Worried About Syndromes

Dealing with hearing loss can be difficult in and of itself, but there are several syndromes which have hearing loss as a symptom. From birth, we've been a bit concerned about Nolan's forehead shape, which seemed to have "corners" and was flat in the front. The pediatrician and ENT never noticed it, so we thought perhaps he would grow out of it, or that it was a minor thing that didn't merit attention.

As he's grown, a silent terror has grown inside of me that something else is wrong with our little boy. We are in the beginning stages of the diagnostic hurdles, and have many doctor appointments scheduled in the coming week. On Monday, I will discuss my concerns about head shape with his pediatrician. Instead of becoming less noticeable, Nolan's forehead is protruding more. I am concerned about craniosynostosis, which can be found with hearing loss. Most of the syndromes including hearing loss and craniosynostosis have other features (such as webbed fingers and/or toes), but I am concerned nonetheless. I have a first cousin who had craniosynostosis as a baby (surgery corrected it at one month of age). This condition is usually dominant, though, and neither Dennis or I ever had it as infants.

Here is a picture of Nolan with his Grandpa on Christmas Day- you can see how elongated his head is becomming:



We will have to wait and see what the pediatrician says on the 7th, and/or what the ENT says on the 9th. He has to have a CT scan for the hearing loss, anyway, so I am hoping they do a cranial X-Ray to make sure the sutures in his head haven't prematurely fused.

My heart will skip a few beats until we get a few more answers. Knowing is always better than not knowing. And I fear for my little guy.

All I Want For Christmas...

..Are some Unitrons! We picked out Nolan's hearing aids today- Unitron Element 8's with brown BTE cases. We were looking at some of the colors, but decided to go with the bronze-ish brown cases so that he could choose any earmold color and not have it clash. We're going with blue-and-white swirl for the earmolds this time around.

We get to pick up the aids on January 9th. Only 3 weeks to go before he can hear better!!!!

Toddler Signs

This isn't hearing loss related, but does involve sign language so I thought I'd post it here.

We've been working on some sign language with Matthew (not for hearing loss reasons- apraxia runs in this family and the kid simply can't talk yet). It's been alleviating a lot of frustration, though a recent "conversation" went as follows:

Mommy: "Matthew, what do you want for breakfast?"
Matthew (signs): Candy.
Mommy: "No candy for breakfast. What ELSE do you want?"
Matthew (signs): Cookies.
Mommy: "No cookies before lunch. Do you want pancakes?"
Matthew: "Aaahhh!" (his version of yes).
Matthew (signs): Cup.
Mommy: "What do you want in your cup? Juice or milk?"
Matthew (signs with a big grin): Candy!!

Sigh...

The amazing thing is that three days ago this conversation wouldn't have been possible. At two years old the kid can only say about 4 words (monosyllabic and not clearly, but he does have four words). The only thing I can hope for is that Nolan skips the apraxia so we don't have to deal with that AND hearing loss. We'll deal with whatever comes our way, though!

I'll post a video of Matthew signing if I can get him to cooperate. Two is generally not a good age for cooperation!

Update: I got a video, but he is in constant motion. He is a bit like a hurricane- as evidenced by the toys strewn around our family room (grin):

The First "Explaining."

I figured we wouldn't have to explain about Nolan's hearing loss until he had aids and the situation became more "visible." We took Matthew to get a haircut last night- his first real one! The employees all came over to coo at Nolan. Nolan, completely oblivious, just stared blankly forward. One person commented that he had eyes only for his mama. I just smiled and Nolan just stared. Another person asked his name and started to call to him, 'Nolan! What a cute baby! Nolan, you're so cute!" Again, Nolan kept staring forward with no clue that these women were fawning over him. "Wow, he really just keeps staring!" was the comment. Then they were REALLY trying to coo to him to get a reaction. I finally said- "he's hard of hearing. You have to get in front of him to get a response- he has to see you." Sigh...

I'm not sure if Nolan really can't hear the voices or if he just has no clue that they "mean" anything and thus doesn't turn to them. From the hearing loss simulator results, he probably can't hear them. Only 2 weeks until we get to pick out his aids and get the impressons made. I am so excited to get his aids in early January. Soon he will be able to hear better!

IFSP meeting

We had our IFSP meeting for Nolan. It was an interesting meeting because Matthew kept sneaking into the living room to play with the nativity set we have. I wonder what the EI coordinator thought when I said, "stop hiding the baby Jesus!" That kid loves the baby Jesus and carries the little statue around our house. We're often missing the main character in the Christmas story, lol. Anyway, Nolan will get speech therapy twice per week and we have established some goals:

Begin babbling
Turn and respond to voices
Start to recognize and respond to environmental sounds (i.e. doorbell)
Begin to vocalize needs as demonstrated in pitch of cries (i.e. hungry, mad, etc)
Vocalizing more to identify/recognize people/objects (e.g. mamama dadadada)

These are goals for the next six months, so these are things he should be doing by the time he is nine months old. We'll start the therapy sessions in January, because we're off to California and Utah for the latter part of December. Skiing and free babysitting, here we come!

Latest Report from the Audiologist

We finally got the results back from the confirmatory ABR. We're no longer mild/moderate but squarely in the moderate range. It doesn't really change much, though, since the result is essentially the same (hearing aids).

His right ear is now registering a bit worse than his left (it used to be the better ear). Our results from October 24, 2007 were:

Right Ear:

500Hz= 55dBnHL
1000Hz=50dBnHL
4000Hz=30dBnHL

Left Ear:

500Hz= 60dBnHL
1000Hz= 50dBnHL
4000Hz= 35dBnHL

Now they are:

Right Ear:

500Hz= 60dBnHL
1000Hz= 60dBnHL
"click"= 35dBnHL

Left Ear:

500Hz = 55dBnHL
"click" = 35dBnHL

We weren't able to get as much information on the left ear because Nolan was not cooperating and kept crying/pulling off the leads, lol. The summary is that Nolan has moderate sensorineural hearing loss sloping upwards to normal bilaterally. Hey- only a few weeks until we get those impressions made! I really can't wait to get him amplified!

Way cool hearing loss simulator!

So, I have been going nuts wondering what Nolan can and can't hear. All of the regular hearing loss simulators on the web are for high-frequency loss, and can' t simulate a cookie-bite or reverse-slope loss. THEN someone on the listen-up listserv told me about this program which can simulate any type or level of hearing loss. They have generic settings, and you can also manually move the bars to get the loss by frequency!

I downloaded it and have been having some "fun" playing with it this morning.

It definitely gives me a better idea of what Nolan can and can't hear (he is actually missing quite a lot).