BackTable / ENT / Podcast / Transcript #16

Podcast Transcript: Congenital Hearing Loss

with Dr. St. John and Dr. Kutz

We talk with Dr. Rachel St. John and Dr. Walter Kutz about Congenital Hearing Loss including causes, available treatments, and counseling. You can read the full transcript below and listen to this episode here on BackTable.com.

(1) Detecting Pediatric Hearing Loss

(2) Assessing Pediatric Hearing Loss Using Birth History & ABR Techniques

(3) The Role of Severity & Symmetry in Diagnosing Pediatric Hearing Loss

(4) CT vs. MRI for Evaluating Mixed Hearing Loss

(5) Managing Pediatric Hearing Loss in the Context of Virtual Learning & Telehealth

(6) Auditory Neuropathy in Pediatric Patients

(7) Pediatric Hearing Loss Due to CMV

(8) Pediatric Hearing Loss Due to Bacterial Meningitis

(9) Cochlear Implants

(10) Contraindications for Cochlear Implants

Listen While You Read

Congenital Hearing Loss with Dr. St. John and Dr. Kutz on the BackTable ENT Podcast)

Ep 16 Congenital Hearing Loss with Dr. St. John and Dr. Kutz

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[Dr. Ashley Agan]
Hello, everyone. Welcome. This is the BackTable ENT Podcast. Here, we bring you conversations with the best and brightest minds in our field with the hope that you can take this information and apply it to your own practice. I'm Ashley Agan, and I'm a general otolaryngologist practicing in an academic setting here in Dallas, Texas.

[Dr. Gopi Shah]
I'm Gopi Shah. I'm a pediatric otolaryngologist practicing at Children's Medical Center in Dallas. We are your hosts, and we are glad you stopped by to check out our podcast today. I'm so excited for the podcast on congenital hearing loss. With us, we have two returning guests, Dr. Rachel St. John and Dr. Walter Kutz. Dr. Rachel St. John was on BackTable ENT Episode 10, Comprehensive Care for Deaf and Hard of Hearing Children. She is an associate professor of otolaryngology at UT Southwestern and the director of our Family Focus Center for Deaf and Hard of Hearing at Children's Health in Dallas. She's a health education consultant at the state and national level.

We have Dr. Walter Kutz. He was on BackTable ENT Episode 4, Managing Eustachian Tube Disorders. He's a professor of otolaryngology and neurosurgery at UT Southwestern. He's a fellowship director of the Neurotology Fellowship and an associate program director for our residency program here. Welcome back to the show, y'all.

[Dr. Walter Kutz]
It's great to be back. Thank you so much.

[Dr. Rachel St. John]
Thank you.

[Dr. Gopi Shah]
How's everybody feeling today?

[Dr. Rachel St. John]
Pretty good. For so many reasons.

(1) Detecting Pediatric Hearing Loss

[Dr. Gopi Shah]
All right. We are super excited. Just to start from, basics. When do infants with congenital hearing loss present to you guys in your clinic? What do you look for? What's in the history? At what point do they come and see you?

[Dr. Rachel St. John]
I'll go first. For me, it's a little predetermined because in order to get into my clinic, you have to have some diagnostic measure of being deaf or hard of hearing, either a behavioral audiogram or an ABR. By the time I see children, they've gone through the audiology process. The parents have at least gotten that initial information that their child is deaf or hard of hearing. If they come to me as infants, it's probably going to be, I'll usually see them around month three or four after they've been through all the diagnostic process and arrive in my clinic. Then, because late onset hearing changes are so much more prevalent than congenital, we get lots and lots of older children, toddlers, school age, teenagers who present in our clinic as well.

[Dr. Gopi Shah]
Before we move on, I just wanted to touch on the screening that leads them to get that hearing loss detected. I know, Dr. St. John, I remember you talking about this when I was a resident and just the importance of making sure we're catching kids at a certain timeline. Can you remind me about what that timeline is? I know, there's the newborn hearing screen that happens in the hospital. What happens after that? Can you have kids who pass that, who ultimately actually do end up seeing you for hearing loss? If you fail that, then what happens?

[Dr. Rachel St. John]
Absolutely. That's a lot. Yes, but I'm going to go through it very succinctly, and then maybe Walt can chime in with this too. Very briefly, there's two different kinds of newborn hearing screening that we use. OAE, which are otoacoustic emissions, and automated ABR, that's an automated acoustic brainstem response. The real quick and dirty on the two of them is that they both-- The thing to remember is they're both screens, they're not diagnostic.

When you do an OAE, which is used fairly widely in the newborn nursery, the well baby nursery, you're basically putting a probe in the ear and emitting a sound and looking for the cochlea to actually make its own microphonic response. A cochlea that is processing sound makes its own sound and that gets picked up by the transducer. There's no interpretation. The machine either picks that up or doesn't, and it spits out pass or refer for more testing.

For kids who have been in the NICU, they are more likely to have something called auditory neuropathy, which I am sure you're going to ask us about later in this podcast. Those kids can actually get through an OAE. They can actually have a typical looking OAE and not be hearing typically. For those kids, it's much more common to use an automated ABR. That's actually checking the entire hearing pathway from an external ear through the middle ear, inner ear, cochlear nerve, all the way to the brainstem. It's looking for a brainstem response in response to a sound. If you have auditory neuropathy, that will get picked up and that will come out as a fail or not passed.

In terms of timeline, the standard timeline for kids just born, without other complication, the American Academy of Pediatrics refers to it as the 136 model. It's screening by one month of age. Then for infants who don't pass that screening, you want a diagnostic with a pediatric audiologist by three months of age. Then for those children who are identified as deaf or hard of hearing, you want to make sure that services are implemented by six months of age. That's the timeline for screening.

There's a lot of kids, a lot, who do pass that newborn hearing screening and then have hearing loss late onset. The place that those kids ideally are supposed to be identified is in the medical home with those pediatric visits. Really, it's very insidious. If it happens during the toddler years, you're basically looking for language development and kids and parental concern that kids are not responding the way that you would expect them. Those would be things that would prompt you to do another hearing evaluation.

[Dr. Ashley Agan]
In terms of the newborn hearing screen, is it true that in the state of Texas, if you're born in a hospital, you should have had a newborn hearing screen prior to leaving, whether it's a healthy baby nursery or the neonatal ICU?

[Dr. Rachel St. John]
It's actually true in most states. The vast majority of states have legislation for newborn hearing screening. That's either a legal mandate through law or that's policy. There's very few states at this point. I would have to go to the CDC Early Hearing Detection Intervention website has all that data in terms of who's got current legislation in place, but it's most of the 50 states that do.

[Dr. Ashley Agan]
I always ask, and I'm sure everybody does, as part of my birth history, because we do have a small percentage or group of babies that are either born at a birthing center or home where the hearing screen is not done and maybe you're seeing them. For us we'll see them for laryngomalacia at two months and yet here we are without a history of a hearing screen. Just things to keep in mind.

