The Cochlear Implant Experience

Is A Cochlear Implant Right For You?

Hearing loss affects 95% of superficial siderosis patients. Specialized hearing aids designed to help word recognition in bilateral sensorineural hearing lossOverview In approximately 95% of superficial siderosis patie... More work very well for most patients in the early stages. Unfortunately, many people will eventually reach a level where it’s simply impossible to understand speech. As a result, a widespread discussion among superficial siderosis patients is whether a cochlear implant is a good option.

Hi Jaclyn, can you share with everyone how you acquired superficial siderosis?

When I was 12 years old I was in a horseback riding accident. The horse I was on started running downhill very fast. I fell off and suffered numerous internal injuries. I had a broken pelvis, broken neck, broken collarbone, bruised ribs and kidneys, my lungs collapsed, and I suffered a traumatic brain injury when I hit the left side of my head which left the right side of my body my tongue paralyzed. 

I was airlifted to Baptist Hospital in Winston-Salem, NC. The first few days were touch and go. The doctors were not sure if I was going to pull through and if I did they believed I would be paralyzed for the rest of my life on my right side. Despite all of these injuries, I did not have to go through any surgeries. To everyone’s surprise, I slowly began to move my right hand, then my right arm, and my leg. I spent a month in the hospital and then I was moved to an inpatient rehabilitation center where I spent the next 2 months learning to walk and talk again. 

Since the accident, I have been completely fine and I have had no symptoms of SS. In fact, I had no idea that I had it until 3 years ago when I began to notice that I had a hard time understanding what people were saying. I went to the ENT and had a hearing test. It revealed that I had moderate hearing loss in both ears. I got hearing aids and everything was fine for a year. Then I noticed a major drop in hearing in my right ear. I returned to the ENT and a hearing test revealed that my right ear hearing loss had moved to the profound category.

At that point, the ENT ordered an MRI. The MRI report came back and I was referred to a neurologist. The neurologist told me that I had atrophy of the cerebellum and that nothing could be done. Something did not feel right to me so I asked for a copy of the MRI report. Upon reading the MRI report I saw the words Superficial Siderosis.

I found the Superficial Siderosis group on Facebook and learned about Dr. Kumar. My husband and I traveled to Mayo Clinic in Rochester, Minnesota to meet with him. He ordered an MRI and a CT Myelogram. However, my leak could not be found. Dr. Kumar said that there could be a chance that my leak was from my one-time TBI and that there is not an active leak. I wanted to know more, so a few months later we traveled to Boston to see Dr. Levy at Mass General. He agreed with Dr. Kumar that there may not be an active leak but the iron from the one-time leak was obviously still there. He prescribed me Ferriprox and I have been taking that for a little over a year.

Could you discuss a little about your hearing history?

I believe that my hearing has been dropping for the past several years but I did not notice until 3 years ago. It got to the point in July 2018  where I noticed that I was having trouble hearing people in noisy situations. That is when I went to the ENT and found out that I needed hearing aids. After a year with the hearing aids, my hearing dropped suddenly in my right ear in November of 2019 and a hearing aid did not help any longer.

I still had great hearing in my left ear with the hearing aid so I remained very social and continued my job as a 2nd-grade teacher. In August of 2020, I returned to work in person (after being virtual for several months) to get ready for the upcoming school year. At work, I noticed I could not understand people at all with their masks on and struggled to hear people even without a mask on when they were across the room from me. I also could not understand people when I was talking on the phone and on google meet I had to start turning the captions on. I returned to the ENT and found out that the hearing in my left ear had significantly dropped as well. At that point, we started talking about a cochlear implant.

I know many of your fellow superficial siderosis community members can relate to your experiences. How did you learn that a cochlear implant might be a solution?

The first time I went to the ENT and found out about my hearing I just had a feeling that this was not the end and it was going to get worse. At the very first appointment, I talked to the ENT about what happens when the hearing aids are not enough and he talked to me about cochlear implants. I had that in the back of my head from the start. The scary thing was that when I learned that I had superficial siderosis I also learned that there was a chance that a cochlear implant may not be successful.

How long did the entire process from planning to completion take?

I qualified for the cochlear implant in January and had my initial appointment with the surgeon in January and I had the surgery in June.

What types of testing were needed to see if you were a good candidate?

The first test is a hearing test with your hearing aids. In order to be a candidate, you have to have less than 60% hearing WITH hearing aids. The very first time I did the test was in September of 2020 right after my hearing in my left ear had dropped. At that point, I was at 62% and did not qualify. Honestly, I was a little relieved that I did not qualify at that point because I was still very unsure about what I wanted to do.

