FY1999 President's Budget Request for the NIDCD

DEPARTMENT OF HEALTH AND HUMAN SERVICES

Fiscal Year 1999 President's Budget Request
for the National Institute on Deafness and Other Communication Disorders

Statement by
Dr. James F. Battey, Jr.
Director, National Institute on Deafness and Other Communication Disorders

Mr. Chairman and Members of the Committee, the President in his 1999 budget has proposed that the National Institute on Deafness and Other Communication Disorders receive $213.8 million, an increase of $14.9 million over the non-AIDS portion of the comparable FY 1998 appropriation. Including the estimated allocation for AIDS in both years, total support proposed for NIDCD is $215.7 million, an increase of $15 million over the FY 1998 appropriation. Funds for NIDCD efforts in AIDS research are included within the Office of AIDS Research budget request. I am honored to appear before you as the newly appointed Director of the National Institute on Deafness and Other Communication Disorders (NIDCD). Several members of Congress were actively involved in the creation of the NIDCD nearly ten years ago. As you envisioned, NIDCD has become the focal point of research in human communication supported and conducted by the Federal government in the fifty states. In the last several years, in my role as the NIDCD's Scientific Director, I was part of the Institute's growth and development, whose goal is to advance knowledge about the mechanisms and processes of human communication, and revolutionizing prevention and treatment of disease and disorder.

Communication Skills at the Center of Successful Life in the New Century

Communication skills will be central to a successful life in the new century for all Americans. For the 46 million Americans with communication disabilities, however, getting up and facing each day is a challenge. The simple acts of speaking, listening, of making their wants and their needs understood, are often impossible. For the individual who has vertigo, or the person who finds himself or herself suddenly unable to hear, the days are challenging. For those who cannot speak without stuttering or for those who are unable to express ideas clearly after suffering a stroke, for those who cannot use their voices to talk with a friend on the phone due to the devastation of throat cancer--each day is challenging. The days are challenging for the child who has autism. They are also a challenge for the individual who cannot participate in activities because his or her tinnitus has become overwhelming. For an older person a loss of balance can result in falls and fractured bones, and a loss of hearing results in isolation. For the young child who begins a struggle with language, that if not for intervention, will be a lifelong struggle-communication disabilities pose a constant challenge. NIDCD made important progress in the disorders of human communication this year and has identified new targets, new tools and new teams for acccelerating discovery in FY 1999.

New Tools: Sensory Imaging

In reviewing the progress of the past year, the advances made in human communication research are frequently based upon new tools, new targets and new teams of scientists looking at research in novel ways. For example, we now have the ability to see the brain at work during human communication through the use of powerful imaging tools. Much of the human brain is used for hearing, balance, voice, speech and the manipulation and production of language, as well as the ability to smell and to taste.

Imaging Provides First Objective View of Tinnitus

Having the ability to image brain activity patterns during various communication events involving stuttering, aphasia, tinnitus, and American Sign Language is revolutionizing our understanding of normal and disordered processes of human communication. In one of many remarkable advances this year, NIDCD scientists are able to visualize brain activity occurring with tinnitus, or ringing in the ears. Tinnitus has been extremely difficult to study without an objective model as it is associated with virtually every kind of hearing loss. These studies provide ground-breaking information about the nature of tinnitus, which will hopefully lead to improved strategies for diagnosis and treatment for the millions of Americans challenged with the incessant or intermittent audiotory sensation that is tinnitus.

Sensory Hair Cell Regeneration Progress

NIDCD-supported scientists are determining the properties of unique sensory cells of the inner ear called "hair cells." These cells are critical for converting mechanical energy from sound or motion into electrochemical signals sent to the brain. Loss of hair cells is frequently the cause of hearing impairment and balance disorders. NIDCD-supported scientists are studying the molecular mechanisms underlying hair cell regeneration in animal model systems with the ultimate goal of using this information to restore hair cells in individuals with hearing impairment and balance disorders.

