Thinking About Cue Camp

Among the various communication options out there for hard of hearing kiddos is cued speech- handshapes that represent the various phonemes found in the English language. Its definitely something I want to learn, since it may help with Matt's articulation issues in addition to being useful with Nolan. On the other hand, classes aren't offered locally and there aren't any transliterators available in our school system. There is Cue Camp New York in Rochester (about 3 hours from us) and I'm thinking of attending that camp.

The camp is really a bargain when you consider what you get for the cost, but it is a high cost when you have a family of four! If all of us go, it will be $700 with the early bird pricetag. If Dennis stays at home, it will be $475. Ouch.

I'm still thinking about it, but we may try for next year rather than this year. This is the one time I wish Matt wasn't a full 2 years old! If he was under 2, he'd be charged at $50 rather than $200. Sigh....

PE Tube Surgery Done

After a long, long, LOONNNGGG night, the PE tube surgery is done and over with. The actual procedure takes about 12 nanoseconds, but the food and nursing deprivation for over 8 hours nearly killed all of us. For some reason, our ENT considers breastmilk a "solid" and none was allowed after midnight. Various doctors regard human milk in different categories (our CT scan docs considered it something between a solid and a clear fluid).

I stayed up until midnight so that Nolan could have the latest feed possible. He woke up at 1:00am, apparently wanting more. He cried until 3:00am, then went to sleep. Since we had to leave Jamestown by 4:00am to make our date at the hospital, I never went back to sleep. I'm working on 2 cups of coffee and one hour of sleep. Forgive me if I'm incoherent!

He was the first infant scheduled and I was there when they put him to sleep. It was difficult to see him struggle against the anesthetic. I am glad he is healthy as a horse- I couldn't bear to go through this process time and time again! I left the room as soon as he was out, and they came to get me about 10 minutes later. The nurse was quite frantic to find me, since Nolan was awake and NOT HAPPY. I was the only one who could provide what he wanted, and since his heart rate was quite high, they wanted to calm him down. As soon as he was fed, his heart rate dropped down and he was a happy camper. Shortly after this, we went home. A piece of cake, except for the no-nursing part of the whole deal.

His ear does have some drainage and we have eardrops for the next three days. I was able to talk to the ENT a bit more today and she confirmed that the CT scan was totally normal (after reviewing it herself with her colleagues at rounds). She also said that every one of her colleagues suggested more genetic tests and they were all surprised by the limited panel the geneticist ran. She's going to call Boston University to see what else we should run, and will update me on April 30th (our post-op date). We'll also schedule another hearing test at some point.

April is looking free and clear of medical appointments- thank goodness! We only have an earmold appointment and the post-op appointment at the end of the month. It's been so long since I've had free time, I'm not quite sure what to do with myself! Being free of waiting rooms and doctor appointments is quite liberating!

CT Scan Results

Nolan’s temporal bone CT scan came back as totally normal, which is really good news. This means that his cochlea (the inner ear hearing organ) is normal, his vestibular aqueducts are normal (if these were abnormal, he would have a progressive hearing loss), and all other osseous (bony) portions of his inner and middle ear are normal. If his hearing loss ever does progress, this means that he would be a cochlear implant candidate.

I also got the genetics report in the mail today (I had already written that those tests came out as normal). The genetics department wrote into the letter that they observed no abnormalities in Nolan suggestive of a syndrome, and that while these three tests came back as negative, it does not mean that Nolan’s hearing loss isn’t genetic. The empiric data show that we have a 18-25% risk of having another deaf/hard of hearing child (if we were to have more children) and Nolan has an empirical 10% chance of having a deaf or hard of hearing child. Matthew’s chance would be far less than this (the same as the normal population), assuming a recessive gene is at play. These are empirical values, however, and not actual risk numbers (they’re just based off the averages from other kids with unknown causes of deafness).

They don’t feel that Nolan’s deafness was caused by environmental factors or illness.

When talking to the ENT, she said she was no longer concerned with his head shape, since it appears to be rounding out and the geneticist was unconcerned. No additional mention was made of the midface hypoplasia, either in the geneticist’s report or by the ENT. They were supposed to do a head CT scan, but I don’t think they did this. I’ll ask again tomorrow with the ENT at Nolan’s ear tube surgery to see if they did the head CT in addition to the temporal bone CT. So far, though, his head appears to be normalizing and doesn’t appear to be causing any problems.

