A bilateral cholesteatoma survivor tells her story

Meet Amy Mackey. Amy is a two-time survivor of cholesteatoma and a Ponto Plus and Oticon Medical Streamer wearer. Amy is a writer, so we could think of no better way for her to share her story than through her written words below.

Name: Amy Mackey
Indication: Bilateral conductive hearing loss
Sound Processor: Ponto Plus Power

“I’m a survivor of bilateral cholesteatoma”

by Amy Mackey 

As an infant and small child, I suffered from repeated ear infections. I remember my mother used to back me up against the refrigerator to give me nose drops instead of the ear drops used nowadays. She always told me she knew when I had an ear infection; I would always be cranky. By the time I was five, I had participated in a group study of bilateral myringotomy (tubes in both ears). From what I understand, I was the only one in the control group not to outgrow my ear problems. Throughout my tender years I had repeated myringotomies, doses of antibiotics, eardrum lancing – occasionally my eardrums would burst on their own from the pressure of fluids.

At 17, I went to my ENT thinking I was suffering from yet another ear infection. I was immediately sent to the hospital for X-rays. CAT scans and MRIs were still a thing of the future. Returning to my ENT specialist after office hours with my suspicions raised, he told me I had a cholesteatoma in my left ear. Less than twelve hours later, I underwent a five-hour surgical procedure.

Placing myself strategically 

I spent several days in the hospital with extreme vertigo, numbness in my tongue and static in my ears – commonly known as tinnitus. I was grateful that my facial nerve had been spared. It took months to relearn how to walk, sit or stand without listing like the Titanic. It was a good thing that I couldn’t feel my tongue, because I chomped on it on a daily basis. But time heals, and my white-walled hair grew back, the vertigo mostly subsided, and I adjusted to a tongue that I could not feel and the constant hum in my head. I was young, tenacious and determined. Even with my single-sided deafness, I learned to cope early on, not going out in the wind without covering my deaf ear, otherwise I’d get vertigo again, strategically placing myself to hear better in groups or classrooms. Sound direction was a big challenge, and I learned to train myself until the point where if I thought it came from the right, I would look left – the opposite of what my brain was telling me.

The calm before the storm

Over the next twenty years, I continued to struggle with many ear infections in both ears, cleanings, antibiotics and eardrops, as well as my “good” eardrum being lanced and drained on a regular basis. Then, I had about 15 years of only minor incidences. This was the calm before the next storm.

Last July, I went to a dinner party and immediately noticed that I was struggling to hear. It was like someone had flicked a switch and turned off my hearing. There was no gradual decline. I had no pain, no drainage, and no odor came from my ear. I felt some pressure in the right side of my face on my cheek, similar to a sinus infection. I assumed my ear was full of fluid.

Self-diagnosis 

Over the years, I had developed a few techniques to self-diagnose my ear troubles. I would tip my head upside down to see whether there was fluid in my ear. I did this and got the classic sound and feeling of fullness and fluid present. I was also taught by my surgeon to plug my nose and gently (I emphasize gently!) pop my ears, keeping the Eustachian tube open to allow the pressure to equalize and drainage to release through the Eustachian tube. I could not pop my ear. Many years ago, a holistic doctor had also taught me a technique of washing my hands and taking a finger, reaching inside my mouth and gently massaging the orifices (nasopharynx) behind the roof of my mouth to help promote drainage. That didn’t work either. So, thinking it was a sinus problem, I tried hot steam with eucalyptus oil and a towel over my head. This period of trial and error lasted for almost a month. Our raspberries popped up in the garden – our first harvest – and they tasted horrible to me, almost metallic. Little did I know that this was another sign that things were amiss. Then, I started getting terrible migraine headaches. I made an appointment with my ENT specialist. In the back of my mind I kept on thinking, “Could it be another cholesteatoma in my good ear?”

