Signal which is eventually something the brain can understand. The third idea is a bit difficult, and this is to understand that each sound is actually made up of a lot of different sounds, a lot of different tones that happen simultaneously and for the brain to understand a sound it has to break the sound into its different parts and then those different channels of information have to go to the brain separately. Rose and when something goes wrong, what happens . When something goes wrong and we might return to this of things that happen and really we should return to it later. And maybe i should continue. Rose okay, continue, you have some slides. And let me show you quickly where this happens in the head. So the outer here carries the sound into the eardrum and then its carried through the middle ear to finally reach the inner ear where the cochlea is. The cochlea is the organ that does this conversion to a neural signal and then the auditory nerve carries that signal to the brain. Here you can see sound waves being collected by the external ear and theyre carried down the ear canal to hit the eardrum. When the sound waves hit the eardrum, it makes it vibrate and this vibration is carried by three tiny bones in the middle ear into the inner ear where the cochlea is. It will inner ear a actually has organs for two different senses. On the right is this snailshaped cochlea that gives us our sense of hearing and the tubular structures on the left give us our sense of balance. To understand how the cochlea works, we can zoom in in much more detail and we can see that theres a special set of about 15,000 cells that do this conversion to neural signals. Theyre called hair cells because if we zoom in even further you can see each hair cell which is about a thousandth of an inch long has a tuft of special sill ya coming from the top. And then if we look closer we can see how the membrane which goes over the hair cells contact it is tip of each bundle of krill ya and when sounds makes the membrane moves it moves the sill ya back and forth. And nerve impulses go go to the brain. So this is an interesting process but theres still something that we dont see about the process and that is how the vibration of the cilia gets converted to an electrical signal. The chip of each psyllium has a couple of key elements. Theres a very fine tip link that connects the tip of one cil yum to the side of the other one if we get a sense of how this moves we can do the conversion. Its davids work who elucidated this process to a great degree. So the next image would show what happens when found with these bundles. The movement of the bundle to the left in which case the pulls the gates of the pores open and then electric charges will be able to move into the cell and they make the inside of the cell more positive, the voltage changes and so thenangeo nerve impulses and thats the magic of the conversion of the vibration to the nerve impulse. So so the third things that that is special about the way a ear works, its not a lot of sound but different sounds that happen at the same time and theyre changing and for the brain to tell the difference between a song and a childs cry it these listen to them. And the separation of frequencys is carried out by the cochlea. Basically the cochlea, it sounds all at the same time but the oak leia are activated by different sounds. And hear you can see one lowpitched sounding a separates one area, a higher pitch another area, and then the nerve fibers that are attached to that area then send the information to the brain or if you played several tones at the same time like a cord hear you could see that several nerve fibers are activated all at the same time. So the cochlea does this separation in into different frequencies and this is the essential thing the cochlear implant these do if its the replace the function of the cochlea. Rose so how did you get involved in the cochlear implant . It all started quite a long time ago and there were prior activities in the cochlear implant field so im kind of the second cochlear implant researcher and i have a big interest in helping people through technology wh from when i was 13 years old. Rose tell me more. How the cochlear implant started . Well, it started really with activities in the late 50s and early 60s in france and in california and at that Time Research was done and then the like singing wire devices were used that gave people an aid to liberating but didnt lead to open set speech understanding without lip reading. The signal pathways like you were showing before. Yes. So in the late 70s the multichannel implant came along and it was first implanted in 77 in vienna, austria and in australia a little later and the other award winner is graham clark from Melbourne University and in the beginning with these multichannel devices there was some patients who spent ours and weeks in the lab to held with us psychophysic experiments to find out which signals we should send to the conduct of the electrodes and then in 1980s the first patient by the name all of a sudden because we had changed the way the signal was sent to the electrodes understood sentences and words without liberating and it was