Am Fam Physician. 2010 Jan 15;81(2):124-126.
In the United States, approximately 12,000 infants per year (or one to three per 1,000 newborns) are born deaf or hard of hearing, making hearing loss one of the most common potentially disabling conditions present at birth.1 Exposure to visual or spoken language is vital during infancy and early childhood. Without full exposure to language and early intervention services, children with late-onset or undiagnosed hearing loss, including mild and unilateral loss, can experience considerable delays in development of language, social, and academic skills.2,3
The Early Hearing Detection and Intervention (EHDI) program is a national initiative promoting identification of hearing loss among infants and young children, and facilitating enrollment in early intervention services that help ensure language development.
The objectives in Healthy People 2010 support this initiative by calling for an increase in the proportion of newborns who are screened for hearing loss before one month of age, have an audiologic evaluation before three months of age, and are enrolled in appropriate intervention services before six months of age.4 Without EHDI programs, the average age of identification for hearing loss is approximately 30 months, which is past the start of the critical period for optimal language acquisition.5
Composed of representatives from the fields of otolaryngology, pediatrics, audiology, speech-language pathology, deaf education, and deaf advocacy, the Joint Committee on Infant Hearing (JCIH) has been influencing EHDI program policy since 1969 and providing internationally recognized clinical practice recommendations. In 2007, the JCIH released its current position statement outlining the principles of the EHDI program, which include recommendations that expand on the Healthy People 2010 objectives. Updates for screening, diagnostic, medical, early intervention, surveillance, and infrastructure recommendations were also included in the statement.6
In July 2008, the U.S. Preventive Services Task Force (USPSTF) revised its recommendations on universal newborn hearing screening. Based on an increasing body of evidence from outcome studies of children with early identification of hearing loss, the USPSTF now recommends hearing screening for all newborns.7
In 2006, hearing screening was documented for more than 90 percent of infants born in the United States.8 However, newborn screening is only the first step in the EHDI process. Diagnosis and provision of early intervention services are essential for the care of infants and children with hearing loss. Despite their importance, adequate documentation of timely diagnosis and initiation of effective intervention does not exist for many of these children.9 In 2006, nearly two thirds of infants who did not pass their hearing screening did not have a documented diagnosis reported to their state EHDI program. Additionally, documentation of receiving Part C intervention services (i.e., services for infants and toddlers provided by states through the Individuals with Disabilities Education Improvement Act of 2004) existed for only one half of infants diagnosed with hearing loss.8,10 These numbers represent infants who might not be receiving necessary services and are at risk of language delays.
In its 2007 statement, the JCIH highlighted “pediatricians, family physicians, and other allied health care professionals” as constituting the medical home of infants and children with hearing loss.6 The JCIH's recommended guidelines outlined roles and responsibilities of the medical home that will help ensure children with hearing loss receive the follow-up services that are critical to their care. The guidelines also emphasized that health care professionals within the medical home should work as partners with parents of infants identified with any degree of hearing loss. Together, they can identify and access appropriate services to develop a global plan of necessary health and habilitative services. These services include audiologic and early intervention services, as well as referrals to an otolaryngologist, geneticist, and ophthalmologist.6
For family physicians, a commitment to become the medical home for these infants and their families is no small task. It requires a collegial interaction with audiologists, interventionists, educators, and various physician consultants. It also requires an awareness of one's own knowledge gaps in the rapidly evolving medical field and a willingness to broaden a commitment to “just-in-time” learning. Additionally, it requires a sense of urgency in guiding families through timely rescreening, completion of audiologic diagnostic testing, and initiation of appropriate intervention—all within the first few months of life.
These actions are critical to the language development of newborns who are deaf or hard of hearing. The availability of new screening technology brings the responsibility of ensuring that appropriate steps are taken for affected infants and their families.
More information on newborn hearing screening and the medical home, as well as billing and legislation, is available at http://www.medicalhomeinfo.org/screening/hearing.html.
Address correspondence to Krista R. Biernath, MD, FAAP, at firstname.lastname@example.org. Reprints are not available from the authors.
Author disclosure: Nothing to disclose.
The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention.
REFERENCESshow all references
1. Finitzo T, Albright K, O'Neal J. The newborn with hearing loss: detection in the nursery. Pediatrics. 1998;102(6):1452–1460....
2. Lieu JE. Speech-language and educational consequences of unilateral hearing loss in children. Arch Otolaryngol Head Neck Surg. 2004;130(5):524–530.
3. Holden-Pitt L, Albertorio J. Thirty years of the Annual Survey of Deaf and Hard-of-Hearing Children & Youth: a glance over the decades. Am Ann Deaf. 1998;143(2):72–76.
4. U.S. Department of Health and Human Services. Healthy People 2010: Understanding and Improving Health. 2nd ed. Washington, DC: U.S. Government Printing Office, 2000.
5. Centers for Disease Control and Prevention. Infants tested for hearing loss—United States, 1999–2001 [published correction appears in MMWR Morb Mortal Wkly Rep. 2003;42(49):1210]. MMWR Morb Mortal Wkly Rep. 2003;52(41):981–984.
6. Joint Committee on Infant Hearing. Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics. 2007;120(4):898–921.
7. U.S. Preventive Services Task Force. Universal screening for hearing loss in newborns: U.S. Preventive Services Task Force recommendation statement. Pediatrics. 2008;122(1):143–148.
8. Centers for Disease Control and Prevention. Summary of 2006 national EHDI data. http://www.cdc.gov/ncbddd/ehdi/documents/EHDI_Summ_2006_Web.pdf. Accessed March 17, 2009.
9. Mason CA, Gaffney M, Green DR, Grosse SD. Measures of follow-up in early hearing detection and intervention programs: a need for standardization. Am J Audiol. 2008;17(1):60–67.
10. Individuals with Disabilities Education Improvement Act of 2004, 20 USC 1400 (2004).
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