Download Topical Session 6-2160

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EHDI
ROYAL PALM 5/6
SPECIAL POPULATIONS:
AN AUDIOLOGIC APPROACH TO MANAGING CHILDREN WITH CRANIOFACIAL
ANOMALIES
MARCH 15, 2016
2:15 P.M.
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ALTERNATIVE COMMUNICATION SERVICES, LLC
P.O. BOX 278
LOMBARD, IL 60148
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This is being provided in a rough-draft format.
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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
over-testing 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, non-cleft. 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 follow-up, 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
non-cleft 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 1-3-6 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 1-3-6 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 one-month 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 six-month 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 physician-driven, 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 follow-up 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 Palate-craniofacial
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, ear-specific, 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
ear-specific 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 44-page 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.
So just kind of an overview of what this included. Including
incorporating high frequency tympanometry, acoustic reflexes,
having that test done at three months of age, the diagnostic
test, regardless of if they passed their newborn hearing test so
that we can get further information, having that baseline test
so that you have something to compare to and monitor and track
moving forward.
And so the next slide here just really talks about some of
those specifics, and so again, we were just looking for answers,
looking for best practice for this population, and so taking it
a step beyond the newborn hearing test, so getting a diagnostic,
ear-specific, three months, recommending ear conduction 5, 2 and
4 and also if that's abnormal or elevated, getting
frequency-specific bone conduction, and not just relying on the
click ABR, but getting more frequency information for these
children, and then, of course, incorporating as they get older,
a structured sequence. So again, really looking at children
that fall into the craniofacial anomaly, so having a test, their
newborn hearing test, having a test at three months, then the
next one being at nine months and then annually between the ages
of two and six. And then kind of after that, it's kind of left
more to the discretion of the audiologist or certainly depending
on what's going on with the child, if they have a permanent
hearing loss. Then that's going to change how often or how
quickly they're being followed versus a child who still
continues to have normal hearing or things like that.
And so I think a big change for us in implementing kind of
some changes to our protocol would be incorporating more
reflexes, so it's definitely something that we do at our
facility often, but maybe we're not always trying it on the
nine-month-old or things like that, but looking at what else
could we be doing to make sure that we're truly assessing their
middle ear or inner ear status and there are guidelines talking
about if you've tried a couple of times and you're unsuccessful
moving to that sedative procedure. I know that you guys, I'm
sure, see it a lot that there may be four, five, six, seven
attempts before we are really determining what that hearing
status is, and so making sure when we're talking about a
population that we already know is at risk for decrease in
hearing sensitivity, that we're not waiting. That we're not
continuing to bring the family back three, four, five, six,
seven times before moving to trying to get further testing or
even coordinating that testing with other procedures.
And then, of course, if the child has conductive loss, we're
making sure that we're referring them to their PCP or if they're
already kind of a part of a craniofacial team, making sure that
the ENT is involved with that and then making sure to do testing
after tubes are placed. We see this probably weekly in our
facility, where we'll have a child that's referred to us that's
had tubes in their ears. They didn't have a test before the
tubes were place and they certainly haven't had a test after the
tubes were placed, so making sure, again, that that's a part of
our protocol, that we're incorporating all of that information
into the testing that we're doing with these children.
So then how does that relate to, again, bringing it back to
our own facility and what we're doing, what kind of changes did
we need to make for that? So again, looking at what role does
audiology currently play? We know before it was kind of more of
a silent partner. Now after we've kind of made some changes,
what role are we playing? And we're definitely an active
participant on a craniofacial team. We're more visible. I can
say from having these conversations and I joined the team a
little over a year ago, actually having these conversations with
the team, making sure that they understand what our role is,
what we're trying to do, how we're trying to monitor them, and
now it's kind of the complete opposite. Now it's no child
leaves the building without making sure they have an idea of
what their hearing is, even if it's not a test that day, but
making sure it is documented in all of their reports. I've had
the geneticist tracking us down to make sure, just wanting to
make sure, you know, do you need to see them, do you not need to
see them, things like that so it's been great to have that
widely accepted by our team and that they really wanted
audiology to step up in that role because that's not their
field, it's not their expertise, and so for us it was kind of an
easy paradigm shift in that.
Another thing that we noticed was that, you know, we have a
NICU and a lot of times these families were meeting the whole
team in the NICU. They were meeting the plastic surgeon, they
were meeting the speech therapist to talk about feeding and
swallowing. We have hearing technicians that do our screenings
in the NICU, but they weren't really provided with other
information about that we're going to be following your child.
We're going to see you when you come in for your craniofacial
clinic visit and things like that and so we started
incorporating that we go over and actually introduce ourselves
to the family and provide them with some information and
incorporating, you know, when they get their packet about
surgeries with their lip and palate, again, there's information
about hearing and audiology included in that. And so parents
are able to get that early on, and so it's not a shock or
surprise when they come to that first visit that, oh, we're
going to be checking in asking about your ears during those
visits.
