Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs

A. Roles and Responsibilities

The success of EHDI programs depends on families working in partnership with professionals as a well-coordinated team. The roles and responsibilities of each team member should be well defined and clearly understood. Essential team members are the birth hospital, families, pediatricians or primary health care professionals (i.e., the medical home), audiologists, otolaryngologists, speech-language pathologists, educators of children who are deaf or hard of hearing, and other early intervention professionals involved in delivering EHDI services ( American Speech-Language-Hearing Association, 1991, 1994). Additional services including genetics, ophthalmology, developmental pediatrics, service coordination, supportive family education, and counseling should be available ( Calderon, Bargones, & Sidman, 1998).

The birth hospital is a key member of the team. The birth hospital, in collaboration with the state EHDI coordinator, should ensure that parents and primary health care professionals receive and understand the hearing screening results, that parents are provided with appropriate follow-up and resource information, and that each infant is linked to a medical home ( AAP Medical Home Initiatives, 2002). The hospital ensures that hearing screening information is promptly transmitted to the medical home and appropriate data are submitted to the state EHDI coordinator.

The most important role for the family of an infant who is deaf or hard of hearing is to love, nurture, and communicate with the baby. From this foundation, families usually develop an urgent desire to understand and meet the special needs of their infant. Families gain knowledge, insight, and experience by accessing resources and through participation in scheduled early intervention appointments including audiologic, medical, habilitative, and educational sessions. This experience can be enhanced when families choose to become involved with parental support groups, individuals who are deaf or hard of hearing, and/or their children's deaf or hard-of-hearing peers. Informed family choices and desired outcomes guide all decisions for these children. A vital function of the family's role is ensuring direct access to communication in the home and the daily provision of language learning opportunities. Over time, the child benefits from the family's modeling of partnerships with professionals and advocating for their rights in all settings. The transfer of responsibilities from families to their child develops gradually and increases as their child matures, growing in independence and self-advocacy.

Pediatricians, family physicians, and other allied health care professionals, working in partnership with parents and other professionals such as audiologists, therapists, and educators, constitute the infant's medical home ( AAP Medical Home Initiatives, 2002). A medical home is defined as an approach to providing health care services where care is accessible, family centered, continuous, comprehensive, coordinated, compassionate, and culturally competent. The primary health care professional acts in partnership with parents in a medical home to identify and access appropriate audiology, intervention, and consultative services needed to develop a global plan of appropriate and necessary health and habilitative care for infants identified with hearing loss and infants with risk factors for hearing loss. All children undergo surveillance for auditory skills and language milestones. The infant's pediatrician, family physician, or other primary health care professional is in a position to advocate for the child and family ( AAP Committee, 2000; AAP Medical Home Initiatives, 2002).

An audiologist is a person who, by virtue of academic degree, clinical training, and license to practice, is qualified to provide services related to the prevention of hearing loss and the audiologic diagnosis, identification, assessment, and nonmedical and nonsurgical treatment of persons with impairment of auditory and vestibular function, and to the prevention of impairments associated with them. Audiologists serve in a number of roles. They provide newborn hearing screening program development, management, quality assessment, service coordination and referral for audiologic diagnosis, and audiologic treatment and management. For the follow-up component, audiologists provide comprehensive audiologic diagnostic assessment to confirm the existence of the hearing loss, ensure that parents understand the significance of the hearing loss, evaluate the infant for candidacy for amplification and other sensory devices and assistive technology, and ensure prompt referral to early intervention programs. For the treatment and management component, audiologists provide timely fitting and monitoring of amplification ( Pediatric Working Group, 1996). Other audiologists may provide diagnostic and auditory treatment and management services in the educational setting and provide a bridge between the child/family and the audiologist in the clinic setting as well as other service providers. Audiologists also provide services as teachers, consultants, researchers, and administrators.

