Not Again?

Today the speech therapist came to visit Elias. She observed and evaluated Elias with his BAHA on. We did not give her any preconceived information. We wanted her judgment pure and clear hoping that she would point towards something that indeed demonstrates that Elias is hearing. You could tell she did not want to say that. She kept asking are we sure this thing is working right and similar statements. I finally came out and said you don’t think he is hearing anything do you? She confessed her disappointment and disbelief. We began to dissect every component and debated several items leading up to the decision to use the BAHA. Her conclusions and opinions for potential explanations are as follows.

*Elias should be showing a response. It could be positive or negative, but none the less a response. He is demonstrating nothing.

*It is possible that there is an undiagnosed neuro-sensory issue. Part of this could also include a component that is not allowing the brain to perceive the stimulation correctly. In other words not interpreting it as sound and channeling it to the proper source or even blocking it all together.

*Potentially there is something that has been missed structurally with the make-up of Elias’ ears, particularly the middle ear and the cochlea. We may need more looks inside, especially the right ear.

* The ABR itself is not accurate.

First of all understand that this is just a very experienced & knowledgeable speech therapist offering up ideas to help explain. She is not attempting to diagnose or figure out the issue. That is for the ENTs and the Audiologist to do. In the case of the first to possibilities above, we have already contacted the FA clinic about and their neurologist. We are waiting for a response if they feel there could be a neurological component to this or if further testing is needed by them. Elias also has been displaying a tick of sorts with his eyes, blinking them in rhythm slowly accompanied by head movements. This began yesterday. We associated it as a sensory input mechanism which was also used to describe other similar activities he does when we met with the neurologist in December. We just thought we’d put it out there as another piece to the puzzle that may help. One thing the therapist commented towards in regards to the potential neuro components was that typically she sees these things in her less cognitively skilled children. Elias is not deficient cognitively, so that could be a plus in favor against this thought process. In regards to the final two possibilities she is basing this on what she knows to be true on how the body works and Elias’ actual presentation. She is very interested to hear what ENT has to say about this. Maybe the MRI scan of the ear ENT has wanted is the missing component that we really need to understand this whole thing. The MRI is something they have placed on the back burner for several reasons. The most important being the sedation is high-risk for his compromised airway and therefore an option not taken lightly. However, as we have added doctors to the mix there are MRI studies that would benefit them too, so if there are enough players to play the game and make it a solid and strong case then the option could be considered. Finally, the ABR itself potentially portraying a false reading was a source of irritation for us, as I’m sure you can all imagine given the results of the first one from Maryland. Her theory for that is actually based on Elias’ speech banana as it is called. It is a diagnostic chart to visually demonstrate to patients and family what their hearing is referencing decibels (dB) 0-120 and frequencies (Hz) 125-8000. According to Elias speech banana, it shows that he should be able to hear sounds without any aiding at about 70 dB @ a frequency of 1000-4000 Hz. Anything below that dB & Hz level Elias can not hear. An example of sounds you find below that are normal conversation speech, a watch ticking, etc. But, anything above that dB reading, not necessarily the frequency mind you, Elias should be able to hear. Examples of high dB sounds are @ 90 dB would be a food blender, 110 dB would be a chainsaw, etc. We did an experiment with Elias without the BAHA on.  While laying flat on the floor and playing with a toy we dropped a 5 section baby play gate to the floor to see what reaction Elias would give. We tried this 4-5 consecutive times at 10 ft and then 5 ft away from Elias. The noise made our ears pop a bit at the startling sound, but Elias did not even blink once. We estimated the noise to be about 85-90 dB clearly something Elias should have heard and responded in some fashion in her opinion. That itself disproves the plots on the page which replicate the ABR result ranges. Her suggestion was to have the ENT review the ABR again and possibly redo the test. She does think we should continue the next three weeks as planned with the BAHA trials since we can not get in to see ENT and audiology until then, but she is not optimistic that things will suddenly improve. So much that we will be rewriting and lowering Elias’ goals for the IFSP next week. Clearly a disappointing step for all of us. However, we made the goals based on Elias ability to begin to hear and I think her proposal to take this action is a dismal acceptance of the reality of what she observed today. She spent an extra half hour with Elias today and has been on the phone all afternoon trying to come up with other solutions and we have to admire her dedication for that. She is truly disappointed, but obviously not nearly as disappointed and hurt as we are at the moment.