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BackTable / ENT / Podcast / Transcript #10
Podcast Transcript: Comprehensive Care For Deaf and Hard of Hearing Children
with Dr. Rachel St. John
We talk with Dr. Rachel St. John from Children's Health about the importance of early language access and collaborative care for children who are deaf or hard of hearing. You can read the full transcript below and listen to this episode here on BackTable.com.
Table of Contents
(1) Dr. Rachel Saint John’s Journey to Pediatrics and Patients with Hearing Loss and Deafness
(2) A Comprehensive Initial Hearing Loss Evaluation: Counseling, Education, and Collaboration
(3) Extensive Evaluation of Deaf or Hard of Hearing Patients: Labs, Imaging, and Genetics
(4) Counseling for Families of Deaf or Hard of Hearing Pediatric Patients; To test or not to test
(5) Hearing Loss with Enlarged Vestibular Aqueduct: Evaluation, Imaging, and Patient Education
(6) Age-Specific Considerations and Needs in Counseling Pediatric Patients with Hearing Loss or Deafness
(7) Sign Language, Language Development, and Language Access Considerations in Pediatric Hearing Loss Patients
(8) Aggressive Intervention in Early Hearing Loss: Coming to Terms with Deafness and Reality of Treatment Options
(9) Single-sided Deafness or Mild Hearing Loss in Pediatric Populations and Patient-Centered Management
(10) Pearls from Dr. Saint John and Additional Resources about Deafness and Hearing Loss
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[Gopi Shah MD]
Hello, everyone. And welcome to the BackTable ENT Podcast. I'm your host, Gopi Shah, here with my lovely co-host Ashley Agan.
[Ashley Agan MD]
Hey, how you doing, Gopi?
[Gopi Shah MD]
I'm doing good. Did you light a candle last night for Diwali? Your text was the first text I got for Diwali, by the way. I appreciated your cultural sensitivity.
[Ashley Agan MD]
I felt like I was going to get some points for being up on that. I was thinking about you.
[Gopi Shah MD]
For sure, for sure. So, happy Indian New Year to everyone. We're here with a very special guest today, Dr. Rachel Saint John. She's here to talk to us about helping children and their families with hearing loss. Rachel is a pediatrician, who joined our department of otolaryngology at UT Southwestern in 2012 to provide a collaborative and comprehensive care service for children who are deaf or hard of hearing. She's the director of the Family-Focused Center for Deaf and Hard of Hearing Children at Children's Health in Dallas-UT Southwestern. And she's also a health education consultant at the state and national level. She was a member of the AAP Early Hearing Detection and Leadership Team and served as an AAP, so the American Academy of Pediatrics, delegate to the joint committee on infant hearing. So welcome, Rachel.
[Rachel Saint John MD]
Hey, guys. Thank you so much for having me.
[Gopi Shah MD]
Thank you for coming onto the show.
(1) Dr. Rachel Saint John’s Journey to Pediatrics and Patients with Hearing Loss and Deafness
[Ashley Agan MD]
So Rachel, you were one of the attendings when I was in residency here. And I remember meeting you and hearing your lectures and talking, and then being like, "How did this amazing, intelligent pediatrician find her way into an otolaryngology department specializing in pediatric hearing loss?" Can you tell us about your background and how you found your way to us?
[Rachel Saint John MD]
It is the funkiest of stories though. Thank you for that incredibly warm welcome. And also, I'm not going to lie. I feel a little old at the moment, because now you are a big full-fledged attending, so, yeah, we're going back in the vault a little bit. Yeah, it's a little bit of a winding road in terms of how, as a pediatrician, I ended up in otolaryngology. And it makes our credentialing people a little challenged every couple of years. Basically, I grew up in northern Virginia, so I was right outside Washington, D.C., which is one of the largest culturally Deaf communities in the US. The population of people who are deaf and use sign language there is quite high. And I happened to go to an elementary school that was the magnet school for my county for the deaf education program. So I had students all around me, when I was very young, who were using sign language. And I became friends with them and learned sign language pretty organically at a very early age. And so, I grew up signing. I grew up with both hearing and deaf friends, and it's kind of always been part of my background.
And then, as I got older and I was going through college and thinking about grad school, I was hearing lots of stories from my deaf friends. Unfortunately, they were not positive in terms of accessing health care, what it was like to go, say, to your GYN appointment as a deaf person, and either not know what was going on or having an interpreter in the room, and have great access but then also that's a fairly vulnerable moment for some people. So kind of some of those stories of how people navigated accessing healthcare as a deaf person, those were very interesting. And it was something that really resonated with me, so when I ultimately made the decision to go to med school, it's something I wanted to do. And I was really fortunate. I went to medical school at University of Virginia and was fortunate enough to get a Robert Wood Johnson General Scholar Program grant to do my medical studies there. And that was specifically with the intention of making healthcare more accessible to deaf and hard of hearing people and families.
So I went to Georgetown for my pediatric residency in Washington, D.C. And I had a deaf-accessible primary care peds clinic, while I was there. Started it as a resident and I stayed there for several years as an attending. And it was a wonderful experience. I had good support. The problem was that it was really hard to do in a primary care environment. Primary care is primarily set up for really high-volume. That had some challenges with it. So while I was in my residency, I did a combo program, so I was part-time resident and part-time getting my masters in counseling at Gallaudet University, which is the deaf university in Washington, D.C. So I was really keeping connected to this the entire time.
In 2010, my husband and I moved to Texas. And I actually took that opportunity... I was a little burned-out. I listened to your guys' first podcast on wellness and burnout with significant interest, because I definitely have had a little bit of experience with that. So I stepped away from medicine for several years. And when we moved to Texas, I worked for the state. They had an early hearing detection and intervention education program, and so I traveled all over the state working with different positions in different places. And I got to know a lot of people in the early-hearing-detection-intervention world in Texas. And one of those people was Dr. Angela Shoup, who was the director of audiology at Children's. And she's the one, who I thought randomly, but I guess it wasn't. In 2012, she called me and said, "We've got this really great new division chief for our Pedi ENT department at Children's. And I just think it would be great if the two of you chatted." And I thought, "Sure, I like to talk to people." I did not realize this was a job interview. I sort of found that out when I got the email with the itinerary of all the people I was going to talk to. And I went, "Oh, I guess I better wear something nicer."