Otolaryngologists are physicians whose specialty includes determining the etiology of hearing loss; identifying related risk indicators for hearing loss, including syndromes involving the head and neck; and evaluating and treating ear diseases. An otolaryngologist with knowledge of childhood hearing loss can determine whether medical and/or surgical intervention may be appropriate. When medical and/or surgical intervention is provided, the otolaryngologist is involved in the long-term monitoring and follow-up with the infant's medical home. The otolaryngologist provides information and participates in the assessment of candidacy for amplification, assistive devices, and surgical intervention, including reconstruction, bone-anchored hearing aids, and cochlear implantation.

Early intervention professionals are trained in a variety of academic disciplines, such as speech-language pathology, audiology, education of children who are deaf or hard of hearing, service coordination, or early childhood special education. All individuals who provide services to infants with hearing loss should have specialized training and expertise in the development of audition, speech, and language. Speech-language pathologists provide both evaluation and intervention services for language, speech, and cognitive-communication development. Educators of children who are deaf or hard of hearing integrate the development of communicative competence within a variety of social, linguistic, and cognitive/academic contexts. Audiologists may provide diagnostic and habilitative services within the individualized family service plan (IFSP) or school-based individualized education plan. To provide the highest quality of intervention, more than one provider may be required.

The care coordinator is an integral member of the EHDI team who facilitates the family's transition from screening to evaluation to early intervention ( AAP Committee, 2005). This individual must be a professional (e.g., social worker, teacher, nurse) who is knowledgeable about hearing loss. The care coordinator incorporates the family's preferences for outcomes into an IFSP as required by federal legislation. The care coordinator supports the family members in their choice of the infant's communicative development. Through the IFSP review, the infant's progress in language, motor, cognitive, and social-emotional development is monitored. The care coordinator assists the family in advocating for the infant's unique developmental needs.

The deaf and hard-of-hearing community includes members with direct experience with signed language, spoken language, hearing aid and cochlear implant use, and other communication strategies and technologies. Optimally, adults who are deaf or hard-of-hearing should play an integral part in the EHDI program. Both adults and children in the deaf and hard-of-hearing community can enrich the family's experience by serving as mentors and role models. Such mentors have experience in negotiating their way in a hearing world, raising infants or children who are deaf or hard of hearing, and providing families with a full range of information about communication options, assistive technology, and resources available in the community.

A successful EHDI program requires collaboration between a variety of public and private institutions and agencies assuming responsibility for specific components (e.g., screening, evaluation, intervention). Roles and responsibilities may differ from state to state. Each state has defined a lead coordinating agency with oversight responsibility. The lead coordinating agency in each state should be responsible for identifying the public and private funding sources available to develop, implement, and coordinate EHDI systems.

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B. Hearing Screening

Multidisciplinary teams of professionals, including audiologists, physicians, and nursing personnel, are needed to establish the UNHS component of EHDI programs. All team members work together to ensure that screening programs are of high quality and are successful. An audiologist should be involved in each component of the hearing screening program, particularly at the level of statewide implementation and, whenever possible, at the individual hospital level. Hospitals and agencies should also designate a physician to oversee the medical aspects of the EHDI program.

Each team of professionals responsible for the hospital-based UNHS program should review the hospital infrastructure in relationship to the screening program. Hospital-based programs should consider screening technology (i.e., OAE or automated ABR testing); validity of the specific screening device; screening protocols, including the timing of screening relative to nursery discharge; availability of qualified screening personnel; suitability of the acoustical and electrical environments; follow-up referral criteria; referral pathways for follow-up; information management; and quality control and improvement. Reporting and communication protocols must be well defined and include the content of reports to physicians and parents, documentation of results in medical records, and methods for reporting to state registries and national data sets.

Physiologic measures must be used to screen newborns and infants for hearing loss. Such measures include OAE and automated ABR testing. Both OAE and automated ABR technologies provide noninvasive recordings of physiologic activity underlying normal auditory function, both are easily performed in neonates and infants, and both have been successfully used for UNHS ( Finitzo, Albright, & O'Neal, 1998; Mason & Hermann, 1998; Norton et al., 2000; Prieve et al., 2000; Vohr, Carty, Moore, & Letourneau, 1998). There are, however, important differences between the two measures. OAE measurements are obtained from the ear canal using a sensitive microphone within a probe assembly that records cochlear responses to acoustic stimuli. Thus, OAEs reflect the status of the peripheral auditory system extending to the cochlear outer hair cells. In contrast, ABR measurements are obtained from surface electrodes that record neural activity generated in the cochlea, auditory nerve, and brainstem in response to acoustic stimuli delivered via an earphone. Automated ABR measurements reflect the status of the peripheral auditory system, the eighth nerve, and the brainstem auditory pathway.

