIS) were retrospectively reviewed. ResultsIn total, 304 CI children were included in the analysis. Approximately 55 % of the children (n = 167) were screened for HL through the NHS, whereas 45 % of the children (n = 137) were born before the launch of the NHS. Both age at the presentation to the CIC (i.e. difference between DOCIC and DOB) and age at implantation (i.e. difference between DOCIS and DOB) were significantly earlier in children who were screened for HL through the NHS than those who were not screened (P < 0.0001). The time difference between the DOCIC and DOCIS was not significantly different between the screened and unscreened children (P > 0.05). ConclusionThe implementation of the NHS in the tertiary center has a significant positive effect on age at presentation to the CIC and age at implantation, but not on the actual CI surgery. Further research is needed to reduce the hospital delays before the actual surgery in order to increase the likelihood of children receiving implantation early in their life.

Audiologist’s perspective on newborn hearing screening in a developing nation: a questionnaire-based survey

IntroductionHearing is crucial for a child's development, influencing speech, cognition, and emotional well-being. Early detection of hearing disorders is vital, yet India faces challenges in implementing effective newborn hearing screening (NBHS) programs. This research explores the perspectives of audiologists, focusing on the major and minor challenges faced and current protocols followed across diverse setups in India.MethodA two-phase study involved questionnaire development and data collection from 116 audiologists nationwide. The survey addressed demographics, current practices, challenges faced, and opinions on NBHS improvements.ResultsFindings revealed disparities in screening protocols, with 42 variations reported. Challenges included ambient noise control, communication gaps, and lack of interdisciplinary support. Audiologists emphasized the need for sensitizing healthcare professionals, free screening, and increased manpower to enhance NBHS efficacy.ConclusionThe study aligns with global challenges in NBHS program implementation. Rural areas face a shortage of audiologists, emphasizing the necessity to train community health workers. The varied screening protocols and delays in screening timeframe underscore the need for standardized guidelines and increased funding.Audiologists play a pivotal role in NBHS success. To overcome challenges, standardizing protocols, sensitizing healthcare professionals, and promoting community-based screening are crucial. The study emphasizes proactive measures among audiologists, continual research, and collaboration to enhance evidence-based practices and policy improvements in newborn hearing screening.

Towards Comprehensive Newborn Hearing and Genetic Screening in Russia: Perspectives of Implementation

The universal newborn hearing screening (NHS) program was implemented in Russia in 2008 to replace the high-risk newborn hearing screening. More than 95% coverage and significant improvement in early detection and intervention is achieved. Meanwhile, it was shown that current OAE-based hearing screening missed 13% of newborns with genetically ascertained hereditary sensorineural hearing loss (SNHL). The aim of the study is to assess the results of genetic investigation and NHS in a large cohort of Russian children with bilateral SNHL and to study the feasibility of implementation of combined hearing and genetic screening in Russia. Genetic, audiological and NHS data of 1292 pediatric patients with bilateral SNHL born in 2008–2021 were analyzed. GJB2 sequencing was performed for all subjects, 644 patients had pathological GJB2 genotype, 406 of them were homozygous for c.35delG variant. The group of 155 GJB2-negative patients were searched for other SNHL genes, The pathological genotypes were identified at 87 patients. The most frequent genes were STRC (21.8%), USH2A (16.1%), OTOF (8%) and SLC26A4 (6.9%). Children with confirmed genetic etiology passed NHS in 21% of cases. The perspectives of implementation of national comprehensive newborn hearing and genetic screening including whole exome sequencing technologies are discussed.

Economic evaluation of newborn deafness gene screening as a public health intervention in China: a modelling study

BackgroundWhile global newborn hearing screening programmes (NHSP) are far from the optimal level, the combined hearing and genetic screening has emerged as an innovative approach of early healthcare interventions. There...

Newborn and infant hearing screening for early detection of hearing loss in Nairobi, Kenya.

