Speech Disorders – Nasal Speech & Velopharyngeal Dysfunction – PediaCast 289
Dr Adriane Baylis joins Dr Mike in the PediaCast Studio for a discussion on nasal speech, resonance speech disorders and velopharyngeal dysfunction. These may sound like confusing terms, but we’ll break the problems down into understandable language and cover their cause, symptoms, diagnosis, treatment, complications, long-term outlook… and the latest research topics!
Resonance Speech Disorders
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Announcer 1: This is PediaCast.
Announcer 2: Welcome to PediaCast, a pediatric podcast for parents. And now direct from the campus of Nationwide Children's here is your host Dr. Mike.
Dr. Mike Patrick Hello everyone and welcome once again to PediaCast. It's a pediatric podcast for moms and dads. This is Dr. Mike coming to you from the campus of Nationwide Children's Hospital. We're in Columbus, Ohio, it is June 11, 2014episode 289. We're calling this one "Speech Disorders", "Nasal Speech", and "Velopharyngeal Dysfunction." Now I realize that's a big word and we'll break it down in just a couple of minutes. First I just want to give a shout out real quick to the Columbus' zoo.
Now I know we have a lot of listeners in Central Ohio but there's also in fact a larger number of you who are not in Central Ohio. And if you find yourself in Columbus, one of the must stop's is the Columbus zoo. It's really the zoo that Jack Hanna built and have a new Africa exhibit, I guess you'd say a new section of the zoo that they opened. And we went as a family a few days ago and it was really quite amazing, really exceeded my expectations for what I thought it would be. They have these cheetahs that when they were little cheetahs, they had yellow labs that they have dogs that have grown up with the cheetahs since they were puppies and cubs together. And so they're like buddies and they chase each other around, and it was really quite amazing seeing cheetahs and dogs interact together.
So that was pretty cool, and then they've got this basically it looks like a savanna and they have the zebra, and the giraffe but it's not just this little enclosure just seems to go on and on. And they've got wildebeest out there and wild hogs and it really looks like you're at an African watering hole. So anyway no kickbacks here from the zoo, I was impressed enough that I think everybody should definitely take a peek at that. It's really cool thing and lot of educational experiences available. Anyway the Columbus zoo here in Columbus where you find yourself in Central Ohio, makes sure you check that out. Alright, so as the title suggests, we're talking about nasal speech and velopharyngeal dysfunction today. And I have to tell you, problems with speech, normal speech, speech therapy, these are all commonly asked questions from you, the PediaCast audience and while I have answered some of your speech questions in the past, I have not yet covered the topic with the full-on nuts and bolts approach like we do for so many pediatric problems.
As I begin thinking about this show, I had this wild idea that we would cover the whole of speech disorders in one podcast. But as I begin researching the topic I quickly realized, I'm not fitting all of that into a single show, not everyone to keep the length of the podcast reasonable. There's really a lot of territory to cover with speech issues which is why speech and language pathologist go to school for a long time to do what they do. So then I thought I have to break this down. So today we're going to cover the resonance disorders. So this is nasal speech and velopharyngeal dysfunction, and again we'll get to exactly what those things are. But look at this as part one because I do have plans to cover normal speech, speech development, apraxia, articulation, voice disorders down the road. It's a long road but we'll get there eventually.
And of course in our usual PediaCast fashion I have an excellent studio guest today to help me cover these disorders. Dr. Adriane Baylis is a speech scientist at Nationwide Children's Hospital. We'll get her settled in to the studio in a moment but first I want to remind you about the 700 Children's blog, it's over at 700childrens.org. Lots of great pediatric topics lined up for you there including "What to Think of Your Child's Weight?" "Safe Airplane Travel with Your Child," "Anxiety and Food Allergies," "What is Allergy Induced Asthma?" And "Treating Crohns Disease with Nutrition Therapy." So be sure to check out our sibling blog, 700 Children's, you can find it 700childrens.org. By the way I have a three parts series coming up over there on "Home Made Baby Food," "Dangers and Safety Tips," so stay tuned for that in the very near future. That site again, 700childrens.org. Why 700 Children's? Well that's been the address of the hospital since about as far back as anyone can remember.
Dr. Mike Patrick We are back. Dr. Adriane Baylis is a speech scientist and director of the Velopharyngeal Dysfunction Program at Nationwide Children's Hospital. Now I realize velopharyngeal dysfunction, the disease process it represents is actually easy to understand and Dr. Baylis is going to break it down for us in just a few minutes. In addition to her role at Nationwide Children's, Dr. Baylis serves as an assistant professor of clinical plastic surgery, speech and hearing science, and pediatrics at the Ohio State University College of Medicine. This is her second appearance on PediaCast. Dr. Baylis who's also a co-director of our 22Q Center.
She joined me a couple of years ago and participated into discussion on the 22Q syndrome that was PediaCast episode 226 so you can look that one up if you're interested. She joins me again today in the PediaCast studio, this time to talk about resonance disorders of speech, nasal speech and velopharyngeal dysfunction.
Dr. Mike Patrick Really appreciate you stopping by. So have you been to the Columbus zoo since they opened this new Africa exhibit?
Dr. Baylis: Yes we go I'd say every other month with my family and I have two little boys and they love the zoo. My 18 month old is really at the stage of making all the animal sounds, so I want to go back for another trip soon.