Cochlear Implants Restore Hearing

In a different way, NIDCD scientists are trying to restore hearing through support of the development of the cochlear implant. The cochlear implant is a sensory neural auditory prosthesis that improves economic and social outcomes for post-lingual hearing impaired individuals. Here is a modern cochlear implant. Let me show you how it works. Part of it is surgically implanted within and behind the ear and the other components are worn. The implant bypasses the nonfunctioning hair cells in the cochlea and stimulates the auditory neural pathway. The prosthesis also includes a voice processor that has been designed to sample and convert sound at high speed and through a brilliant speech processing strategy. As an indication of how well this implantable prosthesis works, many who become suddenly deaf in mid-career are now able to remain in their jobs. These individuals are able to use the telephone again after learning to use the implant through rehabilitation training.

Early Identification of Deaf and Hard of Hearing Infants and Development of Language

NIDCD has continued to investigate the development of language, signed or spoken, in children who are deaf or hard of hearing. NIDCD-supported scientists have shown that the first six months of life represent a crucial period for subsequent development of language either spoken or signed. A number of states are implementing universal newborn hearing screening which begins with a test for auditory function very soon after birth. In 1998, NIDCD-supported scientists will complete a five-year study showing that two screening methods, measurement of otoacoustic emissions and auditory brainstem responses, can be used to accurately identify these deaf and hearing-impaired newborns. These research results will provide much needed guidance for implementing universal newborn hearing screening.

Progress in Understanding Olfactory Reception

The olfactory receptor gene family has about 1,000 members. More than five years ago, scientists discovered this large, multi-gene family, but had difficulty proving that these receptors responded to an odorant. An NIDCD-supported laboratory has shown that a member of the multi-gene family first identified as encoding a putative odorant receptor does indeed code for a protein that is capable of specific odor binding leading to a physiological response. Using a recombinant adenovirus, scientists were able to direct expression of a particular receptor gene in an increased number of rat olfactory neurons. Electrophysiological recording showed that increased expression of a single olfactory receptor gene increases odor and sensitivity to a small subset of odorants. These studies provide the basis for additional research that will correlate olfactory receptor structure with odorant responses.

New Approach for Children with Specific Language Impairment

NIDCD has also made progress in studying specific language impairment (SLI) in hearing children. Specific language impairment, or SLI, is a deficit in language acquisition found in the absence of other cognitive impairment, and is present in about 8% of American school age children. SLI is a common cause of poor academic performance and frustration with learning. For a long time the focus of research was upon language and language strategies. Within the last few years, however, NIDCD-supported scientists have determined that SLI is often caused by a specific inability to process rapidly changing auditory information, such as occurs in some normal human speech. These findings will allow a more precise diagnosis of SLI, and suggests new intervention strategies to help children with SLI.

Molecular Genetics Collaboration Provides Key to Form of Hearing Impairment

Molecular genetics is revealing genes involved in many disorders of human communication. The search for hearing impairment genes is greatly facilitated by the timely, collaborative information exchange among different NIH Institutes working with a common purpose. For example, when a gene for a syndrome consisting of hearing impairment coupled with thyroid abnormalities was identified by a National Human Genome Research Institute scientist, this information was shared with NIDCD intramural investigators. The NIDCD scientists used the information to show that different mutations in the same gene also causes recessive non-syndromic hereditary hearing impairment in some families.

Improved Understanding of the Cause of Recurrent Otitis Media

As every parent knows, otitis media or middle ear infection, is the most frequent reason that a sick child visits either emergency rooms or physicians' offices. The estimated total cost of otitis media is $5 billion a year.¹ Otitis media appears to be increasingly resistant to conventional antibiotic therapy. One of the most difficult aspects of treating otitis media is that relapses often occur within several weeks of antibiotic treatment. This clinical problem was difficult to explain, since most middle ear effusions showed no evidence of bacterial infection when cultured. Using a molecular biology technique (polymerase chain reaction assays), NIDCD scientists were able to detect the presence of bacterial mRNA in a significant number of culturally sterile middle ear effusions. This established the presence of viable organisms in some culture-negative Otitis Media with Effusion. Scientists believe that these elusive bacteria exist in biofilms, a kind of bacterial community that contains intact but indolent organisms deep within the film and away from antibiotics. Otitis media may serve as an ideal model for studying the role of biofilms in other recurrent infectious disease.