[Dr. Rachel St. John]
Interestingly, Texas a couple of years ago passed legislation that now requires both children's hospitals and birthing centers to at least refer those children to a screening site. It used to be those babies that were born at those sites were exempt, for lack of a better word, and they could easily fall through the cracks. At least in the state of Texas, we have legislation in place now. With midwives as well, if you aren't doing the screening, you can't just say, well, I don't screen. You actually have to make a referral to a place that will.

[Dr. Ashley Agan]
It sounds like these babies will present as early as between three to six months to you, to as late as-- When you say late onset, are you talking like 6 months, 18 months, 3-year-old, 10-year-old?

[Dr. Rachel St. John]
Yes. All of the above. In all of the above. It can happen for so many. I'm sure Walter has seen quite a few patients, presenting, they'll have like a late onset acoustic neuroma. That could happen anytime. That could happen in adulthood. That could happen in your late teens. The timeline is life, basically.

[Dr. Ashley Agan]
Dr. Kutz, how do these patients present to your clinic? Is it similar or is it different?

[Dr. Walter Kutz]
Being a tertiary, I'm sorry, I guess being a subspecialist in a tertiary hospital, generally speaking, they're going to be referred in by an otolaryngologist, sometimes audiologist. Typically the pediatricians sending one of the patients to our practice, they'll see one of our pediatric otolaryngologists first. Oftentimes, I'll see the patients once it's been determined they need something like a cochlear implant, or maybe they have atresia and they're considering an atresia plaster or bone-anchored hearing aid. That's typically when I would see patients with hearing loss.

(2) Assessing Pediatric Hearing Loss Using Birth History & ABR Techniques

[Dr. Ashley Agan]
In terms of birth history, prenatal history, I go through how many weeks was the baby born at, ICU stay, any perinatal infections. What other questions in history do you feel like, hey, this is important, but maybe something that we don't always think about that you always make sure you ask every time?

[Dr. Rachel St. John]
That's a good question. I think you covered a lot of them. Prematurity and all of the things that go along with prematurity, there's a ton of risk factors there for hearing changes. Certainly, I think prenatal care is a really big one too, because those moms are going to get testing for TORCH infections and potentially CMV, syphilis, those kinds of things. I think one of the things that I have seen, I think it's just through practice and I've seen enough of these kids, it's not super common, but it happens enough that I've changed my practice, is with kids who have later onset hearing changes. I'm starting to ask much more routinely about noise exposure, both from-- Because, we're sort of in ear pod generation with these kids, they are spending a lot of the day with headphones or earbuds in and are not always mindful of volume.

Also, we're here in Texas, I have a fair number of families and one of the sort of cultural activities that they participate in very regularly is hunting. I have several kids in my practice who have had acoustic trauma and that sort of classic 2,000 hertz drop, because it's just not been the culture of the family to wear ear protection. The kid I saw most recently was really upset, he thought he had done something wrong and he started crying and he said, but Papa doesn't wear it either. We have headphones, but they're always in the truck. Obviously I had to convince him that no, he was fine. Everybody has to wear them, not just him, he's not in trouble. I am much more aggressive about screening for that when I am talking with families.

[Dr. Ashley Agan]
Yes, it's definitely important. When we've had these newborns who are coming in and diagnosed with some congenital hearing loss, what happens next? What's the next step of the workup? Do they need a sedated ABR or a natural sleeping ABR to really confirm. Do you have a preference for that?

[Dr. Rachel St. John]
Yes, I think in terms of the ABR, basically a good quality ABR requires a still person. We can actually do napping ABRs in older people too, if they're cooperative. I think in terms of preference between napping and sedated, I think Dr. Kutz will speak to this as well, but my preference for the child is always if we can get it napping, great. For infants who are poor nappers, even if they do sleep, sometimes the quality of the sleep is fairly light and then you don't get a great ABR.

I think it's whatever gets you the best information. If we have a kid that you sleep deprive and you give them a bottle and they just conk out and they're a great sleeper, you're probably going to get a good quality ABR. Then there's some kids who just fight that eternally and you might end up needing a sedated ABR to get a decent evaluation on them.

[Dr. Walter Kutz]
Yes, I agree with that. I also really listen to the audiologist, I think they're-- Especially for ABR and oftentimes when I'm seeing the child, we're talking about a cochlear implant and it's very important to establish hearing. Oftentimes these kids will have otitis media, so they may need tubes anyway to get a really accurate ABR. We may take them to the operating room, place tubes and then obtain an ABR. The thing is an ABR takes 30-45 minutes in the operating room. There's some concern with children being under anesthesia for a long length of time. I do agree with Dr. St. John, trying to get a napping ABR is definitely preferable. I think we definitely want to have good information if we're going to progress to a cochlear implant.

(3) The Role of Severity & Symmetry in Diagnosing Pediatric Hearing Loss

[Dr. Gopi Shah]
Speaking to that, do you feel like the degree of hearing loss changes your routine workup ever?

[Dr. Walter Kutz]
That's a great question.

[Dr. Gopi Shah]
Are you less aggressive with mild bilateral or mild unilateral than you would be in moderate or profound bilateral?

[Dr. Rachel St. John]
This is interesting. I'm going to be interested to see what Walter says, because I don't, I feel like some of this is based on the patient population you see. For me, in general, I do the same thing, same kind of algorithm for unilateral and bilateral. I think in terms of being aggressive, the timeline might change. For example, if we have somebody who gets an ABR and there's no response and they're a cochlear implant candidate, we're going to really make sure that imaging is part of that. Because we've got anywhere from 6 months to 12 months until the time of implant. Imaging is going to be a really important part of that.

Whereas if I have a baby who is mild to moderate, imaging is probably going to be part of that process, but I'm not necessarily going to feel the need to get a sedated MRI of the internal auditory canals right away, unless it's going to change management. Because I tend to default to genetic testing first in kids who have bilateral sensorineural. The latest data from CDC shows that that's 50 to 60% of babies who are born deaf or hard of hearing, it's going to be genetic because it's often non-syndromic and autosomal recessive. You might not see any family history, even though it's part of the thing that we ask about.

I'm going to go to comprehensive genetic testing first, because that might obviate the need for anything else. If I find out it's connexin 26 and that kid is going to do very well with bilateral hearing aids. I'm not going to image them because that's a gap junction issue, that's a histopathology issue. That's not structural. I would fully expect to see a typical MRI. I think the order in which you do them and the timeline in which you do them changes for me, depending on the degree. Ultimately I'm going to address the same things.

I'm interested to hear what Walt says, because I see patients on a little bit different timeline and in a little different span than he does, so that might change depending on who you're working with.

