Provider Perspectives: Identification and Follow-up of Infants who Are Deaf or Hard of Hearing.

OBJECTIVE: Without timely screening, diagnosis, and intervention, hearing loss can cause significant delays in a child's speech, language, social, and emotional development. In 2019, Texas had nearly twice the average rate of loss to follow-up (LFU) or loss to documentation (LTD; i.e., missing documentation of services received) among infants who did not pass their newborn hearing screening compared to the United States overall (51.1 vs. 27.5%). We aimed to identify factors contributing to LFU/LTD among infants who do not pass their newborn hearing screening in Texas. STUDY DESIGN: Data were collected through semistructured qualitative interviews with 56 providers along the hearing care continuum, including hospital newborn hearing screening program staff, audiologists, primary care physicians, and early intervention (EI) program staff located in three rural and urban public health regions in Texas. Following recording and transcription of the interviews, we used qualitative data analysis software to analyze themes using a conventional content analysis approach. RESULTS: Frequently cited barriers included problems with family access to care, difficulty contacting patients, problems with communication between providers and referrals, lack of knowledge among providers and parents, and problems using the online reporting system. Providers in rural areas more often mentioned problems with family access to care and contacting families compared to providers in urban areas. CONCLUSION: These findings provide insight into strategies that public health professionals and health care providers can use to work together to help further increase the number of children identified early who may benefit from EI services. KEY POINTS: · Infants with suspected hearing loss may not receive timely diagnosis or early intervention.. · We interviewed healthcare providers in Texas along the hearing care continuum.. · Findings suggest strategies to increase the number of children with hearing loss identified early..

Receipt and Timeliness of Newborn Hearing Screening and Diagnostic Services Among Babies Born in 2017 in 9 States.

CONTEXT: By providing timely services at all steps along the continuum of the early hearing detection and intervention (EHDI) process, providers may be able to lessen potential adverse effects of late identification of hearing loss on children's language development. OBJECTIVE: To examine the timeliness of key events in the EHDI process from birth through diagnosis of hearing loss among different populations. DESIGN: Retrospective, cross-sectional. SETTING: Data pooled from 9 states' EHDI information systems were used to determine the extent to which timely screening and diagnosis were achieved by 754 613 infants born in calendar year 2017. Enrollment into early intervention for children diagnosed is not examined here due to incomplete data. PARTICIPANTS: Nine state EHDI programs were selected to participate in this study for their successful experience in using EHDI-IS to collect detailed child-level data. MAIN OUTCOME MEASURES: Age of service, rate of service receipt. RESULTS: Median age of newborn hearing screening was 1 day, and median age of hearing loss diagnosis was 68 days. Early completion of newborn hearing screening was associated with maternal education, maternal race/ethnicity, and admission into a neonatal intensive care unit (NICU). Receiving and completing follow-up diagnostic services were associated with maternal education, maternal race/ethnicity, age of screening, and enrollment into the Women, Infants, and Children program. CONCLUSIONS: Timely completion of the newborn hearing screening is achieved by most of the population among the participating states. Increased efforts may be considered by state EHDI programs to provide additional follow-up and education to underrepresented racial/ethnic groups, mothers with less education, and NICU infants and their families as these groups appear to be at an increased risk for delayed diagnostic testing for hearing loss.

Infants with Congenital Disorders Identified Through Newborn Screening - United States, 2015-2017.