Both OAE and ABR screening technologies can be used to detect sensory (cochlear) hearing loss ( Norton et al., 2000); however, both technologies may be affected by outer or middle ear dysfunction. Consequently, transient conditions of the outer and middle ear may result in a “fail” screening test result in the presence of normal cochlear and/or neural function ( Doyle, Burggraaff, Fujikawa, Kim, & MacArthur, 1997). Moreover, because OAEs are generated within the cochlea, OAE technology cannot be used to detect neural (eighth nerve or auditory brainstem pathway) dysfunction. Thus, infants with neural conduction disorders or auditory neuropathy/dyssynchrony without concomitant sensory dysfunction will not be detected by OAE testing.

Some infants who pass newborn hearing screening will later demonstrate permanent hearing loss ( J. L. Johnson et al., 2005). Although this loss may reflect delayed-onset hearing loss, both ABR and OAE screening technologies will miss some hearing loss (e.g., mild or isolated frequency region losses).

Interpretive criteria for pass/fail outcomes should reflect clear scientific rationale and should be evidence-based ( M. D. Hyde, Sininger, & Don, 1998; M. L. Hyde, Davidson, & Alberti, 1991). Screening technologies that incorporate automated response detection are necessary to eliminate the need for individual test interpretation, to reduce the effects of screener bias or operator error on test outcome, and to ensure test consistency across infants, test conditions, and screening personnel ( Eilers, Miskiel, Ozdamar, Urbano, & Widen, 1991; Herrmann, Thornton, & Joseph, 1995; McFarland, Simmons, & Jones, 1980; Ozdamar, Delgado, Eilers, & Urbano, 1994; Pool & Finitzo, 1989). When statistical probability is used to make pass/fail decisions, as is the case for OAE and automated ABR screening devices, the likelihood of obtaining a pass outcome by chance alone is increased when screening is performed repeatedly ( Benjamini & Yekutieli, 2005; Hochberg & Benjamini, 1990; Zhang, Chung, & Oldenburg, 1999). This principle must be incorporated into the policies of rescreening.

There are no national standards for the calibration of OAE or ABR instrumentation. Compounding this, there is a lack of uniform performance standards. Manufacturers of hearing-screening devices do not always provide sufficient supporting evidence to validate the specific pass/fail criteria and/or automated algorithms utilized in their instruments ( Gravel et al., 2005). In the absence of national standards, audiologists must obtain normative data for the instruments and protocols they use.

The JCIH recognizes that there are important issues differentiating screening performed in the well-baby nursery from that performed in the NICU. Although the goals in each nursery are the same, numerous methodologic and technological issues must be considered in program design and pass/fail criteria.

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C. Confirmation of Hearing Loss in Infants Referred From UNHS

Infants who meet the defined criteria for referral should receive follow-up audiologic and medical evaluations with fitting of amplification devices, as appropriate, no later than 3 months of age. Once hearing loss is confirmed, coordination of services should be expedited by the infant's medical home and Part C coordinating agencies for early intervention services, as authorized by the Individuals with Disabilities Education Act, following the EHDI algorithm developed by the AAP ( Appendix 1; AAP Task Force, 2003).

