Hearing Loss and Cochlear Implants – PediaCast 480
- Drs Oliver Adunka, Krista Winner and Erin Stefancin visit this week as we consider the cause and impact of hearing loss. We explore services provided by audiology and speech-language pathology and uncover the fascinating facts of cochlear implants. We hope you can join us!
- Hearing Loss
- Speech-Language Pathology
- Cochlear Implant
Announcer 1: This is PediaCast.
Announcer 2: Welcome to PediaCast, a pediatric podcast for parents. And now, direct from the campus of Nationwide Children's, here is your host, Dr. Mike.
Dr. Mike Patrick: Hello, everyone, and welcome once again to PediaCast. It is a pediatric podcast for moms and dads. This is Dr. Mike coming to you from Nationwide Children's Hospital. We are in Columbus, Ohio.
It's Episode 480 for January 7th, 2021. We're calling this one "Hearing Loss and Cochlear Implants". I want to welcome everyone to the program.
I also want to welcome you to 2021. It's a new year. Of course, it looks a lot like the end of 2020 and it's going to continue to lot like that for a while. Still, it's good to move on, right, with the fresh start in the new year?
I'll be honest, I feel like the finish line of the pandemic is on the horizon. Now, it may not be until closer to the end of 2021, but we are certainly closer to the end than we were a few months ago.
And I hope all of you had a safe and happy holiday season. And yes, I'm going to marry the holiday season with the pandemic. And in that, my favorite Christmas gift this year, I actually received on Christmas eve, and that was my first COVID vaccine.
And I have to tell you it was sort of an emotional moment because that, really, the vaccine being available. I've looked at the data, I've looked at the research behind the Pfizer and the Moderna vaccine. I've talked to lots of experts whom I trust. And so I felt very confident getting that first COVID vaccine.
And really, that was my favorite Christmas present. Not to diminish the things that family members got for me because I really do cherish and love and appreciate the time that we spent in choosing and buying and presenting presents. But the COVID vaccine, I got to tell you, that was right up there and will be an event that I remember for the rest of my life.
Now, in terms of how well did I fare, I had a little bit of a sore arm just like with any other vaccine, sort of like a flu shot. It hurt for about a day and that was it. Now, I recognize that I'll probably have more symptoms with the second dose, and I will be sure to report that to you as well, because you prime the immune system with the first dose, and then you boost it with the second one.
And so you can expect your immune system to kick in and react when you get that second one because it has some memory from that first one. And so, I expect that after the second one, I'll feel a little feverish. I'll have some aches and fatigue and feel a little sick for a day or two, and then be better after that.
So with this one though, just a little bit of a sore arm for one day and not even the worst sore arm I've had with the vaccine. I would say the Tdap was a little worst. And the Shingrix, so the shingles vaccine that you get after your 50 years old, not to reveal my age or anything, but that one I think really hurts more for sure than the COVID one. The COVID one was kind of on par with the flu vaccine, I feel like.
So that was it. We will be talking more about the COVID vaccine as this year progresses. But for now, I got my first one, waiting for my second one. And I will keep you all updated on any symptoms or problems that I experienced.
We have an expansive and important topic for you this week. Hearing loss affects millions of Americans. And so, we're going to cover the causes of hearing loss, how we screen and test hearing, establishing the diagnosis of hearing loss and the impact that this condition has on language and learning, how can we protect our hearing and prevent hearing loss from happening.
We'll also talk about supporting kids and families with hearing loss and language difficulties with audiology services and the work of speech-language pathologists. Then, we're going to take a look at cochlear implants, what are they, how do they work? They're really fascinating devices that have improved the lives of many people.
You've probably heard of them, right, cochlear implant? But what exactly is this technology and how does it work? Stick around and we will fill you in.
So again, kicking off 2021 with an expansive important topic. And we have three terrific guests joining us this week as we explore hearing loss and cochlear implants.
Dr. Oliver Adunka, he's an otolaryngologist, also known as an ear, nose and throat specialist or an ENT doctor. He's also the director of the Hearing Program at Nationwide Children's Hospital.
We also have Dr. Krista Winner. She's an audiologist with our Hearing Program. And Erin Stefancin, she is a speech-language pathologist, also with the Hearing Program at Nationwide Children's Hospital. They will be here shortly.
In the meantime, I do have some reminders for you. Don't forget, you can find PediaCast really wherever podcasts are found. We are in the Apple and Google Podcast apps, iHeartRadio, Spotify, SoundCloud, Amazon Music, and most other podcast apps for iOS and Android.
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And we do have that handy Contact link at the website, pediacast.org if you would like to suggest a future topic for the program.
Also, I want to remind you the information presented in PediaCast is for general educational purposes only. We do not diagnose medical conditions or formulate treatment plans for specific individuals. If you have a concern about your child's health, be sure to call your healthcare provider.
So, let's take a quick break. We'll get our expert guests connected to the studio. And then, we will be back to talk about hearing loss and cochlear implants. It's coming up right after this.
Dr. Mike Patrick: Dr. Oliver Adunka is an otolaryngologist also known as an ear, nose and throat, or ENT specialist at Nationwide Children's Hospital and the Ohio State University Wexner Medical Center. He serves as director of the Hearing Program at Nationwide Children's and as a professor of Otolaryngology at Ohio State.
