Transcription:
One Chance to Hear: Youngest Ever Documented Child Receives Cochlear Implants

Bill Klaproth (Host): Imagine your new infant child loses her hearing due to an infection, with the window closing on the procedure to restore her hearing. That's what we're going to talk about today on this podcast, we're going to learn the story of young Eleanor Nolan with one chance to hear, the youngest child ever documented to receive cochlear implants. So let's talk with the doctor who did the procedure, Dr. Joshua Wood. Le Bonheur ENT, and Assistant Professor at the University of Tennessee Health Science Center.

This is the Peds Pod, by Le Bonheur Children's Hospital. I'm Bill Klaproth. Dr. Wood, thank you so much for your time. It's great to talk with you. And we're very excited to hear the story about young Eleanor Nolan. So tell us about her and what makes this case so special and unique.

Joshua Wood, MD (Guest): Sure. Thanks for having me on the show. Eleanor's case is very intriguing and interesting and exciting because she is actually the youngest recipient of bilateral cochlear implants that I know of and that has ever been documented. Her implants were done when she was about two months old.

Host: Yeah. So tell us her story.

Dr. Wood: I first met her in December when she was six weeks old. She had been admitted to Le Bonheur Children's Hospital in Memphis, Tennessee and was ultimately diagnosed with bacterial meningitis, which is infection of the covering around the brain and the spinal cord. And about one week after her admission, she had some hearing testing done that showed that she had lost essentially all of her hearing in both of her ears. And that is a known consequence of meningitis, although it's very rare, it's very unfortunate that she had that complication, and it kind of put us in a very difficult spot. So she was at that time now she's seven weeks old with essentially no hearing. And the cure or treatment for hearing loss of that nature would be cochlear implants. The FDA, which regulates all the medical devices has approved cochlear implants down to nine months of age.

We have used them, what we call off label, which is, you know, off of the recommended FDA guidance, in special circumstances. And there have been documented cases of cochlear implants being done in down to three or four months old.

But that was the youngest that, we could find in the medical literature. And so, we had a patient here that needed cochlear implants that was much younger than had ever been reported. That's when we got involved in her case.

Host: So bacterial meningitis. There was no way for her hearing to come back on its own after the bacterial meningitis was treated and taken care of, right? So if her hearing was lost at this point, she would not have hearing for the rest of her life. Is that correct?

Dr. Wood: That is correct, yes.

Host: Wow. Okay. So this is really fascinating. So you meet Eleanor when she's six weeks old, you discover this. You put the implants in when you said she was about two months old. Tell us what was your thought process on implanting the cochlear implants in such a young person like this, that's never been done before. Tell us your thought process on this.

Dr. Wood: Yeah. So, a lot of different thoughts go through my mind. One, from an anatomy standpoint, we know that the cochlea, which is the hearing portion of the inner ear, is essentially full grown at birth. The organ that we would insert the implant into is normally formed, even though she's young. And so from a technical standpoint, I knew that cochlear implant was feasible in such a young age.

Host: Right. Dr. Wood, you said normally cochlear implants are approved, I believe you said starting at nine months of age, is that correct?

Dr. Wood: That's correct? Yes.

Host: So, is there a danger for you waiting until the nine month mark? Was there a reason that you had to do this procedure now?

Dr. Wood: That's a great question. And it's something that, is kind of a time sensitive, urgent matter in cases of meningitis associated hearing loss. And the reason for that is there is a process called labyrinthitis ossificans, fancy words, but essentially it means that the labyrinth, which is the fluid field channels in the inner ear turn to bone. So during the cochlear implant procedure, where the electrodes are actually placed into the cochlea. They're placed into this fluid-filled channel. And so if that fluid filled channel has turned to bone, then there's no opening for us to put the electrodes. And so the surgery is not successful.

Host: So, because it was caused because of meningitis, you had little time, you had to act now basically there was an urgency to this situation.

Joshua Wood, MD (Guest): Correct. wWe had had an MRI scan. that can look specifically at that fluid within the cochlea. And give us some indication if there's that process starting in Eleanor's case, the MRI was, indicative of early phases of this bone formation happening. And so that's why it was, it kind of turned into an urgent case.

wWe didn't have a whole long time to wait. before the surgery was not possible.

