History

I remember like it was yesterday. On September 22, 2008, we made a trip to Minneapolis Children's Hospital for what we thought would be a one time appointment to confirm that Aubrey did not have a hearing loss. After completing the testing, the audiologist looked up with concern and said "your daughter has a rare hearing disorder known as auditory neuropathy." We had never heard of such a thing. So many questions arose in my mind. How did this happen? Was it something I did? What will her future hold? Will we ever hear her say "mom", "dad", "I love you." We were heartbroken and in disbelief.

We never saw this coming. There were no signs. We were just here for a routine visit after Aubrey received ear tubes for reoccurring ear infections. We all thought she had failed the booth hearing test due to her ears being full of thick fluid. After the tubes, we did another booth test and although the results were better, something was still off. We were then referred to Children's for ABR testing. This test proved that Aubrey did have a hearing loss and auditory neuropathy/dyssychrony (AN/AD).

Aubrey was fitted with hearing aids. We were told the aids may not help, as the aids could just amplify the distortion she hears as a result of the AN/AD. We were then referred to several specialists. The first being neurology since AN/AD can be caused by a neurological issue. Then, we went to genetics. There are some genetic disorders that also cause hearing loss. Aubrey also had an eye exam, EKG, blood work, MRI, and a CT scan. We were overwhelmed. I took a leave from work. I threw myself into researching this maddening disorder and becoming Aubrey's advocate and teacher. Aubrey had already been receiving early intervention PT services for her gross motor delay, so we were able to get a Deaf and Hard of Hearing (DHH) teacher to work with Aubrey. The early intervention staff were also shocked at the diagnosis. They had done an extensive evaluation and did not find any signs of hearing loss.

We decided to get a second opinion at the U of M. Another ABR was performed that confirmed the diagnosis. In the meantime, all other tests checked out fine. The MRI and CT showed no neurological concerns or ear anatomy issues. Her eyes were great, her heart looked good, and nothing showed up in the genetic testing. We were relieved. Yet, we were still left wondering how this happened. As of today, we still do not have answers. I have my suspicions, and I think it happened after Aubrey turned one. Something changed right around 15-16 months of age. Aubrey had been on vancomycin for C-diff (antibiotic induced colitis). We later found out that vancomycin is an ototoxic drug, as it can cause hearing loss and AN/AD. The amount she received was so small, and Aubrey's medical staff does not believe this is the cause. It could have been a high fever or it could have just happened. As one ENT told us, it's just bad luck, like a car accident. If you would have left two seconds earlier from the home, the accident may not have happened. I wanted to say "are you calling my daughter a car wreck." Aubrey also does not have the traumatic and/or premature birth or jaundice history that many AN/AD children have. We will most likely never know. Instead, we focus on making sure Aubrey has the tools she needs.

We are so fortunate to live in a community where we have access to excellent medical and special needs services. Aubrey currently attends an auditory oral school for deaf and hard of hearing children called Northern Voices (http://northernvoices.org/). At Northern Voices, Aubrey receives extensive auditory oral therapy five days a week along with preschool instruction.  She also receives speech therapy and works with a DHH teacher through the school district weekly. Our goal is for Aubrey to be as verbal/oral as possible. This is easier said than done. You can spend a fair amount of time engaging in speech therapy, DHH services and wearing hearing aids full time. But, if a child is not receiving a clear, consistent signal to the brain it can be difficult to impossible to learn speech and language.

The fact that Aubrey falls in the "hard to treat" category adds more complexity to the situation. She has mild to moderately-severe hearing loss. We also believe she is in the mild to moderate range of the AN/AD spectrum. So, she's not mild nor profound and her receptive language is pretty amazing, considering. Therefore, the management of her AN/AD is not black and white. Does she do too well for an implant? She receives some benefit from her hearing aids, but is it enough? So, the last two years have been maddening trying to determine if Aubrey should receive an implant and sometimes it's all consuming. What helped give us some peace is that we wanted to do give Aubrey time and try everything else possible before turning to the implant.

Receiving an implant is life changing. Aubrey's brain will need to relearn to hear, any residual hearing she has will be lost during surgery, she will forever rely on technology, and, most frightening, the implant may not work for Aubrey. The list goes on. So, when people ask "why not just do the implant", you can understand the magnitude of this decision. We always said that if we started to notice a plateau in Aubrey's expressive and receptive language, then we would more seriously consider an implant.

Well, over the last 6-9 months we have noticed a plateau in Aubrey's expressive language. At first, I think she could keep up expressively, but as she gets older and the language and social interactions become more complex, it's getting difficult. Once I starting noticing the plateau in her expressive language, I started pushing for the implant and paying more attention to the research regarding implanted AN/AD children. Success rates of implanted children are hard to ignore. Research has shown huge gains in expressive and receptive language for AN/AD children who have been implanted. There are just a small percentage of AN/AD children who have received benefit from hearing aids. Cochlear implants have become the treatment of choice, if the goal is for the child to be auditory oral/verbal. Please refer to the AN/AD page for more details on management of AN/AD.

Receiving approval for Aubrey to receive an implant was not an easy process. It took time before I received approval from the audiologist, surgeon, and even my dear husband. Some desperate measures were taken. Aubrey gives me the strength to fight, and I see her desire to want to communicate and talk. She also loves to sing and dance. She is so full of expression. Right now, she can say 3-5 word sentences, but most people cannot understand the full sentence. I like to say that she has her own language - there is a lot of babble with actual words thrown in here and there. I am feeling confident that the implant will give her what she needs. I continually go back to a quote that I heard at a conference recently. The speaker was talking about his experience working with AN/AD children. He discussed how it was impossible to learn speech and language without a clear consistent signal to the brain. This really resonated with me and prompted my push for the implant. Just imagine if you were relying on a fuzzy signal each day to talk and communicate with people. Now, add hearing loss to this too. 

We take all of this for granted, especially the fact that we learn language by eavesdropping on conversation. It's all to easy for someone who has typical hearing and a clear signal to the brain. People who are hearing impaired will tell you how exhausted they are by the end of the day. They most likely missed conversation that happened that day or relied on their amazing lip reading skills during lunch in the noisy cafeteria. And, keeping up with amplification maintenance and technology is another story. So, I will continue to move through my day hearing the birds chirping, the sound of my car blinker, and water running without notice. Meanwhile, somewhere out there someone is getting their cochlear implant activated and hearing these sounds for the first time in their life. A new appreciation and utter delight for everyday sounds. Amazing.

On to the next chapter...