Well, it's funny. People still talk about it. I came with the East Coast power suit and the heels. And I think it looked pretty professional. It was not comfortable. It was a long day with a lot of walking. But that's where I met Ron Mitchell, our division chief. He's all of our bosses on this call, which is kind of fun. And his goal, as he said, he was really interested in doing something more comprehensive for the deaf and hard of hearing kids we see in clinic. He said, "We've got a multi-disciplinary program for our kids who get a cochlear implant. But for all the other kids who aren't cochlear implant candidates, and that's a much larger number.
[Gopi Shah MD]
Majority, right?
[Rachel Saint John MD]
Yeah, very much so. He's like, "We put tubes in, and we send them home." And he said, "I think we can be doing a lot more." So we all talked about this. And this was my secret pipe dream. It had been on my laptop for years and years and years. And so, they offered me the opportunity to start working sort of as the... I view it as sort of complex care coordination around the hearing status. So for all our deaf and hard of hearing kids, making sure that our parents and families understand what that means, understand that early language access is incredibly important, understand what educational and other resources are available to them. So, that was eight years ago that I started, and I'm still here. And it's grown. I have two nurse practitioners that work with me now. They are fabulous. Huge shout to Ella and Caroline.
And the reality is this works much better in an ENT subspecialty department, where we have access to audiology right down the hall. We have access to each other. It's often the first point of contact for these families. It really works great. So I am your non-surgical party crasher in ENT.
[Ashley Agan MD]
It's probably the best party crasher to have.
[Gopi Shah MD]
Yeah.
(2) A Comprehensive Initial Hearing Loss Evaluation: Counseling, Education, and Collaboration
[Ashley Agan MD]
When I think about visits, our visits with new identification of hearing loss or follow-up audiogram for sensorineural hearing loss, hearing aids. They need to get another audiogram just for hearing aid adjustment. Our visits are probably 15, 20 minutes. Rachel, how long are your visits? How long are they?
[Rachel Saint John MD]
God, I almost don't want to say it, because I'm afraid somebody's going to show up and say, "Ah, there's been a terrible mistake. We need to take this away." One of the things that doctor... Oh, go ahead.
[Ashley Agan MD]
I ask because I feel like there's so much more involved at ENTs, where there's ear doctors, and yet there's so much more involved than our quick 15, 20 minutes in terms of patient counseling and education and resources for these patients.
[Rachel Saint John MD]
It's a great point. I think the ask for anybody who's seeing a patient who's really complex or who has lots of different medical conditions, it's a big ask to take care of everything in 15 to 20 minutes. And I think what patients are expecting is that we're going to view that child as a whole person. And I think that the time table that we work on, and sort of the constraints of managed care, don't always fit with that. So I think you're absolutely right. And I think that there are other models that have taken this approach. You hear about cystic fibrosis centers and complex cardiac centers that have patient navigators or people that sort of work with families more holistically. You also see that in complex care in the primary care realm. And those visits are necessarily longer, because you're not just going over the hearing status, but you're going over what it means for the child. What are you going to do about it? What kinds of things should you be looking for? Are the parents okay? A lot of times, they're not. A lot of times, they're overwhelmed. The information is new, and they're really looking for somebody to help with that.
So yeah, the acknowledgement that that takes more time, I've never taken that for granted. That's something that Dr. Mitchell and I talked about way, way, way at the inception of this. And I said, "If I'm going to do this right, I need time." And so for a new patient consult, I actually have 90 minutes. And for follow-ups, I have 30 to 60, depending on the complexity of the child.
[Gopi Shah MD]
Wow.
[Ashley Agan MD]
That's amazing.
[Gopi Shah MD]
Yeah, I mean, the resource that you provide our patients is amazing. So they might see me, right? Let's say it's a new identified five-year-old with hearing loss. What are the most common things that we, as ENTs, might miss or forget in our workup, in our counseling? What do you get into with the patient? So you see that kid, your first visit, what's that visit like?
[Rachel Saint John MD]
That's a great question. And I'm certainly not going to throw any of you guys under the bus for quote missing anything, because, again, I think what we're being asked to do and what we get time to do often are two different things, when you're talking about a new diagnosis that impacts development so significantly, because that's what we're talking about. When you're talking about kids who are deaf and hard of hearing, it's not just the lack of sound input. It's lack of access to language development. If you ask me, that's the whole reason for me being there. So when I get a child who has a new identification of hearing status, the kind of walk-through we do with parents, obviously, I'm going to read the room, because sometimes I'll walk in, and parents are really collaborative and seeking information. Sometimes I walk in, and they're very emotionally distraught, because they didn't expect this or they don't know what's happening. So a lot of it is sort of supporting families. I feel like I pull on my counseling degree all the time, every day, when I do work, even though I'm not doing any sort of classic therapy. It's a lot of counseling support with families.
But the first thing I do with families is I take their audiogram of their child, and I superimpose it on what we call the visual audiogram. I'm sure you've seen it in clinic, where we refer to it sometimes as the speech banana. So it's an audiogram that is the same graph, but it has all of the information visible. What sounds are where? Where does speech live? Where is a barking dog? Where is a vacuum cleaner? Where is a bird chirping? That kind of thing. And I put those results on there. And then, I go over with the family what the child can hear and what they can't and then what might benefit from things like amplification. And so that's always for the families to keep and hang onto.
And then, we kind of go through a language assessment and see what's been happening. Obviously, that's going to look really different, if you're born deaf, and you have bilateral profound sensorineural versus, "I am older and I have late-onset changes," or it's one ear versus two ears. Those things all play differently into how children acquire language. That's kind of the crux of the visit is what are things you need to make sure that your child can access language and access education and access connection with other human beings. And so everything that we talk about, community resources, education, amplification, whether it's hearing aids, cochlear implants, bone-anchored hearing aids, all of those things go into kind of the psycho-social success of children.