Newborn screening (NBS) identifies infants at risk for congenital disorders for which early intervention has been shown to improve outcomes (1). State public health programs are encouraged to screen for disorders on the national Recommended Uniform Screening Panel (RUSP), which increased from 29 disorders in 2005 to 35 in 2018.* The RUSP includes hearing loss (HL) and critical congenital heart defects, which can be detected through point-of-care screening, and 33 disorders detected through laboratory screening of dried blood spot (DBS) specimens. Numbers of cases for 33 disorders on the RUSP (32 DBS disorders and HL) reported by 50 U.S. state programs were tabulated. The three subtypes of sickle cell disease (SCD) listed as separate disorders on the RUSP (S,S disease; S,beta-thalassemia; and S,C disease) were combined for the current analysis, and the frequencies of the resulting disorders were calculated relative to annual births. During 2015-2017, the overall prevalence was 34.0 per 10,000 live births. Applying that frequency to 3,791,712 live births in 2018,† approximately 12,900 infants are expected to be identified each year with one of the disorders included in the study. The most prevalent disorder is HL (16.5 per 10,000), and the most prevalent DBS disorders are primary congenital hypothyroidism (CH) (6.0 per 10,000), SCD (4.9 per 10,000), and cystic fibrosis (CF) (1.8 per 10,000). Notable changes in prevalence for each of these disorders have occurred since the previous estimates based on 2006 births (2). The number of infants identified at a national level highlights the effect that NBS programs are having on infant health through early detection, intervention, and potential improved health, regardless of geographic, racial/ethnic, or socioeconomic differences.

CDC Grand Rounds: Newborn Screening for Hearing Loss and Critical Congenital Heart Disease.

Newborn screening is a public health program that benefits 4 million U.S. infants every year by enabling early detection of serious conditions, thus affording the opportunity for timely intervention to optimize outcomes (1). States and other U.S. jurisdictions decide whether and how to regulate newborn screening practices. Most newborn screening is done through laboratory analyses of dried bloodspot specimens collected from newborns. Point-of-care newborn screening is typically performed before discharge from the birthing facility. The Recommended Uniform Screening Panel includes two point-of-care conditions for newborn screening: hearing loss and critical congenital heart disease (CCHD). The objectives of point-of-care screening for these two conditions are early identification and intervention to improve neurodevelopment, most notably language and related skills among infants with permanent hearing loss, and to prevent death or severe disability resulting from delayed diagnosis of CCHD. Universal screening for hearing loss using otoacoustic emissions or automated auditory brainstem response was endorsed by the Joint Committee on Infant Hearing in 2000 and 2007* and was incorporated in the first Recommended Uniform Screening Panel in 2005. Screening for CCHD using pulse oximetry was recommended by the Advisory Committee on Heritable Disorders in Newborns and Children in 2010 based on an evidence review† and was added to the Recommended Uniform Screening Panel in 2011.§.

Progress in identifying infants with hearing loss­United States, 2006-2012.

Congenital hearing loss affects one to three of every 1,000 live born infants and negatively impacts children through delayed speech, language, social, and emotional development when undetected. To address this public health issue, jurisdiction-based Early Hearing Detection and Intervention (EHDI) programs are working to ensure all newborns are screened for hearing loss, receive follow-up diagnostic testing (DX) if they do not pass the screening, and are enrolled in early intervention (EI) services if diagnosed with a permanent hearing loss. Although substantial progress has been made in the provision and documentation of services, challenges remain because, unlike screening results, diagnostic test results and enrollment in EI are not consistently reported to the EHDI programs. Therefore, it is difficult for states and territories to know if infants received recommended follow-up services (diagnostic testing and/or EI services), often resulting in infants being classified at either stage as lost to follow-up (LFU)/lost to documentation (LTD). To assess progress toward identifying children with hearing loss and reducing LFU/LTD for DX (LFU/LTD-DX) and EI enrollment (LFU/LTD-EI), CDC analyzed EHDI surveillance data for 2006-2012. Results indicated that the number of jurisdictions reporting data increased from 49 to 57, rates of screening increased from 95.2% to 96.6%, rates of referral from screening decreased from 2.3% to 1.6%, rates of diagnosis among infants not passing their final screening increased from 4.8% to 10.3%, and enrollment in EI among children diagnosed with hearing loss increased from 55.4% to 61.7%, whereas rates for both LFU/LTD-DX and LFU/LTD-EI declined. These findings show sustained progress toward screening, identification, and enrollment in EI as well as highlighting the need for continued improvements in the provision and documentation of EHDI services.