New Teams: Velocardiofacial syndrome

In addition to new tools and new targets, new teams of scientists are providing collaborations for progress. NIDCD is convinced that multidisciplinary teams of scientists working together will be most effective in understanding the basis for human communication disorders. The NIDCD has already observed the benefit of this kind of collaboration. Velocardiofacial syndrome (VCFS) is a genetic disorder resulting in cardiac malformation and cleft palate, which has direct impact upon human communication skills. A team of NIDCD-supported scientists including molecular biologists, human geneticists, otolaryngologists, and cardiologists are searching for the gene or genes that cause VCFS. They are also developing better tools for diagnosis and treatment. This multi-disciplinary team is a model for future partnerships among clinicians and scientists. NIDCD sees an opportunity to support similar teams who will work together to understand the biology and genetics of voice, speech, and language disorders, and translate this understanding into improved diagnosis and better intervention strategies.

New NIH Collaborative Teams
NIDCD Supports Autism and Cancer Initiative

NIDCD was pleased to cooperate on an important autism initiative. Autism is a common developmental disorder found in children, affecting as many as one child in 1000. One of the most striking problems these children face is the inability to communicate with other people. NIDCD has joined three other Institutes (National Institute of Child Health and Human Development, National Institute of Mental Health and National Institute of Neurological Disorders and Stroke) to support research to identify the genes that underlie autism, as well as to develop more effective intervention strategies.

In another collaborative effort, NIDCD is working at defeating cancer of the head and neck. Each year, tens of thousands of Americans develop cancer of the head and neck. Conventional treatment using surgery and radiation therapy is often less than fully effective and results in destruction of organs critical for human speech. Clearly, better treatment modalities are needed. NIDCD has partnered with the National Cancer Institute and the National Institute of Dental Research to conduct and support research to determine the molecular mechanisms critical for the pathogenesis and progression of these malignancies. Cancer of the head and neck is unusual in that the scientist can watch the progression, remission or treatment effectivenes s directly. In addition, these three institutes will work together to translate these basic scientific discoveries into more effective treatment options, sparing organs critical for human communication.

NIDCD Collaborates for Hearing Aid Improvement

NIDCD is collaborating with the Department of Veterans Affairs and the National Aeronautics and Space Administration to improve hearing aids and sponsored a workshop to showcase technologies existing in Federal laboratories to facilitate transfer of those technologies that could improve the function of hearing aids. NIDCD looks forward to funding applications from multidisciplinary collaborations designed to improve speech signal processing for hearing aids.

New Plan

The pace of biomedical research advance is accelerating. To stay ahead, NIDCD is launching an accelerated planning process that will seek the collective ideas of the scientific community as to where the greatest need and opportunity lie in human communication research. New tools, new targets, and more importantly, new teams of investigators will be needed to rapidly and effectively seize the remarkable opportunities before us. We look forward to expanding our understanding of the biology and genetics of human communication disorders that affect 46 million Americans, and translating that knowledge into better strategies for diagnosis, early intervention and treatment.

The activities of the NIDCD are covered within the NIH-wide Annual Performance Plan required under the Government Performance and Results Act (GPRA). The FY 1999 performance goals and measures for NIH are detailed in this performance plan and are linked to both the budget and the HHS GPRA Strategic Plan which was transmitted to Congress on September 30, 1997. NIH's performance targets in the Plan are partially a function of resource levels requested in the President's Budget and could change based upon final Congressional Appropriations action. NIH looks forward to Congress' feedback on the usefulness of its Performance Plan, as well as to working with Congress on achieving the NIH goals laid out in this Plan.

My colleagues and I will be happy to respond to any questions you may have.

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