Sunday, August 3, 2008

Cochlear Implant Progress

In case you haven't been through it (and I realize probably a larger percentage of Evan's blog readers have, vs. the general population), getting a cochlear implant is a long process.

There are a lot of appointments as part of the evaluation. If Evan is determined to be a good candidate and we proceed with the surgery, there will be appointments associated with the surgery (pre- and post-op). Then there will be lots of appointments afterwards to "map" (adjust) the CI and lots of therapy appointments to help Evan maximize his use of the implant.

Tomorrow, Evan and Jeremy are going to Children's to have another hearing test done. He's had quite a few already, but they want to attempt another behavioral one as part of the evaluation. This is the test where Evan is in the booth, and they condition him to look at a moving animal when he hears a sound. The very first time Evan did this test (about a year and a half ago now), he was somewhat cooperative and the audiologist got some results that indicated a moderate hearing loss with one of the aids on. The results were not super-reliable.

Unfortunately he has never performed better on a behavioral test since then. Usually the test is scheduled on a day he has other appointments. So either he's cranky from being poked and prodded during an appointment before the test, or upset because he's just been in the car for two hours. We have tried taking him in for just the hearing test, but he didn't do any better (and that's frustrating for Jeremy--to drive two hours both ways and leave feeling like nothing has been accomplished. At least if there is another appointment, it feels like the drive wasn't a complete waste of time). Evan doesn't like being in the booth--Jeremy has tried sitting in there with him, leaving him in his stroller, we've even tried getting him used to the booth at Hearing and Speech in Yakima as "practice" luck.

Fortunately, Evan does have a history of objective hearing tests (BAERs mostly) that consistently show hearing loss at the severe and severe to profound level. The CI program director looked at the most recent BAER (this was the one that had shown possibly Evan might have better bone-conduction hearing), and felt that the difference was just noise in the data. We can move forward without a good behavioral test result, it's just nice to have one to confirm what the BAER is telling us. Sometimes a person's hearing loss is not as "bad" as it looks on a BAER--behaviorally (subjectively) they can perform better than would be expected. That's why audiologists always say that we don't know with certainty Evan's level of hearing until he can tell us.

Evan is also going to see his urologist (Dr. Lendvey) in clinic. Evan has one last urology surgery we would like to coordinate with the MRI he will need as part of the CI evaluation. Evan has more of a risk with anesthesia than a typical kiddo because of his airway and swallowing issues, so we try to get as much done each time he goes under as possible. Some surgeries can't be combined, but an imaging study is "clean" so it should not be a problem.

The MRI will help the CI surgeon get a better look at the soft tissues around his auditory system. Evan has had a CT scan done previously, which shows his cochleas are not completely normal (they are flatter than they are supposed to be), he also has some abnormalities in the tiny bones that transduce sound from the eardrum to the cochlea (some of the bones are fused or missing). Of particular interest is the path of the facial nerve. In CHARGE Syndrome, it is common for the facial nerve to run an unusual course. It is critical for the surgeon to know where the facial nerve is, because accidental damage during insertion of the CIs could lead to a complete facial palsy on that side (meaning no facial expression). Evan already has a partial palsy on both sides--we do not want to make this worse.

In the next month, Evan has an appointment scheduled with the surgeon and an evaluation with the psychologist. We will continue to update as we learn more.


elizabeth said...

Hope everything goes well -- we're staying tuned! You never know what difference a few months can make. Developmentally, that's a lot of time for such a young child, so you might have better luck with behavioral testing this time around. Good luck!

Leslie, Arlin & Katie Kauffman said...

Good luck with all the hearing tests and CI appointments. It's a long process! Katie has never had consistent behavioral results in the booth, even now, so it's still a guessing game as to how much she hears with her implant and how to map her.

Good luck with getting all the school things worked out, too. It's so exciting to see all the young CHARGErs starting school!

Leslie & Katie