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BackTable / ENT / Podcast / Transcript #33
Podcast Transcript: Single Sided Deafness in Children
with Dr. Anita Jeyakumar
We talk with Dr. Anita Jeyakumar about her approach to Single Sided Deafness in Children, including workup, treatment and long-term care. You can read the full transcript below and listen to this episode here on BackTable.com.
Table of Contents
(1) Presentation of Single-Sided Deafness in Children
(2) Supporting Children with Single-Sided Deafness in School & Beyond
(3) Workup of Single-Sided Deafness in Children
(4) Hearing Options for Children with Single-Sided Deafness
(5) Cochlear Implants for Single-Sided Deafness
(6) When to Consider Cochlear Implant Surgery in Children with Single-Sided Deafness
(7) Etiologies of Pediatric Single-Sided Deafness
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[Dr. Gopi Shah]
This week on the BackTable podcast.
[Dr. Anita Jeyakumar]
When you give them sound with the cochlear implant initially, the sound honestly sounds like garbage. It does. Expecting a child who has a normal hearing ear to listen to a garbage sound and want to listen to it and pay attention to it, I think is an unrealistic expectation. You have to work with them. Fortunately, implant technology supports that. Technically, the FDA approval for single-sided deafness is only for one of the companies, but all the companies do what's called streaming.
Just like we can stream into our headphones, like we're streaming now on this podcast, you can stream into a cochlear implant and you need to have all those pieces in place that the child has to have some daily time on top of working with a speech therapist for an unclear period of time where they're going to be streaming into their affected ear and going to periods where they're going to hear things that sound awful to them with the idea that between time and audiologic programming and speech therapy, that it'll get to a place that they'll have a balance of sound on both sides.
That's what the parental commitment needs to be in. If the parent is not going to do that, if they are going to presume that it's like a light switch, or God forbid, even worse, if we presume that it's like a light switch, the child is not going to do well. Then it's not the right answer for them.
[Dr. Ashley Agan]
Hey, everybody, welcome to the BackTable ENT podcast. Our mission here is to bring you high quality otolaryngology content that enhances your medical education and gives you answers to those burning questions that pop up in your day-to-day work. Quick introductions. My name is Ashley Agan, and I'm a general ENT at UT Southwestern in Dallas.
[Dr. Gopi Shah]
My name is Gopi Shah. I'm a pediatric ENT at UT Southwestern in Dallas as well. How are you doing today, Ash?
[Dr. Ashley Agan]
Hey, Gopi, doing so well. Excited for this podcast. Always excited to be talking to you and our guests on Sunday mornings. It's good!
[Dr. Gopi Shah]
It is good. We have Dr. Anita Jeyakumar. She's a pediatric otolaryngologist practicing in Akron, Ohio, with a focus on ear surgery. She's here today to talk to us about single-sided deafness in children. Welcome to the show, Anita.
[Dr. Anita Jeyakumar]
Oh, thank you so much, Gopi and Ashley. It's a pleasure to be here this fine Sunday morning with you guys.
[Dr. Gopi Shah]
Anita, can you tell us a little bit about yourself and your practice before we get into it?
[Dr. Anita Jeyakumar]
Sure. I am a pediatric otolaryngologist by training, but I've always had a really strong interest in ear surgery. Most of it has been in kids until very recently, where I was given an opportunity to start my own program. It is under the auspices of a hospital. I work in the Mercy Bon Secours system and I work part-time now. I started an otology program there for adult and pediatric ears, as well as a cochlear implant program. It's sort of considered a little bit of an underserved part of the country because Ohio has a lot of big hospital systems. It's a pretty active, robust, but very fun practice.
[Dr. Ashley Agan]
That's great.
[Dr. Gopi Shah]
That's awesome. Today we're talking about single-sided deafness in children, specifically. Let's get into it. Can you talk to us about single-sided deafness? Are we mostly talking about sensorineural hearing loss when we say that? What do we mean?
[Dr. Anita Jeyakumar]
I think when you talk about hearing loss, as you guys are so well aware, it could be such a wide range of things. I think for the focus of this podcast, I was hoping that our focus would be sensorineural hearing loss in children. In general, hearing loss is vastly underestimated, and we don't give as much credit to how much our patients are struggling. Yes, for our talk today, it's really how it impacts children and some of the things that hopefully we can learn together to do together.
(1) Presentation of Single-Sided Deafness in Children
[Dr. Gopi Shah]
Anita, do you find that a lot of your kids are the three-month-old babies that fail their newborn hearing screen in one year? What percentage are older kids that maybe they got through their newborn hearing screen otherwise healthy, and then their first pediatric hearing screen at age four, they had a failed hearing test? How do your patients present, I guess, is my question.