right on the birthday of her brother who had also gone deaf. So at that point in time we knew it worked. We had to make many, many people make it available and then the early 90s a third award winner, blake wilson, published a speech coding strategy, how to encode the signal from the microphone that brought another step in performance, increasing performance and made the speech perception more robust to a noise. And since then further improvements have taken place and the cochlear implant that has the advantage that it really doesnt need a loud speaker in the ear canal and thats why we were able and our able to improve things in terms of how they blend into everyday life for the users. Right now there is even a processor that doesnt have to fit on the ear because we dont need a loudspeaker in the ear canal can be off the near and not in the conflict with glasses even. So a cochlear implant consists of two components, an external audio processor and an implant and the implant is surgically placed and then afterwards the surgeon inserts a long flexible electrode into the snail shaped cochlea and thats done to be able to restore a wide frequency range because each of these electrodes stimulate a different part of the cochlea and sound comes into the audio professor, its transmitted through the intact skin to the implant and then small electrical impulses are generated that stimulate the auditory nerve. And thus the function of the inner ear sr. Bypassed because the hair cells dont function anymore. This is so remarkable because this is first example in which any sensory organ can completely be substituted for. Its absolutely extraordinary. Rose go ahead. And what were going to see now is very special because after the implantation a few weeks later theres a switch on of the audio processor and going to see this on a little child who has never heard before in his life who has not heard his mothers voice. Hey, good job its on. I know hi, cooper hi, cooper hi baby laughing hi, cooper rose wow. One out of every 400 children is born deaf. Thats just amazing there s there a figure worldwide of how many children each year are born deaf. Yes, each year there are 134 million children born and and one in a thousand of those has a real hearing deficit. If. Rose max, i want to turn to you. This obviously helped you. laughter it helped me a lot. Rose tell me what it meant to you. Well, i couldnt tell this to you exactly because ive got my first implant at two years and so i learned my life with hearing. Phenomenon two years you dont really realize your life, you dont and so i have had a usual life. I went to school and to kindergarten, of course, and i go to university now and its an unusual life for me and i think without the implant i dont know exactly what id where i would be now. But it would be completely another life and i would have to learn sign language and i would have it much harder, of course. I dont know exactly if you can imagine how it is if you dont hear. Rose what is so wonderful about the cochlear implant as we see with max is he doesnt have to do lip reading, he doesnt need visual cues, he hears like you and i do. Rose and how long does it take to train . I dont know exactly. Of course i got my first implant at two years that was really, really close because i think with two years its really late to start learning language because over five years its too late. Its not too late but after five years its really late so its very hard but for me i got to the normal kindergarten and school and i learned it fast because i had learned normal language. Charlie, what max is expressing is that he basically had a normal childhood growing up and he took on the context of how it used to be before cochlear implantation before children were born deaf and 90 of children born deaf are born to hearing parents. They were sent to a Residential School to learn sign language so their entire life they couldnt communicate their own parents unless the parents learned sign language and if you dont develop verbal language early on we saw even 20 years ago that the average 18yearold child learning american sign language, they only read at a fourth of fifth grade level and thats mainly because that ability to speak and understand you can imagine literacy is such an important skill for a foundation of the rest of your life. So what max is saying now that going to just a Mainstream School like any other kid is remarkable bauds its completely different 20 or 30 years ago. The revolution treatment of hearing disorders in the last 20 years is remarkable. Its like a war of deaf. Is. Rose tell me about the elderly. The amazing thing about hearing loss in older adults is how common it is. If we look across the life span you see the prevalence how common a significant hearing loss is, it doubles with every age step of life. So what that means is that by the time people are about 70 years old nearly twothirds two of every three people over 70 has clinical hearing loss. Because in a way it used to be seen that, well, if its cho so common how could it be important and increasing the last years we realize thats not the case. People who Vice President age loss just like we will all have