FY2001 President's Budget Request for the NIDCD

DEPARTMENT OF HEALTH AND HUMAN SERVICES

Fiscal Year 2001 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, I am pleased to present the President's non-AIDS budget for the National Institute on Deafness and Other Communication Disorders (NIDCD) for FY 2001, a sum of $276.4 million, which reflects an increase of $14.3 million over the comparable FY 2000 appropriation. Including the estimated allocation for AIDS, total support requested for NIDCD is $278 million, an increase of $14.3 million over the FY 2000 appropriation. Funds for the NIDCD efforts in AIDS research are included within the Office of AIDS Research budget request. Within the last year, we have witnessed outstanding research progress in human communication and communication disorders by NIDCD-supported scientists and clinicians, progress further accelerated by the efforts of other NIH institutes.

Development

How Inner Ear Hair Cells Grow. In humans, auditory sensory cells (hair cells) and other internal parts of the ears develop within the third month of development. These fragile, highly specialized cells, which are essential for the hearing process, are often damaged or lost as a consequence of noise, genetic mutation, drugs or other environmental insults. The resulting hearing impairment is permanent, since these cells do not regenerate in humans. NIDCD-supported scientists are examining the cellular and molecular processes that direct progenitor cells to differentiate into hair cells, leading to new approaches to stimulate hair cell regeneration after damage. These investigators have shown that in the mouse, the Math1 gene is essential for regulating the development of hair cells and progenitor cells. These findings provide novel insight into the molecular mechanisms regulating hair cell differentiation and specification.

Infancy and Childhood

Better Procedures to Screen Infants for Hearing Impairment. The American Speech-Language-Hearing Association estimates that as many as 12,000 infants each year in the U.S. are born with significant hearing loss, making it a common congenital disorder. Research supported by NIDCD has shown that detection of hearing impairment and intervention within the first six months after birth is very important for optimizing language development in young children. In a five-year, multi-center study, NIDCD-supported scientists determined the optimal test procedures for neonatal hearing screening. This study was the first controlled comparison of normal hearing and hearing-impaired infants evaluating physiological responses to sound. The development of precise and timely diagnostic screening techniques for hearing impairment is the first step in providing early intervention strategies that will optimize the development of either spoken or signed language skills. The NIDCD is supporting research to develop and validate intervention strategies that are tailored to the individual with hearing impairment.

Hereditary Hearing Impairment -- Gene Discovery and Implications. Not only is hearing screening becoming available to all newborns, breakthroughs in medical genetics will enable scientists to identify the precise genetic change leading to hereditary hearing impairment. NIDCD-supported scientists have learned that about 1/3 of all recessive hereditary hearing impairment within the U.S. is caused by mutations in the GJB2 gene. But further studies have shown that there is significant variation in the degree and time-of-onset of hearing impairment among individuals with exactly the same mutation in both GJB2 genes. Given this variation, it would be difficult to predict onset and degree of impairment in these infants using only data from a GJB2 genetic test. The NIDCD is interested in pursuing areas of research to develop and validate diagnostic genetic tests, to assess the potential impact of genetic testing and the utilization of genetic information on attitudes and behaviors of various cultural groups and individuals.

Otitis Media -- Vaccine Development and Genetic Susceptibility. In an NIDCD-supported study, scientists have discovered that there is a strong heritable component to prolonged time with and recurrent episodes of otitis media (middle ear infection) in children. The results of this study may have future implications for primary care physicians to identify children and siblings at high risk for otitis media for careful monitoring and early intervention. In addition, with the recent emergence of antibiotic resistant bacterial isolates, it is clear that the best long-term strategy for otitis media is prevention. NIDCD scientists have developed a detoxified lipooligosaccharide-protein conjugate to be used as a possible vaccine against nontypable Haemophilus influenzae, a leading cause of otitis media in children for which there is no vaccine currently available. A Phase I clinical study is nearing completion in adult volunteers to evaluate the safety and potential efficacy of the investigational vaccine. Preliminary data from this study show that the vaccine is able to elicit the production of specific antibodies against the bacteria in a number of volunteer subjects. The results of this trial suggest that this investigational vaccine may be useful for preventing otitis media in children.

Cochlear Implants May Improve Language Achievement in Children. The cochlear implant is an array of electrodes that converts sound into electrical impulses that stimulate the acoustic nerve, restoring the perception of sound. It is the only neural prosthesis in widespread clinical use with over 20,000 recipients, about one-half of whom are children. Scientists supported by the NIDCD conducted a study to measure language achievement in children with cochlear implants. The study, comparing a group of children who had received cochlear implants and a second group who were using hearing aids, showed significant differences in language achievement levels favoring the children using cochlear implants.

Improved Methods for Diagnosing Early Childhood Stuttering. Stuttering is a disorder that typically begins between the ages of 2 and 5. When it persists, the disorder causes serious impairment in verbal communication that is often associated with significant difficulties in emotional and social adjustments. NIDCD is supporting a large-scale longitudinal investigation of children who stutter to examine various aspects of stuttering as it persists or subsides during childhood. In addition, the study is identifying risk factors that can help differentiate between children who develop persistent stuttering and those who tend to recover. The data reveal a strong genetic component to stuttering and differences in genetic liability between different subsets of children who stutter. Based on these findings, NIDCD-supported investigators have initiated a genetic association study to map and identify the genes that predispose individuals to stutter.

