"To ensure that all children whose hearing is defective have the best possible chance of remedial treatment, the writers are convinced that all babies should be given screening tests of hearing, by the ninth to twelfth month." (Ewing & Ewing, 1958)
We have come a long way since those words were penned 50 years ago by these esteemed advocates for children who are deaf. How amazed these early pioneers of infant screening and early intervention would be to know that we now have the ability to screen the hearing of newborns. Today, 96% of newborns in the United States receive hearing screening (National Center for Hearing Assessment & Management, 2009).
Clearly we have a lot to celebrate, but much remains to be done. Beyond newborn hearing screening looms the formidable task of linking all children who have hearing loss, regardless of their place of residence or the circumstances of their birth, with diagnostic and intervention services in a timely and culturally sensitive manner. Only 17% of state early hearing detection and intervention (EHDI) programs link with other data systems (e.g., vital statistics, metabolic, immunizations; White, 2008).
A successful EHDI program can be achieved only when resources (for example, comprehensive medical and audiologic follow-up and quality early intervention programs) and information technologies (such as data management systems to document receipt of diagnosis, enrollment in intervention, and developmental outcomes) close the gaps in the current system. The goal of a comprehensive EHDI program available to all children with hearing loss is worthy and attainable.
In 2008 the Centers for Disease Control and Prevention (CDC) reported newborn hearing screening data from 45 states, two territories, and the District of Columbia based on a 2006 survey of state EHDI coordinators. Almost half (46.3%) of the infants born in 2006 who did not pass their final newborn hearing screen did not complete follow-up and were categorized as lost to follow-up/lost to documentation (LTF/LTD). Jurisdictions reported a range of 1%–99% of infants documented as having received diagnostic evaluations. Of those reported as receiving an audiologic evaluation, only 47% could be documented as having been seen before 3 months of age. Furthermore, only 49% of infants with diagnosed hearing loss were documented as enrolled in Individuals With Disabilities Act (IDEA) Part C Early Intervention or as having received other early intervention services.
We can presume that some children actually did receive follow-up services but were LTD; some families were offered services but declined; and other families were not offered services for a variety of reasons—for example, because treatment was deferred to a later time for a child with recurrent otitis media, or because treatment was not recommended for a child with minimal or unilateral hearing loss.
Regardless of why children were not documented as having received follow-up, it is clear that states vary greatly in the ability to document the linkage of infants who have not passed screenings to diagnostic services and the linkage of infants diagnosed with hearing loss to intervention services. State EHDI coordinators report three factors affecting these high LTF/LTD rates: poor communication between EHDI personnel and families, lack of data management and tracking systems, and lack of facilities and well-trained personnel (White, 2003).
Communication Between EHDI Personnel and Families
Most parents learn of newborn screening programs while in the hospital for the birth of their child—but not before. Parents of infants going through the newborn hearing screening process as well as parents of children with hearing loss prefer learning about the screening process before their child's birth and want user-friendly patient education materials available (Arnold et al., 2006). Furthermore, parents whose infants have gone through a newborn hearing screening program want to be present during the screening but noted that they were not given the opportunity (MacNeil, Liu, Stone, & Farrell, 2007).
Screening results are often provided by technicians who have little knowledge of hearing loss. In an attempt to reduce anxiety in mothers, these untrained personnel often suggest that referrals in newborn screening programs are a common occurrence and they "should not worry about it" (Tharpe, 1999). Clearly, if parents are not educated about the importance of the full evaluation following a failure of the screening, the program loses its efficacy.
Communication with families should not only provide complete and accurate information, but should always honor the culture and values of families (ASHA, 2008a; Marge, D. K., & Marge, M., 2005). When resources are made available to families during times of stress (e.g., during diagnosis of hearing loss), these resources can help reduce a family's stress and, ultimately, improve the social-emotional development of children with hearing loss within their families (Hintermair, 2008).
Data Management and Tracking Systems
Data management systems must have the capacity to report individual infants who receive re-screenings, diagnostic audiologic evaluations, and intervention services. The primary goal of infant screening is to provide timely intervention to all infants with hearing loss. Without the technology to collect and analyze EHDI data, there are no means to measure the benefits and limitations of programs.
The 2007 Joint Committee on Infant Hearing (JCIH) Position Statement describes the Government Performance and Results Act of 1993 (Pub L No. 103-62). That law requires federal programs to establish measurable goals approved by the U.S. Office of Management and Budget that can be reported as part of the budgetary process, thus linking future funding decisions with performance. As such, in addition to data on numbers of infants with confirmed hearing loss and those enrolled in intervention services, the federal Maternal and Child Health Bureau must annually report the percentage of newborn infants with a positive screening result for hearing loss who are subsequently lost to follow-up.
At this time, only a limited number of EHDI programs are able to report accurately the number of infants screened, evaluated, and enrolled in intervention (Marge, D. K., & Marge, M., 2005). As stated so clearly by Muir Gray, program director for the United Kingdom National Screening Committee, "The needs of the population are usually described by public health professionals, but they are often hindered by the lack of program budgeting and the dearth of good-quality information about the incidence or prevalence of disease in a local population" (2004). Our ability to report screening and follow-up data accurately is critical to maintain sufficient funding levels and, thus, to our ability to link infants to high-quality services.
EHDI personnel working with families should have expertise in working with infants and young children who are deaf or hard of hearing (ASHA, 2006; Marge, D. K., & Marge, M., 2005). JCIH has identified a critical need for pre-service and in-service training of professionals—especially audiologists and early interventionists with expertise in hearing loss—related to EHDI activities. Universities are expected to provide interdisciplinary professional education programming specific to EHDI concerns including family systems, the grieving process, cultural diversity, auditory skill development, and Deaf culture (JCIH, 2007).
Examination of the Evidence
Concerned about the high LTF/LTD rates reported by state newborn hearing screening programs, the CDC and ASHA joined forces in early 2008 to examine ways of reducing the number of babies LTF after identification by newborn hearing screening programs. A joint Working Group on Loss to Follow-Up was formed to develop a technical report on the topic based on a systematic review of the evidence conducted by the ASHA National Center for Evidence-Based Practice in Communication Disorders (see sidebar).
State EHDI coordinators also have identified inadequate reimbursement for diagnosis as an obstacle to linking infants with services (White, 2003). Additional barriers have been cited including privacy regulations (NCHAM, 2008), specific family and child characteristics (Folsom et al., 2000; Lui et al., 2008), and proximity to resources (MacNeil et al., 2007).
Other gaps in the EHDI system include a lack of follow-up of children at risk for progressive or late-onset hearing loss who passed their newborn screening. More than 39% of hospitals do not systematically collect data on hearing-risk indicators for newborns. Furthermore, only two of 51 jurisdictions reported that their EHDI programs were responsible for preschool screenings (NCHAM, 2004). The lack of follow-up for at-risk children, coupled with a lack of systematic preschool hearing screenings, creates another gap in the system for preschool children with hearing loss.
Children with hearing loss, with their family's support, can achieve their language, academic, and psychosocial potential when they receive timely and appropriate early intervention services. Success is within reach. However, the value of newborn hearing screening programs is moderated by the extent of their effective linkage to these services.
Where should we focus our resources? It remains unclear which babies are at greatest risk for LTF and how to lower that risk. There is a dearth of evidence to support effective and reliable models of follow-up in EHDI programs. Establishing research priorities in EHDI with requisite funding will lead to the evidence-based recommendations needed to establish explicit practice guidelines that can reduce the gaps in the current system and link all children with hearing loss to high-quality services.