[Dr. Anita Jeyakumar]
Gosh. Probably a combination of both. When you look nationally, the nice thing about hearing loss, particularly sensorineural hearing loss, is that we have a lot of international and national numbers to look at. Overall, when a child is born, they have an incidence about 1 in 1,000 of being born with single-sided deafness. That actually rises to about 14% by the time they reach about age 6 to 19. I see sboth quite a bit, I would say. The older ones usually get caught by their teachers as opposed to necessarily a primary care provider or physician. The younger ones, yes, the goal would be to catch them through newborn screening. Absolutely. If we did screening well and the follow-through well, and all of our states have good and bad times with that, but our hospital is a birthing hospital for the region, so a fair number of my kids that come through do come through the birthing process.
[Dr. Ashley Agan]
Just to clarify, when we say single-sided deafness, we're talking about having a single-sided profound hearing loss, or does this category also include some of the kids who might be moderate, moderate severe? Obviously, I think my assumption is that they would all have one good hearing ear and that the other side has got some sort of loss.
[Dr. Anita Jeyakumar]
That's exactly right. I think the real granular definition of single-sided deafness is that you have profound loss in one ear and completely normal, unrestricted hearing in the other ear. As we know, the practice of medicine is never black and white. Definitely there are kids that range the spectrum where we say profound loss in one ear and normal hearing in the other ear. If they have moderate loss in one ear, normal hearing in the other ear, is that a functional ear for them? I think those are the kinds of things that we don't necessarily always give practice to, that there's a difference between function and objective testing.
[Dr. Gopi Shah]
Yes. In the different age groups, so we talked about the newborn, the preschool school age and the older teenager, how do they present differently in terms of-- how's your counseling or your approach different in each age group?
[Dr. Anita Jeyakumar]
I guess let's start with the babies because that's always the most difficult. It's all difficult because ultimately they have normal hearing in one ear. You're talking to some extent philosophically, you're talking to parents who usually know someone who doesn't have hearing in one ear maybe, or has heard of someone and they've never done anything, but they're "a successful person in life." There, always a question is, why do I have to label my child or why do I have to do anything at all? The reality is there's more and more data. The data has been around since the '80s that if a child has untreated, single-sided hearing loss, they have educational challenges.
It really starts as a baby. You identify them early. We need our early identification to work so well. More importantly, we need our early identification going into early intervention to work really well. It doesn't help to identify the baby if we just sit on them. We have to identify them and direct the family. The intervention, contrary to what people think, doesn't necessarily have to be a device. That is about patient and parent choice. It really needs to be awareness that there are options. I think the days that we said, "Oh, you have normal hearing in one ear, you'll be fine," those days are gone. If they're not gone, they should be gone.
What we want to say is, "Okay, you have normal hearing in one ear. That's awesome. How can we make this better for you?" It starts with identifying as a baby. You got them into early intervention and you just watch them closely. We don't know if they're going to have issues with the other ear. We don't know what the etiology of hearing loss is. There's a pretty wide range of ideology for single-sided deafness in babies.
Then as they get older, the same things, the kids that don't have an intervention have a high incidence of failing or repeating a grade, that is sometimes, in some literature, 10 times the average population, which is sometimes in line with what kids with bilateral hearing loss have that don't have intervention. Really, we know there's an impact to it beyond just directionality and the ability to drive, which is what a lot of people try to boil it down to. It's much more than that.
(2) Supporting Children with Single-Sided Deafness in School & Beyond
[Dr. Gopi Shah]
In terms of the impact of trouble in school, the single-sided hearing loss doesn't even have to be deafness. The impact is at the level of mild to moderate. You know what I mean? I think that as the ENT, it's important for us to take it very seriously and talk to the families, especially the school-age kids about FM system and preferential seating, and how do we help them even in those school environments?
[Dr. Anita Jeyakumar]
Absolutely. In adult world, they talk about the cocktail hour, right? If you have single-sided deafness as an adult and you're at a party or a gathering, something fun, you then lose half the room because your good ear is only pointing in one direction. Imagine a child in a playground, the most fun thing they can be. The kids are all milling around them and playing, but they lose half of the interaction of what's going on in that playground, which is a very fluid environment. A lot of these kids get labeled as being socially awkward or distant, or certainly as we know, kids, adults, people are not forgiving. The kids then get labeled as being unsuccessful people. It really is no fault of theirs. They just don't have access to 50% of the conversation and interaction that's happening, which makes them pariahs, is what ends up happening.
[Dr. Ashley Agan]
Yes. Another thing that I think comes up a lot is the concept of cognitive load and needing to use more brain power to figure out what's going on in the classroom because you may not be hearing things that are happening on one side. Can you speak to that at all?