Dr. Mike Patrick Do they have the dogs with the cheetahs when you were there?
Dr. Baylis: We were there last right before the exhibit opened and they have one little pen with one cheetah and one dog in it and they were just hanging out, taking a nap together.
Dr. Mike Patrick It was amazing, I mean when I walked up and I saw the dog in there I thought, "Oh, the cheetahs are off display so they put a zoo dog I guess in there." And I was like, "No, wow those cheetahs are in there with the dog," it was pretty amazing.
Dr. Baylis: Definitely.
Dr. Mike Patrick Since this episode, we're talking about speech issues and as I mentioned in the beginning I wanted to focus down on some specific speech problems. And nasal speech, or resonance disorders, or velopharyngeal dysfunction, I guess let's talk first about what is resonance and what is it that would make up nasal speech, what's normal resonance?
Dr. Baylis: Resonance is one of the sub-systems of speech. So if you think about the speech mechanism or the vocal tract as a series of different sub-systems that when working all together gives us speech output. So what happens is, we generate airflow from our lungs that goes up into our vocal chords or our larynx and then that creates a tone or our voice signal, and then that further travels up towards our nose and mouth and then resonates.
So that's where resonance occurs as after that tone from your voice begins to move up your throat towards your nose and mouth are kind of echoes. And then from there it transmits out primarily our mouths for normal speech and then you articulate. And so then create bursts of sound or restrictions of airflow, or shaping of your mouth to create different vowel sounds to then produce intelligible or clear speech. So resonance is one of those sub-systems.
Dr. Mike Patrick Great. So really what happens between sound production, and then articulation of that sound into speech and it gives a quality to the voice.
Dr. Baylis: Exactly. So if you have too much of that sound energy from the voice that travels up toward the nose, you have too much nasal resonance or we call that hypernasality and then the contrary being if you don't have enough sound that goes toward your nose when it should then you have hyponasality which is denasal or too little nasality in speech. We've all been hyponasal in our lives, probably several of us many times a year when you have a cold and you just can't get any sound up in your nose.
You sound really stuffy but hypernasality is more of a disordered speech quality that tends to be something that children might be born with, or that someone could develop later on in life.
Dr. Mike Patrick So then this is what we would mean by the term resonance disorders, when there is an abnormal amount of resonance in the voice whether it's too little nasal resonance or too much.
Dr. Baylis: Correct. And usually we're more concern with hypernasality or too much sound coming out of the nose versus hyponasality which is usually temporary or commits too to more common things like enlarge tonsils and adenoids and congestion.
Dr. Mike Patrick Now one of the terms that we have introduced here is velopharyngeal dysfunction and there's something called the velopharyngeal valve. What exactly is that, and how is that related to resonance?
Dr. Baylis: The velopharyngeal valve is basically like the door, or the trap door that closes off your nose from your mouth when you talk. And in addition, it closes off your nose and mouth when you swallow too so you don't have food or liquid escaping out of your nose when you swallow.
But for speech what happens is, all the sounds in english should come primarily out of our mouths when we talk except for M and N, those are nasal sounds. But all the other sounds need to have all the energy, the pressure, the airflow come out of our mouths. And if you have a leak behind your palette, then you'll have that escape out of the nose. So the velopharyngeal valve is created by the soft palette which is the very back of the roof of your mouth that's kind of fleshy or muscular, you can feel it with the top of your tongue if you kind of run your tongue on the roof of your mouth. So that's your soft palette, that's the primary part of this valve, but in addition your throat muscles squeeze in around your soft palette which we don't feel as we're talking but they're actually hugging up around your palette when you speak to close down that valve, or that door way.
Dr. Mike Patrick This is really just something that's going regulate how much air, and so sound energy that enters into the nose since the nose and the back of the mouth are connected.
Dr. Baylis: Correct.
Dr. Mike Patrick So what is then is velopharyngeal dysfunction?
Dr. Baylis: Simply stated, velopharyngeal dysfunction or VPD for short is when this valve doesn't close properly. It could be that the valve doesn't close all the way, so there's a partial leak, or it could be that the valve, and there's little to no movement, and there's a very large opening or gap back there. And VPD can be structural or can be functional. So when I say structural, I'm talking more of a tissue deficiency and so we use the specific term VP insufficiency. Meaning that maybe the palette's too short and it just can't reach the back of the throat when it needs to try to close that valve off. And then the functional side we call VP incompetency and that's when the palette tissue and length all might be fine, the structures look normal but they don't move enough. And so even though that valve is trying to close, it just doesn't have enough effort or movement left in it to shut down that valve to push all speech out of the mouth. So it can be either of those, and some people have a combination of both problems together.
Dr. Mike Patrick When it's the incompetence, you have what you need to close it but it's not closing, is that something then that would be like a neuromuscular type disorder, or something you could be born with, or maybe you've had a stroke or something like that that could cause muscle weakness?
Dr. Baylis: Exactly. It could be all of those issues. Dysarthria is the name of the motor speech disorder that includes basically any weakness or problem with speech motor function. Having VP incompetency is part of having dysarthria for speech. Adults who have neurogenic conditions like ALS can present with something like VPD. In the world of pediatrics where I kind of live, we see there's more neuromuscular based VPD's and kids who've had brain stem tumors, stroke, traumatic brain injury, or kids that are just born with congenital velopharyngeal weakness or dysfunction.