Dr. Krista Winner is an audiologist with our hearing center and Erin Stefancin is a speech-language pathologist also at Nationwide Children's.
They are here to talk about hearing loss in children and cochlear implants. So let's give a warm PediaCast welcome to our guests. We are so glad to have all of you with us this week.
Dr. Winner, let's start with you. Walk us through the mechanics of hearing. So what does the process look like from the source of a sound all the way in to our brain?
Dr. Krista Winner: Yeah, so I like to break up the ear into three sections, the outer, middle, and inner ear. Sound comes into our outer ear through the pinna and it travels through our ear canal.
Once it reaches the eardrum next, that's considered our middle ear space. So the sound will move and vibrate. And it will move these three little bones, what we call ossicles behind the eardrum. The sound is then conducted to the inner ear portion of our ear where we have a small bony-like structure. It's fluid filled.
And inside that, it's called the cochlea, and inside the cochlea, there are tiny hairlike structures. So those then move in response to the vibration, causing the vibration to change into electrical impulses. That electrical impulse is then transmitted from the cochlea to along the auditory nerve and then up to the auditory portion of the brain. And that's how our brain interprets the sounds that we hear.
Dr. Mike Patrick: So this is a pretty complicated pathway, as we move from the source of the sound into our brain and as we experience hearing. And whenever you hear a long, complicated pathway, there is always opportunity along that pathway for things to go wrong.
And as we think about hearing, depending on where in that pathway something happens that affects our hearing, we can either have conductive hearing loss or sensorineural hearing loss.
So Dr. Adunka, kind of walk us through what exactly is conductive hearing loss and what are some of the causes of those problems?
Dr. Oliver Adunka: Yeah, so conductive loss is anything that affects, like the names suggests, the conduction of sound to the inner ear. So it's pretty much any source of hearing loss in, what Dr. Winner said, that external ear or the middle ear.
So simply putting your finger in your ear canal, you have created a conductive hearing loss and when it sounds muffled, right?
Other causes, frequent causes would be, the most frequent one being the middle ear infections, so fluid in the middle ear, the most common cause of hearing loss really in children. And then, of course, holes in the ear drum or more rare, less common problems such as issues with the little hearing bones, the ossicles, like Dr. Winner said.
Dr. Mike Patrick: What about ear wax? Is that something that could cause hearing loss if there's too much ear wax? Or do sound waves pass through ear wax without any problem at all?
Dr. Oliver Adunka: It depends on the amount of wax, we call it a wax impaction. That's really when the wax completely occludes the ear canal and that certainly can have an effect on hearing. And it's actually something we fight frequently in children who do wear hearing aids where the ear canal is blocked. And that usually has a negative effect on how that wax basically makes its way out to the lateral end of the ear canal.
Dr. Mike Patrick: Now in addition to the conductive pathway, we also have something called sensorineural hearing loss. So walk us through what exactly that is and what are some of the causes?
Dr. Oliver Adunka: Yeah, so it's a good question. So sensory hearing loss is more permanent hearing loss. I usually tell folks it's the hearing loss that you get with aging or going to rock concerts, right?
Most sensory hearing loss is sensory in nature, meaning that it affects the inner ear, as opposed to the neural portion. We just bunch them in into sensorineural, but most of them, again, are sensory.
And the implementing step of hearing is really the process where hair cells, they're the receptor cells in the ear, they're very sensitive, take those sound waves that the middle and external ear catch and amplify, and take the sound waves and turn them into electrical impulses the brain can understand.
So hair cells are critical. Unfortunately, we're born with one set and we cannot regenerate them such as birds, for example, would be able to regenerate hair cells. Unfortunately, mammals have lost that ability.
So those hair cells are absolutely critical and it's on our mind every day. The most common cause of hearing loss, not in the pediatric but in the adult population is noise-induced hearing loss, which is usually, the classic one is really occupational. Like going to work and not wearing your hearing protection. That's the most common cause of hearing loss, so working on board of an aircraft carrier on the flight deck.
What we see and what's scary at what pertains to the pediatric podcast we're having right now is that we see recreational hearing losses from wearing over-the-ear or in-the-ear headphones. So a lot of the teenagers have that. In fact, studies from Europe say about 10% of 13- to 23-year-olds have permanent hearing loss from wearing consumer electronics.
Dr. Mike Patrick: So, as we consider pediatrics, we also have to worry about genetic disorders? So there may be some syndromes in which those little hair cells do not form properly. And so we have hearing loss from that.
You can also have infections. And in particular, congenital CMV infection or cytomegalovirus infection is one of those that can lead to hearing loss right from birth. And in fact, we talked about that in one of our CME podcasts, PediaCast CME, Episode 45. That covered congenital CMV infection.
And even though that was a podcast episode for providers, there may be families out there who are impacted by congenital CMV and hearing loss. So you may be interested in listening to that episode. And I will put a link in the show notes for you, so you can find it easily over at PediaCast.org for this episode, 480. Just look in the show notes and we'll have a link to that CME episode for you there, so you can find it.
So Dr. Winner, how common are these forms of hearing loss and who's affected?
Dr. Krista Winner: Yes, so with conductive hearing losses, probably most common in children ages about one year to about five years, mainly because they have a high incidents of ear infections. And as those children get older, they'll typically outgrow the ear infections. But we see a lot of kids who have had such a history that they've got scarring or they have holes or perforations left in their eardrums. And so, they might experience more of a permanent conductive hearing loss as they grow.
