Episode Transcript
00:00:00
(Music up and under)
00:00:03
Host: Hello and welcome to this edition of Cook Children's Doc Talk. It's a very busy day here in the Child Life Zone at Cook Children's so you may hear a little background noise. Today we're talking with the pediatric cochlear implant team. Since the invention of the electrical capacitor in 1745, to the first cochlear implant in the 1960s, to the first pediatric cochlear implant in 1980, the technology and procedure have come a long way Today, thanks to these incredible advancements, the cochlear implant team here at Cook Children's is giving children as young as three months the gift of hearing. Even those with profound loss. Here today to talk about it are otolaryngologist Patricia Purcell and audiologist Mandy Pendleton. First, a little about our guests.
00:00:53
Host: Dr. Purcell earned her medical degree from the University of Texas Southwestern Medical Center. Completed her residency at University of Washington, Seattle, with a focus on head and neck surgery. She completed her fellowship at University of Toronto, Ontario, and is board certified by the American Board of Otolaryngology, and has co-authored several publications related to pediatric hearing and surgery.
00:01:19
Host: Mandy Pendleton is a member of the American Academy of Audiology and American Speech Language Hearing Association. She earned her BA in Communications Disorders from Truman State University in Kirkville, Missouri, and her MS in audiology and an audiology doctorate from the University of Tennessee Health Science Center in Knoxville, Tennessee. She enjoys many facets of audiology, but has special interest in cochlear implant, electrophysiology, and supervising graduate students. Welcome and thank you so much for being here to talk about the exciting advancements in pediatric cochlear implants.
00:01:57
Mandy Pendelton: We're so excited to be here. Thank you.
00:01:59
Patricia Purcell MD: Thanks for inviting us.
00:02:00
Host: Cook Children's launched this program in 2004, can you start us off with a little background on the program since its beginnings to where we are now?
00: 02:09
Mandy Pendelton: This program launched in 2004 like you mentioned, neither of us were here at the time, but we have had the pleasure of looking back and thanking those who started the program. It started with an audiologist and an auditory verbal therapist, really just wanting to provide cohesive care. At the time, Cook Children's did not have an otolaryngology department, and so they did have to start that program with an outside physician, and then in 2017 Cook Children's added otolaryngology, and we had a CI surgeon join our ranks. So from that point, our program has really grown as we're all under one roof, and we're able to communicate very easily and provide cohesive care. I have our 2023 - 2024 yearly report in front of me, still waiting on the 24 - 25 numbers, and the program has implanted 645 ears and a total of 400 patients in that time.
00:02:59
Patricia Purcell MD: I think it's exciting, in general, to talk about some of the trends in cochlear implantation. Over the last 20 years, there's been so many advances in this field in terms of appreciating the benefits of early intervention. I think we've seen children here, and kind of across the U.S., implanted at a much earlier age. You know, the mantra is kind of, as soon as it's safe to do the surgery, we should do it. And then the other idea is the benefits of bilateral hearing. So in the past, when we first started with cochlear implantation, most children just received a single implant. But now, for a child who is profoundly deaf, the standard is really bilateral.
00:03:39
Host: What are some of the more common causes of hearing loss and the most rare you've seen that benefit from a cochlear implant.
00:03:47
Patricia Purcell MD: So I think it's important to remember that most children who have hearing loss are not going to be cochlear implant candidates. Most children benefit from other interventions, such as a conventional hearing aid. So the most common cause of hearing loss is not going to be a cochlear implant candidate. But when we think about what inner ear hearing loss or sensorineural hearing loss, that's where we start to consider cochlear implant candidacy. And the most common cause in children are going to be genetic, and that's different from adults. Most of the adults that you may know who receive cochlear implants, it's from a progressive hearing loss, gradually over time. Whereas in children, if you're talking about a congenital hearing loss, they have not necessarily formed those same auditory networks, and so we have to approach implanting them differently.
00:04:38
Mandy Pendelton: Also, here at Cook Children's, we have the luxury and some of the puzzle piecing that we can see some of those rare, rare cases. So we get the opportunity to work with families whose children have suffered from meningitis, which is not the typical path for a cochlear implant candidate, but it becomes a very urgent path for cochlear implantation, due to the potential of ossification. And then we also get to see children that have complex medical histories and diagnoses that make them challenging candidates and also challenging users.
00:05:12
Patricia Purcell MD: Yes, there are definitely some challenges that go along with implanting infants and toddlers, and some of them are medical so, you know, of course, we're a little biased towards hearing but many of these children with genetic syndromes will have other underlying conditions or having a complex medical history, whether it's prematurity or other medical problems, we know that it increases a child's risk of hearing loss due to exposures early in life. So there may be competing medical challenges, and so we have to figure out how cochlear implantation fits into that picture.
00:05:49
Host: I read in an article that as of the end of 2024 your team has performed over 600 cochlear implants since the inception of the program. What is the youngest patient you've treated, and what is the most complex implantation that you've performed?
00:06:06
Patricia Purcell MD: Yeah, so you kind of touched on that a little bit with meningitis. So there are not very many hearing emergencies, but we do put meningitis in that category because of the condition labyrinthitis ossificans, which basically means that the cochlea, the inner ear, it ossifies and can no longer accept the implant electrode array. So that means that that child, if that process is completed, is no longer able to receive the implant. And it happens quite quickly. It can happen within six to eight weeks of meningitis being diagnosed. And so for children who have a diagnosis of meningitis, an early hearing test, ABR, is critical. That should be part of the workup. And we have made that standard here.