(3) Extensive Evaluation of Deaf or Hard of Hearing Patients: Labs, Imaging, and Genetics
[Ashley Agan MD]
I think that's so amazing to think about you being able to really break it down like that, because I know, in my clinic, I just feel very rushed and hurried sometimes. And frequently, most kids are coming for something straight-forward, like a tube check or something like that. And then all of a sudden, you run into a patient that does have a new diagnosis of sensorineural hearing loss, where there needs to be time to dive into the implications of that. And then all of a sudden, you're like, "I don't have the time. And I don't maybe even have all of the answers." I appreciate being able to refer them to you, Rachel, because that's what I envision, is you actually being able to give them everything that I know that they need in these situations. When I have these kids, one thing that I'm worried about is what are all the studies that I need to be ordering? Does every kid need to be sent for labs and genetic testing? Does every kid need an MRI? What are the nuances of that part of the workup? And how much of that are you involved in?
[Rachel Saint John MD]
That's a big part of what we do, because sometimes we see kids later on down the road, and they've had some of the workups. Sometimes we see kids, and nothing's happened yet. We're getting them sort of fresh. I mean, again, I'm a big fan of looking at the child, but I tend to break this up. I really like sort of thinking about this in the way that was categorized by the 2016 International Pediatric Otolaryngology Group. So the IPOG consensus that was published in 2016, looked at a bunch of pediatric data, about what kinds of things are more common in children versus adults. There's this whole algorithm for adults. When you have sudden hearing changes in adults, one of the first things you think about is a hemorrhagic event. And in kids, that's just incredibly rare. It's not impossible, but it's certainly not at the top of our list. So they've broken it down, and I tend to think about it this way too, whether it's sensorineural or conductive. So if we're talking about sensorineural hearing changes, I really think about it in terms of unilateral or bilateral, and that really drives my workup.
So I think it's good to keep in mind that about a third of the time when you have children who have bilateral sensorineural hearing changes, about a third of the time it's idiopathic, or at least we don't know how to test for everything. And so we can do all the testing we do, and we don't get a diagnosis. But roughly a third of the time, it's genetic. And so the first thing that I go for, and this is supported by the consensus statement, is comprehensive genetic testing. So this is not target testing. This is sending a child to genetics for a full evaluation. And I think that in the past, that didn't happen a whole lot. People would say, "Well, it's not really going to change what we're doing. You might get the name of it, but that's not really going to change our medical management." And I would strongly beg to differ, because if you get a good genetic evaluation, and with the access to testing we have, our multi-gene hearing panels now are upward of 200-plus mutations that are known to involve hearing changes.
And if you can get an identification on a child that's genetic, there's a couple things that happen. One is you either have other things that are medically associated that you need to be on the lookout for that you would not have known otherwise. So we've picked up a couple kids, for example, who have Usher syndrome, and that's inherited sensorineural hearing changes with progressive retinitis pigmentosa. So these people lose vision over time, usually starting in their late teens, early adulthood. Well, if I know that when that child's a toddler, and I can get them into ophthalmology early, and even maybe they're amenable to vitamin A supplementation, that's going to change the course of what we're doing. Conversely, if I get a genetic identification for connexin 26, which is the most common non-syndromic reason for hearing changes that we see, I'm not going to order any other stuff. I'm not going to get a sedated MRI, because that happens at the potassium exchange level. That happens in the channels. That's not structural. So it is going to absolutely change my management. So that's very insurance based, but I always try to get comprehensive genetic testing first.
Certainly, if that doesn't show anything, an MRI of internal auditory canals can be really helpful. I'm a real stickler. I always get an EKG on a patient who has bilateral sensorineural hearing loss, and I don't have a reason, just to rule out inherited prolonged QT. It's really rare, but, man, if you have it, that's a hard way to find that out on the football field or wherever you are.
[Ashley Agan MD]
Yeah, an EKG is easy enough to get. Getting an EKG is much easier than getting a sedated MRI, so why not go ahead and get that.
[Rachel Saint John MD]
You are not kidding. And in 20-plus years of practice, I've never had an EKG denied by insurance. And then for unilateral, imaging is usually first. It's pretty rare. It can happen, but usually if it's genetic, it's going to be bilateral. So for unilateral, I usually go for imaging first.
(4) Counseling for Families of Deaf or Hard of Hearing Pediatric Patients; To test or not to test
[Gopi Shah MD]
So Rachel, I'm glad you brought up the genetics, and your example I think is great. I've heard lots of different arguments in terms of genetic workup. Let's say it's an otherwise healthy kid that has no diagnosed other medical problems, heart, lung, vision. I have heard people say, "Wait till they get older. The genetic testing panels, all that, is going to be more informative, advanced. Wait till they want to start family planning." So when you give us the Usher's example, it makes more sense. Do you have families or patients that don't want to do it necessarily? And how do I counsel them?
[Rachel Saint John MD]
That's an outstanding question. And it does happen. I think if it's the question of a, "Wait till testing gets better," well, you can certainly do it now and then, in 10 years, do it again when we have more information. But yeah, we have had a couple of families. It's not the majority of them, but we do have a few who are leery of genetic testing. And I would say that the majority of the time, that's because they don't necessarily know what it involves. So our hearing panels only test for those specific 200 mutations. They're not paternity tests. Certainly, if they need to go to larger testing, that's something that the genetic specialist will talk to the families about. But usually, this comprehensive targeted testing, it's really specific. Occasionally, and this just happened to me the other week, I did have a parent. She said, "I know you recommended that I go," but she said, "I actually just couldn't handle it if I found out that it was something from my side of the family." She said, "I know that sounds irrational." And she said, "It's not a bad thing." She says, " I was just so uncomfortable with the fact that I might know where it came from."
She was actually much more comfortable just saying, "This is my child. And I accept who she is, and I love her. And actually, that information makes me more anxious." And I really respect that. I do think it's important to go through that with families and help them understand why or why not we wouldn't pursue testing. But I've definitely had families where we've opted to just wait until that child starts expressing interest.