So that will require, obviously, additional treatment or surgeries by the ENT doctors. So risk factors for conductive losses are like people who suffer from allergies or sinus issues. They're more susceptible.
We see a lot of kids with eustachian tube dysfunctions. So, there's a tube that runs from your middle ear down your throat. And if it that becomes clogged or has trouble kind of opening, closing and equalizing pressure, then those individuals I guess could run the risk of having a connective loss.
We also see kids with another risk factors like anomalies of the outer or middle ears. So we see plenty of kids with small and narrow ear canals, stenotic ear canals. They may be the ones too that get a lot of wax trap.
And like Dr. Adunka said or mentioned about wax impaction, kids with missing ears, missing pinnas can cause conductive hearing losses. And then, like Dr. Adunka mentioned earlier, stiffness of those middle ear bones, that also, we see issues with that sometimes with kids.
Dr. Mike Patrick: All right, so you are an audiologist. What exactly is an audiologist and what role do you play in helping kids with hearing loss?
Dr. Krista Winner: So an audiologist is a licensed hearing healthcare professional. And we specialize in diagnosis and treatment of hearing loss imbalance disorders in both adults and children. So most audiologists have completed their doctorate in our profession. Which is why you may hear some of them refer to themselves as doctors, or hearing doctors, I should say.
But we are not medical doctors. So, this is why when we make a diagnosis, before we fit with any amplification, we will refer the child or the adult over to Dr. Adunka and his colleagues to do a further evaluation and make sure it's appropriate for us to move forward and fit these children.
Dr. Mike Patrick: So, we talked about screening babies for hearing. So, there's a newborn hearing screen. And that is a little bit more complicated than it sounds. I mean babies can't tell us whether they hear something or not. So how was this accomplished? What exactly is the newborn hearing screen and how does that take place?
Dr. Krista Winner: So we actually started doing newborn screenings back in the early '90s and now, it is a standard of care in hospitals nationwide. So every baby is tested before they're discharged from the birth hospitals. So, kind of starts there.
We screen the babies in the hospital by putting little headphones over their ears and then we put little sticker or electrodes on the forehead. And so we send a sound, a clicking sound, into these little headphones. And the electrodes pick up the response from the auditory nerve.
If they pass or fail, so we are just trying to identify these babies that would need further testing. So when they come in to our clinic, we're doing a similar screening test which I should have mentioned is called an ABR. But we are doing a similar test, but we're doing a more diagnostic measure.
So we are sending sounds into their ears at different frequencies or pitches. We're sending it at much softer decibel levels. We're trying to find the softest level that we can get that auditory nerve to respond and determine if that is kind of where their hearing threshold is.
And we can do this when the baby is usually from about zero to six months of age. We can do an ABR while they're asleep. So just natural sleep in the parent's arms or we can even just do it in the car seat.
Dr. Mike Patrick: For those interested in learning more about this through some written material, we do have some terrific blog post over at 700childrens.org. That's our hospital's blog.
We have one on Universal Newborn Hearing Screening and another one called Little Ears and Little Voices: Speech and Hearing Care for Preemies. And I'll put a link to both of those in the show notes, so folks can find them easily.
After a baby fails their newborn hearing screen, when we see that there might be a problem, when is the best time to refer those infants to see an audiologist?
Dr. Krista Winner: So once we refer to screen, we would for that referral to be placed by the pediatrician immediately. And so, typically, in the birth hospital, they're given instructions or different facilities that we can follow up with.
So the Ohio Department of Health will also become impacted, so that they will follow up with the family to make sure that the baby is not missed. Our goal is to have every child screened by one month of age. And that we can identify them if they have a hearing loss by three months of age and then fit by six months of age. So sometimes, everything kind of falls right into line and we even meet those goals earlier.
Dr. Mike Patrick: All right, so how about older kids, how is hearing tested in them? So the really older kids who can cooperate and listen to sounds, let you know if they hear them or not as you present to them different frequencies and different loudness levels.
But what about the kids who aren't so cooperative? They're older than babies but they're not quite there in terms of cooperation. And maybe they're fearful of strangers. They kind of clam up. They don't want to be where they are. They just kind of dig into mom and turn their head away from you. How do you accomplish screening hearing in those kids?
Dr. Krista Winner: So usually, cooperation is the challenge sometimes, probably why I chose this profession. I do enjoy the challenge with pediatrics. But testing children, usually around six months and older, we do what's called visual reinforcement.
So we will have them come in to the sound booth which is just a sound treated room. They'll usually sit on their parents' lap. And we'll play sounds through either headphones, depending on their cooperation level and their age.
We'll play sounds through headphones or we'll play them through a speaker. If that child hears the sound, if they turn and look towards the speaker, we'll reinforce them with a little toy or video, just to kind of to keep them interested and responding to the task.
As they get older, we can usually graduate to a little bit different task called condition play. So when they hear the sound, they might drop a little block in the bucket. And then, we eventually graduate to standard hand raise, just raising their hand when they hear the sound, kind of similar to what we remember doing when we did hearing screens in school.
Dr. Mike Patrick: So how often should hearing be screened? Right there, we do the newborn hearing screen. Is this something that should occur at regular intervals?
