Monday, March 16, 2009

Nerve Deafness

I originally wrote this post in my "Book Blog" (which I rarely write in anymore) on August 20, 2005 before my first cochlear implant surgery and thought it was worth repeating.

Nerve Deafness

When I was diagnosed with my hearing loss, my parents were told that I had "nerve deafness." Lately that term has puzzled me because I can hear sounds with the help of hearing aids. How can something be wrong with my "nerve" if I can hear voices and sounds around me? I wear the strongest aids on the market and currently wear Widex digital hearing aids. I've assumed that the doctors, audiologists, and speech therapists that I've had knew what they were talking about and just accepted that diagnosis. I've grown up believing that there was no cure for nerve deafness and never really thought there was anything else out there that could help me except my hearing aids. But, that doesn't seem to be the case at all. "Nerve deafness" can be a major misnomer because it incorrectly implies that damage is to the auditory nerve and not the cilia (hair cells) in the cochlea, which is responsible for much of a person's hearing loss. In reality, the term "nerve deafness" has been around for the last fifty years or so. Until modern medicine, those of us who grew up in the 1950's and beyond are usually given this term if doctors could not find a cause of deafness. In another words, it was a "one name fits all."

I'm beginning to understand that there are several different types of hearing loss. One type is conductive, meaning that the sound comes into the ear but does not get to the cochlea or auditory nerve. That indicates that something is wrong with one or some of the bones in the middle ear. In most cases it is usually the stapes bone which has become stiff, calcified, or hardened and will not move. And when it is not moving, it is not transmitting the sound to the cochlea and auditory nerve. That is what is considered a conductive hearing loss. The cochlea and auditory nerve are usually in fine shape. I noticed on my papers from the doctor and insurance company that I have been diagnosed with sensorineural hearing loss. Which means the sound IS coming into my ear, gets through those three little bones of the middle ear and gets transmitted to the cochlea. But it is not getting to the auditory nerve very well because there is a problem in the cochlea. The cilia (hair cells) in the cochlea are dead or the fluid in the cochlea isn't there or there is something else wrong in that part of the ear. This explains why so many people are labeled with "nerve deafness" because the sounds are REALLY coming into the ear but are not being transmitted like they should to the auditory nerve. That is why I had to do the balance test back in May to determine if I had any fluid in my ear. And I did! This is where the CI comes in. My implant is specially designed to mimic the functions of the human ear like natural hearing. There will be a 22 channel electrode inserted in my cochlea and it is specially designed to protect the delicate walls of the cochlea.

One might ask how a cochlear implant is different from a hearing aid. Hearing aids only amplify sound. No matter how loud the sound the hearing aid produces, it does not provide clarity. That is why I've had so much trouble with amplifiers, speakerphones, loud noises and loud people. I've always tried to explain that "louder" is not always better. I have a severe to profound hearing loss and my ears cannot process the information that they are receiving because sound is still going through the damaged part. A cochlear implant does not make sounds louder. Instead, it bypasses the damaged part of the ear and sends the sound directly to the auditory (hearing) nerve. How cool is that???!!!

I am realizing that some people who qualify for a CI think might that there is no point in pursuing it any further because they have "nerve deafness." But the testing that I've had done has actually shown that I'm missing hair cells in my cochlea. If Mom was still here, I'm sure she would be pleased and tickled pink to know that I haven't given up on the possibility of being able to hear the sounds that have been denied to me all of my life. I have thought about her a lot lately and wished she was here to share this experience with me. I hope to be able to help other people like Susan has helped me. We all need someone to give us that "little push" and hold our hand during the journey. God is so good. He is bigger than any obstacle in my path and knows exactly what I need at the right time. His timing is so perfect!


Anonymous said...

Hey Laurie! I tried to leave a comment and it disappeared. Don't you hate it when it does that? (groan)

I meet so many people in my HLAA chapter or at Fidos For Freedom who have been told they have "nerve deafness" like it's a death sentence for their hearing. So many times it is indeed sensorineural hearing loss and a CI can actually help them!

May I use your post in an upcoming newsletter for HLAA-Frederick County? It comes out again at the end of April. I'd love to share your story with our readers. Let me know!


Laurie said...

Hello Denise,

I have met so many wonderful people "just like me" through my CI and hearing loss journey. Who knew?

Yes, you may share my story and post! I think it is so important to get this information out there.

Take care and be blessed. Laurie

Anonymous said...

Thanks Laurie!

Marines said...

Hello Laurie,

I came across your blog as I was trying to figure out was profound hearing loss really meant. My son, Jacob is hard of hearing and up until today I thought he was mild and moderate bilateral and it turns out it is profound in one ear. I found this out in our first IEP meeting for pre-school... God always knows when we need an encouraging word and your blog was JUST what I needed. Thank you!

coco said...

Hi Laurie,
My big sister was diagnosed as having 'nerve deafness' when she was about 13. The school did a routine hearing test, which they repeated several times as they couldn't beleive that it was possible for a child who was so deaf to have remained undiagnosed for so long. Our parents were not exactly the nurturing types though so it doesn't surprise me. My poor sis, was constantly being yelled at for staring at the teacher when she was being told off, as they thought she was being insolent. She was however trying to work out why she was being told off ! She has gradually lost more hearing as the years have gone by and struggles with her two digital hearing aids. Her balance has always been awful. I wonder if she might be a candidate for CI ? Thanks very much for posting your story, it's certainly worth looking into!

Sharon said...

Hi Laurie =)

I really appreciate your post on this. I was born in 1956 and sometime during infancy had scarlet fever. The doctors told my parents the hearing loss in my right ear was likely caused from that. They did surgery when I was 9yrs old to remove scar tissue. The hope was that it would improve the hearing loss, but it really didn't. My parents were told that the hearing loss that remained was nerve deafness and nothing could be done about it. Several years ago, since I became an adult, I went to a specialist and was told the same thing. According to their tests, I hear some high pitched sounds, but for all intents and purposes, I am deaf in my right ear. Again, I was told nothing could be done, that it was nerve deafness, and the only type of hearing aid that would even work at all for me is the type that you wear on both ears and sound is somehow pulled from the hearing ear (I tested higher than normal hearing in my left ear)and gave you the "feeling" that you were hearing out of both ears, even though, in reality, you were not. Recently we heard about CI and I have also heard about the Baha system. Are you familiar with the Baha system at all? After all of these years of being told there were no options for me, it appears there are finally options! I'm just an appointment away from finding out what my particular options are. Thank you, so much, again. =)