Saturday, February 27, 2010

Audrey's ear

I haven't really felt much like writing about it until now ... I think I needed a little time to process everything.

Short story: Audrey has a mild-moderate hearing loss in her right ear -- which is called unilateral sensorineural hearing loss -- due to a Mondini malformation. Basically, a normal cochlea should have 2.5 turns, and hers only has 2.

Longer story: She failed two hearing screenings in the hospital before we were sent home. We were momentarily upset until the nursing staff and many friends/family told us that she probably just had fluid in her ears from birth and that we shouldn't worry about it. She did need lots of suctioning when born, so we told ourselves it was a non-issue.

We were put in touch with First Steps, which is Indiana's version of the early intervention program that every state has, and scheduled an appointment at a local hospital with an audiologist when she was around 3 weeks old. There she had an auditory brainstem response (ABR) test done, which is actually kind of cool. Electrodes are placed on each side of the forehead and behind each ear and then sounds at various levels are played into the ear via a little earbud. The brainwaves tell the audiologist whether the sounds are being heard or not. I sat and held Audrey as the test was conducted and tried to interpret the brainwaves on the screen for myself. I noticed that the audiologist was doing a bit of sighing and clicking at one point and started to get worried. Sure enough, the test showed that she does indeed have a hearing loss in her right ear (but the left ear was fine). She can hear up to 50 decibels in the right -- normal conversation would be at about 50 db -- but nothing below that. I was given a book titled "workbook for parents of children who are newly identified as hard of hearing" and tried not to cry until I got in the car and called my husband. Hard of hearing. I knew it could be worse, of course, but no one wants their newborn baby to have anything wrong with them.

The audiologist said she strongly recommended that Audrey be fit for a hearing aid so that she didn't suffer from any speech delays. That didn't go over very well with me at the time -- all I could picture was a big, clunky device that would make her a prime target for teasing by her peers in school. She also suggested that we take her to an ENT for follow-up, which we did.

The ENT told us that there are several syndromes, all very serious and some even life threatening, that accompany Mondini malformation in children. After reviewing Audrey's bloodwork from her hospital newborn screening, he told us she didn't appear to have any of those problems. Obviously, that was a relief. The ENT told us we should have a CT scan done so that we could rule out a couple of different anatomical things, one being large vestibular canals that could result in instant and permanent hearing loss in both ears should she ever have a head injury. He also mentioned having her checked out by a pediatric opthalmologist, because kids with hearing loss can also often have eye problems as well.

So about a week later, I found myself back at the hospital with Audrey, this time in the outpatient surgery department. She was going to have to be sedated with general anesthesia, because apparently you have to be entirely still for a head CT or they'd have to keep starting over. My husband stayed home from work that morning to watch Olivia, so I had to deal with it all on my own -- the screaming, hungry baby who wailed the entire way to the hospital because she was hungry (no food 4 hours prior to test due to anesthesia), and the worst part, watching her be sedated via a tiny pink little gas mask that I later learned was scented like cherries. The anesthesiologist assured me that yes, the way she was breathing was normal and that she was already under even though her eyes were open. He asked me if I wanted to kiss her, and I was like, "Well, yeah!" And then it hit me -- he wanted me to leave the room. I think that was my hardest moment as a parent so far: walking out of the room pushing the empty stroller with her car seat attached and turning around to see my tiny baby in her pink and brown striped PJs on this huge table with an even bigger CT machine nearby. A wonderful volunteer distracted me with a friendly stream of chatter as he walked me to the waiting room desk, where I checked in and was given a pager. I realized in my fog that it was the same waiting room where I sat and waited while my mom was having her knee surgery several years ago.

I was thankfully only away from her for about 15-20 minutes, just enough time to scarf down a bagel, call my husband, and call my mom. A nurse came to take me to the recovery room, and I was relieved to see that she was the only one there (aka, no adults coming out of anesthesia and vomiting everywhere). I held her for a few minutes while giving her a bottle of pedialyte, and then they wheeled us -- me on the bed holding Audrey, like we were in some sort of hospital parade -- to another room where we waited for a nurse to take out her IV. I expected her to be pretty out of it for the rest of the day, but she handled it like a champ, napping only briefly in the car on the way home and then getting back to her normal schedule pretty much immediately. Anesthesia is one of my biggest fears, so I was so happy that she came through it ok and with no side effects.

The ENT called about a week later and told me about the Mondini malformation diagnosis. She doesn't have the large vestibular canals, so we don't have to worry about keeping her out of sports. And he said that while he can't predict the future, he doesn't think we need to worry about her hearing getting worse in that ear. Fairly good news all around, and I was happy that we at least had a reason for the hearing loss -- we were told that most of the time, the CT doesn't show anything and you're just left to wonder.

The pediatric opthalmologist was great and said she looks perfect; we go back when she's around 1 for another check and then he said he won't need to see her again if all looks good.

Now we're in the process of getting her hearing aid. The First Steps program pays for all things hearing related until she's 3, which will be a big help. Hearing aids can cost over $1000, and insurance doesn't cover any part of them (which I find ridiculous). She'll be wearing a behind-the-ear (BTE) style that will connect to a plastic piece that will go in her ear. I was told we could pick any color we wanted for the ear mold, but I went with clear with pink glitter to match the baby pink metallic BTE piece. It's actually pretty tiny and cute as far as these things go ... and you can be sure that I'll be posting pictures of her modeling it as soon as it comes in sometime in mid-March. The ear molds need to be custom fit, and the audiologist said that sometimes the kids will outgrow them between the fitting -- where they squeeze pink goo into the ear and let it harden a bit, somewhat like how they do dental impressions -- and when they come back from the lab about 2 weeks later. We'll also be working with a treatment team that includes a parent advisor who has a background in childhood/deaf education and will help us keep an eye out for any speech delays that might occur as a result of her hearing loss. Hopefully that won't be a problem, but you never know.

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