Dr. Krista Winner: So for any child that doesn't really risk factors, and we might get a normal hearing exam, then we usually just recommend as needed. So we'll tell the parents, if parental or medical concurrence arise, bring them back in. It's a pretty non-invasive test.
But otherwise, the pediatrician will do screens in their office at well child checks. The schools doing screening's usually kindergarten, first, third, fifth, seventh grades, I believe. So typically, these kids can get picked up and are monitored pretty close.
For a child with hearing loss, it's a little bit different, especially sensorineural hearing loss. So that will depend on the child's diagnosis. So some of the causes of hearing loss that we find may suggest the hearing will progress. So we'll see those kids a little sooner.
Other kids have a pretty stable hearing threshold so we might push their repeat hearing test out a little further. So it really kind of depends on the diagnosis. And just sometimes, we find conductive losses on top of the sensorineural loss. And so we need to monitor that a little closer as well.
Dr. Mike Patrick: We have another blog post over at 700Childrens. org, Why Are Speech and Hearing Screenings Necessary for Kindergarteners. So if you want to hear more about that, I encourage to visit the 700 Children's Blog.
What are some ways that we can protect our hearing? So we talked about one of the most common causes of sensorineural hearing loss is damage to those little hair fibers. And loud music and exposure to loud noises is what often causes that. So Dr, Winner, what are some ways that we can protect our hearing?
Dr. Krista Winner: Using earplugs when you're around loud noises is something we should think about. We don't always but whether you're attending a concert or you're around loud machinery or equipment, you want to protect those little hairlike structures we talked about in that cochlear that causes that sensorineural hearing loss.
Turn the volume down. So I always tell kids, they're guilty of this with the air pods, but if the person next to you can hear your music, it's too loud. So turn it down.
Give your ears time to recover. So if you've been exposed to some loud noises or just prolonged exposure to noise, give your ears a rest. The Ohio Safety and Health Administration has a great website that you can look at for length of noise exposure and how much is too loud.
And then, one thing I'm pretty guilty of myself but stop using Q-Tips, cottons swabs, sticking things in your ears. As tempting as it is, you run the risk of causing some pretty significant damage to your ears and hearing if it goes in too far.
Dr. Mike Patrick: One thing that families can rely on to help protect their hearing is actually a smart phone loudness meter. So there are apps that you can download that just pay attention to how loud the noises are around you. And so that's one way that you can sort of screen yourself and your family. Are we around loud noises too often? These loudness meters can help you.
And of course, if you're listening to music loudly through headphones or ear buds, it's not going to pick up those noises. But at least, if it's part of your ambient environment, then this can be a way for you to understand, "Hey, this is too loud. I need to alter my environment in such a way that I'm not exposed to these loud noises so often."
One terrific resource from the CDC is the website called Listen Up! Protect Your Hearing. And they point out at that website that half of all young people, so five in ten young people listen to music too loudly or at levels that would be considered dangerous over time. And that four in ten young people are around dangerously loud noises during events such as concerts and sporting events.
Of course, not during COVID. So the pandemic actually, that's one good thing I guess that's come out of it. We're not exposed to as many loud crowd noises as we used to. But again, after the pandemic, when life returns to normal, which hopefully it will, we do have to pay attention to loud noises such as concerts and sporting events.
And again, that's where having an app on your phone with a little loudness meter can let you know, "Hey, this is dangerous, I need to put my earplugs in."
The CDC website also has pictures of healthy and damaged inner ear fibers, so that's pretty interesting. And then, they have links to all sorts of resources, get more tips to help protect your hearing, learn how to use foam ear plugs, choose the type of hearing protection that's right for you, find out how our ear works, learn more about hearing loss in children.
So lots of links and resources. And I'll put all of those in the show notes for this episode, 480, over at pediacast.org, so you can find them easily.
All right, Erin Stefancin, let's move on to you. I want to pull you into the conversation. You are a speech-language pathologist. Tell us exactly what is a speech-language pathologist and what role do you play in helping kids with hearing loss.
Erin Stefancin: Thank you so much. So a speech-language pathologist is a quite wide range of field. We work with treatment, diagnosing speech language, social communication, cognitive communication, and even swallowing disorders. That's all within our scope of practice, both for children and also for adults.
Specifically related to the Hearing Program, my role mostly consists of any evaluation and the treatment of children that are deaf or hard of hearing. Mostly, for my role, I will see patient pretty frequently. After Krista and Oliver see them, I will work with families and provide evaluations that varies in the role of six months, a year.
And then if any family or any child has delay, I will see them for treatment. Typically, that is for 60 minutes in length, usually every week or every other week. And I will see them throughout usually from birth, typically, we will see them pretty frequently if they have a language delay.
Dr. Mike Patrick: One of those blog posts over at 700Childrens.org, what's the difference between an audiologist and speech-language pathologist, and I'll put that in the show notes as well.
So how does hearing affect language development?
Erin Stefancin: So yeah, the big thing when it comes to especially children with hearing losses, early detection, as we previously discussed, early detection, early diagnosis as well as being fit early with hearing aid, or for children who have cochlear implants or any sort of hearing technology.
