DEPARTMENT OF HEALTH AND HUMAN SERVICES
NATIONAL INSTITUTES OF HEALTH

Fiscal Year 2009 Budget Request

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

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

March 5, 2008

Richard J. Turman, Deputy Assistant Secretary, Budget

Mr. Chairman and Members of the Subcommittee:

I am pleased to present the President’s budget request for the National Institute on Deafness and Other Communication Disorders (NIDCD) of the National Institutes of Health (NIH). The Fiscal Year (FY) 2009 budget of $395,047,000 includes an increase of $909,000 over the FY 2008 appropriated level of $394,138,000. The NIDCD conducts and supports research and research training in the normal and disordered processes of hearing, balance, smell, taste, voice, speech, and language. This year, NIDCD celebrates its 20th anniversary. Over the past two decades, extraordinary research opportunities have led to scientific breakthroughs in the study of genes, proteins, sensory and supporting cells, and molecular processes that directly affect our understanding of communication disorders. NIDCD-supported scientists have also made substantial progress in behavioral studies that increase our understanding of how communication processes contribute to a person’s health. The following are notable research highlights built upon two decades of NIDCD support:

Then And Now: Early Identification of Hearing Loss

Approximately 2–3 per 1,000 babies born in the U.S. each year have a detectable hearing loss, according to the National Center for Hearing Assessment and Management. When NIDCD was established in 1988, U.S. hospitals screened only high-risk babies for hearing loss. On average, healthcare professionals and parents did not recognize many children’s hearing loss until they were 2–3 years old. By this age, the critical period for learning language and speech had already passed. In 1997, NIDCD convened an expert panel to recommend standard newborn hearing screening methods. Today, hospitals use these methods to screen nearly 93 percent of newborns in the U.S., and 40 states and the District of Columbia have passed laws that require infants to be screened for hearing loss. By identifying the hearing loss early, parents and healthcare specialists have the opportunity to choose preemptive treatment to optimize these children’s long-term speech, language, and social skills.

Identifying Genes Responsible For Communication Disorders

Effective communication requires an individual to speak, hear, understand, and make judgments about what is communicated. Twenty years ago, scientists were just beginning to learn how best to identify disease-causing genes. Today, with the help of a comprehensive nucleotide sequence of the human and mouse genomes, NIDCD-supported scientists are identifying and describing genes whose mutation results in many communication disorders, including stuttering, speech-sound disorders, autism, and dyslexia, as well as hearing loss. NIDCD-supported scientists have identified more than 60 genes responsible for inherited hearing loss. In FY 2008, NIDCD is serving as the lead institute for an NIH Government Performance and Results Act (GPRA) goal to “identify or study additional genes involved in communication disorders in human and animal models by 2011.” To achieve this goal, NIDCD-supported scientists will use the knowledge gained from the Human Genome Project to track down and describe genes whose mutation plays a role in communication disorders. These efforts will help scientists develop genetic tests to predict communication disorders. In the future, NIDCD hopes to use genetic knowledge to predict which children are likely to have or develop a hearing loss, create personalized treatment based upon the cause of the problem, and prevent some forms of hearing loss altogether.

Preventing Noise-Induced Hearing Loss

Prevention is an important goal for the NIDCD. We estimate that approximately 22 million American adults have suffered permanent damage to their hearing from exposure to loud noise or sound. The NIDCD’s Wise Ears® public education campaign uses knowledge gleaned from NIDCD-supported research to teach Americans how to prevent some forms of hearing loss. The NIDCD launched WISE EARS!® in 1999 along with the National Institute for Occupational Safety and Health (NIOSH) and a coalition of other state, local, and government agencies. WISE EARS!® is working to increase understanding of noise-induced hearing loss (NIHL) among all audiences. By teaching Americans that everyone is at risk for NIHL, the campaign hopes to motivate Americans to actively participate in addressing the problem, including expanding the availability of hearing protection devices, advocating changes in the workplace, educating young people how to sensibly listen to music; and developing hearing loss prevention programs. NIDCD hopes that these efforts will reduce the number of Americans that could acquire NIHL.

