LVAS & Progressive Hearing Loss
Kelin was born a healthy, full term baby girl in March of ’97. The last hour leading up to her birth was less than ideal. Kelin was in distress, doctors from the NICU were on hand. Her umbilical cord had been wrapped around her neck three times! I had been medicated and as a nurse, knew enough to be scared. I vaguely remember grabbing my husband Tom’s arm and pleading “Make sure they’re doing everything right!” That was quite an order for my stressed husband who worked with computers. Kelin was born the color of a plum, but with a little help, was pink and alert. Newborn hearing screening began that year, but not for Kelin who missed it by just a few months. She was perfect in every way, and still is, but I’ll get to that later.
The “Clap Hands” Hearing Test
Kelin was our first baby. I loved reading the “What to Expect” baby books and at nine months, her development was right on target. She was incredibly loud, yelling and yodeling all day long. However, it was around this time that I started to worry about her hearing. Kelin loved to see her dad come home from work, although would never turn toward the sound of the door opening or his voice from across the room. I mentioned this to her pediatrician at her nine month check-up. After the doctor clapped her hands loudly (to which Kelin reacted) and asked about her language, she stated that Kelin was just fine. I might have been relieved by this, but I still worried. Kelin would awaken from naps and sit in her crib, awaiting my arrival. When she saw me, she would be so happy, but she would never hear me quietly walk in the door…hmmm.
Again, this time at Kelin’s one year check up, I shared my concerns with Kel’s pediatrician. She continued to doubt the possibility of hearing loss. Kelin had lots of words, not well articulated, but lots. The doctor gave me a referral for a hearing test at Children’s Hospital. A pivotal moment occurred just prior to that evaluation. I was giving Kelin her evening bath and playing the usual games: “Where’s your nose?” “Where’s your mouth?” She would watch me intently during this game. For some reason, I decided to ask her questions, BUT without sound. She got each and every question right. She was lip reading. My heart sank.
Somehow, this didn’t prepare us for the words of the Children’s Hospital audiologist. “Your daughter has a moderate to severe hearing loss.” Wow. I knew next to nothing about hearing loss. I remember the long ride home that day. I remember feeling numb. Was the test accurate? Was something I did during pregnancy to blame or was it the traumatic delivery? Could something be done to restore her hearing? Would she make friends? What schools would she go to? I was so completely overwhelmed.
Chasing the Diagnosis
As a mom and as a pediatric nurse so I was determined to get an accurate diagnosis…this proved to be quite a challenge. We saw three doctors and each diagnosis was different and accompanied by different recommendations. One doctor told us the bones of the middle ear weren’t formed correctly. He offered to do surgery to restore her hearing. We got a second opinion. This doctor stated he saw no such thing and that surgery was not an option. Her hearing loss was probably genetic and would not improve. What were we to do with two such differing diagnoses? Finally, on the urging of a friend, we went to see our present physician, Dr. Kelsall, who saw something on the CT scan that the other doctors missed. Enlarged vestibular aqueducts, bilaterally, were the cause of Kelin’s hearing loss. He also informed us that her hearing loss would likely progress. Further hearing loss could occur from an injury to Kelin’s head. We finally had an answer, but these were difficult words to hear…another long ride home.
While we were seeking medical explanations for the cause, I began the process of investigating communication options and preschools for kids with hearing loss. We received some loaner hearing aids from her audiologist and Kelin immediately responded so well to them. From day one, Kelin loved her hearing aids. She never took them off unless they were itchy, although where she put them when this happened was unpredictable. I can remember ransacking the entire house for one lost aid. Don’t ask me why I suspected that half empty can of Sprite, but when I shook it, there it was! We felt confident that auditory verbal therapy was a good fit for Kelin because she was always making noise, but in pursuing that, we would need to be open to the future possibility of a cochlear implant. Kelin began auditory-verbal therapy and two months before her second birthday, she was enrolled in an auditory verbal preschool. It went really well.