00:06:54
Mandy Pendelton: Yeah, in my 10 years here, I've seen us go from sometimes missing those kids immediately to working cohesively with otolaryngology and infectious disease to identify and test those kiddos as soon as possible. I've spent hours in the PICU talking with these families. They think they're just here because they have to fight this infection, and then you have to share with them about the loss of hearing and that we need to take some immediate action, and those can be super emotional times, but coming out the other side with those families is so rewarding. I can look back on some of my older kiddos now, eight, nine, ten years old, that were meningitis babies, and you wouldn't know most of them have cochlear implants because they are meeting their milestones so well.
00:07:42
Patricia Purcell MD: Yes, again, it shows the benefit of early intervention, because many of them are implanted extremely early. So the earliest that I have done was five months. The surgeries are pretty challenging because there's always inflammation in the inner ear. And of course, the anatomy is pretty small, but I think we're going to talk a little bit more about the surgery later.
00:08:03
Host: How does hearing loss impact a child's cognition and communication development skills?
00:08:08
Patricia Purcell MD: I think this is another important point where we need to distinguish between congenital hearing loss and progressive hearing loss. So children who have never had access to sound, who are born deaf, the role of the cochlear implant is somewhat different in terms of the listening brain. So the listening brain in a child who is profoundly deaf is almost like a blank piece of paper, and then as they hear each bit of sound is almost like a brush stroke that develops that cortex. And as you can imagine, with more and more brush strokes, that cortex gets richer and more developed. And so if a child has never had that access to sound, then the cochlear implant really has to serve that purpose. And we know that there's a critical period for that type of development, and that's no later than four and ideally less than two years of age. So the brain has the plasticity to be able to develop that cortex during that time. If you miss that window, then that child will never have the listening brain that someone who is heard during that period of neuroplasticity does. And so that's why we encourage early intervention for children with hearing loss, and this same principle applies to all types of congenital hearing loss. So if, even if you're providing a hearing aid, it still offers some of that benefit. But of course, for a child who's profoundly deaf, a hearing aid is not going to create enough stimulation to form that listening brain.
00:09:37
Mandy Pendelton: When I think about my older kiddos who have had progressive hearing loss. I had a very articulate 12, 13 year old a few years ago, and she looked at me the first day we were talking about implants, and she said, "My world is losing color, my world is getting more black and white, and I want vibrancy in my world," and I'm never going to forget that conversation, because it was so profound for a preteen. And I look at her now and and she's thriving, and she looked at me the other day and said, "Thank you. Thank you for allowing me to paint the picture I wanted to." And so even when we have those formed connections in our brain, we're enhancing them, and we are enhancing the quality of life that these patients get to have.
00:10:26
Host: And I love that description of like connecting hearing to like colors, because it's a little easier, maybe, for somebody who hasn't challenged with that understand that. So when they have never heard for the brain to accept it. Do you have to do those in small, slow steps?
0010:41
Mandy Pendelton: Yeah. So I always say we're the tortoise in this scenario, and I tell families the number one priority after activation of that cochlear implant is first-bonding. Bonding between the child and their implant, bonding between the child and their parent regarding the implant, and then bonding between the child and their audiology provider, because it is, it is a slow trickle, and we give them a little bit of sound ... on day one, they're gonna go home hearing me yell at them, because I use an analogy with families, if you had never been in a car before, and you went up to the speedway and got in a car with a professional driver, you went around the track at 120 miles an hour, would you set foot in a car again? Probably not. Okay, so that's how I approach giving kids access to sound with their cochlear implant. We want to drive down the neighborhood at 10 miles an hour so that we feel safe and confident in what we're doing, and then we're gonna ease into it. And thanks to their brain plasticity, sometimes we can go pretty quickly because their brain allows it and their personality allows it. You sometimes have those more sensitive friends who you gotta take a little more slowly, but that's just how each of us approach life, and it's about knowing your patient and following their cues as well.
00:12:01
Patricia Purcell MD: And I think another concept that's important is the hearing age of the patient. I think sometimes when children are provided with an implant, even with the best of situations, we're often activating them around 10 or 11 months of age, so you know, around one and so that means that in terms of their communication development, we would expect them to be, at first, about a year behind their peers, and so it's not a problem. It just means that they need a little bit more time to catch up. But we know the data shows that for a child who uses their implant full time and is able to really focus on those important fundamentals of audio verbal therapy and attending the audiology appointments for appropriate programming. They can achieve normal language development. They can be mainstreamed in the classroom. It really is important to give them a little time at first, but know that they will catch up if we just continue to provide the support that that listening brain needs.
00:12:59
Host: What are the latest advancements in pediatric cochlear implant technology, or surgical techniques that you are most excited about right now. What are the biggest game changers?
00:13:10
Mandy Pendelton: Technology in and of itself, cochlear implants have entered into the streaming world and have entered into the app world, and it's all about ease of use and accessibility to the things that their peers have. If a kiddo is on a long car ride, there's probably a tablet in front of them. They're watching something to keep them occupied. Our kiddos that have cochlear implants now have the luxury of being able to do the same thing without any intermediary devices, and they can stream directly to their implants, which is game changing for a parent who no longer wants to hear the same song on repeat, but also our kids learn so much through what they watch and what they hear, and this is just giving them more global access to sound and those lessons that they experience in that. We're also getting really smart internal electrodes arrays, right? Like, there's some cool stuff coming out.
00:14:10
Patricia Purcell MD: Yeah, I think one of the neatest things about all the technology nowadays is that I think it has reduced some of the stigma about, you know, wearing an ear level device. Lots of people wear them for all kinds of reasons, and they kind of have a cool factor in a lot of ways. So I think that has helped, especially teenagers, feel more comfortable using their hearing technology and definitely talking about individual level programming, where we're looking at kind of the position of the electrode array. We're using imaging to get a better idea about where the electrodes are sitting within the cochlea, so then we can fine-tune the programming based on that to get the best hearing outcome.