[Dr. Anita Jeyakumar]
I think that's a great point. I think there's cognitive load and cognitive fatigue. The reality is that they're having someone who has hearing loss and who's, let's say, presumably adjusted well, is having to constantly be attuned to what's going on in their environment. The way they do that, if they don't have a technology piece to help them, is that they swivel. They're constantly swiveling their heads. That, in and of itself, is exhausting. The reality is then that they have gaps. Regardless of how well they're swiveling, they're going to have gaps in what they get in their attention span. They're in a classroom, let's say, if they're really good at swiveling, they miss 30% of what's being said to them.
Imagine if you miss 30% of all your medical school lectures, that's a lot of reading and extra time you have to put into it to fill in the gaps. Plus, if you miss the wrong word, something that says it's not correct, and you miss the not, well, then you've completely grasped the wrong concept of what's being taught to you. Then so on and so forth, applies to meetings, applies to job descriptions, applies to all of those things. It really ends up cannonballing as things go, and they're exhausted. Sometimes the parents will say they don't want to go out with their friends. Well, gosh, if they have just had a full school day, they're tired. They don't want to go out with their friends, and the world doesn't exactly understand that for them.
[Dr. Gopi Shah]
I feel like for some families, it's easier to describe them, like with glasses or vision, if something is blurry and they're trying to read the board, you're going to get tired of trying to read the board within 5, 10 minutes, you might take a break, your gap, and then maybe you're trying to read again. I feel like sometimes that analogy can help a family understand because hearing I feel like isn't always, just depending on the family or the patient.
[Dr. Anita Jeyakumar]
Absolutely. It's surprising, isn't it? If you tell people, would you leave one cataract in? They probably wouldn't leave one cataract in, or would you wear just one contact lens and go about your merry way? For hearing, that seems to be an acceptable concept still.
[Dr. Ashley Agan]
The other question would be the importance of sound localization. You need two hearing ears to be able to localize sound, right? I think that's another thing that gets overlooked, when we're like, "Oh, well, at least they've got one good hearing ear. They'll be fine." I think that's a pretty big deal when you're navigating the world.
[Dr. Anita Jeyakumar]
I completely agree. If a child can't safely cross a road, well, that's a safety issue, right? I think anyone would say it's a safety issue if a child can't safely cross a road. Yet, by having unbalanced hearing, they cannot safely cross a road. They look left, right, left, but something barrels through on the bad side or affected side, they don't know. It's a safety hazard. That goes into driving too.
[Dr. Gopi Shah]
I was just going to say, we've talked about the impact it can have in school and work. What kind of impact have you found that it has on speech with one good ear and one ear that isn't hearing as good or has no hearing?
[Dr. Anita Jeyakumar]
That's a tough one, because technically, when you have normal hearing, especially if you're in a language-rich environment, you're probably going to have pretty good articulation. It almost makes it harder to get through to families who are making these hard decisions, that this is affecting your child, because they don't feel what the child feels. Their child sounds very similar to them. It's not like they have a bunch of artic[ulation] errors. It's not like they are necessarily answering things completely incorrectly. The concept is there because they get the broad concept, like the forest for the trees is there.
In general, kids with single-sided loss who have normal hearing on the unaffected side, the true definition, should have normal-sounding speech. It makes the paradigm almost more complicated.
[Dr. Gopi Shah]
Yes. That makes sense. When kids are developing speech, there's a little bit of that little kid sound to it anyway. It's like, well, that's how three-year-olds talk!
[Dr. Anita Jeyakumar]
Yes. They're babbling and they're meeting milestones, they're progressing. Yes, it's really hard to-- sometimes I think that the eye doctors have it so easy, just shut off one eye and everyone's like, "Oh, we wouldn't do that." Yet we shut off one ear all the time. It's like, "Why is the ear such a bad guy? Why shouldn't we have two good ears? Two good ears."
(3) Workup of Single-Sided Deafness in Children
[Dr. Gopi Shah]
Then when do you get imaging, and do you prefer a CT or an MRI? What's your workup?
[Dr. Anita Jeyakumar]
Great question. I think if they're older and I'm just identifying them, I probably get imaging right away. I tend to prefer an MRI. There's a fair amount of robust data coming in the literature beyond the radiation issue that in an MRI, you're going to identify more issues that are pertinent to people with single-sided deafness. There's a high degree of hypoplastic auditory nerve, aplastic auditory nerve, and then other cochlear anomalies, which you really should be able to identify on a CT or an MRI. Now, the cell, of course, as otolaryngologist, I'm the same as everyone else. I trained mostly on CTs. I love my CTs. It was a shift.
