Wednesday, September 25, 2013

Another Visit to the Audiologist

Robert went to the Audiologist with me and Zachary and I recorded most of the appointment. Z was tested by Dr. Holly a pediatric audiologist He was tested a week or so ago by Dr. Smith. Today we are here to see if their findings match up and to find out exactly what's going on with Z's hearing.


The structure of the ears look nice and healthy, the ear drums are moving like they should and that looks great. There is a little tiny muscle in the middle ear behind the ear drum. When we have loud sounds they muscle is supposed to contract, now I did see one response but I wasn't able to get normal responses for that in either ear on all the different frequencies that we looked at, in and of itself it does not indicate that there is a problem there, however that wasn't completely normal.
Then we did or OAE testing and what we're looking at with OAE is some different tones, different pitches, or frequencies going into the ear. It travels down into the inner ear and that inner ear has little hair cells in it, now when those are functioning normally sound is going to come in and those little hair cells will send back an echo it's very tiny. There are a lot of things that could block that response from being gained and I did not get a normal response in either ear. The ear canals have no blockage and they aren't inflamed, the ear drum is fine. The organ called the Cochlea has thousands of tiny hair cells which change the vibrations into electrical signals that are sent to the brain through the hearing nerve. The brain tells you that you are hearing sound and what the sound is. So in the test given we found that the little hair cells in the Cochlea did not respond to the tones telling us that more likely than not the origin starts here (the cochlea). There are three nerves going to the brain, one is for balance, one is a facial nerve and the other is the hearing nerve. Are finding are that the damage is starting at the Cochlea
It sounds to me like with the tests that we both did. We are both definitely seeing damage start at the origin of the cochlea. That's the beginning of that neural response. We did not see any kind of issues that would relate this to any type of allergy or ear infection.

An Explanation of the Graph Below

This is an audiogram. This is a graph of the softest sounds that he's hearing. What we are looking at are the softest sounds you can hear. Across the top of the graph we've got base sounds, the boomy low sounds all the way to high pitch, high squeaky sounds. These are the sounds that are important for speech and language. Down the side we've got really soft quiet sounds all the way to very loud sounds. So, basically the farther down the graph you go the louder I had to turn the beep for Z to hear it so he would raise his hand. The red is for the right ear and the blue is for the left ear so you can see they kind of match up they have that same pattern, which is good. If there were a huge difference we might be concerned about other things, so it's good that they are matching up. the normal range of hearing is going to be 20 and above. So you can see that some of those responses come up to that normal range but for the most part they are below the normal. I had to turn up the beeps so they were a little louder for him to hear them. Now that is just hearing the sound. Is the sound there? or not there? That's the first part of hearing so we've got from a ..., and I kind of jotted it down out here so you can remember it later when you take this home, but this is kind of a mild hearing loss in the low frequencies and then it slops down here to a moderately severe in the left and severe in the right. The other things that are on this graph are just everyday sounds anything below your lines you can hear it because it's loud enough, but anything above you either can't hear it at all or you have a really difficult time hearing it. You can tell that the low frequency sounds of speech like vowel sounds those are very loud and boomy they give the volume to speech. Those are audible so he can hear them when you're talking but sometimes understanding what you say id hard because look at all these consonant sounds that give the clarity to speech. Those are above that line so Z can't hear them. He's not hearing a lot of those sounds at all. So that means that his hearing is very muffled, it's distorted and he's not understanding much of what is said in conversation. 


The next part of the testing that we did is involving speech because we want to know how soft does he understand speech and then if speech is loud enough it should be clear enough to hear. How well does he repeat back? That's where he started having some trouble. When we did the speech testing a lot of interesting things happened. Now, we know he's going to miss some things because we now know he is missing all those consonant sounds but I also noticed ... it was a little out of the ordinary not just missing those sounds. You know missing the /p/ sound at the end or the /s/ on the end of the word. It's hard to describe this but it's like he would say a completely different word or just say something that was not your typical response you would see. It was really out of the ordinary. Another observation he kind of twitchy or moving a little bit. The scores were stable from last time to this time which is a very good thing that his understanding of speech did not drop between the two test times. He is understanding speech better on his right side than on his left. Another interesting thing is when we turned up the volume. I went ahead and repeated the right ear I didn't do the left ear because, I mean, the right ear did the same thing where when we turn it up a little bit louder you should still be fine. You should hear it even better, it wasn't to an uncomfortable degree I just turned it up a little bit extra, so you would think, that's easier to hear that word. No, he actually did slightly worse. So he didn't do as well as we thought he would when we turned up the volume, that's out of the ordinary, not something we come across very often. Again goes along with damager in the cochlea or the inner ear. How is he doing in school? 

"He's an A,B student and the teachers haven't noticed anything". 