Improved newborn hearing screening follow-up results in more infants identified.

Longitudinal research suggests that efforts at the national, state, and local levels are leading to improved follow-up and data reporting. Data now support the assumption that the number of deaf or hard-of-hearing infants identified through newborn hearing screening increases with a reduction in the number of infants lost to follow-up. Documenting the receipt of services has made a noticeable impact on reducing lost to follow-up rates and early identification of infants with hearing loss; however, continued improvement and monitoring of services are still needed.

Likely Impact of the COVID-19 Pandemic on Newborn Hearing Screening and Follow-up Services in the United States in 2020.

This perspective aims to highlight aspects of the Early Hearing Detection and Intervention (EHDI) newborn hearing screening and follow-up processes that were impacted by the COVID-19 pandemic and considers factors that likely impacted follow-up after failing newborn hearing screening among infants born in the United States during 2020. Efforts to minimize the potential impact of missed or delayed identification of hearing loss in infants and young children will also be discussed to help guide future program improvement activities.

Assessing Impact of COVID-19 Pandemic on Receipt and Timeliness of Newborn Hearing Screening and Diagnostic Services Among Infants Born in Four States.

The study compares receipt and timeliness of newborn hearing screening and follow-up diagnostic services between the pre-pandemic birth cohort and the pandemic birth cohort in four participating states. Findings from this study will help inform state Early Hearing Detection and Intervention (EHDI) programs in the future should a major public health event occur again.

Overview of newborn hearing screening activities in Latin America.

OBJECTIVE: Ascertain the status of early hearing detection and intervention services in Latin America. METHODS: Between June and November 2007, Gallaudet University, in collaboration with the U.S. Centers for Disease Control and Prevention Early Hearing Detection and Intervention Diversity Committee, disseminated a survey to 11 Latin American countries. It included questions about newborn hearing screening (NHS) procedures, the availability of intervention services for infants with hearing loss, and challenges in identifying infants with hearing loss. In addition, a literature review was conducted to help identify the status of NHS efforts in Latin America. RESULTS: Six countries (Chile, Costa Rica, Guatemala, Mexico, Panama, and Uruguay) and one U.S. territory (Puerto Rico) responded to the survey. Responses indicated that efforts to identify infants with hearing loss vary within and across countries in Latin America. In some countries, activities have been implemented at a national level; in others, activities have been implemented at a single hospital or region within a country. Common barriers to implementation of NHS programs include a lack of funding, screening and diagnostic equipment, public awareness, and personnel qualified to work with infants and young children. CONCLUSIONS: In spite of several barriers, NHS programs have been implemented in at least some facilities and regions in Latin America. Additional efforts are needed to expand NHS activities in Latin America.

Frequency of Early Intervention Sessions and Vocabulary Skills in Children with Hearing Loss.

BACKGROUND: A primary goal of early intervention is to assist children in achieving age-appropriate language skills. The amount of intervention a child receives is ideally based on his or her individual needs, yet it is unclear if language ability impacts amount of intervention and/or if an increased frequency of intervention sessions results in better outcomes. The purpose of this study was to determine the relationship between the frequency of early intervention sessions and vocabulary outcomes in young children with hearing loss. METHODS: This was a longitudinal study of 210 children 9 to 36 months of age with bilateral hearing loss living in 12 different states. Expressive vocabulary skills were evaluated using the MacArthur-Bates Communicative Development Inventories. RESULTS: A higher number of intervention sessions reported at the first assessment predicted better vocabulary scores at the second assessment, and more sessions reported at the second assessment predicted better scores at the third assessment. For each increase in the number of sessions reported, there was a corresponding, positive increase in vocabulary quotient. In contrast, children's vocabulary ability at an earlier time point did not predict intervention session frequency at a later point in time. CONCLUSIONS: A significant prospective effect was apparent with more therapy sessions resulting in improved vocabulary scores 9 months later. These findings underscore the importance of early intervention. Pediatricians and other health care professionals can help apply these findings by counseling parents regarding the value of frequent and consistent participation in early intervention.