Dr. Mike Patrick Do you ever see normal structure and normal function but there's still be an issue with the valve maybe from children not learning to close it correctly?
Dr. Baylis: Yes, that's a great question. There's a little known third category of VPD that we call VP mislearning and it's actually an articulation disorder that kind of masquerade SVPD because it sounds a little bit like it.
So when you have VP mislearning the child may have normal structure, normal function however they've learned to produce certain speech sounds out of their nose as a habit and I can mimic it for you today. Usually we hear sounds like P's, and B's, and D's coming out normally. So they can say, "Pet a puppy," or "Daddy did it," just as clear as anybody else. But then when they get to the continuous sounds, like the F or the S, they've learned to push those sounds out of their nose as a habit. And so instead of saying sissy they'll say [0:14:41.8 Speaking through nose] or instead of fifty five, [0:14:43.0 Speaking through nose] and they're actually pushing those sounds out of their nose because that's how they think they're supposed to do them, so it's just a learned problem that can be untaught or taught to be remediated through speech therapy. So we do see kids with that, they're often referred into our clinic for suspicion of VPD which is appropriate so that we can make the right diagnosis and decide this is definitely just something speech therapy alone can treat or not.
Dr. Mike Patrick Now how common are these kind of disorders?
Dr. Baylis: We don't have great statistics that tack how many children per year are diagnosed with resonance disorders or VPD. We know a little bit about some populations specific information, so we know that children with cleft palate are the highest risk group that can present with VPD, and about 20% of children born with cleft palate even after it's repaired will go on to still demonstrate VPD into their pre-school and school age years which is one that's typically treated. Children with 22q deletion syndrome are also a very high risk pool of patients that can present with VPD as well. And then we see other congenital syndromes, so children with kabuki syndrome, children with hemifacial microsomia which goes by a couple of other names like OAV, oculoauriculovertebral spectrum or goldenhar.
In other children moebius syndrome, the list goes on and on but craniofacial anomalies and syndromes are really where the predominant of children with this condition will present. In addition, interestingly there's also a small percentage of kids who can develop VPD after they have an adenoidectomy. And so prior to adenoidectomy most of the time parents are counseled that it's a remote possibility of surgery. But it occurs and probably one out of every 700 to a thousand adenoidectomies that are performed. So it's quite uncommon but when it does occur it can be long standing, may require additional management.
Dr. Mike Patrick In talking about the epidemiology of this, I just want to bring in one thing and a lot of doctors out there have heard they were switching from ICD9 to ICD10 which is just a way that we come up with diagnosis's. And the original system was done like back in the 70's and so some of these disorders I think that maybe will get better epidemiology moving forward as we go to this new system because you'll be able to find out exactly how many people are getting this diagnosis where that may be more difficult today.
So even though a lot of doctors or it's kind of a pain to move to a new code, well it's probably more painful for the coders than it is for the doctors but this is where we might get some help with the epidemiology of things. So I guess parents hear about ICD9 or ICD10 that what's all about.
Dr. Baylis: Accurate diagnosis's is very important for tracking as well as for making the right management plan.
Dr. Mike Patrick So we talked about underlying medical conditions commonly associated with velopharyngeal dysfunction. And 22Q you've mentioned is one of the risk factors and really is that because of cleft palette that you can see in 22Q or are there other reasons too?
Dr. Baylis: It's usually other reasons, only about 5-10% of kids with 22Q deletion syndrome are actually have a visible cleft palette that requires initial surgical repair.
The vast majority of kids with 22Q will have a sub-mucus cleft palette which is a more subtle presentation of a cleft or actually normal palatal structures that's just don't move while they have more of that VP incompetence or functional problem. The other unique issue with 22Q is that even if their palatal structures are normal and the palette length is normal, we know that the pharynx or the back of the throat tends to be set further back in those children and so the palette, even if it's trying to move often can't reach that back target to touch and separate the nose and mouth. So the long word is palatopharyngeal disproportion, meaning the palette and the throats are kind of inaccurately matched if you will so they can't reach each other to close down that valve. So that's a unique finding in 22Q that seems to be a predominant feature of those kids that have VPD with that syndrome.
Dr. Mike Patrick I guess this is a take home then for doctors out there who are listening. Family practice doctors, pediatricians, nurse practitioners that you may look into kid's mouth and everything looks normal but there's still could be an issue with the velopharyngeal valve.
Dr. Baylis: Please always still consider referring if there's a speech concern even if the mouth looks normal. We have countless kids that we've seen in the clinic who have been turned away by physicians that looked in the mouth and said everything looks OK even though the child had air coming out of their nose, or their speech was really nasally but they thought if it looks normal it must be OK, but clearly that's not the case it can be a functional problem that needs more advanced diagnostic techniques to identify.
Dr. Mike Patrick Are there any other signs or symptoms that parents and doctors should be on the lookout for, or is it just that hypernasal sound to the speech and sounds coming out the nose, or is it more than that?
