Jeno, an adult living with myotubular myopathy, provides us with his personal account of cholesteatoma surgery, to remove a benign lump in his inner ear.
Definition of Cholesteatoma – a growth in the middle ear and/or mastoid process. Presenting symptoms commonly include hearing loss on one side (of the conductive type), ear pain, and drainage. Dizziness is an occasional symptom. Cholesteatomas are not cancerous as the name may suggest, but can cause significant problems if left untreated. Treatment almost always consists of surgical removal. It has been recognized recently in a publication on a German Natural History Study that this might be more prevalent in x-linked myotubular myopathy than first believed, and can occur at any age.
‘As a child I didn’t really have ear problems, perhaps one ear infection at most. I suspect that an early symptom of cholesteatoma was from when I travelled on an aeroplane which resulted in terrible pain in my left ear on landing which got worse and worse. It was strange because when I was younger, I had no hearing or issues with my ears at all – maybe one ear infection at most. Remembering the aeroplane experience now, I believe it was the first sign that something was wrong.
But the first real time I noticed a symptom was when I started to get hearing loss about two years’ ago in my left ear. I started to notice my hearing decreasing a little – but in the first few months I could resolve this by pinching my nose and blowing out of my ears to clear it. I believed the cause was from a sinus-ear infection, so I wasn’t too bothered.
Over the next year my hearing would slowly decrease until at some point, I heard a very loud ringing noise in my left ear and my hearing on that side halved in a day. Because the ringing (or tinnitus) didn’t stop – I went to the hospital to get it checked out. At first, they couldn’t pin point what was causing my hearing problems, only that it was not conventional hearing damage. My local hospital first thought it might be an infection that had gone untreated for too long, as my eardrum was swollen and milky. They tried to place a tympanotomy tube, but immediately after realized that it wasn’t an infection and that the tube wasn’t going to help. They eventually did an MRI on me which revealed that I had cholesteatoma.
I was then referred to another hospital, more specialized in dealing with this ailment. They informed us that the waiting time would be about 10 months. But luckily this ended up being only 2. During this entire time, I was adjusting to living with severe hearing loss on my left side. It became so bad that, if I was in a room with a group of friends, I had to arrange the room in a way so I could optimally hear everyone. It became so hampering to me that, when I knew about the 10-month waiting list I decided to get a hearing-aid fitted, which (though not perfect in any way shape or form) helped me a lot.
After those initial 2 months I was invited for an intake where they made another MRI of my ear which revealed that the cholesteatoma had grown to a point where it had dislocated 2 of my ossicles and completely destroyed one of them. It also revealed that it was growing towards my cochlea. I was then informed that I would have to get surgery to remove the cholesteatoma. The hospital informed me that there was not really a choice because the cholesteatoma would keep growing and could eventually cause me to go deaf in that ear completely, damage my vestibular system and could even potentially cause brain damage if left untreated in the longer term.
For the surgery I planned to have a general anaesthetic, but due to my muscle condition they agreed to use a short working anaesthetic and apply those multiple times until the procedure was done. This was to avoid any post-surgical problems due to my muscle weakness. I wasn’t aware of the surgery itself, even though I was really nervous about having surgery performed. You can read more about ‘Anaesthesia and neuromuscular disorders: what a neurologist needs to know’, 2021, van den Bersselaar et al
Fortunately for me, they didn’t have to open up behind the ear but instead were able to operate through ear canal. The damage was relatively low so the pain post-surgery was not that bad. I recovered fairly quickly, even driving myself home the next day after surgery. But three days later I suddenly started having memory problems – like a delayed reaction to the anesthetic. Fortunately, the memory loss problems have subsided after 2+ months of living with this – but it did concern me as I couldn’t even concentrate on a TV programme and it has affected my ability to concentrate on study. I have just had a brain scan to check that everything is healthy.
Two weeks post ear surgery I had to go back for a checkup, at which time they took off my bandages and checked my ear. Everything pointed towards the surgery being very successful. Even my hearing had improved to the point that I didn’t need my hearing aid the improvement was so remarkably significant that it even surprised the doctor who performed the surgery.
Four weeks later, when I came back for another check, they and I had noticed my hearing ability was decreasing slightly again I thought that I was just imagining it and that I was just getting accustomed to my improved hearing. But after looking into my ear the doctor told me that the titanium prostatic that they placed just behind my eardrum (to replace the ossicles they had removed) had begun to topple over which, they told me, would be the likely cause of this.
I have since been waiting for my hearing loss to stabilise again so I can get my hearing aid refitted. As far as the cholesteatoma goes, I need to return to the hospital for another scan a year after the surgery to see if they succeeded in removing all of it, or if it has come back. We have also agreed that a yearly checkup scan, to see if no cholesteatoma would develop in my other ear, would be a good idea.
After this entire experience I am still left with the terrible unrelenting ringing noise in my left ear, hearing loss to the point where I still need a hearing aid and the complete loss of taste on the left side of my mouth (as they had to completely remove the taste nerve running through my left ear). The enjoyment of things like food and music have changed and I always need to have some sound around me to distract me from the ringing in my ear.
So I urge you, please, if you notice any hearing loss or other hearing related symptoms to get it checked out. Responding in time can save you a lot of extra regret down the road.’
by Jeno, member and patient advocate for x-linked myotubular myopathy at ZNM! (our German sister patient organisation – ZNM-Zusamman-Stark!).
We would like to thank Jeno for taking the time to share his insights about cholesteatoma.
For more information please visit:
For children in the UK, Great Ormond Street Hospital : https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/cholesteatoma/
For surgical recommendations: ‘Anaesthesia and neuromuscular disorders: what a neurologist needs to know’, 2021, van den Bersselaar et al
Or see this US website for guidance and information: https://dizziness-and-balance.com/disorders/tumors/cholesteatoma.html
Important: Myotubular Trust hopes that this personal perspective helps, but recommends you always consult with your usual healthcare team for professional medical advice, and for any specialist referral for treatments or procedures.
