CHILDREN OF A LESSER GOD August 6, 1998

Pre-Movie Introduction:
Dr. Bruce Fuchs:
Welcome back. My name is Bruce Fuchs. I'm the director of the Office of Science Education here at the National Institutes of Health and I'd like to welcome you to the third film in our 1998 summer film festival, Science in the Cinema. Thank you for our patience, for your patience, while we dealt with a few technical problems. It seems like we've had a different set of technical problems each week, just to keep us on our toes. We think we have the sound film... sound problem fixed. I also understand that it's cold in here tonight. Yeah, that's actually, that's not a–for those of you who use Microsoft software–that's not a bug, that's a feature.

Tonight's speaker is going to be Dr. James Battey, who is the director of the National Institute on Deafness and Other Communication Disorders. He will be speaking immediately after our film so let's go ahead and watch tonight's film, Children of a Lesser God. It stars William Hurt and Marlee Matlin. This was Marlee Matlin's screen debut and she won the 1986 Oscar for Best Actress. Let's go ahead and watch tonight's film.

Post-Movie Discussion:
Dr. Bruce Fuchs:
Good evening. We've just watched Children of a Lesser God, a 1986 film starring William Hurt and Marlee Matlin. Marlee Matlin debuted in this film and she won an Oscar for Best Actress.

We're very lucky to have with us this evening Dr. James Battey. Dr. Battey received his bachelor's degree in physics from Cal Tech. He got his M.D. and Ph.D. degrees from Stanford. He then did a residency in pediatrics at Stanford, did a postdoctoral training period at Harvard. And then I suppose he decided he started to need to pay off some school loans, so he joined the NIH in 1983, where he worked at both the National Cancer Institute and the National Institute of Neurological Disorders and Stroke. In 1995 he became the Director of Intramural Research at the National Institute on Deafness and Other Communication Disorders. In 1997 he assumed the acting director role at that institute, and last February–in February of 1998–he was appointed as the director of the National Institute on Deafness and Other Communication Disorders. So please help me welcome tonight's guest, Dr. James Battey [applause].

Dr. James Battey:
Thanks very much for that kind introduction. I very much enjoyed the movie tonight and I hope you did as well. I'll keep my remarks brief so that we can use the time mostly for question and answers.

I thought I'd share with you some of the comments of people who reviewed the movie. John Schuchman [then] from the University of Illinois noted that "in Children of a Lesser God, when James appears lost and isolated at the party of deaf people, the audience also has no idea of what the deaf characters at the party are saying, since he is not interpreting their signs."

"In Johnny Belinda and Children of a Lesser God, the dialogue is complex, but the deaf person is wedded to a hearing person's voice, which reinforces the image of the deaf person as dependent."

"Finally, the most powerful and potentially independent deaf character to appear in a motion picture, Sarah in Children of a Lesser God is a cleaning woman who is dependent on a man who earns his living as a speech teacher."

"I would have admired Children of a Lesser God more if some of its scenes had been played without the benefit of the soundtrack. If a story is about the battle of two people over common ground on which they will communicate, it's not fair to make the whole movie in terms of only one of them. Most of the people who see this movie will be able to hear, and although they may welcome the challenge of a movie about a deaf person, they aren't so interested that they want to experience deafness." That's Roger Ebert.

I'd like to now spend a few minutes telling you what the National Institute on Deafness and Other Communication Disorders is here to do. And that's to support and conduct research and research training in human communication including processes, both normal and disordered, of hearing, balance, smell, taste, voice, speech and language. In our portfolio of grants and contracts, we have studies and research on exploring the basic processes of hearing as well as grants on the acquisition of language, both spoken and signed. We have grants to support knowledge of both oral auditory strategies for young, deaf children as well as studies to try to figure out how to optimally use American Sign Language. And our portfolio includes the development of new technologies and assistive devices, including improved TTY, which is a telecommunication device to help deaf individuals communicate, and computer-aided communication devices (hearing aids as well as cochlear implants, which are devices that bypass the inner mechanisms of the ear, which I'll tell you about in greater detail in the next few minutes, stimulating the auditory nerve directly and facilitating communication and perception of sound by some deaf individuals).

