Living with mitochondrial disease and deafness

3 May 2022

Losing my hearing due to mitochondrial disease was frightening and lonely – until some amazing technology came to the rescue, writes Jojo Wells.

Hi, my name is Joseph, or Jojo for short. I’m 18 and I have a mitochondrial disorder, which affects me in ways such as tiring easily and fatigue. Another way it has affected me is it has caused my hearing to go, leaving me deaf.

I was diagnosed with mitochondrial disorder (more specifically RRM2B) when I was around 12 or 13 years old, and my hearing has been declining since then or even sooner. 

When I first started secondary school I didn’t need any aids for my hearing, but as time went on and my hearing loss progressed it became clear that I needed hearing aids. I was reluctant to wear them though, and this made my life unnecessarily hard. I was working twice as hard as everyone else just to hear what was happening and what was being said. 

One day a big package arrived at my house with my cochlear processors and all the equipment and accessories to go with them. It was like Christmas for deaf people." This went on for a while. Even though my parents and audiologists were begging me to wear my hearing aids, I didn’t like feeling different. I wanted to be normal, but denying it and pretending it isn’t there wont make anything better. 

One day I finally caved in and wore my hearing aids, telling myself I could take them out if I wanted to. But I didn’t take them out. I came home and told my parents they were right, my hearing aids did make it so much easier. Since then I've worn my hearing aids every day. 

A long wait for surgery

Sadly, as time went on my hearing loss progressed, and the small hearing aids I had weren’t enough anymore. This was really upsetting for me and it meant I had to wear larger hearing aids with big ear moulds. Once again I didn’t want to wear them, and once again my hearing got worse and worse until I started to wear them and benefited from it. 

However, last summer I woke up one day and my hearing was completely gone. This was devastating for me. It meant that I needed cochlear implants, which we had actually been trying to get for a while since my hearing in my left ear was a lot worse than my right. But now the hearing in my right ear had gone too, and cochlear implants were the next step. Unfortunately due to Covid appointments where hard to get, and I had to wait six months for surgery. 

This meant I spent six months not being able watch what I wanted to watch, not being able to play the games I wanted to play. But most importantly I couldn’t communicate. I couldn't talk to people and I knew very little sign language, so I either communicated with people through my phone using an app to convert speech to text, or I didn’t talk to anyone at all. It was a very lonely time. Missing out on opportunities, and even not going to college for a while, I was beyond bored. It caused my mental health to plummet. Time continued to tick forwards, and we were counting down the days till my surgery. 

A special delivery

Eventually the day of my surgery arrived, and me and my dad drove to the hospital. The surgery took around four hours but there were no complications, and I only had to stay in hospital for one night. But the healing process wasn’t easy. My head was swollen and I had to wear a very tight headband to keep pressure on the wound. This made life hard, and we had to cut a big hole in a pillow for my ear so I could sleep. During the healing process I was still deaf, as the next step after surgery and healing was getting the equipment and activating my implants. I couldn’t wait. 

One day a big package arrived at my house with my cochlear processors and all the equipment and accessories to go with them. It was like Christmas for deaf people. Now we had my processors, and all there was left to do was activate my implants. The audiologist stuck the processors to my head, connected them to a computer and after a few tests and questions she turned them on. At first everything sounded brushy, robotic, and squeaky. But I knew that as time went on, my brain would learn to hear again using the cochlear implants, and my hearing would get better and better. 

At the time of writing it has been twenty three days since my implants were activated, and my hearing is better than it's been in what feels like a lifetime. I’m wearing my implants everyday and it all sounds natural, as If I have had them my entire life. They even have Bluetooth so I can stream them directly to my phone and listen to it as if I was wearing headphones. My parents say that they have their son back, and I feel as if I have my life back.

Help fight mitochondrial disease by donating today

The Lily Foundation provides vital support to families like Jojo's, whose lives are impacted by mitochondrial disease. We also fund scientific research towards a cure. Please support us by donating today. Even a small donation makes an important difference. 

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