Sunday, December 7, 2008

Part of the Waiting Game

Last I wrote, Aiden was diagnosed with EVAS/LVAS (enlarged/large vestibular aqueduct syndrome), which I still don't completely understand .. in fact, that whole appt. went so fast and so has all the time since then. I've joined an LVAS group and have asked many questions to professionals I have already built relationships with, all of whom told me that yes, my baby could have failed his newborn screening. Yet it still doesn't make sense to me, since they say this is not congenital and that hearing loss in LVAS kids is typically not detected until well after birth.

So here's some of what I've learned:

  • The vestibular aqueduct is a narrow bony canal that runs through the skull, connecting the inner ear (by the cochlea) to inside the skull. In short, the tube within this canal is filled with fluid which typically flows away from the organs of the inner ear. With LVAS, the fluid flows back into the hearing and balance organs, causing balance and hearing problems.
  • This is a syndrome ... it is genetic, it is hereditary. They have traced the gene to be responsible for LVAS, but still have a lot of questions about the syndrome.
  • LVAS occurs sometime after birth and is the result of abnormal postnatal or early childhood development (this is why I question why Aiden failed his newborn hearing screen? Could he truly fail it that fast? Most kids with LVAS are not diagnosed until after 12 months.)
  • People who have LVAS are predisposed to the development of hearing loss (wouldn't this mean it's congenital then that at some point in their life they would have hearing loss??)
  • It can be associated with Mondini (where the cochlea only develops 1 1/2 turns instead of the normal 2 1/2 turns - Aiden has a full 2 1/2 turns). They do say too though that hearing loss is greater if you have LVAS and do not have any other cochlear deformity.
  • It can also be associated with Pendred syndrome - (We will need to get him tested for this although he shows no other signs of this syndrome).
  • If you have LVAS without any other cochlear deformities, the hearing loss can be subject to sudden drops in hearing ... which may explain why Aiden failed his ABRs - was this a point where his hearing was at a sudden drop?
  • The hearing loss can be fluctuating. It can be sudden by the bump of a head, it can continue to fluctuate over time, or continue to be progressive over time.

So where are we at? Aiden is still a candidate for cochlear implants. Our surgeon tells us that LVAS kids are typically prime candidates for cochlear implants depending on the severity of their hearing loss. Aiden's testing seems to just get better and better, with his last aided testing at 45 to 50 db, across all frequencies. THIS IS AMAZING, BUT ...

... is this his highest he will go?

...will his hearing fluctuate more and to a point he reaches even higher db's or continue to decline?

... is this the best we're going to get?

We know we can't turn up his hearing aids any more because there's a point that sounds become distorted. Without his aids Aiden is hearing about 80 db. So his aids work very well for him. If we turned them up any more though, he'd actually lose the "good" sounds. He's at his peak.

So, we're in a holding pen right now. We're playing the waiting game. We have everything done as far as CI candidacy goes and it's all a go. Except the most important part ... the audiogram. With his most recent audiogram, 5 years ago, he wouldn't be a CI candidate. We're on the borderline. Will it get better? Will it stay the same? Will it get worse? Do we just take the next one and run with it? Do we wait even longer for more consistent results? WHAT DO WE DO NEXT?!?

Aiden's daddy and I still feel very strongly about implanting Aiden, on at least one side (although we've been prepared for bilaterals for some time now), and before he is 12 months old. Even if his aided tests went up 5 to 10 db, we're still dealing with a fluctuating hearing loss, we're still dealing with the constant wondering of whether Aiden is receiving all sounds of speech, and we're still dealing with the fact that he could completely lose all of his hearing at any given moment. We want him to have access to ALL speech sounds and learn these sounds when they are easiest accessible to him ... in his prime learning years ... NOW.

So ... next steps include:

  • Continue to work daily with the Ling 6 Sounds (ahhhh, oooo, mmm, eeeee, shhh, ssss), which by the way, he responds to all of them in therapy except the "ssss" sound.
  • We hope that by working with the Ling 6 sounds on a daily basis will truly condition him to listening for these sounds and prepare him for the next soundbooth, which is on January 12th. I'll post a video soon of us practicing these sounds.
  • After the soundbooth, we will meet with the surgeon again to verify candidacy and decide on a surgery date, which, if all goes well, will be in February (Aiden will be 11 months)

That's where we're at with cochlear implants. It's the waiting that's the hardest part. Before the whole LVAS diagnosis (and his last soundbooth), we were looking at bilateral implants by January. Now we're trying to figure out what's going to happen next. I have faith though and I feel a peace about me that everything is going to work out the way it is supposed to. I still anxiously await my baby's hearing birthday, but I know, it's right around the corner. Until then we continue with what we have to do to help our baby hear.

I found an AVT (auditory-verbal therapist) that I really like, so we continue to see her once a week. She used to work for Johns Hopkins (plus, trained under one of my AVTs from Texas!)then started her own AVT business, yet still works closely with our whole team.

Maryland ECI comes out one time per week. A couple weeks ago they came out and evaluated Aiden for his motor skills and language skills. I am happy to announce that Aiden was at 7 to 8 months for both receptive and expressive language!!! This is phenomenal! He's on track with fine motor skills, but behind with gross motor skills (which he tested at 5 months). He is still not sitting completely by himself. He is on his way, yet still has that whole balance issue. It will come though. He definitely gets around - by rolling. ECI will come out at least once a week for both speech and PT.

So, we're underway, trying not to watch the clock (or calendar) and just enjoying this holiday season.


Julie said...

You amaze me! You've come so far on your journey, and Aiden isn't even a year old yet! I think you deserve an honorary degree in being a mom of a deaf/hoh child :0)

Kel said...

What a trip. You are doing such a wonderful job with Aiden! I can't imagine having so much uncertainty, but you just keep pressing forward going for exactly what you feel is best for him. You rock! I hope he remains a candidate and that all goes well for February.

leahlefler said...

Those fluctuating losses can be quite frustrating, especially when you're on the borderline! Knowing he has LVAS and the likelihood of progression is a definite push in the CI direction. We have a fluctuating loss, but it is in the mild-moderately severe range (on the best and worse ends of the testing results)so at least we don't have to worry about candidacy for CI's. We just have to worry about appropriate amplification levels!

Waiting can be the hardest part- I guess you have to keep on keepin' on, as the saying goes.

Good luck in February- may it come quickly!

Renae said...

The unknown IS the worst part! At the same time I bet you can't believe Aiden is already 9 months old and look how far you've come! My husband and I and another couple started saving money from each paycheck 3 years ago, to take a trip, where there is white sand, and clear blue water. Our trip was supposed to be in Feb. 2009. Who would've ever thought that money would be paying for our new baby son to hear instead of sitting on the beach with a drink in our hands. I wouldn't change it for the world and I know you wouldn't either. We have the sweetest boys!! You just never know what life will bring.