We know from our studies that genetic factors cause over 50 percent of the cases of childhood deafness and late-onset hearing loss and that there is more than 300 hereditary disorders that have as part of their expression either deafness or significant hearing impairment. This is a problem for 28 million Americans who are deaf or hard of hearing. One out of every thousand infants born in this country and in fact throughout the world are [sic -- is] born unable to hear and about one child in 22 has a significant hearing problem. It's estimated that about 15 out of a thousand people under 18 in America have some type of hearing impairment and that 415 out of a thousand people over the age of 75 have significant hearing impairment, so it's a problem that affects both the young and the old.

And to conclude my part of the presentation tonight, I'd like to share with you for a few minutes how hearing takes place–what we understand about it because it's really a fascinating subject that involves some very specialized structures that are found only in the middle and inner ear where hearing happens. If you look in the upper left-hand corner, you'll see an image of the outer ear which amplifies the sounds, which then go back to the eardrum or tymphanic membrane where sound gets transmitted through three bones–the malleus, the incus, and the stapes. The third bone impacts upon a window of a fluid-filled cavity, which is shown here. The vibrations are transmitted to the fluid and then the vibrations go through this structure here called the cochleas and, although on this screen it's a very large structure, in reality it's in fact about the size of the tip of your little finger.

Within the cochlea is the Organ of Corti, and in the Organ of Corti we have very specialized cells called hair cells. The yellow are inner hair cells and you have in each row one of those and the red ones are outer hair cells and this is where the vibrations that are moving through this fluid-filled cavity in the inner ear deflect these little things that look like hairs and that's why these cells are called hair cells and it's deflection of these little stereocilia or hair-like structures that causes electrical activity to be sent from the inner hair cells back into the central nervous system. And that's how sound gets turned into neurochemical energy and impulses that are then interpreted by the brain.

It's these hair cells, and particularly the outer hair cells, that are the most common target for diseases and disorders that cause hearing impairment. And on the next slide I'll show you a scanning electron micrograph loaned to me by Bechara Kachar, one of our intramural scientists, and I think you can appreciate the very specialized hair-like structures at the tips that are responsible and maybe can get a feeling for how fragile these structures are and how easy it might be to damage these structures with drugs that harm the inner ear or very loud noises or other things that tend to cause hearing impairment.

And this is just a very close up micrograph again from Dr. Kachar, showing just the little stereocilia, or hairs themselves. And you have this lower column and upper column next to each other and they're connected by structures called tiplinks and when this bundle gets deflected those tiplinks get stretched. And that's in fact the stimulus for starting an electrical impulse going through this cell, which then results in the release of neurotransmitters and electrical signals moving into the central nervous system. And that's the end of my prepared comments, but I'd be happy to answer any questions that anyone in the audience might have, as best I can.

Question: I'm wondering–you mentioned the high rate of people over 75 having hearing problems and I'm wondering two things. First of all, of all the possible things that you sort of outlined, what is the most common cause and to what extent can these be rectified or ameliorated, at least, by any sort of device, either next to the ear or inside of the ear?

Answer: Let me start with the causes. The causes are different among different aged individuals. Among young individuals, infections in utero, infections soon after birth, or genetic causes are perhaps the most common causes for hearing impairment. Among older individuals, hearing is lost by a process called presbycusis, which is a sort of almost natural loss of hair cells that takes place as individuals age. Why it is that certain individuals lose a lot of hearing in their fifth and sixth decade whereas others go into their eighth or ninth decade without significant hearing impairment is a real interesting subject. It's one that the institute is very interested in understanding better, but it's not one for which we have the answer tonight. I hope in a few years as we come to better understand how hearing happens, that we could give a good answer to that question.

