ROUGH EDITED COPY
EHDI
HANOVER A
LARGE VESTIBULAR AQUEDUCT SYNDROME:
IS MY CHILD AT RISK?
FEBRUARY 28, 2016
4:15 P.M.
CAPTIONING PROVIDED BY:
ALTERNATIVE COMMUNICATION SERVICES, LLC
P.O. BOX 278
LOMBARD, IL 60148
* * * * *
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.
* * * * *
> Hello, everyone. Welcome to your final session today. It's been a productive week. Wow. So just wanted to introduce the topic of this session. Huge words. I'll do my best here. Large Vestibular Aqueduct Syndrome: Is Your Child At Risk? So please help us welcome our presenters for your final session.
> KATHLEEN JOHNSON: Hi. Okay. My name is Kathy Johnson, and I'm the audiologist at Montana School for the Deaf and the Blind, and I have been the audiologist there for 37 years so, I've seen all kinds of technology and all kinds of things that have changed over time, and one of the advantages of my job is that my office is actually right in the middle of the school, and so I see the kids walk by every day all the time. So I do get to know my patients sometimes a lot better than you would in a more traditional audiology practice because I go to school with them every day.
Okay. So one of the things that happened about 20 years ago is I found out about large vestibular aqueduct syndrome. And you can just say LVAS. We'll agree to that. I hope. Okay. So what is that? It's a narrow canal between the inner ear and a fluidfilled sac in the brain. And I'm not going to talk about it too much except that that narrow canal, if it's just a little bit too big, then the fluid can go back into the inner ear, especially if there's been a bump on the head or a change in head position. And this can actually cause a change in the sensorineural hearing that can be temporary, or it can be permanent, and it can also cause a conductive hearing loss. Approximately 40% of the time, there can also be a deformation of the inner ear and/or a fistula, which is a leak in the inner ear. So about 40% of the time when the child is diagnosed with large vestibular aqueduct syndrome, they also have some other kind of deformation in the inner ear, or even a leak. And this is diagnosed through a CT scan or measured on an MRI, and the technology that is used to diagnose it tends to vary depending on what part of the country you're in.
Now, this one is a little bit shocking. At any given time, since I started looking for it in about 2000, about 30% of the kids that I consult with who have any degree or any configuration of hearing loss actually have this. Now, this is much higher than other studies have shown on a national level. Other studies have shown more like a 7 to 17% incidence. I have a theory about why that might be low, if my experience is any indication. One of the issues that I've had is that many of these children have had CAT scans in several different places in Montana. And you have to understand, Montana is the fourth largest state, and we might have a million people now. We do have two million cows, but and there's only a few places in Montana, really, that you can even get a CAT scan. And not all the CAT scans apparently are of the same quality. And I'm going to talk a little bit more about that later.
Okay. So what might be an indicator, if 30% of the kids that I know with any degree of hearing loss have this, then the incidence might be a lot higher than people think. So here's some of the signs that your child might have LVAS. Oh, by the way, it's also known as EVAS. And some of the articles will say LVAS, and some will say EVAS, and because I printed all my handouts a while ago, mine say LVAS, and some day they'll decide which one we're all going to use.
Okay. The hearing loss can be of any degree, any configuration, and especially a hearing loss that's progressive. They can have a conductive component to the hearing loss. However, there's no fluid present when the physician checks and there is no ear infection. Their hearing can fluctuate. I actually had a doctor last week that told the parent, no, that's not true. I'm going to show you just how much it can fluctuate. It can go up and it can go down. Also, their understanding ability can fluctuate. So they can have days where their hearing is actually staying stable, but their understanding ability changes. And I actually had one child who would say I'm having a bad hearing day, I don't want to go to public school. I'm just going to stay here, have them send all my information here because I'm just not going to get what I need. Even though she had the interpreter, she still really relied on her hearing. She just didn't want to go on the bad days, and then she'd say, nope, I'm doing better. If you put her in the booth, that's exactly what you got. Her understanding ability was so low that she couldn't understand anything.
Okay. Any time you have a child where you notice a change in their hearing after they bump their head, even a minor bump, they should be evaluated for LVAS. Sometimes the bump can be as bad as, you know, falling down and hitting your head. One little girl had a significant change in her hearing. She stood up because she thought the school bus had stopped, and the bus went forward and she leaned she lurched forward and hit her mouth on the kid's head in front of her, and she had a fat lip. She lost all of her hearing for a couple of days.
