I have so much going on in the next month I’m a little overwhelmed. I’m trying not to think about it. The most important thing is my daughter turns 18 in a week. ONE WEEK SHE’S 18!!! How did this happen? What a long road we had. My daughter was born a little over a year after I was diagnosed with multiple sclerosis. The neurologist took me off birth control, and the pullout method didn’t work. The best accident I ever had. My daughter was born right before the hearing test for all newborns was given. Unfortunately, my daughter had a hearing problem but we wouldn’t know for over a year and a half later.
It was apparent something was wrong but we didn’t know what, she wasn’t talking. She was given a hearing test and she passed. Everyone was at a loss. One day I went to pick her up from daycare, I opened the classroom door, every child’s head turned but hers. The reason she passed the hearing test was because for every sound there was a visual bear that moved with the sound. She was living in a visual world, without full sound, so every time one of those bears move she’d turn her head, also the direction of the sound. That gave the false result of hearing. After the daycare incident, a re-test of her hearing showed a severe hearing loss and then everything went very fast. We ended up with a team of an audiologist, ent and geneticist to find out what was going on.
She had blood tests to find out if it was genetic, hearing aids, cat scans, MRI’s, EEG, sleep studies all by the age of 2. It was finally figured out she has Enlarged Vestibular Aqueduct Syndrome (EVA or EVAS).
EVA can be a sign of a genetic disorder called Pendred syndrome, a cause of childhood hearing loss. According to a study by the National Institute on Deafness and Other Communication Disorders (NIDCD), approximately one-fourth of the people with EVA and hearing loss have Pendred syndrome. Hearing loss associated with Pendred syndrome is usually progressive, which means that a child will lose hearing over time. Some children may become totally deaf.
It’s all explained on this site. http://vestibular.org/enlarged-vestibular-aqueduct-syndrome-evas And https://www.nidcd.nih.gov/health/enlarged-vestibular-aqueducts-and-childhood-hearing-loss. At the time Pendred Syndrome wasn’t diagnosed but my guess is they went together.
After a plane trip to Florida, her hearing went from severe to profound in all of the high frequency sounds. If she was going to talk, hearing aids weren’t going to help. I decided to make the appointment to see if she was eligible for cochlear implants.
A cochlear implant is an electronic medical device that replaces the function of the damaged inner ear. Unlike hearing aids, which make sounds louder, cochlear implants do the work of damaged parts of the inner ear (cochlea) to provide sound signals to the brain.
They were just being done in NYC and were far from a standard choice for a child with hearing loss. I wasn’t even sure she’d qualify, she had to met FDA requirements and guidelines. The best phone call I ever received was that she did.
Her first cochlear implant was at the age of 3 the second at 6.
Tomorrow I will continue with my amazing daughter’s story.