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D. Early Intervention

Before newborn hearing screening was instituted universally, children with severe to profound hearing loss, on average, completed the 12th grade with a 3rd- to 4th-grade reading level and language levels of a 9- to 10-year-old hearing child ( Traxler, 2000). In contrast, infants and children with mild to profound hearing loss who are identified in the first 6 months of life and provided with immediate and appropriate intervention have significantly better outcomes than later-identified infants and children in vocabulary development ( Mayne, Yoshinaga-Itano, & Sedey, 1998; Mayne, Yoshinaga-Itano, Sedey, & Carey, 1998), receptive and expressive language ( Pipp-Siegel, Sedey, VanLeeuwen, & Yoshinaga-Itano, 2003; Yoshinaga-Itano et al., 1998), syntax ( Yoshinaga-Itano, Coulter, & Thomson, 2001), speech production ( Apuzzo & Yoshinaga-Itano, 1995; Yoshinaga-Itano & Apuzzo, 1998a, 1998b; Yoshinaga-Itano et al., 2000), and social-emotional development ( Yoshinaga-Itano, 2001). Children enrolled in early intervention within the first year of life have also been shown to have language development within the normal range of development at 5 years of age ( Calderon et al., 1998; Moeller, 2000).

Therefore, according to federal guidelines, once any degree of hearing loss is diagnosed in a child, a referral should be initiated to an early intervention program within 2 days of confirmation of hearing loss (CFA 303.321d). The initiation of early intervention services should begin as soon as possible after diagnosis of hearing loss, but no later than 6 months of age. Even when the hearing status is not determined to be the primary disability, the family and child should have access to intervention with a provider knowledgeable about hearing loss ( Kennedy, McCann, Campbell, Kimm, & Thornton, 2005).

UNHS programs have been instituted throughout the United States for the purpose of preventing the significant and negative effects of hearing loss on the cognitive, language, speech, auditory, social-emotional, and academic development of infants and children. To achieve this goal, hearing loss must be identified as quickly as possible after birth, and appropriate early intervention must be available to all families and infants with permanent hearing loss. Some programs have demonstrated that most children with hearing loss and no additional disabilities can achieve and maintain language development within the typical range of children with normal hearing ( Moeller, 2000; Yoshinaga-Itano, Coulter, & Thomson, 2000, 2001; Yoshinaga-Itano et al., 1998). Because these studies are descriptive and not causal studies, the efficacy of specific components of intervention cannot be separated from the total provision of comprehensive services. Thus, the family-centered philosophy, the intensity of services, the experience and training of the provider, the method of communication, the curricula, the counseling procedures, the parent support and advocacy, and the deaf and hard-of-hearing support and advocacy are all variables with unknown effects on the overall outcomes of any individual child. The key component of providing quality services is the expertise of the provider specific to hearing loss. These services may be provided in the home or in a center, or a combination of the two locations.

The term “intervention services” is used to describe any type of habilitative, rehabilitative, or educational program provided to individuals with hearing loss. In some cases with mild hearing losses, amplification technology may be the only service provided. Some parents choose only developmental assessment or occasional consultation, such as parents with infants who have unilateral hearing losses. Children with high-frequency losses and normal hearing in the low frequencies may only be seen by a speech-language pathologist, and those with significant bilateral sensorineural hearing losses might be seen by an educator of the deaf and receive additional services.

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E. Continued Surveillance, Screening, and Referral of Infants and Toddlers

Appendix 1 presents 11 risk indicators associated with either congenital or delayed-onset hearing loss. A single list of risk indicators is presented in the current JCIH statement because there is significant overlap among those indicators associated with congenital/neonatal hearing loss and those associated with delayed-onset/acquired or progressive hearing loss. Heightened surveillance of all infants with risk indicators is, therefore, recommended. There is a significant change in the definition of risk indicator 3, which has been modified from NICU stay >48 hours to NICU stay >5 days. Consistent with JCIH 2000, the 2007 position statement recommends use of risk indicators for hearing loss for three purposes. Historically, the first use of risk indicators is for the identification of infants who should receive audiologic evaluation but who live in geographic locations (e.g., developing nations, remote areas) where universal hearing screening is not yet available ( Barbi et al., 2006; Barrenas, Jonsson, Tuvemo, Hellstrom, & Lundgren, 2005; Cone-Wesson et al., 2000; D'Agostino & Austin, 2004; Davis & Hind, 2003; Fligor et al., 2005; Jacobson & Jacobson, 2004; J. L. Johnson et al., 2005; JCIH, 2000a, 2000b; Mestan, Marks, Hecox, Huo, & Schreiber, 2005; Norton et al., 2000; Robertson, Tyebkhan, Peliowski, Etches, & Cheung, 2006; Vohr et al., 2000). This use has become less common as a result of the expansion of UNHS. The second purpose of risk indicator identification is to help identify infants who pass the neonatal screening but are at risk of developing delayed-onset hearing loss and, therefore, should receive ongoing medical, speech and language, and audiologic surveillance. Third, the risk indicators are used to detect infants who may have passed neonatal screening but have mild forms of permanent hearing loss ( J. L. Johnson et al., 2005).