It allowed us to co-treat with patients. So they come in,
especially a child who has a cleft palate, maybe they're having
a visit with speech to talk about feeding and they're doing, you
know, part of the requirements is they wait to feed them until
they come to that appointment so they can work with the speech
therapist on different bottles and the different nipples to use
with that and so let's set you up in the ABR room to do that so
you have your feeding. You're wearing the child out. We hook
them up for the ABR, we're combining these appointments, so
we've had a lot more success in trying to get that testing done
and obtaining this information by coordinating that care. And
part of that is just having those conversations, having that
dialogue, looking at what our current structure was, and so
those have been kind of two big things, and then streamlining
how often we're following them. So if we're recommending
testing annually from 2 to 6, you know, if the child is coming
back at 9 months, we're not going to bring them back for a
separate appointment. Maybe we coordinate it with that
nine-month appointment, and then maybe they're going to have an
ENT appointment if they have tubes in their ears, and another
six months, again, making sure that we're coordinating, even if
it's not just with the craniofacial team, but with their other
appointments so that we can get the information that we need and
really pushing for more of that ear-specific and kind of not
being satisfied with that sound field testing with those younger
ages and certainly we're not talking about the kids that are
super cooperative that you can get all of that but really
pushing those other kids to get more information and looking at
what other follow-up appointments they have and, you know,
trying to combine our testing as a part of that and then, you
know, really that first year of life, really managing their
middle ear status so even if we're not doing a full hearing
test, can we get temps, can we get reflexes, if the child is set
to go for their lip repair, could we do temps and OEs and
reflexes, even if it's not a full AVR to see, okay, they're
about to go under, do they still need tubes and things like
that, do they still have that fluid that's present, when they're
looking at moving forward. Again, coordinating that hearing
test before that so that if they're going to be put under for
something and they need additional services, do we have an idea
of what they're hearing before any of that happens to say okay,
maybe they need some other intervention, maybe we need testing
or things like that so we don't have to schedule separate
appointments for these children.
And then again, what are the outcomes? And so right now,
after putting these changes in place, that's what we're kind of
sitting down and doing is looking at how much information we've
collected, what does that look like, are there other changes
that we need to make for this population.
So then very quickly, because I know our time is almost up,
so I pulled out looking at children with Pierre Robin sequence,
because it is the most common anatomic deviation associated with
a non-isolated cleft. And so I also pulled this out because we
have a current study going on with this population. And so, you
know, when we look at Pierre Robin sequence, it's a sequence
because it starts with the kind of under-development of the
lower jaw, the mandible and then kind of goes on from there
with, you know, displacement of the tongue, and then the
formation of the palate. It can also cause airway obstructions.
With that. And so population-wise, it occurs in one in every
8500 to 14,000 births. Again, kind of a wide range. And then a
pretty high occurrence in twins. And I will say we have a high
population of Pierre Robin sequence children, and a lot of those
are twins, and so again, looking at what's the best management
in monitoring and how are we then following a family that has
two that fall into this category, and when we're doing testing
for that.
And then also just keeping in mind that it can be associated
with other syndromes which goes along with hearing loss.
So just very briefly, it's a retrospective study, so it
includes 34 patients all between the ages of zero and three that
have Pierre Robin or they're a non-cleft patient so that we can
compare the two, and so it's kind of a multi-disciplinary study
so each kind of member of the team is looking at different
things across this entire group. So for example, the speech
therapist is looking at the feeding. So all of these patients
had distractors as part of their treatment, so they all
underwent surgery while they're in the NICU, so they're looking
at pre- and post-surgically, how soon does that child return to
PO feeds, feeding by mouth after they had their surgery and the
time it takes from all of that. From the audiology standpoint,
we're looking at, you know, what are their newborn hearing
screening results? Did we get follow-up testing by that
three-month mark? What kind of treatment, intervention, or
things like that that are in place looking at this population.
And so this is just very, again, just a brief overview. So
we've got 34 as our N and of that, 19 have Pierre Robin sequence
and then looking at that category, again, they all fall within
that zero to three age group. Five of them have an additional
syndrome along with the PRS, whether it's cerebral palsy,
stickler. One of the patients has craniosynostosis, on top of
everything that goes along with that syndrome. Looking at kind
of those 19, eight of them -- only eight of them passed their
hearing screening prior to discharge from the NICU, and then of
those eight, five of them had a normal ABR by that three-month
mark, so very few were passing and continuing to pass.
Of the population of the 19, eight of them have a hearing
loss. Four falling into the sensorineural category, two that
are mixed, and two that have a permanent conductive, and all
eight of them are using some form of amplification, whether it's
hearing needs or an osseointegrated device, and then I thought
it was very just to note that of those 19 that have the PRS, 15
of them have a history of tubes or multiple sets of tubes. So
that means of the 19, only four to date don't have tubes, and
that doesn't mean that they won't be getting a set, or that
they're not about to get a set but at the time that we were
collecting this information, there were only four out of 19, so
that's a pretty high number. Again, that just goes along with
why we need to monitoring and follow them pretty closely so that
we're able to participate and help manage their care.
>> AUDIENCE MEMBER: [Inaudible].
>> BRITTNEY SPROUSE: Yep. So that was pretty much the end.
If there are any questions.
[Applause]
>> BRITTNEY SPROUSE: Thank you.
>> MODERATOR: Thank you so much for your presentation. If
you have any questions, please feel free to ask her. So thank
you so much. And please remember to pass your evaluations in to
me.
[Session concluded]