[Ashley Agan MD]
Yeah, because the question is always how does it change what we're going to do? And if you can safely proceed without that information, then you can talk about holding off or waiting, for sure. What about kids who have a mixed hearing loss?
[Rachel Saint John MD]
Yeah, that's always fun. Again, the POG consensus also has support and an algorithm for conductive changes, which I think is really helpful. And I think, obviously, certainly in the ENT world, we always determine if this is potentially reversible. If you stuck an old jelly bean in your ear, then, yeah, we can probably fix your conductive hearing loss rather quickly. Or if you have fluid, we can put tubes in. But if we're talking about something permanent, that either conductive or mixed, if this is not something middle ear dependent, obviously, looking at that whole kid and seeing if there's any syndromic presentation, because mixed and conductive hearing changes definitely are part of syndromes that involve midface, things like Treacher Collins, hemifacial microsomia, those kinds of things. Branchiootorenal, those may have sort of ear malformation involvement. Imaging, we often do CT for that, but imaging certainly is super helpful.
The mixed presentation is interesting. In my clinic, when kids have a true mixed situation, it's often one of two scenarios. Either they have baseline sensorineural, and then they have middle ear disorder on top that's giving them an additional conductive component. Or they have the famous, and I know, Gopi's going to really want to dive into this at some point, they have the famous enlarged vestibular aqueduct.
[Gopi Shah MD]
Pandora's box.
(5) Hearing Loss with Enlarged Vestibular Aqueduct: Evaluation, Imaging, and Patient Education
[Rachel Saint John MD]
Pandora's box, which I think in the general population is not huge. But certainly in our clinic, there are a large number of kids with EVA. And it's interesting because, again, we can get into that as much or as little as you want to, but that mixed presentation, I've even seen it referred to as a pseudo-conductive component. It's most characterized by a third window situation with the bony involvement with EVA. When I see mixed, particularly late-onset, one of the first things I think about is EVA. And I usually move to imaging.
[Gopi Shah MD]
And do you usually CT? Give the family the option? Because with CT, we think radiation. With MRI, we think sedation, depending on the age and the child's ability.
[Rachel Saint John MD]
I think it's exactly that. I think part of it is... And I've talked to different providers about what they prefer for seeing different things. And I've heard all of it. I've heard people say, "MRI is better for EVA." I've heard people say, "No, a CT is better for EVA." I think when kids have almost primarily or exclusively a conductive component, CT is more helpful, because you are thinking about things like bony involvement or ossicular chain. But I think a lot of it depends on the age of the child and what you're thinking about in terms of sedation versus radiation, length of procedure. Is there something else that needs to be combined with those things? We often will combine hearing tests with MRIs, sedated ABRs, if you need an MRI. So yeah, a lot of it is just based on how old that kid is and what we're looking for.
[Gopi Shah MD]
In terms of EVA, and again, I don't know how much we want to go into this now, but how do you counsel families in terms of activities? We're always taught that with EVA you have to avoid head trauma, significant barotrauma, because that can make the hearing worse. What do you tell in terms of... Do you tell them to have activity limitations, restrictions, head protections? What's that conversation?
[Rachel Saint John MD]
Yeah, and you know it's kids, so that's always a little more challenging depending on what age they are. I think we do all this counseling because it makes us look smart, in the sense that if you look at the literature, the exact underlying mechanism for the hearing change associated with EVA is unknown. So it's not that you have this enlargement of the vestibular aqueduct. That isn't causative. That's not what's making the hearing drop. It's a radiologic marker, at least it's postulated in the data. It's a radiologic marker for an underlying process, potentially at the cellular level. And so, yeah, we're basically like, "Yeah, we usually see kids lose hearing with this, and we're not exactly sure why." Well, none of us want to say that, because that doesn't make anybody feel good, including us. But in terms of counseling, we do know that there are some situations that tend to predispose children to faster or more abrupt drops in hearing when they have EVA. And those tend to be significant trauma to the head or significant changes in barometric pressure.
So in terms of sports, obviously, I always tell families, "Your kid's not in a bubble. You do not need to wrap them in plastic and put them in the attic. That is not the whole point of being a person. They can go do sports." I think the sports that have the highest risk, because you're really thinking about head contact, particularly head to head contact. So the really big ones are football, rugby, and soccer. And then the other stuff tends to be more incidental. "Yeah, you could conk head with somebody playing basketball. It's not supposed to happen, and if it happens, it's an accident." I really try not to get parents super anxious about this. And I really, really firmly believe these kids need to be active, because certainly being sedentary and overweight and all of the things that accompany that are much a bigger issue.
And then in terms of barometric changes, people ask me about planes. Usually, I encourage people to, "If you're flying somewhere, go ahead. The cabin is pressurized. And if you have a sudden depressurization in the cabin that's going to predispose your kid to a sudden drop in hearing, you have way bigger problems at that moment because your cabin has depressurized. So, that really doesn't happen very often." The other thing I really will counsel kids, and it's mostly high school and college-entry kids and young adults is scuba diving, because sometimes schools will offer it for credit. And I actually did have a patient who did not know they had EVA. This is a very, very long time ago when I was in D.C. It was a student who had gone onto college, and they took scuba diving as part of their credit course and went into the pool with a moderate level and went and spent some time and then came up and had a big drop. So, that's always something I encourage families to think about too.
[Ashley Agan MD]
What do the next steps look like for a patient like that?
[Rachel Saint John MD]
Yeah, the next steps really involve, at least from my perspective, making sure that that patient is involved with their own decision making. I mean, it's a lifetime. For example, I had a patient who was a catcher for his baseball team. And he was actually being recruited for college. He was really, really, really good. We had talked about the fact that, again, you're not supposed to get a fastball to the head, but if you're a catcher, the likelihood of that is a little higher than if you're in the outfield. And so, we talked about the risks that would be potentially associated if he ever took a ball to the head at fast velocity. And he was college age, so he was really able to articulate with me and with his parents. He said, "I hear you. I hear all of this. And I am comfortable with the fact that if I have a drop in hearing because of some freak incident, I'm okay with that. It's more important for me to keep this part of my life and pursue this." And I supported him a hundred percent, because he made that choice as a young adult. And he had the support of his family.
