Hearing Loss Intro. #tuneintuesday

I’ve learned more than what I ever thought I would need or want to know about hearing loss in the last 7 months.

My goal is to write a post every Tuesday about hearing or hearing loss or something related to hearing. Before hearing loss affected our family, I knew zero about it. I think it’s important to know some things even if you don’t have hearing loss and other things, well I just want you to know them so I can bring awareness to it. Here’s my #tuneintuesday post for today. This is an intro so it’ll be a little long (edit: really long 😳), but I hope to keep other posts short, sweet, and informative.

My son, John, turns 3 this week. 7 months ago, he was diagnosed with bi-lateral (both ears) hearing loss through a test called an ABR in which he was sedated to be able to have it completed by an audiologist. Since then, that same audiologist has met with us countless times – some expected and some unexpected – due to our son’s frequently changing hearing. I’ll explain more, but let me back up and start from the beginning and how all this happened.

*Sidenote: While I think this is a very interesting story, it may have no meaning to you. That’s totally ok! I just want to document it because I know years from now I’ll want to remember it all. ❤️

The Beginning: March – August 2015

John was born 12 days early on St. Patrick’s Day, March 17, 2015. I had a very healthy pregnancy, I did Crossfit up until I was 37.5 weeks, and taught kindergarten through flu season without ever getting sick. At 37.5 weeks, I had to go on bed rest because I was showing symptoms of pre-eclampsia. I was sent to the hospital for 2 nights to monitor my blood pressure and they ran a few tests. Thankfully, I was sent home with strict orders to not get out of bed and hopefully John would make his arrival before I reached 39 weeks, when they would insist on inducing. I really didn’t want that to happen, so I started taking evening primrose oil and prayed he would come soon!

Sure enough, less than 24 hours later, I went into labor and 3 hours later he was here. It was my dream birth, everything I hoped would happen and planned for the last 9 months, happened almost exactly how I had planned it in my head. It was perfect. He was perfect! We were so excited to finally be holding our little guy here on earth. Other than his hearing tests, everything else he passed with flying colors. His failed the newborn hearing screening in both ears. The doctors assured us that this is something that’s pretty common, not to worry and that it’s probably just fluid. They told us to wait another week or two and then have it tested again. We went to an audiologist when he was around 2 weeks to have him tested. One ear passed and the other failed again. We were sent to another audiologist for a more extensive test, one that (supposedly) can’t be argued and tell you if indeed they can hear or not hear. We waited until he was 4 months to have this one done. Most of the time, babies are sedated because this test is so sensitive. It’s important for them to be completely still to get accurate results. For information that will be better understood later, John was NOT sedated during this test. After almost 3 hours in the testing room with the audiologist, she said he passed. It was weird and we didn’t understand (gosh we know so much more now, we probably could give the test at this point!) because at one point during the test she commented that he couldn’t hear at all then he could then he couldn’t again and here we were, brand new parents, hanging on to her every word. But, to our delight, we left 3 hours later with a confident answer from the audiologist that he was fine. He could hear! Two months later, John had a health scare that ended us in the pediatric emergency room. This was completely unrelated to his hearing loss, but it’s important to note that at 6 months he had a CT scan at that ER visit that would be very important almost 2 years later. You’ll understand what that means in just a few paragraphs!

May – July 2017 – 2 Years Later.

John turned 2 in March of 2015. Being a teacher myself, I knew something was not right with his language. He was talking, but what he was saying just wasn’t making sense. I had one of my teacher friends from the school I used to teach at come over and tell me I wasn’t crazy. 😉 I knew John was “developmentally normal” but his language just seemed off. I was encouraged by so many people, even his pediatrician, that boys can be late talkers, walkers, etc. and not to worry. After all, John didn’t walk until 15 months so he was a little late. I’m not one to overly worry about him, I’m still not, but as much as I tried to tell myself that he was just a “late talker” something (The Holy Spirit is what it was, I know that now!!) told me that it was more. My friend gave us some great ideas to help him with his speech and we hired a speech pathologist to work with him for the summer. We also had him evaluated for Babies Can’t Wait, a grant-funded program to provide services to special needs children who are three and under. While he was getting therapy, he still didn’t seem to be improving. The “weird” ABR test at 4 months never sat right with me or my husband either. We decided to have his hearing checked again, because why not it wouldn’t hurt, right?