00:14:51
Mandy Pendelton: Yeah, so we're using that information to place pitch match. So the cochlea has its own home for each frequency or each pitch that we have, and when that electrode array comes in, sometimes that aligns nicely and sometimes it doesn't. And so this technology allows us to go in and align them so we're not getting any competing sounds, any competing dissonance, and we can have the best clarity of sound for these kiddos, so their brain can continue to adapt as quickly as possible.
00:15:22
Host: In your respective fields, what is the most common misconception you hear from parents when they are first considering a cochlear implant for their child, and how might a referring physician help to address these with the family early in the process?
00:15:36
Patricia Purcell MD: I think as the surgeon, whenever I first meet with the family. Definitely the focus is on anxiety about the surgery and how is the surgery going to go, which is very understandable, but I always want to direct the parents that really the surgery is probably the quickest part of this whole process, while it produces the most anxiety early on. Really the important work happens after the implants are in, and so we need to have a plan in place for device retention and making sure that they are able to attend appointments and all of those other factors that may kind of get forgotten about a little bit when we're so focused on the surgery.
00:16:19
Mandy Pendelton: Surgery also comes up often in my appointments, and I just encourage them to reach back out to the surgeon, because y'all are that expert. But so often I have families that tell me, "We want to allow our child to make the choice for themselves if they receive a cochlear implant or not", and that usually leads to a conversation about bodily autonomy and how it is important, but also how, as a parent, you are able to make those big, life changing medical decisions. And then it launches into a conversation about brain plasticity, and that our chances of catching up to where our peers are. Communication wise, sometimes, you know, other development wise, really, it's so important to have that in place by 2, 3, 4, at the latest, if we want to catch up. And that we don't have to then say other modes of communication are unacceptable. We live in a world that has bilingual, trilingual people, and if you would like for sign language to be an additional language in your family, then make the commitment together to do that, and I'm seeing that with a lot of my teenagers at this point. High schools in the area have introduced sign language as a foreign language option, and a lot of my patients, even with cochlear implants, who've had them since birth, are seeking that because they just want to be aware of their options and involved in the capital "D" deaf community?
00:17:45
Patricia Purcell MD: Yeah, I think it's important to keep that perspective in mind. That there is that window of opportunity for implantation, and once that is gone, it's really closed. So you can't come in later as an 18 year old, when you can consent for yourself and make that decision, but you can, if you are a young adult and you wish to communicate solely with sign, you can do that so having a cochlear implant doesn't eliminate that choice.
00:18:15
Host: From a multidisciplinary perspective, what are the key indicators that suggest a child is a good candidate for a CI versus a traditional hearing aid.
00:18:25
Patricia Purcell MD: So there are a number of things that we look at. As the surgeon, I definitely rely on the audio metric data from ABR testing or even sound booth testing, behavioral testing, but I also need to look at the anatomy to make sure that the child is anatomically a candidate for implantation, and so the children who are congenitally deaf are at higher risk of having an inner ear malformation that may preclude placement of the electrode. And those are always tough conversations to have when we get the imaging and we find that there's really no place to put the implant. And so that's always something on my mind, to get the imaging in a way that is early before getting too far along the process.
00:19:14
Mandy Pendelton: I also think about just how the criteria has loosened over the past 10 to 20 years. You know, 20 years ago, it was just profoundly deaf, like no access to anything. Now we have the ability to consider single-sided deafness for cochlear implantation, and we have the ability to look at those who have a precipitously sloping hearing loss. So might have some normal hearing in those low pitches, and then it just drops off, kind of like a ski slope. And I also just think about we have more tools from our audiologic perspective now. We look at how the child is doing with their hearing aids, but we also look at what's called the speech intelligibility index, and that gives us a percentage of understanding. And we can look at that with the hearing aids and without the hearing aids. And so a lot of times that SII function in kiddos that are cochlear implant candidates. Unaided is 0, 5, 8, and then even with the best set hearing aids, it usually jumps to somewhere in the 60s. And the latest research is telling us that anything under 64% on that aided speech intelligibility index is really a place to consider cochlear implant candidacy. And ,so then that is a launching pad for us to do a full workup. Discuss anatomy like we've talked about, discuss family intervention and motivation, and also discussing hearing aid use, we've alluded to it a few times, but kiddos that are wearing their hearing aids are keeping so much of that brain listening. And if we take those hearing aids off and say, hey, they're not doing anything, we're losing a little bit of that chance to catch up. I like to say that we're keeping the sound highway open by leaving those hearing aids on. Yes, we know, we know he can't hear you when you whisper to him, but we are keeping that auditory nerve doing its job because we know that brain is super, super smart, and if it's not using something, it might make a different decision. So we got to keep those hearing aids on so we have the best chance of outcomes after implantation.
00:21:28
Patricia Purcell MD: Yeah, it's been a good addition. I think the aided SII, I agree, because one of the challenges, especially for infants, is trying to determine how well are they responding to hearing aids? When we think about doing a hearing test, typically think about, oh, raise your hand if you hear the sound. But if you're talking about an infant or toddler, they're not able to participate in that way. We do have ABR testing, but what if we get an ABR test and we find that it's kind of moderately severe hearing, you know. So there are some real borderline gray areas, and I think having that aided SII has been nice for counseling families who fall into those categories.
00:22:12
Mandy Pendelton: Well, and with that gray area, we've instituted quarterly team meetings over the last two years, I think it is. And those can be really great times to look at each other and be like, "Hey, I don't know what it is, but something's holding me up here. Like, do you get that gut feeling too?" And we've got monthly discussions where we can bring our concerns together and then determine who's gonna follow up. So if it's, "Hey, I'm really concerned this kid who isn't wearing their hearing aids as many hours as I would like," I can say, "Hey, Dr. Purcell, I got it. I'm going to bring him in. I'm going to check that data logging, have a quick conversation with the family, and then I'm going to let you know what that updated information looks like. And then we can make decisions together on our comfortability moving forward.
