EPISODE 39

[INTRODUCTION]

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[00:00:48] DS: You're listening to On the Ear, an audiology podcast sponsored by SpeechTherapyPD.com. I'm your host, Dr. Dakota Sharp, Au.D, CCC-A, audiologist, clinical professor and lifelong learner.

Well, I primarily work with pediatric cochlear implants and hearing aids. I am absolutely intrigued by the many areas of audiology and communication in general. This podcast aims to explore the science of hearing, balance and communication with a variety of experts in hopes of equipping you to better serve your patients, colleagues and students. Let's go. We are live and On the Ear, brought to you by SpeechTherapyPD.com.

[INTERVIEW]

[0:01:35] DS: When discussing ideologies of hearing loss, we often think of the invisible causes. Things like degradation of cochlear hair cells, otitis media, auditory neuropathy, the list goes on. But for congenital malformations of the outer ear like, microtia and atresia, the cause is oftentimes something we can see. But how do microtia and atresia affect hearing, and what can we do to better provide access to sound for individuals with microtia and atresia? 

Today's guest is here to explain all of that and more. Meredith Berger has been the director of Clarke Schools for Hearing and Speech in New York for 14 years. Clarke is a national non-profit organization that teaches children who are deaf or hard of hearing to listen and talk. Prior to joining Clarke, Meredith was the Deaf and Hard of Hearing Educational Specialist at New York Eye and EarÕs Institute and Cochlear implant Center from 2005 to 2008. Her previous experiences include working as an itinerant teacher and a classroom teacher for students who are deaf or hard of hearing, and as an early intervention provider. 

Meredith received a Bachelor in Education from SUNY at Buffalo State College and a master's, sorry, not a master's, two master's degrees, one in Deaf Education and another in Educational Leadership from Canisius College. In 2017, Meredith began her doctoral studies at Teachers College, Columbia University, to find answers to her own questions on the needs and outcomes of children who are deaf or hard of hearing, particularly those with microtia and atresia and their families. 

In addition to presenting on the educational needs of children with hearing loss, she also has co-authored pieces on the clinic's school relationship and was a recent contributor to the Journal of Early Hearing Detection and Intervention, their monograph on teleintervention. Meredith is on the New York State EHDI Advisory Board and is a member of the Children's Hearing Institute's Medical and Educational Advisory Board. Meredith is also the parent of two amazing children, one of whom has bilateral microtia and atresia. 

Just a couple of financial disclaimers, I'm the host of, On The Year and received compensation from SpeechTherapyPD.com and Ms. Berger received compensation for her contributions to today's presentation. I am so excited to have Meredith Berger here. She is a leader in the realm of microtia and atresia and her work at the Clarke School has been so beneficial to so many kids across our country. Thank you so much for joining me, Meredith. 

[0:03:38] MB: Oh, it's my pleasure to be here. Thank you for having me. 

[0:03:41] DS: Why don't we just get started, every time I have a guest on who has an amazing, amazing is one more, but also prolific career. Listeners are always so curious, what was their career path? We broke down a little bit of that through this, but I'm curious what led you into your current role. 

[0:03:56] MB: I think that when people hear of that type of bio, they assume that somehow, I was one of those parents who had a child who was hard of hearing or deaf first and then went into the field. For me, it was flipped. I was one of those kids that was very interested in sign language when I was young and that motivated me to pursue something related to deaf people. I just was really interested in the communication piece and realized that there weren't really undergraduate programs in deaf ed. I went through the special ed path and then found my way through to a graduate program in deaf ed. I was lucky, because it was at the beginning of the out of trial approval of cochlear implants for children. 

[0:04:42] DS: Oh, yeah. Turning point. 

[0:04:44] MB: I was in an area that was one of the original approved educational sites that you could only get a cochlear implant if you live near a place where you could get an education and have support from specialized professionals. It was just luck and the timing of it to be involved at that point. I liked working with children. In my early interest started with appreciating the beauty of sign language. Then as I learned about the science and the technology and the possibilities as we moved closer to newborn hearing screening finally being the standard of care, new opportunities opened for me. 

I went from classroom teacher to itinerant teacher. Then was looking for more, more, more information and had the chance to work in a hospital setting and work with families at all different points in their journey, but really being one of the first people to interact with them after their child was identified as having hearing loss which is a real unique and important sacred, almost position to be in and how you can influence that initial feeling and that initial moment with them, but I missed families. I was able to Ð there was a position open at Clarke that coincidentally was around like a corner from my apartment. I was lucky enough that they took me on and gave me time to learn how to run a program. 

[0:06:16] DS: That's amazing. 

[0:06:17] MB: Yeah. It's exciting here, because every state is set up a little bit differently, but the way it's set up in New York state and in New York City in particular, a lot of things are contracted out to agencies where the municipal people are just administering things. Because of that, we're able to work with families, babies as young as three or four weeks of age, so at really early points in the early intervention system and lead them through to understand what they can access and helping them to have high expectations. Really from those early moments on, we can really support them in figuring out what their goal is for their child and their family. 

[0:07:04] DS: That's so great. I think it's really Ð you hit on something there that's really important. I know a lot of listeners are interested in working specifically with pediatrics. I am a pediatric audiologist myself and I completely agree that those early moments with families where you're the person helping guide their boat. You're one of the first members of that team they're going to be building over the next months and years of their child's life. 

It's so great that with your program and in your area, these families have access to professionals who understand the full potential that this child has that when parents can sometimes feel so lost in it, there's someone who says, ÒWe work with families. We work with kids. We know all of the potential your child has. Let us help you on this journey that you're going on.Ó What a great community that you've established and helped grow in that area. 

