My husband and I had our first child, Jack, last September. Jack is an amazing one-year-old little boy who has the ability to brighten anyone’s day with his smile. While he is like many other one-year-old boys in many ways, one thing makes Jack special: He has magic ears! Before last September, childhood hearing loss would have never crossed my mind. Boy how things changed in a year!
The afternoon after Jack made his arrival in to this world, an audiologist came in to our hospital room to explain the newborn hearing screening she was going to do on Jack and that all newborns have to have the test before leaving the hospital.
I knew they completed the test at the hospital, but it never crossed my mind that something could be wrong with his hearing. I watched as she put the tiny tube into his ear and played sounds. Neither ear passed the test. I remember the audiologist telling us that it is not uncommon to not pass the first time and that he may just have fluid in his ears. She let us know that they would stop back before we left the hospital to test again. When she came back, Jack did not pass the hearing test the second time. I went from being overjoyed about taking our little boy home to terrified that Jack may not be able to hear.
We were set up with a follow-up appointment a few weeks later with a local audiologist to test again. The third test showed the same results. This time he was not as cooperative (what one month old would be?); he was awake and moving, so getting an accurate test result was challenging. At the end of the appointment she suggested we see an audiologist specializing in babies and children, so we headed to Cincinnati Children’s.
Jack had his first visit to Cincinnati Children’s Liberty Campus the first Monday in November. He wasn’t even two months old. I remember the anxiety I felt driving to the appointment like it was yesterday. Could Jack hear perfectly fine and it was just fluid in his ears, could he hear but not clearly, or could it be possible that Jack was completely deaf?
When we arrived at the Liberty Campus we were doing our best to keep Jack awake. We were told that for this specific hearing test, he would need to be asleep for sixty to ninety minutes. The audiologist explained that she would be doing an auditory brainstem response (ABR) test and would be putting electrodes on his head and measuring the brain wave activity in response to sound. Jack slept soundly for the entire test. As she finished, she explained that Jack had mild to moderate hearing loss that would require the use of hearing aids. Another ABR would be necessary to confirm the hearing loss and the level of loss, but that hearing aids were likely.
It felt like our world came crashing down, and I had a lot of questions. How could our barely two-month-old baby be given a prognosis that will affect his ENTIRE life? Was Jack going to be able to develop normal speech? Will he be able to attend a normal school with the rest of his peers? Will kids make fun of him because of his hearing aids? Will he go to college? Will he be successful in a career? I was flooded with emotion and then felt guilty for feeling this way, knowing that Brian and I were leaving the hospital with a healthy baby with hearing loss when other families could be leaving the hospital without their child.
The second ABR a month later confirmed his hearing loss at a moderate level. It was then that we learned more about childhood hearing aids (all of the colors we could choose from) as well as what to expect in the future. We set up an appointment with an ENT specializing in hearing loss and talked about what the next few months would hold for Jack.
When it came time to decide on Jack’s hearing aids, Brian and I struggled a little. Did we want something bright and fun, or something more muted and neutral? It wasn’t that we wanted to hide the fact that Jack had hearing aids, but we also didn’t want it to be the first thing people noticed about him.
His audiologist helped us through the decision-making process. People were going to notice them regardless, so why not make them something exciting, why not make them magic? She couldn’t have been more right. For his first hearing aids we chose a silver color and his first molds were blue, yellow and white (the colors of the school Jack will be attending when he gets older). Jack was fitted for his magic ears at the end of January.
I will never forget the moment they were first put in. The look on his face was priceless. It was clear we were getting the help we
needed for Jack to succeed in life. He immediately began reacting to our voices and sounds with smiles and body gestures, as you can see in this video we took. Leaving the hospital that day was the first time I had walked out of those doors feeling excited and nervous but not scared for what the future would hold for him.
Throughout those few months, we were also working with our ENT to determine the cause of Jack’s hearing loss. We were scheduled for genetic testing to rule out the main genetic causes of childhood hearing loss. It was none of those and at that time we were thankful.
Jack was then scheduled for a head CT to test for Enlarged Vestibular Aqueduct (EVA) syndrome. The radiology department put my mind at ease by explaining very clearly what they were going to do. The CT confirmed that Jack did indeed have EVA.
We were once again leaving the hospital with what felt like terrible news. At a follow up appointment our ENT explained that EVA can be caused by Pendred Syndrome, a genetic disorder that causes 5-10% of heredity hearing loss.
The diagnosis of EVA and Pendred Syndrome was extremely hard to cope with. Not only would Jack have to wear hearing aids the rest of his life, but with this syndrome, hearing loss progression becomes much more likely. We began talking about cochlear implants (not that Jack needed them at this time, but that they are an option if his hearing loss does progress).
As if the progression on its own was not bad enough, with Pendred and EVA, a significant hit to the head can cause instant progression of hearing loss or worse, total deafness. That news felt like Jack lost part of his childhood before it even started. For him it means no football, no soccer, no hockey, no trampolines, and no activities in which head injuries are common. It also meant Jack gained two extremely cautious parents. Each fall he takes my heart skips a beat. Since Pendred Syndrome is a genetic disorder, there is a one in four chance that any future children Brian and I have will be born with the syndrome as well.
As we are now a year in to this journey, I can say we are at a much better place than we were last winter. Jack has adjusted well and his progress continues to amaze us. We see his audiologist at Cincinnati Children’s regularly and his growing ears get new molds every three months. He currently has scarlet and gray molds (Go Bucks!). Picking out new molds and showing them off has become a part of the process that we look forward to.
Jack also sees a speech therapist, auditory verbal therapist, and a Regional Infant Hearing Specialist that comes to our home. She works with Jack on developing speech and hearing through every day activities. He has started to associate objects with sounds and we are over-the-moon excited about it! Just this week he picked up his airplane and said ahh-ahh-ahh-ahh.
Jack will continue to have hearing tests twice a year to ensure his level of hearing loss has not progressed. Our main goal for Jack right now is to be introduced to as much language as possible while he is at a moderate hearing loss level. Since Jack’s condition is life-long, we are happy to be at a place where everyone makes us feel at home.
I’m sure the next 18 years will be filled with ups and downs as it relates to Jack’s hearing, but we are confident he is in great hands at Cincinnati Children’s. Brian and I know that the world is at Jack’s fingers, and we can’t wait to see what the future holds for him and his “magic ears!”
Editor’s note: The cover image was taken by Heather Marie Photography in Delphos, OH.
Whitney – you made me cry… really, really hard tonight. Your blog, your story (Jack’s story), and talk of MAGIC EARS… was so much my experience 11 years ago, and it hit me really hard.
From the failed newborn hearing tests… failed ABRs… first hearing aids at 2 months of age… genetic testing… to the RIHP visits… audiologist visits… ENTs… and especially, all of the fear for my daughter’s future, it all came flooding back tonight. My daughter turned 11 this week, and she definitely doesn’t let hearing loss define her… but lately, she is uber aware that hearing loss and hearing aids make her different, and that’s been yet another challenge.
But, I just felt compelled to reach out… let you know that you, Brian and Jack are not alone. And, that you have such great support in the RIHP and Help Me Grow, as well as from Cincy Childrens. (We have lots of experience with the most wonderful pediatric audiologists in the world!) I also gladly offer myself and my daughter as a connection for you and yours, as she can likely offer a unique perspective. Best wishes always!