Dr. Baylis: There's kind of four a different feature or symptoms. So the first was hypernasality as you mentioned which is that kind of constant tone of nasality in speech. Then the second one is audible nasal emission which is actually separate. That's puffs of air coming out of the nose, so instead of puppy you get [0:19:55.1 Speaking through the nose] where you can actually hear a little rashes of air. And then the third one would be reduced pressure.
So the speech sounds are kind of indistinct or muffled, or it sounds like the child's a little bit quieter when they talk and that's because if you can't build pressure with that valve being closed, you're going to lose it out of your nose and then your consonants are going to be less crisper or distinct so you sound kind of muffled. The forth is kind of maybe. So many children who are born with VPD will have this last feature which is abnormal articulation, however children or adults who developed VPD a little bit later on often can have completely normal articulation. So it's the congenital cases have some very unique articulatory habits if you will where they start to try to restrict the pressure in other parts of the vocal tract instead of up in the front of their mouth with their lips and tongue like you and I do. So for example a child born with VPD instead of saying the word puppy they might say a-ih, and they're actually using their vocal folds to articulate instead of using the rest of their mouth. So that's a unique habit we see in kids born with VPD.
Dr. Mike Patrick Are there other things that could cause a similar set of speech issues? I mean is there a differential diagnosis to this? I suppose that they're probably is, what would be included there?
Dr. Baylis: The most common, I call them perceptual confusions if you will that kind of sound like VPD. So we talked about VP mislearning and that's the first thing we rule out is are there some learned articulatory habits that sound like VPD meaning sounds like the child just pushing air out of the nose as a learned habit. So that's one that we see a lot of, and then the second would be apraxia of speech. There are a lot of children that are mislabeled as having apraxia that actually may in fact have VP dysfunction. I think the source of confusion is that, kids with apraxia were classically reported to kind of not move their mouth a lot when they talk. And if a child has some of these more a typical articulatory habits with VPD where they also don't tend to move their mouth very much, there can be diagnostic confusion as to whether that's apraxia. In addition apraxia includes children who've very hard to understand speech, they're highly unintelligible, and children with VPD can be the same as well and both tend to be more challenging to work with in speech therapy.
So I think there's some habits or patterns that co-occur in both but it's really important that a trained speech pathologist particularly one working with a cleft craniofacial program rules out the possibility of VPD if beyond some of the articulatory habits, there's any evidence of nasal speech or air coming out of the nose.
Dr. Mike Patrick Now when we say apraxia, and I don't want go off no another rabbit hole, but what is the general definition of that term?
Dr. Baylis: Apraxia is a speech production disorder that's caused by abnormal speech motor programming or planning. So basically the brain knows what it wants the mouth should do but somewhere along the way the message gets jumbled or doesn't get down to the muscles to tell them how to produce the sounds. So it's a programming or a planning issue. So the muscles of the mouth are normal, there's no weakness, they might be somewhat dis-coordinated as a term that's been brought up but it's more so that the program or the message is not telling the muscles how to execute speech production in the right order or in the right way.
Dr. Mike Patrick And so stay tuned for that because that's a whole other.
Dr. Baylis: Very hot topic in speech pathology.
Dr. Mike Patrick And a whole other episode which we will get to down the road. So then how would you go about telling the difference between these various things? So what exactly would a speech pathologist do who is trained in particular to diagnose PPD, how do you go about doing that?
Dr. Baylis: So for a standard speech evaluation that we would do with any child, we engage them in some conversational speech. So these are usually three, four, five year olds that were really zoning in on the diagnosis of VPD where we have them talk out loud, repeat some words, name some pictures and repeat some sentences. For younger children, we can adopt that protocol down to something that's easier, might be just single words or naming pictures. But typically the confirmed diagnosis of VPD is not made till kids are closer to three or four years of age because it's a speech diagnosis and they need enough speech output so that we can make confirmation that this is what's going on.
Dr. Mike Patrick And then how do you actually evaluate velopharyngeal valve to see if it's really closing or not.
Dr. Baylis: So after we do the listening speech evaluation, we can also do some acoustic testing or some aero-dynamic testing so we can measure the sound objectively with the computer and we can also measure any air coming out of the nose objectively too. And then we go to imaging. So to image that velopharyngeal valve, there's two primary approaches that are used in the US. The first and more common one is called nasopharyngoscopy, and basically we take a very small camera, it looks like black spaghetti noodle and we place it into the child's nose a couple of millimeters and then it peeks over the top of the palette or the top of that velopharyngeal valve. The child has to be awake and talking in cooperative, however we have about a 95% success rate with getting children to be able to complete these exams successfully and all they do with that camera in place is say some words and sentences for about one minute. We video record the whole thing and then we have everything we need to make a treatment plan.
Dr. Mike Patrick You make it sound so easy.
Dr. Baylis: It felt we'd always was. But generally the kids do quite good, they usually sometimes like to watch the video screen and actually see how that works in the back of their throat.
Dr. Mike Patrick Yeah, but they're fascinated by that.
Dr. Baylis: It's pretty interesting if you've never seen how the back of your throat closes when you talk. It's kind of this forgotten mechanism that we don't think about when we talk.
Dr. Mike Patrick Yeah, and especially that the part about with the back of the throat kind of wrapping around the soft palette is you don't think of that.
