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The Institute supports ongoing research in otitis media as it is the leading

cause of acquired hearing loss in children under the age of ten.

We were heartened

by the release of a new vaccine for infants to prevent meningitis, a major cause of

deafness in children.

Congenital cytomegalovirus (CMV) infection is a well-established cause of

disorders of the auditory, visual and central nervous systems.

The NIDCD will

continue to support research on the CMV in association with disorders such as

infant hearing loss, Meniere's disease, perilymphatic fistulae, sudden deafness,

and hearing and balance disorders emerging in AIDS patients.

CMV is the most

common secondary infection among AIDS patients and is estimated to occur in more

than 90 percent of all such patients.

Persons who have AIDS are known to develop

communication disorders the most prominent of which is loss of hearing. We are

studying whether the hearing loss is a result of the AIDS virus or the CMV.

A

model for studying congenital CMV inner ear infection has been developed, and

therapeutic strategies have evolved for the prevention of the loss of hearing from

CMV infection.

Additionally, the NIDCD is concerned with CMV in children.

Approximately

72,000 children are born with CMV each year.

CMV is the single largest cause of

acquired, congenital hearing loss and is estimated to cause deafness in 2,000 to

4,000 infants annually in the United States.

In September 1990, the NIDCD held the first of a series of working group

meetings designed to look at the needs of the several constituencies served by the

Institute.

Each working group has an opportunity to inform the Institute of their

special research and research training needs.

The first working group provided

perspectives of the deaf community; the second, scheduled for April 1991, will

provide the perspectives of oral, auditory hearing-impaired persons; the third, to

be held in October 1991, will address the research and research training needs of

women and women's health issues.

The fourth meeting, planned for April 1992, will

present the needs of minority persons and minority health issues; and, the final

meeting in the series, scheduled for September 1992, will assess the impact of

visual impairment on deaf and hard of hearing persons.

The Institute's National Strategic Research Plan has identified that there is

insufficient incidence and prevalence data on communication disorders.

After

holding planning meetings with the National Center for Health Statistics (NCHS),

the NIDCD is preparing a 10- to 15- year epidemiologic research strategy.

This

strategy in collaboration with the NCHS will yield incidence and prevalence data in

the Institute's seven program areas and will provide reliable and cost-effective

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data in a systematic and comprehensive manner.

The NIDCD is establishing an

Epidemiology Branch in FY 1991 to coordinate these efforts.

The NIDCD is continuing research and development of devices to improve the

quality of life for hearing-impaired persons.

The Institute has made a major

investment in improving technology of auditory prostheses for the deaf.

A new

interleaved-pulse speech processor, developed and tested by NIDCD supported

investigators, is capable of sampling the speech signal at high rates for use by patients fitted with multichannel cochlear implants. The processor provides

impressive gains in understanding speech so important to these individuals.

The

cochlear implant program has, developed limited clinical trials of auditory

prosthetic devices which have promise for major progress for individuals with

congenital and acquired hearing impairment. Furthermore, the application of

patterned electrical stimulation appears to help maintain the integrity of the

central auditory pathways when the inner ear has been damaged.

Another device, the

digital hearing aid, is being studied with great intensity.

NIDCD will continue its initiatives in support of minority-related research

and minority scientists.

In several NIDCD-supported clinical trials on otitis

media, specific attention has been devoted to minority populations. For reasons

not yet clear, there is a higher incidence of otitis media among minority

populations.

Although all clinical trials supported by NIDCD include both males and

females, recent findings in diseases that affect women differentially include

Meniere's disease, otosclerosis, and voice tremor.

Currently, NIDCD is supporting balance-related research on the micromechanical properties of inner ear sensory cells that may explain how these cells detect forces acting upon them during movement of the head and transform this information

into neural signals.

Basic scientists studying the sense of smell have found an indication that

some forms of Parkinson's disease and Alzheimer's disease may be caused by

environmental agents that enter the brain through the olfactory nerve.

Recent research results indicate that normal language learning occurs only

when exposure to the language begins early in life for both signed and spoken

languages.