[Dr. Walter Kutz]
I follow pretty similar decision-making, and every patient's different, right? Some may be premature. They may have family history. I do agree if they have an abnormal connexin test, they're unlikely going to have an abnormal MRI. Of course, if they have severe or profound hearing loss, you're going to do a cochlear implant and you're going to want to do some imaging for preoperative planning. If a child has symmetric, mild to moderate sensory hearing loss, I don't think they necessarily need imaging. I think if there's asymmetric hearing loss that's significant, that's when I would definitely lean towards imaging.

I do agree, in most cases if they have to have abnormality, it's going to be something like-- You're not going to see anything on imaging or they may have an absent cochlear nerve on one side. I do recall a recent case of I think a 12-year-old or 13-year-old that had some asymmetric hearing, retained an MRI and actually had a glioblastoma along the auditory pathway, which is very, very rare. You don't want to base all your decisions off of one case, but we did order the MRI because of the asymmetry of the hearing loss. I think mild to moderate, symmetric, child's doing okay with hearing aids, I probably wouldn't image them, but to carefully follow their hearing.

[Dr. Rachel St. John]
Yes. I think the asymmetry piece is really big. That's something that I always talk about with residents when I'm teaching is, especially if it's late onset, but regardless, it's like, if you've got an asymmetric presentation, you do think more structural and you want to rule out the bad stuff. Especially, and I think this is something you guys are going to get to, but if you have a mixed presentation, one of the things that always starts percolating in my mind is enlarged vestibular aqueduct, because we see a fair amount of it. I think the point about asymmetry is pretty critical to keep in mind.

(4) CT vs. MRI for Evaluating Mixed Hearing Loss

[Dr. Ashley Agan]
Speaking of mixed, and I feel like I asked you this, Rachel, all the time in the clinic when I have like a mixed result, do you like MRI? Do you like CT? How do you decide which scan to get? To me, my rule of thumb is sensorineural, I tend to get MRI just because you see the nerve better. Asymmetry, I like MRI because of sensorineural, make sure there's no tumor, conduct a temporal bone, better bone anatomy and a CT. For mixed, I'm never quite sure which one to get.

[Dr. Rachel St. John]
I call Dr. Kutz and I'm like, "What would you do?" This is where I would really punt to the otologists, although I think I've read a lot of different things in the literature. I've read that CT is adequate for evaluating EVAS, and then MRI is superior for evaluating EVAS. Then I've talked to people who said, I prefer CT or I prefer MRI, and I feel like it's shown up on both studies. I'd love to hear sort of the otology take on that.

[Dr. Walter Kutz]
I think probably the way-- Typically on mixed loss, I think I'd lean towards a CT temporal bone. You're going to most likely see EVA, you can measure the aqueduct pretty easily and see the posterior canal. There's different ways of determining if it's truly an enlarged visceral aqueduct. Then, if you're going to look at acicular abnormalities, you can see the shape of the cochlea. You're not getting MRI. MRI is beneficial if you look at the cochlear nerve status, you know is it hypoplastic or absent, you're looking at inner ear abnormalities, but you can see that on CT scan.

The disadvantage of a CT scan, of course, is there's going to be some radiation involved and it's not negligible with a CT temporal bone. There's a fair amount of radiation. Again, if it's asymmetric or single-sided mixed hearing loss, the kid's doing okay, you can do that CT scan. We don't have to sedate them, or when they're older, to get them out of that young age where the CT scan and the radiation may have more problems in the future. I think I would lean toward a CT scan for mixed hearing loss in most cases.

[Dr. Rachel St. John]
That's great for me to hear.

[Dr. Ashley Agan]
If you're going to wait, like let's say you have like a two-year-old or three-year-old, and you're like, we'll wait to do a CT scan and we'll wait until they're a little older to where they don't need sedation. How long would you wait and how would you follow them while you're waiting on that?

[Dr. Walter Kutz]
You mean for mixed hearing loss?

[Dr. Ashley Agan]
Yes.

[Dr. Walter Kutz]
If it's unilateral, I'm not really worried about anything that's going to harm them. I'm not worried about a tumor or anything of that sort. I think you could wait as long as you-- Until you have to have sedation. A CT scan takes about a minute or two. It's pretty quick. Unlike an MRI where it's 30 minutes plus, and then they have to probably be under anesthesia until maybe, three, four, five years of age, depending on the child, maybe even older. You can also get atresia, the same sort of thing. I think atresia is going to cause hearing loss, is going to present with hearing loss and need imaging.

I wouldn't image them necessarily when they're very young. I'd wait till they're probably three or four years of age, they get a CT scan. I don't think you need sedation so fast for most kids, and you're just rolling out a cholesteatoma at that point anyway. That's my thought. If I'm worried about something, if I'm that they have surgery for a cochlear implant, or if I'm worried about something that's going to harm them, then I think you need to push the imaging up. Otherwise, I think you can wait and just check that box at some point and make sure you have the diagnosis.

[Dr. Rachel St. John]
I always tend to go with things that are going to change my management. I talk about that with families too, so that they know what to expect, because, sometimes we have families who just want to know. Then when we talk about the fact that we're not necessarily going to do anything with that information, they actually feel a little differently about waiting a little bit until that child is older and either can handle sedation better or potentially not need it.

I always let them know that should something change, that's something that-- If I have a child who has had, he was born unilateral and then the other ear becomes involved, I'm probably going to want to image that child. If they have really significant progressive, is there-- Because EVAS doesn't have to present as a mix. I've seen it present as predominantly sensorineural. If there is something there that we might do something differently about, I will always push imaging. I think it's important to look at that and say, am I getting this because I want to feel better having gotten it or the family does, or am I actually going to do something with that information?

[Dr. Walter Kutz]
Great point.

[Dr. Gopi Shah]
For your follow-up, are you doing, every few months, every six months?

[Dr. Rachel St. John]
Yes. Depending on the family. I tend to follow patients annually, minimum. Then if things are shifting, or if we end up with some transitional challenge, like we're changing, we're entering public school or we're shifting from elementary to junior high and the format for learning changes dramatically. If parents are really anxious, sometimes I'll do something sooner. I just base it on what the family needs.

[Dr. Gopi Shah]
Yes. I always worry about people and patients falling through the cracks, so if the plan is, okay, we're going to wait a few years to get the imaging, because it's not going to change anything right now. I just worry that, life happens, and then a few years will pass and then it will just have been dropped or whatnot. I probably have patients follow up too much. I feel like I would rather them follow up too much than not enough. Sometimes there's some social visits in there just to check in.

[Dr. Rachel St. John]
Those can be very valuable.

