Post from Hobb/ Information

[Guest guest]

About the inner ear and RP. An Otoneurologist I saw (Dr. Shaw) had never

had an RP or polyarteritis (Vasculitis) patient before and he was puzzled by

loss of hearing and loss of vestibular function (dizzyness) because there is

no cartilage in the inner ear.

However, there are some tissues in the inner ear that contain Collagen II and

Collagen IX and those are the components of cartilage that RP attacks.

Cartilage simply has more of those components than some other tissues and

that is why cartilage is the main tissue that is affected by RP. Tell your

doctor that RP attacks Collagen II and Collagen IX rather than saying it

attacks cartilage.

One of the things RP can cause is calcification of the sinuses. I am trying

to learn if it can cause calcification of any of the inner ear mechanisms,

too.

The nerves that go to or through the same channel near the middle ear are the

nerve for vestibular function and the one for facial muscles. If anything

happens to narrow that channel (inflammation?) it can put pressure on one or

both of those nerves, imparing function. Some RP patients will have half of

their face suddenly droop (like a stroke) when there is a flare. Some will

be dizzy at the same time. Some will be dizzy but the face is unaffected

(me). In most cases the facial droop goes away as soon as the flare is

controlled. It is an uncontrolled flare that can cause permanent damage to

facial (and vestibular) nerves.

Whatever is the cause of dizzyness -- inflammation in the middle ear or

inflammation affecting the vestibular nerve (or both) -- RP can cause a

complete loss of vestibular function on one or both sides. In my case a

test for vestibular function showed I had lost about 28% of it on the right

side when diagnosed. The same test done about 18 months later showed that

the loss of function had increased to almost 80%. About that same time the

sensation of dizzyness started to go away.

When vasculitis accompanies RP the small blood vessels feeding nerves can be

affected. Sometimes they are destroyed. Nerves do not get the nourishment

they need and neuropathy or even nerve death can result if the lack of blood

is serious enough and prolonged enough. Inflammation of tissues surrounding

critical blood supply to nerves can also choke of blood vessels.

I don't know if you can call the loss of vestibular function because the

vestibular nerve is " starved to death " a kind of neuropathy.

Dr. explained that when something damages vestibular function, the

worse it gets the less dizzy response is left. If you lose all vestibular

function you will never feel dizzy. She said that if healing would cause

vestibular function to improve I would probably have more bouts of dizzyness.

In the case of RP, when the flare has been uncontrolled for long enough the

healing process ends in scarring -- damage that cannot repair itself with

longer healing in most cases. If you can control the flare quickly the

chance of permanent damage is much reduced. High dose (at least 20 mg)

prednisone is required for an ordinary flare. A flare with multiple symptoms

or one that does not respond quickly may need more. If the pain and

inflammation does not respond to 20 mg within a few days an increased dose

may be required to bring it under control.

Deafness is also caused by RP in spite of the " absence of cartilage " in the

inner ear.

Some researchers believe it is often accompanied by other vasculitis that may

be undiagnosed and that can be the actual cause of deafness - causing damage

to the auditory nerve. I don't know if anyone has done any research to

determine exactly what happens in RP deafness -- mechanical or neurological

damage (auditory nerve neuropathy?) or both.

One patient on the list said that he lost all his hearing and all vestibular

function. A cochlear implant makes it possible for him to hear OK. I

believe that requires some auditory nerve function.

List members include others who are completely deaf and one of those believes

he is deaf because his doctor was too conservative in medicating him and let

him continue to flare without increasing his meds. His doctor told me he

thought I was dangerously overmedicated but Dr. West at Denver Univ. Hospital

(who wrote the textbook about vasculitis) is the one who set up my schedule

and I decided to trust his judgement. He is not experimenting to see how

little medication he can use to control a flare with an " oops! " when damage

occurs. He believes in using whatever it takes for quick and certain control

when symptoms are serious. He takes a more conservative approach when

symptoms are not threatening organ destruction.

It is always a good idea to talk over a doctor's treatment philosophy with

him and to discuss when you think it is working or not working. Some

patients respond well to conservative treatment while others need more

aggressive medication.

I believe my kidneys are intact today because of my Rheumy's aggressive

treatment. At one point I was passing 1000 (units) of protein a day -

indicating kidney damage or malfunction - and now that is down to " trace

amounts " . I asked my Rheumy if she thinks the decrease is because the

damaged areas of kidney no longer work at all or because they have healed.

She said there is no way to know but she prefers to believe the kidneys

healed.

I had lost 20% of my hearing on the right when first diagnosed. The last

hearing test at the same time as the balance test that showed almost

complete loss of function on the right side, it had not changed. I get

another hearing test soon. A side effect of the Cytoxan I take is hearing

damage in some patients.

Baseline testing for hearing and balance (vestibular function) are important

so that follow up testing will have something for comparison.

If your Rheumy orders an MRI for inner ear, make sure he/she knows that an

otoneurologist will need side views in very small slices. An otoneurologist

will also look for clusters of small lesions in the brain that are in an area

that affects vestibular function or hearing.