00:22:57
Patricia Purcell MD: And I think another important thing to keep in mind that you briefly touched on is we're appreciating cochlear implants don't have to remove all residual hearing. I think that's been a big move forward in our field, and it has really reshaped kind of our candidacy model too. So especially for low frequency hearing, you can place an implant and essentially have the same unaided hearing test with the implant that you had without it, which is pretty neat with our newer electro arrays and surgical techniques.
00:23:32
Mandy Pendelton: Well, and going back to advancements, we now have the option to do electric acoustic cochlear implants. And so what that means is then we get to keep those low base tones in a more traditional hearing aid setting while those higher frequencies are utilizing the actual parts of the cochlear implant. And then if things change over time, we're able to change those lower pitches into that cochlear implant setting. But we know that the more natural the hearing, the easier the child is going to have at adapting. And so if we can keep some of that natural, low pitch hearing in place, their chances of catching up with their speech and language not having any articulation problems, is really going to make a difference, because it provides a more robust, natural acoustic sound.
00:24:24
Host: So for a child that, say, an infant, gets their cochlear implants early on, versus an older child who gets them later, there's a big difference in them, I guess, kind of catching up in their hearing or
00:24:36
Mandy Pendelton: Yeah. So if we think about those children who are prelingually deafened, so have no language. When they're without hearing, they have no concept for what sound is or what language is. So once we activate that implant, we're starting at infancy. We're starting at a hearing age of zero, and that is something that is challenging to remind parents of, like, "Hey, we are ... we're one month out from activation. What does a one month old baby do when you talk to them? They're not responding to their name at that point, and so when they are, you can really be excited about that. Whereas older kiddos, they have a basis for sound and for speech and language, they know what it sounded like if it changed over time. And so then we just have to help retrain their brain to take what they knew and then what they hear now with the cochlear implant, kind of mesh them together. But since they have an understanding of what language is, that usually is a pretty quick catch up, as long as they're doing some intensive auditory verbal therapy. Streaming has been a game changer for our teenagers who are implanted later in life, because they can go and listen to a book on tape or an audio book or their favorite TV show, and they know those words by heart, so they can listen to them and make those connections in their brain without feeling lost in what's happening, and that's just not possible for children that have no concept of what language is.
00:26:10
Patricia Purcell MD: Yes, I've had several older children tell me that once they receive the implant, it really helped with understanding the clarity of speech and language, and so I think that's another way to show they knew what it sounded like before. As they progressively lost their hearing, they realized they were missing that, and then they received the implant, and it came back.
00:26:28
Mandy Pendelton: My favorite is a little friend who described his implant as sounding like scary Santa, because he knew there was speech there, but he was like, This is not what I'm used to. I mean, over time, that was one of my questions every visit. Hey, how's Santa sounding? And he's like, he's not so scary. And I was like, okay, okay, so they know what speech is, but it's different.
00:26:51
Host: How do you approach the conversation with parents about setting realistic expectations for their child's hearing and speech development, post implantation?
00:27:01
Patricia Purcell MD: I think we've touched on this a little bit with the idea of the tortoise. Take it slow. Understand what the child's hearing age is, which is different than their birth age, and give them grace. Because of that, it's important not to make direct comparisons, even with other children who have implants, because every child is different and has their own challenges. So I think having those conversations early and then also emphasizing the importance of full time device use.
00:27:32
Mandy Pendelton: Yeah, I'd like to start this conversation by asking the family what their expectations are post implant, because I might think I know what they're expecting, but they could have a total different expectation. They could say, Hey, we've done our research. We know it's going to take three years for Billy to catch up and be saying things like his friends in preschool. And these conversations are also so individualized, so if we refer back to our medically complex kiddos, they might not have the ability to acquire full speech and language. So that discussion with that family is going to be very different. It's going to be about sound awareness, safety skills and not full language acquisition. The brain still plays a huge role. We can provide access, but what the brain does with it is up to the brain, and we don't always know what each brain is capable of.
00:28:23
Host: Could you walk us through the surgical procedure for pediatric cochlear implantation, highlighting any unique aspects when operating on children?
00:28:32
Patricia Purcell MD: Yes, I think a lot of parents have questions about what the scar is going to look like. Is it going to be very noticeable? There are concerns about that, maybe based on what they saw on TV or in a movie. And so I try and reassure families that it's a very cosmetically favorable surgery. The incision is placed behind the ear. It's usually only about three centimeters, and honestly, after a year out from surgery, most of the time, I can barely tell which ear was implanted if they have just a single implant. So it really is cosmetically favorable. The other thing to consider is we don't have to do a lot of shaving of the hair to prepare the surgery site or anything like that, so we try and focus on minimally invasive techniques when doing the surgery. The surgery takes, usually about two to three hours to complete. It is done for older children as a day surgery. If it's a young child having both ears implanted at the same time, then sometimes we do observe them overnight, but almost always they go home the next day. So the procedure involves drilling through the mastoid bone to get to the inner ear, to the cochlea. Typically, the insertion of the electrode array is done through the round window, or near the round window in a cochleostomy, if the round window is not immediately accessible. There's often a question about size. You know, can we use the same implant and an infant as in an adult? And actually we can. Which is pretty remarkable. The cochlea is fully formed, so we don't have to take the implant out when they turn five or six and replace it. They can keep that same one as long as it will last. Now, the mastoid bone is much smaller in size than an adult, obviously. So the mastoid bone in a infant or toddler is about a quarter of the size of an adult. So definitely, with surgery, you have to be thoughtful about that and use equipment that in an appropriate way for a much smaller patient. But yes, in a lot of ways, the surgery is quite similar to adults.