Dr. Baylis: And then the other approach that's used although less commonly here is called videofluoroscopic. It's the same technology used for a swallow study. So children who have swallowing disorders may have the same type of procedure. But basically it's a movie x-ray. The radiologist takes pictures from a couple different views while the child talks with a little bit of barium that kind of lights up the structure so we can see them better on the x-ray. And again the child produces speech and that's all video recorded and interpreted later. We think image qualities best with nasopharyngoscopy.
We have live in-color pictures and we can make treatment plan right there, sometimes the videofluoroscopy exams little more interpretation later and involve some radiation although it's minimal and we think we get the best pictures with the direct shot.
Dr. Mike Patrick It's kind of an older mechanism for diagnosing this but now that we have thinner, high definition cameras who can get better imaging directly than we used to be able to.
Dr. Baylis: Exactly.
Dr. Mike Patrick So let's say you have done this evaluation and a child you just decide does have velopharyngeal dysfunction. How do you go about treating that?
Dr. Baylis: Well treatment and diagnosis are really an inner disciplinary problem. And so once we got through the speech evaluation and the imaging part, there's also a plastic surgeon involved in our VPD clinic and so he does a medical evaluation of the child, also looks at the mouth, also does the imaging exam with us together. And then we review all that information and come up with a joint treatment plan.
And that will include likely surgery, and we'll talk about some of those options. But it could also include speech therapy in terms of what role will speech therapy play in treatment for that child if any. And then possibly speech prosthesis which are our current kind of none surgical modality that we can use to treat VPD.
Dr. Mike Patrick What exactly is that?
Dr. Baylis: A speech prosthesis? So they're older in terms of that was kind of the first treatment we used to use many decades ago before we had some of the advancements in surgery. Basically it's a retainer with a tail, that's how I describe it to parents. So it looks like a normal pink orthodontic retainer that kids would wear, but it has as extension kind of like a whale tail shapes little paddle at the back of it and it pushes up and lifts up the palette. The child wears it all day when they talk and then they have to take it out at night. it sounds very simple but sometimes it's a real challenge to get kids to enjoy wearing a device that extends into the back of their mouth because they may be a little bit gaggy or not want to cooperate to wear it all the time.
Dr. Mike Patrick I know. My kids don't want to wear their retainer even when it didn't have the tail on.
Dr. Baylis: So we reserve that if someone's not a surgical candidate or there are some other medical reason for why they can't proceed with surgical treatment.
Dr. Mike Patrick Do you ever use it before surgery, or do you usually get surgery schedule pretty quickly?
Dr. Baylis: Usually surgeries are first line, it's highly effective, 85-90% have resolution of VPD or at least significant improvement to the point where speech is judged to be acceptable. So VPD surgery is very reliable when done in the hands of an experienced surgeon and typically these are surgeons that work with our cleft cranial facial programs because they have a lot of experience in palette related surgery. In terms of deciding on a prosthesis or surgery, usually if you know it's one surgery procedure that could potentially remediate this problem for the rest of that child's life. That's often our first choice but that's a discussion we have with the family to make sure it's a good fit.
Dr. Mike Patrick In terms of these specific surgeries, what kind of procedures do you do to fix this problem?
Dr. Baylis: Our plastic surgeons in the clinic Dr. Kirshner and Dr. Pearson who are both cleft cranial facial surgeons as well. There are three main procedures we offer meaning they perform, not me, that's the speech pathologist. But what they do is based on the VP imaging. So we have to look at that exam and we look at the size and the shape of that gap or leak back there, how much movement's occurring, and then we also look at that child's medical history because there are factors related to their syndromic diagnosis, do they have 22Q or not? Do they have history of airway issues in particular because these are surgeries that can affect breathing, in particular breathing out of the nose? So based on all that information they would offer either a furlow z-palatoplasty which is a specialized technique that repairs the palatal muscles and gives it a little bit of length. A pharyngeal flap is sometimes it's called a posterior pharyngeal flap which basically makes a bridge of tissue from the back of the throat and then connects it to the palette.
And then the third being a sphincter pharyngoplasty which takes two little pillars of tissue in the back of your mouth which are behind your tonsils and then disconnects them and then saws them back together in the very back of your throat to kind of singe up or tighten up that VP mechanism while it's at rest. It makes the resting hole or gap smaller to begin with. So they're very different approaches that are individualized to that patient's anatomy and diagnosis.
Dr. Mike Patrick And then just to be complete, you have mentioned chronically enlarged tonsils, or chronically enlarged adenoids could cause a resonance problem, just hypo-nasal speech because you've just got the bulk in there. So a kid who had a chronic issue with that, then tonsillectomy and adenoidectomy may be something to consider?
Dr. Baylis: Possibly. You know it's interesting because usually specially enlarge adenoids because they're filling up that space right where the sound may need to resonate on your M and N sounds in particular. Usually if the problem is that significant for speech. So someone sounds that stuffed up and blocked up that we're worried about speech. There're probably are other medical indications that are more important for why the surgery should occur if it needs to happen.
Dr. Mike Patrick Yes, like obstructive sleep apnea.