So if we are able to have that early detection, early identification and being fit early with hearing aid. Our goal is that they don't have a language delay, that children don't go a very long time with a significant hearing loss. That's our main goal. But for some kids, that timeline might be a little but delayed or for some kids that might have a more severe hearing loss, that might require cochlear implants which they won't get typically when they're nine to twelve months of age.
They might have a language delay because they don't have access to sound and access to be able to listen to people. So we develop language by listening and taking in the environment around us. So we develop spoken language that way. So for children that have hearing loss, especially as they get to the more severe degree, they do not have access to that as readily without hearing technology.
So for children, especially children that go a little bit longer and we get a child that has cochlear implant that doesn't fit them until that between that nine- and twelve-month range, we provide lots of resources for families and treatments to jumpstart learning spoken language, having access to communication with the families and giving them a bombardment of resources to jumpstart their language and close their gap between what their chronological age is.
So if they're a year old, and maybe what their age is for their language, so maybe they are at a six-month range, but they're really a year old, we'll work on closing that gap.
Dr. Mike Patrick: When should kids first see a speech-language pathologist and how often do you see them?
Erin Stefancin: So within our program, typically, as long as things are working as they're supposed to be, we'd like to see families, we'll see kids as early as typically between three and six months for their first appointment, usually not earlier. That includes children that have a variety of hearing technology, hearing aid, children with varying degree of severity in their hearing loss.
And then, for children, we typically see them every six months for an evaluation. For kids that have graduated speech, we'll see them every year.
And so, we will see children that are very, very young. Parents will sometimes come to me between three and six months, "How do you know that my son has a language delay?" So kind of, "What are you using for getting that information?"
And a lot of that, especially in that first three to six months range, seeing what the child is doing and also getting the baseline of skills. So at this current age, this is what they're doing.
We might meet the family once a month. And then six months later, we'll do another evaluation and be, "Okay, this is where they're at now." So that kind of give us the baseline of their skills, to see where they're at in that moment and at most, providing a lot of parent education for the family.
Usually, if it's between that three to six months, we'll talk about some strategies for promoting listening, like maybe reducing background noise at home. When you're having a communication interaction, maybe don't have the dishwasher running that have that extra added background noise.
We'll talk about having parents narrate their whole life, everything they're doing throughout the day. So maybe when the parent is folding laundry, then, "I'm going to do my shirt next. I'm going to fold it up really nice. This is a beautiful purple shirt."
So we'll kind of talk them through the strategies that we would like parents to start implementing even at a really young age of only three months old.
Dr. Mike Patrick: Wow, so I would not have thought. And I think a lot of parents would not think that you should see a speech and language pathologist as young as three or four months of age. Because certainly, when we think about speech therapy, at least I think back to when I was in grade school and I had speech therapy in first and second grade, so I could learn how to say my R's correctly.
It's the kind of vision that we have of speech therapy. But really, there's a lot more to it in educating parents and caregivers on the best way to communicate with young kids even at the baby level, and especially if those babies have hearing difficulties to begin with. Really important stuff.
Now, as I think about my own speech therapy and learning how to say my Rs correctly, I have a picture of that in my brain, and I think a lot of other listeners probably can think back to their speech therapy. Is the process of speech therapy for older kids, is it the same as it's always been or is it different these days?
Erin Stefancin: So because we have such big range of what's within our scope of practice for speech therapist. So there are speech therapists that teach children how to do their speech sounds. There's the big nine is the nine areas of practice. So doing articulation and speech production of individual counts, that's within our scope of practice. As well as for really young children, there's early intervention piece that typically is at birth-to-three age, we're mostly focused on overall language skills.
So there's this very critical period of time from birth to three for the language development in the brain. And mostly, what speech therapy might look like for those kids from that age range, it will look like asking a lot of parent coaching with them, help in setting up that environment.
As they get older, we will continue to monitor their skills. We might work with children that has hearing loss on individual sounds. Sometime, higher frequency sounds like the S and S-E sound, or difficult to be perceived with some hearing technology. So we might work on the production of those sound.
As we go through, especially in that birth-to-three range, it mostly is early intervention, parent education and training. And then, as they get older, it might be kind of similar to when you had speech therapy for your production of your R sound. Typically for older children, that will be something that we can also provide for them. We have a quite big scope of practice and that is still included in every speech-language pathologist.
And part of the scope that I provide to even patients that don't have hearing loss is to work with kids that need help with their speech sound production.
Dr. Mike Patrick: Another one of their 700 Children's blog post, we have so many of them related to hearing and language and speech, it is Speech and Communication Development: Birth to 3 Years. And I'll put a link to that in the show notes. I think that will be helpful for parents as well, especially if you have young kids at home.
All right, I want to pivot the conversation here. We've talked about hearing and audiology and speech-language pathology and how hearing and language and speech is related to learning, all important things. But I want to pivot to topic that I think most folks have heard about, it's a procedure called a cochlear implant.
And even though a lot of us have heard of cochlear implants, I think that there probably is some confusion of exactly what it is. So Dr. Adunka, I'm going to bring back you in. What exactly is a cochlear implant?
Dr. Oliver Adunka: Yes, so it's a great question. It's quite a complex machinery, and it's really a semi-implantable device. So there's an implant part and there are externally worn parts of the cochlear implant.