[0:07:50] MB: Thank you. It's exciting in the sense that we say that there's a point where parents are still shell shocked at having a baby, and then having a baby and having to run to these appointments with specialists they've never heard of before. While they're figuring out how they're ever going to shower again. That's such a vulnerable period. At the beginning, many of them don't necessarily believe whatever this is in front of them is going to work, but they believe that we believe it and at the beginning that's enough. That's a lifeline. 

[0:08:21] DS: Wow. Yeah. What a great way to put that and how important professionals like early interventionists and teachers of the deaf and hard of hearing, that's their role here, right? It's so, so critical in those early stages especially, but even into those first few years. Switching gears then, so you've been in this role with Clarke School and or at least in the early intervention space all this time, no personal connection to the world of hearing loss or children who are deaf and hard of hearing, just a passion to serve that group of people. Then you have a child who has microtia and atresia, right? 

[0:08:54] MB: I do. To give some context to that, I've been at Clarke for 14 years and just before, about a year or two before I came to Clarke, I got married and my husband is deaf, is a bilateral cochlear implant user. We had our first child who was eight months old when I started working at Clarke. We decided Ð I had always been interested in adoption and we talked about the different possibilities for how to grow our family we ended up adopting from China. Our second child has bilateral microtia atresia, but it wasn't a surprise. People are a little confused when I say my husband is deaf and has cochlear implants and my daughter has microtia atresia, because they try to figure that connection out. 

[0:09:43] DS: Yeah. What's the connection? Yeah, I get that. 

[0:09:45] MB: Yeah. We try to jump in and explain that. It's not genetically related in any way. My path to being a parent is a little bit different than some other families. But I will say that the interest that I had in how we as a field, as professionals, support children and families was piqued for this population before I had my daughter. But it made me realize that you shouldn't need 20 odd years in the field to be able to effectively advocate for your child and have their needs met. I think that's where there is this synergy in both parts of my life. 

[0:10:28] DS: Yeah. That makes a lot of sense. I think, it's so interesting, because tonight our goal is obviously to talk more on microtia and atresia, but with your career and the work you've done, clearly, there's like a lot of different topics we could get into. I do think whether it's related to microtia atresia or just hearing loss in general. I'm hoping a little bit later, we'll just talk to more in your experience, the advice you would give to clinicians who are helping families along that journey or families who are listening in, who have questions about how they can better support their children. 

I just think you have a really great perspective front Ð there's multiple aspects of how your perspective is really interesting here. One is just your career prior to having your child and then raising your child and then your new clinical perspective on children with microtia and atresia, it's just so multi-layered. It's really, really interesting. 

[0:11:13] MB: Well, and it's interesting for me too, because there are times, weÕre dealing with some of the bureaucracies around having children, the educational systems. I think what on earth are they saying to other Ð like if they're saying this to me, like what is happening with families with limited English proficiency, with families who just trust the person who thinks they're the head of the meeting, has their child's best interest in heart or knows what they're talking about? I find myself with lots of really interesting stories of how bureaucracies don't always support our kids in the best ways possible. 

[0:11:53] DS: Yeah. I was going to say, I feel like I definitely want you in the room if my child has an IEP meeting. We definitely want her in the room for that conversation. 

[0:12:04] MB: I appreciate that. I do think I was a frustrated lawyer in a past life or something, so that might be connected to it, too. 

[0:12:14] DS: Then let's dive in a little bit on microtia and atresia. Let's just assume someone listening in isn't as familiar with the condition. I guess you could even start with just the terminology, right? I mean, there's microtia, there's oral atresia. A lot of times people will use the term microtia atresia, so I'm just curious if you want to give us a little bit of a breakdown of the condition itself and maybe a little bit of the terminology and what that means. 

[0:12:35] MB: Sure, and to make it even more confusing if you look on the CDC website for the condition that they track like the different physical malformations. They use birth defects, which makes me cringe a little bit, but that's what their site is called. They use microtia anotia. 

[0:12:52] DS: Got it. 

[0:12:53] MB: The microtia anotia piece is really, microtia, literally means like, little ear. Typically referring to the pinna being smaller than expected. There are different grades of that. One is the mildest where there's, it's slightly smaller, it doesn't have all of the details of the Ð a typical pinna, all the way to four, which is anotia, meaning there's no outer ear at all. Three is the most common. Typically, three people will describe as looking almost like a packing pinna. 

The microtia anotia really refers to no ear canal, so they think of it that way. They don't seem to distinguish between atresia, which is that closed canal, often blocked by bone, and microtia, they think of it as degrees of microtia. Then the anotia piece would be if the canal is affected. From the CDC's point, and I think that's part of the confusion among all of us is, are we talking about an outer ear malformation, or are we talking about the atresia piece, which is often what really is creating the hearing loss. 

[0:14:03] DS: Yeah. Then I know sometimes, I've heard there can just be a narrowing of the ear canal, at least, unrelated to a congenital malformation, right? Like you could just have, I don't know, severe otitis externa, maybe could cause a narrowing of the ear canal. How often are microtia and atresia happening together? Is it more often just microtia? Is it only anotia is going to result in atresia, or can you have some form of a developed outer ear, but no ear canal at all? 