Early detection of hearing loss and subsequent intervention leads to better speech, language and educational outcomes giving way to improved social economic prospects in adult life. This can be achieved through establishing newborn and infant hearing screening programs. To determine the prevalence of hearing loss in newborns and infants in Nairobi, Kenya. A cross-sectional pilot study was conducted at the National hospital and at a sub county hospital immunization clinic. A total of 9,963 babies aged 0-3 years, were enrolled in the hearing screening program through convenient sampling over a period of nine months. A case history was administered followed by Distortion Product Oto-acoustic emissions (DPOAEs) and automated auditory brainstem response (AABR) hearing screening. The screening coverage rate was 98.6% (9963/10,104). The referral rate for the initial screen was 3.6% (356/ 9,963), the return rate for follow-up rescreening was 72% (258 babies out of 356) with a lost to follow-up rate of 28% (98/356). The referral rate of the second screen was 10% (26/258). All the 26 babies referred from the second screen returned for diagnostic hearing evaluation and were confirmed with hearing loss, yielding a prevalence of 3/1000. Establishing universal newborn and infant hearing screening programs is essential for early detection and intervention for hearing loss. Data management and efficient follow-up systems are an integral part of achieving diagnostic confirmation of hearing loss and early intervention.

Family-Perceived Barriers and Facilitators Toward a Tele-Audiology Infant Diagnostic Testing Approach in Victoria, Australia.

Background: Universal newborn hearing screening programs allow for early identification of congenital hearing loss. However, some families experience difficulties accessing diagnostic audiology services following a refer screen result. Methods: This study aimed to assess the opinions of families who had experienced infant diagnostic audiology assessments regarding a telehealth option for these appointments within Victoria, Australia. Families who attended in-person infant diagnostic audiology appointments were sent a questionnaire exploring their experiences of the service and their opinion regarding a proposed telehealth option for infant diagnostic audiology (50 responses received). These results were also compared to those of families who were surveyed following testing in 2020, where the audiologist conducted the appointment remotely to comply with COVID-19-related social distancing recommendations at the time (10 responses received). Results: There were not significant differences between the duration or number of appointments, perceived understanding of results, or concerns regarding a tele-audiology model between families who experienced face-to-face and tele-audiology infant diagnostic audiology appointments. Opinions of infant diagnostic audiology appointments utilizing telehealth technology were largely positive, and minimal technological difficulties were identified. Conclusion: Overall positive attitudes of many families with infant diagnostic appointment experiences toward a tele-audiology option of this service suggest that offering a telehealth model of appointments may be an appropriate model to improve service access for families requiring infant diagnostic audiology in Victoria.

The Cost Effectiveness of Newborn Hearing Screening Program: New Approach of Iran Ministry of Health and Medical Education in 2022

Background: In Iran’s newborn hearing screening (NHS) program, OAE and AABR are administered to early identify hearing impairment in the primary health care (PHC) system. Objectives: The aim of this study was to conduct a cost-effectiveness analysis of this program integrated into Iran's health system. Methods: We examined the cost-effectiveness of AABR and OAE screening procedures in infants less than 1 month old. TreeAge 2022 software was used for cost-effective modeling. In this study, QoL and QALY were considered as the outcome measures. To evaluate the robustness of the modeling results, we used one-way sensitivity analysis. A ± 20% variation was utilized to determine target variables and estimate the costs per identification. Results: In 2022, a total of 1,106,072 babies were born in Iran, of whom 1,006,293 underwent hearing impairment screening (coverage rate: 90.97%). Overall, 3,359 of the newborns screened were diagnosed with hearing problems, indicating a mean prevalence of 3.3 per 1000 births. The ICER was equal to 3297.2 US PPP (Purchasing Power Parity) per QALY. According to our results, the dominant cost-effective strategy for hearing impairment screening was OAE plus AABR. After 1000 iterations, the second cost-effective strategy was found to be OAE alone, according to Monte Carlo simulation modeling. Conclusions: In this study, we evaluated the cost-effectiveness of the NHS program for detecting hearing impairment in over a million births in Iran. A two-step screening approach, including OAE and AABR, was found to be the dominant cost-effective strategy to identify newborns with hearing impairment.

