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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