In language research on adult aphasia, recent findings suggest that the

immediate administration of Type I gangliosides in stroke patients may prevent

gross aphasia.

NIDCD investigators continue to study the onset and development of stuttering

in early childhood.

Preliminary results show that girls have a significantly

earlier onset, were more likely to have sudden onset, and show a stronger tendency

for amelioration than boys.

NIDCD intramural scientists have demonstrated that botulinum toxin injections

are effective in reversing the effects of muscle reinnervation and restoring the

voice in the treatment of spasmodic dysphonia.

The first three National Multipurpose Research and Training Centers have been

established.

The Centers will stimulate important areas of basic and clinical

research while providing needed research training opportunities. They will increase the numbers and broaden the capabilities of investigators in the

communication sciences.

Continuing education programs will disseminate research

results to physicians, other health professionals and the public.

As we approach the new century, the age of communication is coming into maturity. Every aspect of human life is dependent upon skilled communication.

Communication processes and technologies have become increasingly critical to

success in the workplace.

Persons with disabilities have been guaranteed the

rights they have always deserved.

However, when a child is born with a

communication disorder or acquires one later in life, that child faces undeniable challenges throughout his or her lifetime. Often the challenges are economic. The

earliest possible diagnosis and intervention are critical to improving the quality

of life for individuals with communication disorders.

Research in molecular biology and, specifically, molecular genetics holds

great promise for the use of gene therapy in hereditary deafness and other

disorders of human communication that are clearly hereditary.

NIDCD has an ongoing commitment to research that develops strategies and devices to ensure the best possible outcomes for individuals with communication disorders. To the degree that these individuals are able to improve their

productivity and independence, research will have contributed to major savings and,

more importantly, to the quality of their lives.

It is of the greatest importance

that the age of communication is truly accessible to all Americans.

Mr. Chairman, the FY 1992 budget request is $146,321,000.

I will be pleased

to try to answer any questions you have.

BIOGRAPHICAL SKETCH OF DR. JAMES B. SNOW, JR.

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Education:

B.S., University of Oklahoma, Norman, Oklahoma, 1946-49;
M.D., Harvard Medical School, Boston, Massachusetts, 1952-56

Professional History: Intern in Surgery, Johns Hopkins Hospital, Baltimore,
MD, 1956-57; Resident in Otolaryngology, Massachusetts Eye and Ear Infirmary,
Boston, MA, 1959-60; Captain, Consultant in Otolaryngology, 1960-61; U.S.
Army Medical Corps, 8th U.S. Army, 121st Evacuation Hospital, Korea;
Otolaryngologist, U.S. Army Hospital, Fort Leonard Wood, MO, 1961-62;
Assistant Professor and Vice-Chairman, Department of Otorhinolaryngology,
University of Oklahoma Medical Center, Oklahoma City, OK, 1962-64; Professor
and Head, Department of Otorhinolaryngology, University of Oklahoma Medical
Center, Oklahoma City, OK, 1964-12. Adjunct Professor, Department of Oral
Medicine, University of Pennsylvania, School of Dental Medicine, 1974-17;
Professor and Chairman, Department of Otorhinolaryngology and Human
Communications, University of Pennsylvania, School of Medicine, Philadelphia,
PA, 1972-89; Director, National Institute on Deafness and Other Communication
Disorders, Bethesda, MD, 1990-.

Society Affiliations: Alpha Omega Alpha; American Academy of Facial, Plastic
and Reconstructive Surgery; American Academy of Otolaryngology-Head and Neck
Surgery; American Association for the Advancement of Science; American
Broncho-Esophagological Association; American College of Surgeons; American
Laryngological Association; American Laryngological, Rhinological and
Otological Society; American Medical Association; American Neurotology
Society; American Otological Society; American Society for Head and Neck
Surgery; Association for Research in Otolaryngology; Association of American
Medical Colleges; College of Physicians of Philadelphia; Collegium Oto-Rhino-
Laryngologicum Amicitiae Sacrum; International Bronchoesophagological
Society; John Morgan Society; Massachusetts Eye and Ear Infirmary alumni
Association; New York Academy of Sciences; Pan American Association of
Otolaryngology and Broncho-Esophagology; Pennsylvania Academy of Opthalmology
and Otolaryngology; Pennsylvania Medical Society; Phi Beta Kappa ;
Philadelphia Laryngological Society; Sigma Xi; Society for Ear, Nose and
Throat Advances in Children; Society of University Otolaryngologists-Head and
Neck Surgeons; Southern Medical Association.

