Microtia and Aural Atresia Program at Lurie Children’s with Stephen Hoff, MD, and Akira Yamada, MD, PhD

The Lurie Children's Microtia and Aural Atresia Program is dedicated to improving hearing, communication and cosmetic outcomes for children born with microtia, a congenital birth defect affecting the ear's form and function. Leading this multidisciplinary initiative are co-directors Stephen Hoff, MD, and Akira Yamada, MD, PhD. On this episode, they discuss both the aural and reconstructive aspects of microtia treatment, as well as the program’s team of experts offering families the most innovative therapeutic and surgical options all in one place.

“It's really great when we see kids, and really satisfying, who have had their ears reconstructed, and they show their ears off. And what that means is they have their hair up for photos and they're showing that ear, you know, in their yearbook, or whatever it is, where they're not just hiding their ear, they're showing it off.” 

Stephen Hoff, MD 
Attending Physician, Otorhinolaryngology-Head & Neck Surgery; Endoscopic Ear Surgery Director; Director of Microtia Surgery 
Associate Professor of Otolaryngology - Head and Neck Surgery, Northwestern University Feinberg School of Medicine

Akira Yamada, MD 
Attending Physician, Plastic Surgery 
Professor of Surgery (Pediatric Surgery) and Surgery (Plastic Surgery), Northwestern University Feinberg School of Medicine 

Show Notes 

• Microtia is a congenital birth defect that can impact both the form and function of the ear. Typically discovered after birth, microtia is usually an isolated condition in otherwise healthy children. (1:44) 
• Drs. Yamada and Hoff work as a team, each with their own area of expertise. Dr. Hoff focuses on the aural component of treating microtia, as the condition can cause degrees of hearing loss.
• Dr. Yamada, a plastic surgeon who has studied the precise anatomy of the ear’s curvature for 15 years, focuses on ear reconstruction. (7:38) 
• Hearing loss caused by microtia can affect speech and language development. For this reason, use of hearing devices (such as a bone conduction device) is recommended in young children.
• As a child ages, comprehensive audiology and imaging like CAT scans are utilized to refine treatment options. (8:46) 
• Rib cartilage is generally used to reconstruct the ear; the body tends to accept its own tissue and rib cartilage allows for sensation in the reconstructed ear. (14:08) 
• The stigma around wearing hearing aids is diminishing, making them more socially acceptable, especially for young children, and even desirable due to their technological capabilities. (20:25) 
• It’s expected that future research for hearing will be tailored towards more accurate, higher fidelity devices that are even more discrete, with reconstruction potentially making use of 3D printing using biocompatible material grown in a lab. (21:00)  

Transcript 

[00:00:00] Erin Spain, MS: This is Precision: Perspectives on Children's Surgery from Ann & Robert H. Lurie Children's Hospital of Chicago. I'm your host, Erin Spain. On this podcast, we introduce you to surgeons at one of the country's most renowned children's hospitals to find out how they're transforming pediatric medicine. Improving hearing and cosmetic outcomes in children with congenital birth defects of the ear is the focus of the Lurie Children's Microtia and Aural Atresia Program. A team of experts offer families the most innovative therapeutic and surgical options all in one place. Here with details about this program are its co-directors: Dr. Steven Hoff and Dr. Akira Yamada. Welcome to the show. 

[00:00:23] Stephen Hoff, MD: Thank you. 

[00:00:24] Akira Yamada, MD, PhD: Thank you for having me. 

[00:00:25] Erin Spain, MS: Could you both introduce yourselves and your areas of expertise? We can start with Dr. Hoff. 

[00:00:31] Stephen Hoff, MD: Yeah. Thank you. So, Stephen Hoff. I'm a pediatric otolaryngologist here at Lurie Children's. My specialty within pediatric ENT is mostly children who have issues with their ears, so otology, and that includes kids with hearing loss, kids with microtia, and anybody who has issues with their ears and kind of ear pathologies mostly related to hearing loss. So we try to get them hearing again and optimize their speech and language. 

[00:00:57] Erin Spain, MS: Lovely. Dr. Yamada, tell us about you. 

