Deaf Again

There's a lot to say in this post, so I'll get right to the point: Becca was diagnosed yesterday with permanent hearing loss.  Right now, she's testing at mild loss in her left ear and moderately-severe loss in her right ear.  They know it is permanent because it is nerve damage, not middle ear issues (though there are some of those going on, too).  She will need hearing aids, and we are in the process of setting up a series of appointments to confirm her levels, get them fitted, pick out sparkly ones, etc.  

We don't really know why her hearing has gotten progressively worse.  After her first set of tubes, she was hearing perfectly (or close to it).  In the past year, we've noticed that she's had trouble hearing, and then, as you know, she tested in the hearing loss range.  That test showed middle ear issues (fluid) and nerve damage, but we were hoping that the nerve damage findings were an anomaly due to the extreme amount of fluid in her ears.  Apparently it wasn't.  The middle ear part improved some with the tubes, but not completely.  One of her ear canals is full of blood (just gunk draining out through the tube - nothing they are concerned about except that it's acting like a big glob of wax and plugging that ear up), so we are starting drops to help clear that out  before her next appointment.  The most likely reason her hearing has deteriorated is that the ototoxic medicines and high bilirubin from when she was in the NICU have caused delayed hearing loss.  That sounds very weird, but apparently it's not unheard of.  The audiologist said that it's kind of like the little hair cells got smooshed down some then and the damage compounds as time goes on.  If that's the case, her hearing will probably continue to deteriorate.  (Even if it does, the treatment is basically the same - just keep adjusting the aids to provide more support.)  It may be that there are some structural issues in the middle ear that would predispose her to hearing loss - something about the auditory nerve canal or something or other that could suffer further damage through activities like football, dancing, gymnastics, standing on her head while watching t.v. (her typical pose), etc.  If that's the case, we'd have to try to keep her from doing those activities, but the damage won't necessarily get worse.  The other possibility out there is that there is some genetic issue that we haven't caught yet.  I don't think genetic in this respect means hereditary - but that there is something askew in her genes (mutations or such).  Along these lines, the hearing loss could be related to her septo-optic dysplasia diagnosis.  If you recall, we aren't really sure if she has that diagnosis (because she only shows some signs of the syndrome), but the part of her brain that she is missing is her septum pellucidim, and we don't really know what it does - so when something like this crops up, there's always a chance that it relates to that missing piece, I guess.  Another possibility is that she had CMV when she was a baby and showed no symptoms of it (apparently this can happen), but having the virus as a baby can lead to delayed-onset hearing loss.

So here's where we go from here:

• Give drops in the right ear to try to clear the blood clot out of the canal.
• Go back to our old genetics doctor for them to run further tests.  (I know nothing about what these are.)
• Go back to the ENT/audiology in 4-6 weeks to see if the blood is gone and to retest her hearing to try to replicate these results.
• On that same day, we'll do a CT scan of her temporal area so that they can get a better look at the structures of her middle ear.
•  If we don't get all the way through the hearing test with good participation from Becca, we'll go into the main/adult hearing clinic (Bill Wilkerson Center) for a more extended hearing test.
• Meet with an audiologist for a hearing aid selection appointment - pick out the devices, pick colors, get fitted, order the aids.
• When they come in, we'll go back in to have them fitted, adjusted, etc.
• For the next year at least, we'll do hearing tests every 3-4 months.  If her hearing has stabilized, we'll reduce the frequency of tests after a year; if it still seems to be changing, we'll continue to go more frequently.

John and I are kind of reeling from this diagnosis - not that it was completely unexpected.  We're worried about the logistics of it all (more appointments, more costs (since most insurance plans don't cover hearing aids, which is ridiculous), possibly more therapy if her speech ends up being affected (thankfully, though, she was hearing in the most critical stages of language development), more special ed needs).  We're sad that there is more we have to "do to" her that she won't like.  We just want to leave the kid alone for once.  We're discouraged that every time it seems like she's making lots of progress (SHE'S POTTY TRAINED!) and starting to leave prematurity behind, something else crops up.  She was getting so close to "normal" and fitting in.  I'm particularly sad that this is coming just as she is starting to notice that she is different than her peers with her button and all.  We're mostly sad that this is a disability that will never go away.  There's no way to "therapize" our way out of this.  She will always be hearing impaired.

Obviously, things could be much worse.  We know we are lucky beyond measure that she is alive and doing as well as she is.  And we could have received a diagnosis of something life-threatening like cancer.  But right now that really doesn't soften the blow that she will always be disabled in this way.  And we know that in the world of disabilities, hearing loss is not a huge deal.  We've seen our friend Sarah live with it for a long time now, and she gets by just fine.  But it's especially hard to find out something like this so late in the prematurity game.  I mean, we were prepared for her to be deaf when she was an infant.  She came home deaf, and we knew there was a good chance that she's always be deaf.  But then her hearing turned out okay - and that was great!  We thought we had dodged that bullet.  It's just weird and unsettling for it to come up now, when she is 4 and a half.  B's a tough cookie and will do fine in the long run...I just hate that it's one. more. THING. for her little self.