Living With a Cholesteatoma, the Tumor That Destroys Your Hearing

In honor of today being "Rare Disease Day" and March being Cholesteatoma Awareness Month, I’ve decided to share my experience, as well as what I have been going through with this disease over the past year. While it is a rare (1 in 10,000) condition, more awareness is needed and support for those in the community suffering.

I’m a bit of a cyberchondriac. Undiagnosed, of course. But I regularly worry about my health and Google my "symptoms" for medical ailments that I do not have. And while I regularly self-critique in aims to better myself, this is something I’ve never quite had a desire to change. Mainly because I figured if my body did develop something unwanted, I would be the first to know. And I was right.

In October 2016, I experienced my first sinus infection. This was the start of the end of my ear health. Handy Google told me the best way to get rid of one was to use a neti pot, tilt your head, and "blow" gently through your nostrils.

Yeah, so please don’t do that. I’m confident that is how water traveled into my ear and why I ended up with an excruciating ear infection later that evening. But the infection cleared and shortly after I was good as new.

Months went by, and I was feeling great. It was a new year, my health was dandy (or so I thought), I had started a new job, and overall everything was going well. Except for my earwax.

It’s not something you probably stare much at, but you’re probably familiar with what stares back at you on a Q-tip. Mine was off. A little brown, a little wet, sorta sticky. A darker than normal color. The average person wouldn’t think much of it, but I’m a not-so-average person when it comes to my health.

My GP was off on maternity leave so I headed to the clinic. They looked inside my ear, saw inflammation, and wrote a prescription for antibiotics (?). Not sure why since I had weird earwax, not an infection, but okay, trust your doctor.

A week passed with the same gunk in my ear, so I went back—for another look, another prescription, and another “you’re fine, it’s just some inflammation.”

Third time's a charm. It had been two weeks of gunk at this point. I was starting to get a bit concerned, so I asked to see a specialist. I pay taxes for a reason. I wanted an appointment with an ENT doctor.

The first ENT doctor was completely useless. He spent more time in my nose than he did in my ear. He went on a ramble about my "deviated septum," and told me I had some fluid in my ear that will drain in a couple weeks. He wished me well and sent me to wait over 45 minutes for a hearing test.

I walked out.

I wasn’t satisfied. I wanted a better look. Something felt off and I needed someone to take my concerns seriously.

So back to the medical clinic I went, for the fourth time, to request a different ENT. Persistence is key here, folks.

We got somewhere. Dr. Marshall Hay didn’t tell me I had an infection. He didn’t prescribe any antibiotics. He didn’t even say “you’re fine.” And he most definitely did not look in my nose.

Dr. Hay asked me a few questions. He figured there was more going on behind my ear drum than what he could see. Dr. Hay sent me for a CAT scan.

Aren’t the best doctors the ones who take precautions and double check your suspicions instead of walking you out the door?

A week later, he diagnosed me with a cholesteatoma.

A cholesteatoma is a nasty little bugger. Essentially, it’s a benign (not cancerous) tumor made up of dead skin cells that collect and grow into a mass. It’s completely painless so a lot of times patients don’t even know they have it. It’s more or less symptomless, but it grows and the bigger it gets, the more havoc it creates. No one really knows the cause. Dr. Hay said it really came down to a matter of luck. Thankfully, if caught early, the tumor can be removed without too much hearing loss. If caught late in the game, however, facial paralysis and even death can sometimes be the end result.

Mighty good thing I was persistent.

On January 23, 2018, I had a 2.5 hour surgery to remove the cholesteatoma. The scariest part were the risks, the major one being complete and irreversible hearing loss as well as possibly facial paralysis, say if the tumor was wrapped around my facial nerve or the surgeon accidently nicked it. Thankfully, my surgeon had a good night’s sleep, but I double-checked when I awoke by smiling. Both sides of my face still moved? Check. Hearing? A little iffy.

While the surgery was overall a success, it was bigger than they anticipated. If even one skin cell is left behind, the cholesteatoma will sprout all over again. The cholesteatoma ate away my three primary hearing bones, but my cartilage was used to reconstruct them as best as possible. I’ve been warned that there is a high possibility I will have a second surgery later this year.

As for now, my hearing is a bit of a waiting game. I have my first hearing test post-op in a few weeks, and while I have come to terms with the fact that my hearing will never be the same, it’s still tough.

My primary reason for writing this, aside from documenting the experience for myself (because for me, writing is therapy), is to really bring awareness to this disease. Prior to this, I’ve never heard of a cholesteatoma. And now anytime I tell anyone with a medical degree that I have had surgery to remove one, it sparks the same reaction every time, “Oh wow! They’re so rare, I’ve never met someone with one before.” My rare condition really seems to light up a medical professional's world.

Maybe it’s because they’re so hard to catch—you’ve got a whole eardrum blocking the view and it’s painless. Anything could be going on behind there, which means even more awareness is needed (and persistence on your part).

When I was first diagnosed, I spent hours on the internet, trying to learn more and find other young people suffering with this same condition. Through a few searches and Twitter hashtags, I was able to find a few people I could chat with to see how they’ve been affected and their healing process. I also found an online Facebook forum dedicated to people who have been diagnosed with a cholesteatoma. The online community is amazing. It’s also helped me better understand the disease and realize that I’ve been very lucky. Most people don’t get diagnosed as early as I did.

The statistic of 1 in 10 people diagnosed with a "rare disease" is often quoted, yet it’s truly hard to determine because there are not enough people trained to diagnose rare diseases and some conditions are either too severe to be compatible with life (or too mild so are missed). If you would like to donate to the Rare Disease foundation, or just want more information on rare diseases in general, click here.

https://www.rarediseasefoundation.org/donate

And if you have, or had, a cholesteatoma and stumbled upon my post, and want someone to reach out to, please shoot me a message. I understand the need for support, and it’s even harder when there’s limited resources on your condition. I welcome anyone wanting to chat.

And to all you healthy individuals out there—trust your gut instinct! If you feel something is off, push for the services you believe you need. Ask to see specialist. Get a second opinion. Don’t settle. Make your health a priority. No one else will.

You only get one life, but thankfully in my case, you get two ears.