as we age it quintuples your risk of dmaepl shah in some cases. It increases your risk of progressing disability and requiring institutionalization and were realizing that this is probably through the direct effects of hearing loss. The most intuitive ideas that hearing loss contributes in some way, shape and form to social isolation and the amazing thing about that is if we believe that hearing loss contributes to some loss of social engagement, thats fundamentally something profound. Going back over a hundred years now that pathway exists for social isolation, we know hearing loss it leads to a load on the brain. The brain is constantly having a very, very degraded auditory message from the ear. The brain needs to rededicate Processing Resources to deal with that impoverished sound. The third idea to which hearing loss direct these outcomes in later life is we see hearing loss can lead to changes on the brain. Parts of the brain that are important for language and speech processing are also important for memory. If its faced with the impoverished auditory signal from the ear is you literally seeing parts of the brain atrophying faster as we develop hearing loss. Let me turn thattohearing aids, tell me how they work. Well, in fact, we have had hearing aids around since the beginning of time i imagine and in that people have done this since the beginning of time. And probably in the 1800s more than prior to that we had the socalled ear trumpet. This particular picture is of a typical ear horn, ear trumpet. Davids mother, speaking to her grandfather. So the grandfather in this case that has hearing loss and so we have no electronics id like to point out here but rather a way by way of speaking to just to get the signal up to the ear. If we go to the next slide you see a whole collection of beethovens, ear trumpets or ear horns. So before we got into the age of electronics we have choices in terms of that collecting of sound like i showed you in the beginning weve been doing but collecting it into the device and bringing it into the ear. Fortunately beginning about 1901 we started having access to electronic hearing aids and in reality those first electronic hearing aids were nearly as big as this table and eventually they got more smaller sized and so on. We went to body style nadz the 40s and 50s, ear level aids in the 60s at least and these days hearing aids are pretty excellent i would like to point out to be positive before we look at how a hearing aid works. All hearing aids, even since the beginning of electronic hearing aids at the turn of that century all hearing aids had to have a place to pick up the sound, that microphone. They had to have something to amplify the sounds so it would be louder and then the sun was set by these loudspeakers into the ear canal. Basically thats still the style or the makeup of a hearing aid, again, because we have had such changes in technology in the last ten years or so that amplifier you see there is now a electronic chip. And as i often times point out to patients that electronic chip is a computer sitting up at the ear so it can analyze sound, it can determine what will be the amount of amplified sound coming out and so on. Again we have accomplished what many of our persons with hearing loss help us to accomplish and that is to make them less obvious and thats kind of a thing we fight in this country more than in some countries of the world. And technologically weve come a long way. Hearing aids have signal processing schemes that are pretty advanced. There are noise reduction schemes that happened digitally, there are microphones that come off and on and so and and as you eluded to earlier, charlie, and you eluded to, most of the people with hearing loss in this country cant use a cochlear implant so their choice is to either seek no help or seek help via a hearing aid. And unfortunately for us i guess only about 25 of those people who could use and should use a hearing aid are doing so. Rose why is that . Because of vanity or Something Else . A variety of reasons. Thats part of what what my research has been looking into. Why do people reject hearing aids . But vanity is a big one. Cost is always a big one. I would say probably the bigger reason for rejection might be because people who seek hearing aids want those hearing aids to help them in difficult listening situations and that means noise in the background. And hearing aids in a background of noise, hearing impairment in abackground of noise is difficult at best. And so we still dont have we dont a way with that particular design of hearing aid to make hearing in a background of noise easy, better and all of the above. So in terms of technological advances, i would say the newest advantage in hearing aid has oz the do with all of the Wireless Connectivity we have available. So even if we were wired here, it is a Wireless Connectivity. Were wearing a microphone thats being projected in some receiver somewhere. That is an option for people. That is an option for people with their hearing aids. Hearing aids because theres a chip in each one can kind of talk to each other and kind of take in information from a computer, from a t. V. Can hear a