We had our follow-up visit with Elias ENT that is focusing on his ears and hearing. In his surgical update we mentioned that the repeated ABR that was performed a few weeks back returned the same results of a conductive hearing loss. This of course means that the BAHA hearing aid should in fact be working. We know that it is not. So now what? After our appointment we walked away with a deeper understanding of the potential how and why, but not with a final answer.
It will not surprise any of us to learn that the probable issue Elias is having is a rare phenomenon. Elias certainly has excelled at being unique and making all his doctors work very very hard to find answers. His hearing is no exception. For the moment the doctor is putting most of his eggs in this one basket, not all, but most. Those were his words. His best guess for now is that Elias has some sort of central component to his hearing loss. In other words the area of the brain (bottom back lobes) that process central system information may not be mature or awakened. This has nothing to do with cognitive ability or intelligence, which is what some audiologist have kept eluding towards. A prevalent issue most people can relate to with central nervous system issues is apnea or sleep apnea. This may also unlock the mystery behind Elias infamous episodes of apnea particularly awake. Not that these two things are related in any way or the cause for one another, but when you put the puzzle pieces together you begin to correlate the picture in the picture a little clearer. Focusing back on the hearing there is unfortunately no way to image or study this specifically to determine if this is indeed the root of the issue. This is why most of the eggs are in one basket as this is the most logical at the moment. To put it in better perspective, Elias may be hearing but this portion of the brain is not able to define or even acknowledge that sound is what is occurring. A great example that Katharine came up with is the air conditioning in your house. We don’t usually hear that noise when it is running, unless you focus on it specifically. It becomes ambient background or white noise if you will. We tend to tune out and not hear the typical daily background, but can tune it in and bring it to focus when we want. It is possible that Elias central system is simply processing everything as white noise thus not allowing him to hear.
So how do we fix this or improve it? This is the disappointing part. Elias will need to undergo intensive Aural Rehab Therapy. Where Speech Therapy focuses on the output, Aural Rehab focuses on the input. We will need to work with a specialist that can attempt to “train” or awaken the central lobes to process hearing. During this time we will still need to continue usage of the BAHA. Elias cochlea are responding just not interpreting, but this is like a muscle. If they do not continue to receive stimulation then atrophy could set in rendering it impossible for him to hear. Here is the disappointing part….It can take 4 to 5 years before this could be effective and Elias begins hearing and there is not a 100% guarantee this Aural Rehab will work. So what is next? We begin the Aural Rehab Therapy July 14th. All of his sessions will be video taped so that all members of Elias hearing team may have an opportunity to observe the progress and also be able to offer input based on their own evaluations. We will be moving forward with the purchase of Elias own BAHA, as we are still using the loaner. We are moving on a parallel path towards total communication with assistive technology and sign language consisting of multiple speech therapist focusing on specific items while everyone and all aspects work toward a common goal. It is a very long and complex process we are about to embark on yet again.
It is very disappointing to hear all of this, but overall our feeling at the moment is a little subdued. While we were really hoping for some definitive answers we feel as though we have a very strong and logical explanation for what is going on. For that at least we have a little sigh of relief as we have battled this for over 2 years now. The reality that it may take Elias 4 to 5 years before he will even begin to hear is no exactly the best news, but at least there is hope. By taking the total communication path along with this Aural Rehab Therapy Elias should stay on developmental course which if he eventually does hear will actually help him catch on more quickly. Elias hands and arms do present some challenges with sign language and depending on who you talk to we over the years we have a mixed bag of opinions in regards to this. We are sadden by this result, but remain hopeful as we always have tried to be. It isn’t always the easiest to be that way when you feel like you are constantly battling something, but Elias gets us through the rough times. If anyone is going to overcome this it will be Elias! He has overcome so much already and it is obvious he is destined to continue down this difficult path. We appreciate everyone’s continued support, thoughts & prayers as we continue to tackle each issue day by day, one by one and watch Elias continue to win out over them all.