Reading Proficiency Trends Following Newborn Hearing Screening Implementation.

OBJECTIVES: To investigate trends in population-level school-aged reading scores among students with hearing loss in an urban Colorado school district after implementation of universal newborn hearing screening (UNHS) and Early Hearing Detection and Intervention. METHODS: The final sample included 1422 assessments conducted during the 2000-2001 through 2013-2014 school years for 321 children with hearing loss in grades 3 through 10. Longitudinal hierarchical linear modeling analyses were used to examine reading proficiency (controlling for birth year, grade in school, free and reduced lunch status, additional disability services, and English not spoken in the home). The Colorado Student Assessment Program was administered to students in third through 10th grades throughout the state. The test years chosen included children born before and after implementation of UNHS. RESULTS: After implementation of UNHS, significant longitudinal reading proficiency improvements were observed by birth year and grade overall and for all subgroups. However, gains in reading proficiency were substantially less for children eligible for free and reduced lunch and those with moderate-severe to profound hearing loss. With each succeeding birth cohort and grade, increased numbers of children participated in testing because of improved language skills, with higher proportions identified as proficient or advanced readers. CONCLUSIONS: Notable improvements in reading proficiency after Early Hearing Detection and Intervention implementation were demonstrated, as all groups of children with hearing loss became more likely to achieve proficient and advanced reading levels. On the other hand, some disparities increased, with greater improvements in reading proficiency for children in economically advantaged families.

Newborn hearing screening and follow-up: are children receiving recommended services?

OBJECTIVES: Newborn hearing screening programs have been implemented by all 50 states and most U.S. territories to detect hearing loss in infants and prevent delays in speech, language, and social and emotional development. To monitor progress toward national goals, the Centers for Disease Control and Prevention (CDC) collects data from state and territorial programs. This article summarizes findings from the CDC Early Hearing Detection and Intervention (EHDI) Hearing Screening and Follow-up Survey (HSFS) and provides a summary of recent efforts to identify infants with hearing loss in the U.S. METHODS: The HSFS was sent to representatives of U.S. EHDI programs to gather aggregate screening, diagnostic, intervention, and demographic data for 2005 and 2006. We analyzed these data to evaluate progress toward national goals. RESULTS: In 2005 and 2006, more than 90% of infants were screened for hearing loss. Of these infants, 2% in both years did not pass their final screening. Out of those not passing the final screening, approximately two-thirds were not documented as having a diagnostic finding. In both years, the reason reported for the majority of infants was loss to follow-up/loss to documentation (LFU/LTD). Although the majority of infants with permanent hearing loss were receiving intervention, more than 30% were classified as LFU/LTD and could not be documented as receiving intervention services. CONCLUSIONS: The HSFS enables the collection of more complete data that highlight the progress in screening infants for hearing loss. However, data indicate improvements are needed to reduce LFU/LTD and meet the national benchmarks.

Kindergarten Readiness in Children Who Are Deaf or Hard of Hearing Who Received Early Intervention.

BACKGROUND: Children who are deaf or hard of hearing (D/HH) have improved language outcomes when enrolled in early intervention (EI) before the age of 6 months. Little is understood about the long-term impact of EI on outcomes of kindergarten readiness (K-readiness). The study objective was to evaluate the impact of EI before the age of 6 months (early) versus after 6 months (later) on K-readiness in children who are D/HH. METHODS: In this study, we leveraged data from the Ohio Early Hearing Detection and Intervention Data Linkage Project, which linked records of 1746 infants identified with permanent hearing loss born from 2008 to 2014 across 3 Ohio state agencies; 417 had kindergarten records. The Kindergarten Readiness Assessment was used to identify children as ready for kindergarten; 385 had Kindergarten Readiness Assessment scores available. Multiple logistic regression was used to investigate the relationship between K-readiness and early EI entry while controlling for confounders (eg, hearing loss severity and disability status). RESULTS: Children who were D/HH and entered EI early (n = 222; 57.7% of the cohort) were more likely to demonstrate K-readiness compared with children who entered EI later (33.8% vs 20.9%; P = .005). Children who entered early had similar levels of K-readiness as all Ohio students (39.9%). After controlling for confounders, children who entered EI early were more likely to be ready for kindergarten compared with children who entered later (odds ratio: 2.02; 95% confidence interval 1.18-3.45). CONCLUSIONS: These findings support the sustained effects of early EI services on early educational outcomes among children who are D/HH. EI entry before the age of 6 months may establish healthy trajectories of early childhood development, reducing the risk for later academic struggles.