And really, what a cochlear implant does, it addresses the most common cause of permanent hearing loss, all right? So what we discussed early on in this podcast are those little hair cells. The cochlear implant bypasses them so it's inserted into the cochlear, which is part of the hearing portion of the inner ear.
That's where those hair cells live. And it basically stimulates the little nerve endings that are the most distal part of the hearing nerve. And the reason cochlear implants work is it then code the different pitches that we hear and brings those codes to the corresponding nerve endings.
So then, we use something that's unique to the hearing system in the human, basically, nervous system. And then we got is that each frequency has a certain and specific place in the cochlear. And we mimic that with the cochlear implant.
They're really called multi-channel cochlear implants. So they were FDA approved in 1985. So cochlear implants actually been around, but they've been increasingly been used since.
And obviously, there's a lot of myths and a lot of bad news about cochlear implant and information. There are also very good ones. As Erin and Krista and you mentioned earlier today as well, a biggest concern with managing pediatric hearing loss is really spoken language development, right? Because we can't learn to talk if we don't have a good input.
And for children who either had no hearing or hearing that's not good enough to support spoken language development, that's when a cochlear implant would come in. And how you have to imagine that is that if a child has a moderate to profound hearing loss, so quite a substantial level of hearing loss, and we would fit a child with a hearing aid. So we'd ask Dr. Winner and colleagues to do that.
In the meantime, Erin and her team will work on the child's speech development. But if we feel like that that hearing is not enough with the hearing aids to support spoken language development to a normal degree, that's our goal, then we would switch to cochlear implant.
The process is somewhat easier with children who are born without any hair cells. Like you mentioned, some genetic disorders for example would do that. Or CMV can sometimes do that. Then, no hearing, that sort of makes it a little easier for us. It's really of when the complex algorithm of hearing tests that Dr. Winner mentioned.
She hasn't taken enough credit for that. Pediatric audiology is very very hard, and it really takes a village. As you noticed, from just our group, here and there, so many team members of the Pediatric Hearing Program at Nationwide Children's said, "It really takes a village to do what we do," right?
And there are a lot of visits. And, unfortunately, one of the first things I tell a family when they come in and see me and the child has hearing loss, I'm like, "Just bear with us." Early on, there are ton of appointments. A lot of families feel overwhelmed and like just stick with us, believe me, it's worth it.
And we frontload all the work, right, because that's when the brain's like a sponge, that's when speech and language development, the basis for really for what's going to happen, is created. So that's when Erin and her team, Kris and her team, myself, that's really where all the wheels are turning in that. So it's quite a complex algorithm.
Dr. Mike Patrick: Another one of those 700 Children's blog post, What Are Cochlear Implants and How Do They Help a Child's Hearing? We'll add that to the long list of resources that will help our folks in the show notes. If you would like to learn more, you can find all of those links over at pediacast.org in the show notes for this episode, 480.
So who are candidates for this procedure and when is it performed?
Dr. Oliver Adunka: So just this year, cochlear implant, the labeling with the FDA varies from manufacturer. But one manufacturer, and I think the others will follow too, has now been approved for nine months and older. So nine months is really the general age we will put a cochlear implant in.
And one important concept is that with what Krista said earlier, with this series of hearing test. Usually, it's not just one test that will determine the child's hearing. Usually, it's a series of tests. And like with a lot of things in life, we want two separate things showing us the same result before we feel confident about it.
So oftentimes, we get to ABR test, which is an electrophysiologic objective tests, so the child doesn't have to cooperate with us. And we want that supplemented by the behavioral test, the visual reinforcement audiometry, before we actually would recommend a cochlear implant. So we do want those tests to all align, the data to align before we would recommend the cochlear implant.
Dr. Mike Patrick: All right, so it sounds like this is an easy procedure, you know, "Hey, we just put this implant in the inner ear." But I'm sure it's more complicated than that. So how exactly is this procedure performed.
Dr. Oliver Adunka: So a cochlear implant is actually a very standardized procedure. I remember when I first saw a cochlear implant in 1990s. It was like a four- or five-hour procedure. There's a lot on the Internet about the complexity of the surgery and all that.
And I do it for a living, but trust me, for someone who is a good ear surgeon, a cochlear implant is sort of one of the simpler procedures. It takes about 45 minutes to an hour. It's a little incision behind the ear. We typically do not even shave any hair. We squeeze in behind in the crease behind the auricle. And the vast majority of children go home the same day.
So it's an outpatient procedure. We used to wait about a month to activate the implant. So you have surgery and then you go home, and then you come back to audiology. What I tell the parents, now, the work begins, right? Because my part was the easy part. And so, nowadays, we usually activate the implants two to three weeks after surgery.
So, really, it became, it has become a much less involved procedure. Partly because we have learned as surgeons, but also really the manufacturers have really helped us a lot and the new devices are fabulous. They hardly ever feel anymore. They're much smaller.
The extra components, when I recently gave an interview to NPR, they asked me, "What's new?" And I'm like, "Well, what's really new about hearing aids and cochlear implants is that they talk to your consumer electronics." That's really a big deal these days, right? That it's iPhone compatible, right?
So it's really evolved quite a bit. So what the cochlear implant really has, a receiver simulator that is implanted behind the ear. And that just picks up the signal that's sent from the outside, from the external. But then there is cable, really a wire. And the wire, we call them electrode, but it's really an array of electrodes.