Defining and Identifying Specific Language Impairment in Children. Specific Language Impairment (SLI) is a language disability observed in the absence of any other cognitive disorders, affecting as many as 8% of all kindergarten-age children. Research to understand and treat SLI has been hampered by the lack of uniformity in the definitions and measures that are used to identify preschool-aged and older children, adolescents or adults with SLI. NIDCD-supported researchers have developed definitional guidelines and research directions that will lead to enhanced abilities to diagnose and assess SLI, determining that a brief non-word repetition task is a powerful predictor of SLI. This test differentiates between children who will benefit from .language intervention and children who will not require intervention to achieve normal language skills.

Eliminating Health Disparities in Hearing and Language Disorders. As research moves forward to reduce the burden of disease in America, the NIDCD is committed to the idea that all segments of American people should benefit from this progress. In comparison to the general U.S. population, Native American children have one of the highest rates of otitis media. The NIDCD is continuing its support of a study on the epidemiology of this disorder and hearing loss among Native American infants, from birth to age two, at the White Earth Reservation in Minnesota. Recent assessment shows that intervention programs should focus on parental smoking as a significant risk factor for otitis media in Native American infants. The study also includes the development and implementation of prevention strategies to reduce the burden of otitis media such as promoting breastfeeding.

Treatment for Deafness Caused by Neurofibromatosis Type 2. The NIDCD is conducting research on neurofibromatosis type 2 (NF2), a genetic disorder that often results in bilateral tumors of the acoustic nerves causing deafness in children and adults. Scientists supported by the NIDCD have determined that specific mutations in the NF2 gene result in different levels of severity of the disease. This finding will facilitate early DNA-based diagnoses that will improve disease management and increase the preservation of hearing in NF2 patients. For many individuals with NF2, surgical intervention required to remove tumors also involves resection of both acoustic nerves, so that sound perception cannot be restored with cochlear implantation. To help these individuals, NIDCD is supporting research to develop a specialized auditory prosthesis for NF2 patients. Multiple, ultraminiature microelectrodes have been implanted directly into the ventral cochlear nucleus of animals, the portion of the central auditory system where the acoustic nerve fibers once made connections. These animal studies have demonstrated the safety of this technique and deaf NF2 patients are now scheduled to be fitted with these devices within the next few years with the hope of restoring auditory perception.

Adulthood

The Hazards of Noise-Induced Hearing Loss. When an individual is exposed to sounds that are too loud, the hair cells needed to detect sound in the inner ear can be damaged, resulting in noise-induced hearing loss (NIHL). NIHL is a major health concern, but it is preventable. In a public outreach effort, the NIDCD has launched the WISE EARS!^®campaign, where a national coalition of over 60 government agencies, public organizations, businesses, industries and unions is working to inform the public about the risk of NIHL.

Molecular Mechanisms Governing Our Sense of Taste. In humans, the loss of taste sensation can contribute to the loss of appetite and poor nutrition, a particularly common problem for older Americans. In a collaborative effort joining molecular biologists supported by the National Institute of Dental and Craniofacial Research, NIDCD, and investigators at the University of California, San Diego, candidate sweet and bitter taste receptors have been cloned and characterized. These receptors are selectively expressed in a non-overlapping subset of taste receptor cells on the tongue. This research is an important step in determining the molecular pathway activated by sweet and bitter substances, and will guide future research studies in identifying additional molecules in this poorly understood pathway.

Genetic Association and Age-Related Causes for Hearing Loss. A recent NIDCD-supported study has demonstrated that a genetic component exists for age-related hearing loss. It is likely that different mutations in the same genes that cause profound hereditary hearing impairment in children also cause age-related hearing loss (presbycusis), a common problem for older Americans. With the ability to predict who is at increased risk, better strategies to minimize or delay hearing loss within the aging population can be developed.

NIDCD/Department of Veterans Affairs Hearing Aid Clinical Trial Yields Important Results. The prevalence for hearing impairment significantly increases with age and hearing aids are the most common means of assistance for persons with hearing loss. The Department of Veterans Affairs and the NIDCD conducted a multi-center trial, which included elderly volunteers, to compare the efficacy of three commonly used hearing aid circuits. Data from the trial showed that performance differences among the three hearing aid circuits were minimal. Of greater importance, the trial demonstrated that each circuit improved speech recognition with improvement observed under both quiet and noisy listening conditions. NIDCD remains committed to support research leading to smaller and better hearing aids, capitalizing on bioengineering advances in microelectronics.

The NIH budget request includes the performance information required by the Government Performance and Results Act (GPRA) of 1993. Prominent in the performance data is NIH's first performance report which compares our FY 1999 results to the goals in our FY 1999 performance plan.

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

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