[0:14:34] MB: You can have a fully formed ear and no ear canal, which is rare. It's rare to not have microtia and just have atresia. You can also have a degree of microtia with a tiny narrow canal. If there's any combination, the most common is, is to have that Grade 3 microtia, which looks like that packing pinna with a closed ear canal. Sometimes it's hard to tell if the canal is actually fully closed. It can look like there's a canal opening, but it's so small. They can't really see into it, often though it could be pitted, meaning it doesn't go anywhere. It looks like there's a canal and then it's almost like has a dead end, so to speak. 

I've seen children who have clearly microtia atresia on one side, and the other ear looks fully formed. Then when they do more assessment, they realize that the ear that looks fully formed actually has atresia, as well. Those things happen, so that obviously, the referrals to the right people after a baby is born is critical to make sure that there aren't assumptions made based on the initial appearance. 

[0:15:47] DS: That's a great point. I'm curious with these different degrees of microtia, I'm guessing there aren't degrees of Ð well, I guess, maybe. I'm thinking like there's either a patent ear canal or there isn't, right? Or maybe there could be different levels of narrowness of the ear canal.

[0:16:02] MB: Right. You think there would be, and I think that's where the confusion comes in about whether it's a stenotic canal, it's atretic, so it's completely closed, or it's open, but sometimes you don't know. Sometimes these kids have slightly unusual anatomy that really can't be seen initially, but they have Ð and microtia atresia is most likely to be unilateral, bilateral happens, but it's much rarer. 

There are these assumptions that it's unilateral and the other ear is fine, but sometimes it's not. It might have ear infections or fluid and need tubes. There could be a conductive component, because of the canal having a slightly different width or shape that's just not picked up on immediately, because of the look of the ear and the assumptions that are made. I think there's still a lot we don't know, because it's such a small population that it's hard to get a critical mass of kids to get researchers interested. 

[0:17:04] DS: Yeah. That makes sense. 

[0:17:05] MB: Yeah. 

[0:17:06] DS: I'm also curious when it comes to middle-year development. Are children who are born with microtia or atresia more likely to have underdeveloped ossicles, or is the tympanic membrane, I guess where the atresia that might be a different scenario, right, because everything's closed, but with different levels of microtia, is there a lot of middle-year involvement usually? 

[0:17:25] MB: There would be. I think, again, it's about 500 or even less babies are born with microtia atresia in the US, every year. When you think about what that means, like how small that really is, it's hard to know, because most children who are born have microtia atresia, not microtia like with a stenotic canal. I don't know that there's enough information for me to say, maybe for an otologist or one of the, like a cranial facial clinic.

[0:17:58] DS: Sure, sure.

[0:17:59] MB: It's hard to know. I think that Ð depending on the initial information families get, which can be really not what we would think of as best practice. Some of those kids who do have maybe like a milder microtia and maybe a narrow canal. They may never go to specialists unless language delays start or behavior problems start. If they seem to be just fine, often the medical professionals, the family comes in contact with don't consider that to be a child with a hearing loss or at risk for hearing loss, and they don't get the same information and follow up. 

[0:18:37] DS: That's really interesting. I mean, I do Ð I want to dig in a little bit to more of what that population looks like and what the diagnosis process is. Maybe before we get to that, if we want to talk about what the intervention options are. If you want to play out a scenario, a baby's born with the most typical, let's say unilateral grade three microtia with Ð you said that was probably the more common one, right? What does that process usually look like? 

Previously, I was doing a lot of natural sleep ABRs for babies who referred on the newborn hearing screening. I didn't have a baby in the Ð it was a brief time. I guess with only 500 babies a year, it's not surprising. I didn't have a baby with microtia come through, but in that scenario, I would try to get bone conduction information on that side, try to get some masked information for that ear. Then what intervention options are we talking about with babies at this point at hopefully diagnosed by that three-month mark and we're thinking of intervention by six months? What does that look like for children in this scenario? 

[0:19:39] MB: Well, ideally or practically?

[0:19:44] DS: I guess maybe start with ideally and then give me what's really going on. 

[0:19:48] MB: Ideally, with the, the updated JCIH recommendations in 2019, it's actually recommended that children who have an ear malformation like microtia atresia, skip screening altogether and go right to ABR. 

[0:20:05] DS: Okay. 

[0:20:06] MB: They should have diagnostic testing and that it can even happen as soon as like in the hospital while they're there. That they should immediately be referred to early intervention. 

[0:20:16] DS: Got it. 

[0:20:17] MB: I don't think that's happening. It's probably happening in some cases, but when you think about the fact that we Ð I think many of us say the one of the weak links in the 136 model is like waiting for ear molds to come back. 

[0:20:32] DS: Sure. Absolutely. 

[0:20:33] MB: The fear of over amplifying little babies, because if you're going off of the ABR, it's not really a hearing test per se, so people want to be cautious and all of those things, right? But if you can fit a baby shortly after birth, but that's probably not going to happen. You can fit a baby at a couple of weeks old with a bone conduction device on a soft band. If they didn't have a hearing loss, it would be a headband with a funny box. 

[0:21:03] DS: Got it. 

[0:21:03] MB: You'd have to Ð if I put on that same type of device right now as a person with normal hearing, it wouldn't bother my ears. I wouldn't notice it unless I plugged my ears, right? Because the vibration is so subtle. There's no Ð I think of the do no harm. There's no harm in amplifying early with bone conduction devices if you find out later maybe that the canal is enclosed, because just as babies grow, if the canal is stenotic, but not closed, you might realize that it actually does go all the way through 20 or drop, so you can make other decisions then, but babies can be fit that early. 

