Early Hearing Detection and Intervention

December 2007

Brandt Culpepper, PhD, CCC-A

Updates in the Year 2007 Position Statement of the Joint Committee on Infant Hearing: Principles and Guidelines for Early Hearing Detection and Intervention Programs

In October 2007 the Joint Committee on Infant Hearing (JCIH) issued its eighth position statement. The JCIH continues to endorse the early hearing detection and intervention (EHDI) for infants with hearing loss with the overall goal of maximizing the linguistic competence and literacy development for infants and young children who are deaf or hard of hearing. The 2007 statement continues to follow the "1-3-6 EHDI Plan" as outlined in the first three of the eight principles presented in the position statement. These principles are:

All infants should have access to hearing screening using a physiologic measure at no later than 1 month of age.
All infants who do not pass the initial hearing screening and the subsequent rescreening should have appropriate audiological and medical evaluations to confirm the presence of hearing loss at no later than 3 months of age.
All infants with confirmed permanent hearing loss should receive early intervention services as soon as possible after diagnosis but at no later than 6 months of age. A simplified, single point of entry into an intervention system that is appropriate for children with hearing loss is optimal.
The EHDI system should be family centered with infant and family rights and privacy guaranteed through informed choice, shared decision-making, and parental consent in accordance with state and federal guidelines. Families should have access to information about all intervention and treatment options and counseling regarding hearing loss.
The child and family should have immediate access to high-quality technology including hearing aids, cochlear implants, and other assistive devices when appropriate.
All infants and children should be monitored for hearing loss in the medical home. Continued assessment of communication development should be provided by appropriate professionals to all children with or without risk indicators for hearing loss.
Appropriate interdisciplinary intervention programs for infants with hearing loss and their families should be provided by professionals who are knowledgeable about childhood hearing loss. Intervention programs should recognize and build on strengths, informed choices, traditions, and cultural beliefs of the families.
Information systems should be designed and implemented to interface with electronic health charts and should be used to measure outcomes and report the effectiveness of EHDI services at the patient, practice, community, state, and federal levels (JCIH, 2007, pp. 900–901).

Wisely, key updates from the previous position statement (JCIH, 2000) are presented in the initial pages of the 2007 manuscript. The primary updates are broken down into eight categories: (1) the definition of hearing loss, (2) hearing screening and rescreening protocols, (3) audiologic evaluation, (4) medical evaluation, (5) early intervention, (6) surveillance and screening in the medical home, (7) communication, and (8) information infrastructure. Stakeholders in EHDI systems at all levels are encouraged to review the JCIH recommendations and guidelines, determine which aspects of their program need modifying to meet the recommendations, and determine the action steps necessary within their program to address the modifications needed to meet the new guidelines. A link to a checklist of questions highlighting the JCIH 2007 recommendations is provided at the end of this article to assist in determining where program modifications are needed.

One update that has an impact on hospitals with neonatal intensive care units (NICUs) in particular is the expanded definition of hearing loss . In addition to targeting infants with unilateral or bilateral congenital sensory hearing loss or permanent conductive hearing loss, neural hearing loss (auditory neuropathy/dyssynchrony) is now included in the definition. As such, it is recommended that separate hearing screening protocols be used for newborns in well-baby nurseries and NICUs. Since research has indicated that infants from NICUs are at the highest risk of having a neural hearing loss (D'Agostino & Austin, 2004; Sininger, Hood, Starr, Berlin, & Picton, 1995; Starr, Sininger, & Pratt, 2000), the JCIH now recommends that all infants who stay longer than 5 days in the NICU be screened for neural hearing loss. That is, since otoacoustic emissions alone are not sufficient to identify neural hearing loss, automated auditory brainstem response (AABR) measures need to be included as a part of the hearing screening protocol in the NICU.

The JCIH 2007 statement also recognizes that the current physiologic screening technologies are effective in identifying congenital hearing loss of moderate or greater degree (Norton et al., 2000). Milder degrees of hearing loss or hearing loss in an isolated frequency region, however, may not be detected (Cone-Wesson et al., 2000; Johnson et al., 2005). The JCIH therefore encourages that interpretive criteria for pass/fail outcomes used in screening programs be evidence based using clear scientific rationale (Hyde, Davidson, & Alberti, 1991; Hyde, Sininger, & Don, 1998). Using hearing screening devices that incorporate automated response detection will eliminate the need for individual interpretation of results, reduce the effects of screener bias or operator error on screening outcomes, and will 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). As there are no national standards for calibration in OAE or ABR instrumentation, audiologists must obtain normative data for the instruments and protocols that are used in hearing screening programs.

A second key update rests in the recognition that UNHS programs cannot identify a late onset or progressive hearing loss. Similarly, not all infants and young children who will later develop a permanent hearing loss will have a risk indicator at birth. JCIH 2007 therefore 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, regardless of the presence or absence of a high-risk indicator. The burden of ongoing surveillance of infants and young children with risk indicators for hearing loss is therefore proposed to be shared by EHDI systems and the medical home providers.

