A New Bump in the Road

Some days, all we can do is laugh. And in the last few days we’ve been doing quite a bit of shocked chuckling. Evie apparently doesn’t think she’s unique enough, so she decided to add a new challenge.

Evie saw her audiologist on Tuesday, where we discussed some of the results of Evie’s sedated hearing test back in June.  This type of test measures the response of the auditory nerve to different sounds, but on Evie’s left side, the nerve did some “weird” stuff. Yes, that is an exact quote. “Weird”.  Rather new in its discovery in the audiology world, this type of response is known as Auditory Neuropathy. Here’s a quick and simple explanation:

“Think about driving your car with the radio on, but you have a frayed wire somewhere between the radio and the speaker system. The sound is entering the perfectly operational radio but is not able to get through the wire and into the speaker in the correct way. Now replace the words radio, wire, and speaker in that sentence with the words cochlea, nerve and brain.

The sound is entering the perfectly operational cochlea but is not able to get through the nerve and into the brain in the correct way. With the bad connection you get sound fading in and out and it sounds out of sync. You can turn the volume control knob up to try and correct this but the static sound just gets louder and louder and you still can’t understand most of the words because volume doesn’t correct the bad connection. This is why hearing aids rarely help the auditory neuropathy child.”

So…bummer. The good news is that Evie only has unilateral auditory neuropathy, so her right ear still hears very well with her hearing aid. The bad news is that unilateral is even more rare than bilateral which is already rare, so trying to find information on the best approach has been a little rough so far. I believe the phrase I’ve seen in regards to unilateral AN has been “a handful of cases”. Oh, Evie, you do like to be different!

When the audiologist did some testing in the sound booth, Evie always turned to her right in response to a voice even if the sound came from the left. She would however turn her head to the left in response to static noise on that side. So now the question is, do we continue with the hearing in the left to give her the benefit of the doubt (hoping the static isn’t too bad) or do we stop amplifying that ear because it may actually hinder her ability to hear with her good ear?

Many feel that cochlear implants are a good approach for AN, but there’s no guarantee that they will work in an individual child until you try and since CIs wipe out all natural hearing, that’s not something we’re willing to do at this point. As she gets older, she will hopefully be able to communicate with us on how that left ear really is working, but until then, please pray for wisdom on our parts. These first few years are such a critical time for language development and we want to give her the best opportunity to hear the words as we continue to work on sign language, since she cannot vocalize.

Let me be clear, this is not devastating news. We’re not devastated. I think we’re more just tired of the rollercoaster. I cannot even tell you how many times we’ve been given good news about Evie only to have it snatched away later. We thought her hearing would be fine with the hearing aids. We were incredibly grateful that her hearing could be improved so easily, so to now not know if that’s possible is difficult for us.

And also let me be clear, Evie is doing amazingly well. She’s doing more sign language all the time. She’s getting better at pulling to standing with her AFOs (ankle braces) on as she strengthens those leg muscles. She’s vibrant and growing and enjoying life. And we’re enjoying her.

Please just pray as we research and seek out specialists (umm..well…one specialist. There is only one specialist it seems.),that we get the necessary information to do what’s best for Evie. Thank you all for your continued prayers! They are being felt daily!


4 thoughts on “A New Bump in the Road

  1. At one point we were told that Laynie had AN but then they said they didn’t know. We are still trying to figure out what is going on with her hearing. It is frustrating because AN is so difficult to deal with. I am sorry that Evie has this! She is such a cutie though. I love your blog:)

  2. Our little ones love to be different, huh? Ronan has had his cardio case presented at a conference, and we have actually attended a pediatric ophthalmology conference before since both those aspects of him are “unusual”. :). I know it is hard, but I admire you for all the time, effort, and love you put into researching what is best for Evie. And I appreciate you sharing that with me! Like I say with Ronan, he is so much more than doctors ever said he would be, and so is Evie.

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