Development of audiology in Ghana: past, present, and future

Objectives This paper provides an overview of Ghana's audiology journey, tracking its evolution since the 1970s. It explores the historical context, transitions, and current state of audiology in Ghana, highlighting the challenges and prospects. Design Descriptive and analytic approach drawing from diverse qualitative and quantitative data sources. Study sample The study encompasses various sources and data types, such as reports, academic literature, and statistical data. It also incorporates information related to developing audiology programmes in tertiary institutions, mobile van outreach units, cochlear implant surgeries and newborn hearing screening. Results Ghana's audiology journey has transformed significantly since its inception in the 1970s. From its humble beginnings with few audiologists and limited facilities, audiology has transitioned from an unknown field to growing recognition. The emergence of audiology programmes in tertiary institutions signals progress. Challenges persist such as disparity in facility distribution, low professional employability, and limited access. Nevertheless, initiatives like cochlear implant surgeries, and newborn hearing screening programmes reflect proactive steps. Conclusion Ghana's audiology journey is a testament to visionary thinking, dedication, and collaborative efforts. While the journey has been marked by significant developments, bridging disparities, ensuring equitable facility distribution, and formulating comprehensive policies are pivotal.

Parental Knowledge and Attitudes to Childhood Hearing Loss and Hearing Services in the Kingdom of Saudi Arabia

Abstract Background: Childhood hearing is critical to the development of speech, language, and social skills. Hearing loss (HL) can interfere with learning and requires early detection and management. This study aimed to evaluate parental knowledge with attitudes toward childhood HL, and available hearing services across the Kingdom of Saudi Arabia. Methods: This cross-sectional study was conducted among parents of children from different regions of Saudi Arabia. Data were collected using an online self-administered questionnaire and analyzed using SPSS version 23. This study examined the relationships among socio-demographic characteristics and knowledge and attitudes toward HL using a Chi-square test. Results: Assessment of the prevalence of various aspects of knowledge and attitudes among parents toward childhood HL revealed that 732 (58.4%) participants had a good level of knowledge, while 521 (41.6%) had poor knowledge. Furthermore, the attitude analysis revealed that 871 (69.5%) participants expressed a good attitude, whereas 382 (29.5%) had a poor attitude. A significant association was noted between age group and attitude (P = 0.005). Conclusion: The findings demonstrated a gap in the level of knowledge and significant variation regarding a certain set of risk factors responsible for HL, which highlighted the need for establishing educational programs across the Kingdom to mitigate these harms and promote parental awareness. Parental attitudes toward auditory services were satisfactory; however, efforts are needed to familiarize parents with newborn hearing screening programs that are widely available in the Kingdom.

Late-onset, progressive sensorineural hearing loss in the paediatric population: a systematic review.

To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection. PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL. Electronic searches were performed through Medline, Embase, Cochrane, and Emcare. 37 studies were included. 21 showed an association between late-onset SNHL and congenital cytomegalovirus (cCMV) infection (age at hearing loss diagnosis 0.75 to 204months, mean 45.6 ± 43.9), while 16 between late-onset SNHL and other congenital or perinatal factors, namely Neonatal Intensive Care Unit (NICU) stay, prematurity, neonatal respiratory failure, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) support, hypocapnia, hypoxia, alkalosis, seizure activity, congenital diaphragmatic hernia (CDH), inner ear malformation, and gene mutations (age at hearing loss diagnosis 2.5 to 156months, mean 38.7 ± 40.7). cCMV infection may cause late-onset SNHL, which can be missed on standard NHSP. There is, therefore, evidence to support universal screening programmes to enable detection in even asymptomatic neonates. Ongoing audiological follow-up for all children with cCMV is advisable, to enable timely treatment. In the paediatric population presenting conditions such as NICU stay > 5days, prematurity ≤ 34weeks gestation, severe neonatal respiratory failure, mechanical ventilation, ECMO support, and CDH surgery, an audiological follow-up from 3months of age up to at least 3-4years of age, and at least annually, should be recommended.

