Ears

CMV is a leading causes of childhood hearing loss. It's a leading cause of a lot of problems, actually, but ENTs and audiologists have their finger on the pulse of CMV whereas other specialties don’t. One of the few official standards to follow when it comes to medical supervision and care of kids born with congenital CMV is to monitor their hearing. Kids born with CMV often fail their newborn hearing tests. Some pass their newborn test but lose their hearing later. Many suffer progressive hearing loss.

Starting from birth, Keira has had her hearing tested every 6 months. She's had tubes placed a couple of times; not because of ear infections, but just to keep them clear of fluid and pressure so we could get adequate hearing test results. Keira has actually had very good hearing. In fact, I found it somewhat annoying that so much focus in CMV research and literature was in regards to hearing. Hearing, I felt, was the least of my worries. When you've got a child who can't see well and can't speak and can't walk and can't eat, hearing loss doesn't seem like the most urgent thing. But the truth is, many kids born with CMV have hearing loss and no other unfortunate disabilities, so hearing is of utmost importance to them. I get that.

In October, Keira had her first sedated ABR hearing test. This was a bittersweet milestone. Until then, Keira could have all of her hearing tests done while she was awake (by putting probes on her head and reading the brain responsiveness to various sounds). However, she has recently gotten too wiggly for a non-sedated test. The increased movement is awesome, though the flip side of that is that now she has to go under general anesthesia for her hearing tests.

The results of her first sedated test were bad! Her right ear tested normal, but her left ear tested as moderate to severe hearing loss. That was a huge decrease in under a year's time. It was a pretty big blow. Once again, when things felt somewhat steady and predictable for Keira and for us, we were reminded of the precarious nature of her health, her fragility, and of course the idea that she could rapidly decompensate and leave us for good. 

I wasn't altogether surprised about the hearing loss, but the audiologist surely was. The audiologist thought that we were pretty much out of the woods, believing that the longer Keira went without hearing loss, the less likely she was to ever have that happen. I wasn't so confident. One think I knew best about CMV was that it causes progressive hearing loss in children. Plus, I'd heard Dr. Park's recent updates regarding his vancyclovir studies.

When Keira was born, she took vancyclovir, a dangerous antiviral medication, for 6 months because Dr. Park had shown that this reduced the incidence of progressive hearing loss for children born with CMV. It was (and still is) the only standard recommendation for treatment of babies born with CMV. Keira took the medication with no problems, took her routine hearing tests, and worrying about hearing loss was put on the back burner.

In September, I attended the national CMV conference in Vermont. I listened to Dr. Park speak about his updated research. They were beginning to find, unfortunately, that the vancyclovir was not so much preventing hearing loss as it was delaying it. The children in his study were still losing their hearing, but it was happening later. This is unfortunate, but the vancyclovir is still beneficial because it is of huge importance for kids to have their hearing early in life to help understand language and communication.

So I thought that his lecture was interesting and within weeks after returning from Vermont, we got this negative result from Keira's ABR. In response, I shot Dr. Park and email. I basically said, "You know that thing you were talking about? Yeah, we just saw that happen with our daughter." Dr. Park responded personally and informed me that he was doing some new research on a treatment method that was showing some modest results and he'd be happy to talk to us about it if we were interested. Jeff and I made plans to go and meet with him at Primary Children's in Salt Lake over Christmas.

In the meantime, I went into high gear, trying to do anything and everything I could think of to help Keira in the event that she went completely deaf, like, tomorrow. I got super critical of her current speech and language therapy and decided they were moving too slowly. I got her started with a 2nd, additional speech therapist to do some supplemental work with Keira. I got her on the waitlist to see a 3rd, one who is renowned across the valley for expertise in AAC (alternative and adaptive communication). We got in contact with the Tobii Dynavox company, one of just two that makes AAC devices with eye gaze tracking. We initiated a 4-week trial of a "Tobii" device. "Just to see."