speaker or a talker who happens to be leading a discussion or leading a Church Service or whatever. Because wirelessedly talkers voice can be projected to the hearing aid. Rose can all of us lead to some having been able to delay whatever might be in terms of the cognitive losses seen with hearing loss in the elderly. Charlie, thats the key question. Until now when i put on my happen as epidemiologists, these results are fascinating, increase our risk of dementia and memory abilities over time. With the key question Going Forward that we treat hearing loss well with hearing aids and Cochlear Implants, can we delay the risk, can we delay the onset of dementia and right now as we the evidence we just havent done the studies quite yet, we know now definitely for many people hearing loss clearly improve social engagements but terms of the longer studies looking at over time can reduce the risk of cognitive dementia, those are studies were just beginning with the National Institutes of health with Clinical Trials. Rose where are you look at this and you have to be excited about the recognition of your work. What other cutting edges are things that are happening in the field of hearing loss . One of the things to point out is that the cochlear i plant does a beautiful job of replacing the function with the cochlea. What wed like to be able to do is restore the function of the cochlea. And so many of the different causes of hearing loss whether theyre hereditary or caused by loud noise end up killing those little hair cells in the cochlea. So something a lot of people are working on is to try to figure out a way that we could regenerate the hair cells. And the hair cells are interesting because youre born with about 15,000 of of them. Thats all you get. If you lose them, you get no more. If you cut your skin, new skin cells grow. If you kill your hair cells no more ones grow. But if a hair cell dies, then theres still cells reminding behind and if you could figure out what turns a cell into a hair cell, what are those instructions that you might be able to put those instructions into the remaining cells and turn the remaining cells into hair cells. This would be a hair cell regeneration. There were a lot of laboratories around the country that are trying to do this. First figure out what makes the cell a hair cell and then figure out one what are the factors and how to deliver them into the cochlea to regrow hair cells and restore the function of hearing. Rose where are we on gene therapy . Gene therapy ten years ago got kind of a bad rap because there was a lot of work, people were very excited and perhaps patients demanded a little too much progress too quickly and there were some unfortunate case i was saying the last two or three years theres notable successes in gene therapy. Meaning in my area theres been a tremendous use of gene they areny the retina and examples where replacing a defective gene in the retina with gene therapy has restored at least functional vision. Now gene therapy for the inner ear is a little further off. Theres been in the last year or two two striking examples of success where gene therapy has restored at least some measure of hearing in animals. But there are no human Clinical Trials and thats something other groups are working on. Rose why not . Its just too early. We need to make sure theyre very safe in animals before you put Something Like that that into a human. And what do we know that from tests that have been done from animals so far. The tests have gone into animals specific type of hereditary hearing loss and they have either correct add defective gene or replaced a defective gene. And when they measure the hearing of the animals after a certain number of weeks and they can see theres a great deal of function. Rose with respect to hearing aids, the question is its restoring hearing has a pervasive effect across both your life and your health. So the question really becomes how do you correct if you have mild hearing loss so people dont simply wait until theyre almost deaf in order to get some kind of corrective whether its a hearing aid or what it might be. Give us the sense of where we are which is correcting mild hearing loss. Yes, and back to the hides dhishgs a pretty good job of that. I think part of what i was trying to say before was that theyre not always received as positively as they should be by the persons who could obtain a lot of benefits. Rose there are a lot of people who wear hearing aids and they make no they readily say its made a difference in my life. The one thing i would put out is rose including former president s. Yes, the ones that used this publicly. Hearing aids are called aides because thats what they are. An aid to hearing. And, again some of the negative vibe that goes with having a hearing aid has to do with the fact that it doesnt correct a hearing problem. When we have a cochlear implant that can do the stimulation in the right place in the cochlea to present the signal its sort of a solution. What im doing here is putting on an amplifier and i can shape the sound coming out of the amplifier to accommodate the hearing loss. Hearing loss can be shaped in all kinds of ways but it doesnt