Usually, anywhere between 12 to 22 electrodes that are implanted in the cochlea. So, it's a wire essentially that we advance into the cochlea. And cochlea is Greek for snail. So the inner ear looks like a snail, right? And we just open it at the base and we advance the wire gently into the cochlea.
We usually try to get at least 360-degree insertion. So we try to wrap that hearing nerve around one time, at least. Again, that varies a little bit from manufacturer to manufacturer. And they all have some marketing claims, we're going be better or not, but the truth is, as long as you get a turn in, then we don't really have measurable differences in most children.
So, that's really, the actual implantation of the electrode is usually actually the quickest part of the whole surgery. So, the very first cochlear implant actually had a wire that came out through the skin, with the associated concerns and issues with that. But that's long gone, right?
So, right now, every cochlear implant has a radio frequency link. It's like charging your cellphone wirelessly. It's the same the principle and it sends the signal through the intact skin. So yeah, what I usually tell folks is, adults and children, that you'll look the same.
Dr. Mike Patrick: So what kind of hearing results from a cochlear implant? I mean, is this normal hearing like you and I have right now? Or is this different in some way?
Dr. Oliver Adunka: So eventually, it is. What a lot of the adults, for example, if you and I would lose our hearing and we would get a cochlear implant, we would say, well, it sounds like going to Disney. It sounds like Micky Mouse and Donald Duck. Eventually, most folks will say that after several months, the brain will get used to that and it just sounds like your normal hearing.
And interestingly, now that we've started to implant more and more adults, as well as children with what we call single-sided deafness, traditionally, cochlear implant is for folks who can't hear on both sides. But now, we've actually implanted, especially at Nationwide Children's, we've implanted a lot of children with unilateral hearing loss. And those children will eventually say the sound's almost the same. It's really interesting.
Dr. Mike Patrick: All right, I want to bring Dr. Winner back into the conversation. How do audiologists support kids with cochlear implants?
Dr. Krista Winner: So after Dr. Adunka does his easy insertion, which we got to give him more credit or he needs to give himself more credit for pretty detailed process, but once that implant is in, we'll see the kids back about two weeks after the surgery. We get to do, I think, the fun part of activating them.
So for families, it's a very exciting time, but it's also a very anxious time because for many of these kids, it's their first time hearing. So there's a lot of emotions that can happen but we always make sure that the child leaves comfortable.
And that new sound, it doesn't sound like normal hearing, they're going to need to come back for several appointments. So we'll see them a week later. We'll see them two weeks later. See a month, three months, six months, until we can find that we have a good what we call map or program on the cochlear implants.
So each appointment, when they come back, we kind of reprogram that cochlear implant. We put them in the sound booth. Whether it's their parent's lap or doing condition play we talked about earlier or hand raise, or just trying to find what's the softest level they can hear at different pitches and frequencies.
And then, we can go back to the programming room and we can make changes that are necessary to make sure that they have good access to all the sounds necessary for speech and language development.
Dr. Mike Patrick: How about you, Erin? How do speech-language pathologist support kids with cochlear implants?
Erin Stefancin: So I'm going to kind of talk about two different groups that we might see. And each child is different, but there's kind of two groups especially with cochlear implants and you treat them a little bit differently.
So I'm going to talk first about maybe kids that are born without hearing involved with their ears, that might need two cochlear implants at that nine month or older range. For those families, we typically see them, like I was saying about three to six months at birth.
And one of the really main things that we talk about with families is at that time, that child might be wearing hearing aids. But they may not be given enough access which is why they're going to get a cochlear implant. And one of the main things that we'll talk about with families is we'll talk about this piece of joint attention or shared attention between a parent and a child or between the children.
And joint attention is a really important aspect for children to learn words and for language and communication in general. So for a child that might be able to hear at that time we're seeing them, maybe three to nine months of age, they get the implant, we're really focusing on lots of opportunities for families to have that moment of joint attention and connect with their children as much as possible.
Once they get their cochlear implants activated, implanted by Dr. Adunka and once it's activated by Dr. Winner, then they come and see me. We will work on jumpstarting their language and also working as much on focusing on listening.
So that's kind of in some of those setting up their environments. We might be working on using some devices. There are different systems that can have parents directly give sounds to the cochlear implant.
And we work through developing our language kind of from the bottom up. So we might start with working on children participating and listening to songs, something that is very fun for the child. We might work on clack and twist rattle or might start working on some animal sounds. We call them learning-to-listen sounds. They're the sound-object paired association.
So pairing together and understanding that "baa" is sheep, and moo is a cow, all the way up through developing spoken language words when we work together. So we kind of move through the typical development of a child's spoken language but we try to compress it in that first year by giving, especially intensive therapy and intensive parent training.
For a child that might get a cochlear implant, as Dr. Adunka said, for a single-sided deaf, a deaf this case, a child that maybe has normal hearing in one ear and that's cochlear implanted in the ear that has no significant hearing loss, we might do a lot of direct streaming.
So it's been really beneficial to the telehealth because we're able to work on direct streaming or maybe putting a sound right to their cochlear implant. So that's the one that they're listening back, we input to their ears.
They might do some training in similar ways with songs, understanding words. And we'll work on you having the ear that is newly implanted with this cochlear implant and work on having them start having their brain understand this new way of hearing, and work more towards that ear as opposed both.