(5) Managing Pediatric Hearing Loss in the Context of Virtual Learning & Telehealth

[Dr. Walter Kutz]
Even with telehealth, I haven't done that as much in my pediatric practice, but that's helpful just to touch base. It's convenient. At this point, I don't know if the online audiograms are all that great for, especially for kids, but it may be an easy way just to touch base, hey, is school going okay? Any problems with speech and language? Then, that'd be an easy way of doing that more frequently, because I think it's a good point, we do lose patients to follow up and then worry what happened.

[Dr. Gopi Shah]
I was just going to say, I find that with virtual school that has started in the last year, these kids that have had baseline hearing loss are newly identified, because of the virtual and the ability to control the volume on the computer or the iPad. These kids are actually doing well. I actually recently had a new identified moderate sensory or hearing loss in a 13-year-old girl. Her hearing started to change. She noticed it changed a year ago, didn't really say anything in school. She's been an A student in the past 12 months because she's been virtual learning. The classroom hasn't affected it and she skated by until she failed a hearing screen at her pediatrician's office. It's interesting how some of the changes have also-- Maybe have actually been beneficial to some of these kids.

[Dr. Rachel St. John]
That's a very interesting point, because our counseling has changed dramatically with online learning. There's all sorts of accommodations that you can talk about for captioning, for streaming Bluetooth through your amplification. There's all sorts of things we can do at home. It's been interesting, because there's been two very distinct camps of these kids. There's a, I would argue it's a smaller group of kids who have done better, but it's for the reasons exactly that you said, Gopi.

You've modified and almost eliminated competing background noise, so there's nobody whispering next to you in class. There's nothing going on. You can really contain the sound and you can control the volume and some of these kids do much better. Then there's a lot of these kids, the lack of structure that they had with in-person learning, it's really rough. It's been really tough for these kids. They've had wildly different experiences. I think it just all goes back to the fact that it really depends on the kid that you're talking to.

[Dr. Gopi Shah]
Absolutely. Just personally having younger kids, first grade and third grade, when it's virtual at home, that lack of structure is difficult and my kids are fortunately hearing, they don't have any hearing concerns, but.

[Dr. Rachel St. John]
No, it's been, I think, fairly brutal across the board with that lack of-- you can't replicate that at home. It's just hearing is just one more level that you're working with.

(6) Auditory Neuropathy in Pediatric Patients

[Dr. Gopi Shah]
All right. Can we talk a little bit about the auditory neuropathy kids? I would love to-- If you could just explain that to us, and then are these kids different in your mind?

[Dr. Rachel St. John]
We're like looking at each other, like who wants to take this first? It's the Pandora's box for sure. I think it's a great thing to talk about. Walter, do you want to start with that one?

[Dr. Walter Kutz]
Yes, I can. Oftentimes these kids will come over because they're potentially a cochlear implant candidate. Like Dr. St. John said, they're going to have oftentimes intact acoustic reflexes. They're going to have abnormal ABR. Oftentimes the ABR will show a mirroring when you change the rarefaction condensation, they'll mirror each other. There's this distinct pattern, for classic auditory neuropathy that's seen. I think an important thing to remember is that sometimes kids can be born with auditory neuropathy and then it'll actually improve.

These kids we'll definitely go a little bit slower if we're going to consider cochlear implants and they're very carefully followed by audiology, and frankly the audiologists are experts at this. I really listen to their interpretation of how their child's doing as a team and we'll talk about this later, we get together and decide, is a cochlear implant in the best interest of this child. I think auditory neuropathy definitely makes it more interesting and challenging, for those kids, especially.

[Dr. Rachel St. John]
I agree. I think from a parent expectation point of view, this is probably one of the most challenging things that comes through my clinic. Because by nature, AN is very idiosyncratic. Every child looks a little different in terms of how they're-- It's cortical level hearing processing. The sound gets to the brain, but then there's-- It's not always processed the way that we would expect. Not only do these kids look different from each other, so you might have one kid who doesn't benefit from hearing aids and one that really does and their behavioral thresholds look fairly similar, but these kids can actually look different from day to day.

Parents will classically describe having good hearing days and bad hearing days and that is the absolute nature of AN. It's its own thing. I think in terms of counseling parents of what to expect, that's much more difficult, because we don't always know what to expect. We don't know if the character of that's going to change, as Dr. Kutz mentioned. If you have AN and you get cortical maturation, you may see that waveform change around 15 or 18 months of age. Whereas children who have like a really significant brain insult, like there are kids with really severe hypoxic-ischemic encephalopathy, that's sound processing at a cortical level that's not happening. That's not going to improve because the insult is what it is, and then it's, for lack of a better word, fairly static after that. What parents expect is tough.

Then I think what happens is parents sometimes get very mixed messages from different providers. That has been one of the bigger challenges for me in working with these families. I had a family, there was an audiologist, it was an outside audiologist who said, "Oh, well, your kid needs a cochlear implant because those kids always do better with a cochlear implant." While we know that sometimes the quality of the input with a cochlear implant might be better than what they get through hearing aids, this is a kid whose behavioral thresholds were testing in the mild to typical range. We just knew that the message wasn't the standard message. They still had that AN waveform. That's not somebody who would even qualify for a cochlear implant.

It's some of those blanket statements. It's like, oh, if you have AN, then you do this. I think this is the absolute poster child for making sure that you are looking at that individual child and that individual family, because the medical advice changes, for sure.

[Dr. Gopi Shah]
Do you refer these kids for hearing aid evaluation? What are their resources or options? What counseling do you, or resources can you provide these patients?

[Dr. Rachel St. John]
Absolutely. These kids often will benefit from hearing aids. It's just a question of, is the quality helpful for them? Some of these kids, it is, and some of them, they're not. In talking with our audiologists, the goal is always to-- For anybody who's even considering a cochlear implant, we always see benefit to hearing aids first. That's part of the picture. For these kids in particular, it's really important to see how they do with hearing aids. One of the things that the audiologists and I have talked about a lot is that if they're going to program, they really need behavioral thresholds. You can't really get good threshold information off an ABR when you have auditory neuropathy. You can't state for sure, this is the level of hearing that I'm testing today. That really has to come from a behavioral evaluation.

Those kids necessarily have to be a little bit older and they have to be able to get some behavioral information and then program based on response. I think that our pediatric audiologists are the absolute best allies in this because they are really trained to get some really subtle information from kids about what they're doing with that acoustic information. I lean on them very heavily in collaborating with them with these patients in particular.

[Dr. Gopi Shah]
The diagnosis of auditory neuropathy is made based on the pattern seen on the ABR. Is that correct? Okay, and so you could have a patient that has a pretty good or mild, moderate looking behavioral audiogram, but they have auditory neuropathy and maybe it has or has not been diagnosed if they haven't had that ABR that shows that pattern. Is that correct?

[Dr. Rachel St. John]
Yes, as far as I am aware, you're not going to be-- I would punt to audiology on this one unless Dr. Kutz has some other input, but you're not--. Kids who are getting behavioral testing, there's no mechanism for testing for auditory neuropathy. That's my understanding, it's based on the ABR pattern that we see.

