Reverse Slope Hearing Loss: Hearing Aid Journey

A few months ago I was diagnosed with severe hearing loss.  Since then my life has been turned upside down with researching, doctors visits, and attempted hearing aids.  The last time I updated, I had finished my hearing aid consult and was going to get my hearing aids soon.

The only thing my AUD (audiologist: hearing aid doctor) mentioned about my type of RSHL (reverse slope hearing loss) was that “because my hearing loss is unique, I could use any type of hearing aid and they should all work.”  (Looking back, that was so very wrong…but I will get to that in a minute.)  Insurance responded to my request saying that they would pay up to $2,000, and anything above that would have to be paid out of our pockets.  The highest model hearing aids are around $6,000, meaning we would have to pay $4,000 out of pocket if I went with the best model.  After discussing this with my AUD and my husband, we decided to start with a low-mid level model and only paid about $1,000 out of pocket.  I wanted something as discrete as possible, so we decided to go with an in the ear hearing aid.  Unfortunately for me I have very small ear canals, so they had to make my hearing aids protrude out of my ear more than they should have, making them quite visible.  My AUD played with the program a bit asking me if one setting sounded better than another.  I did my best to answer the questions, but all the new sounds coming in my head was very overwhelming.  After a little while she said I was good to go and we would schedule another appointment in a week to adjust them more.

I was so disoriented as I tried to stumble through her office to the receptionist desk.  I could barely put words together to form a sentence to schedule my next appointment.  The door hinges, the pens clicking, the receptionist typing, all were so overbearingly loud I just wanted to cry.  I was so confused I left my bag at the desk and they had to chase me down to give it to me.  I got in my car, and the sound of my car door shutting made me burst into tears.  What was happening to me?  I barely was able to drive home, the sound of the cars driving past, the tires on the road, my turn signal blinking, all overwhelmed me.  Once I got home I was supposed to make dinner.  I stood in the kitchen just staring at the refrigerator, at the cabinets, not being able to remember what I was supposed to do.  I literally could not put thoughts together.  I felt like a zombie.  I felt weak.  I gave up making dinner and sat on the couch and cried.  I texted my friend this:

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I posted about my hearing aids and how I was feeling on Facebook, as well as two support groups for Otosclerosis, the disease I have.  Everyone kept posting similar words of encouragement.  Someone they knew or they themselves got hearing aids and it took them a few days to adjust, but their life was so much better.  “I could finally hear the birds chirp again!”  “My father said it was the best thing to ever happen to him!”  Why wasn’t I feeling this way?  My final conclusion was that I must be weaker than everyone else.  This was not the best thing to happen to me, I couldn’t stop crying for days and I couldn’t even make myself toast.  I couldn’t put words together.  “It will get better, it is a big adjustment.”  I didn’t want to adjust to this new world.  Opening a wrapper was so loud I started crying, but now I couldn’t hear my daughter crying in her bedroom when I could before.  My ears felt plugged up, like I was under water and listening to the world on a really crappy microphone and speaker.  Everything sounded fake.  Voices were unrecognizable.  The front door, the microwave door, the refrigerator door, the cabinet door, once all made different unique sounds, now all sounded the exact same.  Hearing myself speak was horrible.  There was a crazy feedback and distortion, so I opted for just not talking as often as possible, and when I did I whispered.  My hearing aids also made me nauseous.  When I was wearing them I would get so dizzy that once I even threw up, but the second I took them out I instantly felt better.  Was this my new reality?  Everything I knew about the world was now different.  But not a good different like everyone else seemed to experience with hearing aids.  I could NOT hear new sounds, but instead sounds I used to be able to hear were either absent altogether, or excruciatingly loud, or distorted to the point of unrecognizable.  I was weak.  I couldn’t handle this.

I called my AUD and explained everything to her.  We decided the in the ear aids weren’t working for me and we were going to try a behind the ear model instead.  I came in a few days later to pick up my new aids.  She chose an open dome ear piece, which was going to let in the most natural sound possible.  After a few tweaks, she again sent me on my way.

These aids were MUCH better.  I no longer was nauseous and a lot of my natural high pitched sounds were coming through nicely.  Sounds weren’t as distorted as they were before and things sounded so much more like natural sound.  “I found my aids!!” I thought! Though they were so much better, they still had some issues.  My left aid was making that same distorted/cracking sound a broken speaker makes when it hits certain pitches or frequencies.  This sound was worse when I spoke, so I found myself subconsciously not talking as much or trying to pick shorter sentences so I didn’t have to hear the sound.  I also turned my hearing aids way down so the sound wasn’t as loud, but obviously that then turned down everything else as well.  When I was in my car I would play with the radio, taking one aid off, then the other, then both, seeing if I could hear any differences between them.  I put my left aid, the one making that broken sound, in my right ear, and I still heard it!  I was convinced my speaker was broken.  I put my right aid in my left ear and I did not hear the sound in my left ear, so to me that ruled out the problem being in my ear.