cure anything. Its extremely important to treat early because you participate in the conversation. If you cant follow it, you drop out and this is a common feature, the social isolation, and the Cognitive Impairment that may be directed to it and although we dont know the result its possible that having good hearing aids may prevent that. Good hearing care, i would like to say. Part of it is just making sure that you have somebody who understands all of that. All of this were talking about. So theres the aud logic care, sometimes the simplest devices and so on just to make it so the hearing is improved. Rose this is an important point for us to make, that people should have themselves tested from 55 on and if they need hearing aids they should use them. Yes, one shouldnt wait too long, neither with the hearing aid nor to get the cochlear implant. In fact most of the Cochlear Implants recipients now have some hearing left, considerable hearing and its our task to be really gentle with flexible electrodes and careful surgery to enable, for example, little children who might have a Life Expectancy of 120 years now of the in the future be able to pro fit from technologies or therapies we dont know yet that were mentioned or even some others were rose the powerful impact cochlear implant has on children. What stands in the way of making them available to so many more Young Children . Whats the issue that prevent mrs. Cochlear implants in children . Globally its a factor five thats missing from whats being done today which is 25,000 per year. We would need five times as more to enable every deafborn child for a year on earth to receive a cochlear implant. Is that the issue in . The cost. Its expensive but also awareness thats lacking and infrastructure in some countries and we will make them cheaper as economies of scale come into play. Rose so whats the cost element today of a cochlear implant . It depends on what they are but approximately you can get one for 15,000. Rose 15,000 . Thats really the lowest price because its not only the implant its also the work at the clinic before to identify the child and then the surgery and the therapy. Rose for one or both ear . This would be for one ear. So 30,000. And, if its both years in fact, max was the first child implanted in both ears makes a big difference to localize sound for example. And you hear better in noise and you have a much easier time at school because those children who are deaf in one ear theyre usually they usually miss a year and have to repeat the year they have a harder time to concentrate. Rose so frank i make you the czar for hearing. What are you going to do . Give us your Mission Statement and mandate and agenda so i think the big thing overall is we talk about low rates of hearing, we talk about Expensive Technology and you realize its all sort of true that overall the big picture that fupldmently now as a society were understanding how that hearing and the ability to communicate effectively is its fundamental to everything we do. So as we age, as we develop agerelated hearing loss the goal is to make sure we can functionally be at our best every single day. And i think unfortunately thats a lot of people dont understand that yet. So i think one big aspect is we need thirdparty reimbursement for Hearing Health care overall. I think there needs to be a much broader understanding across the whole society that hearing and Hearing Technologies and being able to hear effectively is integral to what we do on a daily basis. I think there needs to be a lot of changes on health care. If theres the perspective this that if you have hearing loss and you get a hearing aid that youre cured. And hearing a lot of times isnt enough. We need hearing loop induction technologies so we can transmit sounds wirelessly. We can go back and forth to audiologists and that can be a large standard but for a lot of with us moderate hearing loss, we may not be able to make that may not be able to go through that. And the last big thing for all of us 20 or 30 years from now when dwip hearing loss, too, one of the Big Questions that remains unanswered is what is the impact of treating hearing loss in older adults . Does it justify the costs . Can we reduce the risk of dementia over time . This topic is timely. Rose and those metrix would be powerful. Thats why just in the few months we have institute of medicines which is major arbitor of u. S. Government health care policy, theyre taking on this topic and hosting a twoday workshop in january on tissue of hearing loss and Healthy Aging. What can with we do to address hearing loss now, to preserve Healthy Aging for all of us as we go forward. You not only increase agerelated memory loss, it increases the incident of alzehimers disease so if you can prevent the incidence of alzehimers disease or delay it with good hearing devices thats a Major Public Health advance. Rose so what what would be on your wish list, david . The hair cell regeneration . But keep in mind a lot of hearing loss doesnt have to happen. Some of it is the natural process of aging but a lot is the terrible thing dwos to our ears. Exactly. The sound and the environment. In fact, if you took the