RE: Question - please respond

[Guest guest]

Anne,

I have had one other patient who experiences "fluctuating" hearing loss, depending on how his mito status is doing. He has been diagnosed as everything from profoundly heraing impaired to mild/moderately heraing impaired...just varies with how his system is doing on any given day. Go figure...this mito stuff can sure be crazy.

ruth

[Guest guest]

Boy can I write a book on this one!

The hearing nerve is VERY energy intense -- it has over 2000

mitochondria per cell as opposed to your arm muscle that has around 500.

The hearing nerve, cranial nerve 8, or CN VIII, runs through a part of

the brain called the Pons which is located at the brain stem. The pons

is the most mito active part of the brain with Cranial Nerves 5,6,7, and

8 running through it.

My son was born profoundly deaf. At seven months of age we had a

cochlear implant put in. This device converts sound into a signal that

the brain can process. After activation, I was disappointed that my son,

who could obviously hear me now, would purposely take off his processor

so he couldn't hear. I soon surmised that he was tired from hearing

because he would take it off about the same time each day and then

proceed to nap. After a year of being able to hear I noticed that

was no longer responding to sounds like he once was. After many test, my

gut told me it wasn't the technology but my son's brain that had been

the culprit. I convinced the doctors to remove the implant and run a MRI

scan of his brain. What we found only confirmed my gut feeling. Cranial

Nerve 8 was gone -- it was no longer sending the signals from the ear to

the Pons for processing. Not only that but the Pons was malformed, a

condition that is progressive. Unfortunately, this particular

malformation has very little research but a doctor in Chicago is

collecting data on brain stem malformation as part of his research on

Joubert's syndrome.

Sue Ann Bube

President, UMDF Indiana

Question - please respond

Okay, I just got back from Asher's 2nd audiological appointment. The

first appointment concerned me b/c Asher showed " dysnchrony " in his

right auditory nerve and the docs were concerned that this indicated

brainstem problems. This time it looked normal - yea!, but left me and

the docs with the question - is this what you see in mito patients? Even

hearing fluctuates depending on " good " days and " bad " days.

Please let me know your experiences.

Anne R - mom to Asher (almost 4 yrs. old! Complex III defect, possible

Complex I defect)

Please contact mito-owner with any problems or

questions.

[Guest guest]

Sue Ann,

This was REALLY informative, although i hate to read about your son's difficulties with his implant. I used to work at the House EAr Institute so CI is of special interest to me. You go, girl...if we all would follow that little "mommy voice" in our brains that tells us something's off, we would be such great advocates for our kiddos.

ruth