Mother had schwannoma removed I Am now having symptoms and have questions?

[Mrs.S]

Hello everyone hope you are well!

My mother was diagnosed with a schwannoma on the 7th and 8th cranial nerve that grew into her cochlear. They did the surgery three months ago and it left her paralyzed on the left side as the nerves bad to be removed because the tumor took over her nerves unfortunately. She is struggling with the new her and still falls randomly as well as struggles eating now that she has no feeling in that side. Just figured I would give some info on her before I explain why I am questioning everything. Me and my mother both have a rare genetic disease called Fabry Disease it's a genetic metabolic disorder. When she was diagnosed with the schwannoma and I was questioning if it was genetic as I had similar issues I was told no she does not have NF2 and nothing has showed on the other side as of yet. Well it's been a few years that I have dealt with tinnitus and the stuffy feeling in my ear that also causes a muffled sound as well as not being able to use phone on that side because I can't really differentiate words well. 3-4 years ago I was told I have some high pitch loss and they will keep an eye on my hearing because Fabry disease can also cause hearing loss but typically a lot later I'm 25 now. So the last 2 years my ear pain and headaches are severe. I was told I'm having cluster headaches and have been treated with several rounds of antibiotics and steroids with no relief. My ear feels like someone is driving a pencil through it I cannot take one more day of this pain or I'm going to lose it which is why I'm here! I'm supposed to be having a brain MRI but all my docs keep dropping the ball or putting it off or can't get me in until September. Just saw an ENT who is having me take 800 mg of ibuprofen three times a day to act as a steroid and set me up for a hearing test in a few weeks... Meantime I feel like punching myself in the ear!!!! My mother says to me you need an MRI that's exactly how I started and I remember it all not to mention the face twitch and tingle that's fun. I wish I could get a dr to order this MRI stat I'm at the end of the rope and I'm contemplating going to the ER but feel it's a waste of time. Any suggestions??

[arizonajack]

If you want to rule out AN with a contrast MRI I suggest you see a medical provider who specializes in AN.

The ANA website has a list of facilities:

https://www.anausa.org/resources/medical-resources/medical-center-directory

And a list of specialists:

https://www.anausa.org/resources/medical-resources/physicians-directory/47-menu-articles/9/418-physician-directory-by-state

If you can't find any on those lists that are convenient, tell us where you are.

[ANGuy]

I do not have NF2.  I asked my ENT if my kids were at risk of having an AN and he said they had no higher risk than the general population.  In other words, for one of my kids to get an AN, it would be like lightning striking twice, pretty much not going to happen.

As far as pain, while many of us have a "full" feeling that can border on being a dull pain from time to time, I have never had unbearable pain like you describe and I don't recall ever reading about any AN sufferers having unbearable ear pain.  To the best of my knowledge, ear pain on the level you are describing is not associated with AN's.

Further, only having high frequency hearing loss is not typical of AN's eaither.  Typical AN hearing loss shows on one side only, with both low and high frequency loss with the middle range relatively normal.  Your graph should look like a mesa, low than a fairly broad and flat middle section dropping off to a low level.

Like AZJack says, an MRI with contrast is really the only way to know for sure.  There are a few reasons why your Dr may not have ordered one.  He/she may know what they are doing and know that your symptoms are not due to an AN, your insurance company probably needs strong justification to authorize an fairly expensive test like an MRI, or your Dr is stupid and you need to find a new one.

[Mrs.S]

Thanks for the tips my mother went to the facility in Ohio I am just waiting for my doctors to call back and order the MRI my genetic doctor wants me to have one but they always take forever to get back with me. As far as the pain my mother was having all of these pains as well and she never got answers and they told her the tumor doesn't typically cause those types of pains but she believe whole heartedly it had caused the bad pain so that's why I was relating to her. With us having Fabr disease we also have a lot of nerve pain as is which I am on medication for. She was told by one doctor that he doesn't believe it's NF2 because she doesn't have them on both sides another doctor said he wasn't ready to rule it out. When she had the surgery they weren't really planning on taking the nerve out they said they truly was not aware how involved it was until they went in. I have met a few other Fabry women who have an AN that were just diagnosed so I guess I'm just confused if Fabry disease somehow makes you higher risk they told my mother there is no correlation because at the time no other Fabry patients were getting the tumors well now there are several that have. There is not a lot of research done on Fabry disease or awareness of the brain other than the white matter. I am going to push for the MRI because I know something is off and nobody seems to find anything based on typical appearance of my ear. My mom had unusual symptoms which is why I know question whether or not having a genetic disease makes me higher risk for an AN. Once I get a MRI I will keep you posted! Thanks for listening.

[ANGuy]

According to this http://www.webmd.com/a-to-z-guides/fabry-disease hearing loss and tinnitus are a symptom of Fabry disease.  The article also says patients with Fabry should get head MRI's, but it doesn't say why.

I don't know enough about Fabry, nothing really, but it seems you should be getting periodic MRI's regardless of the possibility of having AN.

As for her not having NF2, I asked my Dr if I had NF2 and he said "no".  I asked him how he knew and he said at my age, 47 at the time, I would already have had multiple tumors.  While that is not as definitive as gene testing, the guy is no slouch and is a leading researcher of this stuff at Duke.  His answer was a bit cocky, but I trust him and think that it is a black and white type issue, if you are middle age and only have had one tumor, you are negative for NF2.

Again, none of this is medical advice, I didn't even graduate college.  I just think you should get an MRI and not get ahead of yourself in the meantime.  What you can do is learn more about Fabry from more detailed sources than webmd.  By knowing more about Fabry, you would be in a better position to demand an MRI and to assess whether your current Dr is the right one for you.

[Cheryl R]

The older onset of NF2 does happen.     I was 49 at time of my first AN and the one on the other side didn't show up till 2 yrs later.     I was able to wait 3 yrs before surgery on that side.     Before then a facial neuroma also grew suddenly and had surgery for that so ended up with 3 tumors.      It has been 7 yrs since last surgery so hope that it is it for any more ever.   I am 63 today.      There was no prior ANs in the family that know of.                Cheryl R

[Debbie N.]

Who was your doctor at Duke?  I just had my surgery there this past January. 

[ANGuy]

David Kaylie at Duke.  I am WW, so I can't give any feedback on surgery, but I am happy with every aspect of the interactions I have had so far.