SWOT analysis of Russian universal newborn hearing screening program

Introduction. The negative impact of congenital hearing loss on child’s development, education and socialization can be reduced by early identification and intervention as a result of universal newborn hearing screening. The screening program needs continuous improvement by monitoring of outcomes and correction of algorithms. The SWOT analysis can be applied to make effective decisions. &#x0D; The purpose of the study is to identify strategies for the improvement of the Russian newborn hearing screening program based on SWOT analysis.&#x0D; Materials and methods. The study was based on the experience of two pediatric audiological centres in Novosibirsk and St. Petersburg. &#x0D; At the first stage, in accordance with the SWOT methodology, internal and external factors, the strengths and weaknesses of the Russian hearing screening program were identified, as well as the opportunities and threats that can affect the audiological care for children. At the second stage, pairwise analysis of strengths, weaknesses, opportunities and threats was carried out.&#x0D; Results. The strengths of the Russian hearing screening program are national level implementation, high coverage of newborns in maternity hospitals, the use of modern objective audiological methods. Weaknesses are associated with a lack of medical personnel, need to equipment replacement, number of cases lost to follow-up. Opportunities are the availability of high-quality domestic diagnostic equipment, possibility for active interaction of specialists and families. The threats are caused by an imperfect legal framework and funding mechanisms, low awareness of parents, related professionals and society about the problem of congenital hearing loss. &#x0D; Limitations. The study results are limited by the expertise of the authors without involving the wide range of stakeholders.&#x0D; Conclusion. The developed strategies can increase timely audiological service to hearing impaired children and their families and to plan the necessary resources in the face of modern challenges.

Qualitative Analysis of Newborn Hearing Screening Program in Iran.

Early diagnosis of hearing loss and timely interventions are important to minimize the consequences of this condition, especially for children. This research was conducted to analyze the newborn hearing loss screening program in Iran. This qualitative study was conducted using the content analysis method and based on the CIPP model in 2023. The snowball method was used to recruit a sample with maximum diversity. The criteria for selecting people for interviews included having at least three years of experience in the newborn hearing loss screening program and sufficient knowledge in the field. To ensure the reliability of the results, four criteria proposed by Lincoln and Guba were used. Data analysis was conducted by MAXQDA2022 software. In the current research, using content analysis in the form of the CIPP model, based on the viewpoints of the interviewees (40 people), the management requirements of the newborn hearing loss screening program were categorized into the four main categories of context (texture), input, process, and output. Eight subcategories were identified in the context dimension, four subcategories in the input dimension, seven subcategories in the process dimension, and four subcategories in the output dimension. According to the findings of this research, in order to properly implement the newborn hearing loss screening program, there is a need to conduct pilot studies, need assessments, evidence-based programs, and epidemiological studies and to prioritize services and resources. Also, communication between service delivery levels needs to be improved, and attention should be paid to personnel motivation and screening programs.

Newborn and Infant Hearing Screening for Early Detection of Hearing Loss in Nairobi, Kenya

Background: Early detection of hearing loss and subsequent intervention leads to better speech, language and educational outcomes giving way to improved socioeconomic prospects in adult life. This can be achieved through establishing universal newborn hearing screening (UNHS) programs. The objectives of this study were to assess the feasibility of implementing a UNHS program and to also determine the prevalence of hearing loss in newborns in Nairobi, Kenya.&#x0D; Materials And Methods: A cross-sectional pilot study was conducted at the National Hospital and a sub-county hospital immunization clinic. A total of 9,963 babies aged 0-3 years, were enrolled in the hearing screening program through convenient sampling over nine months. A case history was administered followed by Distortion Product Oto-acoustic emissions (DPOAEs) and automated auditory brainstem response (AABR) hearing screening.&#x0D; Results: The screening coverage rate was 98.6% (9963/10,104). The referral rate for the initial screen was 3.6% (356/ 9,963), and the return rate for follow-up rescreening was 72% (258 babies out of 356) with a loss to follow-up rate of 28% (98/356). The referral rate of the second screen was 10% (26/258). All 26 babies referred from the second screen returned for diagnostic hearing evaluation and were confirmed to have hearing loss, yielding a prevalence of 3/1000.&#x0D; Conclusions: Establishing universal newborn and infant hearing screening programs in Kenya is feasible and essential for early detection and intervention for hearing loss. Data management and efficient follow-up systems are an integral part of achieving diagnostic confirmation of hearing loss and early intervention.