So I think a lot of it is making sure that when those decisions are made, that they involve the child who's becoming an emerging adult, and that they end up taking some of the responsibility for that decision-making. Because it's not about avoiding everything that could possibly drop your hearing, because you would never come out of your house.
[Ashley Agan MD]
So it's all about risk benefits. And certainly if the joy of your life is playing baseball, then sometimes it's worth those risks to be able to live a full life.
[Rachel Saint John MD]
Absolutely. I think a lot of it is just informed decision-making.
(6) Age-Specific Considerations and Needs in Counseling Pediatric Patients with Hearing Loss or Deafness
[Ashley Agan MD]
Well, I love the fact that it's important... I love that you see older patients, because you're right. The needs are different, and the decision-making's different once the child is in high school, college. And it all starts even earlier than that, late elementary and middle school, in terms of the child wanting to have an FM system, the child being willing to wear a hearing aid. How does age come into play with your counseling? And what are the different needs for the different age groups to help these kids to understand their hearing loss?
[Rachel Saint John MD]
I'm so glad you brought that up. I actually think it's incredibly important, because it's not just limited to these children and young adults understanding. It's their family understanding what that means for them. I think you had mentioned earlier, what's something that we tend to forget in ENT. But I think it's not just ENT, it's all of medicine. I cannot tell you how many times families and children as they get older have told me, "Wow, you're the first doctor that's come in and talked to my kid directly. You're the first person who asked them what they wanted." And I do it starting at a very early age. I just gage it developmentally. If I've got a kid who's really developmentally precocious at age five, I'm going to involve them to whatever extent I can in the visit. As soon as kids are showing more independence, I'm going to try to have as much of that visit independently with them as I can with support from the family.
Because what unfortunately tends to happen, and it's not just with deaf and hard of hearing kids. It's with lots of different kids who might have multiple medical conditions. Some families are a little vulnerable to the fragile child script. And I totally understand how it happens. You have parents who have to make tons of decisions for their children when they're very, very young about their medical care. And so they get very used to that. And as their child is getting older, sometimes parents are more comfortable speaking for their children. And I think as physicians, sometimes we're pretty relieved if we walked into a scenario and we're not totally comfortable with it, and then the parents start interacting with us. Well, we can just go ahead and interact with the parents. I think it happens a lot with kids who are deaf and hard of hearing, because sometimes they're missing part of that conversation. Sometimes they engage in communication that a physician isn't familiar with. So I happen to be fluent in American Sign Language, and that has helped me tremendously, because a lot of the kids that I see use both spoken and sign language.
And so, sometimes kids, and young adults in particular, will tell me, "Yeah, nobody talks to me. They just immediately start talking to my mom because it's easier." And it is easier. It's faster, and sometimes you get the information you need quickly. All that's good, but it really takes that patient out of the equation. So I involve kids and older kids and young adults to whatever extent that I can in the visit that's appropriate to their development level.
(7) Sign Language, Language Development, and Language Access Considerations in Pediatric Hearing Loss Patients
[Ashley Agan MD]
Mm-hmm, yeah. I think that makes a big difference. How does sign language come into your treatment plan? Do you help families connect with resources for using sign language?
[Rachel Saint John MD]
So, yeah, this is the best thing you could've ever asked me, because I think Gopi knows this is a little bit of a soapbox for me. The answer is a resounding yes. I think this is where our field, and a lot of other fields that are involved in working with deaf and hard of hearing kids, tons of fields, audiology, speech pathology, early intervention, I mean there's so many players in this... But I think this is where things get a little fractured in terms of how we approach working with these kids and giving them access to language. My biggest thing over the last 20 years, or whatever it's been, that I've been working with deaf and hard of hearing children and their families, the most important thing, as a pediatrician and as a developmentalist, is early language access. There's a small but growing body of data that's looking at deaf kids and language emergence in both spoken and sign language. And all of the recent data, the peer-reviewed data with sound methodology, that I've seen going back to probably the early '90s all the way up to now, it really points to early language acquisition being the cornerstone for all of this development, not mode.
So I think that there's some people who really cling to, "If you want to develop spoken language, you can only do spoken language. And if you do sign language, it's going to somehow take away from that." Well, it's not what the data shows. It's certainly not what my last couple of decades of experience has shown me, in terms of outcomes for kids. The much, much more concerning thing, that unfortunately I see a lot in my clinic, are kids who don't get early language access. And so, they missed a lot of that critical language window. They end up with what's called early language deprivation. And they can't catch up the way that other kids can.
[Gopi Shah MD]
Rachel, let's dig into language access. Let's dig into it more. It makes sense, language versus sign language. Can you just break it down in terms of what exactly language access means? Because we get these questions of, "If my child does sign, he's not going to 'speak'?" or, "I think my child has speech delay, but I think it's because one person speaks English and the other one speaks Spanish, and that's too many languages." Tell us what language access means and how to answer some of those questions.
[Rachel Saint John MD]
Woof! I mean, it's the million dollar question. And if we can answer this in this podcast, then we are amazing.
[Gopi Shah MD]
You are amazing. That's why I'm asking you.
[Rachel Saint John MD]
So I heard something in a talk recently that was being given on VPI. And it was from one of the speech pathologists. And she said, "It's really important to remember that speech articulation is an oral motor, but language originates in the brain." And I think that's where this goes off the rails immediately. Speech and language are not the same thing. Language is the thought construct that we develop as humans to generate a concept. And then the way that we share that concept is through a mode, whether it's spoken language, English, Spanish, French, whether it's visual sign language, whether it's ProTactile, so that would be sign language by touch for people who are deaf-blind. The mode is not the language. The language is the thought construct in the brain.
