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Re: hearing loss in CMT

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Beata,

Hearing loss/problems are associated with several types of CMT, both dominant

and recessive. I am pleased to learn that you have found something that helps

you.

Gretchen

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Hi Gretchen,

Thanks! The cochlear implant helps to some extent, but I still  have

difficulties. I am also a positive person and a great lip reader. Also, my

hearing was perfect until I was 25, so I finished schools, university and got a

great job before I noticed the hearing loss, but then it got worse fast (but now

it gets better again, as I wrote before!).

The cochlear implant also got back music for me. I could not hear music at all,

it was like noice. I missed music so much, because I used to like it. I also had

to stop playing the harmonica. But with CI I can hear some rythm based music,

usually not the type I liked before, but it is great to be able to hear music

again, and I play the harmonica again too!

Beata 

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I have noticed that my hearing is becoming impaired. I wondered if CMT had

anything to do with it. I have to turn my TV up to almost full volume and my son

always tells me I talk so loud on the phone.

Tamara

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Tamara,

So is mine and family is getting a little disgusted with me.

They say go get an hearing aid, guess they do realize the cost

and that they are not covered by ins. Some days I can hear better then

others,I'm told that it is CMT related.

Geri

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Hi Tamara-

The attached NIH source says that hearing loss is not uncommon to those of us

with CMT disease. (check the fourth paragraph). I would also suggest checking

with your Neurologist and ENT physician.

http://ghr.nlm.nih.gov/condition=charcotmarietoothdisease

-wheels

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> I have noticed that my hearing is becoming impaired. I wondered if CMT had

anything to do with it. I have to turn my TV up to almost full volume and my son

always tells me I talk so loud on the phone.

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> Tamara

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I am moderately deaf in both ears due to Meniere's disease, but I have seen

hearing problems and neuromuscular disease occur more frequently that statistics

expect.

Meniere's disease and neuromuscular diseases cause balance issues far beyond

what either would cause alone.

I have graduated from a single crutch to bilateral this summer as my right leg

went south in June.

The double crutches really help with balance -- and getting seats on buses and

trams. It took a whole year to convince my physical that my rotten balance

wasn't due to just CMT. She was so frustrated that after the strengthening she

achieved on my left leg(I no longer need an AFO) I still looked like a drunken

sailor when walking without a crutch.

Digressing from hearing, here is a link to a animation of the four different

gaits that can be used with two crutches:

http://www.walkeasy.com/interact/crutch_gait.asp

These clips are worth looking if any crutches are in the near future.

I have been using the WalkEasy model 495 for four years, and had a pair sent to

me here in Rome this summer. They are made in Denmark, but impossible to find

here in the EU.

Regards.

Larry

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Tamara,

Tt seems that the evidence is mounting that hearing loss can be a component in

some CMT cases. In my immediate family 3 of us have significant hearing loss and

4 of us (including the 3 with deafness) have balance issues and have been told

at one time or another that we have Menieres disease. I believe that it is more

likely that it is purely CMT and if Menieres is diagnosable it is because it has

the same symptoms, but probably not the same cause.

Menieres is caused by too much fluid in the inner ear, I believe dizziness due

to CMT is more likely to be because of irritation of the cochlear nerve where it

runs through the middle ear -- which also explains the hearing loss. The name of

it actually doesn't matter that much because there is little treatment of

either.

I just like to make sense of things and it makes more sense to me that the known

DNA mutation that I have is likely the cause of most/all of my symptoms and not

that I was so unlucky to be struck by several different things.

My poor sister has needed hearing aids since her mid-30s. I get " stillness

sickness " where I'm fine with motion but keep feeling it after it stops for

days/weeks.

You are not alone!

Holli

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For most of my life I have heard a high pitched whine that other people

claim not to hear. I learned early on not to ask if they could hear it.

Since joining this group, I have gotten the impression it is CMT related.

I now notice that it is worse on my bad CMT days. Anyone else notice this?