Okay. Some of these children have significant balance problems, especially if the balance issue seems to come with vomiting or eye movements. You see their eyes go back and forth. That's called nystagmus. They may have sorry they may have a family history of Pendred Syndrome. The child may also hear ringing in their ears, or tinnitus. They hear sirens when none are present.
Their hearing may decrease after they have a fever. It may decrease after they play on a trampoline or a bouncy house at a birthday party. There may be a change in their hearing after they fly especially if they fly in a small, nonpressurized plane. In fact, some people will not take their child on anything but a pressurized plane. And they can have a change in their hearing after they dive into deep water. If they go scuba diving or snorkeling or something, that doesn't usually happen in Montana, but some of you might live somewhere more fun.
This can also be unilateral, where they only have it in one ear, and it may be that 30 this is 30% of my caseload, not 7%, because I actually look for this. I'm the LVAS Nazi. This is important. It's extremely important to find out if your child has this, because the hearing loss progression may be prevented if the family knows and is taking precautions.
In some areas, all babies who are diagnosed with a hearing loss get a CT scan. This is not happening in Montana. They may the baby may need to be at least one to one and a half years old before the duct can be measured accurately. That's just what the doctors do in Montana. I don't know if that's necessarily true everywhere, but that's what they say.
There's been some research about the fact that the measurement may change over time, and some people say that that's nonsense, so I don't know. But anyway, one thing that all the experts do agree on is that the size of the vestibular aqueduct does not predict how or when the child's hearing levels change. So you can have a child that has a huge measurement and a huge duct, and that doesn't necessarily mean that their hearing is any worse than any other child.
The thresholds can go back up to previous levels, up to 9 to 12 months later. I know some studies show six months but I have actually seen children where a year later their thresholds went back up.
Okay. This little girl I started seeing, if you look at the audiograms, when she was two years old, and then this was when she was six years old, the bottom one is when she was eight years old, and when she was eight, that okay. She's she had a CAT scan when she was two years old in one town in Montana that was read as normal. She had a CAT scan when she was four years old in Great Falls that was read as normal. When she was eight years old, she had a terrible vestibular episode. She fell down, she hit her head. She was vomiting. She had nystagmus. When I tried to do atympanogram on her ear, she had nystagmus. When I tried to put a more powerful hearing aid on her ear, she got nystagmus, so that's the point at which we went to a different ear, nose and throat specialist and he said I want to do another CAT scan. I said this kid's had two CAT scans already that were read as normal, and the doctor there's one doctor in Montana that I refer to for this, by the way, and he said nope, we've been having problems with some of the CAT scans, and I want her to have one done here. So I said, fine, you know, whatever you want. So we went to Missoula, she had her third CAT scan, and I was in the room when the doctor put the xray up and showed us, and even I could see that the child had Mondini dysplasia. Her inner ear was not formed right. She also had large vestibular aqueduct syndrome. Okay. So this is her third CAT scan. The other two CAT scans were misread. Okay. This happened about this happened back in 2001, it looks like. Since 2001, the CAT scans aren't necessarily getting better in all the other places in Montana, so I still send everybody to Missoula if I can talk them into it. Why should you put a kid through a CAT scan if the results are not going to be able to be read or are not read accurately? So that's my soap box.
Okay. Over time, her hearing dropped. And she actually had different times when oh, wait. This is the wrong kid. Sorry. This young man actually had just a slowly progressing hearing loss, and he also had a CAT scan that was read as normal, and then we went I sent him, when he was about 11 years old, for another CAT scan in Missoula, and that was the result, and he also did have large vestibular aqueduct syndrome but we could not restrict his activities because he wouldn't restrict them. If you told him not to play basketball, he would just go somewhere else and play basketball. So his hearing stayed has stayed pretty stable, and he also has a basketball scholarship. And he's done okay.
