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Federal Document Clearing House
Congressional Testimony
March 30, 2000, Thursday
SECTION: CAPITOL HILL HEARING TESTIMONY
LENGTH: 3320 words
HEADLINE:
TESTIMONY March 30, 2000 JAMES F. BATTEY JR. DIRECTOR NATIONAL INSTITUTE ON
DEAFNESS AND OTHER COMMUNICATION DISORDERS SENATE
APPROPRIATIONS LABOR, HEALTH AND HUMAN SERVICES, EDUCATION LABOR HHS APPROPS
BODY:
DEPARTMENT OF HEALTH AND HUMAN SERVICES
Statement by: Dr. James F. Battey, Jr. Director, National Institute on Deafness
and Other Communication Disorders on Fiscal Year 2001 President's Budget Request
for the 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 Mathl 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. NEDCD-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
NIF2 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. NIDCDI 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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