O

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Hi O,

 

It sounds like you are describing Tinnitus.  I have this as well, but my husband

does too and he doesn't have CMT.  It's very common in the general population.

 

Most people who experience it have some hearing loss (like in the higher

ranges) and the brain fills in the lost tones with this filler noise. 

 

http://en.wikipedia.org/wiki/Tinnitus

 

 

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Hi Tamara and Geri,

Hearing loss can be CMT-related because the hearing nerve is a peripheral nerve

(just like the affected nerves in our hands and feet). Unfortunatelly, hearing

aids are not good since the nerve itself is involved and not the ear. Making

sounds louder does not mean they will be clearer.

With severe hearing problems, cochlear implants can be used. I got mine through

the Swedish healthcare system for free, but they are extremely expensive and

require a 4 hour surgery to attach electrodes (21 for me) to the nerve. But it

is worth it.

Beata 

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Hi  Geri,

I tried a lot of hearing aids too, but they were no good for me. I went to

tuning for 6 months, trying different programs for the hearing aids, but I could

not get used to them. They only made my tinnitus worse, without helping me

understand speach better. Not to mention the blowing of the wind, which sounded

like a storm or a car driving past sounding like a Jumbo Jet. Sometimes, during

the try-out period, I had to remove my hearing aid to be able to hear what

people said. So I gave up. I did not think that the cochlear implant would be

very good either, since the doctors said that there were not many people with

nerve injuries who had recieved it. Usually, people with hearing problems

located in the ear get the implant. But for me the implant is very good. My

hearing was 25% before, and now 65% (they test how many words out of 100 you can

hear, but you must listen to a 5000 word text which you must repeat word by

word, phew!).

So I must say it is a lot better with the implant. But if your hearing is still

ok, (I guess around 75% or more) then the implant is not an issue. Also good

thing with the implant is that my hearing improves each time they test. I get a

higher score (it is different texts all the time!). So from feeling that I would

get totally deaf before my implant to feeling that I hear better and better

after receiving it - it is really wonderful!

Beata

 

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HI!

I am in the process of trying the newer generation of high frequency behind the

ear hearing aid -- I have CMT 1A -- am 49 yrs old and I have noticed mild

hearing problems the last 3 -4 years.and audiology report supported mildness.

I have a Widex 440. It makes a huge difference -- especially in crowds, etc.

Went from hearing 48% of words with background noise to 96%. They are expensive

but seem great.

SK

> Hi Tamara and Geri,

>

> Hearing loss can be CMT-related because the hearing nerve is a peripheral

nerve (just like the affected nerves in our hands and feet). Unfortunatelly,

hearing aids are not good since the nerve itself is involved and not the ear.

Making sounds louder does not mean they will be clearer.

>

> With severe hearing problems, cochlear implants can be used. I got mine

through the Swedish healthcare system for free, but they are extremely expensive

and require a 4 hour surgery to attach electrodes (21 for me) to the nerve. But

it is worth it.

>

> Beata 

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By definition the Auditory or Vestibulocochlear nerve in not a peripheral nerve

but a cranial nerve. There are 3 sources of nerves defined by where arise --

cranial, spinal, and peripheral.

Cranial nerves (there are 13) arise from the brain itself or the brain stem.

Nerves 1-3 arise from the brain, and nerves 4-13 arise from the brain stem. The

Auditory nerve is number 8.

Spinal nerves arise directly from the spinal column. Each of these nerves is

paired right and left. There are 31 pairs of spinal nerves. Neurological

diseases such as MS and ALs are classed as nerve root diseases as they are

associated with pathology of the one or more than one nerve roots. I maintain

that CMT and MS are analogous diseases as both are demyelinating diseases.

Peripheral nerves are all nerves that don't reside in the central nervous

system. CMT is a peripheral disease. Very simply, out issues are primarily below

the elbow and below the knee. Why? The upper arm, shoulder, and thigh are all

enervated by spinal nerves. The forearm, hand, lower leg and foot are enervated

by peripheral nerves.

