A Not-So-Brief History

Hi Everyone-

Mommy wants to tell you a story of my care history, so you can understand what our family has done to get ready for this exciting new phase! Here she goes (Beware..she can talk a...l...o...t!!)

There is a wonderful Doctor who lives in Tampa, FL named Dr. Chuck Berlin. When we first got the diagnosis of Auditory Neuropathy/ Dys-Synchrony, when Audrey was one month old--we immediately found that he is the utmost expert in this rare field of hearing loss. We were told that there is about 2 cases of AN/AD diagnosed in newborns each year in the state of Washington, approximately 100 cases each year in the entire US.

Dr. Berlin is an amazing resource for parents, in fact, he was holding a parents' conference a few weeks after we received our diagnosis, and we were so blessed to be able to fly across the US to spend a weekend with his team and other parents all hoping to learn how to best help our children.

We had multiple opportunities to see growing kids with AN/AD, talk with parents about their choices for care and to attend classes covering many topics including the newest research available on the diagnosis.

Upon our return to Seattle, we started Audrey's developmental testing at Children's Hospital. When you are diagnosed this young--it turns into a bit of a waiting game, while the kiddos grow and develop. We joined an Early Intervention program focused on helping us live our best life with Audrey's challenges, started learning sign language and focused our attention on therapy skills to use in our home to maximize Audrey's ability to gain language.

Auditory Neuropathy is confusing. To put it briefly, Audrey is a deaf kiddo that can hear. Her ears work properly, but there is a problem with the signal as it leaves her ear and travels to her brain. By the time the signal gets processed, it is distorted, late and garbled. Adults describe the sounds around them as buzzing bees, a radio between two stations or like a robot speaking very mechanically. So---to add to all the confusion of the rarity of this condition and lack of available information---sometimes she'll have a "hearing moment" and get our hopes up that she's getting better...

But over time, and after hours and hours of therapy work in our home and with our professionals, we realized that there was just no progress being made with her spoken language. We have tried hearing aids on her for over a year--and we didn't see any positive effects.

We travelled back to Tampa for a second parents' conference this spring and were able to gain so much more understanding after living with the diagnosis for a full year. We came back energized, organized and ready to start making the difficult decisions about how to best care for Audrey as she entered the important language learning age.

We asked Children's Hospital to review Audrey's case and consider us for a Cochlear Implant. We weren't ready for a surgery date, but wanted to have all our testing complete and paperwork ready, if and when we got to the point that her spoken language was delayed. Despite all our therapy work and all our work in educating ourselves on the latest information on AN/AD, Children's declined to consider Audrey for a Cochlear Implant. Unfortunately, they have only had about 15 cases of AN/AD in their center and are grossly under-informed about the benefit of implanting based on Language delay.

I was told that Seattle Children's only implant kids that fail their hearing tests at the most severe levels. Audrey registers hearing on the hearing tests, but we were seeing more and more that this hearing wasn't meaningful to her--because she wasn't gaining any spoken words.

We didn't take "no" as an answer the first time, so we came back to Children's to meet with the other director of the Implant program. The two doctors make team decisions on each case. Unfortunately, this appointment was a disaster. The doctor was confusing, drifted off task- to prove that she was more knowledgeable than us and gave Audrey a hearing test that actually brought tears to my eyes. The testing was very unreliable and I could see her registering "hearing" that was definitely NOT.

She listened to us give our points on Audrey, but in the end, she was very reluctant about the Cochlear Implant. I took this refusal as the catalyst to get to work on the language side to prove what I knew in my heart--that Audrey was not going to develop spoken language--even though she was ready to communicate with us. We found our amazing therapist, Miss Leticia, and immediately got busy testing Audrey on all the standardized tests we could, to build data for me to resubmit to Children's.

In the meantime, Audrey blossomed with her sign language. In less than 2 months, she gained 60 signs that she could sign to us! ...And not one word formed. At this point I was convinced it was a signal problem.

Dr. Berlin from Tampa helped me regroup and refocus my energy on finding a different center that would see the benefit in giving Audrey access to consistent sound through the CI. And that's just what we did!

If you are interested in learning more about Auditory Neuropathy, and seeing the amazing work that Dr. Berlin has compiled, visit:
www.auditoryneuropathy.com

Here is a simulation of what it sounds like to hear when your anatomy is affected with Auditory Neuropathy/Dys-Synchrony:

http://www.ucihs.uci.edu/hesp/Simulations/simulationsmain.htm