I'll just give you an example, if you handed me a paragraph of Italian, I could probably read it for you. I don't know how good my accent would be, but I could probably limp my way through and read it for you. I would have no idea what I'm saying, because I have speech and a language. So the language is absolutely the building block that we're looking to build. And again, the data supports building that building block, the thought concept, and mode is irrelevant. And we actually have a lot of good data that shows if you expose kids to sign language, it doesn't impede the development of their spoken language as they move on. In the past 20 years, I've only seen one study come out that overtly says, "Sign language is harmful to the spoken language development of kids," and it's been hotly contested in the community. So it's really kind of in the face of everything that's come along.
So the idea of too many languages, I've only seen that be a problem when we have a kid who's not neurotypical. So if you have a child who's dealing with some other conditions, maybe autism is part of the plan or some other developmental conditions, sometimes I've seen that be more of an issue for being hit with the cognitive stress of several different languages in early childhood. But most of the time, if you have a child, and the parents speak English and the nanny speaks French, and they go to Spanish schoolhouse, and they do Spanish at school, and then they're doing a little bit of sign language with their friends in the playgroup, they're going to develop all of them. They get a little mushy in the first couple years. There's a lot of code switching between them. And you see some of one overlapping with the other. But if you measure it all as a whole, these kids are not delayed. And usually by about age four or five, they've ironed out who they need to put in what box and use what language with.
[Ashley Agan MD]
Mm-hmm.
[Gopi Shah MD]
I love that. It's all at the level of the brain, and it's just different modes of self-expression. I absolutely love that. To make connections, it's beautiful.
[Rachel Saint John MD]
And then if you miss that window, for early language development, it's not the kind of thing where you can go, "Okay, when they turn 10, we're going to hit this thing really hard." You've sort of missed the boat on that one, and that's really sad.
[Ashley Agan MD]
And what is the window?
[Rachel Saint John MD]
It's debatable. What I have seen in terms of the physiologic stuff, so brain myelination, and sort of neuroplasticity and the end of temporal pruning, the human brain is fully myelinated around age four. And so that's when a lot of that flexibility, that, "If I have a pathway and I use it, I'm going to cement it." That's where that starts to really decline. And so, I would say four is kind of one of those periods, physiologically, where if you don't have a first language in place by four, if you're not getting good language access before age four, you're going to have some real sort of neurological stiffness in terms of acquiring a first language beyond that. But having said that, year zero to one looks real different from year three to four. That first year, where you've got billions of excess brain cells, that's a critical moment. It's a big use-it-or-lose-it moment.
And so, for example, if you're born completely deaf, and you're waiting for a cochlear implant, and the earliest that that's going to be covered by third-party insurance is six months. And the earliest that that's going to be covered by Medicaid is 12-months. So if you're going to sit around for those first 6 to 12 months and do nothing linguistically, that child is behind the eight-ball. Whereas if you start with sign language, which is usually available, you're starting to build that brain thought concept that, when that sound input eventually starts coming, you have something to link it with. Yeah, I'm not a fan of letting children languish without language for the first 6 to 12 months of their life. Some parents will get that very advice, not from our department, thank goodness. But I've worked with lots of families who have heard that kind of thing, "Oh, just wait till we do the cochlear implant, and then we'll work on spoken language." That, unfortunately, sometimes sets families up. Some kids do okay, and some do not.
[Ashley Agan MD]
So it's never too early.
[Gopi Shah MD]
Yeah.
[Rachel Saint John MD]
No, it's never too early, ever.
[Gopi Shah MD]
What are some other considerations that you have for your patients with hearing loss, Rachel?
(8) Aggressive Intervention in Early Hearing Loss: Coming to Terms with Deafness and Reality of Treatment Options
[Rachel Saint John MD]
That's a great question. A couple of things I always think about, one is aggressively referring to early childhood intervention for our kids who are birth-to-three, because those are the people who are going to work with families for acoustic positioning, helping them wear their amplification, teaching them sign language, getting them ready for school, supporting parents. And unfortunately, that referral doesn't really live with anybody specifically. It's like if you're going to get your preventative shots, you do that with your pediatrician. I don't think ENT is clamoring to take over the world of preventative vaccination. Everybody knows, you go to your pediatrician to get your shots. Anyone can make an ECI (Early Childhood Intervention) referral, which is great and also what makes it a little problematic, because that doesn't always live with any one provider. And so I always encourage the residents in peds and ENT, our colleagues, "If you have a kid, birth-to-three, who's not been referred to ECI, that's a wonderful opportunity."
And the other consideration I think is super important... And you can yell at me if I sound preachy about this. I'm not trying to. But I think it's very easy to forget that whatever we do for and with these kids, so no matter what kind of technology we put behind their ears or in their ears or even inside them surgically, it actually doesn't fundamentally change who they are at all. At the end of the day, they're still deaf. So if you get a cochlear implant, and you take it off to swim, to take a bath, you're going to bed, you're waking up, it's broken and it's being repaired and you're waiting for it to come back, you're a deaf person. And I think that sometimes the messaging we give families is contrary to that, "Oh, we'll do this, and it'll just make everything fine." That's understandable that that message comes, because we're hearing-centric people. If you're hearing, you function from a hearing perspective. I get it.
But if we don't acknowledge that these kids are deaf, and we don't help families acknowledge that these kids are deaf, and we spend a lot of time trying to make them hear, we set these kids up for some really significant identity crises when they go through adolescence. I've seen it happen. I've seen kids get super angry with parents for not giving them certain opportunities when they were younger, when parents, again, were just making the best decisions that they knew how based on the information that they had. I think it's really important to acknowledge that kids, who are deaf, are deaf. And we give them access, and we give them technology that helps them access the hearing world better, but it does not fundamentally change who they are at baseline. And if we don't support that messaging with families, we can set families up for a bit of tough time down the road.
[Ashley Agan MD]
Yeah, I think that's a really important thing that you lay out so well for us. I think the big thing is that we're not curing them of being deaf. A cochlear implant is a tool. So we're giving them a tool to access spoken language. But kind of helping them with their identity and what this means for the rest of their life is going to be a lot more helpful than just kind of throwing a hearing aid on them and kind of saying, "See you later." I mean, I think something I admire so much about what you're doing is you're actually helping them kind of step through this diagnosis that is there forever.