Over the next few months, I could tell that my hearing was continuing to get worse. I began to have trouble understanding people even when they were not wearing masks.  I went back for a re-test in January and I was at 55%. This time I felt relieved that I qualified. Hearing had become even more difficult and I was becoming more and more isolated. Before going in for the test I had already made the decision that if I qualified I wanted to move forward with the surgery as quickly as possible. After the hearing test, I had to have a CT to look at my cochlea. The CT checks to make sure that your cochlea has a normal shape and that there is no growth that could interfere with the placement of the implant. I also met with the ENT so that she could go over all of the risks of the surgery and what to expect.

How long did the implantation procedure take?

We arrived at the hospital at 7 am the morning of the surgery and were home by 2 pm. The surgery was an outpatient surgery and the actual surgery took about 2 hours. The whole process was much easier than I expected. When I woke up I was in some pain and the ride home from the hospital was miserable, but by the fourth day, I was off of pain meds completely and back to normal activities.

How long was the time from when you had surgery until you returned to have your implant programmed?

I had my implant activated four weeks after surgery. Those four weeks were hard because I was so anxious about if the implant would work. The side that was implanted felt very plugged up and hearing people during that time was even more of a struggle than usual. I asked the ENT about this and she said it was from fluid during surgery and it would go away within a few weeks.

The good news was I actually did keep some residual hearing in my right ear (sometimes you lose all residual hearing after implantation). My husband reminded me to hold strong to my faith and that no matter what the outcome God was in control and we would get through it. For me, having a strong support system with my husband and my mom has been crucial.

My mom went with me on my activation day. The audiologist told me all about the processor and showed me how to put it on. She told me to count the number of beeps I heard when she played a series of beeps. At first, I did not hear any beeps. I was so nervous and was on the verge of getting very emotional. Then, I started to hear them. We went through this several times where I counted the number of beeps and then we did something where she played a beep and I had to tell her if it was very soft, just right, or very loud. This process is called “mapping.” MAPs are programs that help to optimize the CI user’s access to sound by adjusting the input to the electrodes on the array that is implanted into the cochlea. After we finished, she said some words, and I had to repeat back what she said. I got them all right! On the drive to our home, I could hear EVERY word that my mom said. This would have never have happened before as the noise of the car always made it hard for me to have a conversation. At first, voices sounded a little strange, like Mickey Mouse but I had expected this and knew that it would get better.

How often do you have to return for programming tune-ups?

The first time I went back was two weeks after activation. Then a month after that. At this point, I will go every three months.

How would you rate your ability to understand speech or hear music now? Has there been an improvement over time?

I have been blown away by the improvement and all of the things I can hear now! Some examples that have been huge for me are hearing the doctor at my children’s appointments even when she is wearing a mask, hearing crickets, hearing birds chirp, understanding what my kids are saying from the back seat, being able to understand people on the phone again, having conversations with my husband when we are driving down the road, understanding every word the pastor says in his sermon, going out to eat and hearing my friends even with all of the background noise, watching TV without the captions, being able to understand my favorite podcasts in the car again, and more.

Hearing loss can be so isolating. I had found myself withdrawing from social situations, dreading family get-togethers, and browsing Facebook instead of engaging in conversation with my kids and my husband. I was considering resigning from my job as a teacher because it was just too difficult to hear the students and my colleagues. Having a cochlear implant has truly been a miracle for me.

Unfortunately, we know cochlear implantation may not be successful in every case of superficial siderosis. So what advice can you give someone thinking about a CI as an option?

I would say go for it! I know it is scary knowing that it may not be successful but my hearing was so bad in my right ear that I could not hear regular conversations at times. If the CI had not been successful, that would have been so hard, but at least I would have known that I tried. I did not have much residual hearing left in my right ear but my doctor knew that I was anxious about it being successful so she put me on heavy steroids before and after surgery and gave me steroids through an IV during surgery. The steroids helped to preserve any residual hearing that I did have.

So, if the CI had not worked I would still be in the same place that I was with my hearing before the surgery. I would also say talk to your audiologist and ENT about Superficial Siderosis. My ENT and audiologist did a lot of research to learn more about SS and they were both aware that the CI may not be successful. Also, join a cochlear implant group on Facebook. I joined one when I was trying to decide about the surgery and it was a huge blessing for me! I connected with other teachers and moms in the group who have a CI and they answered many of my questions. I read through posts with people’s experiences and I was able to share my own experience with the group.

Finally, I would say get a good support system. My friends and family rallied around me and lifted me up in prayer constantly. They were prepared to stick by me whether the CI worked or not. My husband and my mom even learned “Cued speech” with me. I know that having that support and trusting in God’s plan is what ultimately helped me to make the decision to move forward with a CI and I am so glad I did!