I'm not a neurotologist by training. I had to teach myself how to look at MRIs and not freak out that I didn't have all my bony windows and cuts and things like that. Then you have to often sedate them. If they're under double digits in age, you do have to weigh that risk of sedation. The problem is there's a fair amount of data out there showing that if they have a normal CT with normal IACs, they still can have an aplastic or hypoplastic nerve. Getting a CT temporal bone on someone with single-sided deafness, while it might appear normal to us, it may not necessarily be normal.
That definitely feeds into some of the options we might want to give them, is that if they have a hypoplastic or aplastic nerve and you don't know that, and let's say you move forward, jumping right ahead into one of the potential treatments, cochlear implantation, then you probably haven't done your due diligence in counseling the patients about their outcome with implantation because you don't know that they have an aplastic or hypoplastic nerve. Not to say it's the wrong option, but the counseling needs to be different.
(4) Hearing Options for Children with Single-Sided Deafness
[Dr. Ashley Agan]
That might be a good place to get into hearing options, hearing aid options and what are the different options for children who have single-sided deafness?
[Dr. Anita Jeyakumar]
The nice thing is that it's a wide array of options. It's good and bad. One of my friends put it the nice way, it's like back in the day, 20 years ago, you had a bicycle. If you were single-sided deaf, you got a bicycle. Now you have a Cadillac of options, like you have a Corvette and a Cadillac and a Mini Cooper and it's really, really overwhelming. What are our options? You do have the option of saying, "I'm just going to watch them." I think watchful waiting is an appropriate option for the appropriate family.
What does watchful waiting mean? It means that one, you acknowledge that they have this and the kids need to be watched closely. They need to get audiologic evaluations at least every year because you're watching the affected ear as well as watching the presumably unaffected ear. Once you've done your medical workup and you're watching how they're doing in school, are the teachers noticing an issue? Do we need to get them an IEP in school and make sure that they get preferential seating? Do we need to start with an FM system at school? Once you've marched through those educational options, then you talk about some of the technology options.
We as otolaryngologists, we love our technology and we will touch base about some of this. Sticking a technological option on a child without teaching them and their family how to use it is really not very helpful. I think we do miss the boat on that quite a bit. Before we go into that and my personal soapbox, the technology options that we talk about is you can do a CROS hearing aid, you could do some kind of bone conduction device. Then technically, per FDA rules, once they're above five, then we can talk about that too. You can do a cochlear implant, but right now cochlear implantation in kids is only approved for age five and older, which definitely has come back to some of their current data.
The differences though, are that really all the options except for cochlear implantation, you are bypassing the affected ear. The CROS hearing aid is sending the signal to the good ear. I will say CROS-technology has improved substantially from when most of us were in training and that it's not quite as clunky and quite frankly, awful as it was. It's better. Some kids, teens, do like it for certain situations. It's not an absolute no. When I first came out, I used to think that I will never prescribe a CROS for my patient because they were horrible. I would say that's evolved to being better.
The BAHA technology, I will say, in fairness to my audience, I am not a fan of BAHA technology for single-sided deafness because I just personally haven't seen too many kids that use it long-term. I've seen a lot of non-users because it's directing sound to the other side. They have to wear it. It's sometimes echo-y, is what a lot of my teens will tell me. Especially with CI being an option, particularly when done properly, I probably tend to-- if they're talking about a surgical option, I often will try to persuade them to do a CI as opposed to a BAHA to get function in the affected ear.
[Dr. Ashley Agan]
Can I stop you for a second and just have you elaborate on some of those terms for maybe our non-physician listeners? When you say CROS, can you go into what that means for the patient? Questions that frequently come up is, so am I wearing one hearing aid or two hearing aids? What does that look like from a patient standpoint, what your patients feel?
[Dr. Anita Jeyakumar]
Thanks, Ashley. I know we get excited and we forget ourselves with our acronyms. A CROS is basically hearing aid technology. Technically, they're wearing two hearing aids. There's one in the affected ear and one in the unaffected ear. They're both designed a bit differently because you don't want to mess up the sound in the good ear, so to speak. Usually, the hearing aid in the good ear doesn't have a full seal that's blocking off the ear canal and things like that to mess up the good hearing that they have. Essentially, the hearing aid in the affected ear is sending the signal to the hearing aid in the good ear. The child learns to understand that that signal is coming from the affected side.
If it sounds complicated, it is, because children don't necessarily understand that right away. It's called CROS because it's crossing over to the good side, essentially. There's also something called BiCROS, but that's for people who have hearing issues on both ears. That's not the situation we're talking about right now.