I had no idea where to start. No one recommended that we get his hearing checked, everyone was more worried about his speech. He did qualify for speech services through BCW, so naturally the next step was speech therapy. I don’t know if it is because hearing loss is so rare, but I am so thankful our mama and Daddy intuition kicked in and we decided to have his hearing re-checked. The first thing we did was book an appointment with a pediatric ENT. She conducted a very quick hearing test. John was awful during the test (this was one where he did not need to be asleep).We had to bribe him with goldfish and YouTube kids. (Big thanks to YouTube kids – getting us though most of our audiology appointments! 😬🤷🏼‍♀️) Anyway, he referred in both ears. The ENT wasn’t really concerned because she said that it could not be an accurate representation of his hearing because he was eating and a video was playing. Looking back, I think she was just trying to be nice, but whatever. Because he failed both ears during that test, we were referred to the children’s hospital to have a sedated ABR, aka the SAME test we were supposed to have when he was 4 months. I don’t think I’ll ever get over that darn test.

Fast forward about a week, and we are at Children’s Healthcare of Atlanta (CHOA, I’ll refer to as from now on) getting a sedated ABR. We met with the audiologist beforehand and she explained it would take about 20 minutes, not to worry he would be monitored the whole time he was sedated, etc etc. CHOA is an AMAZING place and we felt good and hopeful that everything was going to be fine. Right? Because who actually thinks they’re going to get bad news?!?! Not (always optimistic) me!

Well, about 45 minutes later Steven and I are actually freaking out, the audiologist comes in and gives us the results. Thank goodness we got them immediately.

I never forget how quickly she related information and was so straight forward and honest about it, while also being empathetic and compassionate. The Lord knew we needed someone to guide us through this process, and John’s sweet Doctor was a perfect fit. We found out that John had bi-lateral hearing loss. She told us his right ear was moderate leaning towards severe and his left ear was mild leaning towards moderate. She also said that he would need hearing aids. Our first questions, which you are probably thinking of too, were: Will he have to wear hearing aids for the rest of his life? and will his hearing get better? Yes, he will have hearing aids for life and no it will not get better. Actually, it will get worse, but we do not find that out until later. Once John was up and moving, we immediately walked next door and had him fitted for hearing aids. Two weeks later, we were back in her office trying on John’s hearing aids. It was a tough transition for Steven and me. I was in the midst of a breast cancer diagnosis and going through surgery right when all of this was happening. I was fine, but it was just a rough few months for us to process what was happening to us. Thankfully, John took to the hearing aids perfectly fine! It was better than we all expected. He actually loved wearing them and we never really had any trouble with him keeping them on throughout the day. Even now, 7 months later, the kid is a complete champ. He makes sure we never forget to put them on and is so responsible with them. I never thought I’d say that about a toddler.

In between getting his hearing aids and his sound booth check a few months later, we made a few changes, got a few things sorted out, and learned way more than we ever imagined about hearing loss.

First, we had to meet with an ENT who could help walk us through this process. We found a great one and she helped explain hearing loss and what may have caused it for John. We were going to have to go through a series of tests to check his kidneys, heart, and eyes because apparently there are life threatening symptoms related to hearing loss that we knew nothing about. We were also going to get genetic testing in case anything was life threatening, we wanted to know more about it and the cause. No-one on either side of our families have early on-set hearing loss. We left the ENT feeling pretty confident with a plan to determine the cause of his hearing loss and next steps.

