Phew! It's the end of a very busy but very positive week for Maria. This will probably be a long one, so get comfortable.
Back in January she got a BAER (Brainstem Auditory Evoked Response) test which showed that her ears were working, but her brain wasn't getting the signals. This can indicated a problem with the auditory nerve (auditory neuropathy -- AN) or with parts of the brain itself (auditory processing disorder -- APD). The recommendation was to try her on hearing aids with the eventual goal of a cochlear implant. An oversimplified description of the implant is that it's a sophisticated hearing aid, most of which is implanted directly into her skull. However, drawbacks include the fact that it is major surgery, it destroys any residual hearing in that ear, and although it has had the best success with auditory neuropathy it still very often fails to help the patient. The problem is that doctors right now really don't understand either AN or APD so aren't sure how to treat them.
For the past few months we've struggled with hearing aids, and you can imagine how hard it is to keep them on a 13-month-old baby particularly now that she is getting better at deliberately pulling them out. At first, I thought she could hear _differently_ with them, but I couldn't see if she heard _better_ or not. She'd get quite fussy in loud environments like a restaurant and we'd have to take them out. In addition, though her hearing was bad before, it got MUCH better very abruptly back around March and has stayed quite good. More recently, we can't really see a difference in her reactions either with or without the hearing aids. She hears fairly well, but we don't know how well she discriminates what she is hearing. A good metaphor for these kinds of disorders would be dyslexia. A dyslexic can see the letters but can't make sense of them. Someone with these types of hearing impariments can hear sound, but the sounds are so distorted they may not be able to tell what they are hearing.
Unfortunately, as we have complained about before here, all of the people involved with her hearing impariment have been...well, insert your own profanity here. They have been condescending and unhelpful and never believed us when we would say that Maria's hearing was better. Heck, they didn't believe when she said her first hearing aids didn't work! I mean I can see being in denial about her hearing, but what possible purpose would it serve to lie about the hearing aids??? However, to be fair, their experience is with more typical forms of deafness that doesn't improve and they just don't seem to understand that AN/APD do sometimes get better as a baby's brain develops and repairs the damage. From my point of view, they are basically treating her for a now-invalid diagnosis so it is hard to take their opinions too seriously.
This week, Maria went through a number of tests to evaluate whether or not she was a good candidate for a cochlear implant. When this idea was first brought up in January/February, I could see the benefit of it. However since then, since Maria has improved, I've turned against the idea. Cathy has probably always been against it. We aren't closed-minded about it. We are willing to be convinced to do this, but we need to see that it really is the best choice for Maria and not just a "well, we don't know what else to do, so let's do surgery" thing.
Tuesday she had her speech evaluation. A speech therapist (not her usual one but a new one) examined Maria and talked to us about what kinds of sounds Maria makes and responds to. Although she didn't score as well as a typical 13-month-old, she has made a lot of advances recently that are very encouraging. Her babbling continues to increase in complexity and she discovers new and interesting sounds all the time. There have been several times she has used words that _seem_ to be in context (milk, more, up, bye-bye, mom) but they tend to be things she'll do one time and never again. On average, she seemed to score about in the 6-to-9 month range. The therapist said that she was on the fence about whether to recommend the implant. She was the first of the people involved with Maria's hearing who seemed to understand that AN/APD can get better and can be erratic as her hearing changes from day to day...or minute to minute. She was encouraged particularly that Maria is making progress and clearly has some significant hearing.
Thursday she had her appointment with her normal speech therapist. The developments there weren't hearing/language related. During the hour appointment, Maria did some amazing things. She rolled belly to back using only her trunk muscles, not her arms. Then using this newfound ability while on her belly with her arm trapped under her body, she rolled off her arm, pulled it out, and rolled back. Shortly after, she rolled from back to belly unassisted for the first time (she actually "fell off" the pad onto the carpet, and the little 3/4" elevation change obviously helped her complete the roll, but it still showed her it can be done). And, with some assistance from the therapist on balance, she pushed herself up to a sitting position. There were several other less obvious feats in the way she moves and holds her body. She hasn't done any of these things since (except the back-to-belly which she did off a pillow onto the floor) but her movements continue to get more coordinated and more directed. The vision therapist thinks that as her head control has improved her visual field has become steadier. This has allowed her brain to start processing images better. As her vision improves, she has more incentive to move to grab a toy or to roll because she's more aware things are happening. This, in turn, leads to better vision and so on. Yesterday and today we all commented on how she seems to be seeing people at longer distance (10-20 feet). Three times she has looked at my face and smiled, which she's never done before. There are many more visual and physical things that are new. She is on this incredibly fast track to development and we are very excited.
Today was the long day of the long week. She had an MRI, CT scan, and another BAER test, all under general anesthesia. We haven't heard the results of the first two, though we don't expect anything earth-shaking from them. They were mostly just to map out the area of the surgery to see if there was any strange geography for the surgeon to be aware of. The doctor who interpreted the BAER was the head audiologist. He hasn't worked with Maria before and we were very glad that he was the one involved because he has a very good reputation. He said her BAER is still abnormal. There is still little to no brainstem response to sound. However, unlike the others, he doesn't dismiss our claims that she hears. He said he has had a couple of patients whose BAER tells him they should be completely deaf, then he goes out and has a normal conversation with them and they can clearly hear just fine. It goes along with the idea that they don't really understand these conditions yet. He also said that her cochlear microphonic (the part of the test that shows whether her ears are responding to sound) was "the biggest response I've ever seen in my career and I have no idea what it means".
The most important thing is that his recommendation is that we adopt a wait-and-see attitude, do another BAER in 6-8 months, and see how she develops. No surgery. He still thinks we should use the hearing aids, but feels we should keep the volume very low like they have been. This is pretty much what we thought we should do. OK, we'd like to dump the hearing aids, but they aren't making things worse so we'll keep using them.
So what could have been a stressful and demoralizing week has turned into a very positive one. Maria continues to be the miracle baby and we hope to have more good news soon.
