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NCHAM: National Center for Hearing Assessment and Management, Utah State University

Last Updated: 06/30/2011

NCHAM Research Projects

Title: Early Development of Children with Hearing Loss (EDCHL)
Web Site: http://www.speechdevelopment.org/EDCHL/
Time Period: September 2003 to August 2008
Funding: National Institute on Deafness and Other Communication Disorders

Researchers at Utah State University have just received a grant to study the development of young children (ages 12 months to 4½ years) with hearing loss. (Start date is September 1, 2003.) This project is funded by the National Institute on Deafness and Other Communication Disorders and is headed by Dr. Susan Nittrouer. The study will be conducted over a 5-year period and will look at the development of speech, language, cognitive, and psychosocial skills in young children with hearing loss. Specifically investigators will be examining whether there are differences in the development of these skills as a function of the age of initiation of intervention and whether the intervention approach is auditory-oral or is supplemented by the use of signs. Results will help educators and clinicians design early intervention programs that ensure the best outcomes for all children.

If you are a parent of young child with hearing loss, please consider participating. If you are a professional involved in early intervention for children with hearing loss, please consider helping us conduct these tests. For more information, contact either Susan Nittrouer (Nittrouer@cpd2.usu.edu), Dan Robertson (Danr@cpd2.usu.edu) or Alma Burgess (Alma@cpd2.usu.edu).

Title: EHDI Program Cost Analyses
Time Period: October 2000 to September 2005
Funding: Centers for Disease Control and Prevention and Utah Department of Health

The recent expansion of newborn hearing screening and intervention programs has led to many questions about the costs, cost-effectiveness, and cost-benefit of Early Hearing Detection and Intervention (EHDI) programs. Although a few cost analyses of EHDI programs have been done, most of these have been very limited in scope, have relied on hypothetical data, or have not used standard analysis techniques. Working in conjunction with economists at the Center for Disease Control and Prevention and the Early Intervention Research Institute at Utah State University, NCHAM is conducting a series of cost analyses of EHDI programs during the period from.

Title: Genetics of Hearing Loss
Time Period: October 2000 to September 2005
Funding: Centers for Disease Control and Prevention and Utah Department of Health

About 1 in 300 infants have congenital mild profound hearing loss. It is estimated that genetic causes account for at least 50% of such hearing loss, and about 90% of genetic hearing loss is non-syndromic with autosomal recessive inheritance representing the most common etiology. Mutations of the Connexin 26 gene represent about 50% of recessive hearing loss. Thus, as much as 20% of all prelingual hearing loss may be due to mutations of the Connexin 26 gene. Several other genes have been identified as causing hearing loss (e.g., those associated with Wardenburg syndrome and some forms of Usher syndrome).

The goal of this project is to provide better understanding of the etiology and pathogenesis of genetic hearing loss which could lead to prevention or therapy. This will be done by doing genetic evaluations of all infants identified through the Utah Statewide Newborn Hearing Screening Program. For every family who chooses to participate in the project, the first part of the analysis will include examining records, documenting the child's pedigree, and conducting a physical examination to determine syndrome status. All families without a well-established etiology based on this analysis will be offered Connexin 26 mutation testing (genotyping) based on a small sample of blood. If a Connexin 26 mutation is not present, mitochondrial mutations associated with non-syndromic hearing loss will be tested.

As a result of these analyses, participants will be classified according to the cause of hearing loss (i.e., acquired, genetic, or unidentified). This will significantly enhance our general understanding of the causes of hearing loss in infants identified in universal newborn hearing screening programs.

Title: Efficacy of a Two-Stage (OAE/AABR) Newborn Hearing Screening Protocol
Time Period: October 2000 to September 2002
Funding: Centers for Disease Control and Prevention

Efforts to minimize false-positive newborn hearing screening results without sacrificing the sensitivity of the test have led to various screening protocols. A protocol which is being used more and more frequently is to screen all newborns with Otoacoustic Emissions (OAE). If the baby passes, no further testing is done. If the baby does not pass the OAE screen, a screen is done using Automated Auditory Brainstem Response (AABR). Because of anecdotal evidence which suggested that a significant number of babies with mild hearing loss might be missed using this OAE/AABR approach, the Centers for Disease Control and Prevention issued a request for proposals to do a study of the OAE/AABR protocol. NCHAM is participating in a multi-center study involving 12 hospitals (all of which are using the (OAE/AABR protocol) and the University of Hawaii. Of approximately 50,000 babies born at these hospitals during the study period, it is expected that approximately 1,650 will fail the OAE screen, but pass the AABR screen. Normally, these babies would not be followed. However, in this study, families of the babies will be asked to return for a complete diagnostic audiological evaluation when the baby is 7 months old. Results of this evaluation will be used in conjunction with demographic and medical information about each baby to determine if a significant number of babies with hearing loss are being missed using the OAE/AABR screening protocol.

Title: Integrating EHDI Tracking and Data Management with Other Public Health Information Systems
Web Site: http://charm.health.utah.gov
Time Period: October 2000 through September 2005
Funding: Centers for Disease Control and Prevention

The goal of this project is to improve the timeliness and appropriateness of Early Hearing Detection and Intervention (EHDI) services to infants and their families by:

  • refining and expanding Utah's existing surveillance and tracking system for EHDI; and
  • integrating the EHDI surveillance and tracking system with other relevant public health information databases and service systems, including, but not limited to, the Electronic Birth Certificate, Heelstick Screening, Birth Defects, Early Intervention, the Utah State Immunization Information System, and Women, Infants and Children.

Over the past five years, the Utah Department of Health (UDOH) has made substantial progress in developing a public health information infrastructure. Most local health departments are now linked to the State's Wide Area Network, internet access about key health indicators is available and being expanded, an Electronic Birth Certificate has been developed, a statewide EHDI surveillance and tracking system has been implemented, and the Utah Immunization Information System has been created. What has not happened is the integration of those various components, along with other relevant databases and information systems, into an integrated system which can be used to improve services to children and families, make better public health policy decisions, and advance knowledge about children with special health care needs. By creating electronic linkages among these various systems and providing procedures for secure internet access and transmission to authorized users, this project will improve the quality and effectiveness of health care services for children.

Title: A Statewide Evaluation of Utah's Early Hearing Detection and Intevention Program
Time Period: April 2000 to September 2004
Funding: Utah Department of Health

NCHAM has contracted with the Utah Department of Health to do an annual evaluation of the state's Early Hearing Detection and Intervention program. Data collected for the evaluation consists of results of questionnaires from each birthing hospital in the state, analyses of data from the state's EHDI tracking and data management system, site visits to a sample of hospitals, and questions completed by a sample of parents and physicians who have participated in the EHDI program during the past year. Results of the evaluation from each year are used to judge progress and improve the program.

Title: Updating Hearing Screening Procedures for Migrant, American Indian, and Early Head Start Children
Web: http://www.infanthearing.org/earlychildhood/
Time Period: September 2001 to August 2004
Funding: Administration on Children and Families

NCHAM is conducting the Hearing Head Start pilot study with Migrant, American Indian, and Early Head Start programs to update their hearing screening practices using OAE hearing screening technology and an effective screening and follow-up protocol. The project was initiated in Oregon, Utah, and Washington and was recently expanded to Kansas and North Carolina. NCHAM has trained over 100 Head Start staff, representing 19 Grantees serving across 69 sites, to conduct OAE hearing screenings on children 0 – 3 years of age enrolled in their programs. Screening and outcome data has been collected on over 2800 children to date. Training is also being provided to each state's existing Early Hearing Detection and Intervention program to sustain and expand the Project.