On the morning of May 21, 2010 my mom and I drove Benjamin to the Diagnostic Neurophysiology building in Tulsa for his scheduled sedated ABR (Auditory Brainstem Response) procedure. (That was all a mouthful, I know!) His first birthday was the very next day, and I could think of a hundred things I would rather be doing. Our son’s first year was full of so many hospital stays, doctor visits and medical procedures, however, that I had pretty much come to accept them as a normal part of life. And whether the results were good or bad, I was hoping that we would finally get some concrete answers that day about Benjamin’s hearing.
During his recent bout with infantile spasm seizures, he had a routine hearing test with his audiologist, a recommended intervention for children with Down syndrome, as around 50-60% of this population suffer from some degree of hearing loss. There are three possible types of hearing loss:
1. Conductive—referring to a build of fluid in the middle ears that creates interference with the conduction of sound vibrations in the middle ear
2. Sensorineural— referring to a damage to bones and/or nerves in the inner ear that affects the transfer of sound from the inner ear to the auditory nerve
3. Mixed—referring to the presence of both types of hearing loss
One common characterstic for children with Down syndrome is small ear canals, which can often increase the problem of fluid retention in the inner ear and lead to chronic ear infections, which in turn can cause conductive hearing loss. The good news about this type of loss is that it is not permanent. The surgical insertion of ear tubes to allow the inner ear to drain is often all it takes to return hearing to normal levels. Sensorineural hearing loss, on the other hand, is not curable. In young children with this type of hearing loss, the damage to the inner ear is often present at birth. It is the most common type of permanent hearing loss, and hearing aids are the most common solution to help improve hearing ability.
I am not wanting to sound like I’m writing out of a textbook, but I believe some basic information about the types of hearing loss will enable me to share our story with more credibility and meaning. When Benjamin had his hearing test in February 2010, the results were not good. He was not responsive to the level the audiologist would like to see. Behavioral hearing tests, however, are difficult with small children and leave a definite margin for error, but they can assist in determining if there may be a problem. We tested again in March, but the results were not improved. In daily life Benjamin was not as responsive as we would like either. The seizures had definitely played a part in this, but we couldn’t assume they were the only reason for the delays. The audiologist recommended us to a Ear Nose and Throat specialist for the purpose of having ear tubes inserted. The hope was that Benjamin’s potential hearing loss was conductive, and we would see an improvement after having the simple surgery.
His surgery was scheduled for the end of March. It was a simple out-patient procedure, but we still dreaded our son having to be sedated again for another operation. Hospital settings had become all too familiar. Our ENT was very helpful and kind, but he told us before surgery that Benjamin’s ear canals were some of the smallest he had ever seen, even in children with Down syndrome. He could not guarantee that he would be successful in inserting the ear tubes. Thankfully, though, the surgery was a success, but he told us that he saw very little fluid in our son’s ears. We still hoped we would begin to see improvement in Benjamin’s responsiveness, but this was not the case.
For this reason, I found myself in Tulsa the day before my son’s birthday for the ABR test. In this particular test, the child must be asleep for the entire procedure. Benjamin was given oral medication to make him drowsy, and once he was asleep, the audiologist prepped him for the procedure while my mom and I waited in a small office across the hall. She told us the test itself would take around an hour for each ear, so we settled in for the wait. In an ABR, small electrodes are connected all over the child’s scalp and a small microphone in placed in the ear canal. A computer then charts the brain’s responses to sounds sent directly into the ear. It is the most accurate hearing test for small children.
I don’t remember how long we waited in that little room, but I do remember that the time seemed to drag by. My mom had flown in for a visit earlier in the week, and I was grateful for her company. It was an early morning test, and we were both sleepy. I was anxious as well, wanting answers, but afraid of what the answers might be. When the ABR was finally finished, the audiologist came to talk with us. To my dismay, she gave the report I didn’t want to hear. According to her test results, Benjamin had sensorineural hearing loss, mild in his right ear and moderate in his left. I tried to process what she was saying and asked if there was any chance his hearing would ever improve. She gently explained that his loss was permanent, and there was a possibility it may get worse over time. I felt numb. She recommended that we have him fitted for hearing aids soon, an intervention he would need for life. An excerpt of the official report reads as follows:
IMPRESSION: The above test battery is consistent with a moderately sloping most likely sensori-neural hearing loss in the left ear, and a mildly sloping most likely SN loss in the right. (Bone conduction thresholds abnormal).
1. Medical management of blocked vs extruded PE tubes au.
2. Bilateral hearing aid evaluation.
3. Close monitoring of hearing levels due to the possibility of progressive hearing loss.
My heart sank. Did this have to be the hallmark for the end of Benjamin’s first year? Hadn’t he been through more than enough already? Our little boy had experienced more medically in one year’s time than some people do over a life time. How much more would we have to deal with? For Shawn and me it was “one more thing” to throw on top of the pile.