My worst nightmare came true

My regular ENT specialist who I had been seeing for 25 years was downsizing, so I was introduced to a new partner. He came into the room and I casually said to him, “I think I just need my eardrum lanced so it can drain, and then I'll be on my way.” I explained some of my history, and he took a peek in my ears and said he would like to look under the microscope. After looking under the microscope, he very solemnly told me I had a cholesteatoma in my right ear – my “good” ear. My worst nightmare had come true.
I was horrified, for I am profoundly deaf in my left ear from the first cholesteatoma 36 years ago. I sat there and cried, thinking I would be deaf for the rest of my life. I do know some sign language, but as my surgeon pointed out, not everyone does. My children can sign, but my husband can’t. How am I going to communicate? How big is this infection, and has it eaten all the hearing bones like the last time? Over the next few days, and after the CAT scan, I began to accept my deafness.

I was a prime candidate for a bone anchored hearing system 

A week later, I had a hearing test and a follow-up appointment. We discussed whether I should go to a bigger city to have the surgery, possible reconstruction of my middle ear and the eligibility of a bone conducting implant or bone anchored hearing system (BAHS). After some debate, we decided that my doctor could do the cholesteatoma removal here in Montana. My audiologist and surgeon concluded that I would be a prime candidate for a BAHS implant after the removal.

Thirty-six years ago, there was not much available to help with my hearing loss. I began researching bone conducting implants. There was not a lot of information out there, but I was amazed at what I did find. It is such a simple concept, yet I could not imagine how I could hear with it vibrating throughout my skull. Everyone would ask whether it was a cochlear implant. I kept explaining that it is a bone conduction titanium implant that is placed in half the depth of your skull, with a sound processor attaching to the abutment that sticks out of the skin. The sound processor picks up the sound and vibrates it to the auditory nerve through the bone. The implant should last a lifetime, and if it becomes loose, it can be easily removed and placed in a new location.

Simple surgery

My surgeon called me about five days later and suggested that I have the BAHS put in first, so that I would not be totally deaf when I had the tympanomastoidectomy. He also decided that I needed the more aggressive canal-wall-down surgery for the cholesteatoma – just like my left ear. The aggressive surgery has only a 10–20% rate of reoccurrence, whereas the less aggressive has an 80% chance of returning in the first two years. I agreed; I never wanted to go through this again – twice is plenty. He also assured me that if it did reoccur, it could be taken care of in the office rather than through major surgery.

On September 23, 2013, I had the implant surgery. It’s a simple one-hour surgery that is commonly done on an outpatient basis. I think the hardest part of the implant surgery was the five-week wait for the sound processor. I had endured four months of nearly total deafness and 36 years of single-sided deafness. I had no fathomable idea of what was to come.

At that moment in time, I could not hear my own footsteps – not a good thing living in bear country. Nor could I hear the phone ring, water running, the cry of an eagle, vehicles running or many other everyday life noises. The tinnitus had taken on several forms and was now in both ears, creating static, ringing and pulsating with my heartbeat. Migraines were almost a daily occurrence, totally debilitating me and turning my right eye red. Conversations were almost impossible, even in a quiet setting. In large groups, it was horrifying – all this garbled sound, not understanding anything, getting only bits and pieces and trying to make sense of it was physically and emotionally taxing.

A changed life

My grandson, Michael, went with me on the day that I got my Ponto – both of us bubbling over with excitement. The first voice I heard was Michael’s. It had dropped an octave in the four months since I had lost my hearing; no wonder I couldn’t hear him. I cried and instantly realized how much I had been missing with my hearing loss. When I drove the 35 miles home, I had my window down and noticed I kept flinching when oncoming traffic passed me. Everything was so loud – it was a whole new world.
 
In November came the cholesteatoma removal. I feel so fortunate. My surgeon, who is not only a skilled professional but also a kind and compassionate physician, successfully removed the cholesteatoma in four hours. He was able to repair the single hearing bone that was not destroyed with a fascia-cartilage graft, in order to retain what little hearing I still possess. The area for reconstructing the other bones was too small, so I was not able to have middle ear reconstruction. He also grafted a partial eardrum to prevent bacteria from entering the Eustachian tube. My facial nerve was once again spared, but I did not get so lucky with my tongue, and I have lost most of my sense of taste. I am five months out now, still healing and adjusting to hearing more than I have done in 36 years.
I consider myself a survivor and am grateful every day. I still have the many forms of severe tinnitus, the occasional vertigo, a mild form of recruitment (distortion of speech), fewer migraines and a tongue that no longer tastes. But thanks to the Ponto Plus Power and Oticon Medical Streamer, I can hear. It has basically changed my life.