Early intervention for children with unilateral and mild bilateral degrees of hearing loss.

Some children with unilateral hearing loss or mild bilateral hearing loss are at risk for developmental delays, educational difficulties, and other adverse effects. However, these children face several challenges in receiving services that can prevent such problems. Many do not meet existing eligibility requirements for early intervention services in their state. Information disseminated to professionals and parents often does not convey the significance of unilateral and mild bilateral hearing loss. Some professionals indicate that there are insufficient guidelines defining appropriate intervention services for these children. Factors that influence developmental outcomes in this population are not well understood, making it difficult to determine which children can benefit from intervention services. Additional data are needed about how to minimize or prevent adverse outcomes in these children. This article presents suggestions for intervention and future research that were developed by participants of the 2005 National Workshop on Mild and Unilateral Hearing Loss.

Hearing screening and diagnostic evaluation of children with unilateral and mild bilateral hearing loss.

More than 90% of newborns in the United States are now being screened for hearing loss. A large fraction of cases of unilateral hearing loss and mild bilateral hearing loss are not currently identified through newborn hearing screening. This is of concern because a preponderance of research has demonstrated that unilateral hearing loss and mild bilateral hearing loss can lead to developmental delays and educational problems for some children. To help address this probable underidentification of unilateral hearing loss and mild bilateral hearing loss among infants and children, the Centers for Disease Control and Prevention Early Hearing Detection and Intervention program and the Marion Downs Hearing Center convened a workshop in Breckenridge, Colorado, in July 2005. During this workshop, several issues related to screening and diagnosing unilateral hearing loss and mild bilateral hearing loss were identified, as well as recommendations for future research in this area. Issues identified included the lack of standardized definitions for permanent unilateral hearing loss and mild bilateral hearing loss; the use of screening protocols that are primarily designed to identify bilateral and unilateral hearing losses of a moderate degree or greater (eg, above 40 dB); calibration of screening equipment; availability of facilities that can provide the full range of audiologic, diagnostic, and management services to this pediatric population; and an overall lack of awareness by many professionals and families about the potential effect of unilateral hearing loss and mild bilateral hearing loss. Suggestions for future research, such as identifying ways to improve the identification of cases of unilateral hearing loss and mild bilateral hearing loss, were also discussed.

Measures of follow-up in early hearing detection and intervention programs: a need for standardization.

PURPOSE: To demonstrate the need for standardized data definitions and reporting for early hearing detection and intervention (EHDI) programs collecting information on newborn hearing screening and follow-up, and types of information best collected in a standardized manner. METHOD: A hypothetical birth cohort was used to show the potential effects of nonstandardized definitions and data classifications on rates of hearing screening, audiologic follow-up, and hearing loss. RESULTS: The true screening rate in this cohort was 92.4%. The calculated rate was between 90.0% and 96.5%, depending on the measure used. Among children documented as screened and referred for follow-up, 61.0% received this testing. Only 49.0% were documented to have been tested. Despite a true prevalence of 3.7 per 1,000 births, only 1.5 per 1,000 children were documented with a hearing loss. CONCLUSION: Ensuring that children receive recommended follow-up is challenging. Without complete reporting by audiologists to EHDI programs, accurate calculation of performance measures is impossible. Lack of documentation can lead to the overstatement of "loss to follow-up." Also, standardization of measures is essential for programs to evaluate how many children receive recommended services and assess progress toward national goals. A new survey has been implemented to collect more detailed and standardized information about recommended services.