The following day we celebrated our son’s first birthday with family and friends. Overall, the party went well, and we were all able to enjoy ourselves. I had some anxiety leading up to the party, however. I wanted everything to be perfect, and little details were blown up big in my emotions, making me feel tense and irritable. Shawn was encouraging me to relax and just enjoy myself, but I was struggling at first to do so. Inwardly I was chastising myself for behaving this way. Today was supposed to be a day of celebration, and I wanted to enjoy it. I soon realized the root of my intensity. During Benjamin’s first year, I felt so out of control of so many things. I felt so helpless to help my son on numerous occasions. His first birthday party felt to me like something I finally had a measure of control over; something special I could do for him, and I wanted it to be perfect since so many things had been less than perfect since his birth. Recognizing where I was helped me to relax some and regain perspective. The important thing that day was that we enjoy Benjamin and enjoy our loved ones celebrating with us. The other details were secondary.
Within a few weeks Benjamin was fitted for hearing aids, and they arrived less than two weeks later. On June 17, I drove my small son to Tulsa again, this time to The Scholl Center for Communication Disorders, to pick them up. I found it strangely ironic that this day marked the one year anniversary of Benjamin’s homecoming from the NICU. Who knew how much one year could hold! The audiologists at The Scholl Center were very kind and demonstrated to me how to insert and care for the new hearing aids. I was thankful that everything appeared to be quite simple. Benjamin didn’t put up the fuss I thought he would when they were first put in his ears. We were given a set of small fabric casings that slipped over each hearing aid and were attached to a string that could be clipped to the back of Benjamin’s shirt. This would help to ensure that they wouldn’t be lost if they fell out (or he pulled them out). I was also advised that a small strip of tupee tape on the back of each hearing aid would help hold it in place against my son’s head without any discomfort to him. In the weeks and months that followed, we discovered that we preferred to use the tape for day to day life at home and the casings for outings. Benjamin had two hearing tests that day (and/or a few weeks later at a follow up visit—my memory escapes me), one with and one without the hearing aids. With the hearing aids in place, his hearing tested close to normal levels. Shawn and I were so encouraged!
The drive home from Tulsa that day was a new experience. It was close to rush hour , so there was extra sound and activity on the highway. Benjamin screamed the whole way through Tulsa. I realized he’d never heard the amplified sounds of heavy traffic before, and the new sensation scared him. I was sad for him but excited as well!
Over the next few weeks, Benjamin adjusted to the hearing aids better than we could have imagined. It was obvious that they were helping him, and he liked having them in. He was still a long way from crawling at this point but was rolling all over the place, which did create a challenge for keeping the aids in place. However, it was rare that he actually pulled them out himself. If the molds weren’t fitting quite right, and he was getting a lot of feedback he’d take them out; otherwise they seemed to become a part of him. Shawn and I noticed a marked difference in his responsiveness as well, as did his therapist. Overall he was more alert and a happier little guy. We were so thankful!
Since children grow so quickly, Benjamin had to have new molds cast about every four months. He never did appreciate this procedure. After having his inner ears checked by the audiologist, he would have to be held down while a tiny piece of cotton with a string attached to it was pressed down inside his ear canal to protect his inner ear. Then the audiologist would fill the opening of his ear with a gooey paste that reminded me remarkably of Laffy Taffy, though I’m sure it didn’t taste the same! It had to set for a minute or two to stiffen, and when it was pulled out, it gave the exact replication of Benjamin’s ear. These molds were then sent to a lab where the actual hearing aid molds would be cast. I was able to choose from a variety of colors for the ear molds, but I always stuck with some shade of blue, though one time we did a blue and white swirl. I thought the blue brought out Benjamin’s eyes. For the hearing aids themselves, I had picked a neutral color that was the mix between a tan and a light brown. They would last for at least a few years. We had a sheet of hearing aid stickers to decorate them with, and I usually changed Benjamin’s stickers whenever he received new ear molds. I always wondered what his preferences would be for colors and stickers when he was old enough to choose himself.
The hearing aids quickly became a normal part of life, though Shawn and I never stopped asking God for a miracle in our son’s ears. We had already witnessed Benjamin’s healing from seizures, and we knew we had a miracle baby! Whenever there was the opportunity for others to pray for his hearing, we eagerly sought it out. If we went to hear a guest minister speak, we took Benjamin up to receive prayer. When we shared his amazing testimony of healing from seizures at the One Thing Conference at the International House of Prayer in Kansas City, the whole audience joined in praying for him. He was prayed for at our own prayer gatherings on a consistent basis. And, of course, we prayed over him in our home all the time. However, Benjamin couldn’t tell us if anything was happening to him, and so we just trusted that time would tell... (to be continued).