[Dr. Ashley Agan]
The big thing with CROS is that you're not actually aiding the deaf ear. You're just taking any sound that's coming from that side of the body and routing it over to the good hearing ear.
[Dr. Anita Jeyakumar]
Right, exactly. Again, if we go back to our cocktail hour or playground, if a child is actually focusing on someone on their good side, suddenly there's a signal coming from the other side. They're like, "What's happening over there?" It may not be a relevant signal. Theoretically, it can be good. I think in a classroom setting, you can see how it would be helpful. A lot of our measures are what we call best case scenarios. In the real world, most people are not in a classroom. Most classrooms are not quiet. An audiologic booth, which is where we do testing and testing in quiet is really not reflective of real world for 99.9% of people.
[Dr. Gopi Shah]
The other hard part with a CROS hearing aid or hearing aids in general is with one normal good ear, at least in the state of Texas, and then the other ear, insurance doesn't cover it. It becomes a major cost issue here. Then there are certain foundations where families can apply to aid if they're like, "Listen, we just don't want surgery," which is a very reasonable decision. There's obviously a money issue. There are foundations. Then I don't know. I know there's loaner hearing aids in the school. I would imagine there's loaner CROS ones. You have to have a school system that has the resources, the audiologist equipment and the support system for that too.
[Dr. Anita Jeyakumar]
No, 1000%. There's no doubt that our system is flawed, that you don't have hearing aid coverage from most commercial insurance or Medicare for that matter, but yes, they will pay for a BAHA or they'll pay for a CI. You have people coming to you that just want to skip the payment part. They just want to skip the medical part and jump right to surgery because it's actually covered. They also want help. Yes, a lot of programs, including ours, are focusing on loaner banks and things like that. The problem is you're dealing with humans, right? Loaner banks, they can sign away their first child to you, but those hearing aids may be gone, gone.
Just because they look nice and tell you all kinds of stuff doesn't mean that those hearing aids are going to come back. You do have to be careful. I'm a trusting person. I'm like, "Oh, yes, they'll bring it back." The audiologists just pat me on the shoulder and they go, "No, they're not bringing it back."
[Dr. Ashley Agan]
Okay, Dr. Jeyakumar.
[Dr. Anita Jeyakumar]
Exactly.
[Dr. Ashley Agan]
Getting into the details of a BAHA. When you say BAHA, what does that look like? Because I agree, it does always seem weird that insurance would cover a BAHA but not a CROS hearing aid just because one is surgical and one is not.
[Dr. Anita Jeyakumar]
BAHA technically is a generic term that encompasses a whole bunch of stuff. It traditionally means a bone conduction device. It's some technology that's either worn or surgically placed on the affected side that's, again, routing the signal through bone to the unaffected ear, to the unaffected hearing ear. You don't need to have an intact auditory nerve or anything like that on the affected side for you to have a BAHA. If you're super young, meaning that you're too young as a patient to meet the criteria to get the surgery, you can wear it on a soft band.
The nice thing I will say about the BAHA is that you can truly try it non-surgically and get some sense of whether it will help the patient or not, which I really do love because you can't do that with a cochlear implant. A cochlear implant is like you're all in or all out, but you can wear it on a soft band. Now, I will say also that my teens, males, and boys in general, are not enamored by wearing anything on a headband. If you stick a bow on it, they're even more ticked off. There are ways that our audiologists have come up with like rigging baseball caps and things like that that you can put it on and get that band-like sensation.
They can really have a real-time attempt to see if they like it before they go for the surgery. I tend to encourage my patients to do that because, one, we have the capability. Two, from a billing standpoint, if you can get approval for BAHA surgery, they can get the device and you don't have to jump right to the surgery. They can truly try it for a several-month period without burning any bridges for them. Again, they're not hearing on that affected side, which, gosh, it's really hard for us to conceptually understand that. That I'm doing a surgery on, let's say it's a right single-sided deaf ear, but I'm really not accessing the right side except anatomically. I'm really using your left side.
No matter how many times you say that, the patients don't understand that. Certainly other medical professionals don't understand that. They're like, "Well, they're hearing in that ear." No, they're not. They're really not. It's really confusing, but it does have a role in our system. If you have people who have been single-sided deaf for a long, long time, who have normal hearing, well, the data on cochlear implantation is not as robust in getting them sound quality in the affected ear. For them, if they just want to drive or certain situations, the BAHA might be the best solution for them. Now, back in the day when BAHAs were made, you used to have what was called an abutment, meaning that it was a screw that went in the skull. Then there was essentially a screw that poked through the skin. That's what you clipped the hearing device on if you went surgically.
[Dr. Gopi Shah]
Back in the day, Anita, that was 10 years ago.