What Contribution Did Economic Evidence Make to the Adoption of Universal Newborn Hearing Screening Policies in the United States?

Universal newborn hearing screening (UNHS), when accompanied by timely access to intervention services, can improve language outcomes for children born deaf or hard of hearing (D/HH) and result in economic benefits to society. Early Hearing Detection and Intervention (EHDI) programs promote UNHS and using information systems support access to follow-up diagnostic and early intervention services so that infants can be screened no later than 1 month of age, with those who do not pass their screen receiving diagnostic evaluation no later than 3 months of age, and those with diagnosed hearing loss receiving intervention services no later than 6 months of age. In this paper, we first document the rapid roll-out of UNHS/EHDI policies and programs at the national and state/territorial levels in the United States between 1997 and 2005. We then review cost analyses and economic arguments that were made in advancing those policies in the United States. Finally, we examine evidence on language and educational outcomes that pertain to the economic benefits of UNHS/EHDI. In conclusion, although formal cost-effectiveness analyses do not appear to have played a decisive role, informal economic assessments of costs and benefits appear to have contributed to the adoption of UNHS policies in the United States.

Progress in Documented Early Identification and Intervention for Deaf and Hard of Hearing Infants: CDC's Hearing Screening and Follow-up Survey, United States, 2006-2016.

The national EHDI 1-3-6 goals state that all infants should be screened for hearing loss before 1 month of age; with diagnostic testing before 3 months of age for those who do not pass screening; and early intervention (EI) services before 6 months of age for those with permanent hearing loss. This report updates previous summaries of progress on these goals by U.S. states and territories. Data are based on the Hearing Screening and Follow-up Survey (HSFS) conducted annually by the Centers for Disease Control and Prevention for the years 2006-2016. Trends were assessed using 3-year moving averages, with rates of newborns lost to follow-up or lost to documentation (LTF/D) also examined. During this period, the percentage of infants screened before one month increased from 85.1% to 95.3%, while the percentage receiving diagnostic testing before three months increased from 19.8% to 36.6%, and the percentage of infants identified with permanent hearing loss enrolled in early intervention (EI) before six months increased from 25.1% to 47.2%. Percentages of infants who ultimately received screening, diagnostic testing, and early intervention services - regardless of timing - were higher. During this period, LTF/D declined from 42.1% to 31.3% for diagnostic testing, and 39.4% to 20.3% for EI services. Diagnoses of hearing loss recorded increased from 0.9 to 1.7 per 1,000 infants screened, likely reflecting improved data.

Determining the effect of newborn hearing screening legislation: an analysis of state hearing screening rates.

OBJECTIVE: This study was conducted to determine the effect of state Universal Newborn Hearing Screening legislation on the percentage of infants having their hearing screened within one month of birth. METHODS: Hearing screening data for 2000-2003 were obtained from state hearing screening programs. States with Universal Newborn Hearing Screening legislation were categorized according to legislation type and implementation status, and hearing screening rates were compared between states with implemented legislation and states with no legislation. RESULTS: Hearing screening rates among states that implemented Universal Newborn Hearing Screening legislation were significantly higher than rates in no-legislation states throughout the study period, although the mean screening rate among no-legislation states increased substantially from 2000 through 2003. The percentage of states attaining a 95% national screening quality indicator in each year was substantially greater among states with implemented legislation. In 2003, 76% of states with implemented Universal Newborn Hearing Screening legislation reported screening at least 95% of infants, compared with 26% of states without legislation. Although there is a greater likelihood of meeting the national screening target with Universal Newborn Hearing Screening legislation than without, other factors such as collaborative relationships and federal funding can also influence this outcome. CONCLUSION: State legislation has had a positive effect on hearing screening rates and is one tool states can use to help ensure that infants are screened for hearing loss.