Host: Right. Wow. This is a fascinating, so you saw that on an MRI and it was already starting to close up and you had to get in there. So can you just briefly explain to us how a cochlear implant works so we in our minds can understand this?

Dr. Wood: A cochlear implant is a way that we can essentially restore hearing in most cases of deafness. It works very different from normal hearing. So, you know, the normal hearing pathway, the sound waves come in through the ear canal. They vibrate the eardrum, which then vibrates the three little bones of hearing in the middle ear, which then transmit into fluid waves in the cochlea. And then those fluid waves are picked up by very tiny, sensitive receptor cells that then transmit to the cochlear nerve. Which then sends the impulses to the brain that we interprete as sound.

And how a cochlear implant works is it essentially bypasses everything, and puts electrical stimulation on the hearing nerve directly. And so it essentially bypasses everything that's been going on in the natural form of hearing, and stimulates the cochlear or the hearing nerve directly.

Host: So are there certain people that qualify for cochlear implants and others that don't.

Dr. Wood: There are, yeah, there are pretty stringent criteria for who qualifies and who does not qualify for a cochlear implant. And it has to do with a lot of different factors, some of which are, the degree of hearing and most patients that would qualify for a cochlear implant would be what we'd classify as severe to profound hearing loss.

Which is essentially they have no hearing that usable. And in that degree of hearing loss, even a hearing aid, doesn't give them enough benefit to make anything useful. Other factors we look at are anatomical factors, such as the inner ear itself. If there's a malformation of the cochlea or the cochlea is not formed right, it may preclude putting the implant in correctly. And then there are also some children that do not have a functioning hearing nerve. And so in that case, the implant doesn't have anything to simulate. And so it it doesn't work. And patients don't meet criteria for the implant.

Host: Got it. So back to Eleanor, you've got the implants in right now. So will she be able speak? Where will she be at? Will she need some speech therapy in the future?

Dr. Wood: Generally the process and, in Eleanor's case, we followed our normal protocol, which is that, we have them come back to clinic, in about three or four weeks after the surgery itself. That's when we actually turned the device on.

Host: So when you put it in it's off, she's not able to hear right away.

Dr. Wood: Correct? Yeah.

Host: Oh, so tell us what happened when you turned the cochlear implants on. We've seen pictures of people that are able to hear for the first time. And their face lights up like oh my God what happened. So does that happen with an infant. Is that what happened with Eleanor?

Dr. Wood: It did. It was very cool to see. Eleanor had normal hearing for the first month of her life. So she knew her parents' voices and things like that. And so when we turned the implants on, you can immediately see when her parents are talking to her that she was looking around. Trying to find mom and dad and was smiling at them again. And so you could tell that there was definitely some hearing going on and she was recognizing things again.

Host: That's amazing. I bet that was an emotional, I'm going to start crying. What an emotional moment for her parents. Right?

Dr. Wood: It was very touching. And so over the next six months or a year, there is some fine tuning and adjusting of the implant that will have to happen to make sure that she's hearing as best as she can and hearing with the cochlear implant is not exactly normal hearing, but it's pretty darn close.

Host: I was going to ask you that if it modifies the sound at all, if it's different, than regular human hearing?

Dr. Wood: It is a little bit different, because we can only stimulate at certain frequencies. So we don't have, all the fine tuning of the natural hearing ear that we're all used to. But with the modern cochlear implants, people can appreciate music and those kinds of things. And so it's come a long way from the early, early implants. But in Eleanor's case, since she is implanted so young, you know, she's going to be able to develop speech and language, probably just like any other one year old, 18 month old, two months old.

She may need a little bit of speech therapy just to kind of, fine tune, a little bit of pronunciation or those kinds of things, but all in all, she'll be able to develop pretty normally.

Host: Well, thanks for sharing your thoughts on that. We appreciate it. And thank you for your time today. This has been a fascinating story about Eleanor Nolan. Thanks for your time again, Dr. Wood, we appreciate it.

You're very welcome. Thanks for having me.

And once again, that's Dr. Joshua Wood. And to learn more, please visit LeBonheur.org and be sure to subscribe to the Peds Pod on Apple podcasts, Google podcasts, or wherever you listen to your podcasts. You can also check out LeBonheur.org/podcast to view the full podcast library. And if you found this podcast helpful, please share it on your social channels.

This is the Peds Pod by Le Bonheur Children's Hospital. Thanks for listening.