Saying that, it doesn't mean that the upper arm, shoulder and thigh are immune

from CMT based issues. They are often involved by the stresses of gait change

and forearm weakness. I believe the major cause of fatigue in CMT is the extra

energy needed by the thigh and pelvic muscles to move what is nearly (sorry

Gretchen, after seven years I still refer to)dead weight.

Having lifted amputated legs during surgical procedure, they are surprisingly

heavy, and as the force of walking requires the thigh to move the lower leg as a

third class lever which in classical physics requires more input energy that

what is output.

Regards.

--Larry

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Thanks for the neurology lesson, Larry. I learned a lot in a few paragraphs

:-)

Joan

On Mon, Nov 2, 2009 at 2:18 PM, Lawrence <shieldlp@...> wrote:

>

>

> By definition the Auditory or Vestibulocochlear nerve in not a peripheral

> nerve but a cranial nerve. There are 3 sources of nerves defined by where

> arise -- cranial, spinal, and peripheral.

>

> Cranial nerves (there are 13) arise from the brain itself or the brain

> stem. Nerves 1-3 arise from the brain, and nerves 4-13 arise from the brain

> stem. The Auditory nerve is number 8.

>

> Spinal nerves arise directly from the spinal column. Each of these nerves

> is paired right and left. There are 31 pairs of spinal nerves. Neurological

> diseases such as MS and ALs are classed as nerve root diseases as they are

> associated with pathology of the one or more than one nerve roots. I

> maintain that CMT and MS are analogous diseases as both are demyelinating

> diseases.

>

> Peripheral nerves are all nerves that don't reside in the central nervous

> system. CMT is a peripheral disease. Very simply, out issues are primarily

> below the elbow and below the knee. Why? The upper arm, shoulder, and thigh

> are all enervated by spinal nerves. The forearm, hand, lower leg and foot

> are enervated by peripheral nerves.

>

> Saying that, it doesn't mean that the upper arm, shoulder and thigh are

> immune from CMT based issues. They are often involved by the stresses of

> gait change and forearm weakness. I believe the major cause of fatigue in

> CMT is the extra energy needed by the thigh and pelvic muscles to move what

> is nearly (sorry Gretchen, after seven years I still refer to)dead weight.

>

> Having lifted amputated legs during surgical procedure, they are

> surprisingly heavy, and as the force of walking requires the thigh to move

> the lower leg as a third class lever which in classical physics requires

> more input energy that what is output.

>

> Regards.

>

> --Larry

>

>

>

--

Joan E. nte LLC

PO Box 3233 Oakton VA 22124

703 855-1048 Fax 800 779-0816

Email joan.lisante@...

www.joanelisante.com

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I am on board with the logic expressed here and agree with what should

be impacted by CMT. There is evidence that other functions can be impacted as

well. What is the explanation for those impacts (hearing, phrenic nerve, ...)

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According to Merck:

 

" The peripheral nervous system refers to parts of the nervous system outside the

brain and spinal cord. It includes the cranial nerves and spinal nerves from

their origin to their end. "

http://www.merck.com/mmpe/sec16/ch223/ch223a.html

 

In other words, the peripheral nervous system is NOT separate from the cranial

and spinal nerves.  The cranial and spinal nerves are the peripheral

nerves.  Anything outside the brain and spinal cord is a peripheral nerve. 

 

Any of the spinal and cranial nerves can be impacted by CMT, or all of them. 

The greater the distance the nerve signals need to travel, the greater the

likelihood of problems, but problems with nerve signals can occur anywhere

within the peripherial nervous symptom, i.e. the auditory nerve, the diaphragm,

the back, the hips, the digestive system, etc.