Dr. Baylis: Exactly, or drooling, or other breathing problems. And so hyponasality is often treated with our otolaryngologist and they will then do the tonsillectomy, or adenoidectomy, or both. If the child also has other cranial facial anomalies, sometimes we tried very carefully on that or do partial adenoidectomies. For example, if we think this is also a child who we could induce VPD on as well. So they're very well aware of those other co-occurring conditions that require a little adaptation of the treatment plan.
Dr. Mike Patrick Now what kind of complications can stem from a delay in the diagnosis of this? You'd mention that there's probably significant number of kids out there who are labeled as having apraxia who really have VPD. So if those kids go on just to regular speech therapy and you don't really get to the structural or functional problem, what kind of complications can you see down the road?
Dr. Baylis: Certainly the goal will be to allow this child to have normal speech as soon as possible, and the average age nationally for VPD surgeries between four and six. SO our goal is to offer management as soon as the diagnosis is made and then based on age and medical criteria that child's a candidate to proceed with treatment. Things that can hold it back like not getting to the diagnosis till later. Certainly if there are other articulation problems going on such as a child who we think may have apraxia or at least these other very atypical articulation airs. The longer those are not responding to treatment, or perhaps if the wrong type of speech therapy's being utilized because there are differences in techniques we'd use then that disorder will keep persisting longer and longer become harder to remediate. So we know that the longer speech air stay in grain, then that child's pattern becomes a longer term habit. It's going to be harder and harder for that speech therapist to change that and to make that normal let's say in an eight, a nine, and 10 year old, we would much rather fix those speech airs when the child's two, three, four years of age to give them normal speech as early as possible.
Dr. Mike Patrick And I suspect there's also academic and learning issues that can happen if you don't have good speech and communication problems and there could be social issues because other kids are making fun of your speech and that sort of things. So it's definitely something you want to get corrected as soon as you can.
Dr. Baylis: Definitely.
Dr. Mike Patrick Now just to be fair, on this show we always talk about not all of the complications of not treating, but there's also potential complications with treating, that's where we get risk-benefit, we talk about that. What are some of the complications that can stem from surgical treatment of VPD?
Dr. Baylis: Luckily there are few and they are relatively rare which is the good news for management. But the more common we talk about is obstructive sleep apnea and the general risk we think is around 5% of kids who have this velopharyngeal procedure. So the pharyngeal flap and the sphincter pharyngoplasty specifically. But the research studies are still relatively inconclusive, there's a lot of issues with how the studies were designed, and how the patients are tracked, and how long term they've been followed.
So I don't think we have the final answer on that but we see it pretty rarely clinically here which is the good news and we do get sleep studies on a lot of the patients before or after the surgery in particular if they're demonstrating any clinical symptoms after surgery. But parents are always counseled that that's a possibility and certainly right after surgery when the back of the throat's really swollen and things are still healing. A lot of the kids are really heavy snores, they may have some temporary apnea until all that healing and scarring has settled down and then I tend to normalize for most of the patients.
Dr. Mike Patrick And just so parents can understand, if the hole's too big and you're trying to make the hole smaller so that that valve can close better, you're going to get obstructive sleep apnea, I don't want to say that you've over corrected but if there's not enough space then air's going to have trouble getting through and that's going to give you that snoring sound.
Dr. Baylis: That's correct.
Dr. Mike Patrick What about not fixing it enough? So do you still see persistence of the problem even after surgery?
Dr. Baylis: Yes. So there are some children that will need a revision procedure. So if they've had VPD surgery and were around a year post surgery and not satisfied with the outcome, they may be a candidate to have a revision and there's different ways the surgeons can approach that based on what procedures have already been done. In addition we know that children with syndromes who tend to also have hypotonia or any other neuro-muscular problems, they're at a slightly higher risk for needing the revision than children who don't otherwise have a syndrome, or otherwise healthy especially from a muscular status. So there's different for factors that might predict the need to go back and have more surgery but it's important for parents to be aware that if a child did have one of these procedures and the result has not been optimal that sometimes revisions are possible and can give a much better speech result.
Dr. Mike Patrick" Now what about longer term?
So once these is fixed and in the first couple of years let's say that there aren't a need for revision, is this something that usually sticks? I mean is it then fixed for good, or do you see recurrences like longer down the road into the teenage year or adult years?
Dr. Baylis: We don't usually see recurrences meaning over a long period of time that someone comes back five, 10 years later saying, "Now my VPD is back." Usually we know the results within 6-12 months after surgery and they're going to stay pretty stable at that point. Because these procedures reconnect anatomy and then obviously there's still some growth of your throat and your mouth and all those speech structures, the speech mechanism tends to adapt to those changes. So we really don't see it's kind of new VPD or new onset coming back in again later just because of growth. One of the things that can change is adenoids. All of us are born with an adenoid pad that's a size of bulky pad of tissue in the back of our throat and it sits right about where velopharyngeal closure tries to occur especially in children.
So a lot of kids use their big, bulky adenoid when they're younger to help close off this valve and let's say the child has VPD and they do have a surgery like a furlow palatoplasty in particular. Sometimes they could potentially need more surgery later because they might still be using their adenoid pad and then kind of outgrow that but more so it's that the adenoid shrank down or kind of went away too fast or too much for them to tolerate. So the adenoid is more of kind of the unpredictable factor depending on the child's anatomy and surgery history.
Dr. Mike Patrick Is there a way to prevent this from happening in the first place? Probably not since it's mostly structural.