Because some important indicators, such as family history of hearing loss, may not be determined during the course of UNHS ( Nance, 2003; White, 2003), the presence of all risk indicators for acquired hearing loss should be determined in the medical home during early well-baby visits. Risk indicators that are marked with an asterisk in Appendix 1 are of greater concern for delayed-onset hearing loss. Early and more frequent assessment may be indicated for children with CMV infection ( Barbi et al., 2006; Nance, Lim, & Dodson, 2006; Pass, Fowler, Boppana, Britt, & Stagno, 2006), syndromes associated with progressive hearing loss ( Nance, 2003), neurodegenerative disorders ( Nance, 2003), trauma ( Lew et al., 2004; Vartialnen, Karjalainen, & Karja, 1985; Zimmerman, Ganzel, Windmill, Nazar, & Phillips, 1993), or culture-positive postnatal infections associated with sensorineural hearing loss ( Arditi et al., 1998; Roizen, 2003); for children who have received ECMO ( Fligor et al., 2005) or chemotherapy ( Bertolini et al., 2004); and when there is caregiver concern or a family history of hearing loss ( AAP Committee, 2000).

For all infants with and without risk indicators for hearing loss, developmental milestones, hearing skills, and parent concerns about hearing, speech, and language skills should be monitored during routine medical care consistent with the AAP periodicity schedule.

The JCIH has determined that the previously recommended approach to follow-up of infants with risk indicators for hearing loss only addressed children with identifiable risk indicators and failed to consider the possibility of delayed-onset hearing loss in children without identifiable risk indicators. In addition, concerns were raised about feasibility and cost associated with the JCIH 2000 recommendation for audiologic monitoring of all infants with risk indicators at 6-month intervals. Because approximately 400,000 infants are cared for annually in NICUs in the United States, and the JCIH 2000 recommendation included audiology assessments at 6-month intervals from 6 months to 36 months of age for all infants admitted to a NICU for >48 hours, an unreasonable burden was placed on both providers of audiology services and families. In addition, there was no provision for identification of delayed-onset hearing loss in infants without an identifiable risk indicator. Data from 2005 for 12,388 infants discharged from NICUs in the National Perinatal Information Network indicate that 52% of infants were discharged within the first 5 days of life and these infants were significantly less likely to have an identified risk indicator for hearing loss other than NICU stay. Therefore, the JCIH 2007 recommends an alternative, more inclusive strategy of surveillance of all children within the medical home based on the pediatric periodicity schedule. This protocol will permit the detection of children with either missed neonatal or delayed-onset hearing loss, irrespective of the presence or absence of a high-risk indicator.

The JCIH recognizes that an optimal surveillance and screening program within the medical home would include the following:

• At each visit, consistent with the AAP periodicity schedule, infants should be monitored for auditory skills, middle ear status, and developmental milestones (surveillance). Concerns elicited during surveillance should be followed by administration of a validated global screening tool ( AAP Council on Children, 2006). A validated global screening tool is administered at 9, 18, and 24 to 30 months to all infants or, if there is physician or parental concern about hearing or language, sooner ( AAP Council on Children, 2006).

• If an infant does not pass the speech-language portion of the global screening in the medical home or if there is physician or caregiver concern about hearing or spoken language development, the child should be referred immediately for further evaluation by an audiologist and a speech-language pathologist for a speech and language evaluation with validated tools ( AAP Council on Children, 2006).