Dr. Brodsky (the ENT) said she would present his CT scan before her radiology rounds tomorrow to discuss the next step. She has not seen the actual scan herself, but has received the report. The hospital was supposed to give us a disc of the scan (we asked several times and they wouldn’t). Unfortunately, this means she hasn’t had the chance to look at the scan herself. She always double checks the results (we don’t expect her to find anything the radiologist didn’t, however). She was quite surprised that they ran only a limited genetics panel. She said that they typically run a lot of markers on deaf/hard of hearing kids and they only ran 3 on Nolan.

We are not quite sure what the next step is. His ear tube surgery is tomorrow (he has visible fluid in both ears, worse in his left) and then we have a follow-up (post-op) on April 30. Dr. Brodsky does want him to have a follow-up hearing evaluation (he’ll have one at least every six months during his early childhood). Since we don’t know the cause, we don’t know if his loss is progressive or stable and he’ll take continual monitoring for a while to see how stable he is. Configured losses (like Nolan’s) are more likely to progress, but that doesn’t mean that all configured losses progress. He will have an eye exam at some point, but probably after he is a year old or so. Other than that, we’re pretty much at the end of the diagnostic process- we’ll probably have more genetic markers run and an eye exam, but after that we’re done. He’ll be monitored by the ENT until he is no longer a pediatric patient (or we move) and will be monitored by the audiologist.

We may never know what caused his hearing loss, but we know what DIDN'T:

• Jervell Lange Nielson Syndrome
• Branchi-Oto-Renal Syndrome
• Alport Syndrome
• Large Vestibular Aqueduct Syndrome
• Mondini's Dysplasia (or any other cochlear dysplasia)
• Connexin 26
• Connexin 30
• Mitochondrial A1555G

So now we just watch and wait!

I'm Changing my name to Dada. Or Car.

Nolan has begun babbling. We officially have a consonant!! After all the work with "mamama" and "mmmmm," we finally have.... "dada!" Argh. I was really, really hoping for a mama first this time around! Nolan tends to be quiet most of the time, but when he decides to make noise, let's just say LOUD is an understatement. "AHHHHHHHHHHHHH DADADAAAAAADA" at maximum volume is heard until he tires of his own voice. And I love every second of it.

Since Matthew is talking more and more, I was hoping to finally (after 27 months) get a "mama" out of him. No dice. He can say "help," "two," and "my dad," but not mama. At bedtime two nights ago, Dennis told Matt to say goodnight to me. He came up, gave me a kiss, waved, and then said, "bye car!" He likes cars a lot, so I consider it a compliment. I'm still pining for a "mama," though!

What It Isn't

The department of genetics called back today and gave me Nolan's test results. Negative for Connexin 26, Connexin 30, and for the mitochondrial A1555g mutation. Darn! I was really, really hoping for one of these since they are non-syndromic and the Connexins tend to be non-progressive.

They didn't run any other genetic tests, so now I am on pins and needles waiting for our March 26th appointment with the ENT to discuss the CT scan results. I called the ENT's office and they have the results in the office, but didn't discuss them with me over the phone. I wonder if they found something, because you would think they would say, "his scan came out normal" over the phone...

About 10-20% of the time, they never find a cause for deafness. We may be part of that statistic. I don't really care what caused the hearing loss, but do want to rule out anything syndromic. I get to practice being patient waiting for the CT scan results. If there is one thing I am NOT good at, it is waiting for results (especially when the doctor's office already has them)!

In My Totally Unbiased Opinion..

Nolan is brilliant!

Hearing Aid Envy

I was wondering where all of my sewing thread went...

Now the real problem: how to make a 2 year old understand that we put things in Nolan's ears, but NOT in Matthew's ears. At least he didn't stick something more dangerous in there!

As for other updates, Matt is starting to talk more and more. He probably has around 25 words now (up from just six about 3 weeks ago- amazing). Signing has really helped clarify his points, though he is beginning to drop sign as his verbalization increases.

