Any audiologist knows that having a hearing impaired child can happen to any family. Current statistics indicate that 3 in every 1000 children born will have hearing impairment, 90% of which are born to normal hearing parents and only 50% of which can be attributed to a genetic cause. This leaves 50% of the cases of hearing impairment in children of unknown cause.
It became clear after the loss of our second child that we had a better chance than average of having a child with severe hearing impairment. My husband – who has worn a hearing aid since childhood – was diagnosed with a genetic condition called branchiootorenal (BOR) spectrum disorder. In this very rare condition, kidneys, ear, and facial structures can develop abnormally. It is an autosomal dominant condition, meaning that only one abnormal copy of the gene needs be passed along by either parent. With every pregnancy, there is a 50/50 chance of passing along the gene, and it turned out that our older child also had the condition.
My third pregnancy went well, and no major kidney issues were found in the baby. Some 3D imaging three weeks before our due date indicated everything looked fine. Yet within seconds of delivery my husband informed me that our child had the syndrome. His ears had barely formed and there were no ear canals visible (a condition called bilateral microtia). As you can imagine I was devastated. I was not prepared for this. How could I have a hearing impaired child?
In the midst of my grief I was somehow able to tap into my audiologist side and do what needed to be done. I contacted an Ear, Nose and Throat physician I worked with and requested a CT scan to find out if middle or inner ear structures were missing. Luckily, we were able to have one completed while still in the hospital and we learned within the day that the middle and inner ear were intact. This meant any hearing loss would mostly conductive, so once released from the hospital I contacted our local Early Intervention audiologist to schedule my son for an Auditory Brainstem Response test. The test revealed that bone conduction was within normal limits, so later that same day a bone conduction hearing aid was ordered. My son was fit with his first hearing aid at 3 weeks of age, and he started speech-language therapy at 10 months. As an audiologist, I knew what needed to be done and did it; as a mom, however, I continued to struggle with the “how could this happen?” aspect. Thankfully I had a very supportive husband and extended family. It took some time to reach a place of acceptance, but I did get there eventually.
Fast forward four years. My son is now on his second hearing aid and has been in speech therapy for almost four years. His speech is understandable to most listeners, and his grandparents constantly tell me how much his speech has improved when they talk on the phone or via Skype. He LOVES his hearing aid and will not go without it unless he is playing in water. He even sleeps in it!
We are now in the process of getting him a bone anchored hearing aid. Due to age requirements, he must be five years old to have this surgery. I will keep you posted!