00:30:34
Host: Once you've implanted it, is it there forever? Or is there a point where it has to be replaced?
00:30:40
Patricia Purcell MD: We would like it to stay in as long as possible. And that is a common question. How long do they last? I usually quote, hopefully at least 15 to 20 years occur. The newer technology that being said, if there's a event where the implant takes a direct hit, I have seen the device malfunction in that situation. So it is important for children who have cochlear implants, as for everybody, to wear helmets and avoid direct contact to the implant site.
00:31:11
Host: You also mentioned that you have to drill through the mastoid bone. So does the bone grow back around, where you place it, or does it just go in and it's not in the bone.
00:31:21
Patricia Purcell MD: It can, yeah, a lot of that bone will regrow. It will kind of grow around the implant as the child grows. So in children who've had their mastoid bone drilled for almost any reason, if we go back in, we'll see that most of that bone has actually reformed on the outside, what we call the cortical part of the bone.
00:31:39
Host: So then you just do have to remove that? Or do you just drill through another space? Like, how do if you're replacing…
00:31:45
Patricia Purcell MD: If you're replacing, yes, sorry, yeah, you just redrill it again. Okay, if you need to, you can drill it again. There's not any downside to doing that. Yeah.
00:31:53
Host: So what criteria do you use when determining the timing of implantation? Like, bilateral versus unilateral age considerations?
00:32:03
Patricia Purcell MD: Like we've talked about, earlier is better, as long as it can be done safely. So for children who have genetic syndromes associated with other medical problems, sometimes we do need to address those other medical problems first. Do they have congenital heart problems? Do they have airway problems? So we need to make sure that medically, they are able to go through the surgery safely and then also participate in the programming and everything satisfactorily afterward. But now, with the growing appreciation for the benefits of early intervention, we are able to offer cochlear implantation for children down to nine months of age by FDA approval, and for children who have meningitis, obviously we're implanting even earlier than that.
00:32:49
Host: What is the typical post operative recovery timeline, and what are the signs parents and physicians should monitor in the days and weeks following surgery?
00:33:00
Patricia Purcell MD: I think that families are really surprised how quick their kids are back up and at it, because we talk about, Oh, you want them to take it easy, not to bump their head or fall or trip too much during those first weeks after surgery, and we'll have the post op visit usually is scheduled about one week after surgery, and the parents will tell me it's so hard to get them to stay off the couch, jumping on the couch so the recovery isn't too bad. Tylenol and ibuprofen is all that I typically recommend for pain. I do put a mastoid or a head wrap type dressing on for 24 hours after the surgery to help reduce swelling, and then there's gonna be some skin glue over the incision that keeps it sealed. But parents really don't need to do wound care or anything like that.
00:33:50
Mandy Pendelton: Wow. Yeah, they are always surprised. They're like, we had family recently, we brought out the PlayStation, and even that wouldn't keep her still. And I was like, "Hey, we're glad she's up. We're glad she's feeling herself. Now it's time to get her some sound," which really could lead right into our next question about, what does activation look like? So timeline wise, we usually turn a kiddo on or have initial activation three to four weeks post surgery, and that's a surgical preference of the timeline to allow for the swelling to go down and kind of everything to settle. Activation day is probably one of my favorite days. It always is. It's just a day full of possibility, and kiddos and adults can be very similar. We start with a lot of objective measures, and so we want to make sure that that implant is communicating from the outside piece, the external piece, to that internal piece. And then we can get some evoked compound potentials. And that is really looking to see where that auditory nerve is firing and at what presentation level. So how many charge units do I need to put in there for that to occur? Yeah, and then after that, things kind of deviate from kiddo to adult or older teenager. There's a reflex in our ear called the electrically evoked stapedial reflex, and we know that by getting those responses, we can set an implant at the optimal level, but it requires you to sit really still.
00:35:21
Host: Yeah, so toddler ...
00:35:24
Mandy Pendelton: Yeah, it's not always great in my toddlers and babies. Sometimes, you know, we can aim for one electrode to get those on. I've had babies come in asleep, bring them in after a therapy appointment, let them kind of just settle down, fall asleep, and then we can get those measurements, whereas a 15, 16, 17 year old, you can say, Hey, stay here, read your book, and I'm going to do this measurement. But then after that, we get to kind of just turn on the sound around them. So I take those measurements, and I know that's where I want to end up. But again, if I turn them on right there, we're not going to have a great rest of our day. So we turn 'em down, and then I like to involve everybody in this process. So I've got a box of musical instruments. I've got some cabinets in my room, and I give everybody in the room a job. So if there's big brother there, he's got a tambourine, and when I point at him, he gets to just shake, shake, shake. Usually I have dads stand and give that loud dad clap to see if we can get a startle response. And these days, sometimes it's just mom, dad and the kiddo. Other days, I've had 20 family members come to activation, and everybody wants to be a part of it, and there are usually tears all around, mom, dad, grandparents. What possibility the future holds with access to sound?
00:36:45
Patricia Purcell MD: Yeah, I wish I could go to the activations. I've only been to a few, but I think it's important for families to realize that every activation is different. And so again, this is where kind of having those expectations, maybe something that you saw online, or something that you saw represented on social media. It may ... your child's experience may be different. They may be sobbing by the time they're out of you know, ready to go, and that's okay, but the fact that they're responding to the sound is so important.