[Dr. Anita Jeyakumar]
It was back in the day because I hated those screws. I could not stand those screws. I was like, "Oh," I was like, "Oh my God, I just made my kid look like Frankenstein." The audiologists look at me funny, but cosmetics matters. It matters so much in our field and it matters to children who already can't hear and are getting bullied and whatnot. For an atresia kid, absolutely. I get it. Atresia is a different category. We're not talking about that today. Atresia means they have no ear, for anyone who's wondering. For a lot of kids, especially with the variable hit or miss with single-sided deafness, I really struggled when BAHA was the only option for these kids. Now we have BAHA options that are not abutments.
You can put a similar kind of device underneath the skin and it's based on a magnetic principle. What they wear on the outside is attached by a magnet. Quite frankly, I'm so much more excited about this. One is that the hearing outcomes are very similar to having something coming through skin. Two, cosmetics, again, it does matter. I think if we can't justify doing it in our family and our children, we need to think about what we're setting up other people's children for and not take it for granted because they're the ones that have to wear it and are out there.
Plus, there's so much data showing that when they have that abutment screw sticking through, kids, for whatever reason, have such a high incidence of getting infected all the time. No, it's not a cleanliness issue, contrary to what the general population thinks. We don't know why, but kids just get them infected all the time. It's just this hot mess, quite frankly.
[Dr. Ashley Agan]
Yes, that's exactly what I was going to say, is that there was all the issues with the skin overgrowth, infection, drainage and all that.
[Dr. Gopi Shah]
Then they're not wearing it for four months because they got another infection and they stay dry for two months and they got another infection.
[Dr. Ashley Agan]
Yes, it's a chronic issue.
[Dr. Anita Jeyakumar]
Yes. The whole purpose of it is not helping.
[Dr. Ashley Agan]
Yes. When you're thinking about CROS versus BAHA, really, the advantage of the Baha is that you're not having to wear anything on the contralateral good hearing ear. Is that one of the main differences?
[Dr. Anita Jeyakumar]
Correct. That's exactly right. Perfect. Couldn't have said it better myself.
(5) Cochlear Implants for Single-Sided Deafness
[Dr. Ashley Agan]
Let's get into the cochlear implant for single-sided deafness. We said the FDA approved it for kids 5 and up. Who makes a good candidate and how do you decide?
[Dr. Anita Jeyakumar]
All great questions and if I had all the answers, I'd be retired for sure, but I will do my best to tackle it. One, the FDA approval for kids only came in 2019. It's really recent. I would say probably most of the data we look to are really coming from Europe. They have been 10 years ahead of us in terms of hearing outcomes and hearing technology. When you look at them, what their data seems to suggest is that it makes sense in some ways. It says the shorter the duration of the single-sided deafness, the better your chance of getting hearing back in that ear.
How is that shorter duration defined? Some papers suggest somewhere between 4 to 7 years. I'm saying these vague terms like suggest and all of that because we're not really sure. We think that if there's a ton of auditory deprivation, that we know that the visual cortex takes over the auditory cortex in the brain, meaning your eyes work harder and your ears work less. That data is really for people who have bilateral hearing loss. What does it mean when one side of your brain is hearing perfectly and the other side is hearing not at all? We don't 100% know.
It's really hard to look at someone who has just heard about cochlear implantation and they're 8 and their parents want to pursue it and say that this is not something you should try. That being said, I do think that because you're giving sound to a ear that has not heard, you can't simply put the device on and not have a mechanism for them to learn to use the device and expect that they'll be successful. I think that's a recipe for failure. What do I mean by mechanism? Meaning that you have to have a team. We as the surgeons can get the device in, right? Almost all of us have seen, done, do implants as otolaryngologists. It's not a matter of us being the variable. Hopefully we know how to do the surgery well.
We come out with the certification as a general otolaryngologist. The issue is, do we have a team in place that can teach them to listen? Do we have a program in place that these kids can go in and constantly work on their affected ear, their implanted ear? Because when you give them sound with a cochlear implant initially, the sound honestly sounds like garbage. It does. Expecting a child who has a normal hearing ear to listen to a garbage sound and want to listen to it and pay attention to it, I think is an unrealistic expectation. You have to work with them. Fortunately, implant technology supports that.
Technically, the FDA approval for single-sided deafness is only for one of the companies, but all the companies do what's called streaming. Just like we can stream into our headphones, like we're streaming now on this podcast, you can stream into a cochlear implant and you need to have all those pieces in place that the child has to have some daily time on top of working with a speech therapist for an unclear period of time where they're going to be streaming into the affected ear and going to periods where they're going to hear things that sound awful to them, with the idea that between time and audiologic programming and speech therapy, that it'll get to a place that they'll have a balance of sound on both sides.