[Rachel Saint John MD]
Yeah, thank you for that. And I think part of that comes from the fact that there is, for people who are not familiar with it, there's a cultural component to being deaf. There is actually a cultural... It's the capital D, Deaf communities, so Deaf spelled with a capital D. And I don't represent the Deaf community. And I don't speak for them, because I'm a hearing person. But in working with folks, I think it's very helpful for providers to know that there are people who are deaf, who are out there who actually being deaf is not only part of who they are, it's a very positive part of who they are. They actually wouldn't become hearing if they had the opportunity. And they do all the same things that hearing people do that make us successful adults. They go to school. They have relationships. They have families. They get terminal degrees. They have careers. They have community. They get involved in hobbies and spiritual communities, whatever, all the things that make us people. They do the same things. They just do them through a visual medium and they're their own culture. And that's a group of people...
You'll hear me. I mean, I'm not the PC police. I am not a terminology policier. But I personally don't use the term hearing loss or impairment when I talk about this. And it's not because it's a dirty word. I mean, it is offensive to a group of people, but there's a reason we use it medically. It means something. But I think when we use those terms with the families, and families hear terms like loss and impairment very early on, even though we're talking about it related to a very specific aspect of that child, families almost always, and we know this from data from pilot programs, families hear those words and really view their child globally in that manner. And so, we're basically coming from a place of limitation in those early, early conversations.
I use very neutral language when I talk with families for the first time. I talk about the fact that their child has hearing changes and, "Here's how it's different from what we would expect." And I let families learn about these things so that they go on and figure out how that child wants to talk about themselves, how that family wants to talk about that child. So I am very fortunate to work in such an enlightened otolaryngology department as the one that I do with you folks, because it takes people at the top recognizing that this is something that's important for families and making space for it. I don't even begin to think that what I do would work just anywhere. I think the fact that I work where I work, with the people that I work with, and the fact that you guys take advantage of the fact that I'm there in such a positive way, makes this really successful. So this is my opportunity to tell you guys thank you.
[Ashley Agan MD]
Thank you. I think you're such a wonderful addition to our department. I always learn so much talking with you. It's very special to be part of a department that is working so hard to give kids a better quality of life and to be able to kind of live with the cards that they've been dealt and to live their best life. So it's very cool.
[Rachel Saint John MD]
Yeah, I like that you end with that. That's so great.
(9) Single-sided Deafness or Mild Hearing Loss in Pediatric Populations and Patient-Centered Management
[Gopi Shah MD]
This could be a Pandora's box. But last quick question, so do manage or counsel your patients differently? Or how aggressively, in terms of counseling or work up, differently when it's unilateral versus bilateral or mild? Do you do anything different for those kids? Because I always think of school performance, as well as language. Does it change the way you manage your kids?
[Rachel Saint John MD]
Yeah, the answer is yes. And it's a phenomenal question. I think it's kind of telling that we sort of brought it as, "Here's the closer," because this is a population of patients that's very easy not to see.
[Gopi Shah MD]
They're easy to miss.
[Rachel Saint John MD]
Yeah, the kids who are single-sided deafness, or kids who have mild-level hearing changes. So I see the literature often will describe this as MMHL, which is minimal or mild hearing loss. And there's a lot of research going back to the '80s. Bess and Tharpe were sort of the folks who did a ton of research, and then Anne Tharpe did a whole retrospective thing in, I think, 2017 or 2018. And nothing has changed. It's exactly what you say, other than the fact that we're much more aware of it. These kids, who are deaf in one ear or who have sort of mild hearing changes, they all function socially quite well. They all have language. They all develop language. Sometimes there might be some speech articulation stuff. They all develop language, but their access to language varies wildly depending on what environment they're in. So if they're at home, and it's quiet and it's one-on-one, and they're able to see people's faces, often they'll get a much better picture than, as you mentioned, when they're in school, and there's lots of competing background noise and it's open set, so teacher could be talking about anything, and it's new, so they don't even have anything to draw on if they have to fill gaps in cognitively.
So these kids are definitely at higher risk of having to repeat a grade. They have a higher usage of special education plans. And unfortunately for these kids, because socially they look so good, they're doing all the things that people expect, it often presents as behavioral. The teachers all complain, "This kid needs Ritalin, because they don't pay attention. They don't focus. You ask them a question; they give you a totally out-there answer. They never know what's... We're lining up, and they're off doing their own thing." It looks like inability to focus or inability to pay attention a lot of time, or being just defiant. But a lot of times, it's kids trying to compensate. So you'll have kids who are the class clown because they're deflecting, because they don't want to get called on. You'll have a kid who will try to close to the best of their ability with the 70% of information they got, and then put out the wrong answer. You'll have kids who won't volunteer, and they're very quiet. And they're the kids in the parent-teacher conference where the teacher's saying, "Hey, you really got to work on your kid not being so shy in class. They're missing all these learning opportunities." And they're deathly afraid to participate, because they're not working with the full Rolodex of information that everybody else is being given.
So yeah, I think looking at the visual audiogram with those families is incredibly important, because it's really important for those families to acknowledge, "Yes, your kid hears tons. But here's where things are falling out. Here's where things are the most challenging in school." The counseling that we do, even for mild or single-sided, is pretty significant. And a lot of it is around making sure that school is accessible.
[Ashley Agan MD]
And do you find that you have resistance or any kids who just don't want to wear hearing aids? Or they just cringe at the thought of having something that outwardly tags them as being different?