00:37:17
Mandy Pendelton: My key phrase there is any response is a positive response, because any sort of response signifies that that child understands there's a change, and whether it's the inquisitive child who just kind of looks around at everybody trying to take it in and put those puzzle pieces together, or the kid who sobs, or the kiddo who does just light up and looks at his mom when she calls his name, and who is the perfect Instagram story or whatever it may be, all of those responses are equally positive in in my eyes.
00:37:52
Patricia Purcell MD: Yeah, because they highlight that there is some intact auditory network there. And again, there are, unfortunately, sometimes when children have inner ear malformations or don't have a well formed auditory nerve, even with an implant, we're not able to get that kind of a response. So sometimes, for children like that, we will still try, especially if they're profoundly deaf. But again, this is where highlighting any response is good, because it means that there's an intact network there.
00:38:23
Host: How has the rise of early intervention influenced your approach to pediatric CI surgery?
00:38:29
Patricia Purcell MD: I think it just really continues to push us to get children in as soon as we can, to realize that while hearing is not necessarily the same emergency as a cardiac arrest or something. It is something that should still be given priority, especially in congenitally deaf children. If the goal for the family is for that child to achieve fluent spoken language, we need to provide them with the support to make that happen to the best of our ability, and so that sometimes means we need to rearrange our clinic to squeeze those patients in. And we're always happy to do that. So if there are any primary care providers who have a concern about a child's hearing, I would recommend that they reach out to ENT, audiology, to really try and get that hearing status confirmed sooner rather than later.
00:39:26
Host: And that brings up the question of the primary care, whether it's a primary care physician or a pediatrician, which is still primary care. But at what point do they get involved? And then what is their long term involvement for those patients?
00:39:38
Mandy Pendelton: Sometimes those are the first providers to refer them to audiology or ENT if they fail their newborn hearing screening, which became universal in the early 90s. But a lot of times we have techs that are completing those screenings within the hospital, and a technician is just not always equipped to know the implications of a failed screening, or to pass along potentially challenging news to new parents. So if you do get a child that failed their initial newborn hearing screen, we like to repeat it at one time, and then after that, the recommendation is to go straight to a full diagnostic workup with audiology. The faster we can get them in to see us, two - three months is great. So we can really hit those early intervention goals of screened by one month, identified by three months, and fit with technology by six months.
00:40:33
Patricia Purcell MD: Yes, those goals are so important. And I think as we have been able to learn more about the cause or etiology of hearing loss on a national level, there's been a lot of interest in trying to improve detection of conditions like congenital CMV, which are highly associated with sensorineural hearing loss, and also much quicker, to refer children to genetics for genetic testing, because oftentimes we can identify the cause of the hearing loss from that as well. And there's a lot of really important research going on in terms of the genetics of hearing loss too. So I think there's just been a growing appreciation for if we can make these steps earlier in this child's life, it will impact them throughout their lifetime.
00:41:16
Mandy Pendelton: When I think of the implications that genetics has been able to play in our family's lives as well. So if you are able to identify the cause of your child's hearing loss, you're then also able to know what additional steps might be needed for future children, and be able to kind of move a little bit faster in those cases as well.
00:41:38
Patricia Purcell MD: Yes, I definitely have had situations where children were born into a family with siblings who were identified in a delayed fashion, but then the younger child was able to be implanted before 12 months of age because of their siblings history. So I think there is growing recognition by identifying the cause, it definitely makes a difference in terms of our counseling.
00:42:01
Host: So could you explain the process of initial activation and mapping for a young child, and how is this different from mapping an adult?
00:42:10
Mandy Pendelton: So we've talked a little bit about how an older child can sit there and is able to do some more testing, whereas a younger child, I'm really watching for an eye shift to a loud response, or even like a little mouth twitch up in that direction, whereas an older child can say, Oh, I hear that there was something there. It sounded very odd, but there was something there. Then from that appointment, they're able to go home with some preset programs and able to work through some additional, essentially, volume and make some progression between appointments with me. And usually we see them at about two weeks post activation, and they've worked their way through some of those programs and are typically detecting moderate to loud sounds. Sometimes our overachievers are definitely either understanding some things or hearing some softer voices, and then we follow them up at one month post activation, and then, typically, every three months for a while. And every child is different. Some are slower to adapt, and so they need a few more appointments to get those adjustments made, but all the while, we're checking in the sound booth with their devices on to say, "Hey, where are you hearing me now? How much progress have we made, and how much progress do we still need to go to get you detecting sound with those cochlear implants in the normal hearing range?
00:43:37
Patricia Purcell MD: Yes, and I think something for the younger children that really comes up is just the retention. So just keeping the devices on. You know, for older children, we can focus on the programming and such. But sometimes with the toddlers, it's a win if they can just keep those implants on for six hours a day, you know, and gradually just see that number go up because the magnet, there's not really a way to affix it so that it won't come off if the child pulls it off or gets really active. There are some great tips, though, that you can use, like clips or pilot caps or pilot caps
00:44:16
Mandy Pendelton: Hearings, hearing bands, little headbands that take some of that pressure off of the ear for a kid who is overwhelmed by that sensory aspect, or their ears just aren't big enough to hold the actual device on. And this is where parents' creativity really, really shines, because they come in with things I would have never imagined. 3D printing now is a thing, so I've had some parents get really creative with that aspect and provide their own retention solutions with technology and resources they have, and then we're able to share that among each other and among other patients. We kind of just build this own little support system.
00:44:55
Patricia Purcell MD: Yeah, I know some parents even market their things online. And such, they've found a talent with that. So there's a lot of options for retention. We don't ever want parents to feel like they're out there on their own when it comes to those kinds of challenges, because we definitely can provide some support.
00:45:14
Host: I love how challenges bring out human ingenuity.