As far as various assistive devices, they really fall into two classes. One is the hearing aid, which is basically an amplifier. It takes sound and it makes the sound louder. And that's a bit of an oversimplification because it does a little more than that. The good hearing aids, the best ones available today, amplify low sound more and don't amplify the louder sound so that it makes them more effective for facilitating communication. Those are useful devices for individuals that have hearing–some residual hearing–but for individuals that have essentially no hearing at all, the device that's perhaps most assistive for facilitating their communication is the cochlear implant. And the cochlear implant is an array of electrodes that a surgeon, such as an otolaryngologist–would actually implant in that cochlear structure that I showed you and the electrodes would each be responsible for a given frequency. For example, one would do very low sounds, one would do higher sounds, higher pitched sounds. And what happens is that there's a radio transmitter that transmits into the cochlear implant after having taken the sounds in the environment and processing them. This allows individuals that couldn't hear to perceive sound again and in many of those individuals, it restores some communication skills that they will have lost when they lost their hearing.

Question: Hi, I have two questions. The first one is what is the–when someone is deaf and–what's the difference between when someone is deaf and then they can be aided with the listening device and when someone is deaf and they cannot be aided by a... I mean, what's the cause of that kind of a distinction?

Answer: Well, that's a really good question. Let me begin by first of all saying that it makes a big difference when you lose your hearing. If you lose your hearing before you have acquired language skills, then that presents an entirely different situation than if you lose your hearing later on in life. But I think the real question that you're asking is: why do these assistive devices like cochlear implants work very well on some people and not so well on others? And that is the $64 question in cochlear implant research today. And we don't know the answer, but we would hope to get it very soon, because by knowing that, we might be able to make the devices more useful to a bigger spectrum of individuals.

Question: Could hearing loss be caused by pressurized cabins of aircraft for people who are traveling? I've heard a claim by one or two people who've thought they lost hearing shortly after flying.

Answer: I'm not going to say it's impossible, but I know of no systematic study that would support that contention.

Question: In my occasional opportunities for intercommunicating with deaf people, we've used various media–some elementary signing, some finger spelling, but resort finally, when necessary, to writing back and forth. Nowhere in the film, it surprises me, was there any such occasion of communicating through writing.

Answer: I found that very interesting as well. I don't know the explanation, but perhaps I could guess that because both James and Sarah could use sign language, that they chose to communicate by that route, rather than by writing things down on a pad of paper.

Question: I think that the film is extremely beautiful and it contains a lot of water because water from one hand, it has noises like ocean, like waves and from the other hand, it's total silence, as you know, under the water. That's why the director found the best solution so to represent the dual nature of both heroes as the dual nature of water–sound and silence. And so may I ask a totally unscientific question? Where did the director find these beautiful landscapes, rocky islands, and lakes.

Answer: Unless I am mistaken, that is the state of Maine. [movie credits indicate Canada]

Question: Two-part question. In terms of children and cochlear implants, what is the effectiveness or success in children who have never heard speech and thus aren't relearning how to perceive it and what are the ethical considerations given to minors in terms of such invasive treatments?

Answer: Well, let me tackle the first question. The efficacy of cochlear implants in pre-lingual children is an area that we are just now beginning to have an adequate length of experience with the modern multichannel implants to begin to get an answer. And the answer that's emerging is that there is an enormous range in terms of how effective the cochlear implant is in improving the language skills of children whose only hearing has ever been–or perception of sound rather–has only been using a cochlear implant. We know of individuals who now have had their implants for a decade and some of those individuals can use the telephone. Those represent the people who are getting the most benefit. Others don't receive anything like that same level of benefit, and again, the big question–one of the big questions for cochlear implants and for our institute–would be to figure out why there is that range because it would be nice to be able to predict who is going to benefit and perhaps who would be better off developing another mode of communication such as sign language rather than the auditory oral route that is facilitated by the cochlear implant. The ethical considerations–they're the same ethical considerations that go into any decision that a parent has to make for a minor child as far as a procedure goes. And that's that the parent is making a decision that will affect the outcome and the life of the child and there's always an ethical dilemma there because the child is presumably not in a position at that young an age to make a decision for themselves.

Question: I'm hearing impaired myself. To follow up on that question and I do happen to have personal experience with hearing parents who have chosen a cochlear implant for their child who as a teenager in the rebellion stage, managed to figure out how to turn it off. So I think that the child will prevail no matter what the parent chooses. My question was regarding the budget. How much money do you have to work with in research and development in this area? And do you do any studies in the clinical center on campus?