In fact, the Ð I'm going to get their name wrong. I feel like Ð forgive me, anyone who's on this committee in advance, the north Ð I want to say it's the North America Bone Conduction Work Group that put out a paper in 2020 outlining best practices in managing non-surgical fitting of bone conduction devices for children. So specifically, talking about soft bands. They say as young as two months. When I was actually at a conference and said, ÒWell, why not younger?Ó Because we would really say for like one, three, six is almost one, three, four, six. It's to fit with amplification Ð 

[0:22:28] DS: Yeah. Yeah. Yeah Ð 

[0:22:28] MB: Diagnostics, you can fit with amplification the same day a baby with microtia atresia is identified, because you don't need to wait for the ear mold. 

[0:22:38] DS: True. 

[0:22:38] MB: Right. The comment that the speaker came back was we thought two months was reasonable in terms of the process of applying for insurance, so that there isn't a reason it couldn't be younger other than they thought practically that might be the earliest. 

[0:22:54] DS: Sure. Sure. Sure. 

[0:22:55] MB: That's what we would want to see, because it practically what often happens is that it takes much longer and parents are given much different advice from professionals. Even those who work with children with hearing loss about what the next steps are to follow up that are not consistent with the JCIA recommendations. It leaves parents confused. Things like surgical repair of the ear, which some parents are interested in. The earliest you can do it is at the age of three, depending on which approach you might be taking, but it's expensive and often not covered by insurance, so there are a lot of hurdles to overcome to get to that point, but birth to three is that critical language period. Hoping that the child has an ear like Ð is able to get surgery for a fully formed ear, three years or four years or seven years later, but not thinking about the immediacy of language access is a concern for me as an interventionist. 

[0:24:06] DS: Yeah. That makes sense. If you're with these families from that really early stage, it's really at the top of your mind, we're working now for a goal that's years from now. I'm curious, I honestly haven't worked very much with bone conduction hearing aids in my past, but with babies really sitting up at like five, six months, if a lot of their time is spent laying down is placement of the headband or I guess of the soft band different? Is it still in that mastoid placement? If so, are babies rolling on to the device more or what's been your experience with that? 

[0:24:41] MB: Yeah. Even for bilateral babies, because their little heads are really tiny. If they get that early point, you want them to have access like full access, consistent access to the optimal auditory signal, right? One at the very beginning is okay, because they're sleeping most of the day. They're on their back or being held in a certain way. What you'll often see is a baby with a soft band positioned in a way that the device is located around the forehead, right? 

Ideally it would be great to be on the mastoid, because it's closer to the cochlea, but their heads are really tiny. Their forehead is really close to the cochlea, too, so that works. Then when they have more head control, they're sitting up more independently, that if they're not always in that like in a stroller, in a high chair, in a carrier or something like that. If it's a child who is bilateral, microtia atresia that's one we'll look to fit the second one. 

For the first one, depending on the parents, they may keep it in the front. They may start to move it back a lot. It depends on just I think their lifestyle and how much downtime meaning physically down that child is, because my daughter was 20 months old when she came home from China, so she was well past that always on her back or Ð 

[0:26:01] DS: Sure. She was always on the move. Yeah Ð

[0:26:03] MB: She was on the move, but when she fell asleep and she was bilateral, so like, she was knocking those things off all the time. There is, it's that risk of putting like expensive delicate equipment on children. I can't tell you how many weird places we have found devices over the years. I think there is a weighing out of what is the benefit in certain situations and what's the drawback, what's the risk of losing something. I think, like just the way toddlers head sit in car seats is exactly where a bilateral BAHAs would be sitting on the mastoid. If they fall asleep and you hear that whistling for the next hour when you're driving. 

My daughter was actually really an amazing, has amazing auditory skills. If we try to turn it off while she was sleeping, it woke her up. Then she would scream, because she wanted to hear. Really, it was just not a pleasant experience until she was a little older and she could turn off the BAHAs or something. 

[0:27:09] DS: Yeah. That makes sense. I work mostly with kids with cochlear implants, so the car, I talk to families all the time, the car is the no man's land. ThereÕs no rules apply in the car. If you've got to take them off so they don't get thrown out a window, I think everybody understands. 

[0:27:23] MB: Yeah. It is crazy what kids can do. My daughter is actually a little bit type A, which is good, so we had a really good run until she was about eight and was skiing and decided she got to the point where she felt confident enough to ski without them and the sound was annoying when she was skiing, so she took them off and put them in her pocket and lost them, both of them. 

[0:27:47] DS: Oh, no.

[0:27:47] MB: Thank goodness a month before the expiration of the warranty. We got them replaced, but yeah, I was looking for every previous version we had on the shelf that we had just not gotten rid of to see what worked till we could get that replacement in. I mean, as a parent, practically speaking, that's really stressful. That's one of those things also like, if the minute I see a parent who has a child that's just gotten devices, I look for the safety line and talk to them about it, because often they're so overwhelmed when they're being dispensed equipment. 

They don't think about it, but IÕm like, ÒThis is going to come out so many times and the safety line gives you a fighting chance of getting it before it hits the ground.Ó You're doing like the dive across the room to catch it or something. So just to be to be aware of that, because it's such a pain. It's so much stress in so many hurdles to replace devices that yeah, we should bubble wrap them, bubble wrap the kit, bubble wrap the device.

[0:28:53] DS: Bubble wrap all of it. Speaking of the devices themselves, I'm curious if you could break down a little bit more for us the different intervention options for this kind of conductive hearing loss. We know of bone conduction devices, but then within that category, there's so many sub categories, right? There's soft band and then you move on to a surgical option. I know there's an abutment and then I know there's also devices that don't have an abutment. Again, this is Ð subcutaneous maybe, this is like, so out of my wheelhouse, because I donÕt work in this population very much. Could you just give us a quick breakdown of some of those options? 

