Our Hearing Loss Journey

In honor of Deaf Awareness Month, I wanted to share with you all in more detail about our journey with Rhett’s hearing loss. I know I have shared bits and pieces throughout previous blog posts, but I wanted to take the time to focus on hearing loss specifically, and share how that has affected Rhett and our family summed up into one post.

I am going to start from the very beginning. We found out about Rhett’s hearing loss very early on. A few days after birth, once Rhett had been transported from the hospital he was born at to the children’s hospital right down the road, Rhett had a CT scan to find out what was going on with his nasal passages. It was after the scan that we found out about Rhett’s inner ear abnormalities and how that could affect his hearing. This scan told us that Rhett had a malformed cochlea in his left ear and that he was missing his semi-circular canals in both ears. It was also after this scan that we were told it was highly suspected that Rhett had CHARGE Syndrome. This news hit me like a ton of bricks. There were so many unknowns and I just couldn’t wrap my head around my new sweet baby having to face so many challenges. Even though I knew hearing loss was very likely, I prayed that was not the case. I asked God to please let him have hearing. I could not imagine how in the world I was going to be able to parent a child with hearing loss. So I prayed very hard for 2 days that Rhett was able to hear.

The day that Rhett had surgery to have the trach placed, he also had an ABR hearing test done while under anesthesia. An ABR shows the brain’s response to sound which lets us know what his level of hearing is. Hearing is measured in ranges based on the pitch and volume of a sound. The ranges of hearing loss goes from mild-moderate, moderate-severe, and severe-profound. When you are in the severe-profound range you are considered deaf. The results of the ABR were that Rhett had mild-moderate loss in his right ear and severe-profound loss in his left. While this was not the results I had hoped for, I remember being so relieved. I think I knew deep down he was going to have some degree of hearing loss based on his anatomy, but he was not completely deaf. He had some useful hearing, and I was so thankful for that. The plan was to have him fitted for a hearing aid in his right ear, and begin therapy to help us learn how to most efficiently utilize the hearing that he had. We were told that a hearing aid on the left ear would not benefit him since his hearing loss was so severe, and that he also would not benefit from a Cochlear Implant in that ear because his anatomy was not typical. We were also told at that time that he did not have an auditory nerve in his left ear, and you can not do a Cochlear Implant without having an auditory nerve.

A month or so after being discharged from the NICU, Rhett had an appointment with audiology to check his hearing and to be fitted for his first hearing aid. We tried to do another ABR test while Rhett was napping in my arms, but it was difficult to keep him asleep with all of the things attached to his head. The information from this test, what information we could get, was pretty consistent with the other ABR which let our audiologist know where to start with programming his hearing aid. I remember during that visit I had to choose the color of the outer part of the aid and also the color ear molds. I tried to match the outer part as close as possible to his hair color and I chose clear ear molds. I remember thinking how Rhett had so many accessories already with the trach and g-tube that I didn’t want to bring attention to one more thing. I guess that was me being vain. I love that sweet little hearing aid and the sound it gave to Rhett, but I did regret the colors I chose on several occasions as little ones do not always leave their devices in their ears. When you have such a neutral color, it tends to blend in with everything. Talk about having a heart attack several times because the aid had been lost. I have even pulled over on the side of 52 several times to frantically search my car for a missing hearing aid, but these are stories for another time.

We monitored and tested his hearing pretty frequently the first few years. He had to go back to audiology every couple of months to have new ear molds made since his little ears grew and changed so quickly over the first few years. We have had some great audiologists, and we spent the first 3 years of his life in therapy with an early interventionist who specializes in children with hearing loss in the birth to 3 years age range. Our early intervention teacher, Preston, gave me so much guidance the first 3 years. She helped me focus on specific goals for Rhett’s language development, and also helped me learn so many signs. I will forever be grateful for her.

We were initially told that Rhett would not be a candidate for a Cochlear Implant in his left ear because an auditory nerve was not visible. Once we started traveling to Cincinnati for Rhett’s medical care, we learned that a CI for his left ear was an option. Rhett does have an auditory nerve to the left ear, but it is small. The doctors at Cincinnati are so familiar with children who have CHARGE that they were confident that Rhett would benefit from a CI in that ear, and he has. We are still unsure of what speech sounds he can actually hear with that ear, but we do know he is having some level of hearing and understanding through his Cochlear Implant.

We put off going forward with Rhett’s Cochlear Implant surgery because we were so focused on his airway for so long. Once we were at a point of his airway management that we knew we just needed to give him time to grow before his trach could be removed, we decided to then move forward with the left CI surgery. He was implanted in September of 2018, and activated in October of 2018. He has had sound to that ear for two years now, and in the last 6 months I’ve been confident that he is getting some level of hearing to that ear.

Right after surgery and activation, we spent our time and audiology appointments focused on maximizing the use of his new implant. During this time we sort of neglected his right ear. I started to notice Rhett responding less and less to people speaking, but I honestly thought he was being a toddler and ignoring us when he was focused on playing. Boy was I wrong and I still kick myself for not seeking more information about his right ear. During an appointment that we were checking on his right ear, his audiologist noticed him responding less during booth testing. I figured he was bored or uninterested in the task, but she decided Rhett needed another ABR test to get more information about that ear. Since Rhett was having scopes so frequently, we added that onto one of his routine scopes. Once he had the ABR, we learned that his hearing had regressed from mild-moderate to moderate-severe. I felt so awful because Rhett had been going about life without having the best access to sound, and I don’t know how long that had been going on. We had his hearing aid turned louder and I started paying closer attention to Rhett’s response to sound. Several months later when Rhett was under anesthesia for another scope, he had a repeat ABR done. This time his hearing had regressed even more to the severe-profound range. I cried. I was so heartbroken for him and so mad at myself for not noticing sooner. I was heartbroken because I knew how much he enjoyed songs and animal sounds and I feared he would miss out on that now that his hearing loss was to the point of deafness. He had his hearing aid turned up to the max, but often times having it louder does not help with hearing sound clearly. He wore his hearing aid at the max level for almost a year before finally having the second implant done. Once his hearing loss in that ear was at the severe-profound range, we knew he would most likely benefit from a Cochlear Implant on that ear as well. We began the long process of going through more testing and insurance in order for the surgery to be approved. We finally had surgery scheduled for the spring of this past year, but then COVID happened and postponed it. He was implanted and activated this past June, and he is already doing so well. He is responding quicker and quicker to speech, and I know it is helping so much with his understanding of things. He has also started to become more vocal although sign remains his preferred form of communication.

