Copyright 2000 Federal News Service, Inc.
Federal News Service
February 16, 2000, Wednesday
SECTION: PREPARED TESTIMONY
LENGTH: 2173 words
HEADLINE:
PREPARED TESTIMONY OF DR. JAMES F. BATTEY, JR. DIRECTOR NATIONAL INSTITUTE ON
DEAFNESS AND OTHER COMMUNICATION DISORDERS
BEFORE THE
HOUSE COMMITTEE ON APPROPRIATIONS LABOR, HHS AND EDUCATION
SUBCOMMITTEE
BODY:
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 heating process, are often damaged or lost as a consequence of noise,
genetic mutation, drags or other environmental insults. The resulting heating
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-Heating 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 heating screening. This study was the first controlled
comparison of normal heating 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 heating screening becoming available to all newborns,
breakthroughs in medical genetics will enable scientists to identify the precise
genetic change leading to hereditary heating 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.
END
LOAD-DATE: February 26, 2000