Newborn Hearing Screening Benefits Children, but Global Disparities Persist.
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ABSTRACT:
SUBMITTER: Neumann K
PROVIDER: S-EPMC8746089 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Early intervention for newborns who are deaf or hard-of-hearing leads to improved language, communication, and social-emotional outcomes. Universal physiologic newborn hearing screening has been widely implemented across the United States with the goal of identifying newborns who are deaf or hard-of-hearing, thereby reducing time to diagnosis and intervention. The current physiologic newborn hearing screen is generally successful in accomplishing its goals but improvements could be made. In the past ten years, genetic testing has emerged as the most important etiological diagnostic test for evaluation of children with deafness and congenital cytomegalovirus has been recognized as a major cause of childhood deafness that may be treatable. A comprehensive newborn hearing screen that includes physiologic, genetic, and cytomegalovirus testing would have multiple benefits, including (1) identifying newborns with deafness missed by the current physiologic screen, (2) providing etiologic information, and (3) possibly decreasing the number of children lost to follow up. We present a framework for integrating limited genetic testing and cytomegalovirus screening into the current physiologic newborn hearing screening. We identify needed areas of research and include an overview of genome sequencing, which we believe will become available over the next decade as a complement to universal physiologic newborn hearing screening.
| S-EPMC6831511 | biostudies-literature
Project description:Scope:Jurisdictional-based Early Hearing Detection and Intervention Information Systems (EHDI-IS) collect data on the hearing screening and follow-up status of infants across the United States. These systems serve as tools that assist EHDI programs' staff and partners in their tracking activities and provide a variety of data reports to help ensure that all children who are deaf/hard of hearing (DHH) are identified early and receive recommended intervention services. The quality and timeliness of the data collected with these systems are crucial to effectively meeting these goals. Methodology:Forty-eight EHDI programs, funded by the Centers for Disease Control and Prevention (CDC), successfully evaluated the accuracy, completeness, uniqueness, and timeliness of the hearing screening data as well as the acceptability (i.e., willingness to report) of the EHDI-IS among data reporters (2013-2016). This article describes the evaluations conducted and presents the findings from these evaluation activities. Conclusions:Most state EHDI programs are receiving newborn hearing screening results from hospitals and birthing facilities in a consistent way and data reporters are willing to report according to established protocols. However, additional efforts are needed to improve the accuracy and completeness of reported demographic data, results from infants transferred from other hospitals, and results from infants admitted to the Neonatal Intensive Care Unit.
| S-EPMC6945814 | biostudies-literature
Project description:Introduction In pregnancy, the mother and fetus share body structures based on the maternal organism. Exposure to psychoactive drugs in this period may have repercussions on the baby's hearing. Therefore, it is necessary to investigate this association. Aim Analyze the results of newborn hearing screening (NHS), the occurrence of associated risk factors, and the incidence of hearing loss in newborn exposed to psychoactive drugs during pregnancy. Methods This is an observational retrospective study done from a database analysis. From this database, records were selected about the use of psychoactive drugs by mothers during pregnancy, then the neonates were divide into two groups: the study group (146 babies exposed to drugs) and the control group (500 babies not exposed to drugs). The NHS failure rate, the presence of risk factors for hearing loss, and need for audiological diagnosis were analyzed in both groups. From these variables, absolute frequency and prevalence rates were calculated and the results compared between groups. Results There was no statistically significant difference in the comparison of NHS failure rates between the groups (p = 0.267). The occurrence of risk factors for hearing loss was greater in babies exposed to drugs (p < 0.0001). There was only one diagnosis of hearing loss, which occurred in the control group (p = 0.667). Conclusion The use of psychoactive drugs by mothers during pregnancy did not affect the NHS failure rate of this sample. However, the occurrence of significant risk factors in the study group showed a possible sensitivity of babies exposed to psychoactive drugs during pregnancy.
| S-EPMC4296945 | biostudies-other
Project description:Newborn hearing screening is an effective strategy for early identification of hearing loss in the newborn which result in early intervention and best outcome. However implementing universal screening strategy is a challenge in many resource constrained settings. There are various limitations towards successful implementation of hearing screening program in the developing countries. The cost effectiveness of the screening program also needs to be considered in a resource constrained settings. We attempt to provide a viewpoint that can be potentially helpful for the successful implementation of hearing screening in a resource constrained settings of the developing countries.
