Newborn Hearing Screening Benefits Children, but Global Disparities Persist

There is substantial evidence that newborn hearing screening (NHS) reduces the negative sequelae of permanent childhood hearing loss (PCHL) if performed in programs that aim to screen all newborns in a region or nation (often referred to as Universal Newborn Hearing Screening or UNHS). The World Health Organization (WHO) has called in two resolutions for the implementation of such programs and for the collection of large-scale data. To assess the global status of NHS programs we surveyed individuals potentially involved with newborn and infant hearing screening (NIHS) in 196 countries/territories (in the following text referred to as countries). Replies were returned from 158 countries. The results indicated that 38% of the world’s newborns and infants had no or minimal hearing screening and 33% screened at least 85% of the babies (hereafter referred to as UNHS). Hearing screening programs varied considerably in quality, data acquisition, and accessibility of services for children with PCHL. In this article, we summarize the main results of the survey in the context of several recent WHO publications, particularly the World Report on Hearing, which defined advances in the implementation of NHS programs in the Member States as one of three key indicators of worldwide progress in ear and hearing care (EHC).

Application of otoacoustic emissions and brainstem evoked response audiometry in newborn hearing screening

<p class="abstract"><strong>Background: </strong>Newborn hearing screening was conducted in a tertiary care hospital in a step by step manner using otoacoustic emissions (OAE) and brainstem evoked response audiometry (BERA) and details were recorded.</p><p class="abstract"><strong>Methods:</strong> A prospective institutional based study was conducted. All the newborns born in the hospital over a period of 18 months from December 2018 to May 2020 were considered in the study. Healthy newborns were screened bedside within 24 hours of delivery and NICU (Neonatal Intensive Care Unit) babies were screened in the NICU. Handheld OAE apparatus was used as the initial screening tool. A total of 3 OAEs were done for babies with a “refer” result in the OAEs, which were done 1 month apart. Babies with a “refer” in the third OAE were subjected for BERA.</p><p class="abstract"><strong>Results:</strong> A total of 14226 babies were screened at 24-48 hours of birth. Among them, 13,069 babies passed the first OAE screening in both ears. Remaining babies were referred for further follow-up. After subsequent follow-ups and successive testing, 11 babies were found to have hearing loss, which was diagnosed within 4-5 months of the child’s birth.</p><p class="abstract"><strong>Conclusions: </strong>Universal newborn hearing screening is the need of the day. OAE is an effective screening tool for newborn hearing loss. When complemented by BERA, majority of congenitally deaf babies can be diagnosed at a very early age. This helps in early intervention.</p>

Clinicians’ Views and Experiences with Offering and Returning Results from Exome Sequencing to Parents of Infants with Hearing Loss

Exome sequencing (ES) is an effective method for identifying the genetic cause of hearing loss in infants diagnosed through newborn hearing screening programs. ES has the potential to be integrated into routine clinical care, yet little is known about the experiences of clinicians offering this test to families. To address this gap, clinicians involved in a clinical study using ES to identify the cause of infants’ hearing loss were interviewed to explore their experiences with offering and returning results to parents. Interview transcripts were analysed using inductive content analysis. Twelve clinicians participated: seven genetic counsellors, four clinical geneticists, and one paediatrician. Most clinicians were supportive of offering ES to infants with hearing loss, primarily because results may inform the child’s clinical management. However, some expressed concerns, questioning the utility of this information, particularly for isolated hearing loss. Clinicians had differing views regarding the optimal time to offer ES to families; while some felt that families can manage everything at once, others recommended delaying testing until parents have come to terms with their child’s diagnosis. These findings show the complexity involved in determining how ES should be offered to families following the diagnosis of a child with hearing loss, particularly with regards to when testing is suggested.

Hubungan Lingkungan dan Sosial Budaya dengan Pengetahuan Orang Tua Terhadap Deteksi Dini Pendengaran Bayi Baru Lahir di Rumah Sakit Mitra Husada Pringsewu Lampung