[00:01:00] Akira Yamada, MD, PhD: Yes, my name's Akria Yamada with pediatric plastic surgery for the last 25 years. I do all kinds of pediatric plastic surgery, including the cleft lip, cleft palate, craniofacial surgery, and microtia. And microtia is my passion for my life. And I'm happy working with an expert like Dr. Steven Hoff. 

[00:01:22] Erin Spain, MS: The two of you work together with families and children, these children who have microtia or oral atresia, which often go hand in hand. Now these are congenital birth defects that can impact both the form and function of the ear. These conditions are typically discovered after birth. Dr. Hoff, can you tell me about when this is typically discovered and what do the ears look like? 

[00:01:44] Stephen Hoff, MD: Right. So, as you said, it's usually discovered after birth. So usually parents discover that their child has microtia, which means a small ear or a little ear on the day they're born. This is something that can be picked up on prenatal ultrasound, but the ear is not a vital organ, so typically the ultrasounds that mothers get, you know, the 20 week ultrasound looks for vital organs, heart, liver, lungs, things like that. And generally the ears are really not in that category of, let's see what the look of ear is, on an ultrasound, so usually it's a surprise. It's a big surprise for families. Most of the kids with microtia don't really have any other medical conditions. So mostly it's an isolated condition. There are certainly exceptions to that. But usually it's a bouncing baby who happens to have a very small ear. So what it looks like if you kind of look at somebody's external ear, there's different portions of the ear. The external part is the part that sticks out from the head and has all the different nooks and crannies, all of which are named, as well as the ear canal, going down to the eardrum and the structures deep to that which include the middle and inner ear. So typically a kid with microtia will have a very small ear on that side or sometimes both sides. And they're almost always missing the ear canal. There's different grades of microtia. So grade one would be a smaller looking ear. Grade two generally means about half the structures of the ear did not form, either the top half or bottom half. And there's wide variation there. The most typical kind is what we call grade three or typical microtia. And that's where there is skin and cartilage there, but it really doesn't look like an ear. Sometimes it's referred to as kind of a peanut ear because it looks kind of like peanuts you'd get at a baseball game or something like that. Kind of just stuck to the head. So it is kind of a curvy structure there, but not really resembling an ear, sometimes in a bit of a different location than an ear typically is, so a little more forward and down. And there's a fourth category where there's really nothing there whatsoever. So typically it's a real surprise to parents. Most times they have a lot of questions very early on, so we tend to see kids very early on. Much of that is just reassurance that they have a great healthy baby and they just happen to have a small ear. So much of the early visits are setting the course of what can we do and when during this child's lifetime and especially in their early childhood years, and reassuring them that they've got a great looking kid and they just happened to have a small ear. We sometimes use the Nemo analogy where Nemo from the movie Finding Nemo has one fin that's smaller than the other. So sometimes we say it's kind of like their small fin. 

[00:04:12] Erin Spain, MS: Dr. Yamada, you said that you are passionate about this particular surgery and helping these kids. Is there a message that you share with parents when you first meet them who are concerned about the cosmetic part of this diagnosis? 

[00:04:28] Akira Yamada, MD, PhD: Yes. Insurance companies actually don't like the term cosmetic, but I think this is a cosmetic surgery itself. But also this is something about the function of the external ear, which is including to be able to wear the mask, to be able to wear the glasses for vision or reading. Also to be able to wear the sunglasses for the protection of the eyes from the sunshine. So there's a both functional and the cosmetic purpose. We can provide the care to build up the new ear construct. That's my message to the parents at the time of the first encounter. 

[00:05:04] Erin Spain, MS: Dr. Hoff, how common are these conditions and is there a genetic or environmental link that has been discovered? 

