An Audiologic Approach to Managing Children with Craniofacial Anomalies

ROUGH EDITED COPY

EHDI

ROYAL PALM 5/6

SPECIAL POPULATIONS:

AN AUDIOLOGIC APPROACH TO MANAGING CHILDREN WITH CRANIOFACIAL ANOMALIES

MARCH 15, 2016

2:15 P.M.

CAPTIONING PROVIDED BY:

ALTERNATIVE COMMUNICATION SERVICES, LLC

P.O. BOX 278

LOMBARD, IL 60148

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This is being provided in a roughdraft format. Communication Access Realtime Translation (CART) is provided in order to facilitate communication accessibility and may not be a totally verbatim record of the proceedings.

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> BRITTNEY SPROUSE: All right. Can everyone hear me?

> MODERATOR: Just before you get started, if you don't mind. Hi, good afternoon. Before we begin our lecture, this is Special Populations, an audiologic approach to managing children with craniofacial anomalies.

> BRITTNEY SPROUSE: So my name is Brittney Sprouse and I'm an audiologist at the University of Chicago Medicine Comer Children's Hospital and so really I just wanted to kind of shift gears. The previous conversation was maybe are we kind of overtesting in certain populations, and really focusing on children who have a craniofacial anomaly, so not really looking at craniofacial syndromes, but really those that fall into this category of having a craniofacial anomaly, and before I get started, I just want to kind of gauge the room. How many audiologists do we have? Speech therapists? Early intervention providers? Any parents? And any physicians?

Perfect. Well, thank you for joining me. This is a very quick session on, again, looking at our approach to treating and managing children who have a craniofacial anomaly.

So first, when looking at this, again, just talking about the importance of early monitoring and management, looking at the current protocols and guidelines, and then just a tidbit of information on a study that we have going on at our own center that's looking at children who have Pierre Robin sequence. So when you think of a craniofacial anomaly, a word that typically comes to mind is cleft, and so in that sorry, I didn't change the slide there that would be correct. So cleft lip and palate. How common is this? It's most likely something that you're going to see kind of come across your doorstep there. So occurring in one in 600 births every year. The recent CDC has reported that about 7,000 children are born with cleft lip and/or palate annually, and so when we talk about how does this relate to audiology, if we're seeing a child that has a diagnose you know, if we're looking at just cleft lip in isolation, that doesn't necessarily make them more at risk for hearing loss, but certainly in seeing a child who has a cleft lip, you may be looking to see does that go along with an additional syndrome or diagnosis. But cleft lip by itself doesn't make them more susceptible to a hearing loss.

On the flip side of that, when we talk about a child who has a cleft palate, we should be thinking that they're more at risk for middle ear abnormalities, fluid, conductive hearing losses and things like that, and so really looking at how can we better manage, track, test these children.

The next category, when we think of children who have a craniofacial anomaly, kind of switching gears, noncleft. What does that look like? That occurs in one in 1600 births. So not as common as the lip and palate, but one in 1600 births. Let's look at microtia, so we're talking about the actual external ear, the abnormality can be range from a type 1, which is a little bit more mild, all the way to a type 4, which can be the complete absence or also known as anotia. More common to be unilateral versus bilateral but you can see it in the bilateral case. We've got one to five for every 10,000 births. So again, not as common as your cleft lip and palate, but still something that we're seeing in the clinic quite often. And again, we're talking about how is their hearing affected. And so if we are unable to get that sound through with having, you know, a reduced or accent kind of external ear, again, thinking about that conductive hearing loss and how often we should be monitoring and managing these children.

And then taking it kind of a step further. We've talked about kind of the malformed or absence of that external ear, looking at atresia. Now, in our field a lot of times, people will use microtia and atresia interchangeably, but really they're two different things. And when we're talking about atresia, we can have a stenotic ear canal, very narrow, all the way to that kind of complete absence, and you can have atresia in addition to microtia. You may have that microtia in isolation, but really when you're switching gears looking at atresia, most likely you're having microtia and atresia and when we think of atresia, there's some sort abnormality with the middle ear or even the inner ear. There's not uncommon for there to be again complete absence of that canal or very stenotic ear canal, so again, when we talk about those kids, these are more of that permanent conductive component and so it's not something that's going to be resolved in the same way that maybe we see fluid being treated with tubes or things like that, and so really, we know that these children are more at risk for changes in your hearing or, you know, if it's at birth with microtia, atresia, that this is what their hearing is going to be so we really need to make sure that we're following them and tracking them and getting them kind of directed in the right direction.