Less than 5 minutes after we left, we got a call from the ENT who we had just finished meeting with and she had a diagnosis for John, a reason for his hearing loss that needed to be confirmed with a CT scan. As it turns out, the CT scan that he had from an unrelated trip to the ER at almost 6 months old showed the problem. She was able to see his ears on the scan and a radiologist confirmed what we now know is the cause of John’s hearing loss, EVA. Enlarged Vestibular Aqueducts are present in both of his ears. This is a developmental abnormality that he has likely had since birth, thus confirming his hearing loss that has been present since he was born. EVA is tricky though, it’s changes and is inconsistent. Because it’s related to his Vestibular system, it explains why he may have walked later than typical children and why he is sometimes clumsy and seems off balance.

We had another CT scan to confirm EVA and to check for anything else that the CT scan at 6 months didn’t pick up. During the CT scan, we found out that in addition to EVA he also has mondini malformations in both of his cochleas. This means that John doesn’t have the same amount of turns of the cochlea as a normal person. Most people have 2 1/2, John has 1 1/2. More on Mondini and EVA in another post!

During this time, we decided it would be best to start John on Audio Verbal Therapy (AVT). One day in another post, I’ll explain more and how this is different from speech therapy, but in a nutshell John has to learn to listen before he can speak correctly and that’s what audio verbal therapy does. We go to audio verbal therapy every other week and in between work with him at home.

We also decided to enroll John in a program at the Atlanta Speech School. They also have an audio verbal program and it has been a great fit for him.

December 2017 – January 2018: By the time December came around, we were getting into our groove with BCW therapy every week and traveling to Atlanta for speech school and AVT. December was the month we had been preparing for since August, when John got his hearing aids. December was his first “check up” appointment with the audiologist in the sound booth. Little did we know, this would become a frequent occurrence with EVA. The first appointment did not go as we hoped. His right ear declined much more rapidly than we thought, but it wasn’t so bad that hearing aids couldn’t help. However, cochlear implants went from “well he may need them in the next 10-15 years to ok now he will need them in probably the next 3-4 years.” We were honestly shocked. Both of us not really understanding the magnitude of how bad this really could be.

Then, a month later, we got the news that we never even thought was possible. After a bump on the head at school (we think was the cause but we’ll never know for sure because what toddler doesn’t bump their head?!?), John completely lost his hearing. He stopped responding to us and we knew he couldn’t hear us at all. We didn’t even know that was possible, but it’s a typical thing that can happen with kids who have EVA. So, our audiologist being as awesome as she always is, has us come in immediately for a sound booth test and confirmed that his right ear had significantly worsened. Thankfully, we were able to have a conference call with our ENT and she suggested putting him on steroids for a week to help boost his hearing in hopes of it returning back to where it was. If not, we were going to have to rush to have cochlear implant surgery ASAP. Thankfully, after much prayer and petition, his hearing began to come back around day 4/5 and we were so very thankful.

January – March 2018:

January – March continued to be more “seemingly bad news” with declining hearing in the sound booth. His right ear fluctuated and his left ear got worse. Then his right ear got worse again. We have been in the sound booth way more times than we ever thought we’d have to be at this point, but thankfully it provides us with a picture of what John’s hearing looks like since he can’t exactly tell us what we want to know. Now that the shock of John losing his hearing in January has passed, we’ve moved into the “let’s talk about implants” stage. Steven and I are so thankful that either through hearing aids or implants, John will always be able to hear. We think that we will be starting the cochlear implant journey in as soon as the next few months to the next year or so. The Lord has definitely “eased us” into this journey with all that has happened since January and we feel prepared and ready. Cochlear implants can be tricky, especially for EVA kids, because there’s not really a “good” time to implant. It’s honestly up to us as parents. We can try to wait it out and let the hearing aids do their job for as long as possible or go ahead and do the inevitable.

It’s honestly a tough decision, but thankfully we have an amazing team of doctors and support at the speech school to walk us through the whole process.

So that’s where we are as of today, a week before John’s 3rd Birthday. I just can’t believe that 3 years into this parenting journey and I could already write a book on what we’ve been through. I’m certain though, that all of this is not by mistake. The Lord knows what he’s doing through all of this and his plan is absolutely perfect.

Staytuned!