 

From: Lawrence <shieldlp@...>

Subject: Re: hearing loss in CMT

Date: Monday, November 2, 2009, 10:18 AM

 

By definition the Auditory or Vestibulocochlear nerve in not a peripheral nerve

but a cranial nerve. There are 3 sources of nerves defined by where arise --

cranial, spinal, and peripheral.

Cranial nerves (there are 13) arise from the brain itself or the brain stem.

Nerves 1-3 arise from the brain, and nerves 4-13 arise from the brain stem. The

Auditory nerve is number 8.

Spinal nerves arise directly from the spinal column. Each of these nerves is

paired right and left. There are 31 pairs of spinal nerves. Neurological

diseases such as MS and ALs are classed as nerve root diseases as they are

associated with pathology of the one or more than one nerve roots. I maintain

that CMT and MS are analogous diseases as both are demyelinating diseases.

Peripheral nerves are all nerves that don't reside in the central nervous

system. CMT is a peripheral disease. Very simply, out issues are primarily below

the elbow and below the knee. Why? The upper arm, shoulder, and thigh are all

enervated by spinal nerves. The forearm, hand, lower leg and foot are enervated

by peripheral nerves.

Saying that, it doesn't mean that the upper arm, shoulder and thigh are immune

from CMT based issues. They are often involved by the stresses of gait change

and forearm weakness. I believe the major cause of fatigue in CMT is the extra

energy needed by the thigh and pelvic muscles to move what is nearly (sorry

Gretchen, after seven years I still refer to)dead weight.

Having lifted amputated legs during surgical procedure, they are surprisingly

heavy, and as the force of walking requires the thigh to move the lower leg as a

third class lever which in classical physics requires more input energy that

what is output.

Regards.

--Larry

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What Merck got wrong -- Mark Twain said " Don't believe anything that you read

and only half of what you see " -- were the special defenses given the cranial

and spinal nerves, and denied the peripheral nerves.

The brain and cranial nerves are protected by what is called the Blood-Brain

Barrier(BBB). This barrier is defined the the different permeability of the

capillaries within the brain that prevent (usually) certain drugs, (hopefully)

all bacteria, and (thankfully) most viruses. Without this mechanism we would be

speaking of a brain cold and not a head cold.

The spinal nerves are protected by the Cerebral Spinal Fluid (CSF) that bathes

both the brain and spinal column in a liquid enveloped by a tough membrane

called the dura. This is the fluid that is drawn off by a spinal tap or in the

myelogram. Similar filtration of drugs, bacteria, and viruses occur within the

CSF, but not as efficiently as be BBB as nerves leave the protection of the CSF

to enervate parts ob the body. The parts of the spinal nerves that are protected

are the roots that contain the cell bodies if the nerve.

The protective layer of peripheral nerves is the myelin sheath which members of

this group know breaks down in CMT. Myelin is mainly made up of 20% lipids(fat)

and 80% protein.

In my understanding, the BBB and the CSF protect the cranial and spinal nerves

from the demyelination that affects our peripheral nerves. Logic states that the

farther away from the protective BBB and CSF a nerve travels the greater chance

that some demyelination may occur, but not at the rate of the unprotected

peripheral nerves.

That being said, I have difficulty assigning the causative effect of what

appears to be the high incidence of deafness in CMT patients. I think that the

other factors yet unknown but " catalyzed " by the same imbalances that affect our

peripheral nerves.

The phrenic nerves arise from the 3rd, 4th, and 5th cervical spinal nerves. The

enervation of the diaphragm is almost the distal termination of the phrenic, and

probably more susceptible to demyelination. I find it interesting that cranial

nerves X -- the vagus -- are much longer than the phrenics, but are not affected

due to the stronger protection of the BBB.

Regards.

--Larry

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I play percussion in a community band where one of the other percussionists is a

neurologist.

Last night I asked him if a peripheral nerve disease would effect cranial nerves

and his reply was " Of course. "

He said there are tests that can determine if response times are delayed, a

sharp sound for hearing or a flash of light for sight.

O

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