[Dr. Gopi Shah]
You have to have that suspicion to test for it, to get the ABR, you know what I'm saying? If you have a kid that their behavioral testing looks pretty good, but yet there's report of problems or hearing loss, someone has to be like, oh, maybe this is auditory neuropathy and we should get an ABR, or no, am I thinking of this wrong?

[Dr. Rachel St. John]
I think the way it bears out practically, it's the opposite, which is you have kids who, based on either the fact that they're a baby and they can't behave early test, or they have some condition, HIE, seizure disorder, microcephaly. Something that's going to potentially cause AN situation, kernicterus. Those kids also often can't condition if they're older to behavioral testing. By default, you end up with an ABR to get accurate information. I don't know, Walter, if you've had a different experience with this, but most of the time, the ABR information comes necessarily with these kids, because it's really the only mechanism that we've had to get a full evaluation on them.

[Dr. Gopi Shah]
That makes sense.

[Dr. Walter Kutz]
Yes, and I agree with that. Usually they've undergone ABR for-- there's always a suspicion or they can't do behavioral testing, and that's where the auditory neuropathy. On the adult side, you may see-- I don't see it that commonly on the adult side or older children, but it can happen, delayed Charcot-Marie-Tooth or some other conditions that can cause auditory neuropathy. These patients will come in and their pure tone testing is pretty good, but their speech discrimination scores do not correspond at all. Then we'd send them for an ABR. We may detect auditory neuropathy in the older patient population, but it's just not that common. I do think they typically come in with the ABR for other reasons.

(7) Pediatric Hearing Loss Due to CMV

[Dr. Ashley Agan]
That makes sense. Then just switching to some of the other groups of kids that we might see, CMV comes to mind, EVA comes to mind, history of meningitis comes to mind. How do those different groups, or I think of it, I always put them in little buckets. How does your thought process work up management for, we can start with CMV change, or how is it different? Versus the meningitis patient who has had meningitis at four months of age?

[Dr. Rachel St. John]
Walter, I'll take CMV if you want to take meningitis. You want to divvy it up? We can do a tag team.

[Dr. Walter Kutz]
That's a fair deal, I'll take that.

[Dr. Rachel St. John]
Walter is like sold. Also, I think Dr. Kutz is probably a really good person to speak on meningitis because the timeline for intervention is pretty unique for that, so he's the guy. CMV is tough for a number of reasons. Congenital cytomegalovirus, it's every place in the United States right now, in all of our 50 states, because we handle things at the state level, it's all being handled differently. A lot of it depends on what's been put in front of the legislature based on what's been happening.

The thing about CMV that's so tough. If it's the neurologic presentation for CMV, all depends on when the mother contracted CMV during her pregnancy. If it's an early, like first trimester infection and she did not have native antibodies, if it's the first time she's seen CMV, that baby will usually come out. People will mostly know that the baby likely has CMV. They're small, they have problems with clotting, they have terrible problems with jaundice. They often have vision, hearing. There's a whole host of symptoms that go along with that.

The tricky part is the later, the closer to the third trimester infection, because while those babies sometimes are born deaf or hard of hearing, it is much more common for that to be a delayed onset phenomenon. That's where screening gets tricky. UT Southwestern, very fortunately, was part of this study back in 2007 called the CHIME study. I think there were six or five or seven centers, but we were one of them. Where basically from 2007 to 2012, so it was a five-year period, every child that did not pass a newborn hearing screening in our hospital system, and that included Parkland, got a PCR test for CMV at birth. That was almost 100,000 kids by the end of the study. It was 999 something.

They looked at all those kids who didn't pass their newborn hearing screening and saw how many of them had congenital CMV and it was an extremely low number. It was 0.4%. It's translated to 400 some kids out of 100,000. The problem with that is, first of all, you only catch the kids who are presenting as deaf or hard of hearing. It caught about, it was close to 60% of them, but the other 40% are kids who had CMV and they were delayed onset. The newborn hearing screening wasn't your pathway to figuring out who those kids are.

The much bigger problem is that CMV is ubiquitous in the community. If you look at Karen Fowler's data, she's an ID professor at University of Alabama, and she has done a ton of publications on congenital CMV. If you look at her data and you look at the CDC data, it's around 8 to 12%. If you split the difference, 10% of kids who are completely asymptomatic who have CMV go on to develop delayed onset hearing loss. If you look at the number of those kids, if you look at the incidence of CMV in the general community, it's one in 200, it's really high.

In the state of Texas, we're talking about roughly 400 kids a year that would be born who passed their newborn hearing screening and will go on to develop late onset hearing loss. Anything we do in the newborn period to try to identify that with newborn hearing screening, we're not going to catch it. It's a late onset phenomenon for these kids who are born and don't have other symptomatology of CMV. It all rests on suspicion and following in the medical home. If you know these kids are CMV positive at birth, you're going to look at them a little more closely, but it's really hard.

The delayed onset stuff is really tough, especially in the sort of toddler pre-K group, because really you're looking for concern about responsiveness and lack of expressive language delay. That can be not picked up. That can be really tricky. It can be minimized. It can be overlooked. It can be denied. CMV is challenging, but if we know about it in the newborn period and they're symptomatic, that can change our management. Some of those kids will qualify for ganciclovir treatment, which has-- And I'm not a super guru with the ganciclovir studies, but there's been enough data to show that can ameliorate some of the progression with the hearing.

We've definitely had patients who have been identified early and have gotten ganciclovir therapy, and have gone on with frequent testing to see how that has affected their hearing.

[Dr. Gopi Shah]
Does that identification happen because they failed their newborn hearing screen and so then they get the blood test?

[Dr. Rachel St. John]
It only happened in the context of the CHIMES program. Universal CMV screening is almost nowhere, because it's a huge undertaking in terms of money and then linking that to newborn hearing screening. Targeted testing, which is what the CHIMES program did, I think philosophically we love it, but when you look at the data, it caught 0.4% of kids who didn't pass their newborn hearing screening. That's not winning over people who are planning budgets. That's not something that's making people run to fund newborn hearing screening protocols and testing for CMV in those patient populations. The American Academy of Pediatrics just last month established a CMV committee that I have joined because for just this reason, it is really tricky. It's tough to get funding, but we know what the longitudinal implications are. We're all very passionate about it. It's just figuring out realistically how to make that happen in a coordinated way. It's very challenging.

[Dr. Ashley Agan]
Is the hearing screen for this progressive onset, has baby passed his newborn hearing screen, but there was CMV in the history. Are you getting behavioral audios, ABRs? Is this something they can just do in the pediatrician's office? Do they need a pediatric audiologist? I guess, what's the timeline and how often and how are we checking their hearing to test for progressive hearing loss?