[0:29:27] MB: Sure. For under five, everything is non-surgical in the US. There are different criteria in Europe. Everything is essentially using something to keep the bone conduction device in place, so whether it's a soft band or one of the company cochlear has like the sound arc, which almost looks like the attachment, if you were wearing sports glasses that have the line that goes around the back of your head. 

[0:29:57] DS: Yeah. I've seen Ð it's like if you put your glasses on backwards, the rest of them on your earÕs thing. Yes. Yes.

[0:30:02] MB: Exactly. There are some kids who like that, because there's no headband kind of thing that it's visible to other people. One of the companies has an adhesive type of attachment where there's a connector with adhesive that goes along the mastoid and then you connect the device to that. They all work the same way, right? But we know just from other research and studies that the best auditory access is really direct bone stimulation and all of these are not doing that, so you lose a little bit of sound. 

The FDA has criteria about the, any of the surgical options, so the earliest you're supposed to do it is five, but it's also based on skull thickness, because of the way it's inserted. If you do it when the skull's too thin, you run the risk of possibly fracturing the skull or the piece that is put into the skull to keep it in place, getting pushed out as the bone grows back. 

[0:31:04] DS: Oh, yikes. 

[0:31:06] MB: That either way, but there's a period typically of about four months if it's that abutment that you need implanted where that it has to Osseo integrate before you can use it. Some of the newer devices are put in more like cochlear implants in terms of every Ð like receiver simulator under the skin in a surgical procedure and you wear a processor on the outside that connects through a magnet, that type of thing. Those technologies tend to address some of the issues that the other surgical options can't or have issues with. Like some of the traditional abutment type of thing like, there's a lot of risk for infection. If you get the one with the magnet that can connects to that abutment, the older style like it's not as strong a connection. 

If you make it too strong, you can have skin breakdown. If you don't make it strong enough, it can get knocked off easily. But these newer devices are using other technologies, so this is not my wheelhouse at all, so someone will no doubt let that I can describe this possibly very wrong. They're using this other technology to simulate the vibration. The fact that it's not actually vibration means that they're reducing some of that feedback issue that you get when you try to amplify high frequencies. 

[0:32:30] DS: Got it. 

[0:32:31] MB: Those have shown promise and when you see the audiological results from the last four or five years that those devices have come into the market and you see that they're getting like that high frequency like at 4,000, they're getting them up to like 10 dB, 15, where a BAHA, typically like you hit 25 and you're starting to squeal. You have difficulty getting those voiceless high frequency consonants well amplified. It's really nice to see those happening that they're just more Ð I think they're nicer to look at for kids like the abutment, it worked. It got you sound, but it felt a little strange. You have this little snap on the side of your head and if you even if you cleaned it well, you ran the risk of infection. 

I think that as the FDA keeps lowering the age for these newer devices, more families will probably want to consider those, because of the easier maintenance and the less medical risk and the greater auditory access it provides. We'll see. It's exciting. But the thing is, and I go back to you, because I've been all over the map in terms of settings and ages of kids and teenagers and young adults that I've worked with, but my heart is in that birth to five. Earliest you can do surgery related to amplification for bone conduction devices is five. It's something to think about, but right now, we need language. 

[0:34:06] DS: Yeah. Absolutely. Access is so critical that early. It sounds like there's a lot of different options that are starting to be more and more available. I know that with that improved frequency response, I guess for the bone conduction device. Also, you're getting better access to those soft high frequency sounds. Are you seeing a lot of mixed hearing losses in this population? Is it mostly just purely conductive like, total conductive hearing loss with oral atresia, anotia kind of a situation? I mean, I guess, is there risk for progression in those sensory neural thresholds over time? 

[0:34:41] MB: It's an interesting question. Most of the Ð in my experience, most of the kids have conductive loss and often will just say like maximum conductive loss for convenience and the difficulty in testing children and the difficulty in testing on the tri otic ear and masking issues, those types of things. It's usually, strictly a conductive hearing loss, not always, but often it is, because the inner ear and the outer ear form at different times. They're not Ð like they're being an issue with one is usually not related to an issue with the other, unless it's something that there's more craniofacial involvement like, a syndrome, where it could affect the cochlea either by the nature of whatever the syndrome is or just from the anatomy perspective. 

[0:35:31] DS: Got it. Got it. That makes sense. 

[0:35:33] MB: In theory, I guess that someone who has a conductive hearing loss, it could have sensory neural hearing loss like related to age or damage to their hearing from drugs or something like that. I haven't run into that per se, but like theoretically, it should be possible. You could have both, but not. I haven't run into it yet in terms of like profound deafness, but you started with a conductive loss. 

[0:36:01] DS: Sure. Sure. Sure. I know I've Ð I mean, again, not having had much experience. I do know that feedback can be such a persistent issue with bone conduction devices. Anything that can help combat that, I'm sure is a very appreciated feature. 

[0:36:14] MB: Yeah. The companies have done better at addressing that. The beauty of competition is that the companies try to do better. They try to have better features, including feedback controls and stuff like that, but there are things that come up. I often, if I'm presenting about like things to think about if you're working with families. I think about like just getting a winter hat. If you have a child with microtia atresia and they're wearing their BAHA and personality wise like, my child was rigid. Like she woke up and it went on and it was us at the beginning, but it was definitely her, so at times where it was below zero and we were like, ÒListen, it's more important for your head to be warm.Ó She was having none of it. She is like 100% committed. 