[00:05:11] Stephen Hoff, MD: Great question. They're generally not very common at all. There are some kind of ethnicity biases, which is to say it's more common in certain ethnic groups. But generally they're pretty rare, maybe one in 10,000 up to one in 6,000 children who are born. So relatively rare. Most parents who have a child with microtia, they do not have microtia themselves, and frankly, most of them have never seen a kid with microtia because it's just not something that most people encounter on a frequent basis. One of the things we do like to emphasize with families is this is not something that the mother did wrong or caused during her pregnancy. Most cases were not entirely sure why the ear became mycotic, but something impeded the growth sequences of that ear. So yes, there have been genetic causes that have been identified. There's also different pathways that generate blood vessels and skin and what we call growth factors, all of which can be impaired and then lead to kind of a cascade of events that means that ear really doesn't grow. All ears come from kind of six different blobs of tissue we call the hillocks of hiss, and they have to meld together and fuse together in just the right way into what's a relatively complex structure. If you look at somebody's ear, there's lots of nooks and crannies and it's very consistent across humanity what ears look like. And so it's a complex thing, so if any one of those pathways is interfered with it can cause that to really shut down and have a small ear. But generally it is not something that is common. It's not something that's passed down within families. And as genetic testing and research has improved, we have identified more genes involved with children with microtia. But it's usually what we call a spontaneous thing, which means it's just one of those genes or those growth pathways turned off early on and just made it so the ear itself didn't generate, which also includes the ear canal. Usually things like the inner ear where the cochlea and hearing nerve are all the way deep, those actually generated at a different time in the genetic sequence. And so almost always those are normal. Those children can hear. It's just there's impedance of the sound from getting from the outside to the inside. 

[00:07:21] Erin Spain, MS: Dr. Yamada, there's a wide range, as Dr. Hoff was explaining, of what the appearance of the ear is like. Tell me how you approach each patient when it comes to helping them create an ear that is able to function as if it was an ear that they were born with. 

[00:07:38] Akira Yamada, MD, PhD: There's several type of the microtias as Dr. Hoff said, but the goal is the same. Most of a child with microtia is unilateral. 10% is bilateral. The goal is to create normal anatomy in a normal location. So that's a goal to achieve. So for that reason, I did a study of the normal anatomy of what the ear is in terms of the curve, what kind of curve the normal ear has. So I’ve been working on that for 15 years, to find out the ear has only probably six or seven types of the curves available for the human's auricle. So based on that we identify the normal size or parents ear shape and using the template and create a shape of the auricle based on that anatomy. That's what I do. 

[00:08:30] Erin Spain, MS: So there's many different avenues that can be taken to treat children, depending on if they have hearing loss or not. Tell me how your team sits down with a family during that very first visit. How do you set families up for a positive experience, because they may be coming back for months and years? 

[00:08:46] Stephen Hoff, MD: Right. I am very fortunate to work with a great team for microtia. So we have many different subspecialties represented. So, Dr. Yamada does the plastics and reconstruction portion. I do the hearing and hearing surgery portion. Dr. Ittner, one of our audiologists who specializes in children with microtia and types of hearing loss both conductive and sensory neural, and then nurse coordinator Armando Morales. So basically the four of us get together and are able to be there at the same time during our microtia clinic visit. So the families, parents and children, come in and meet each of us in sequence so that they can kind of hear from each of us what some options are, what the plan is. So as we alluded to, there's kind of two main things to talk about during these visits. One is the look of the ear or kind of the appearance of the external ear as well as the hearing. And those don't necessarily coordinate as far as their treatment plan nor timeline, which is to say you can fix one without the other or both. I am always kind of focused on the hearing portion. So most kids we see are pretty early on and they're very young. So what we want to do is make sure that they have consistent hearing tests throughout their life, but particularly early in life because we want to know what their hearing is. Most of these kids with microtia will have hearing, what we call a maximal conductive hearing loss, which is the same as if you stick your finger in your ear. That's a maximal conductive hearing loss. You can still hear on that side, but it's very muffled. So many of these kids do okay without any hearing devices or options. But we always like to optimize the hearing for these children which helps them with speech and language in what we call the critical speech and language learning window within the first few years. So most of these kids, after we do comprehensive audiology, will get some type of hearing device. Typically, it's what we call a bone conduction device in which we fit them early on. These are devices that they wear and they pick up sound and then they can actually send the sound through the bone and bypass that missing ear canal and restore hearing on that mycotic ear side, the side with what we call aural atresia, which is missing the ear canal. So they just get used to wearing these hearing devices later on when they are older and we have some imaging. Typically we'll get a CAT scan, somewhere around age five. Then there are other options available to them. So historically there was one main option called a baha, which is basically a headband they wear and that sends the sound signal through. It's still a great option. Still many of our families use that. But as they get older, there are more and more devices that can also pick up that sound signal and send it in and basically restore their hearing on one or both sides that are missing it. So much of their early visits are confirming their hearing loss or their hearing type. And then restoring that with which of our devices are appropriate for that patient, as well as what's available based on their age. So much of the visit is talking about their options with their kind of best options and really coming to a group consensus on how to optimize. 