[Dr. Rachel St. John]
I believe the way we handled it through the CHIMES program is that those children were followed up with audiology every three to six months to see, at least in those first three years of development. I think it was a combination of both screening and testing just to get as much data. If a kid had an atypical screen and they were too young for behavioral testing, that would buy them an ABR. If they were old enough to behavioral test, they would try to get as much data there.

They were followed very closely by audiology for several years. I think the average age of hearing loss in this patient population for kids who have delayed onset is between two and two and a half years old. That's a tough time. Also, language-wise, what's going on there? The kids are frustrated because they don't have enough language if they're hearing and there's so much development that's happening. They were followed very closely for several years as part of that study.

Certainly, if we have kids and we know that they had congenital CMV, I tend to make sure those kids are plugged in with audiology pretty frequently. If they go to audiology and they'll always write their recommendations, it's always come back within three months, six months. It's never, "Oh, we'll see you next year," because that often is a moving target.

[Dr. Ashley Agan]
In terms of screening, are we talking about behavioral sound field, OAEs, and temps, or hey, I can't get behavioral sound field because I can't get the baby to condition at 10 months? I have normal temps and OAEs, that's sufficient? Are we talking now, hey, we might need an ABR and this might need to be under anesthesia because the baby doesn't nap anymore. What's the minimum amount of screening do you feel for the CMV babies is adequate?

[Dr. Rachel St. John]
That's tough. I think that part of it is you have to look at your individual kid. If you've got a kid who has signs of developmental delay, I'm not going to be messing around with acoustic reflexes. I'm going to want full ear-specific data on that child because that's going to affect, are we using hearing aids, what education plan, what kind of ECI support, you want to know.

For kids who are asymptomatic, we happen to know that they have congenital CMV and they have typical OAEs, they're seeing them and they're doing screening, and that kid is developing normal language, that would probably be a little less likely to push into a sedated ABR just to know what was happening. Again, for those kids that are-- in my clinic, it doesn't matter if we're talking about CMV or not. If you've got expressive language delay, I need your specific information.

I think when we've had kids who have had sound field testing for three years and haven't been able to condition and they've got language delay, I'm like, "That kid needs an ABR. We need to know what we're working with. We're only getting information on the better hearing ear."

[Dr. Ashley Agan]
We can't miss a lot of it.

[Dr. Rachel St. John]
No.

(8) Pediatric Hearing Loss Due to Bacterial Meningitis

[Dr. Ashley Agan]
It's already been missed. That's super helpful. Walt, let's talk about the meningitis picture, the child with meningitis. Tell us, with meningitis, the infection can lead to hearing loss. While they're in the hospital, many times they'll get an audiogram and you might have a child that has moderate to severe bilateral hearing loss. What are the next steps? When do you see these kids and what is your thought process in terms of if we are going to need to think towards a cochlear implant?

[Dr. Walter Kutz]
Bacterial meningitis can certainly cause severe-to-profound hearing loss. Viral meningitis typically does not do this. It's going to be bacterial. The kid's going to be very sick in the hospital. Oftentimes, the first goal is to make sure the child survives the infection, and doesn't have any neurological complications. Oftentimes, these kids are really sick. Once they're stabilized and we recommend testing, a lot of times that may have to be an ABR because they're not going to be neurologically responsive.

It's really important to identify hearing loss very early because they can develop labyrinthitis ossificans, which is where the cochlea can ossify. This can happen within weeks very quickly, within two or three weeks even. If we identify a child with bacterial meningitis that has severe-to-profound hearing loss, we'll try to place a cochlear implant as soon as possible that week or the next week if we can.

The problem is if it's delayed and they've had meningitis, they develop labyrinthitis ossificans, placing a cochlear implant can be very difficult. As a matter of fact, you may even have to drill out the cochlea and just lay the electrodes into what's left of the cochlea after doing that. Those results are definitely inferior to what you would get by placing a cochlear implant before ossification. There's definitely a timeline that's critical for a patient with bacterial meningitis.

[Dr. Ashley Agan]
Is that common for patients to get labyrinthitis ossificans? Is that happening to most meningitis patients?

[Dr. Walter Kutz]
No, that's not most patients. That's typically the really sick patients, but even patients that they're recovering from bacterial meningitis that maybe weren't as sick as others, they definitely need testing quickly. They need to be followed at probably regular intervals. Typically, that may be every four weeks for a while, especially if there's a mild hearing loss or moderate hearing loss after bacterial meningitis, you want to follow those kids very carefully to make sure their hearing loss isn't progressing.

We don't see it all that often. I would say once or twice a year, if even that, we have a child we do a cochlear implant on because of bacterial meningitis. Obviously, at Children's Medical Center, there's many kids that have bacterial meningitis. It's not that common, but it's something we should be aware of.

[Dr. Ashley Agan]
Got you.

(9) Cochlear Implants

[Dr. Gopi Shah]
I think that's probably a good segue into talking about cochlear implants. Dr. Kutz, we wanted to get into, what is the pathway? What does the evaluation look like for these patients who are born with congenital hearing loss and who will ultimately end up getting a cochlear implant?

[Dr. Walter Kutz] We've set up a really nice multidisciplinary team, which I think is essential, especially for children. They'll typically come in, they'll fill their newborn hearing screen, or they may come in later because of CMV or some other progressive hearing loss. I would say most of our kids come in, they're six months of age, been identified, or even three months of age. Then we typically have two meetings.

It's a multidisciplinary clinic. They'll come in, they'll meet the otologist, which would be myself or three other of the otologists in the practice. We always have the patient see Dr. St. John because of her input. I think it's a good checks and balances to make sure we've done all the testing. We want to do imaging. Typically, we get an MRI. The only time I get a CT scan is if there's some inner ear abnormalities. I'm worried about a facial nerve or something of that sort.

Then we'll have them see ophthalmology, do genetic testing, we'll get an EKG, and that's going to be our typical workup. I think by having seen Dr. St. John in our clinic a couple of times, we make sure to get all that covered.

We're also very careful these kids get vaccinated. They need to have their Prevnar 13, which is part of the normal vaccines until two years of age. When they're two years of age, they'll get a Pneumovax as well because with a cochlear implant, these kids have a higher risk of meningitis. Actually, Dr. St. John started a program this year, a quality improvement initiative that we're proud to say with her leadership, we made sure 100% of kids had their proper vaccination over the past year, which is, it's a big undertaking. It's easy to miss that, but--

[Dr. Rachel St. John]
Congratulations. That's huge. You're just, you're being nice because these guys were so tolerant of me just hounding them by email every couple of weeks when I was running the data, they were awesome. It was a Herculean team effort for sure.

