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 then this voltage change is converted to 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 i