[00:11:46] Erin Spain, MS: Dr. Yamada, at what age or at what stage do you start working with the child on the construction or reconstruction of an ear? 

[00:11:54] Akira Yamada, MD, PhD: Oh, I use the patient's own rib cartilage for the ear construct and the rib cartilage has to grow enough to have enough amount to be able to create a perfect three dimensional ear construct. And for that reason, I think the optimal timing is typically age 10. Also with chest, a lower portion of chest circumference is 24 inches or 60 centimeters. So that's a typical timing to do the surgery. And at the age of 10, most children are aware of what's missing and they are keen to do something.  

[00:12:30] Erin Spain, MS: You're an international expert in this particular surgery of using a patient's own rib cartilage to perform your reconstructive surgery. When did you start doing this? And tell me about that surgery. It might sound a little intimidating to parents. There's rib cartilage that's used. 

[00:12:46] Akira Yamada, MD, PhD: Yes, and I agree. A long story short, I learned from the master surgeon named Dr. Nagata who passed away two years ago. I happened to watch his surgery three times, and by watching his surgery, I thought I couldn't do it because it was too complex and too difficult. Before Dr. Nagata, I saw many operations done by somebody else. It doesn't look like an ear, but Dr. Nagata creates such a beautiful ear and I want to master his technique. To do that I just became a disciple. So his disciple for three and half years, spend time with him. And after that I studied by myself. That's why I started. So that's a story of myself. The surgery is a two-stage surgery and an ear reconstruction used to be four to six stage surgery, but now my approach is the two stage surgery. The first stage is to create the shape of the ear. And the second stage is to create the ear elevations to be able to wear the mask. So second stage is also important for the functional purpose of the reconstructions. It is a bit painful, but we have a team to relieve the pain, including the anesthesiology and to place the catheters to relieve the pain in the OR. By the time that he wakes up, is not much pain and the patient isn't gonna suffer from that. 

[00:14:02] Erin Spain, MS: Dr. Hoff, could you comment on the work that Dr. Yamada does? You look at ears all day. 

[00:14:08] Stephen Hoff, MD: Yeah, Akira's ears are beautiful and I'm so fortunate and it's so nice to work, you know, side by side with him. So, it is amazing, you know, these kids have these little peanut ears and by using their own cartilage, the nice thing about that is the body doesn't reject it, which is to say you literally have cartilage to work with and then you use that to sculpt it. So it's almost like a block of marble that you sculpt. Literally in the OR, he is sculpting this ear using typically the other ear as a template for size and measurements. And then each of those nooks and crannies, the helix, antihelix, tragus, antitragus, and all these little folds in the ear are sculpted individually, tied together, and then placed just where they should be. So it works out really well. I kind of used to do both portions of that. I was fortunate to have great training when I was at Stanford in rebuilding ears. And then I did study with Dr. Firmin, who is another world leader in auricular reconstruction. But when Akira joined us, it was just fantastic because his reconstructions look good. And it also allowed me to focus much more on the hearing side of things which I really enjoy. Restoring hearing, and that's kids, you know, who need cochlear implants and kids who need ossicular prosthesis and other things. But for microtia in particular, it lets me focus on all these different avenues for hearing, and then he focuses on the reconstruction, both of which are so important. And honestly, it's better to do it as a team than just solo. 

[00:15:37] Erin Spain, MS: Dr. Yamada, as Dr. Hoff was saying, there's an art to this obviously when you're sculpting a new ear. Why is it so important for families to seek out someone with high levels of surgical expertise such as you and Dr. Hoff? People who have done many of these procedures have studied under mentors, who are experts in this field. Why is it so important? 