The JCIH proposes that an optimal surveillance and screening program within the medical home would allow that infants and young children 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 (American Academy of Pediatrics [AAP], 2006). A validated global screening tool is administered at 9, 18, and 24–30 months (consistent with the AAP periodicity schedule) to all infants or, if there is physician or parental concern about hearing or language, sooner. Infants who do not pass the speech-language portion of the global screening, or for whom there is concern about hearing or spoken language development, should be referred immediately for further evaluation by an audiologist and a speech-language pathologist. Siblings of infants and young children with confirmed permanent hearing loss should be referred for audiologic evaluation, and all infants with a risk indicator for hearing loss, regardless of surveillance findings, should be referred for an audiologic assessment at least once by 24 to 30 months of age. More frequent assessments should be established for infants at greater risk for developing a hearing loss, such as those with cytomegalovirus (CMV) infections or those who received extracorporeal membrane oxygenation (ECMO).

The Early Hearing Detection and Intervention Program Checklist [PDF] may be used to determine how well EHDI programs are meeting the JCIH recommendations. The categories JCIH designated as having key updates in the 2007 Position Statement define the areas for examining existing programs. For each "No" answer, a review of that program component is suggested to determine the feasibility of modifying the current program to reach the recommended guidelines.

About the Author

Brandt Culpepper, PhD, CCC-A, works at Northside Hospital as a pediatric audiologist. Northside Hospital holds the honor of having the highest annual birth rate in the nation, with more than 18,000 babies born there each year. The Audiology Department provides newborn hearing screening and follow-up services to all babies born at Northside, and also provides follow-up services to many infants and young children throughout the greater Atlanta area and Northeastern Georgia. Brandt also serves as ASHA's newest representative to the Joint Committee on Infant Hearing. Contact her at brandt.culpepper@northside.com.

References

American Academy of Pediatrics, Council on Children With Disabilities, Section on Developmental Behavioral Pediatrics, Bright Futures Steering Committee, Medical Home Initiatives for Children With Special Needs Project Advisory Committee. (2006). Identifying infants and young children with developmental disorders in the medical home: An algorithm for developmental surveillance and screening. Pediatrics, 118, 405–420.

American Academy of Pediatrics, Task Force on Improving the Effectiveness of Newborn Hearing Screening, Diagnosis, and Intervention. (2003). Universal newborn hearing screening, diagnosis, and intervention: Guidelines for pediatric medical home providers . Elk Grove Village, IL: American Academy of Pediatrics.

Cone-Wesson B., Vohr, B. R., Sininger, Y. S., Widen, J. E., Folsom, R. C., Gorga, M. P., & Norton, S. J. (2000). Identification of neonatal hearing impairment: Infants with hearing loss. Ear and Hearing, 21, 488–507.

D'Agostino, J. A., & Austin, L. (2004). Auditory neuropathy: A potentially under-recognized neonatal intensive care unit sequela. Advances in Neonatal Care, 4, 344–353.

Eilers, R. E., Miskiel, E., Ozdamar, O., Urbano, R., & Widen J. E. (1991). Optimization of automated hearing test algorithms: Simulations using an infant response model. Ear and Hearing, 12, 191–198.

Herrmann, B. S., Thornton, A. R., & Joseph, J. M. (1995). Automated infant hearing screening using the ABR. Development and validation. American Journal of Audiology, 4, 6–14.

Hyde, M. L., Davidson, M. J., & Alberti, P. W. (1991). Auditory test strategy. In J. T. Jacobson & J. L. Northern (Eds), Diagnostic Audiology (pp. 295–322). Austin, TX: Pro-ed.

Hyde, M. D., Sininger, Y. S., & Don, M. (1998). Objective detection and analysis of auditory brainstem response: An historical perspective. Seminars in Hearing, 19, 97–113.

Johnson, J. L., White, K. R., Widen, J. E., Gravel, J. S., James, M., Kennalley, T., et al. (2005). A multicenter evaluation of how many infants with permanent hearing loss pass a two-stage otoacoustic emissions/automated auditory brainstem response newborn hearing screening protocol. Pediatrics, 116, 663–672.

Joint Committee on Infant Hearing. (2000). Year 2000 position statement: Principles and guidelines for early hearing detection and intervention programs. Pediatrics, 106, 798–817.

Joint Committee on Infant Hearing. (2007). Year 2007 position statement of the Joint Committee on Infant Hearing: Principles and guidelines for early hearing detection and intervention programs. Pediatrics, 120, 898–921.

McFarland, W. H., Simmons, F. B., & Jones, F. R. (1980). An automated hearing screening technique for newborns. Journal of Speech and Hearing Disorders, 45, 495–503.

Norton, S. J., Gorga, M. P., Widen, J. E., Folsom, R. C., Sininger, Y., Cone-Wesson, B., et al. (2000). Identification of neonatal hearing impairment: Evaluation of transient evoked otoacoustic emission, distortion product otoacoustic emission, and auditory brain stem response test performance. Ear and Hearing, 21, 508–528.

Ozdamar, O., Delgado, R. E., Eilers, R. E., & Urbano, R. C. (1994). Automated electrophysiologic hearing testing using a threshold-seeking algorithm. Journal of the American Academy of Audiology, 5, 77–88.

Pool, K. D., & Finitzo, T. (1989). Evaluation of a computer-automated program for clinical assessment of the auditory brain stem response. Ear and Hearing, 10, 304–310.

Sininger, Y. S., Hood, L. J., Starr, A., Berlin, C. I., & Picton, T. W. (1995). Hearing loss due to auditory neuropathy. Audiology Today, 7, 10–13.

Starr, A., Sininger, Y. S., & Pratt, H. (2000). The varieties of auditory neuropathy. Journal of Basic Clinical Physiology and Pharmacology, 11, 215–230.