[Dr. Walter Kutz]
That's right, it is important. You just feel terrible if one of your patients got meningitis and aren't probably vaccinated. It's extremely important. Anyway, there's a lot that goes into that. Then at the meeting, it's going to be an otologist, a cochlear implant audiologist, we have a psychologist to meet with the family, a social worker. The team will generally meet with the patient twice.

One, we want to make sure the patient is a good cochlear implant candidate. We want to make sure they're going to-- The real work of the cochlear implant journey starts after the surgery. That's when the kids have to wear the implant, the parents have to be supportive. A lot of these young kids are just saying, "I don't want to wear it." That first week or two, the parent has to make sure the child's wearing the cochlear implant all the time.

We want to make sure they understand some of those issues. There could be behavioral issues, a child could have autism, they may have some other severe neurologic problems that may not be a great cochlear implant, they're just not going to be able to do anything with that input. It can be very complex decision-making. That's how we do it. Two clinic visits with our multidisciplinary team, and it seems to work pretty well.

[Dr. Gopi Shah]
What's the average age of implant for most patients?

[Dr. Walter Kutz]
We try to get them implanted by one year of age if they're Medicaid. If they have private insurance, we'll try to get them even sooner, 9 to 12 months of age. That doesn't always happen, unfortunately. There's some socioeconomic issues that come up, but we do the best we can. The earlier you can implant a child with severe-to-profound bilateral hearing loss, the better they're going to do.

Once they get over three years of age, I think their potential for being an excellent cochlear implant user really starts to decline. Average age, hopefully, we implant them by one year of age. I'm sure it's a little older than that, but that's what we shoot for.

[Dr. Rachel St. John]
One thing we've been doing with CI team, which I have found incredibly helpful, and I hope Dr. Kutz has as well, is that we do a monthly multidisciplinary team meeting by-- well, now by Zoom with COVID, which is I think made it a lot easier, honestly, in terms of scheduling. That's where we actually look at cases that are particularly more complicated, whether it's ethical, psychosocial, medical, all of the above.

We've had a case of a child who had unilateral sensorineural hearing loss. She actually had access to language, but she was deaf in one ear, and mom was really looking for a cochlear implant, and helping her understand that that wasn't going to change that child's access to language because she had the one ear that typically, that wasn't necessarily going to change the child's trajectory.

Having a multidisciplinary meeting where we all reviewed the case and reviewed what had been done and reviewed what mom had been coming to the team with, was really helpful, or families that have been declined elsewhere and are seeking a second opinion. I think I found that to be incredibly valuable, both in helping families with appropriate expectations. Then also, sometimes we will talk about things and say, "Okay, do we have a policy when we have a particular situation like this? How do we tend to handle it? What's our database to norm?" Sometimes it's easy and sometimes it's stuff we haven't seen before. It's a first time. I think that's been a really helpful part of the CI multidisciplinary process.

[Dr. Gopi Shah]
That's amazing. From the patient side of it, I think I would be very excited to know that so many people are thinking about my case and trying to help make the best decision. I think that's awesome. Rachel, for your part of it, I assume when they're seeing you during that first year before they're implanted or before this decision has been made that that's a good recommendation, what kinds of things are happening when they see you? Is there a role for any other types of hearing aid?

[Dr. Rachel St. John]
Hearing anything?

[Dr. Gopi Shah]
Yes. What's happening in your clinic? Sign language.

[Dr. Rachel St. John]
All of it. It's important. I think the way that I have always approached my piece, my role with the cochlear implant team is helping parents navigate that process and helping them understand what's coming next, what should they expect, what should they not expect. A big part of my talk as they're in processes, cochlear implant team is, what do cochlear implants mean for children? Helping parents understand because there's a lot of stuff that parents look at on YouTube, and it's cold.

I'm just going to give you an example. When a cochlear implant is activated, so Dr. Kutz puts an implant in a child, they heal up for two to four weeks, and then the audiologist turns it on. Then there's this series of programming, mapping that we do to gradually increase the programming and match it to what that child needs. A child who's, say, 12 months old, who's been deaf since birth, who's never heard any sound input whatsoever, the moment you give them sound, that can be a very interesting moment for a child.

I think it's very important for families to know that it can look just like YouTube where they turn it on and the baby lights up like a Christmas tree and everybody cries and laughs and claps. It's great. It can also look, the baby can be absolutely petrified and break down or just get really quiet and scared. Those are all typical responses. They are all expected. That first time that a baby hears sound, we don't know what they're going to do.

If parents don't know that and they have watched all the pretty YouTube-- because the babies crying when the cochlear implant is turned on, those don't make it to YouTube. You are not going to find those, but they're very important. That's a very normal expected response for a child who's experiencing something completely new for the first time. For families to know that it's okay if the baby freaks out a little bit, that little thing is so important.

I've had parents come to me with cochlear implants in a bag right after they've been activated and said the baby gets so upset. I'm like, "Okay, let's put one on and we're going to cry. It's going to happen. Then we're going to just keep talking, and then we're going to let the baby get used to it. Then we're going to be okay. Then we'll put the other one on. The baby's probably going to cry again." There's a process there.

I think a lot of what I do is helping families understand what is the range of outcomes. Cochlear implants have never been better. The technology has never been better. The surgical technique is continuing to evolve. It doesn't mean it's going to be hearing like the parents have. We know that. It's not a bionic ear. We might get there one day, but we're not there yet. Understanding that children are all unique, and so there's a range of potential outcomes.

We're shooting for access to listening and spoken language. Depending on the risk factors involved, we expect to get there, but we also have to be prepared for the fact that that may not happen. It's not to be doom and gloom with families, but just to make sure that they don't have this expectation that we're going to put this thing on and we're going to turn it on and everything's fine.

When we talked about this on the last episode, you have a deaf child and they will always be a deaf child no matter what we do. Helping families embrace that and helping their child embrace that, that's my job. Getting deaf role models involved, hooking parents up with family support programs like Texas Hands & Voices, which is just a bunch of other parents with deaf and hard of hearing kids.

Walter and I have been doing this collectively for, I'm guessing, many decades. I'm not the mom of the deaf child. We don't have deaf children. We don't sit in that seat. Sometimes connecting a family with another parent is much more powerful than anything I can tell them. That's my role in the cochlear implant process is helping families understand the range of possibility that's coming with the cochlear, but then also more globally for their child.

(10) Contraindications for Cochlear Implants

[Dr. Gopi Shah]
That's wonderful. I love it. One last thing is if I can end up having to wrap up soon, Dr. Kutz, are there any absolute contraindications for a cochlear implant? I guess if there's no nerve.

[Dr. Walter Kutz]
Interestingly, that's a great question. If there's no cochlea, definitely a contraindication. We have done implants for kids that there's no visible nerve and there's been reports in our study, kids had no visible nerve. They really maybe got some pattern awareness. That could have been more of a sensation than an auditory signal. There have been other reports where some kids have gotten some very limited closed-set speech. That's traditionally been a contraindication is a totally absent nerve on imaging.