This is the little girl I was talking about before. Sorry. This was her first audiogram when she was three years old, and this is after she bumped her head. And this is after she bumped her head again. She had a significant drop, the ringing in her ears. She refused to wear a hearing aid because she couldn't hear. Okay. So this is the one on the left is September 9th, 2004, and the one in the middle is September 27th, 2004, and it was at that point, in 2004, that she had her third CAT scan. She also has had fluctuating hearing that's gone up and down. Her hearing she had a fistula in one ear, which was repaired by the ear, nose and throat specialist, and her hearing has kind of stabilized, actually kind of more similar to the audiogram from when she was three. This young man was identified in the first he passed kindergarten screening, but he was identified in the first grade as having a unilateral hearing loss. He had a CAT scan done in my town, which was read as normal. So we sent him to Missoula, and he did, indeed, have large vestibular aqueduct syndrome, but it's only in one ear.
This is the most interesting one. Okay. This little boy passed the newborn screening. He passed kindergarten screening. In the first grade, he had a hearing loss in his right ear, and normal hearing in his left ear. So he had a CAT scan that was read as normal in Great Falls. And but by the second grade, kind of the springtime of the second grade, he had a hearing loss in both ears all of a sudden. So I said well, maybe we need a second opinion, sent him to Missoula, and he was identified with large vestibular aqueduct syndrome. So I asked his parents, is there anything that changed, any activities, anything that you did different than the last year or so? Well, they bought a trampoline. And he was in karate and he was in gymnastics. And I said well, you know, maybe those activities had something to do with the change in his hearing. So maybe for this summer, maybe we could try golf and swimming. So he did golf and swimming, and they ordered the hearing aids for him through Tricare, and that takes a while. So he came back in September, and his hearing was like this on the right side. So his hearing had actually come all the way back to normal after we restricted his activities, and then this is the last time I saw him, which was in the spring of the next year, and he was still holding on. And then they moved away, so I don't know what happened. But that's pretty amazing.
Okay. So here's some suggestions that I have written up for families that have a child with large vestibular aqueduct syndrome. Prevention of further hearing loss means taking a lot of precautions and safety measures. It might mean restricting the child's activities, and educating everybody in the child's life. This is very stressful. I'm just going to say that up front. Be strict about helmets for all of your children and yourselves. There's also the possibility that someone else in the family might also have LVAS, so that everyone needs to be wearing their helmet. Remove benches, bar stools, backless stools, anything that the child might climb up on and tip over. One little girl that I was showing you the second time, she actually fell off a bar stool when she was developing normally. This is before newborn hearing screening. She was developing normally, fell off a bar stool in the kitchen and went around for a day or two calling mommy, mommy, mommy? And then she stopped talking. That's probably could be when she lost her hearing.
Keep bicycles and tricycles and all other toys in good working order. Teach your child how to use them appropriately and safely, and then you need to be watchful. Carpet the hard floors, and remove throw rugs that the child might trip over. Keep the floors clear and picked up. Be sure that everyone in the family, that babysitters, child caregivers, are aware of the problem and so they can tell you that they need to report that to you if they have even a minor head bump. The reason that you need to know is you need to watch what their hearing is doing, and some doctors recommend that the child go on steroids for a couple of days. If you catch it quick enough, and they're with the idea that this may allow the hearing to come back, this is controversial, but the doctor says to do it so that's what we're doing.
If your ear, nose and throat specialist has suggested it, ask to have standing orders for the steroids already on file so you can go you can call the pediatrician, go right straight to the pharmacy and get them. Keep a supply if you're going camping or on vacation. Make sure that the school is aware of the situation and all of the playground aides, gym teacher, classroom teachers, know what the restrictions are, if there are any on the child. Remind the school that the child might have good hearing days and bad hearing days, where they just don't understand, have little patience with them. It's frustrating for everybody. It's frustrating for the child. As I said, they can have days where their understanding ability is affected, and they might be able to hear the speech signal, but it's just not clear enough that they can understand what's being said.
Be sure to let the school know if they're on steroids. That can also change their behavior and make them very busy.
It's a good idea to have a written sheet of instructions to be given available to be given to substitutes, especially in gym class, as the child is often too shy to tell someone that they shouldn't be doing something, especially if it's an adult, and especially if it's an look for alternative to sports that will still give them something to do. So that means it's okay as long as they don't dive to the bottom of the pool. Dance class can help them with balance and coordination and it might help actually prevent falls. Let the child have a say in what they give up for some of the kids, giving up football is more than they can handle or basketball, and everyone has to weigh out what that might mean.