He was beautiful and fat and full of energy. At nine pounds, 15 ounces, my son was a commanding presence on the maternity ward. In addition to his size and energy, he also commanded attention because of his little oddity.
My son was born with one normal ear and one nub where his other ear should be. From some angles it looked like a tiny ear, from others angles it was clearly not an ear. It had no opening and was about 1/3 of the size of his other ear. I was certain there was a problem from the moment I saw it in the recovery room but the nurses tried to convince me it might just need time to “unfold.” I appreciated their delicacy, but it was wasted on me — when he repeatedly failed the newborn hearing screening, my fears were confirmed.
No one used the term birth defect until he was five months old. I guess the term “defect” was too harsh. Though I spent the first six months of his life taking him to a variety of specialists, compiling information and fielding calls from numerous early intervention groups, it never occurred to me to use the term birth defect either.
Aside from his ear – a more visible defect – he also had a misshapen kidney that didn’t drain. An ultrasound had picked up the kidney issue during my pregnancy, so we knew it was coming and that it was repairable. The kidney was easier for me to accept — no one can tell if your baby has a bad kidney, but a missing ear is hard to ignore.
For months, we were bombarded with an overwhelming amount of advice from parent groups, interventionists, audiologists, ENT doctors, and developmental and occupational therapists.
We head everything:
“You should learn ASL (American Sign Language) to be safe.”
“You should get him fitted for a BAHA [bone anchored hearing aid] before six months. You don’t want speech delays.”
“You should do genetic testing. He might have a syndrome.”
“Protect his good ear. Ear infections can cause deafness.”
“Don’t blame yourself. Birth defects happen.”
But I did blame myself. It was my body that failed to form his. Something I had done must have caused this, I thought. What started as a tiny ear nub snowballed into a hard-of-hearing baby who began missing milestones and earning a new diagnosis with every doctor visit. My older son had never had more than a cold, but by the time our little guy was six months old, he’d already had more hospital visits and tests then his father, brother, and myself combined.
Why didn’t he roll over?
Why is he so quiet?
Why isn’t he babbling?
Does he sound funny when he laughs?
He didn’t look when I called his name; does that mean his good ear is going too?
With every milestone he missed, my anxiety rose. I was so worried about what might happen that I was missing what was happening. He was a happy, chubby baby who loved to grin, giggle, and play with his older brother. So I made the decision to put his early interventionists on hold, wait on his hearing aid, and just give it time to see what would happen.
By 18 months, we got a tube in his good ear and his balance finally developed. Six months after that, he had reconstructive surgery on his kidney. When he had no language by age two, we started to learn ASL and got him a hearing aid. Last month, we enrolled him in a speech intensive deaf school for fall.
Ultimately, he was diagnosed with a genetic syndrome found in only one of 50,000 cases. In his case, his obvious birth defect was more than cosmetic. It was an outward clue to something much bigger – and thanks to that tiny ear, we know about it.
Doctors can build him a new ear after he turns six, but he still won’t be able to hear. It would be cosmetic, a correction to a defect. We’re leaving that decision up to him. The ear is more noticeable as he gets bigger; he looks lopsided. I notice it, but I seem to be the only one, for now.
Fortunately, most people can’t see past his smile.