The school was expensive, added to the fact that I stopped working to stay home and take care of Kelin, now almost two, and her baby sister, Molly. At the time, there was no state funding for auditory verbal therapy. Our insurance denied therapy as well as her hearing aids. We went through a very lengthy struggle with our insurance company and eventually got partial coverage of her hearing aids and a small amount towards therapy. I remember what a difficult time that was. Tom had to take a job that required traveling to pay for all of this. I felt that it was so unfair. We had insurance for our young, healthy family, although we received little to no coverage for our daughter’s needs! The important thing was that our children were healthy and happy, and Kelin was doing so well.
Adventures in Advocacy
Another big struggle was on the horizon: obtaining services at our local school district. When Kelin was three, we decided to pull her from the private preschool which was far from our home, and mainstream her in our local public preschool. Because Kelin’s language was age appropriate, she was denied services altogether. What ensued was a long and stressful series of meetings, eighteen hours of IEP meetings to be exact. We did obtain services but continued to negotiate as to what was and what wasn’t appropriate for our daughter. We were lucky to have another family in our district seeking the same services for their child, and we were a great support to each other, attending each other’s IEP meetings. What resulted was a great preschool environment for both of our kids.
Despite our hopes for the contrary, Kelin’s hearing loss did progress and yes, it was because she injured her head. On one occasion, she fell backwards onto a wooden floor. The second injury happened when a friend fell off a chair and their heads collided. That was the worst. Kelin was only three and I couldn’t convince her it wasn’t the hearing aid malfunctioning. My heart broke for her. After each of these drops in hearing, Kelin required ear surgery to repair a perilymphatic fistula, a condition that kids with Kelin’s ear formation are prone to develop. Despite this, we have always encouraged Kelin to be adventurous and active, yet we’re diligent about using a helmet for certain activities.
Kelin coped with changes in hearing by using good digital hearing aids and also an FM. We were so lucky to have found a great audiologist in Allison Biever, because we basically lived at her office during those early years. I was often wrong when I suspected further losses in hearing, based on my own crude measurements, i.e., making noises while hiding behind a chair. Poor Kelin and Molly, sometimes I think they’ll have a lot to tell a therapist! Yes, I also subjected Molly, now 10, to my less than scientific methods of hearing assessment, not to mention the fact that I regularly tried to fit her with Kelin’s hearing aids….they looked so much alike from behind!
Preschool became very difficult for Kelin as she lost hearing. She began to avoid group play at preschool and I could see that she was exhausted by the work of listening. We decided to investigate the possibility of a cochlear implant. Just before Kelin turned four, she had surgery and received a cochlear implant in her right ear. We chose this ear because Kelin still had quite a bit of hearing in her left ear. We had always planned on getting a cochlear implant for Kelin so it was not a difficult decision for us. The surgery was, however difficult for everyone. She was very nauseated as well as dizzy afterwards. Because of the large vestibular aqueducts, her dizziness persisted and was resolved only several months after surgery. I was anguished over this. Although her otologist reassured us that the dizziness was temporary, I felt guilty and questioned our decision for our daughter during this period. That is a really difficult thing.
There was an initial period of adjustment to the implant. Kelin had to orient to the new sounds of familiar things, such as her name, for about two weeks. After that, everything clicked. Within a month, life was definitely easier for her. Soon we noticed that she became more social in preschool and had more energy. She wasn’t working so hard to hear anymore. A funny side benefit of this was that when Kelin started to hear and pronounce sounds, like /s/ and /f/, Molly’s speech improved because she mimicked all of Kelin’s words.
Much has happened between then and now. Kelin is currently a happy and healthy 11 year old. She still has one implant (pink and green) and wears a hearing aid in her other ear (covered with bright stickers) and always insists on braids or a ponytail. She is a great listener but also relies heavily on lip-reading depending on the environment. She is mainstreamed in a public charter school and has lots of friends. She plays soccer, sings in the school choir, reads obsessively, and is really into Hanna Montana and the Jonas Brothers. Both Kelin and Molly began taking an ASL class with their friends at school. Kids are like sponges with this language. It’s been great.
I do know that Kelin has to work harder than hearing kids and certain social situations are trying. While difficult to see as a parent, I am always impressed by her tenacity and confidence. Despite the struggles, Kelin’s hearing loss has been a gift in so many ways. I hope that her challenges will give her great perspective and strength for whatever her future holds.