Dr. Baylis: I would say generally no with the exception of the post adenoidectomy. Some of the studies interestingly have shown that the vast majority of cases of VPD after adenoidectomy were in children who already had another pre-disposing factor like 22Q deletion syndrome or an identified sub-mucus clef palette. So it's always important that whoever's performing the adenoid procedure, always check the palette to see if there's any signs of a sub-mucus cleft like a little split in the uvula or to look at medical history and listen for any symptoms of VPD prior to the surgery.
Dr. Mike Patrick Now is the surgery itself considered a cure, or these kids still going to need speech therapy?
Dr. Baylis: I never think of cure in speech therapy, to me that's more of like a medical disease thing but I know what you're getting at. I mean I think we say we effectively manage it in a vast majority of cases like I said 85-90% success rate in the literature for these procedures. But in terms of, is it a 100% gone? Maybe I could find a little blip of air coming out of a child's nose after the surgery. So did I say we cured them or we made their speech certainly acceptable in a whole lot better? So I think it's these grades of change and grades of acceptability because speech is subjective and it's finding the right balance between normalizing resonance, not over correcting because we don't want to make them hypo-nasal by over correcting or inducing some complication like sleep apnea.
So it's this delicate balancing act between airway and speech resonance. So I think we can treat this. In tandem with it is speech therapy. So the children who have VPD but also have articulation airs, they will need to keep going to speech therapy and they should already have been in therapy prior to seeing them in the clinic and diagnosing and treating the VPD too in most cases. But even if they have surgery it doesn't mean all their articulation airs will magically go away. Speech therapy is the tool we use to correct the articulation and surgery's what we use to correct the structure or the VPD itself which is the resonance, the air, and the pressure.
Dr. Mike Patrick And since those things are often present together, you really need both arms of it and so it's not just a surgery, it's done deal. There's still work to be done.
Dr. Baylis: Exactly.
Dr. Mike Patrick What about research in this area. Are there any hot topics right now in terms of research in VPD?
Dr. Baylis: There are few. It's a small group of researchers I'd say that are interested in the topic and a lot of us tend to live in the clef palette craniofacial circles..
I think some of the more current or relevant topics are still we're trying to decide which surgical procedure is "better" and I think every surgeon has their bases, beliefs, and different training. But the two main procedures, the pharyngeal flap and the sphincter pharyngoplasty, I think they will be ongoing debate through the rest of my career as to which one people think is really better and has lower complications with sleep apnea. Because the literature right now is pretty inconclusive and it make things sound like they're pretty even. So generally what I recommend is it's the procedure that's best in the hands of that surgeon and it's usually what you done the most of and what you're best trained in but it also has to take into account all those patient's specific factors. 22Q and treatment of VPD is an ongoing hot topic because traditionally this is a clinical population that's harder to manage, they may need a little more aggressive VPD surgery and what I mean by that is they may need a bulkier flap of tissue for some of the procedures we do because they tend to have larger leaks or larger VP gaps.
And then lastly outcomes. As a whole we're still kind of debating what is a good outcome, and what is that mean, and who should judge that? You know, should it be the speech pathologist, should it be the surgeon, or the parent? And ideally I think it's a combination of all of those but we're really kind of as a field in the area of clef palette, speech, and VPD getting to the point where we're starting to try to do more objective, unbiased outcome assessment.
Dr. Mike Patrick How common are programs that really look just at kids with velopharyngeal dysfunction? I mean obviously we have a program here at Nationwide Children's. Is this something that's common at children's hospitals or is this a unique program?
Dr. Baylis: It's more unique, not every children's hospital will have a program dedicated to this and not all children's hospitals will even have a cleft lip and palette center which is kind of the other place for many of these children may be managed.
Parents should be looking for a program that has specialty program decoded to VPD or at minimum a cleft lip and palette center where there are surgeons trained and experienced as well as speech pathologists trained and experienced in this. The typical speech language pathologist has very minimal training in VP dysfunction and resonance, it is a very small component of required training but they may have never seen a patient with VPD or maybe one or two kids with cleft palette in all of their training. So it's really important to get to a pediatric center where that's where they're seeing a high volume of patients. In Ohio we do have a couple of centers that do see patients with VPD and we do see patients who have cleft palette VPD as well as those that have all these other causes of VPD as well.
Dr. Mike Patrick And I guess this is something that parents whose kids have speech problems especially if there's a hypernasal component or air coming out the nose but even if it's just they have been labeled as apraxia and things aren't getting better with standard speech therapy that's something that they should consider and maybe tried to get to a center with a little more expertise on diagnosing and managing kids who have these problems.
Dr. Baylis: Exactly. We've had kids with that exact same background that the speech pathologist out in the community said, "You know I'm just wondering, I want to make sure I've ruled this out because I have this little suspicion and sometimes it is VPD and sometimes it's not, but at least we have a second set of eyes and ears to just offer an opinion on that."
Dr. Mike Patrick And for speech pathologists that are out there that maybe listening, family practice doctors, pediatricians and parents as well, in the show notes for this episode 289 we will have a link it's called "Connect Now With a Speech Specialist from Nationwide Children's" and will basically take you to our welcome center where parents can put in the information with the problem is and someone from the hospital will get back and just help to arrange the referral or help to facilitate an appointment. So we'll make sure that we have that in the link in the show notes. So in the velopharyngeal dysfunction program, so it's a multidisciplinary clinic, what specialists are involved with this?
