Anyone have malformed cochleas?

W

wizardofozzz

New Member
Joined
Sep 17, 2010
Messages
6
Reaction score
0
Does anyone have malformed cochleas without enlarged vestibular aqueducts? This type of malformation is often called, Mondini dysplasia, cochlear dysplasia, or Mondini-type cochlear malformation.

I would love to know the following:

1. What does your audiogram look like?
2. Has your hearing loss been progressive?

I have mondini-like cochlear malformations (2 turns rather than 2.75) with EVA's, which has resulted in a profound bilateral reverse slope/reverse cookie bite hearing loss that is progressive. My also has the same cochlear malformation but without the EVA (or LVAS). They tell me that his mild hearing loss will be progressive, but I'd like to find others who also have malformed cochleas and see what their experience has been. My experience doesn't really count since I think the LVAS accounts for a most of my hearing loss.

Thanks!!




but my son only has the mondini-like cochlear malformations. Our cochleas do 2 turns rather than two and three quarter turns. My hearing loss is profound but I assume most of that is due to my EVA's. So, far my son seems to have a mild hearing loss. They tell me it will be progressive, but I'd like to hear from others who actually have this type of cochlear malformation.
 
Mine are not malformed, they are just gone, like my ear drums.
 
I am missing "hair cells and I hearing test look like a slope ,it drop way down.
 
How do they know you have malformed cochleas? As opposed to missing or damaged hair cells, the cause of most people's hearing loss?
 
That's a midline defect. There are some hearing loss syndromes that are associated with midline defects. You may want to have an expert check your eyes, kidneys, etc. I don't know where you are but you want to look for specialists who are familiar with hearing loss syndromes. PM me if you want more info. Welcome to AD! :wave:
 
HHIssues said:
How do they know you have malformed cochleas? As opposed to missing or damaged hair cells, the cause of most people's hearing loss?
Click to expand...

By your hearing test and how you speak ? ? ? My daughter was bit by a dog as child and the doctor that did the plastic surgery had me go see him. He thought I had malformed mouth because of the my speech. The doctor was shocked that my mouth was fine , he could not believe I had no help with my speech. He said there was no reason for me talk like that .
 
The original poster probably had an MRI. That's really to only way for drs to confirm a malformation. I don't think that the dr can tell from a CT.
 
I'm the original poster. Yes, I have an MRI that shows both LVAS and Mondini. My son has an MRI and CT that show Mondini malformation. It's been a while since my original post and my son's hearing has declined to severe in the low frequencies rising to normal in the high frequencies. Still interested in anyone with just a Mondini-like malformation and how your hearing loss/deafness has progressed.
 
Have no clue what my deafness is caused by I never bothered to ask why. Interesting thread.

I just know im so deaf beyond help of hearing aids.
 
Back
Top