Nolan's had a cold, and I'm hoping he gets over it soon. We don't need any additional fluid in his ears, and the CT scan is now less than a week away. We had his six month old well-baby appointment: he is now 26 1/2" long and 15 pounds, 6 ounces. His head is 46cm, which is a bit big (especially considering his head used to be in the 50th percentile). The CT scan should shed some light on that situation.

I might also have a lead on a speech language pathologist who has worked with deaf/hoh kids before! I have no idea if she has room on her caseload, but you can bet that I am planning on being as persistent as I have to be to get her. Maybe there is no TOD in this area, but there IS a SLP who has worked with hearing loss before. I want her!

Mommy Panic Attack Over

I've talked to the EI coordinator and to a speech pathologist at the center where Nolan has his hearing checked- they both have said that he cannot be "graduated" from early intervention because he has a permanent condition with a high risk of causing a developmental delay. Basically, our SLP can test him until the cows come home - even if Nolan gave a dissertation on the works of Sophocles, he still qualifies.

The SLP at our hearing and speech center stated that all babies (deaf/hh and hearing kids) are similar until sometime AFTER six or seven months of age. A language delay is often not noted until the children are between 12-18 months of age, so six months is EXTREMELY premature to pull the plug on services. She is sending me an informational packet to share with our current speech therapist.

I can relax and get my panties out of the knot they are currently in. Now I just need to work on my next project: finding a teacher of the deaf (TOD) willing to work with early intervention in Chautauqua County. When we revisit Nolan's goals, the request for a TOD will definitely be in the wish list.

Got My Mama Bear Fighting Gloves On

We had a list of specific goals for Nolan in the Early Intervention system:

• Begin babbling (not yet- at least with consonants)


• Turn and respond to voices (check! accomplished)


• Start to recognize and respond to environmental sounds (i.e. doorbell) (doens't recognize these yet)


• Begin to vocalize needs as demonstrated in pitch of cries (i.e. hungry, mad, etc) (Check! accomplished)


• Vocalizing more to identify/recognize people/objects (e.g. mamama dadadada) (not yet)

Our SLP believes he has met his goals and is now age appropriate. She is going to test him on Monday so that EI can disenroll him from the system.

AAAAAARRRRRRGGGGGGHHHHHH!

Why does EVERYTHING have to be a fight? There are so many reasons I want Nolan to stay in the early intervention system. Among them:

• Nolan has a moderate hearing loss. This level of loss is educationally significant in prelingual children. Heck, it's educationally significant in postlingual children!


• We do not yet know if his hearing loss is stable or progressive.


• Meeting goals at six months does not mean that he will remain on target for speech and language at a later date.


• Prime language acquisition time is from the ages of 7-12 months. Kicking him out of the system just before that time period could be detrimental to his development.


• Kids with hearing loss qualify for EI regardless of performance on standardized tests.

I would never forgive myself if I allowed the system to disenroll Nolan, and then find out that he had slipped further behind because services were discontinued just as he really needed them!

Thanks to some very helpful advice on the listen-up email list, I am going to ammend his IFSP to state different goals. Goals that are more far-reaching, such as including a goal to be included in a mainstream school setting. I am going to request that if speech therapy stops, then the services of a ToD (Teacher of the Deaf) begin.

Wish me luck as I call EI tomorrow, and begin the fight to keep my little boy at an age-appropriate level for language development!

The Miracle of Speech Therapy

I truly love speech therapy. Matthew has been showing some improvement already, which is thrilling to this mama! He has added "car" and "two" to his very short list of spoken words. He uses both of them spontaneously, and uses them to communicate (instead of just imitating the word). He doesn't know the exact meaning of the word "two," but knows it is more than one! When given a cookie, he'll always say, "two, two!" in the hope of getting as many cookies as possible.


Nolan isn't babbling with consonants yet, but this isn't really delayed even for a baby with normal hearing. The range for babbling with consonants is 6-9 months, so we're keeping a sharp eye out for hearing that first "mama" or "dada" or "baba." He is taking turns with making sound, imitating an open mouth, and making lots of "aaaahh" noises. He's localizing to sound and I'm a little anxious for that first booth test (not yet scheduled).



The boys are starting to interact, which makes getting a good picture of the two of them very difficult. One takes a lot of silly faces, and the other takes bribery. For those of you with even basic ASL knowledge, take a guess at what I bribed Matthew with (I know, I know- bad mother of the year award):