Cochlear Implants

A cochlear implant is an electronic device that provides a sense of sound to individuals who are profoundly deaf or severely hard-of-hearing by processing sounds from the environment and directly stimulate the auditory nerve, bypassing damaged portions of the inner ear. Cochlear implant technology is based in part on NIH-funded research that dates back to the early 1970s. When NIDCD was first established, cochlear implants were somewhat rudimentary devices that enabled users to detect sound, but lip reading and other environmental cues were still very important. Twenty years later, however, NIDCD-supported scientists have made significant improvements in this device. With the appropriate training and support, deaf and severely hard-of-hearing individuals who receive a cochlear implant can enjoy an enhanced quality of life because they can listen and participate in conversations as they typically occur throughout our society. According to the U.S. Food and Drug Administration, approximately 23,000 American adults and 15,500 children have received a cochlear implant. At present, cochlear implants are most successful in children who receive them at a young age, when the brain can still adapt to processing sound and the window for language development is still active. NIDCD-supported scientists are working to understand how the developing brain processes sounds, and why the brain has a limited window of opportunity for language development. NIDCD hopes its scientists can learn enough about language development and brain-implant interaction to improve cochlear implant training, and to make cochlear implants more personalized for individuals who have been deaf since birth or for a prolonged period.

Hearing Aids

NIDCD is also actively supporting research to improve hearing aids. A hearing aid is a small electronic device worn in or behind the ear that amplifies sound. According to the NIDCD, nearly 15 percent of American adults report a hearing loss, and nearly half of adults aged 75 and older have a hearing impairment. In the twenty years since NIDCD was established, hearing aid technology has improved significantly. Hearing-impaired individuals can now opt for digital hearing aids, which can be programmed to amplify some frequencies more than others, and can be adjusted to certain listening environments. Nevertheless, only 20 percent of people who could benefit from hearing aids wear them. A recurring complaint of hearing aid users is the aid’s tendency to amplify most sounds, making it difficult to focus attention on a conversation that occurs amidst a lot of background noise, such as in a crowded restaurant. NIDCD-supported researchers have developed a new and exciting technology, based on the ears of a small, parasitic fly with acute directional hearing. The scientists used manmade materials to imitate the fly ear’s biological structure. By copying nature, the scientists have succeeded in copying a unique and important ability of the fly: the ability to zero in on one particular sound amidst background noise. Scientists are hard at work miniaturizing this prototype device, and incorporating it into hearing aids. The device offers significant promise in improving a hearing aid user’s ability to listen to conversations amidst background noise. NIDCD hopes this research will lead to the development of hearing aids that are more personalized, better mimicking normal hearing.

Hair Cell Regeneration

NIDCD support has led to significant progress towards understanding how we might one day regenerate the cells that detect sound in the human inner ear. Our ability to hear relies on hair cells, small sensory cells in the inner ear. Hair cells have bundles of microscopic hair-like extensions, called stereocilia, that project from their top surface. These “hair bundles” convert sound vibrations into electrical signals, which travel to the brain by way of the auditory, or hearing, nerve. Hair cells can be damaged by disease, injury, aging, or from certain drugs. When hair cells are damaged, an individual experiences hearing loss. Although fish, amphibians, and birds can spontaneously grow new hair cells to replace damaged ones, mammals cannot regenerate hair cells.

NIDCD support has helped scientists study development of the mammalian inner ear. Consequently, we now understand more about how the developing inner ear determines which primitive cells become hair cells, and which become the supporting cells that help maintain the hair cells. These basic studies provided the foundations for more recent breakthroughs. NIDCD-supported scientists have been able to identify the specific genes that determine an inner ear cell’s fate. They are also refining their knowledge of how the inner ear’s sound-detecting structures are set up. By combining their knowledge of anatomical development and gene expression, NIDCD-supported scientists have made a breakthrough discovery and are able to grow new hair cells in laboratory mammals, in some cases restoring some hearing to these mammals that could not hear before treatment. These promising results provide the hope that we might someday be able to regenerate hair cells in humans.

Finally, Mr. Chairman, I would like to thank you and Members of this Subcommittee for giving me the opportunity today to present this sample of the exciting scientific advances made with the support of the NIDCD. I am pleased to answer your questions.

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.

U.S. Department of Health and Human Services
National Institute of Health (NIH)
Office of Budget
Deputy Assistant Secretary for Budget, HHS

Richard J. Turman
Mr. Turman is the Deputy Assistant Secretary for Budget, HHS. He joined federal service as a Presidential Management Intern in 1987 at the Office of Management and Budget, where he worked as a Budget Examiner and later as a Branch Chief. He has worked as a Legislative Assistant in the Senate, as the Director of Federal Relations for an association of research universities, and as the Associate Director for Budget of the National Institutes of Health. He received a Bachelor’s Degree from the University of California, Santa Cruz, and a Masters in Public Policy from the University of California, Berkeley.