[00:15:59] Akira Yamada, MD, PhD: The microtia reconstruction has to be successful as a first surgery is most important. Okay. So using the patient's own skin, which is critical because if you use the patient's own skin without any scar, you can create the ear with sensation. Okay. And that's one of the things I'm studying right now. And most patients have over 95% of the recovery of the sensation to the anterior surface. So if a patient comes from outside, and unfortunate to the outcome, they'll have to do reconstructions as a secondary case, we have to use some other material like fascia flap from the temporary area covered with a skin grafting.That's going to create an ear with no sensations. Okay, so that's a big difference between the two. So that's the reason the primary surgery has to be successful and it has to be done by who knows what to do. 

[00:16:56] Erin Spain, MS: Describe some of the success stories that you two have been a part of over the years. 

[00:17:00] Stephen Hoff, MD: Well, I'll start. Some of the nice things that we've seen particularly are kids that are just proud of their ears. I say that because some of the kids, you know, it's very common when kids are younger that they're embarrassed about their small ear, their mycotic ear. Usually the very young kids really don't notice. But, you know, once other kids start to notice, and elementary and especially junior high, kids are not always pleasant and kind to each other in those age groups, and so they can get teased about it. For kids who are having issues with that, we have resources available, but most of these kids will grow out their hair and try to hide their ears. It's really great when we see kids, and really satisfying, who have had their ears reconstructed, and they show their ears off. And what that means is they have their hair up for photos and they're showing that ear, you know, in their yearbook, or whatever it is, where they're not just hiding their ear, they're showing it off. That's really great to see. On my side of things with the hearing, we actually meet monthly and kind of talk about any updates we have on people. You know, we start them from a young age with their hearing test, and we know they've had hearing loss their whole life, but you know, when they're in high school and things like that, they become musicians and things that are directly related to sound and hearing and language and, you know, critical music skills. Or even beyond into college, choosing careers that have to do with communications and talking to people and things that really rely on good hearing. And so when somebody has a device that we've put in that has restored their hearing and they go on to kind of show off their ear and show off their communication skills, that's really satisfying. We've really been a part of what could have been a disability for them, has now turned into kind of something that they use as their centerpiece of identity. 

[00:18:38] Akira Yamada, MD, PhD: Yeah, The other thing, in addition to what Steve said, in the past I thought I did a good job to create a nice shape, but a problem that patient grow up and become the university student to become the pharmacist. But she said,"In class, I cannot hear very well from one side." You know, microtia side, she cannot hear very well. Okay. From the side of the microtia, she can't hear what the teacher said. She has to do a lot of lab work. It's a noisy environment, but she cannot hear at all. So that makes the study much more difficult. That kind of thing happens if you don't have a collaboration with such a good partner like Steve and the reconstruction ear at the same time. So it's nice working together. So that's the reason microtia clinic is great for the patient. 

[00:19:22] Erin Spain, MS: And another reason why a multidisciplinary program like the one at Lurie Children's exists is to make sure that you're addressing all of the child's needs. Is that right? 

[00:19:30] Stephen Hoff, MD: Right. It's really great. You know, as I mentioned, we kind of have the three of us right there, but then there's a broader access too for things like if kids need counseling or kind of some advice on how to handle things like getting teased or genetic testing, certainly available to all these kids and imaging and, you know, high level expertise on the different anatomic variations we can see. So it is really great to kind of have this team and be able to work with them and kind of bounce ideas off each other. From the hearing side of things, I work with Dr. Ittner quite a bit with audiology about which kind of devices are the best for these kids and there is no one size fits all. We try to individualize each kid's care and it's fun. 

[00:20:12] Erin Spain, MS: Last question here. You both are involved in clinical research in this area. There's a lot of research taking place to improve hearing and these reconstructive outcomes. What can we expect from the field in the future? 