[0:37:04] DS: Yeah.

[0:37:05] MB: She took it very literally, waking hours. For like three years, she had one hat that I look at pictures of and we ended up losing it. I cried when we lost it, because it was the one hat that fit over the BAHAs that didn't cause feedback. 

[0:37:22] DS: Wow.

[0:37:23] MB: I look at it like just before we lost it. I was afraid to wash it, because I didn't want it to lose its shape in case it affected it. Man, that thing was dirty. I mean, like I'm sure people were like, ÒWhat is going on with that parent? Wash your child's hat.Ó Those types of things like shouldn't be a big challenge. Wearing masks over the last couple of years, for someone who's using amplification and it's bilateral, it's so much easier, because she used her BAHAs. She just hooked the looped the mask around her BAHAs and it was fine. But if they are not using amplification like that constant reminder of like, ÒHow do we figure this out? My child doesn't have an ear to put the mask on.Ó 

[0:38:04] DS: Yeah. That's a really great point. I saw some, I want to say a child with microtia atresia, it had a 3D printed. I saw this in somewhere online like a 3D printed mask holder. That was like a really inventive way to solve that problem. 

[0:38:19] MB: There are different ways around it. It's funny, because my daughter uses cochlear BAHAs and she went from the five to the six and the profile of them are different like the six is smaller, but it's also the profile in terms of the way it connects is closer to the connector on the soft band, so that the loop from the mask didn't actually fit under it, the way it did with the five. 

[0:38:46] DS: Interesting Ð 

[0:38:46] MB: We had to Ð we went through a lot of different scenarios to figure out how to make it possible for her to wear a mask, which she needed to do for school, initially anyway. Like I would sew buttons on and try to do it that way. She ended up using bobby pins and was fine with it. We knew which masks were best that gave her enough room to be able to tie the mask loops, but it's been rough. 

[0:39:13] DS: Yeah. That just speaks to the forced ingenuity of parents of children who are deaf and hard of hearing. They just come up with the best things. There's a parent of a child who has a cochlear implant and the companies hadn't designed a way for her to maintain her processor or coil set in a way that clipped into her hair really well. So she just went about it on her own, designed and 3D printed it. Now she's running a total shop where she sells these CI retention solutions hair clips out of necessity. They needed a solution and they came up with themselves. 

[0:39:44] MB: Well, it's interesting, because what I see a lot of parents doing is looking on like Etsy or something like that to get more interesting soft bands than the ones that the companies make. The thing that always makes me nervous is that they don't realize that the soft band is actually part of the medical device like it's gone through testing, it has to be approved, whatever. The thing you're getting off of Etsy may not have tight enough elasticity or whatever to get the contacts you need for the cochlear to be stimulated with the vibration. I always tell parents, if you're going to do that, if you're going to buy something from Etsy, before you use it, go to your audiologist and have them test your child in the booth using it. 

[0:40:31] DS: That's a great point. That's a really, really good point. 

[0:40:33] MB: We actually did that with the white hat that was like gray by the time we lost it. We did that. We were like, ÒHow does wearing this hat over her BAHAs effect what she's hearing?Ó We Ð I don't know, I think it was like she lost 20 dB, 25 dB maybe with this hat on. But I was like, ÒThat's a good trade off.Ó Like, if it's that cold out that she's got to have something, but we went in and checked. I really advise parents to not, to recognize that the soft band is a medical component that has gone through testing. You want it tight enough to maintain that contact at whatever bony area you're putting it, otherwise it's not going to give your child the auditory access they need. 

[0:41:21] DS: Yeah. I just thought of a very random question. This might be a dumb question, but I have to ask this. 

[0:41:25] MB: Yeah.

[0:41:26] DS: Normally, when we think of feedback with a hearing aid user, a lot of times it's at a frequency where most high frequency hearing losses, the person, the listener at least wouldn't even realize the feedback was occurring, right? But for a child with a bone anchored hearing aid, they probably have normal high frequency hearing. Does it get picked up in a way by the microphone that it also bothers the person with the bone conduction device? 

[0:41:49] MB: They definitely know it's there. They know that it's there. You'll see them naturally, just learn Ð the way many young children who use hearing aids do where like, they'll check it by touching it, check to see if it's working by touching it to get the feedback. Yeah. So that they do, they typically do have normal cochlear function. Once they have access with the bone conduction aid, their responses are typically within normal limits. They have the same issues as other people with hearing loss in terms of like the problems with background noise and distance and things like that. In terms of access, they have access across frequencies in most cases. 

[0:42:31] DS: Got it. Sort of thinking about parent ingenuity and then thinking of that more from like an early intervention or this question might be something you haven't interacted with, because I'm thinking more even like at the school age level, but specifically a child who's unilateral with their microtia atresia. We think about an FM system or some system in school to give them better access to the teacher. I mean, my thought would be, if you have them set up with some FM system that is streamed directly to their bone conduction device, technically both ears are going to receive that information due to zero dB in an aural attenuation that we have from our skull. Is that true? Is that the mentality when it comes to using an FM system for kids who have microtia and atresia? 

[0:43:14] MB: I mean, I think the general recommendations in terms of the access that whether it's like an FM router system or remote mic use, I think those general recommendations are the same. I think that similar to other kids with unilateral hearing loss, there are times where what should work in theory isn't what works for that child, whether they're getting some distortion or what have you. I think that's where a really good audiologist is between your primary and educational audiologist, if youÕre in a school or a district that is using one to try to figure out what the best set up is for that child. How do they hear the best? What's going to be used the most? 