At my appointment two weeks later I brought this concern up to my AUD.  She listened to my aids through her machine and said that not only did she not hear that sound I was talking about, but my left aid sounded better to her than my right.  What?!  I was shocked and confused and embarrassed.  I KNEW I heard a sound.  She was a good sport and put a brand new speaker on my left aid, and I still heard that sound.  She said that the sound was probably in my head somehow, and it “should” “hopefully” get better as I got used to the aids.  She tweaked a few things and sent me on my way again.  As I drove home I thought about it more…how could this sound be made up in my head?  I heard it in only my left aid, in both ears, and my left ear didn’t hear the sound with my right aid.  There has to be some other explanation.

During this process I researched as much as I could about both Otosclerosis and reverse slope hearing loss.  One thing both my ENT (ear, nose, and throat doctor) and my AUD didn’t make clear is my type of hearing loss.  I have reverse slope hearing loss, and not just that, but the kind I have is steep and severe.  My hearing loss is very rare, and yet neither doctor thought that was important to tell me.  After much research, I know this is important for me (and my Dr) to know for a few reasons.  1.  There are only about 3,000 of us with RSHL in North America, that means that ENTs and AUDs can go their entire career without seeing someone like me.  2. Not all hearing aids are meant for all hearing loss.  In fact, ALL hearing aids on the market are made for traditional ski slope hearing loss.  In a nutshell, all hearing aids are made to work the exact opposite of how I need them to work.  If you have an AUD who is not familiar with the unique nuances of RSHL, they wouldn’t know not only which hearing aids will work best for you, but also how to program them properly.  Programming hearing aids, especially RSHL, isn’t just a plug and play type of deal.  After doing a ton of research, I have heard of people with RSHL that needed their aids programed almost the exact opposite of what the computer recommended for them.

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After playing with my hearing aids more in the car, I realized two major things.  1.  I still wasn’t really hearing any new sounds coming through my aids.  Louder, sure, but volume was never my issue since I have above average high pitched hearing to begin with.  I still could only hear the same low pitched sounds I could without my aids.  2.  After taking my aid on and off during a song, I realized I could not hear any different in my left ear, not even volume.  I could not tell any difference at all, except I could still hear that broken speaker sound overpowering everything else.  I came to the conclusion that not only were my hearing aids not giving me new sounds, they were now hindering the good hearing I had.  It was time for them to go.  I realized that my current AUD would never be able to get my current hearing aids “just right,” though I am not even convinced there is a such thing with RSHL.  My trial period was coming to an end, so I returned my hearing aids to my AUD, thanked her for all her help, and walked out the door.

I have asked others with Otosclerosis and I got mixed answers as to whether or not their hearing continued to decline with each subsequent pregnancy.  As I mentioned before, my Oto was brought on by pregnancy, which is common even though scientists still aren’t sure why.  I have been asked if in hindsight if I would have still had my daughter if I knew I was going to lose my hearing.  Though it makes me sad, it is a valid question.  I have read stories from others who were diagnosed with Oto before having kids and decided to not have any children in hopes of not making their hearing worse.  It is a very personal and tough decision, but I know deep in my soul that I was born to be a mother, and it has brought my the greatest joy in life.  I would not trade Torey for anything in the entire world.  My husband and I have decided that we will have at least one more child.  To me, completing our family is more important than my hearing.  The only thing I am worried about is a 50% chance of giving Oto to my children.  We will remain diligent and continue to have my children get routine hearing tests.  By then, hopefully modern medicine will catch up and there will be more opportunities for them than I currently have.  Once we are done having children, I will find an ENT and AUD that specializes in RSHL, even if I have to travel very far to see them.  Then I will get another hearing test and re-evaluate what I could do from there.  For now, I get to just sit back and watch The Walking Dead with subtitles.

During this journey I made a friend online who also has RSHL caused by Oto, which is a very rare combination.  It turned out that she happens to live less than an hour away from me, so last week she and I got dinner with her friend who also has RSHL since birth.  It was great to sit and talk with two other ladies who have experienced the same challenges I have.  This Saturday I am going with them to a HLAA meeting (hearing loss support group) where I look forward to meeting other people and expand my support team.

If you want to read what I wish someone told me the day I got diagnosed with RSHL, click here.

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