Dr. Baylis: At minimum when a child's referred they'll see a craniofacial speech pathologist. So myself or one of my colleagues on our cleft lip and palette team and a plastic surgeon. And in addition to that based on what we have in terms of referral information, it may also include a geneticist as well as an otolaryngologist or even a dental specialist. And that again just depends on history. And so if we're suspicious that the child might have a syndrome that is related to their VPD like 22Q for example then we want genetics to do an evaluation as well or we'll refer for genetic testing because over half of case of VPD will be associated with some type of genetic diagnosis or syndrome. I think having an otolaryngologist is important as well because there's often complimentary needs related to tonsils and adenoids and other hearing and speech issues that they will be able to collaborate on with our team as well. And then also having dental professionals if the child needs a speech prosthesis, they will then check the teeth to make sure that if we do have to have a retainer made, do they have appropriate dental hygiene and tooth support to be able to wear a device in their mouth if that's the pathway we have to take.
So based on it referral information we customize the team visit to that child's needs but all those professionals are available in the clinic. So the beauty of it being inter-disciplinary is that when you come in, you see your specialist all in one day and have your treatment plan that day. It's not a matter of having to go from doctor to doctor and then having people talk to each other, it's all done right there in one setting.
Dr. Mike Patrick And the nice thing with that too since it is a specialized program that a lot of places don't have. A lot of your patients are coming from a distance and so it's really convenient to be able to have one stop shop so to speak and get it all done in one visit rather than multiple ones and lots of trips.
Dr. Baylis: Exactly. We want to make this family friendly because it is a lot of time if you have to do multiple visits with missing school, or work, and things like that.
Dr. Mike Patrick Well we have some great links for listeners. If you head over to pediacast.org and click on the show notes for episode 289, we have a link to the velopharyngeal dysfunction program at Nationwide Children's. Also a link to PediaCast 226, all about 22Q. And I also have a link to the American Speech, Language, Hearing Association because they have lots of great educational information for families there as well. And of course that "Connect now With a Speech Specialist from Nationwide Children's". If you're interested in having your child evaluated here at Nationwide Children's, that's a great way to reach out and we'll get back in touch with you. Alright so before you go, and I think we might have done this the last time you're here and then I haven't done it though for at least a year, maybe more. But in the last episode we had listener that wrote in and said, "Hey what about family games because you used to ask your guest about what kind of games they like to play at home." And so a lot of families were getting suggestions for getting new games and play, having a family game night.
So did we do that last time you're here?
Dr. Baylis: No.
Dr. Mike Patrick It has been a while since I've done it. So the question is just thinking about your family or from your own childhood, what was a favorite game that you guys like to play?
Dr. Baylis: So my kids are still pretty young, they're four and on. So my four year old is really into UNO right now in particular, Jake the Never Land, Pirate UNO in particular and we also have a Jake and the Never land pirates like treasure game. So those are probably the ones we do the most with him. I think we just like to do informal things still like have a dance party where my kids want to put the music on and then everybody runs around, and dances, and it's pretty fun watching our toddler get into that now too. I mean in general any game that just gets us together and have fun, but usually we're at different stages given the age so my kids got to do one game because the 18 month old grab all the pieces and run away.
Dr. Mike Patrick And then I remember my daughter would play Hi Ho Cheerio which does have little chokeable pieces so you have to be careful with that. But I remember a time when she would lose and like she would get angry and throw the pieces across the room.
Dr. Baylis: It's really hard to not win all the time, so try and teach our four year old that it's OK to let somebody else win. It's a very tough lesson in life.
Dr. Mike Patrick Speaking of dancing and all that, I remember when my kids were young charades was really a lot of fun, although sometimes it's hard to guess the younger part but it's always good for a laugh. But we do want to encourage families to do stuff together and i think playing games, although it's nice as it's been here, but during the winter when we had that terribly cold winters could do quite games around the table.
Dr. Baylis: Thank God we can get outside now because I think everyone's feeling really coped up. SO we've been in the back yard a lot.
Dr. Mike Patrick Same here. Alright, we really appreciate you stopping by and talking about speech disorders and in particular velopharyngeal dysfunction, thank you.
Dr. Baylis: thank you again.
Dr. Mike Patrick Alright we are going to take a quick break and i will be back with a final word right after this.
Dr. Mike Patrick Alright we are back and really with just enough time to say goodbye, I want to thank all of you for taking time out of your day to make PediaCast a part of it. I also want to thank Dr. Adrianne Baylis, director of the Velopharyngeal Dysfunction Program here at Nationwide Children's hospital, I really appreciate her stopping by and sharing her expertise with all of us. That does wrap up our time together. PediaCast is a production of nationwide Children's Hospital.
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Announcer 2: This program is a production of Nationwide Children's, thank you for listening. We'll see you next time on PediaCast.
pediacast.org. Until next time, this is Dr. Mike saying stay safe, stay healthy, and stay involve with your kids. So long everybody.
Announcer 2: This program is a production of Nationwide Children's, thank you for listening. We'll see you next time on PediaCast.