My daughter is now in seventh grade. We're dealing with the non-compliance issues. I realize there are a lot of things that go into that. Sometimes like you need really creative people on your child's team who won't just assume that the recommendations they gave to five other children are the same for your child, because it might not be. Then sometimes you'll hear about kids who are using some type of like FM roger on their Ògood earÓ to give them better access to the teacher as opposed to Ð I think there are so many different configurations that it is individual. I think, some of the same conditions that make listening for someone with unilateral sensory neural hearing loss difficult occur for children with microtia atresia and often people see it as behavior as opposed to hearing. 

[0:44:56] DS: Absolutely. I see a fair bit of children with single sided deafness. I mean the impacts to localization and understanding and noise, I mean 100% we get reports of behavioral problems that aren't actually behavioral problems. I think it also Ð I think the similar concept applies to maybe this child gets a cochlear implant, but what is the best in school option for access to the teacher? I don't want to just stream it to your implant. I want your normal ear to also get access to that teacher. I think, yeah, it's important to have a good team. 

[0:45:28] MB: Yeah. A lot of times we don't Ð we're not doing it consciously, but we're really expecting the child to somehow tell us what they need. We actually have to teach them what good hearing is, what good auditory access is, so that they know how to advocate when it's not there. 

[0:45:47] DS: That's a great point. That's a really good, really good way to describe that. Hopefully developmental aspect of advocacy, that they need to understand what good access is if they're going to be able to advocate for it. That's a great insight. 

[0:45:59] MB: Yeah. Yeah. Well, you have to be used to something being present and expect that it's just there for you to be able to stand up for yourself, especially to adults when it's taken away. If the lights are off and the teacher started teaching, someone would say, ÒHey, the lights are off. I can't see the board.Ó Right? But if everyone just acts, if a child is used to having inconsistent access or poor signal, they don't know that what they're hearing is any different than anyone else, really, right? They just think they're missing something or they tune out. Yeah. Self-advocacy is a whole other interesting topic related to the way we unintentionally, intentionally put the responsibility on like a five or six- or seven-year-old to advocate for themselves for something we're responsible for teaching them. 

[0:46:49] DS: That is a really great point. I think we're coming up towards the end of our time, but I did want to like give you an opportunity. I think that might be something if you want to share a little bit more on or just like I was saying earlier, you just have such a really multi-layered and really insightful perspective on all of these different topics that we've talked about. 

There's a lot of students who listen to this podcast, audiology and speech students. I'd be curious, what advice you would give them when it comes to working in this space, whether it's an early intervention perspective or early intervention specifically with microtia atresia, just what would be an important Ð there's been a lot of important nuggets throughout our conversation, but I'm wondering if there's anything that you just feel like is a really important thing to share with that group or maybe clinicians who are new to this world of clinical care. 

[0:47:37] MB: For any of us working with families, realizing how vulnerable they are, how new this is, it's not something they expected or went to school for, they're trying to understand it and you do have to repeat things more than once. You can't assume, I can't tell you how many audiologists over the years have said, ÒBut I told them that.Ó It's like, well, it's like teaching. It's like, if you're teaching and no one's learning, are you teaching? If you're counseling and advising and the parent has shut down, you're talking to yourself.

[0:48:11] DS: Yeah.

[0:48:12] MB: Realizing that there are very few parents who don't want the best for their child and their child to become happy, healthy, independent, successful adults. If they're making decisions that seem to go against what you think, one is to step back and think about that like, do they have the information? Right? Because most parents, if they have the information to do something to help their child will, and if they don't, if they're not, either is there a barrier that you can help them with or is it something that they're choosing to do something else and it's their right to. 

To me, I think particularly in that early intervention space, or for late identified kids whose family miss early intervention and they've gotten no support, it's such a learning process for families that making assumptions based on the last time you talk to them will leave them without the support from you that they need, because they need that information, but they can't process it any sooner. They need as much accurate information that unbiased information is a weird thing, because it's really hard to be unbiased. 

Accurate information so that they can make those decisions and providing them with the support and I always say with kids with microtia atresia, the parents are often self-diagnosing in the delivery room or in the like recovery while they're waiting to go to a room. They're googling on their phones. Often, they're meeting people who have no idea, ENTs have told parents that, ÒDon't worry, I think it'll unfold.Ó 

[0:49:59] DS: Oh, interesting. 

[0:49:59] MB: I'm like, ÒAre you kidding me? Like, where do you graduate in your class?Ó Like how does an ENT not Ð even if they haven't seen it, how do you not know that an ear doesn't unfold? 

[0:50:11] DS: Wow, that's really interesting. 

[0:50:12] MB: It's scary. Parents are often, their heads are reeling before newborn hearing, the newborn hearing screener comes in or what have you. They're looking for information and they don't ever have that period of forgetting, right? Like, if a baby fails newborn hearing screening, parents can still, can be in denial about what's going to happen at that follow up testing, right? They have that period of time. This family, every time they look at the baby, they're reminded. It's something that's there and they have people around them who are not giving them accurate information about the time they get to you. It has been traumatic. 

[0:50:51] DS: Yeah. Wow. That's a really great insight and a really good reminder. How you approach counseling in this situation has to be so different. You're exactly right. There's a completely different history this family has gone through in just those couple months before they get to you as a clinician. It's so important to be mindful of that. 

