The mystery that is Gracie's Hearing

Gracie's hearing continues to perplex us all.

Many of you have emailed me about her hearing after seeing her with an aid on again... so here is the scoop in a (believe it or not!) abbreviated version. (I started this post a couple of weeks ago... now that I have time sitting here at Gracie's hospital bedside, I have a little time to catch-up.)

She has been seen by four different audiological groups... all have been left stumped by her presentation. As an infant and toddler, her ABRs (Auditory Brainstem Response test) showed a severe to profound loss. Behaviorally (tests in the booth and her social/behavioral responses at home) showed a moderate to severe loss.

When she had her PE tubes in her ears, she had a constant drainage problem - - and because she is so open on the left side, we would even see the color of things she tasted come out her ear. She constantly was on eardrops for the drainage/infection issue and over time we started noticing a decrease in her hearing behavior. Then she got pretty sick with her GI issues and heart problems in summer of '06 and by spring of '07, it seemed she wasn't hearing anything at all. And she wouldn't even consider putting on her bone conduction aid.

In September, we took her to the California Ear Institue to try and get to the bottom of her hearing situation. They have a great CT machine that you can sit in the seat and in about 90 seconds it gets the shots they need. Unfortunately, it was too scary for Gracie and she wasn't still enough. After explaining her history and what we've been observing, the audiologists decided not to put her in the booth and it was recommended we have a new ABR, OAE, ASSR and CT done - - all under sedation. So that's what we did - - and at the end of it all, the audiologists came out and told us that there was absolutely no brain response at all... TOTAL DEAFNESS... given her recent hearing behavior, we weren't floored by these results, but were a little surprised that there wasn't at least some brain response. They also told us to give up on the hearing aids because clearly, there was no point. Gracie had a CT to determine the presence of the auditory nerves and the cochlea. Unfortunately, since she is "structurally complicated" and her nerves are very small, the CT was inconclusive and an MRI was recommended. Well... Gracie has a pacemaker and thus, cannot have an MRI. So, our only option in regard to giving a cochlear implant a try was to have the surgery and they would find out in the OR whether or not the nerves were present/viable. We assume they are present since we had previously witnessed hearing behaviors, but felt that at this point, we weren't entirely comfortable taking the surgical route with no guarantees of success and if she really did have some hearing potential, that could be destroyed with the operation. Plus, our guts were telling us that all that fluid was causing an additional conductive problem... that the loss might not be complete or permanent. The team at CEI noted the fluid in their reports, but insisted that the fluid wouldn't have resulted in a complete lack of brain response. (I should note that Gracie is neurologically complex and her brain structure is not normal... we reiterated this to them and wondered if it's possible that this fact would affect the results... but they were confident in the results.)

In November, we requested the ENT take out her left tube (the right had come out on its own in the spring). He did... and patched the eardrum. At the same time, thanks to the persistence and help of Gracie's DHOH (Deaf & Hard of Hearing) teacher, the district approved an evaluation with an educational audiologist. She came in December for a few visits... right around the same time that we were certain that Gracie was turning to some of Preston's shrieking (after having thought it for a while, now we really felt certain). Katie, the audiologist, brought a nifty machine (the name of which slips my mind) that has a piece that looks a little like a giant stethoscope head at one end and has a microphone attached to the other end of the box. The "stethoscope" piece was placed on different areas of Gracie's head and body and then various sounds were made - - voices, banging of a drum, etc. The "stethoscope" piece provides vibro-tactile stimulation in response to the sound. Gracie loved it... so we started trials with her bone-conduction aid again and had positive responses/behaviors from her.
Once we made what seemed to be real progress, the aid broke and we were without it for over two weeks. So... now we have essentially started over with her auditory training.

We are still in limbo on this issue... so complex is our girl!