If there's an internal ear canal, there's a cochlea, there's a chance cochlear fibers could be traveling on the vestibular nerve or even the facial nerve. I think that's a little bit of the discretion of the surgeon in the cochlear implant team. If a patient has no cochlea, then there's nowhere to place the cochlear implant. Then we can get into the whole other episode about the brainstem implants for kids, which we won't get into that, but that's a possibility.

[Dr. Ashley Agan]
I think we got another episode coming up.

[Dr. Walter Kutz]
That's a tough one. No one's paying for that right now. That's pretty tough. Otherwise, I do think it's important and it's why we have the multidisciplinary team. Dr. St. John is such a critical part of that team. If a child has other severe neurologic disorders, we have to decide what are our expectations. You can never guess that 100%. We don't have the crystal ball we'd like to have.

You really do need to talk to parents. "Listen, we're putting your child under a two-hour general anesthetic with a $30,000 implant. That does have risks, although they're low risk for surgery. There's a risk of meningitis and other problems. As a team, we think the benefit is going to be extremely limited." It's always, in my opinion, very difficult to have that conversation and to be like, "It may be best to look at alternative ways of communication besides a cochlear implant for your child." Of course, that's a discussion between the family and the team.

I don't know that's an absolute contraindication, but there are some reasons you may not want to pursue a cochlear implant despite having normal anatomy and these sort of things.

[Dr. Rachel St. John]
I just really quickly would just say, too, that it's one of those things where it's very kid-related, but it's also very parent expectation-related because we've had parents who have said, "Environmental awareness of sound is really important to us and our kid." Maybe that's not the goal that we would all ideally want, but that can actually be really important for a family. It can be important for safety. It can be important for engagement.

Dr. Kutz knows this. I encourage families to pursue multiple avenues of language regardless of what they're doing. Whether they get a cochlear implant or hearing aids or whatever or don't, I always support sign language being involved because there's not really a big downside to bilingualism.

For some kids, that's going to be more critical because we do have to be really honest with families and say, "We're not here to tell you, no, this is not allowed, but here's what we're seeing on imaging and we're seeing with your child. Your kid, it rubs their head on their chair all day long and you can't keep a hearing aid on. A cochlear implant's probably functionally not going to help them a whole lot." We do have to be really honest about that.

I agree with Dr. Kutz, that's a hard discussion, but we've had kids whose parents have elected to get cochlear implants with minimal gain. Sometimes I look at that and go, wow, that's a long way to go to get it. Then this child is just so happy with that input and that little bit of increased awareness and connection with others, even if they're not using it to understand or speak language. I have to give that a lot of respect because that child's existence looks really different, in the same way that sometimes parents say, "That is not worth it to us."

[Dr. Ashley Agan]
Everyone's different.

[Dr. Walter Kutz]
If I can add one quick point about cochlear implants, the cochlear implants have recently been approved for single-sided deafness. We've done a few kids with single-sided deafness. We've done a few kids with single-sided deafness. Again, that's a whole nother episode, but just for the audience to be aware, that is something that's going to be the next-- a few years, I think we'll really sort out when a child may be a good candidate for single-sided deafness cochlear implantation.

Right now, they have to pay approvals for five years of age. Sometimes we'll do things off-label, but I think that's something to be aware of and something that the family should at least know about it. If they want to have more questions answered, I think it'd be important to refer them to a cochlear implant center so they can at least be informed of what the options may be.

[Dr. Rachel St. John]
I think that's going to be huge for our congenital CMV kids because they often will start with unilateral changes, and then progress to include bilateral. That's a group that would benefit from an early unilateral implant. If we know that that other ear potentially may change, that might be really helpful for them. It's a great point.

[Dr. Ashley Agan]
Awesome. I think we've really done a great job of exploring this topic today. Are there any last final thoughts, anything that we've missed that you guys want to leave our listeners with?

[Dr. Walter Kutz]
Just a quick thing, I always think about this. I hear these cases, if a child has bilateral aural atresia or bilateral conductive hearing loss, please make sure they have a bone-conducting hearing aid on as soon as possible. I have unfortunately seen cases where they were never fit because they can't have a hearing aid because they don't have ear canals. That's something I think very important to keep in mind.

With bone-conducting hearing aids, a child will develop normal speech. Without them, they won't. That's something I see sometimes. It's just devastating. Something for the audience to remember.

[Dr. Ashley Agan]
Absolutely.

[Dr. Rachel St. John]
I guess my parting thought, it's probably very similar to what we talked about before, is that for these kids who are deaf and hard of hearing, particularly at birth or very early age, the one in a thousand thing from my perspective in working with families is access to language early. I think Dr. Kutz alluded to this a little earlier when we're talking about when you get into late cochlear implants and your language outcomes tend to go down, it's a use it or lose it.

That first year of life is a very critical year of life in terms of brain development and pruning and making sure that these kids all have access to language regardless of mode, regardless of whether it's spoken, sign, protactile, whatever you're doing, depending on the child, not sitting around and waiting for language, waiting for something to happen, waiting for the next piece of technology or intervention or resource or person who's going to work with them, is I think really critical. Once that ship sailed, it does not come back.

[Dr. Gopi Shah]
Thank you both for spending this time with us. For our listeners, you can find us at BackTable ENT on Apple, SoundCloud, Spotify, iHeartRadio. Reach out to me or Ash. We'd love your feedback, your input. If you want to come on the show, topics.

[Dr. Ashley Agan]
If you're digging on Dr. Kutz and Dr. St. John, you can check out their episodes, episode 4 and episode 10. As far as social media, I know Dr. Kutz, you're Earduct1 on Twitter. Is that right?

[Dr. Walter Kutz]
That's right.

[Dr. Ashley Agan]
Rachel, you're not tweeting, right?

[Dr. Rachel St. John]
Still blissfully free of social media platforms.

[Dr. Walter Kutz]
You didn't get blocked, did you? You weren't blocked from Twitter, right?

[Dr. Rachel St. John]
Oh, all the time. No. I've never delved into the Twitterverse. I'm just sitting over here with my phone doing whatever.

[Dr. Ashley Agan]
If you want to connect with either of them, you can always reach out to us and we can get you guys connected to them. They're both great resources, but we appreciate our listeners and we hope you'll subscribe, rate, and share our podcast with anyone you feel might find this topic interesting. That's a wrap. Bye.

[Dr. Rachel St. John]
Thanks, guys.

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. Joe Walter Kutz

Dr. Joe Walter Kutz is a neurotologist and Professor of Otolaryngology and Neurosurgery at the University of Texas Southwestern Medical Center in Dallas, TX.

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, February 16). Ep. 16 – Congenital Hearing Loss [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.