Another category would be the ear tags and ear pits. And so I put this on the list, one because it's pretty common. Five to ten in every thousand births, and so making sure that you're doing a good visual inspection, so making sure that that gets documented, so whether it's at the point of their newborn hearing screening or certainly at that followup, making sure that we're looking at the ears, not just looking in the ears, but looking around the ears for these skin tags and skin pits, just knowing how common they can be. Again, that doesn't mean that they may have a hearing loss, but it certainly can be indicative of another syndrome, something abnormal with the structure, and so we want to make sure that we're ruling that in or out with these children.

Malformed eyes. So this falls more into that category of not directly affecting the hearing loss, but certainly associated with syndromes or conditions that can affect the hearing loss. So whether it's hypertelorism, where the eyes are more widely spaced, or more closely space, if you look at hypo. A coloboma. Again, when you're seeing these things and you're looking at the child, we need to be thinking that there could be more that's going on, even if it's not directly related to the ears. But do they have some sort of other syndrome or condition that's associated with that. You know, we think of a coloboma, it's not uncommon to see that in children. Again, more likely to have some sort of hearing loss component to that syndrome.

A hemifacial microsomia, so we put this on here because it's the second most common facial anomaly after the cleft lip and palate, occurring in one to 3500 or 4500 births, unilateral or bilateral, and when we see this, again, we're thinking that it's going to involve the ears. And so that degree of hearing loss really depends on how much of that ear structure is involved with that case, and so just making sure that we're documenting, following and monitoring these children.

And then the last of the categories of these kind of noncleft craniofacial anomalies are craniosynostosis. So it's the premature fusion of the skull bones and it can lead to maldevelopment of the cranial cavity and other cranial features, and so again, when we're seeing this, it can certainly occur in isolation, but it's not uncommon to be associated with a syndrome. 15 to 40%. And that's a wide range with that. And so again, you may see it in isolation, or we may be thinking that it's in conjunction with another syndrome, and so when we look at these other syndromes, whether it's Apert or proson, again, those are all associated with hearing loss and so putting all these kind of pieces of the puzzle together and making sure that we figure out what's the best way to track and monitor these children. So again, it may not be that this craniofacial anomaly causes the hearing loss, but it may be that it's in conjunction with something else that does.

So now that we've kind of reviewed what some of those craniofacial anomalies are, how does this relate and tie in to EHDI? How do we make sure that we're hitting all of these components when we're talking about this specialized population of children who have a craniofacial anomaly? So if we look at the is JCIH recommendations, we already know that children that fall into this category are at risk for changes in their hearing, whether it's present at birth, delayed onset or things like that, and so we know, you know, I think the biggest message I'd like to say is just when you see children that fall into this category that we should automatically be thinking of EHDI, making sure that they're getting connected because that hearing loss may not be there right away, but we know that it can be there. We know that it can change. It could be more in that fluctuating category or it could be something that's more in the permanent category, and so how do we, as audiologists, how do we as providers, make sure that we're meeting all of these components of EHDI with this very specialized population.

So of course we have to look at the 136 model. And so again, we talked about that they're at increased risk, making sure that we're monitoring, managing them, looking at what these other program goals are, providing that support, that education for families, and then again thinking craniofacial anomaly. We should automatically be thinking of EHDI. So what does this look like? How is that 136 model affected? Well, if we've got a child who's born with some sort of craniofacial anomaly, depending on the severity of that, maybe they were born premature, maybe they're still in the NICU. How quickly are we getting that screening done? Are we getting it done at one month of age? If we've got an extreme premie, no, we're waiting until they're much older to get that testing done. It could be a child that was born full term but maybe they're still in the NICU for other reasons for feeding or growing with their cleft palate or things like that, and again, or certainly if there's something structurally going on maybe they need a distractor to help with their mandibular hyperplasia, so maybe they've had their jaw surgery, they still have the distractors in, they're not ready for the hearing screening to be done. That puts them past that kind of onemonth mark for getting the screening. Then looking at do we get a diagnostic test done by three months of age? Again, if they're still in the hospital, if the family has multiple appointments, complex medical care, all of that impacts how quickly, how soon we're getting all of this testing done.