It's very subtle, because he has that drop where he's got a mild loss and then he slopes down in those high frequencies and you can kind of see on this graph. If the lines above that sound, that's typically that you should be able to hear those sounds pretty well so these consonant sounds he's going to struggle here because his line drops below, but the vowel sounds he should hear pretty well. But you can see where it's borderline right here. I think he's getting by on having a little bit better hearing in the low so when you talk about amplification or hearing aids. It kind of helps span that gap and gives you a little bit of a boost where you need it and a lot more boost where you need it there. Unlike testing under headphones or listening to TV or something like that. He may turn the TV up here to try and grab these. Well it going to be way to loud for right there. (The Dr. is pointing at the above chart as she describes this to us). So if he's listening to the TV or to music or something like he may really try to jack it up so he's catching those consonant sounds, but when he does that it over corrects on those low sounds and could cause further damage. 

 

"How would hearing aids fix that"?

Hearing aids? We can't say that they totally correct your hearing like a pair of glasses corrects your vision. It's not quiet the same but hearing aids amplify or give us a chance at being able to modify so that ..., basically where he doesn't need as much power we don't give him as much and where he needs more we can give him more. A basic hearing aid even the good basic you may only have four sections where we can actually make changes to it. So, it gets kind of dicey where he goes from a mild and slopes pretty quickly to a severe to moderately severe level. Thats one reason why I would plan on the total cost being in that 4 - 5 thousand dollar price point. I can extend a little bit of a discount and we will work with your insurance on that, but you should plan on there being some out of pocket expense. We will try to get the best basic aide that we can but its going to be a lot harder to fit his hearing loss and also get a hearing aid that will function well in the school type setting. You really want something now that manages that speech and noise.
You can compare the different levels of hearing aids to cells phones take technology for instance you take like an old basic cell phone vs. a smart phone. It's a computer chip on the inside that's what we're dealing with and so the smart phone computer chip and the processing is happening quickly it can do a bunch of things automatically running the background. Whereas the other one you literally have to push buttons each time to do what you want it to do. So, hearing aids are based on a computer chip and so when you go top of the line everything is more automatic, it's a faster processing it a more sophisticated of a noise manager so someone who has a more active life style if you think about it he will be going into different environments, for example he will be going into this noisy classroom, and then into a cafeteria where it's this big open space with a lot of noise and then he will be going home where it's very quiet and he will be focusing on mom or a TV. 
"So the hearing aid automatically adjusts the volume" 
Yes, exactly! It's automatically going to detect noisy rooms from quiet ones. Let's say I'm in a coffee shop and there's a guy talking over there while I'm trying to listen to my mom the hearing aide is going to alleviate that noise so that I can hear my mom or it will lower the range of where you are listening. With a basic hearing aid you have to physically look at the person you're talking to and it's only going to lower the volume from the back or behind you, so it's just very basic. And then you have technologies that are between there. 

So a top tier, it used to eliminate back noise or it would minimize or eliminate what was going on behind you. Now it actually will kind of zoom back if like someone was behind you telling you to do something it would recognize that there is a prominent noise from behind and it would zoom to the back, whereas before you would have to turn so you could see who was talking to you. They are always going to work better with anything within 6 feet and more face to face but that's just an example of the newest offering in the high or top tier level. The other thing I would consider for Zachary is doing the water proof or water resistant it's a little bit bigger casing but its going to be a little more durable for a child. Given to how we are uncertain to how this will progress I would put him in a Rick that way we have a lot of flexibility for the life expectancy of that hearing aide. If we start seeing his hearing change then we're not coming back in a couple of years and saying we need to get a different hearing aide. If his hearing progresses to where it is off this graph then we would be talking about cochlear implants. Hopefully we wont see it progress rapidly like that, but it is something we will want to watch for. I would recommend we have him tested every 6 months for the first couple of years and if it remains stable then we can see him once a year. 

Essentially Zachary is going to need some support in the classroom. Being moved to the front of the classroom and the FM System we discussed last time we met and possibly a note taker as he gets older if he needs it.

Zachary do you have ringing in your ears? yes 
Are you in any pain today? No 

I just wanted to be sure pain wasn't a factor as sometimes if a child is in pain they won't be as focused during the testing.

"Does the constant tone in his ear have any affect in him hearing some of the higher pitched sounds?" 

No it will not create a problem for our testing, but it can be a little disruptive throughout the day. Sometimes it can have an impact on sleeping, getting to sleep at night, because its kind of annoying. We look at that too when we're managing that hearing loss.





Monday, September 16, 2013

Definitely Experiencing Hearing Loss

Audiologist said that Zachary is definitely experiencing hearing loss and wants us to bring him back to be tested again by a pediatric audiologist that she works with to make sure they are seeing the same thing and then wants to test nerve function with a machine she shares with another audiologist. She'd like to have this done before the end of the month. She was an awesome doctor. I told her about the Superficial Siderosis. She totally looked it up and studied it for about 15 minutes. She said she had never heard of it before. I like doctors that listen and take the time to study up on their patients.

