Do You Hear What I Hear?

Great news....Olivia can hear!

Sensorineural deafness (or nerve deafness) is one of the many negative side effects of Sanfilippo syndrome. Olivia has had her hearing tested on an annual basis since she was two (even before she was diagnosed with Sanfilippo syndrome). In the last few years we've had to transition to having sedated hearing tests because Olivia just isn't able to actively participate in the traditional audiology exam. Olivia had her first sedated hearing test in September 2010 when she was having her port removed. Unfortunately the test showed that Olivia had mild to moderate hearing loss in her left ear and severe hearing loss in her right ear. The audiologist recommended hearing aides and took impression molds so that Olivia could be fitted for the hearing aides. We were advised to follow up with a local audiologist so that we wouldn't have to travel far if a hearing aide was damaged or needed a new battery, etc. We took a copy of the report and the molds and were on our way. I spent weeks trying to find an audiologist in the area who would work with the information we were given at Duke. Each audiologist I spoke with said they weren't comfortable working with that info and suggested we contact someone else. Add that to the hurdle of finding someone who worked with our insurance company and it was becoming an incredible frustrating situation. I went back to Olivia's primary pediatrician and explained the situation. He suggested that we try UNC and put in the appropriate paperwork for us to be seen there. I faxed over the report from the audiologist only to be told that the audiologists at UNC were not comfortable working from the Duke data. They suggested we start all over by seeing a UNC ENT and then moving forward (because of course you can't do anything with audiology without first going through an ENT). UNC is an in-network provider with our insurance company while Duke is out-of-network. With our particular insurance we aren't given the option of just paying the difference between the in-network and out-of-network providers, it's a all or nothing situation, they either pay all of it or none of it so we are always advised to try the in-network option first. So off to UNC we went, of course the pediatric ENT at UNC had a waiting list almost 2 months long. I went back Olivia's pediatrician and explained our situation, he then suggested that we go back to Duke and put in the appropriate paperwork requesting that Olivia be seen at Duke. I called Duke audiology hoping to FINALLY schedule the appointment to get Olivia fitted for her hearing aides. By this time I'd spent almost 6 months trying to get this resolved. The Duke audiologist said that Olivia had outgrown the old molds and the report was out of date so they recommended another visit to ENT and another sedated hearing test.

So frustrating! Sanfilippo syndrome is accompanied by so many health risks that complicate anesthesia and sedation. I hate to have Olivia sedated and we try and combine procedures as much as possible.

Olivia was being sedated for her MRI last week at Duke so we tried to coordinate with audiology so that they could do the repeat sedated hearing test at the same time. Unfortunately there was a back up with MRI and scheduling glitches so they were not able to do the test.

We headed back to Duke today for a sedated hearing test. I fully expected that they would take another set of impression molds and I'd even gone so far as to schedule an appointment with audiology in 2 weeks so that Olivia could be fitted for her hearing aides. I canceled that appointment today!

The audiologist walked out to find me in the surgery waiting area and we headed into the consult room. I was expecting the worst...increased nerve damage, not repairable with hearing aides, etc....

The audiologist told me that Olivia's hearing tested NORMAL in both ears, her right ear is significantly improved. Both ears are well within the normal limits! We went on to discuss that sometimes patients with Sanfilippo syndrome experience improvement in their hearing post transplant. As soon as she said it I remembered going over that when we were deciding about whether or not to move forward with transplant. But to be honest with all the life/death stuff we've been dealing with hearing was way down on the list.

On the heals of the news we received last week about Olivia's MRI and improved brain activity today's news is icing on the cake! Such an unanticipated blessing!