When Ben was born, it took us about 4 days (I think) to get diagnosed with Goldenhar spectrum or syndrome (depending who you are talking to). At the time, I didn’t really know what that meant, I only knew that it affected his eye, his ears, and his spine. We were transferred to our local hospital for this diagnoses and each specialty came in and did a consult. I would eventually gather all these consults and study them beginning my journey into understanding the medical world. I didn’t study them to argue with my doctors, but more to learn the language and be prepared with what to ask. It’s an intimidating world to maneuver and learn. I used to study before each specialist appointment to try to understand what they were looking for – I studied audiograms, eye prescriptions, xrays, ct’s, bloodwork, and doctor’s notes – all searching for proof that everything was going to be ok.
My first “crash” was when Ben was about 4 months old. We were to have an ABR (auditory brainstem response) test to help us determine Ben’s hearing loss. This is a test where they put the patient to sleep through anesthetics and run sounds in his ears while monitoring brainwaves to determine what he is actually hearing. It is generally quite accurate. I had done my homework and I was ready! There are two types of hearing loss you can have: conductive or sensorineural. Conductive is more like sticking your fingers in your ears. The quality of the sound is the same, but it can be really muffled/muted. Generally hearing aids work well with this type of loss. Sensorineural loss is when some of those millions of hairs in your inner ear (cochlea) are damaged or missing. We have no way of determining sound quality, and clarity of sound can sometimes be difficult even when aided. Anyways, I was ready for this test. I studied Ben’s diagnoses – I looked at his previous hearing charts – I read specialist notes, I read CT’s and I knew knew knew that his results would come back as a conductive loss. I was prepared and able to deal with that. Because we had to be sedated for the test, I was waiting in day surgery with him while he recovered. The audiologist came in and presented me with the news…it was a sensorineural loss on both sides. Cue nervous breakdown #1. In hindsight I’m not why that should be the news that pushed me over the edge, but it was; it did. I also believe that the audiologist might not have been prepared for my emotional response, but I feel like they handled it pretty well. My sobs shattered the recovery room and I kept saying over and over “why can he just catch a break”. They managed to calm me down, saying all the right things. Afterwards I sent a three page letter to the audiologist apologizing for my tears and also including my documented research as to why I still thought it was conductive (sometimes I just can’t stop myself). Even today I wonder why nobody’s filed a restraining order yet!
I remember after Jesse and I received the initial diagnoses we thought – well that is that. At least now we know what to expect and I was able to slot our little problem areas into nice tidy compartments…ENT….opthamology….neuro….spine….etc. We were confident that we knew what to expect and we would be able to find ways to deal with each issue. Little did we know at that time that Ben would come very close to dying in less than 6 months.
Oh…I should mention here that five and a half years after Ben’s first sedated ABR, and a million tests later his audiologists have come to the conclusion that Ben has conductive hearing loss in both ears, so we are quite pleased with that (I say tentatively in case it should change). On that note – I have a favour to ask, which I am happy to pay for. Ben will be receiving new hearing aids called BAHA (bone anchored hearing aids). Normally these are anchored onto the head through a screw, but we want to wait a bit for that to happen (we’ve totally surpassed our head screw allotment for this year…maybe we’ll try again in 2018). So, the hearing aids that we are getting are attached to a headband. We have done a trial of these and Ben really does not like the headband at all as he finds it quite uncomfortable. I thought that we could find a way to incorporate the headband into a boys baseball cap and cut out holes in near the ears to allow the BAHAs to access the bone. I know that I impressed the heck out of everyone with my beanbag surgery, but I feel like this type of seamstressory (yes, it’s a word) is beyond my capabilities. If anyone knows of anyone who I can contact to help me with this project, I would be super happy.
Ben seems to be doing well. He is strong and continues to move forward. Tomorrow we will be doing X-rays again.