So those kids really are hearing with two different ways. They have the acoustic hearing, what they normally can hear, and then the electrical hearing with the cochlear implant. So there's a lot going on for that child. So we'll work on that with the family.
And for those kinds of children, we typically don't see a language delay because they do have normal hearing in one ear. There, really, the typical therapy is mostly focused on that listening piece and direct streaming to their newly implanted ear.
Dr. Mike Patrick: So Dr. Adunka, what is the long-term outlook for kids with cochlear implants? Are these devices that lasts a lifetime? Or do they need some maintenance or upkeep or replacement from time to time? So what does the long-term outlook look like for these kids?
Dr. Oliver Adunka: So it's a good question. So first of all, a long-term outlook for the device would be that we expect devices to theoretically last forever. Practically, I would say that after about 20 years we would see that a device, the technology upgrade would make sense.
So right now, we're typically upgrading still functioning devices that were implanted in the 1990s, all right? So that will be consistent with this. Usually, the external processors, those are actually the brain of the device. And we do upgrade those just without surgery, of course. That will be something for audiology and depending on the patient's insurance, et cetera, those would allow certain periods for upgrading.
In terms of what the long-term outlook is for the family, what we would say that coming back to what I said earlier, that we frontload our work, right? When Erin really starts working and her colleague starts working with a child, when things calm down on the audiology side a little bit, when we see them less and less often, then we see that about 8% of children that are non-syndromic, meaning that hearing loss is their only issue, that 80% of children actually have mainstream schooling with very little or no support. Which basically mean they will be discharged from Erin and her care and they would sit in classroom with your kids or my kids. And they would see Dr. Winner once a year for maybe just a hearing check and device support. So that is the a long-term.
I'm sorry, I know your question was more pertaining to the device, but you know, I wanted to put this out there, right? It's really important that our goal for all children and there's an educational goal, obviously, that we achieve by providing hearing and speech and language, that this goal is typically obtained in about 80% of people. And that's actually well document in prospective clinical trials as well.
Dr. Mike Patrick: So Dr. Adunka, tell us about the Hearing Program at Nationwide Children's. I would assume it's a multi-disciplinary approach. So who all is involved with your team?
Dr. Oliver Adunka: We're obviously extremely proud of our program. We have three physicians. So I'm one of the physicians. We have a large team of audiologists. It's almost 20 audiologists really who take care of hearing aids, hearing testing, cochlear implants.
There are also bone-anchored hearing aids or middle-ear implants that we service. So really a full spectrum kind of shop.
We have several speech-pathologists who work with hearing impaired children. Erin is one of them. And what we do every Thursday, that's a time to shine, I guess in the Hearing Program is when we really discuss cases as a team. And we want each specialty.
And I shouldn't forget we have a social worker, part of our hearing team. And we closely collaborate with behavioral health. Sometimes, they show up at our meetings. We closely collaborate with radiology because every child usually gets an MRI at least or some children, just a CT scan, so part of their medical workup, right?
So our model is a medical model of managing hearing loss. It involves all those group. Again, it does take a village. And people don't want to have more meetings. I do get that and back to back Zooms are sort of what we've all gotten used to.
But those are critical meetings for us and we enjoyed these. And frankly, oftentimes, you go in to this meeting and you've seen this family. And you have this your single-minded, preconceived notion and you hear all the other opinions, and all of a sudden, it looks a lot different.
So we really value the multidisciplinary nature of the input that we all receive. And oftentimes, I'll learn from Krista and her team or from Erin. What we basically say, our mantra is that if you take care of a child... So Erin for example would see a child with a hearing aid, that she feels like they should make more progress than they are, right, so then we really lower the threshold to basically zero for presenting that child.
And then, we would discuss, hey, is there anything else we can do? Have I done all the medical stuff I can do? Has Krista done all the audiology she can do?
Is there a social concern? Do they have barriers to making it to Children's? Can they do telehealth? Do we need to help them with a computer or something like that?
So those are all things I think that are really relevant. So we try to remove barriers for the child's success. And oftentimes, since there's a large educational component of hearing loss, we really try to rope in the school district. And that's really where speech-language pathology comes in and their educational efforts that grew along oftentimes with very close collaboration with the child's school system.
Dr. Mike Patrick: All right, I will put a link in the show notes to the hearing program at Nationwide Children's Hospital, so folks can find you easily. There are lots of links and educational items, resources for families. If you are interested in learning more about hearing loss, speech-language pathology, audiology, cochlear implants, all of these things, we do have links for you in the show notes for Episode 480 over at pediacast.org.
So with that said, Dr. Oliver Adunka, Dr. Krista Winner, and Erin Stefancin, thanks once again to all of you for stopping by today.
Dr. Krista Winner: Thank you.
Erin Stefancin: Thank you so much.
Dr. Oliver Adunka: Thank you, Dr. Mike.
Dr. Mike Patrick: We are back with just enough time to say thanks once again to all of you for taking time out of your day and making PediaCast a part of it. I really do appreciate that.
Also, thanks to our guests this week, Dr. Oliver Adunka, otolaryngologist and director of the Hearing Program at Nationwide Children's Hospital. Also, Dr. Krista Winner, audiologist, and Erin Stefancin, speech-language pathologist, both with the Hearing Program at Nationwide Children's.
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