amount of sound traces that a chain saw puts out, in two hours that would completely destroy the hair bundles. The sound makes the bundles move back and forth and tears them apart. And the hair cells can recover to some extent but the you do that over and over again and there are plenty of guys who use a chain saw two hours a day the hair cells die and you never get them back. And we worry also about personal music players like ipods. You can crank those up as loud as a chain saw and theres the possibility of causing hearing damage. Rose so people might be doing damage to themselves. The yes, at 15 or 20 years old. I know people who have headphones all the time in broadcasting and complain they suffer hearing loss. So where is the db next whats the next thing to happen do you think . Next break through. I would stay next break through is in learning to regenerate the hair cells. Rose how close are we today . Its coming along but i would say in ten or 20 years when somebody goes into the clinic with hearing loss they would probably get a biological treatment of some sort. Not next year, not the year after, but 10 to 20 years. Rose what does that mean biological treatment . That would be some kind of gene therapy that might bring those instructions into the other cells. Rose stem cells . A lot of people difficulty with stem cells is its difficult to surgely chri implant them into the ear to get them to go into the right place and turn into hair cells. So we tend to use stem cells in a different way just to use them to figure out what terms a cell into a hair cell and use those factors that are in the cochlea. Just in the last few years as people recognize that hearing loss is a big critical Public Health issue that some Major Pharmaceutical Companies pfizer, sanofiaventis theyre developing pipelines to see if they can develop therapeutics for hearing loss. The holy grail is regeneralive the process but pfizer is in an early phase of a drug that could boost hearing on an everyday basis. Its too early to say whether it will work but its a phase 1b study. I think even among the Big Pharmaceutical Companies now, until now hearing loss was under the radar screen. Just in the last couple years with population and graphics and the importance of hearing loss and cognition theyre recognizing that there may be a pharmacologic target that may be improving hearing similar matally. It sounds far fetch bud going back 20 or 30 years there have been more farfetched you mean finding a channel and making it conduct effectively. Well, they either function peripherally to boost the signal or to help with auditory processing. These are early phase drugs that are just being tested for the first time in humans but its encouraging when you have big pharma coming into the picture in a good way investing in scientists, investing basically the money to basically look for the solutions, theres no reason why it couldnt happen. Rose what would you like to see done . Perhaps youve all eluded to this awareness. We talk about rose awareness and answer the . Awareness and then ultimately acceptance. Whether its the young person, ill mention something about that, or the older person, lives change because of use of some prosthetic device. Whether its cochlear implant for the more severe or hearing age for the less severe hearing loss lives change and so more awareness of doing this thing called getting your aud logic evaluation and see whats up. I would like to mention the other endoftheage spectrum, the Young Children who have mild hearing loss are at a disadvantage even though they have a mild hearing loss. That mild hearing loss does not make them candidates for cochlear implant and often times we call in the our clinic the invisible disability or handicap and parents want to ignore it. But we finished five years worth of Data Collection on an n. I. H. Grant and found out those kids really are i hate to say this out loud but sort of smarter. If you consider that rose those with hearing aids . Those with hearing aids relative to those who are not fit with hearing aids. Appropriately fit hearing aids. But because their verbal intelligence is higher, their academic success is higher they are going to want to be productive citizens relative to counterparts who were not fit with those hearing aids appropriately. And appropriately is a key word id like to point out. Rose so wheres your creativity being applied . Now that youve received this award and this recognition for your achievements whats the next step for you. Oh, theres tons of Research Projects ongoing in Cochlear Implants to improve them. There are even other hearing implants that are approved in this country and are being used. A middle ear implant. But were living in a split society in a way because theres so Much Research and there will be some so many improvements but on the other hand there are so many people in need of these solutions who dont have access to them and we have to go on both sides of that and i really want to work for many, many more years in both aspects of that have to make progress. Rose what is medel . Medel produces Cochlear Implants. Its a private company and weve helped about 80,000 people to get