ABSTRACT:THE CORRELATION BETWEEN ENVIRONMENT AND SOCIO-CULTURAL WITH PARENT’S KNOWLEDGE TO EARLY DETECTION OF HEARING FOR NEWBORN BABY AT MITRA HUSADA HOSPITAL PRINGSEWU LAMPUNG Introduction: The incidence of deafness in newborns is 0.001% to 0.5% and increases in infants with risk factors reaching 1% -5%, which occurs in about 4-6 babies in 1000 live births in developing countries. Newborn hearing screening is a program to reduce the increasing incidence of hearing loss in the world. Knowing the child's hearing condition from an early age is an important thing to do where parental knowledge plays a role in early detection of hearing in infants. One of the factors that influence knowledge is environment and socio-culture. Purpose: To determine the environmental and socio-cultural relationship with parents' knowledge about early detection of hearing in newborns at Mitra Husada Pringsewu Hospital, Lampung. Method: The design of this study was an observational analytic with adesign cross sectional. The sample selection used total sampling of 60 respondents. Results: The data obtained were analyzed using thetest Chi-Square which showed that there was a significant relationship between the environment and parental knowledge where the p-value was 0.000 (p-value <0.005) with an OR of 13.00 and 95% CI 3.11-54.26 and the results of the analysis between social culture with parental knowledge showed significant results where the p-value was 0.015 with OR 4.42 and 95% CI 1.27-15.38. Conclusion: There is a significant relationship between environment and socio-culture with parents' knowledge of early detection of hearing of newborns at Mitra Husada Pringsewu Hospital, Lampung. Keywords : Parent’s knowledge, Early Hearing Detection, Environment, Socio-cultural INTISARI : Hubungan Lingkungan Dan Sosial Budaya Dengan Pengetahuan Orang Tua Terhadap Deteksi Dini Pendengaran Bayi Baru Lahir Di Rumah Sakit Mitra Husada Pringsewu Lampung Latar Belakang: Insidensi ketulian pada bayi baru lahir 0,001-0,5% dan meningkat pada bayi dengan faktor risiko mencapai 1%-5%, dimana terjadi pada sekitar 4-6 bayi pada 1000 kelahiran hidup. Skrining pendengaran bayi baru lahir merupakan program untuk mengurangi angka kejadian gangguan pendengaran di dunia yang semakin meningkat. Mengetahui kondisi pendengaran anak sejak dini merupakan hal yang penting, dimana pengetahuan orang tua berperan terhadap deteksi dini pendengaran pada bayi. Salah satu faktor yang mempengaruhi pengetahuan adalah lingkungan dan sosial budaya. Tujuan: Mengetahui hubungan lingkungan dan sosial budaya dengan pengetahuan orang tua tentang deteksi dini pendengaran pada bayi baru lahir di Rumah Sakit Mitra Husada Pringsewu Lampung. Metode Penelitian: Desain penelitian ini adalah analitik observational dengan rancangan Cross Sectional. menggunakan Total Sampling sebanyak 60 responden. Hasil: Data yang diperoleh dianalisis dengan uji Chi-Square menunjukan terdapat hubungan yang signifikan antara lingkungan dengan pengetahuan orang tua dimana p-value ­0.000 (< 0,005) dengan OR 13.00 dan CI 95% 3.11-54.26 dan hasil analisis antara sosial budaya dengan pengetahuan orangtua menunjukkan hasil yang signifikan dimana p-value 0.015 dengan OR 4.42 dan CI 95% 1.27-15.38. Kesimpulan: Terdapat hubungan yang signifikan antara lingkungan dan sosial budaya dengan pengetahuan orang tua terhadap deteksi dini pendengaran bayi baru lahir di Rumah Sakit Mitra Husada Pringsewu Lampung. Kata Kunci: Pengetahuan Orang Tua, Deteksi Dini Pendengaran, Lingkungan, Sosial Budaya

Newborn hearing loss in the south of China: a cross-sectional study

Objective Newborn hearing screening can identify congenital deafness and hearing loss. The current status of newborn hearing screening in the south of China is unclear. We aimed to assess the hearing loss of newborns in Dongguan, China. Methods A total of 62,545 newborns were enrolled in this retrospective, cross-sectional study between September 2015 and August 2020. The screening procedure was carried out using a two-step hearing screening. The trends were examined by the Cochran–Armitage trend test. Results From 2015 to 2020, the total initial newborn hearing screening rate was 98.16%, and it significantly increased over time (Z = 2.488). The initial screening pass rate of newborns was 90.08%, and no significant difference was observed in the initial screening pass rate between different years (Z = 0.845). After two-step hearing screening, the overall hearing screening pass rate of newborns was 94.65%. The overall hearing screening pass rate in normal newborns was higher than that in high-risk newborns (95.70% vs. 93.59%). Conclusion The initial newborn hearing screening rate increased yearly in the study period, but there was still an approximately 10% referral rate. The initial screening pass rate in China needs to be further improved.

Concurrent Newborn Hearing and Genetic Screening in a Multi-Ethnic Population in South China

Hearing loss is a common sensory deficit in humans with intricate genomic landscape and mutational signature. Approximately 1–3 out of 1,000 newborns have hearing loss and up to 60% of these cases have a genetic etiology. In this study, we conducted the concurrent newborn hearing and genetic screening in 20 mutations (18 pathogenic variants in GJB2, SLC26A4, and MT-RNR1 and 2 uncertain clinical significance variants in GJB3) for 9,506 normal newborns (4,977 [52.4%] males) from 22 ethnic population in South China. A total of 1,079 (11.4%) newborns failed to pass the initial hearing screening; 160 (1.7%) infants failed to pass the re-screening, and 135 (1.4%) infants presented the diagnostic hearing loss. For the genetic screening, 220 (2.3%) newborns who presented at least one of the screened mutations were more likely to fail the hearing screening and have diagnostic hearing loss than mutation-negative newborns. In comparison to the differences of distribution of mutations, we did not identify any significant difference in the prevalence of screened mutations between Han group (n = 5,265) and Zhuang group (n = 3,464), despite the lack of number of minority ethnic groups. Studies including larger number of minority ethnic populations are needed in the future.