Universal newborn hearing screening program in Saudi Arabia: Current insight

Newborn hearing screening (NHS) programs are essential to identify hearing loss early in life and to improve outcomes in children. In Saudi Arabia, the national NHS program has been operational since 2016; however, few studies have evaluated its status, and none have covered all provinces across the country. This cross-sectional retrospective study provides an overview of the program's status across all provinces, focusing on screening coverage rates, referral/fail rates, and follow-up procedures. In 2021, 199,034 newborns were screened, with a coverage rate of 92.6% and an overall referral/fail rate of 1.87%. These performance measures provide a foundation for future progress and improvements. This study highlights the importance of ongoing efforts to enhance the program's effectiveness and sustainability.

Evaluation of newborn hearing screening program in Jordan.

The Newborn Hearing Screening (NHS) program was officially launched in Jordan in 2021. Since its inception, no studies have examined the effectiveness of the program. This study seeks to assess the effectiveness and outcomes of the NHS program in Jordan. A retrospective cross-sectional study was conducted to investigate the program coverage rate, referral rate, loss to follow-up rate and the hearing status of newborns who successfully completed the necessary diagnostic assessment. Live births in all hospitals administered by the Ministry of Health (MoH) in Jordan from July 2021 to November 2023 were included. Out of 25,825 newborns delivered, 99.4% (25,682) were screened. A referral rate of 0.7% (189) was recorded. Approximately 61.9% of those referred (n = 117) had normal hearing, while 31.7% (60 infants) were diagnosed with hearing loss. The prevalence of congenital hearing loss was 0.14%, and the mean age for identifying hearing loss was 11 months. The current status of the NHS program in Jordan is promising. The program has achieved most benchmarks recommended by the Joint Committee on Infant Hearing (JCIH), demonstrating encouraging outcomes. There is a need to investigate and address the factors causing delays in the identification of hearing loss in Jordan.

Evaluation of newborn hearing screening results of infants with phenylketonuria

ObjectiveThe purpose of this study is comparing the newborn hearing screening failure rate between phenylketonuria (PKU) infants and matched healthy control infants. Patients and methodsBetween April 2021 and April 2023, data obtained from the national newborn hearing screening program were examined for patients who presented to the pediatric metabolism clinic of Konya City Hospital with a clinical and genetic diagnosis of PKU. A healthy control group, matched for age and gender, was established. The newborn hearing screening test results, demographic information, and pregnancy data of infants with PKU and the control group were compared to assess risk factors. ResultsIn the initial screening test, 23 out of 73 PKU infants (31.5%) and 12 out of 73 infants in the control group (16.4%) failed the test (p = 0.033). Among the 23 PKU infants who did not pass the first test, 9 (35%) also failed the second test and were referred. In contrast, all 12 infants in the control group who did not pass the first test passed the second test. The failure rate in the second test was significantly higher in PKU infants compared to the control group (p = 0.003). It was observed that the maternal age of PKU infants who underwent the second test was significantly higher than that of the control group (p < 0.029). Diagnostic hearing test results were found to be normal in all nine PKU patients who failed the secondary screening test and were referred to a tertiary center. ConclusionIn our study, it was determined that infants with phenylketonuria (PKU) who did not have any risk factors for hearing loss failed the hearing screening test significantly more than healthy infants.