• Once hearing loss is diagnosed in an infant, siblings who are at increased risk of having hearing loss should be referred for audiologic evaluation ( Fortnum & Davis, 1997; Nance & Kearsey, 2004; Orzan et al., 1999; White, 2003).

• All infants with a risk indicator for hearing loss ( Appendix 1), regardless of surveillance findings, should be referred for an audiologic assessment at least once by 24 to 30 months of age. Children with risk indicators highly associated with delayed-onset hearing loss, such as having received ECMO or having CMV infection, should have more frequent audiologic assessments.

• All infants for whom the family has significant concerns regarding hearing or communication should be promptly referred for an audiologic and speech-language assessment.

• A careful assessment of middle ear status (using pneumatic otoscopy and/or tympanometry) should be completed at all well-child visits, and children with persistent middle ear effusion lasting 3 months or longer should be referred for otologic evaluation ( AAP Subcommittee, 2004).

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F. Protecting the Rights of Infants and Families

Each agency or institution involved in the EHDI process shares responsibility for protecting infant and family rights in all aspects of UNHS, including access to information about potential benefits and risks in the family's native language, input into decision making, and confidentiality ( NIDCD, 1999). Families should receive information about childhood hearing loss in easily understood language. Families have the right to accept or decline hearing screening or any follow-up care for their newborn infant within the statutory regulations, just as they have for any other screening or evaluation procedures or intervention.

EHDI data merit the same level of confidentiality and security afforded all other health care and education information in practice and law. The infant's family has the right to confidentiality of the screening and follow-up assessments and the acceptance or rejection of suggested intervention(s). In compliance with federal and state laws, mechanisms should be established that ensure parental release and approval of all communications regarding the infant's test results, including those to the infant's medical home and early intervention coordinating agency and programs. The Health Insurance Portability and Accountability Act (Pub. L. No. 104-191 [1996]) regulations permit the sharing of health information among health care professionals.

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G. Information Infrastructure

In the 2000 position statement, the JCIH recommended development of uniform state registries and national information databases incorporating standardized methodology, reporting, and system evaluation ( JCIH, 2000a, 2000b). EHDI information systems are to provide for the ongoing and systematic collection, analysis, and interpretation of data in the process of measuring and reporting associated program services (e.g., screening, evaluation, diagnosis, and/or intervention). These systems are used to guide activities, planning, implementation, and evaluation of programs and to formulate research hypotheses.

EHDI information systems are generally authorized by legislators and implemented by public health officials. These systems vary from a simple system collecting data from a single source to electronic systems that receive data from many sources in multiple formats. The number and variety of systems will likely increase with advances in electronic data interchange and integration of data, which will also heighten the importance of patient privacy, data confidentiality, and system security. Appropriate agencies and/or officials should be consulted for any projects regarding public health surveillance ( CDC, 2003).

Federal and state agencies are collaborating in the standardization of data definitions to ensure the value of data sets and to prevent misleading or unreliable information. Information management is used to improve services to infants and their families; to assess the quantity and timeliness of screening, evaluation, and enrollment into intervention; and to facilitate collection of demographic data on neonatal and infant hearing loss.

The JCIH endorses the concept of a limited national database to permit documentation of the demographics of neonatal hearing loss, including prevalence and etiology across the United States. The information obtained from the information management system should assist both the primary health care professional and the state health agency in measuring quality indicators associated with program services (e.g., screening, diagnosis, and intervention). The information system should provide the measurement tools to determine the degree to which each process is stable and sustainable and conforms to program benchmarks. Timely and accurate monitoring of relevant quality measures is essential.

Since 1999, the CDC and the Directors of Speech and Hearing Programs in State Health and Welfare Agencies (DSHPSHWA) have collected annual aggregate EHDI program data needed to address the national EHDI goals. In 1999, a total of 22 states provided data for the DSHPSHWA survey. Participation had increased to 48 states, 1 territory, and the District of Columbia in 2003. However, many programs have been unable to respond to all the questions on the survey because of lack of a statewide comprehensive data-management and reporting system.