| S-EPMC7691834 | biostudies-literature
Project description:The universal newborn hearing screening (UNHS) component of the multi-center EUSCREEN project is being piloted in Albania since January 1st 2018. The aim of this study was to explore mothers' perceptions about various elements of UNHS in Albania. A cross-sectional study was carried out in the three sites of UNHS in Albania, namely in Tirana, Kukës and Pogradec during May-June 2019. During this period 512 consecutively approached mothers giving birth to included maternity hospitals were interviewed face-to-face about different aspects of UNHS. Basic socio-demographic and socioeconomic information was also collected. Mean age of participating mothers was 28.6 years ± 5.5 years. The overwhelming majority (93%) of mothers knew what their baby was being tested for, 33% were aware that hearing screening was offered in maternity hospital, 94% were very satisfied/satisfied with UNHS and about 62% were very stressed/stressed waiting for screening results, with significant sociodemographic and socioeconomic differences. The main information source about UNHS was screening staff in the maternity hospitals where mothers gave birth, reported in 67% of cases. All mothers (100%) agreed on the importance of early detection of newborn hearing problems, all mothers were willing to be informed early if their newborn baby had a hearing problem and all mothers were willing to contribute financially for testing the hearing of their newborn baby. These findings should guide information and education campaigns about UNHS in Albania. The public willingness to financially support neonatal hearing testing should be considered as an opportunity to achieve universal newborn hearing screening in the country.
| S-EPMC7444524 | biostudies-literature
Project description:IntroductionHearing loss is the most common congenital condition screened for at birth in the United States, and more than 95% of newborns are currently screened for hearing. Newborn hearing screening is most effective when infants receive timely and effective interventions. Unfortunately, follow-up rates for newborns not passing their initial hearing screenings are as low as 50% in some states. Midwives are well-positioned to encourage families to follow-up with their neonatal providers when newborns are referred for further testing. Newborn hearing screening is a relatively new practice in the United States and, to date, there has been no research regarding the informational needs and practices of certified nurse-midwives or certified midwives related to hearing screening. This study examined the knowledge, attitudes, and follow-up practices of midwives related to newborn hearing screening and intervention.MethodsA survey instrument was developed and sent to 5255 American College of Nurse-Midwives members in 50 states and 2 territories.ResultsFive hundred and eighteen surveys were returned, yielding a response rate of 9.9%. Only 68% of respondents said it was very important to screen all newborns for hearing loss. Respondents reported significant gaps in their knowledge about screening procedures, steps for referral, and the availability of resources when newborns did not pass the test. Midwives also reported the need for information about hearing loss conditions and genetics, screening guidelines, protocols for follow-up, referral networks, and therapies available.DiscussionCurrent practices in newborn hearing screening and intervention programs can be enhanced by strengthening the basic midwifery knowledge of and rationale for follow-up when newborns fail their hearing screenings. Midwives can play an integral role in optimizing hearing, speech, and family interaction by assuring that each newborn has access to the best hearing screening and referrals.
| S-EPMC3068862 | biostudies-literature
Project description:ObjectivesAutosomal recessive long QT syndrome (LQTS), or Jervell and Lange-Nielsen syndrome (JLNS), can be associated with sensorineural hearing loss. We aimed to explore newborn hearing screening combined with electrocardiograms (ECGs) for early JLNS detection.Study designIn California, we conducted statewide, prospective ECG screening of children ≤ 6 years of age with unilateral or bilateral, severe or profound, sensorineural or mixed hearing loss. Families were identified through newborn hearing screening and interviewed about medical and family histories. Twelve-lead ECGs were obtained. Those with positive histories or heart rate corrected QT (QTc) intervals ≥ 450 ms had repeat ECGs. DNA sequencing of 12 LQTS genes was performed for repeat QTc intervals ≥ 450 ms.ResultsWe screened 707 subjects by ECGs (number screened/number of responses = 91%; number of responses/number of families who were mailed invitations = 54%). Of these, 73 had repeat ECGs, and 19 underwent gene testing. No subject had homozygous or compound heterozygous LQTS mutations, as in JLNS. However, 3 individuals (with QTc intervals of 472, 457, and 456 ms, respectively) were heterozygous for variants that cause truncation or missplicing: 2 in KCNQ1 (c.1343dupC or p.Glu449Argfs*14; c.1590+1G>A or p.Glu530sp) and 1 in SCN5A (c.5872C>T or p.Arg1958*).ConclusionsIn contrast to reports of JLNS in up to 4% of children with sensorineural hearing loss, we found no examples of JLNS. Because the 3 variants identified were unrelated to hearing, they likely represent the prevalence of potential LQTS mutations in the general population. Further studies are needed to define consequences of such mutations and assess the overall prevalence.