That's what the parental commitment needs to be in. If the parent is not going to do that, if they are going to presume that it's like a light switch, or God forbid, even worse, if we presume that it's like a light switch, the child is not going to do well. Then it's not the right answer for them. That was a very long answer to your question.
[Dr. Ashley Agan]
No, that was really great because I think that highlights how difficult this all is. It's not black and white and it sounds like these kids are very different than the child that's born with bilateral profound sensorineural hearing loss.
[Dr. Anita Jeyakumar]
Absolutely. I think when I first started doing single-sided kids, I would go for some of their early programming appointments because I needed to understand. I don't have single-sided deafness, and I wanted to see what it took for them to even just go through just a programming appointment. The audiologist had to basically plug the good ear, stream in the implanted ear and have a semi-cooperative, non-screaming child to follow through. Then, it answers the question. The data suggests younger is better.
For those of us who have children, you start to think, "Okay, well, when is my child going to sit for 30 minutes and put a block in a plug or whatever it is they're supposed to do?" My children are older, but I do have good memories of their attention span. It was like a fly. You'd be like, "Oh, a fly, they're gone."
[Dr. Anita Jeyakumar]
You're supposed to program them to get good hearing!
[Dr. Gopi Shah]
Mine are 7 and 9 and it just flies. That's it.
[Dr. Anita Jeyakumar]
Yes. You're like, "Well, how are you supposed to do this?" Then you're supposed to program 22 electrodes or whatever. It's a lot. It's just a lot.
[Dr. Gopi Shah]
It sounds like it's maybe not necessarily age dependent, but child and family dependent.
[Dr. Ashley Agan]
Very much so.
[Dr. Anita Jeyakumar]
I would say, I think we all know programs that call themselves destination programs. This is not a critique per se, maybe it is. I don't know. If a family is flying to a program to get an implant, and particularly if they're single-sided deaf and the program implants the child, as a lot of very high esteem programs do, but they don't have any mechanism for this kid to go through all this other stuff. Guess what? This kid has not had any favors done for them. It doesn't matter. A lot of programs can do the surgery. There's something to be said for having all the pieces in place. One of the silver linings in COVID is that telemedicine and all this stuff has become better.
Since, again, you can use streaming technology, these are actually probably pretty good candidates for streaming programming. The parents have tools on what to do with their child when they're home, whether they're remote or rural or whatever they may be. We know most of America does not live in urban America. Most of America lives in farming country and rural America. These people need access too, but it has to be a comprehensive package.
(6) When to Consider Cochlear Implant Surgery in Children with Single-Sided Deafness
[Dr. Gopi Shah]
Anita, what are the concrete things? I guess, do you tell the family like, "Okay, this is a day to surgery. We turn the implant on at this time." How many visits or is it just a once a week thing? How does it work in terms of the nuts and bolts so that family has an idea of a timeline?
[Dr. Anita Jeyakumar]
If we start, we identify the child. We do our medical workup, which includes imaging. Let's say it's best case scenario, there's normal anatomy, a normal cochlear nerve. I will have them do a non-surgical trial. Some of that is for the family, some of that is for insurance because we need to get it paid for. Since it was just FDA approved in 2019, it's hit or miss whether it gets paid for. I find that the odds are better if you can show that they have failed a non-medical trial. The non-medical trial might be, quite frankly, a hearing aid in the affected ear, which as you guys all know, is a bunch of crock because it's not going to do any good, but hey, you do what you have to do.
The flip side of that is that you teach the child to wear something on their affected ear, even though it doesn't do anything for them. Because initially, guess what? Your implant is not going to do a whole lot of good for them either. Then they meet the speech therapist, they get an idea of how many appointments it's going to take. I usually say, yes, they have to come either through telemedicine or in-person once a week. Every patient is going to be a different story. For a growing and developing child, it may be many years before you really get to where you want to get.
Parents will say, "Oh, doc, we'll come and see you forever." That's awesome. Is your job going to let you see me forever, once a week? You do have to talk about some of these difficult things and figure out how you're going to make it work for the child. Then we get to implant, activate in two weeks. Then yes, really, at that point we're done, right? As a surgeon, as the interventionalist, we have done our job, but no, then you watch with the team. I do think we have a responsibility to our patients to do that a bit more. I know that doesn't work with insurance and billing and all that.
Really, to understand what's successful and not successful, then you have to watch and get feedback from the therapist.