The Government Performance and Results Act (GPRA) of 1993 (Pub. L. No. 103-62) requires that federal programs establish measurable goals approved by the U.S. Office of Management and Budget (OMB) that can be reported as part of the budgetary process, thus linking future funding decisions with performance. The HRSA has modified its reporting requirements for all grant programs. The following GPRA measures must be reported to the OMB by the MCHB annually for the EHDI program:

• the number of infants screened for hearing loss before discharge from the hospital;

• the number of infants with a confirmed with hearing loss no later than 3 months of age;

• the number of infants enrolled in a program of early intervention no later than 6 months of age;

• the number of infants with confirmed or suspected hearing loss referred to an ongoing source of comprehensive health care (i.e., medical home);

• the number of children with nonsyndromic hearing loss who have developmentally appropriate language and communication skills at school entry.

The following is one GPRA measure that must be reported to the OMB by the CDC annually for the EHDI program:

• the percentage of newborn infants with a positive screening result for hearing loss who are subsequently lost to follow-up.

EHDI programs have made tremendous gains in their ability to collect, analyze, and interpret data in the process of measuring and reporting associated program services. However, only a limited number of EHDI programs are currently able to accurately report the number of infants screened, evaluated, and enrolled in intervention, the age of time-related objectives (e.g., screening by 1 month of age), and the severity or laterality of hearing loss. This is complicated by the lack of data standards and by privacy issues within the regulations of the Family Educational Rights and Privacy Act of 1974 (Pub. L. No. 93-380).

Given the current lack of standardized and readily accessible sources of data, the CDC EHDI program, in collaboration with the DSHPSHWA, developed a revised survey to obtain annual EHDI data from states and territories in a consistent manner to assess progress toward meeting the national EHDI goals and the Healthy People 2010 Objectives. In October 2006, the OMB, which is responsible for reviewing all government surveys, approved the new Early Hearing Detection and Intervention Hearing Screening and Follow-Up Survey. To facilitate this effort, the CDC EHDI Data Committee is establishing the minimum data elements and definitions needed for information systems to be used to assess progress toward the national EHDI goals.

The JCIH encourages the CDC and HRSA to continue their efforts to identify barriers and explore possible solutions with EHDI programs to ensure that children in each state who seek hearing-related services in states other than where they reside receive all recommended screening and follow-up services. EHDI systems should also be designed to promote the sharing of data regarding early hearing loss through integration and/or linkage with other child health information systems. The CDC currently provides funds to integrate the EHDI system with other state/territorial screening, tracking, and surveillance programs that identify children with special health care needs. Grantees of the MCHB are encouraged to link hearing screening data with such child health data sets as electronic birth certificate, vital statistics, birth defects registries, metabolic or newborn dried “blood spot” screening, immunization registries, and others.

To promote the best use of public health resources, EHDI information systems should be periodically evaluated, and such evaluations should include recommendations for improving quality, efficiency, and usefulness. The appropriate evaluation of public health surveillance systems becomes paramount as these systems adapt to revise case definitions, address new health-related events, adopt new information technology, ensure data confidentiality, and assess system security ( CDC, 2003).

Currently, federal sources of systems support include Title V block grants to states for maternal and child health care services, Title XIX (Medicaid) federal and state funds for eligible children, and competitive U.S. Department of Education personnel preparation and research grants. The NIDCD provides grants for research related to early identification and intervention for children who are deaf or hard of hearing ( Roush et al., 2004).

Universities should assume responsibility for special-track, interdisciplinary, professional education programs for early intervention for infants and children with hearing loss. Universities should also provide training in family systems, the grieving process, cultural diversity, auditory skill development, and deaf culture. There is a critical need for in-service and preservice training of professionals related to EHDI programs, which is particularly acute for audiologists and early interventionists with expertise in hearing loss. This training will require increased and sustained funding for personnel preparation.

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H. Benchmarks and Quality Indicators

The JCIH supports the concept of regular measurements of performance and recommends routine monitoring of these measures for interprogram comparison and continuous quality improvement. Performance benchmarks represent a consensus of expert opinion in the field of newborn hearing screening and intervention. The benchmarks are the minimal requirements that should be attained by high-quality EHDI programs. Frequent measures of quality permit prompt recognition and correction of any unstable component of the EHDI process ( Agency for Health Care Policy and Research, 1995).

