Complicated Hearing

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Children with Goldenhar syndrome have very complicated hearing issues – they are (usually) not simple by any means. Ben and I recently had a pre-surgical consult with his surgeon to discuss BAHA (bone anchored hearing aids) implant surgery – a minor surgery that implants a piece of metal abutment in his skull on which his BAHA will rest. Yup….more screws, but super fancy screws – only the best for our kiddo! So, here’s the low down. FYI – a BAHA is a hearing aid that (for now) is attached by an elastic headband. The hearing aid rests on Ben’s bone behind the ear and vibrates the sounds through his bone to his cochlea (inner ear). This allows him to bypass his middle ear, which, frankly, is a bit of a mess. Ben has been wearing his BAHA’s most of the time since 2017. I occasionally make him take off his BAHAs and put on his ‘in the ear’ hearing aids to compare the two – it is still unclear to us how Ben hears. Occasionally he wears all four hearing aids – I’m not sure why, but who am I to judge – Jesse said he once wore two pairs of underwear , but he had a good reason (I didn’t ask). (Another FYI – A BAHA is different from a cochlear implant – a BAHA bypasses the middle ear and transfers the sound directly to the cochlea whereas- a cochlear implant bypasses all the crappy hearing parts and delivers the sound directly to the auditory nerve – it is a far more invasive procedure.)

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Although bypassing the middle ear seems pretty logical when your middle ears are screwed up, things are never as simple as they seem. Ben’s can’t easily hear some frequencies, so he is still missing sounds – especially when they are quiet. Currently the most difficult sound for Ben to hear is the ‘s’ sound. The letter ‘s’ is a quiet sound, and it is also what we call a high frequency sound (not because we say it a lot, which we totally do), but high on the sound wave frequency scale. I can’t imagine how amazing this kid is that he has learned to decipher language and communicate so effectively without having easy access to this sound. There is a possibility that Ben might be able to hear the ‘s’ sound easier with his implantation surgery, but it is still unclear.

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The biggest hearing concern we have now is the different hearing abilities between the two ears. Ben has one ear that can hear significantly better than the other ear. (Interesting fact – the ear he hears best with is his little ear – the one you would think would be worse because it is smaller and misshapen…but it’s NOT – weird hey! Ben also calls it his favourite ear – it’s my favourite ear too). Ben’s left ear has difficulty distinguishing a lot of noise unless it is super amplified. To put this into simpler terms, he could be sitting beside a lawnmower and not necessarily hear it if he had to depend on Lazy Lefty. At one point we were told that his left ear had profound loss (deaf). I don’t think it is that bad, but it does suck quite a bit. Ben’s right ear is also screwed up but in a way more positive way. It appears the right ear just has middle ear problems, so the BAHA allows us to bypass all those shenanigans and go straight to the cochlea – we have way better hearing opportunities this way. Because righty is stronger, smarter, and tougher than lefty – all the sound that goes to Benjamin’s head is sorted and processed by the right cochlea (bypassing the lazy, left cochlea). This in itself causes a problem because Lazy Lefty just gets lazier. I’m not sure why this is a problem, but here is my best guess. I think they (the audiologists) think Ben’s left ear is pretty screwed (they use way more technical terms), and if it gets more screwed up over time – we may have to have a cochlear implant in his left ear one day, which I think is pretty unheard of in today’s hearing world. However – ten years ago having two BAHA’s was pretty unheard of, so I’m gonna roll with this being a potential future possibility. I think a big part of the worry for Ben is that if we let the left cochlea get too fat and lazy, we might find that we need it one day and it will no longer be functioning like it should. I feel like that is the gist of the last hundred audiology consultations I’ve had (in which I was – unfortunately – entirely sober). Why don’t doctors discuss things over cocktails – I feel my retention would improve?

So, when making a decision about implanting Ben’s BAHA we had to decide whether to implant one or both. As I said before, we have three different audiologists – all of whom I respect and – with three somewhat varying opinions. Ben’s right ear is a no brainer – we will be implanting it – the BAHA seems to work best for him in that ear.

To decide whether to implant both ears, we took into account the following considerations:

Ben currently uses two BAHAs in school, so implanting the second one will not be a big change in the way we have been hearing. In fact, he should have better overall quality sound with the implantation surgery because there is less interference from his headband, hair, etc. (he also might be able to hear ‘s’ sounds more easily.

He could still use ‘in the ear’ hearing aids post-surgery if he chooses to.

Double the BAHAs = double the chance for infections/complications

By bypassing the left cochlea – which is what we think will happen – the cochlea’s function might erode over time.

We have discussed doing the right, then doing the left later – this would involve a different surgery down the road. Things to consider for this is each implant surgery is actually 2 surgeries, so postponing a surgery will be adding on two more surgeries later. With surgery comes intubation and full sedation – which is riskier for Ben, so less is better. The surgery itself is not that invasive – it is just a day surgery.

If we end up putting one in on the left side and we later decide to not use it, we can just remove the abutment and use in-the-ear hearing aids. We are not destroying any physical real estate by doing so.

So….after much discussion…(drumroll please), we have chosen to implant both at the same time because less surgeries was the most important factor for us. As well, Ben has been using the BAHA’s for the past two years, so it’s not like this is some way out there thing we are doing. We are currently using this equipment, so by implanting it we are just hoping for better sound quality and convenience. So, we are currently on the waiting list for that to happen.

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5 thoughts on “Complicated Hearing

  1. Wow! Big decisions but with all the thought that has gone into it, you can’t go wrong. Don’t have regrets in life when you make the best decisions with the information you gather at the time. I love you guys and your attitude! Thank you for sharing your journey. xoxo Aunty Sue (Aunty Zap It)

  2. I love hearing about the BAHA from another person’s perspective. I have Goldenhar Syndrome as well and am a candidate for the BAHA. When I put the external BAHA (not the surgically implanted one) on for the first time I couldn’t believe how noisy the world was. That moment was a once in a lifetime moment. Thank you for sharing your story. 🙂

    1. Thanks for responding Sarah! Ben’s been using his BAHA headband for about 2 1/2 years…he seems to like them! They just called us to do his implant surgery, but I have to delay it til spring or summer as he will be having other surgeries this winter. I have been told that once he implants them he will hear his s sounds better! I checked out your blog – it’s awesome!

      1. I have heard that the surgery helps along with sound quality. I’m sure you are very excited to see Ben’s reaction to sound after he receives the surgery. I wish you and Ben luck!

        And thank you so much! That means the world. 🙂

  3. I love hearing about the BAHA from another person’s perspective. I have Goldenhar Syndrome as well and am a candidate for the BAHA. When I put the external BAHA (not the surgically implanted one) on for the first time I couldn’t believe how noisy the world was. That moment was a once in a lifetime moment. Thank you for sharing your story. 🙂

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