[0:51:11] MB: It is and in terms of people having fidelity to their role and the work they do. Other than that, probably the single biggest thing is to try to connect them with other families or other resources, other parents who get it. Often parents will say that that was what the lifeline at the beginning was just finding another parent who had been there, who could help guide them a little bit to next steps, because even the early interventionists like, early interventionists in general like, hearing loss is a small part of what an early intervention system deals with, let alone microtia atresia. Trying to connect them with other families, so that they can develop a new normal, a new safety net, a new community is really critical for their emotional health as they start this very lifelong process. 

[0:52:04] DS: Wow. That's really, really helpful information. I got to ask you one more thing and then I guess we'll wrap up. With that in mind, so you give us a really great philosophical counseling piece of information to take into appointments, working with families and with children who have microtia and atresia. But from a parent's perspective. Other than the retention clip, which I do think is really critical, what other hack for like families of kids with bone conduction devices, do you wish audiologists or early interventionists knew to be sharing with families to say, ÒThis is that thing that families don't know about, but they need to know it exists or this is the tip that can be so helpful.Ó What do you have for that one? 

[0:52:46] MB: A thing that comes up a lot is bike helmets, like Ð I have these pictures now saved in a folder on my phone, because they're a couple of microtia parent groups on Facebook. Someone else say, ÒWe can't find one that works with their device. What do we do?Ó I now have the pictures and the company, but looking for those almost old fashioned cut helmets that sit up a little higher, that typically you can maneuver the BAHAs if they're wearing Ð assuming they're wearing them along the mastoid so that it doesn't feedback. We ended up having to do that for a number of years when my daughter was skiing. I had to balance that, ÒOkay, what's the risk of her getting hurt wearing a bike helmet instead of a ski helmet?Ó 

[0:53:33] DS: Yeah.

[0:53:33] MB: What kind of a bad parent am I taking that risk? But she would not ski without wearing amplification. She really doesn't understand how anyone could consider not being bilaterally amplified if you have bilateral microtia atresia. I had to take the risk and use that bike helmet until she just grew enough that she could use a traditional ski helmet if we took the pants out of the ear area. 

[0:54:03] DS: Got it.

[0:54:03] MB: That was fine, but the bike helmet is definitely one of those things that comes up a lot. We also didn't talk about ear reconstruction and though some of the considerations, but one of the things is that some of the plastic surgeons advise not getting girls ears pierced if you're considering reconstruction, because of what they want to use the cartilage of your child does have in terms of the reconstruction, so like, there are little things like that. 

[0:54:32] DS: Yeah. That seem important to know that goes beyond a tip. That's like, you need to know that. 

[0:54:37] MB: Yeah. Yeah. I think the other thing parents ask a lot like how will I know if my child has an ear infection? Which is good Ð

[0:54:45] DS: Yeah. ThatÕs a great one. 

[0:54:46] MB: Well, it's a differential diagnosis in terms of like, they're running a fever, they're pulling on their ear, they can get ear infections if they have a middle ear space, but most children haven't had imaging to know. It's a process of elimination. 

[0:55:02] DS: Yeah. Interesting. Well, there's so many things here that we're just now learning about right at the end of our time. I don't want to keep you too late. Thank you again for joining me. You have a lot of stuff going on this year. Do you want to give the listeners a quick breakdown of where they can find you? Maybe if they have any questions, if they want to connect with you, or if they want to just tune into another one of your amazing talks or the projects you're working on. 

[0:55:28] MB: Definitely, anyone is free to reach me here at Clarke. My email is mberger@clarkeschools.org C-L-A-R-K-E-S-C-H-O-O-L-S.org, so I'd love to hear from other parents or professionals. I'm also going to be presenting at the EHDI Conference in March in Cincinnati on microtia atresia. Hopefully I'll get a chance to meet more parents there. I'm co-chair Ð I'm not talking about microtia atresia, but I'm co-chairing the Children's Hearing Institute's controversial issues in pediatric audiology on March 23rd and 24th. It's virtual in. It's from New York City, but it's a virtual conference this year. Hopefully people can tune in to hear some of the amazing speakers we have lined up. 

[0:56:23] DS: One of UNCs own Dr. Lisa Parks can be talking on unilateral hearing loss and cochlear implants. 

[0:56:28] MB: Yes. I'm very, very excited to hear that. Such a great team at UNC. So there are so many topics we could have just heard two days of UNC people, because it's like how Ð theyÕre just great people there. In addition to work and kids, I'm taking two courses this semester. I'm a little bit more scattered. The next three months are going to go really quickly Ð 

[0:56:53] DS: ItÕs a busy, busy year for you. Absolutely.

[0:56:54] MB: Yes. I keep thinking I'm going to pare down and then something comes up that just sounds too good. 

[0:56:59] DS: Yeah. You just got to make it the summertime. 

[0:57:00] MB: Exactly. That's my goal. 

[0:57:03] DS: Well, it's really been so fantastic to have you. I'm so grateful that you took the time and thank you for sharing your email for people who have any other questions. It's been a great night. Thanks again for joining me. 

[0:57:13] MB: Thank you so much. It's been a pleasure.

[END OF INTERVIEW]

[0:57:16] DS: That's all for today. Thank you so much for listening, subscribing, and rating. This podcast is part of an audio course offered for continuing education, through SpeechTherapyPD. Check out the website if you'd like to learn more about the CEU opportunities available for this episode, as well as, archived episodes. Just head to SpeechTherapypd.com/ear. ThatÕs SpeechTherapyPD.com/ear.

[END]

OTE 39		Transcript

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