[00:20:25] Stephen Hoff, MD: Well, I think we can expect a lot. And I'll kind of touch on both sides from the hearing side. There's always advancements in optimizing the hearing, and that's particularly speech language and music. And really providing what we would consider normal hearing. So, using devices that can restore hearing, but also that the devices are easy to use, easy to place, easy to interact with via, you know, Bluetooth technology and streaming technology and, fairly nondescript. So, you know, historically it was really obvious if somebody was using a hearing aid type of device, and used to be a bit I don't wanna say shameful, but you know, people really wouldn't show that off. Now wearing technology is just such a part of modern life that it's just not that unusual for somebody to be wearing something. And honestly, a lot of these kids show it off because they can stream Bluetooth directly to their device and other kids think it's pretty cool. So I expect that future research for hearing will be very much tailored towards more accurate, more higher fidelity devices that are frankly more hidden or more, you know, appealing to wear, and easy to wear. I think long-term from a reconstructive standpoint, and Akira probably has much more to say about this, you know, we currently use rib cartilage. There's been some research into other materials. Hopefully someday 3D printing will be, you know, accessible. And that's just either with a biocompatible material or even somebody's own tissue, you know, taking a sample of cartilage and then growing or printing with bio inks in the lab. That's still gonna require an expert surgeon to place that into the patient and really make sure it thrives. But I do think that there's a lot more to be done in this field, and it's fun to see each step along that way. 

[00:22:07] Akira Yamada, MD, PhD: Yeah, I think Steve said that the tissue engineering has been the hot topic for the ear reconstruction for the last 30 years. And progress is very slow at this point in time. But, I think we are hoping that's going to happen. Some companies, you know, harvesting the patient small piece of cartridge they grow in the lab, and try to build up the 3D framework, construct, and put it back to the patient. And that is still in growing face right now, that's maybe the future to try to avoid the sacrifice of the rib cartilage. 

[00:22:43] Erin Spain, MS: As we wrap up today, is there anything else that either of you would like to share to people who are listening? Maybe it's a family who has just received this diagnosis, or someone who's interested in learning more about the research that's taking place or the procedures taking place. What would you like them to know about the program at Lurie Children's? 

[00:23:00] Stephen Hoff, MD: One of the things I say to families that come in for the first time, usually with a new child with microtia, very young child, is just treat your child as you would otherwise, which is to say, read to them, sing to them interact with them in every way.These are beautiful kids. There's great resources to help with. And there's also a really wide network of people available. So I think we have a wonderful team here at Lurie Children's, but many times parents want to hear from other parents who are going through the same thing. So there's online resources. We're so fortunate one of our own patients wrote a book about it. She and her mom wrote a children's book and it's illustrated and published. It's called Rebecca's Super Ear. So it's about one of our patients, Rebecca, who has microtia and about how her journey through you know, learning about her ear and honestly showing it off as her superpower. It's so fun to have that family, and other families have these kinds of connections. And so that's one of the things we like to do, is not just kind of provide that counseling, but then kind of resources for parents who may feel a bit isolated and alone, because this is not a problem that most parents have on their radar before they are handed a child with microtia. So, that is one of these things just to reassure people that there's a lot out there, there's a lot to learn, but also there's a lot to help. 

[00:24:14] Akira Yamada, MD, PhD: The only thing I didn't dive into is occasionally associated with facial deformity, it's called hemifacial microsomia. One side of the face is smaller and soft tissue skin is small, muscle is small, jaw is small. As a craniofacial surgeon I can also help to do that. One other promising thing I discovered is using the patient's own fat tissue and harvesting from mostly the buttock or thigh and injecting it to one side of the face, which really helps to make the face more symmetrical and it lasts much longer than the adult. So child fat tissue is very promising for the future. Also working with an orthodontist to correct the jaw as well. So that's part of the microtia clinic. 

[00:24:58] Erin Spain, MS: Thank you both for your time today and really explaining what's taking place in the program and all that you can offer to these patients. It's really inspiring, so thank you so much for your time today. 

[00:25:08] Akira Yamada, MD, PhD: Yeah. Thank you very much, Erin. Thank you for having us. 

[00:25:10] Stephen Hoff, MD: Thanks very much. This has been great. 

[00:25:13] Erin Spain, MS: For more information, including how to make a referral or an appointment, visit LurieChildrens.org.