And then certainly on to that sixmonth mark of are they getting into early intervention? Again, if they're still in the hospital, that kind of puts delays all of that, and so really looking at this population and how are we doing with hitting that mark and what are we doing with that when we talk about the importance of early identification, again, making sure that we have access to these children right away, and as soon as possible, and really, what's the role of the audiologist in this model? Where do we fall? How are we you know, sometimes we can be the gate keepers with some of these patients so how do we make sure that they're steered in the right direction and getting the care that they need and making sure, again, that we're hitting all of those components of EHDI?

So when looking at this, I had to take a look at our own facility. So what are we currently doing? How are we implementing that? And so on the screen is a list of kind of the team members that make up our craniofacial clinic team, and those that are listed with an asterisk are the ones that are kind of on call. So everyone else, they're seen that day by the whole team. The ones that have an asterisk are kind of called in for certain patients. They may see them in their own clinic, down in ophthalmology or certainly if we need them to come up that day for that particular patient, they may be seen kind of with the whole team, so just kind of differentiating that and so we really had to take a look at what is our current structure, how does this fall in to place, what is the protocol, what is the guideline when we're looking at these children, how does that fit into our team model with our what was currently happening on the craniofacial clinic team.

And so in that, it kind of led to again, what is the role of the audiologist? What's being implemented? Where can we improve upon this? And so when looking at what role we play, previously or historically, audiology is kind of a silent partner on the team. And so whenever children were referred for hearing test, it was kind of physiciandriven, maybe from the ENT or the plastic surgeon of how often or when they needed a hearing test, or maybe occasionally the parents might request that, but it wasn't really steered or directed from the audiologist.

And then what was implemented, of course, routine hearing test, assessments, looking at what amplification is needed, fitting them of their amplification, doing the followup testing for the newborn hearing screenings. But again, you know, some of it was managed by audiology, but more of it was really driven by other team members on that, and some of it was logistics, when they were holding the craniofacial clinic team, it was on a day when there was a high volume pediatric ENT clinic going on and so there weren't as many resources for the audiologist to participate actively on that team, and so a lot of this required education and really a structural change in our model, and then how could we improve on that? How can we make sure that we're getting these children in sooner? How can we coordinate their care? And so in looking at all of this, it really led us to what is best practice for treating children with a craniofacial anomaly. And so in doing that, where do we go? What is the information?

So naturally starting with kind of our organizations, AAA, ASHA, what's out there? What information tells us how soon we should bring them back, how often should we follow up with them, and so there's very limited information with this specialized population. There are a few guidelines kind of here or there, but there's really nothing that says specifically you should bring them back at this time, this is what frequency information you should get, things like that. And so it kind of, again, just looking at what is the next step, where should we go, and knowing that these children are at more of an increased risk. We need to find out what those answers are. So the next step was looking, okay, we looked at ASHA, looked at AAA, looked at the research. What about the American Cleft Palatecraniofacial Association, what does that say about how often these children should be monitored? Not very much. The only recommendation is having a diagnostic test ear specific by three months of age. Great. What about after that? What do we do next? And then the only other kind of main recommendation they talked about is having an audiologist participate on the craniofacial team. So great. We need to have an audiologist on your team. Get a test done by three months of age, and then I guess call it a day from there. But so we didn't really stop there. We kind of continued to look and try to find out what that information is. And certainly if you go on to their Web site, they have a lot of materials that talk about, of course, screening and monitoring these children, so but there's not really anything specific other than having that diagnostic test, earspecific, three months of age and having an audiologist on your team.

So then we started to look at what are other facilities doing, benchmarking with other teams, benchmarking with other children's hospitals, pediatric audiologists to find out what was going on. And so we came across this document that came out of the British Columbia in Canada, and so they kind of had the same questions. They looked at the research. Looked at the recommendation and also looked as what was currently being done in their territory, and what they found was just looking at the ACPA guideline, that that wasn't even being met across the territory. So these children were not getting a diagnostic test earspecific by three months of age. And then they found that audiologists were recommending kind of a wide variety of things. There's no consistency or standardization across the field. And then there was really no distinct protocol, so that led them to develop the audiology clinical practice guideline for cleft palate/craniofacial and syndromic patients. And so you can actually find the document online. It's a 44page document that you can find that kind of lays it out, and what's nice about this is that it kind of puts it into the category of, you know, children who have a cleft palate, children who are being followed for tubes, how often you should monitor them, certainly if they have a syndrome, kind of what track to put them on, and so that's kind of the closest thing that we have in our field right now to having specifics on how often or how soon we should be following and monitoring these children.