Cochlear Implants. Im really proud of that. Rose where do they go to receive them. Oh, to clinics, about 2,000 clinics in the world. Rose the company is based in austria. But if you watch the Television News you know that you need a cochlear implant either for the elderly or, in fact, if thats possible certainly for children or your child, what do you do . You go to an e. E. N. T. Clinic and find out more about whether youre a candidate. Charlie, im a cochlear implant surgeon, my Clinical Skills as a cochlear implant surgeon so increasing cochlear implantation is revolving around specialized clinics. The surgery is the easy part. The surgery is a straightforward routine surgery and you want routine surgery. Its breadandbutter surgery. The hard part gets to why in terms of expanding a lot of times its not necessary the surgery but the work force around in terms of the training, the rehabilitation, the programming, thats why increasingly Cochlear Implants are done in Specialized Centers where you have the expertise in one place. But most major Academic Medical Centers with a large e. N. T. Department will do Cochlear Implants. They get refer there had by their primary care doctor or local e. M. T. And go through stichblgs getting their hearing tests to see whether youre a candidate and its about an hour and a half surgery, you go home the same day. About a month later you get a cochlear implant turn on for tht time which is an exciting day for everybody. Then thereafter for about six to 12 months after implantation you follow a routine every few months to get the device programmed and adjusted and basically learn to use language again for people who are later in life literally learning to hear again for many and for the first time. And kids born deaf they learn sound the first time to develop language. Rose whats the earliest age you can receive a cochlear implant . Typically about six months. People have gone a little lower than that but in general we dont doless than six months of age. Is weve implanted adults up to 95 years old at Johns Hopkins now. Age is not a contraindication. Rose i want to return to this question. Help us understand from your personal story the difference its made in your life. I have a normal life. Im a young man andty rose everything . Yeah. I think so. I changed everything. Because if i couldnt hear everything i would be kind of a special school. inaudible i can do whatever i want. I can hear and i can inaudible and its a normal life for me and i wouldnt have a normal life and i cant believe it system . Rose how did you become aware of it . How did you learn of it . From his mom rose ah you were only two years old. Yeah, exactly. So i was only two years old and my mom went to the hospital with me and they said yes, you can come here. And i was one year old and my mom found a little favor in the hospital where you can learn something about the Cochlear Implants and then she had to try one year to get the hospital to implant me because at that time they said you cant implant a child with one year or two years old cant do this. You have to be a lot older and its very dangerous. Rose tell me about the hereditary part again . About one in a thousand children is born deaf or with severe hearing loss. And we they probably about half of those cases are due to some sort of hereditary effect. Theres some sort of a gene that may be defective that may be inherited from the parents and when one or another gene is defective it means probably the hair cells never worked, the child is born deaf. And you think theyre probably about 300 different genes that are critical for hearing. Rose 300 . If you have a defect in any one of them you may be deaf. Rose how many have been identified . 70 . Only about 70. Thats right. So theres a huge amount of work to do to identify more of these deafness jeans. So the reason thats important is because if the child comes in that is deaf and you can gate blood sample and figure out what the genetic defect is it tells you something about what the prognosis is. Will it get worse. Or there some kind of treatment we can offer for this particular genetic deafness or is the child a good candidate for a cochlear implant . It can vary from one or another form of hereditary hearing loss. And theres a lot of work being that sets around the country. Rose finally, this eric. How does this dmekt a sense we understand because of the explanation in the beginning but where do you put this in terms of ongoing understanding of the brain. I think this is a perfect example of what the Obama Initiative wants to do. To develop new technologies to, number one, understand the brain better and to be able to treat disorders of the brain. I think this is a paradigm and the thing comes to my mind and perhaps this is something we can think about in the future is to what degree can this be applied to other sensory losses . Wouldnt it be wonderful to have treatment for blindness . Wouldnt it be wonderful to have treatment for people who dont have a sense of smell . Or people who have difficulty with touch . So this is a major step forward that not only is an end in itself but it challenges the imagination to think of what other things might