Zachary was at a really good part of his book he didn't want to be left with a cliffhanger when the doctor called him back.


Z being tested, I asked him if he was ready and if he studied he said he didn't get a study guide.


Into the silent room he goes! 



The Results! 
  

Dr. Beth Smith advised me to go to the school and ask about their hearing loss services and reccommend an FM System for Zachary to use. I got the ball rolling over at the school today.
Hearing loss is the most common symptom associated with Superficial Siderosis. The research that I've done, hearing loss doesn't occur until age 40 plus, however we are in uncharted territory as Zachary is the only documented child case with SS. 


You know with the news of Z losing his hearing, there were some pretty serious emotions going on today, mostly from me. Zachary? He's so laid back and just goes with the flow, he doesn't really seem to concerned about it. Then today after years of trying to ride a bike. Today, the Lord sent us a Tender Mercy! Z was able to balance himself enough to ride a bike. Whoo Hoo! I just couldn't hold back the tears. Nothing is more rewarding than to see your child that happy. I'd like to pocket this memory, for it was definitely a good one. 





Wednesday, September 11, 2013

The Run Around

Went to Dr. Westerkamm (the ENT doctor) today and he did the exact same thing that our family doctor did. He put a cone shaped cup onto a light and looked in both ears. We were in and out of the room in less than 5 minutes. Guess What? Z has no blockage! What? You're kidding me! You mean my family doctor got it right? Shocking!! It aggravates me to no end when doctors give you the run around and charge you all kinds of money. Seriously I didn't see any since in that appointment at all. We totally could have skipped that one and went straight to the Audiologist.


Z walked out with the biggest toy from the toy chest. That toy will only cost us a couple hundred dollars.

I just get so aggravated when we are handed the run around. One, I don't have the time for it and two, I don't have the money for it.

Friday, September 6, 2013

Reply from Dr. Levy & Dr. Jordan


Hello Ms. Huguley,

Thanks for the update. I'm glad to hear that Zachary is doing so well. I will let Lori Jordan know.

It will be important to track the hearing loss over time. If Zach continues to lose hearing year after year, you may want to think about iron chelation therapy. 

Michael Levy, MD, PhD
Assistant Professor, Johns Hopkins University
Director, Neuromyelitis Optica Clinic
1800 E. Orleans St.
Pathology 509
Baltimore, MD 21287


So glad to have this update Mrs. Huguley.  I'm glad that  Zachary is overall functioning so well. Though I am sorry to hear that he has some hearing loss. Hopefully it stays minor. 
I am here and am always happy to see Zachary if neurology care is helpful. 
Best, 

Lori Jordan MD, PhD
Department of Neurology, Divisions of Pediatric Neurology and Stroke
Vanderbilt University School of Medicine
2200 Children's Way, DOT 11-242
Nashville, TN 37232

Thursday, September 5, 2013

Hang on!! The roller coaster in on the move again ..

This year has been such a good year for Z. There just really wasn't much to write about, however, it seems the roller coaster is on the move again. We're buckling our seat belts and preparing for the ride. In the past few weeks we've found that Z is losing his hearing ...

Wednesday, September 4, 2013

Update on Z to Dr. Levy, Dr. Jordan & Dave Hill

I'm not sure if you will remember us or not, as it has been nearly a year since our last correspondence. Our son, Zachary was diagnosed with SS when he was 9 years old. I wanted to contact you to give you an update on him.  Zachary's pain is more under control now than when we first found out about SS. The pain does tend to sit on the back burner for a time, and it does occasionally rear it's ugly face when we least expect it. In all of this we have learned how tough our little boy really is.
We found that he did suffer from a torn ligament in his neck from whiplash and Dr. Brown has gotten Z to a point where he can at least be pretty close to a normal 11 year old boy. We took him in to have his hearing checked by our family doctor last week, as I was encouraged by you to keep a look out for that sign of SS. It is with a heavy heart that I tell you that he has suffered some minor hearing loss and is being referred to an ENT. We were so praying this would not happen, don't get me wrong we knew it was a high possibility, but we always hope and pray for small miracles. I truly pray for all those who suffer with SS and constantly hope that a cure is near enough to help all of them.

If you would be so kind as to forward this to Dr. Jordan I would be so grateful. I do not have her contact information anymore and I know she would want to know how Z is doing. Thank you in advance!

I will keep you updated
Michelle Huguley

Monday, September 2, 2013

Checking Z's Ears

Zachary seems to be having some trouble with hearing so I took him into our family care doctor to have it checked. We left with the diagnosis of some minor hearing loss and were referred to an ENT doctor.