| S-EPMC3943925 | biostudies-literature
Project description:Neurodevelopmental changes occur with asymmetric hearing loss, limiting binaural/spatial hearing and putting children at risk for social and educational challenges. These deficits may be mitigated by providing bilateral hearing in children through auditory prostheses. Effects on speech perception and spatial hearing were measured in a large cohort of >450 children who were deaf and used bilateral cochlear implants or bimodal devices (one cochlear implant and a contralateral hearing aid). Results revealed an advantage of bilateral over unilateral device use but this advantage decreased as hearing in the two ears became increasingly asymmetric. Delayed implantation of an ear with severe to profound deafness allowed asymmetric hearing, creating aural preference for the better hearing ear. These findings indicate that bilateral input with the most appropriate device for each ear should be provided early and without delay during development.
| S-EPMC6123397 | biostudies-literature
Project description:ObjectiveTo conduct a scoping review on etiologic investigation of prelingual hearing loss among children <2 years of age in the era of universal newborn hearing screening (UNHS).Data sourcesPubMed, Embase, PsycInfo, CINAHL, and Cochrane Library databases.Review methodsWe searched for articles published from January 1, 1998, to February 19, 2020. We reviewed studies that (1) included children identified with either congenital or delayed-onset hearing loss before 2 years of age among cohorts who had undergone UNHS and (2) investigated ≥1 etiologies of hearing loss. We defined hearing loss as congenital when confirmed after UNHS failure and as delayed onset when diagnosed after ≥1 assessments with normal hearing.ResultsAmong 2069 unique citations, 115 studies met criteria for full-text assessment, and 20 met our inclusion criteria. Six studies tested children diagnosed with hearing loss for genetic etiology, 9 for congenital cytomegalovirus (CMV) infection, and 5 for both. Among 1787 children with congenital hearing loss and etiologic investigation, 933 (52.2%) were tested for genetic mutations and 1021 (57.1%) for congenital CMV infection. The proportion of congenital hearing loss cases attributable to genetic etiology ranged between 7.7% and 83.3% and to congenital CMV infection between 0.0% and 32.0%.ConclusionData are lacking on the identification and etiology of delayed-onset hearing loss in children <2 years of age in the UNHS era. The proportion of congenital hearing loss cases attributable to genetic etiologies and congenital CMV infection appears to vary widely.
| S-EPMC7541667 | biostudies-literature
Project description:Evidence-based guidelines for the prevention, diagnosis and treatment of congenital cytomegalovirus (cCMV) were recently released by two independent expert groups. Of particular emphasis was the relationship between cCMV and sensorineural hearing loss (SNHL), a major component of the virus' overall disease burden. In this study, a literature review was performed to estimate the proportion of cCMV-related SNHL cases, which might be identified through selective cCMV testing following failed newborn hearing screening. Furthermore, it was of interest to estimate the potential benefit of emerging antiviral therapies. Currently, at most 10% of cCMV-related SNHL is likely to be identified clinically. Through use of a selective cCMV testing protocol, however, a significant improvement in the identification rate can be achieved. Recent expert group statements strongly recommend antiviral therapy in cases of moderate-to-severe disease, especially in the presence of central nervous system involvement. Though differences exist between recommendations in instances of isolated SNHL or SNHL in combination with only mild symptoms, the majority of experts in both groups offered at least a weak recommendation for antiviral treatment. Available results suggest antiviral treatment could therefore benefit a meaningful proportion of newborns referred for cCMV testing following failed newborn hearing screening.
| S-EPMC7510248 | biostudies-literature