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Challenges

All of the following listed challenges are considered important for the future development of successful EHDI systems:

• Too many children are lost between the failed screening and the rescreening and between the failed rescreening and the diagnostic evaluation.

• There is a shortage of professionals with skills and expertise in both pediatrics and hearing loss, including audiologists, deaf educators, speech-language pathologists, early intervention professionals, and physicians.

• There is often a lack of timely referral for diagnosis of, and intervention for, suspected hearing loss in children.

• Consistent and stable state and federal funding is needed for program sustainability.

• When compared with services provided for adults, pediatric services in all specialties are poorly reimbursed.

• Access to uniform Part C services is inadequate among states and within states.

• There is a lack of integrated state data-management and tracking systems.

• Demographics and cultural diversity are rapidly changing.

• Funding for hearing aids, loaner programs, cochlear implants, and FM systems is needed.

• There is a lack of specialized services for children with multiple disabilities and hearing loss.

• Children may not qualify for services (state Part C guidelines) before demonstrating language delays (prevention model vs. deficit model).

• Children may not qualify for assistive technology (prevention model vs. deficit model).

• There is a lack of in-service education for key professionals.

• There are regulatory barriers to sharing information among providers and among states.

• No national standards exist for the calibration of OAE or ABR instrumentation, and there is a lack of uniform performance standards.

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Opportunities for System Development and Research

• Establish programs to ensure the development of communication for infants and children with all degrees and types of hearing loss, allowing them access to all educational, social, and vocational opportunities throughout their life span.

• Develop improved, rapid, reliable screening technology designed to differentiate specific types of hearing loss.

• Develop and validate screening technologies for identifying minimal hearing loss.

• Develop state data-management systems with the capacity for the accurate determination of the prevalence for delayed-onset or progressive hearing loss.

• Develop state data-tracking systems to follow infants with suspected and confirmed hearing loss through individual state EHDI programs.

• Track the certification credentials of the service providers for children with confirmed hearing loss receiving Part C early intervention services and early childhood special education.

• Track genetic, environmental, and pharmacologic factors that contribute to hearing loss, thus allowing for tailored prevention and intervention strategies.

• Continue to refine electrophysiologic diagnostic techniques, algorithms, and equipment to enable frequency-specific threshold assessment for use with very young infants.

• Continue to refine techniques to improve the selection and fitting of appropriate amplification devices in infants and young children.

• Conduct translational research pertaining to young children with hearing loss, in particular, genetic, diagnostic, and outcomes studies.

• Initiate prospective population-based studies to determine the prevalence and natural history of auditory neural conduction disorders.

• Conduct efficacy studies to determine appropriate early intervention strategies for infants and children with all degrees and types of hearing loss.

• Conduct additional studies on the efficacy of intervention for infants and children who receive cochlear implants younger than 2 years.

• Conduct additional studies on the efficacy of hearing aid use in infants and children younger than 2 years.

• Conduct additional studies of the auditory development of children who have appropriate amplification devices in early life.

• Expand programs within health, social service, and education agencies associated with early intervention and Head Start programs to accommodate the needs of the increasing numbers of early-identified children.

• Adapt education systems to capitalize on the abilities of children with hearing loss who have benefited from early identification and intervention.

• Develop genetic and medical procedures that will determine more rapidly the etiology of hearing loss.

• Ensure transition from Part C (early intervention) to Part B (education) in ways that encourage family participation and ensure minimal disruption of child and family services.

• Study the effects of parents' participation in all aspects of early intervention.

• Test the utility of a limited national dataset and develop nationally accepted indicators of EHDI system performance.

• Encourage the identification and development of centers of expertise where specialized care is provided in collaboration with local service providers.

• Obtain the perspectives of individuals who are deaf or hard of hearing in developing policies regarding medical and genetic testing and counseling for families who carry genes associated with hearing loss ( Brick, 1999).