00:45:17
Group: Yes.
00:45:18
Mandy Pendelton: Well, you know, I talked earlier about trust with your device as a child, and also trust with your providers. And I cannot tell you how many times I've just spent a whole session on the floor with the child to get the device on their head. And stuffed animals are key in that, as are family members and trusted friends who get to be a part of it. So we never want families to feel overwhelmed by the process and if retention is their biggest struggle, hey, we do not have to do a traditional mapping session. Let's figure out what your biggest struggle is, and we're gonna sit right there, because our job is to help you along this journey, and that's exactly what we want to do.
00:46:02
Host: What role does early and consistent auditory verbal therapy play in maximizing the outcomes of a cochlear implant? And what resources do you recommend to families?
00:46:13
Mandy Pendelton: Auditory Verbal therapy is the other component to this team. These are our specially trained speech therapists who have a very vast understanding of how children with hearing loss have different speech and language struggles than a typical hearing child. And the heart of auditory verbal therapy is actually family centered care and coaching. So our AVTs want to provide skills and knowledge that the family can take home and implement in order to get those brain pathways moving as best they can. And so we recommend that our families are enrolled in consistent auditory verbal therapy prior to surgery, and then that they resume following surgery, so that they have an understanding of what is happening. They're looking at audibility. So what is the child hearing? What are they not? They can then report that back to us as the audiologist, they see those children on a weekly basis as well. So those are really are detectives on the team. I think if they see something that's concerning, they can reach out to the surgeon, the audiologist, and then we can all work together to find a solution, whether that is as a team or collectively with the family. And then, really they're kind of the feet on the ground, again, reinforcing that full wear time of the device. They are able to know if a child comes in, and you can tell the difference in a kiddo who came from school and didn't have their devices on, and then parents pulling it out of the bag and slapping it on their head, that kiddo is a little more resistant, sometimes, to wearing their device and participating in therapy. Whereas a child who is wearing it all waking hours is going to feel that magnet fall off, and they're going to make some effort to put it back on, or to signify to their trusted adults or guardians, something happened. My battery died. They take it off and they hand it over. And so those AVTs are really helping the families say, hey, that is your child's way of saying something's wrong. They're not taking it off because they just want to take it off. They're taking it off because they need you to help them fix something.
00:48:27
Patricia Purcell MD: Yes, I think the AVTs are definitely, if there's the three multidisciplinary specialists involved in hearing health, they're just as important as audiology and ENT and for a lot of the children we talked about initially, they're borderline candidates for a cochlear implant. We want to see how well they're doing with their hearing aids. They're wearing their hearing aids full time, but the speech and language growth is just not where we would expect them to be. That's where we really rely on the auditory verbal therapist to weigh in and say, Should we start talking with the family about cochlear implantation? And they've definitely played an important role in surgical decision making for multiple families. Another thing I think that is important for AVT is realizing that a lot of that work, again, is done at home as well, so it's not something where they can just attend the appointments and see the results. It really does need to play out in the home, but I think giving families the resources so that they can do that on a daily basis is going to maximize that child's speech and language outcome.
00:49:34
Host: What are the most frequent device management challenges like maintenance troubleshooting that parents and kids face and what is your best advice for them?
00:49:44
Mandy Pendelton: Device Management is so tricky. These families go home with a huge backpack per ear. So goal number one is, let's worry about daily management. Let's be able to change the batteries. Let's be able to get it on your child's head. We usually go over that with them as an audiologist, but we also have great resources from the cochlear implant companies that are able to support the families in that. And then these families go and they build their own support systems, meeting other families in the waiting room, knowing someone local that has a similar situation, joining support groups online, that sort of thing. My first piece of advice is, let's get the mastery of every day. Let's get it on their head. Let's know what to do if the cable breaks. And then, second of all, find an organizational system that works for you. I am not one who thinks leaving everything in a sealed box is a great way to find something when you need it. And these families get photo organizers, jewelry organizers, tackle boxes, and they put all of their parts and pieces in there, and then they have a quick and convenient way to troubleshoot when something comes up.
00:50:55
Patricia Purcell MD: And I think another challenge, especially for the younger children, is figuring out how this device management can also be transitioned to the daycare or preschool setting, where the caregivers are not going to be as familiar with hearing technology, and sometimes that can involve really an educational session with them, a teaching session. We're happy to always provide whatever support we can, whether that's letters or even a phone call, just to really help to make sure that that child has access to sound all day, not just when they're at home.
00:51:30
Host: Beyond pure speech understanding, what are some of the other key benefits you observe in children with CIs social academic safety? Safety is a big one.
00:51:43
Mandy Pendelton: I think I see these benefits for all of them, but looking back over the years, I've been doing this most powerful benefit I see is inclusion. Inclusion with their families, inclusion with their peer systems, and the psychosocial implications that go along with that, patients that previously had no way to communicate with their families and tell them what they were feeling or what they needed. But we've talked about kiddos who are thriving and hear well and have met their speech and language milestones and are in a mainstream classroom. I tell families all the time, what are your dreams? What were your dreams for this child before you found out that they had hearing loss, those dreams are not gone. Those dreams are still achievable, and we're going to encourage that child to continue dreaming of all the possibilities as they get older. So safety. Yes, we can hear the fire truck coming down the street. We can hear the fire alarm going off at school. I've got kiddos that are participating in marching band, choir, theater, where 10, 15, years ago, I feel like a lot of them might have been missing out in those extracurriculars. I've got kids playing football and all sorts of other sports. So they're out there and they're living the life they want to live, regardless of their hearing status or the use of their cochlear implants.