Rhett currently does Auditory Verbal Therapy twice a week to help him learn how to hear with his devices, speech therapy once a week to work on his communication skills, and twice a week he works with a teacher for the deaf and hard of hearing to learn new signs and vocabulary. He is a busy boy and a lot goes into helping him communicate as effectively as possible. Getting a Cochlear Implant was the best decision for him, but it is certainly not an easy fix to his hearing loss. Rhett is deaf with or without the Cochlear Implants, but they are a tool to help him best access language. We are using what is called a total communication approach to Rhett’s language development which uses a combination of spoken language, sign language, and pictures. Rhett is doing so well, but he still has a long road ahead. This journey has been hard, but it has taught me so much. My biggest take away from Rhett’s hearing loss journey is the never ending work he puts into understanding language. Let’s all learn a lesson from Rhett and work hard with whatever God gives us.

Let’s Talk Disability

Let me preface this post by saying that what I have written is based on what I have been thinking and feeling. I have been listening, researching, and reflecting from others who either have disabilities or from other parents who have children with disabilities. I am bringing this topic up because I think it is one that needs to be discussed more frequently so that it is not looked upon with negative connotations.

So the word I want to discuss today is “disability.” I will admit, this word has been a difficult one for me to accept when it comes to Rhett. In my mind, he can do absolutely anything he wants to do, which is true. The way he accomplishes things may look different than what others do, but he can still reach any goal he wishes. The word disability has become sort of taboo, which is what needs to change. I have had a lot of difficulty accepting that word as a term to describe Rhett, but why? Just because he has disabilities does not make him less. It does not change who he is and how amazing he is. It’s just another word to describe his uniqueness. Now I’m getting off my point. What I am trying to say is that the word disability should not be treated as a bad word. It is a word to describe a person, but the disability is not the person.

Let’s take a look at what the word disability means. The prefix “dis” means being the opposite. For example dislike means to not like something. So disability means not having the ability. One of Rhett’s disabilities is deafness. It has taken me a long time to say that word when talking about Rhett and his hearing loss, but it is the truth. Rhett does not have the ability to hear (without devices), therefore, has a disability of deafness. Now the point I want to make is that Rhett is deaf, but being deaf does not define his abilities. He is still able to communicate, learn, play, and be a kid. Having a disability does not mean he does not have many abilities, but those abilities may look different than what you are used to. His abilities might shine through with the support of an interpreter or special devices like his Cochlear Implants, but the abilities are there. Some people may have a disability of being unable to walk, but they are able to get around with a wheelchair. Some people may have a disability of blindness, but they can learn to read with a special text or walk using a cane. Some people may have a learning disability, but they are still able to learn when taught with certain modifications. With all that being said, a person who has a disability may need accommodations in order to accomplish all they are able to do.

People with disabilities can teach us important lessons. They show us that there is not one right way to do something or reach a goal. They show us that though there may be barriers or challenges, they are still able to do so many things. It’s time to look past the word disability being a negative way to describe part of a person. It is time we all acknowledge when someone has a disability, recognize there may be barriers for them, but see the person for all their amazing abilities. Let’s embrace the term in a positive light so that we may have a more accepting and inclusive world for those who have disabilities.

Quick Update

We are almost one full week post op with the CI surgery, and Rhett is healing wonderfully. He is pretty much back to his normal self, and we are only giving pain medicine as needed. His incision is looking great, and he doesn’t seem to be bothered by it at all besides the itching from healing.

The surgeon said that everything went perfect during surgery. This ear’s anatomy is more typical than his left ear, which means his response to the implant should also be more typical. Rhett did spend one night in the hospital to be monitored, and he slept a big part of that time. We were able to keep his pain managed by alternating Tylenol and Motrin, and he was discharged the next day. Rhett took a few days to get back to his happy playful self, but I can’t say I blamed him. He did take naps the two days following surgery, which is not his norm. He left his head band bandage alone for the most part, until day 3 of wearing it. Having it on seemed to itch and bother him due to how hot it was. Thankfully he does not need to wear that any longer.

We will be going back to Cincinnati next week for the activation. I am so excited to see how he responds with this new hearing device. I hope and pray that it will give him better access to sound, especially speech sounds. We will continue to use sign language, but I am still hopeful for him to speak one day. During our trip next week Rhett will also have a sleep study done. This is another step in the process to get the trach removed. Please pray that the study goes well, and we get good results. I do not have any reason to believe we should find out anything negative, but I do not put anything past Rhett. He has a way of functioning completely fine even if there are underlying problems. Also pray that he will handle all of the things they have to put on his head and body, and that he will go to sleep. We will be leaving Brooks with Nana and Papa once again, so pray that Brooks does well while we are away. We will update again as soon as we get back and have the results!!

Pre-surgery selfie
Rhett did a lot of sleeping following surgery.
Day after surgery. He wasn’t smiling a ton, but we were able to see some smiles with the Minion movie.