[Rachel Saint John MD]
No, not at all. Every single one of them loves their hearing aids. Yeah, it happens all the time. And it's funny. I'll get parents who say, "All right, Dr. Saint John's going to talk to you about these hearing aids, and you're going to listen to her." And the first thing I say to that teenager who doesn't want to wear their hearing aids because they look different from their friends, the first I say is, "That's normal. You're supposed to feel that way." And these parents look at me like I'm just this charlatan. But my next discussion is, "That's appropriate. That's appropriate for your age. It's okay to feel that way. But here's what's going to happen if you don't wear them. And because you're as old as you are, all this consequence is on you. So when you're not wearing your hearing aids, because you don't want to look different to your friends... " Sometimes I'll ask kids, "Well, what do you want to do after high school?" And they'll say things like, "Go to college." And I'm like, "Okay, so what do you need to get into college?" "Well, you need good grades." "Well, what grade are you getting in your science class right now?" "A D." "That's not going to get you there."
A lot of it is really having that conversation with the child, and the parent is peripheral. But the parent also needs to hear that that's normal. A lot of times these parents are like, "It doesn't matter. You just have to wear them." Sometimes these kids just need to be acknowledged that, "Yeah, it stinks when people make fun of you. But I'll tell you what, if it weren't the hearing aids, it's going to be something else, because that's just how things go when you're in junior high school and high school. It ain't just you." So I think normalizing that is really helpful for these kids. It's also really helpful when I show them mine. I'll take them off and show them that I'm wearing them. Sometimes they didn't even notice. The technology now is a lot smaller, and you can get them the color of your hair or you can get rockstar glitter blue if you want. Every kid's different. Some kids don't mind at all. I've had kids who've never had an issue. And then some, it is absolutely the worst thing that they've ever been asked to do. It's really crushing for them. And I think just normalizing their feelings about that being okay is incredibly helpful, for the parents as well.
[Gopi Shah MD]
Well, I learned so much today, Rachel. Thank you so much. I feel like the whole concept of language access, you enlightened me from since I was a fellow to eight years later, so thank you so much for your time today. Do you have any final pearls or tips that you'd want to leave us with?
(10) Pearls from Dr. Saint John and Additional Resources about Deafness and Hearing Loss
[Rachel Saint John MD]
First, I just want to thank the two of you for inviting me to be part of this. I love what you guys are doing on this podcast. I think you're very organic interviewers. And it's a lot of fun just kind of chatting with you guys. But it's also fun to talk about work. Sometimes it's fun to not talk about work. Yeah, I mean, I think it's not anything new. I think the entire reason, in my opinion, that I have the job that I do, and what I think is the most important about it, is making sure these kids have access to language. I think it's very easily forgotten. I think sometimes we focus on all the micro stuff. We check off boxes for things like speech therapy and hearing aids. But the big thing is looking at the entire person. And I realize that I am in a clinic situation where I am built to do that. These are things that take time. But one thing I always tell parents when we look at an audiogram, I say, "I never look at an audiogram and determine if somebody needs hearing aids. I always look at a child."
So if you have a mild hearing change, and you're a straight-A student, and socially you're doing great, and you're crushing everything, am I going to double the quality of your life by putting a pair of hearing aids on you? Versus you have the same audiogram, and you're borderline failing a couple classes, and you're really struggling, and you are really hating school, that's somebody who would benefit from hearing aids substantially more. So I think that's a conversation that I think is great to have with families. But it really does take looking at the child that's in front of you and not just that piece of them in context. Because at the end of the day, this is all about function.
[Ashley Agan MD]
Great. I think that's super helpful. Thank you so much for what you do. If people want to find you after the show, are you one social media? Are you online? Or do you recommend any online resources for people if they want to learn more, want to read more?
[Rachel Saint John MD]
So I am kind of one of those weird Luddite people. I am not on social media.
[Ashley Agan MD]
That's awesome.
[Rachel Saint John MD]
I used to be. And I made that decision a couple years ago to get off, and it was incredibly liberating. So no, I do not have any social media. But we do have the website for the clinic on the Children's main website, so it's https://www.childrens.com/specialties-services/specialty-centers-and-programs/ear-nose-and-throat/programs-and-services/ffc-for-deaf-and-hard-of-hearing-children and our clinic is there. I think we actually have some of our resources posted there as well. There's a lot of great resources out there that are available online for people who are looking to learn more about this. In terms of professionals who are looking to learn more, a great place to look for some... It's kind of a really nice clearinghouse website for information regarding early hearing detection and intervention, so that kind of early language acquisition, birth-to-three. What are we supposed to be doing with kids in terms of screening and testing and monitoring? It's the American Academy of Pediatrics EHDI website. And if I gave you the website, it would be very long. But if you just go into a search engine and type in AAP EHDI, it's the first thing to come up. And there's actually some really great resources there for professionals who are looking to learn a little more about this stuff.
[Ashley Agan MD]
Cool.
[Gopi Shah MD]
Awesome. Well, thank you so much, Rachel. It was great to have you. For our listeners, you can find us, our BackTable Twitter, @_backtableENT. Reach out to us. Send us suggestions of topics, speakers, if you want to come on the show. Is that a wrap, Ash?
[Ashley Agan MD]
I think that's a wrap.
[Gopi Shah MD]
All right. It's a wrap.
[Ashley Agan MD]
Thanks, everybody.
[Gopi Shah MD]
Thank you.
[Ashley Agan MD]
See you next time.
Podcast Contributors
Dr. Rachel St. John
Dr. Rachel St. John is a practicing ENT and Associate Professor of Clinical Medicine at Children's Medical Center Dallas/UTSW Department of Otolaryngology.
Dr. Ashley Agan
Dr. Ashley Agan is a practicing ENT and assistant professor at UT Southwestern Medical Center in Dallas, TX.
Dr. Gopi Shah
Dr. Gopi Shah is a practicing ENT at UT Southwestern Medical Center in Dallas, TX.
Cite This Podcast
BackTable, LLC (Producer). (2020, November 24). Ep. 10 – Comprehensive Care For Deaf and Hard of Hearing Children [Audio podcast]. Retrieved from https://www.backtable.com
Disclaimer: The Materials available on BackTable.com are for informational and educational purposes only and are not a substitute for the professional judgment of a healthcare professional in diagnosing and treating patients. The opinions expressed by participants of the BackTable Podcast belong solely to the participants, and do not necessarily reflect the views of BackTable.
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