Answer: The budget for the NIDCD–and this would be both for its intramural and its extramural laboratories (or research supported in extramural settings)–is a little over $200 million for this fiscal year, making it one of the smaller institutes here at the NIH. We do have a component in the clinical center. Our intramural research program has a clinical arm to it and right now in that clinical arm, we have three principle investigators, one of whom studies the pathogenesis of head and neck tumors, and one of whom studies the brain activity patterns associated with normal and disordered language, and a third who studies normal and disordered processes of voice and speech.

Question: I guess you've sort of talked a lot about the medical and scientific issues at the heart of disordered hearing, but what are some of the culture issues that you've encountered in your time? I think that there's been a lot–I read an article in the Atlantic Monthly a while back about decisions regarding deaf parents who make a decision to raise their child deaf. Do you have any comments on the cultural issues that you've encountered?

Answer: I think the culture issues are very important. They underscore the fact that there is no one size that fits all for communication among human beings and that in different families and that in different settings and different circumstances, different ways of promoting language skills and communications will work better.

Perhaps in a family where we have a deaf child born to parents that can't hear, that American Sign Language would be the preferred route for promoting communication for that child. The institute's point of view is that what's critical is language skills and communication. And the way in which those language skills and communication happen is much less important than that they do happen--they happen at an early age so that the child can develop a full spectrum of language and realize their full potential.

Question: How effective would this cochlear implant be for a person who is 97 years old, they have given up on hearing aids on her, and I just wondered if there is any possibility this might help and how invasive a procedure would it be for her?

Answer: Let me begin by addressing the issue of how helpful it might be. One thing we know about cochlear implants in post-lingual people–people who have developed language skills and who have lost their hearing as a consequence of age–is that the longer the duration of time between when the hearing is lost and the implant is activated, the less likely the cochlear implant is to work well. And the reason for that is that the central auditory pathways, if not stimulated for long periods of time, essentially atrophy and you lose them. So it would depend on how long the individual had been without hearing in terms of how well one might expect a cochlear implant to work in such an individual. As far as the invasive nature of the procedure, it involves making an incision behind the ear and threading the electrodes into that spiral structure that is the cochleas, then healing the wound back up again and using a speech processor to convert the sound energy and speech into radio frequency signals, which get sent into the implant itself. I'm not a surgeon myself, so I don't know how difficult the operation is to do, but my colleagues who do know how to do this say that it's pretty straightforward surgery.

Question: I'm interested in the other communication problems part of your title. I have a hearing loss, but it's complicated by the fact that I also have a process problem. That is, sometimes I will hear someone say something and I will think that I did not understand it or I did not hear it, but then about one or two seconds later, all of a sudden without them repeating it, I will understand what they said. What is happening in your institute in work on that?

Answer: We have millions and millions of dollars in grants and contracts to understand what happens to sound, or the perception of sound, within the central nervous system after it leaves the ear. We believe that problems in the rate of auditory processing may be absolutely central to other forms of language disorders and language appreciation problems that people might have and in fact are supporting investigators to explore that avenue of research right now in 1998. So we think that's a very important area.

Question: I'm wondering about deaf education and how it's changed in the 25 years since we saw it in the film.

Dr. Battey: In what respect, changed in what respect?

Questioner: I'm wondering if it's the same–if it's still like what we saw in the movie or if there are new ways that they teach or.....

Question: I have heard that the sign language as used by congenitally deaf people has many characteristics of a language...in its own right. Has this been studied?

Answer: Well, it's a very rich language and it allows people to express themselves very clearly, and the institute is certainly investing a good bit of its research energy towards optimizing the communication skills that are possible using sign language and specifically American Sign Language, which is the form of sign language used in this country.

Dr. Bruce Fuchs:
Before I ask you all to help me thank our guest speaker, I will invite you to come back next week when our film is going to be As Good As It Gets, a popular Academy Award-winning film from this year. We are expecting our largest crowd, so I would give a hint to those of you who are here, come early if you want to sit down here. And I would now ask you to help me thank tonight's speaker, Dr. James Battey [applause].

Dr. James Battey:
Thank you.