be approached therapeutically. I think its a fantastic paradigm. Rose thank you for coming. Back in a moment, stay with us. Rose on the next charlie rose, costa gras vas a filmmaker. His new movie explores the world of global finance. Its not so much about politics its about the human beings who vote and they get so attached to someone without any criticism, without saying oh, this guy he did the wrong thing, its not good and keep voting sometimes for the same person again and again and justify him. I think were not enough critical without politicians. With our politicians. Rose whos a politician that you admire . De gaulle. Used to be a very important politician. Rose you admired him . Yes, very much. Very much. Rose why didnt you make a movie about him . Oh, my god he was very difficult to make a he was so big, so difficult, so complex as a character. Rose you can make a movie just about 68; and there were movies made about 1968. Yes, but its there, still, 68. They did that. Very difficult. You had an important president , i think, eisenhower was a great president , too. Rose both war heroes. Yeah. Right. Rose but back to de gaulle because im fascinated about de gaulle. What was it about de gaulle . Because he drove churchill and roosevelt crazy. His ego was he defined himself i am france. It was not his ego for him, it was for the count are ray. The only person de gaulle used to love was the country, france. Probably not so much the french people, but the country and the idea of his country, absolutely. And he really fought very strongly with the small army he had to destroy the germans, to destroy the nazis with churchill who was probably one of the best this is another great politician because he Rose Churchill . Yes. Rose of course, he lost the election after the war. Yes, but you know, again, this is the public. Rose but tell me about de gaulle beyond what we just said that makes you in fascinating view. First of all, he wrote a great memoir he was a great writer but first of all i liked de gaulle for decades. You know, when he left from power he was supposed to have a salary of the president and also for the general. He only took samry for the general. Rose he took the generals salary. Not the president s. Rose he pictured himself as a general rather than a president . No, because he liked to not go after money. And he changed the industry of france. For example, he created the first plane. If since then, the french, europeans, the European Industry of planes has become a very important one. He started the algerian war and stopped also algerian war and started also the colonies. The black colonies france used they have no africa. Rose right. He was really, really a very important president. Rose then he came back to power. He left power and came back to power. He came back in 68 and he was he used to do elections every two years and he was voted every two years and he was saying if i wont be electedly run away from politics. Rose and what did he do when he retired the last time . He finished his writing his memoirs. Thats all. He didnt do anything. Rose but he lived outside of paris. Outside of paris. Rose did you meet him . No. Never. This is the other thing. He used to meet very little people. Rose i know. That was series of interviews done by one man of him that were famous interviews. He was amazing. He never tried to seduce the journalists or to promote promote things and sayly do this and that. Captioning sponsored by Rose Communications captioned by Media Access Group at wgbh access. Wgbh. Org im alan cumming, and this is masterpiece mystery meyerson im starting to get the impression that ive inherited an organization that is somewhat complacent. Id be grateful, mr. Foyle, if youd prove me wrong. If we have another security breach, i need to know. screaming foyle it appears he was a member of the nkvd. As mr. Foyles assistant, youll work in this section. Harris sam really is an asset. Get her out on the campaign trail as much as possible. Sam i saw something at work today that might interest you. As long as its not top secret. Ive come to give you another opportunity to tell me the truth. You dont have the authority to question me. What is it you dont want me to find . How much does foyle know . thunder woman wailing National Funding for masterpiece is provided by. T of historic cities and landscapes on a river, you see things differently. You get close to iconic landmarks, to local life, to cultural treasures. Its a feeling that only a river can give you. These are journeys that change your perspective on the world, and perhaps, even yourself. Viking river cruises. Proud sponsor of masterpiece. Ralph lauren designing for me is a creative journey. It starts with a feeling, a mood of a certain place, the style of a heroine, her journey, her dreams. I create a world around her. A world beyond fashion. The world of ralph lauren. Additional funding is provided by major support is provided by donald and darlene shiley. And by Conrad Prebys and debbie turner. And by the following and by phone ringing kensington 3487. Yes. Who was it, darling . door opens birds chirping car Engine Running evelyn . coughing groaning faint voices dr. Ross