DEPARTMENT OF HEALTH AND HUMAN SERVICES
NATIONAL INSTITUTES OF HEALTH

Mind, Brain and Behavior

Witness appearing before the Senate Subcommittee on Labor-HHS-Education Appropriations

James F. Battey, Jr., M.D., Ph.D., Director
National Institute on Deafness and Other Communication Disorders

March 26, 2007

Richard J. Turman, Deputy Assistant Secretary, Budget

Mr. Chairman and Members of the Subcommittee:

I present the President's budget request for the National Institute on Deafness and Other Communication Disorders (NIDCD). The Fiscal Year 2008 budget for NIDCD includes $393,682,000. The NIDCD conducts and supports research and research training in the normal and disordered processes of hearing, balance, smell, taste, voice, speech, and language. These processes are fundamental to the way we perceive the world and to our ability to communicate effectively in modern society. Disorders of communication impose significant economic, social, and personal costs. Accordingly, the goal of the NIDCD strategy is to produce outcomes with a significant impact on the health of Americans. Driven by the public health need and scientific opportunity identified in the NIDCD Strategic Plan, NIDCD prioritizes its research investment to fund the most promising scientific opportunities in diagnosis and treatment of communication disorders. The following are notable highlights from the past year that are the result of NIDCD support:

Genes and Communication Disorders

The NIDCD recognizes that functional genomics—determining the identity, structure, and function of genes—is one of the most rapidly developing areas of research. Inherited genes account for approximately 50–60 percent of the severe to profound cases of childhood hearing loss. NIDCD scientists are working to understand the normal function of these genes, and how they are altered in individuals with communication disorders (such as hearing loss, stuttering, speech-sound disorders, autism, and dyslexia). These research investments to understand the genetic basis of communication disorders will help scientists develop diagnostic tests and better treatments for the millions of Americans with hereditary hearing impairment.

Preventing and Diagnosing Communication Disorders

The Centers for Disease Control and Prevention (CDC) reports that two to three out of 1,000 babies born each year in the United States have a detectable hearing loss, and estimates the average lifetime cost for one individual with hearing loss to be $417,000 (in 2003 dollars). Accordingly, NIDCD places a high priority on understanding causes, possible treatments, and progression of hearing loss during early childhood. NIDCD-supported research demonstrates that children not exposed to language during their first 3 years of life due to hearing loss will have more difficulty developing spoken or signed language and reading skills. Early identification of hearing loss enables parents to pursue interventions early enough that their child can learn to communicate on par with his or her hearing peers.

However, childhood hearing loss does not always show up right away. Congenital cytomegalovirus (CMV) is the most common viral infection passed from a mother to her unborn child, with 40,000 infants born infected each year. According to the CDC, approximately 10 to 15 percent of these children have some degree of hearing loss. Scientists believe that CMV infection present at birth is a leading cause of sensorineural hearing loss in children. Hospitals do not test newborns for CMV unless they already show signs of the disease. NIDCD is funding the CMV and Hearing Multicenter Screening (CHIMES) Study to identify asymptomatic children and follow them to determine if hearing loss develops. Scientists will screen approximately 100,000 children at birth for CMV infection, and those who test positive will undergo follow-up diagnostic hearing testing to determine the onset, severity, and progression of hearing loss. The scientists will use these data to understand the relationship between CMV infection and hearing loss and to determine whether CMV screening together with hearing testing can improve the detection and prediction of permanent hearing loss in children.

Although success in establishing early screening programs has identified a new population of children with hearing loss, we do not know which interventions provide the best outcomes. Current intervention and outcome data are limited to those children whose hearing loss was detected later in life. Hearing health specialists need research data that considers not only the intervention strategy but also the parent-child interaction, socio-economic factors, and language exposure. To address this need, NIDCD held a workshop on "Outcomes in the Child with Hearing Loss" in December 2006. NIDCD is using information from this workshop to develop FY 2008 initiatives focused on prospective and longitudinal research. These initiatives will be part of a multi-agency collaboration designed to close the gap between children with hearing loss and their hearing peers, and will provide sorely-needed information on the best strategies to achieve this goal.

Developing Assistive Devices

NIDCD-supported basic research on the ears of the tiny fly Ormia ochracea has inspired a new generation of hearing aids. The fly's ear structure permits ultra-sensitive time coding and localization of sound, and scientists used it as a model to develop miniature directional hearing aid microphones that can selectively amplify speech rather than amplifying all sounds. NIDCD-supported scientists are now working to make these directional hearing aids widely available. Individuals with hearing loss who use hearing aids fitted with these improved directional microphones will experience improved quality of life because the aids will do a better job of helping them to understand spoken language amidst background noise.