Honors and Awards: Regent's Award for Superior Teaching, University of
Oklahoma, 1970; Certificate of Award, American Academy of Opthalmology and
Otolaryngology, 1971; Honorary Master of Arts, University of Pennsylvania,
1973; Second Vice-President, American Academy of Ophthalmology and
Otolaryngology, 1973-74; Consulting Professor, Shanghi Second University of
Medical Sciences, Shanghai, People's Republic of China, 1985; Honorary
Fellow, Japan Broncho-Esophagological Society, 1985.

HEARING LOSS

Senator HARKIN. Thank you very much, Dr. Snow. Millions of Americans have different there's different kinds of hearing loss, and some are affected with a hearing loss that if you are tested in the booth, the sound chamber—I don't know what you call that, but the testing room.

Dr. SNOW. Yes.

Senator HARKIN. Your hearing comes out OK. But if you are in a crowded room or something like that and you are talking to someone, you cannot understand them because of the background noise. I have become aware of this, in fact, having a little bit of a problem with it myself.

But I am just wondering what kind of research is being done in this area. What kind of new hearing aids are being developed? I have heard about some, but I don't

really have much knowledge about what is being done in that area.

Dr. SNOW. Yes, sir; we all have trouble hearing in background noise but, of course, this becomes very severe, particularly in those who develop the loss of hearing due to aging, the condition known as presbycusis. And then when one tries to solve the problem of loss of hearing with a hearing aid, just the thing that you point out, the variation among individuals becomes a very major problem.

One of the hopes is that digital hearing aids will help solve this problem, and we are sponsoring research in which hearing aids are being tested both in quiet and in noise, in which there are men's voices in noise, women's voices in noise and each of those in quiet. Our research in hearing aids really is addressing three major approaches: The amplitude compression, the reduction of noise, and the use of binaural hearing aids, each one fitted differently to each

ear.

DIGITAL HEARING AIDS Part of the problem with the development of digital hearing aids has been that one can work out strategies for different listening circumstances for a given individual, but there is not a great deal of application to other individuals. And that has hampered the production of these for widespread use. What has been worked out in the laboratory for a few subjects is applicable to less than 50 percent of the population that needs a hearing aid, and this makes it a very difficult and complex matter. We are emphasizing the study of the amplified speech signal in a hearing aid and the characteristics of the hearing impairment, trying to match those better than has been done in the past.

Senator HARKIN. One last question. I have a number that I will submit to you in writing.

I am informed that the Defense Department has been doing a lot of classified funding of research and development in certain kinds of equipment, such as eavesdropping equipment, that if available to those working on improving the quality of hearing aids, could lead to some dramatic breakthroughs in quality and sophistication of hearing aids. In other words, the Federal Government has already done some research in another branch on this.

Have you had any discussions with the Department of Defense on this? Are you aware of this?

Dr. SNOW. No; we have not. We are developing the interagency coordinating committee that was authorized by the legislation so that we can be aware as much as possible of what other Government agencies are doing in deafness and other communication disorders. I do not know anything about the classified work.

Senator HARKIN. I also serve on the Armed Services Appropriations Subcommittee, Defense Appropriations. And I am going to get my staff to look into that and try to coordinate with our staff here and with you, and let's see if we can't get you the appropriate classification, whatever you need to look at this. OK?

Dr. Snow. Yes, sir.

Senator HARKIN. I am not certain that I am totally aware of it. I have been told about it. I don't have a firsthand knowledge of it myself, but I would like to have you take a look at it and see if there is anything there.

Dr. Snow. I would be very pleased to do so.

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