For us, every week we talk about our patients who are ongoing and who have had surgery. Yes, for some patients, it's a quick, Joe is doing great. That's awesome. That's great. For some, it's like, I might think they're doing great. The audiologist might think they're doing great because they had their surgery, they came for activation, the audiologist did their programming, their data logging looked okay. The speech therapist might say, "Well, actually, for the last two weeks, they came with their implant in their purse." Guess what? It doesn't do a lot of good in the purse. Then we have a check-in and say, "Okay, what's going on?" All of those things have to be in place for an unclear period of time for a child really to do well.
[Dr. Ashley Agan]
Do you give people an estimate of like, weeks? months, a year, don't commit to a time, it just depends.
[Dr. Gopi Shah]
This is like allergy shots, two to five years.
[Dr. Anita Jeyakumar]
I say for a long time. The average American family moves quite a bit. I say you need to be established with either me or a reputable program for the duration of your child in high school. My goal tends to be to get your child to graduate high school, which sounds crazy to us because we're all highly educated and have done all this school. It's not intuitive for most children with hearing loss, actually, who are deaf and hard of hearing. That always tends to be my goal, that, hey, if your kid is doing really well, we will spread out the visits as best as we can and all of that.
The follow-up is needed to make sure that if the classroom changes, if you suddenly have a second language requirement that you didn't before, if your location changes and you're suddenly in remote schooling or everyone's wearing masks, does this sound familiar? If all those things change then we have to be in touch with you so we can help you.
[Dr. Gopi Shah]
Right. This is super helpful. One last question for you. It sounds like the CI is better, at least surgically, than the BAHA, in your opinion, which is great. Are there patients, I think you said this though, is the atresia patient really the only patient you'd be like, "No, definitely a BAHA," for?
[Dr. Anita Jeyakumar]
I think it depends on your practice. Certainly for people who have had a canal wall down mastoidectomy for really bad ear disease or people who have a chronic draining ear that cannot tolerate a hearing aid, I think the BAHA has its role for sure. Those are all conductive losses. For a conductive loss, a bone conduction device is amazing. We really shouldn't be talking about an implant for a conductive hearing loss. This category though is like the sensorineural hearing loss. I personally, and probably a pretty robust group of people like me, are not huge fans because again, you're just not utilizing the affected ear.
[Dr. Ashley Agan]
Yes, that makes sense. Wrapping it up, what have we missed? Is there anything that we have failed to ask or that you would just want to leave with our listeners as we round out the show?
(7) Etiologies of Pediatric Single-Sided Deafness
[Dr. Anita Jeyakumar]
Oh gosh, I could go on forever. Wrapping up, I will say, I think the only thing that we didn't really touch on is etiology. If a baby is coming with a new diagnosis of single-sided deafness, a couple of things that we need to talk about beyond the nerve hyperplasia is that there is a strong association with congenital CMV. Now, congenital CMV, it's its own beast in and of itself, and most places in the country don't screen for congenital CMV. The only ones that are caught are about the 10% of kids who have symptomatic congenital CMV as defined by a neonatologist. Unfortunately, congenital CMV, even in asymptomatic kids, has a pretty high degree of hearing loss.
Up to 15% to 20% of adolescents with asymptomatic congenital CMV will develop sensorineural hearing loss. Just something to keep in mind, another plug to get imaging, because most of the times you can't get access to their blood spots by the time they come to see us as otolaryngologists. You can usually find some of those periventricular calcifications on an MRI when you do imaging for workup for single-sided deafness. Another reason. Is that definitive? No, it's not, unfortunately. It gives you a pretty good idea when you put all the pieces together. Intervention is important, whatever that intervention is. I know I dogged on BAHA a little bit, but the key is intervention is important. Someone needs to be paying attention to these children, and helping their parents help them is really key.
[Dr. Gopi Shah]
I think that's right on. Thank you so much for joining us, Anita. It was so nice to have you here today. Thank you for all of our listeners who joined us. New listeners, thank you for stopping by, and our returning listeners, we appreciate your time always. You can find us on SoundCloud, Spotify, iTunes, and Apple. You can follow us on Instagram and Twitter @_backtableENT. We'd love feedback, reach out to us for topics, ideas, speakers, or if you ever want to come on the show. Ash, what am I missing?
[Dr. Ashley Agan]
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Podcast Contributors
Dr. Anita Jeyakumar
Dr. Anita Jeyakumar is a practicing Pediatric Otolaryngologist in Ohio that specailzes in ear surgery.
Dr. Gopi Shah
Dr. Gopi Shah is a pediatric otolaryngologist and the co-host of BackTable ENT.
Dr. Ashley Agan
Dr. Ashley Agan is an otolaryngologist in Dallas, TX.
Cite This Podcast
BackTable, LLC (Producer). (2021, October 12). Ep. 33 – Single Sided Deafness in 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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