Some individuals with severe to profound sensorineural hearing loss may benefit from a cochlear implant (CI). The NIH's support has played a significant and important role in the development of CI technology over the last three decades. A CI converts sound into electrical impulses on an array of electrodes surgically inserted into the inner ear, bypassing the damaged hair cells that normally detect sound (see illustrations of cochlear implants). The CI stimulates the auditory nerve directly and restores the perception of sound to individuals who are deaf.

The Food and Drug Administration (FDA) estimates that approximately 36,000 Americans have received CIs, and one half of the recipients were children. The FDA approved the use of CIs in children as young as 12 months of age. NIDCD supported research demonstrates that the sooner a child with profound hearing impariment receives the benefit of a CI, the greater the benefits and improvements in speech perception and language production. Because of the rapid development and plasticity of their brains, young children implanted with a CI usually show age appropriate brain responses within six to nine months after the CI is turned on.

CIs are expensive (costing approximately $60,000 for the device, associated surgical expenses, and postoperative fitting and training) and many insurance companies were initially unwilling to reimburse for this cost, citing a lack of evidence that the device is cost effective. To address this concern, NIDCD-supported scientists conducted an initial cost utility analysis of the CI in children to examine whether the benefits of the implant outweigh its costs. The study showed that CIs improve the children's quality of life, and result in a net saving to society. The cost benefit is the result of fewer demands on special education and greater wage earning opportunities for CI recipients, providing an estimated life savings per child at $53,198. This landmark study has helped make CIs a standard treatment for severe to profound nerve deafness, and many insurance companies now cover them.

An NIDCD-supported study assessed the sound-localization abilities of children (ages 5 to 14 years) wearing two cochlear implants as compared to one. Children in the study located the source of a sound more accurately when they were wearing two implants as opposed to one. The greater the experience with two implants, the more adept he or she became at localizing sound. The research team is now investigating the effects of bilateral implants on word learning and language acquisition in infants and toddlers receiving CIs at a young age.

NIDCD-supported scientists are currently using lessons learned from their cochlear implant research experiences to develop an implanted device to help restore the sense of balance. The prototype vestibular implant has the potential to benefit over 90 million Americans who have experienced a dizziness or balance problem.

Strategies to Protect Your Hearing

The NIDCD shares Congress's concerns that approximately 10 percent (over 22 million) of American adults have suffered permanent damage to their hearing from exposure to loud sounds or noise at work or in leisure activities (CDC NHANES). In 1999, the NIDCD collaborated with the National Institute for Occupational Safety and Health (NIOSH) to launch WISE EARS!®. WISE EARS!® is a national campaign to prevent noise-induced hearing loss (NIHL) in the general public, including the workplace. NIDCD has built a coalition of nearly 90 partner organizations and disseminated information and promotional materials through the media, at professional conferences and health fairs, and over the Internet. In 2006, the NIDCD conducted an evaluation on the WISE EARS!® Public Health Campaign to obtain an accurate picture of how far WISE EARS!® has progressed in achieving its goals and to identify those needs that have not yet been addressed through current educational and promotional methods.

Finally, Mr. Chairman, I would like to thank you and Members of this Subcommittee for giving me the opportunity today to present exciting scientific advances from the NIDCD. I am pleased to answer any questions that you have.

Biography

U.S. Department of Health and Human Services
National Institutes of Health (NIH)
National Institute on Deafness and Other Communication Disorders (NIDCD)
Director

James F. Battey, Jr., M.D., Ph.D.
Dr. Battey is currently serving as the director of the NIDCD within the National Institutes of Health (NIH), Department of Health and Human Services. He has served as the NIDCD director since February 1998. Dr. Battey served as the Chair of the NIH Stem Cell Task Force from 2002 until December 2006. He received his Bachelors of Science degree in Physics from the California Institute of Technology in 1974. He received a M.D. and Ph. D. in Biophysics from Stanford University School of Medicine in 1980. After receiving training in Pediatrics, he pursued a postdoctoral fellowship in Genetics at Harvard Medical School under the mentorship of Dr. Philip Leder. Since completing his postdoctoral fellowship in 1983, he has held a variety of positions at the NIH, including serving in the National Cancer Institute (NCI), the National Institute of Neurological Disorders and Stroke (NINDS), and the NIDCD. He has been married for 26 years to Frances Battey, and has two sons, Michael and JJ.