The Bopp Family Story: 

Microtia and Unilateral Hearing Loss: Choices We Made

By Lyn Bopp

I was one of those neurotic pregnant women obsessed with having the healthiest pregnancy and baby possible. I read all the books, followed all the advice and did everything in my power to ensure I had a “perfect” baby. So when Cameron was born it was a shock to see that his right ear was malformed. No one in the delivery room knew what it was or what to do and he was rushed away for ultrasounds, x-rays, blood tests, and CT scans. The doctor on call at the hospital warned us that he may be totally deaf and that he may have kidney problems or no kidneys at all since they form at the same time as the ears. He informed us that he may have a syndrome that could have devastating implications. He scared us to death. In fact, Cameron was born with a condition called microtia which literally means “little ear.” His right outer ear was malformed and he had no ear canal. His hearing loss was identified immediately since it was the result of a birth defect. Numerous tests, many months, and several ulcers (mine) later we realized that Cameron had normal hearing in his left ear and a profound conductive loss in his right. He did not have a syndrome or kidney problems or any of the other health issues suggested by the doctor of let’s-jump-to-conclusions. 

Cameron’s first year was wrought with worry about what the future would hold. When would he learn to speak? Would he learn to process language normally? Would his speech be affected by his hearing loss? Would he be teased? Should we pursue surgery to have him “fixed” or just let it go and teach him that he didn’t need to be “repaired”, that we loved him just as he was? Would he learn to read at a reasonable age? Should he wear a hearing aid? As time and milestones passed our fears were put to rest one by one. He did everything right on cue. He spoke early and OFTEN. He became a happy, social, well-adjusted toddler who kept us hopping. As he grew and changed we tried not to think too much about his ear deformity since we’d heard that no doctor would operate to reconstruct the ear until he was about six years old. In fact, we were so conflicted about our options that we weren’t even sure we would pursue surgery for him. So we used that time to bury our heads in the sand and focus on enjoying our son. 

When Cameron turned three, we had another little bundle of joy to dote on. Our second son, Chris, was born. He was a gregarious little package of energy who kept us smiling and running! One day when he was almost two, he presented us with a new challenge. Chris broke his leg while playing quietly with a friend. The x-rays revealed a large mass in his left tibia and off we rushed to Children’s Hospital. It took 10 days to diagnose his problem and determine that he did not have cancer. He had a condition called osteofibrous dysplasia and would need surgery to remove the tumor and bone graft the site. The condition is ongoing and the tumors will return. He will need the procedure repeated several times until he is skeletally mature. That got our minds off Cameron’s ear for a while!

When Cameron turned five, we flew to California to meet with Dr. Burt Brent, a specialist in rib graft ear reconstruction. Cameron was absolutely not interested in having surgery and the doctor refused to operate on unwilling patients, but we made the trip because we felt that we needed to make an informed decision. By the time he was six Cameron had done a complete about-face and asked for the operation. He had become more self-aware and somewhat self-conscious about his appearance. He wanted to look like other kids and make a big ear out of his “little ear.” I lost a lot of sleep worrying that the series of four surgeries would be painful and traumatic. Imagine my relief when he woke up from surgery each time without pain! He was a fabulous patient and didn’t complain of pain after any of his surgeries. All he wanted to do was go to the beach. Our trips to CA turned out to be as much about vacation as stressful life events! Cameron looked forward to going to California and couldn’t wait to get back to the ocean each time. We were so proud of how he dealt with this challenging time in his life! Fortunately, first-graders tend to be sweet and concerned about their friends when they show up at school with their heads shaved and bandaged. He had plenty of support from family, teachers and friends and was perplexed as to why everyone was making such a big deal of his surgeries. It’s just a testament to the resiliency of kids and the undue stress that adults put upon themselves. Kids can really teach us lessons in coping with whatever life throws at us.

Cameron is 12 now. Holy cow, where did the time go?!? He has matured into a fine young adolescent boy (as it turns out, the words ‘fine’ and ‘adolescent’ are not necessarily mutually exclusive)! He has performed very well in school every year, much to our relief, and has built strong social relationships with his peers. He enjoys a variety of extra-curricular activities including sports and music and has a positive outlook on life. What more could we ask for?

Now to address some of the decisions we’ve made and why we made them. 

1. Hearing Aids.  We chose not to aid Cameron simply because the audiologists we spoke with when he was very young all told us that since he had “one good ear” he would develop speech normally and compensate for the unilateral hearing loss.  In 1996, the consensus among professionals (at least the ones we met) was that there was no need to aid UHL kids.  The pendulum has swung recently, however, and now most of the families I meet who have children with unilateral hearing loss and/or microtia have chosen to aid their children.  Most professionals in the field of hearing loss are now recommending aiding these children on the basis of new data that suggests children with UHL are at significantly greater risk of failing a grade in school than those with normal bilateral hearing.  At this point it is unlikely that Cameron will ever be aided, but it will always be an option for him and hearing aid technology just keeps improving!

2. Surgery. There are multiple options for children with microtia. They can opt not to have surgery, choose rib graft reconstruction, Medpor implants, or prosthetics. This is entirely a personal family decision. Children with microtia may be candidates for a procedure called canalplasty, which opens the ear canal and can offer the possibility of restoring hearing to the affected ear(s). Why didn’t we go for it? Seems like a no-brainer, right? Well, the procedure is actually considered neurosurgery and is a very sensitive operation with high risks. The child could lose hearing in both ears due to hair cell damage from drilling out the ear canal. Also, the facial nerve could be severed causing paralysis of half the face. Yikes!! Another risk is the ear canal closing up after surgery. This is not uncommon after canalplasty. After Cameron’s fourth reconstructive surgery for the microtia, he was done. We encouraged him to meet with the doctor about canalplasty, but he would not even consider it and we didn’t blame him. This is a procedure that he can have done at any time if he changes his mind. We are confident that he will be a successful, productive individual with or without an ear canal!

3. IEPs and 504 plans. Although Cameron would at least qualify for a 504 plan, we have never pursued one. He has been fortunate to have teachers who are compassionate and responsive to his needs without the paperwork. He maintains an excellent GPA, so the IEP is unnecessary for him. We are working hard to teach him to self-advocate so that he can speak for himself when he needs a seating accommodation or repeated instructions. It’s all about self-confidence and that can be challenging when you’re in middle school. It’s good to know the law is there to back us up if he ever runs into trouble.

Going forward, we expect more of the same! It is sad and scary to think that 2/3 of our time to raise Cameron to adulthood is gone, but it is reassuring to know that we’ve made it this far and Cameron has a wonderful life ahead of him regardless of his hearing loss. We all have our challenges, strengths, and weaknesses to deal with and his will not hold him back!