After Christmas, we left the boys with their aunt Shelly and cousin Sam and took Keira Salt Lake City. We met with Dr. Park, which was quite satisfying. As someone who knows CMV, he agreed that it’s likely Keira could lose the rest of her hearing in both ears and quickly. Bad news, yes, but so encouraging to have my suspicions validated. I felt like the doctors in Idaho thought I was overreacting. Park told us about a study he is doing that was just, I mean JUST, proving to have some measurable results. After following his protocol, some kids were not only having their hearing stabilized, but actually improved! 

The protocol is this: As soon as we got home, we put Keira on a 3 week regimen of oral (G-tube) steroid (prednisone). Then, after a few weeks we’ll have her hearing tested again and if there is ANY improvement, they will put tubes in her ears and we’ll give her steroid ear drops indefinitely. He also recommended testing her hearing every 3 months. 

Keira survived the oral steroid. Park told us that most kids take that dose without issue. I didn’t tell him that Keira’s mother once had to discontinue a steroid eye drop, an eye drop!, because it was making her crazy. During the first week especially, Keira was cranky, flushed, and sometimes inconsolable. But it got better as we tapered her down. Plus we got some interesting results right away. 

We had to coordinate the care protocol with a local ENT. While at that appointment, the pressure in Keira’s ears tested normal! Normal! That hadn’t happened in years, if ever, and is most certainly due to the steroid. This gives us hope that she will test well enough to get tubes and ongoing intervention. 

In the meantime, Keira received her very first hearing aid. It’s been unremarkable. She tolerates it swimmingly, but we don’t really notice any difference in her hearing or her behavior. And we did her 4 week trial of the Tobii Dynavox. 

Keira's response to the Tobii was really incredible. It broke my heart and made it soar all at the same time. 

To train her to use the device, first we have to teach her how it works. It came equipped with several games for that purpose. For example, on “Pie Face,” there is a small picture of one of her family members in an otherwise black screen. When she looks directly at the picture, it triggers animation of a pie in that person’s face then switches to a new photo in a different portion of the screen. 

The Tobii is basically a glorified iPad that has a strip across the bottom that tracks her eye movements. When setting it up and calibrating it, you can see where exactly she’s looking, which was fascinating. We saw that when trying to purposefully look for something, she was really only using one eye, her right eye. Then we observed that she had a hard time looking at things on the left side of the screen. To compensate, she would use all of her strength and rapidly move her head to the left, trying to capture the picture with her right eye. After 10 minutes of this, her face will be flushed with the effort. But, oh, was she ever proud of herself! She was controlling something in a way she never had before. And in spite of her visual impairments, she was getting it!

Eventually, the idea is that Keira would graduate from the games to communication boards. With her eyes, she would choose pictures off a grid to represent what she wanted and the Tobii would “speak.” We’ve got a long way to go with that. Just like any computer, the Tobii has its glitches and limitations. The programs available for young children are few and with the eye-gaze adaptability even fewer. What I had anticipated was an experience like we’d had with the boys on the iPad. I thought we’d be able to choose from a variety of apps featuring cute, colorful characters teaching the alphabet and manners and rhyming. I was sorely mistaken but found instead that I can create these programs myself. (Insert picture of my head exploding). Great. I’ve always wanted to dabble in computer programming and curriculum building. Not. 

I practically kicked my heels when I was able to finally put that damned machine back in the mail. It felt like so much pressure. It’s much more cumbersome, too, than just asking Keira which thing she wants and waiting for a response in her expression. But, she may not be able to hear us always, so we’ll keep on keepin on. In fact, we expect a delivery today of the PRC, the other option in AAC eye-tracking equipment, to begin another trial. 






So all this brings me today. As I write, Keira is getting sedated for her ABR. If the results are what we hope, she’ll have tubes put in her ears. While we’re at it, she’s also going to have Botox injections, xrays, and dental work. 

If K’s hearing has not improved, then we'll just deal with what comes as far as her hearing is concerned. The worst case scenario is more hearing loss, another hearing aid, and eventual cochlear implants. 

She was as sweet as can be this morning. She gets nervous at the point that we go into the pre-op room, but she understands what we are telling her enough that we can visually see her trying to stay calm. She chose peach chapstick for her mask this time and she has Lovey and Peppa along for the ride.