00:53:06
Patricia Purcell MD: Yes, I agree. I think it's again, you mentioned this at the very beginning of the podcast, that it's important to understand the family's expectations to sit down with them. You know at the first meeting after hearing loss has been diagnosed, especially if it is profound or deafness, and say, what are the family's goals? What do you envision your child's goals to be in the long run? Because some families, their goal is for their child to communicate with sign language, and everybody in their community communicates with sign language, and that's where they see their child fitting into that community, and so they may not opt for a cochlear implant. So I think that for families that are functioning in the hearing world communicating with spoken language, the cochlear implant can be a way that that child can participate in that environment to their fullest extent, and that offers them benefits in shaping their careers, forming friendships, all of those things.
00:54:09
Host: So as these patients grow into from childhood into the adult world and adult medicine, how do they transition from that pediatric care into that adult care?
00:54:20
Mandy Pendelton: From the audiologic perspective, I start that conversation early. I start that conversation right around 14, because here in our system, we're able to continue seeing these kids into their early 20s, oftentimes, but we need for the child and the team to be able to advocate for themselves. We have a lot of discussions as we transition from high school to the college setting, because a lot of that support changes and that advocacy goes from the parent to the actual child or teen. So we're working through those preteen and early teen years to move that on, and then usually we're working throughout those years to put a plan in place. I encourage them to reach out and start to form relationships with other providers as I'm still on their team, so I can provide that support, or if they go and have an experience that they're uncomfortable with, we can take that back and see where we need to fill in the gaps and what direction we might need to go from there. But it's also looking at, as they age out, I like to see them three months beforehand and say, Okay, this is it. This is our last visit. What do you need from me? How can I launch you into your adult world? And you have everything you need. Is that getting an upgraded processor with the newest technology? Is that sitting there and you're explaining to me your hearing loss as if I had no idea what hearing loss was, so that you can walk into a job setting and advocate for yourself. It's about preparing them to get what they want out of life and give them the tools to do so.
00:55:57
Patricia Purcell MD: And I think you have a different perspective in some ways, from the ENT side of things, because as an ENT, I really hope that I'm not having to see my patients too frequently when they get to this age, because I want that implant to be functioning perfectly without any medical complications. If I'm seeing a patient with a cochlear implant frequently in clinic, it usually means there's something going on, but we definitely want to be available to support their transition into the adult world. They definitely need to establish with an ENT, because they'll need to be able to have medical clearance for technology upgrades and things that come up, but hopefully they're not needing to see an ENT more frequently than every few years.
00:56:40
Host: As we wrap up, there are so many steps along the way from diagnosis to implant and beyond. If you could give one piece of advice that a referring physician could provide to a family that just found out their child is a candidate for a cochlear implant, what would it be?
00:56:56
Mandy Pendelton: My recommendation is have a conversation. A conversation is not a commitment. Learn as much as we can and make the right choice for your child and your family.
00:57:10
Patricia Purcell MD: I agree. I think that, again, trying to focus on that family's expectations and figure out the timing of any intervention within that structure, that framework, with the understanding that, due to neuroplasticity, we know that earlier intervention is better, and if the goal is for fluent spoken language, that the sooner the child is implanted, the more likely it is that they will be able to achieve normal language.
00:57:37
Host: And finally, with all the amazing innovations now available for pediatric patients with even profound hearing loss. Where do you see the field of pediatric cochlear implantation heading in the next five to 10 years and beyond?
00:57:50
Patricia Purcell MD: From the surgical side of things, we're always trying to make it less invasive. We've come a long way in terms of how much operating needs to be done. We're drilling less we're using smaller incisions, and I know that that process will continue to play out over time as the technology gets even finer. So I think that there'll be that there'll also be more ongoing work to tailor the intervention to the type of hearing loss. So there's been research that's showing that there may be other treatments, even genetic treatments, for hearing loss, and that may mean that cochlear implantation is not always the recommendation for children who are profoundly deaf. So there's a lot of exciting research in that area as well. There's so much to still learn.
00:58:35
Mandy Pendelton: Yeah, looking back, I don't know that I would have said 10 years ago that we would be where we are with implantation. So I think we're gonna see more personalization of implantation and tailoring that to each child in each scenario. I think we're gonna see a continued growth of technology and how the implants interact with the world around them. But I think there's the potential that I don't even know what's coming.
00:59:02
Host: Things get smaller and smaller and smaller. And I think the research that happens is just incredible. So, yeah, I think it's an exciting time to see where things go. So is there anything else you want to add before we close?
00:59:17
Patricia Purcell MD: I just want to emphasize that a hearing test is so minimally invasive, if there's ever doubt, just just do it to confirm how well a child is hearing. And again, this podcast has been really focused on cochlear implantation, but most children with hearing loss are not needing cochlear implants. They're not profoundly deaf. They could just have a mild or moderate hearing loss and still have major speech and language implications because
00:59:40
Mandy Pendelton: of that, I would agree we're here to help. And if there are concerns on the part by the parent, concerns on the part of the teacher or you as the physician, we just love to take that concern and either move forward as needed, or just be able to kind of wipe that away and take one thing off of that parent's to do list.
00:59:59
Host: Fantastic. Well, thank you both so much for being here and for doing the work that you do for all the kiddos that you do it for.
01:00:06
Mandy Pendelton: Thank you.
01:00:07
Patricia Purcell MD 1:00:07
Thank you. It's been a pleasure.
01:00:09
Host: For our listeners, you'll find more information about our cochlear implant program on our website at CookChildrens.org. You can also access clinical pathways on the health professionals section of the website. And while you're there, sign up for our Doc Talk newsletter. Want more Doc Talk? Get our latest episodes delivered directly to your inbox when you subscribe to our Cook Children's Doc Talk podcast from your favorite podcast provider. And thank you for listening.
01:00:07
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