One large unilateral tumor- later the other one appears? Am I Nf2? Help.

[4cm in Pacific Northwest]

I am up here in the wee hours of the morning sort of freaking out. (OK OK really freaking out  )

I had one atypical unilateral XXL acoustic neuroma tumor removed 2 years ago. Prior to that surgery I had 100% word recognition plus it was such a weird shape that some neurotologists were NOT convinced it was actually an acoustic neuroma in the MRI ( before the surgery). So nothing in my acoustic neuroma case has been typical.

All hearing was sacrificed in the AN side so I am SSD now. Since I woke up from the surgery I have had terrible hyperacusis in the “good” ear. It never went away. I tried the various audio-desensitizing tapes, reducing earplug use etc- nada. In my support group meetings I am THE ONLY one who has to always wear an earplug in the good ear. This seemed to be the case when I attended the recent Chicago symposium also.
“Why did I get the hyperacusis, in the good ear, so much worse than the rest of them?”  I often ask. 
The hyperacusis has not improved and some days I think it is gradually gotten worse.


I made a remarkable “balance” recovery from that surgery- (my face I cannot brag about since I have synkenisis.) Over this summer however I am noticing a bizarre but subtle regression in my balance. I have been getting periodic tinnitus in the “good” ear. This past month I have had some communication break downs where I have heard incorrectly- on the phone in my good ear (Similar to what I experienced in my old phone ear that is now 100% deaf- before diagnoses of the AN- as hearing on higher frequencies fastly reduced).

This morning I woke up with a full ear sensation in my GOOD ear. The hearing is muffled and the tinnitus (which is pulsating  ) seems constant – however at a different frequency than the other ear that had the acoustic neuroma removed. You would think with the muffled hearing the hyperacusis would be reduced  - NADA. No matter how many times I yawn- this will not go away. I do not have a cold or sinus issues at the moment (although I did at the beginning of August)

I DO have a family history of unilateral deafness. The reasons never known as there were no MRI’s in those days. My father has unilateral hearing issues, balance issues and some facial weakness. Both he and my sibling have constant tinnitus. He thinks the balance problems is due to the 2 hip replacement and the other symptoms are just due to age.   (I have written to him- yet his ENT sees no need to do an MRI …and he is Canada) My youngest child has had on going ENT issues and too is showing signs of hyperacusis. (I won’t even start in her early childhood radiation exposure – with infant chest x- rays etc) When there is deep bass music in her dance class I see she is not maintaining her balance as the others in her class are. (I too had this issue in my youth) She is also showing facial asymmetry in her growth with her nose (as I have). She is just age  10 ½.

As far as I know, because my tumor was huge and unilateral, that an NF2 pathology was NOT performed on the dissected tumor.  I do not  think NF2 was even considered a possibility in my case.

Quite frankly I feel I am showing signs now of an acoustic neuroma in the GOOD ear. I have woken up very deaf this morning (although not completely)

Freaking out here- not wanting to mention anything to my family until I get the follow up MRI results and talk with a neurtologist.  (The MRI order is overdue with my Dr- as I wait for them to get their forms in.)

Did any of you NF2 folks ever have just one large tumor first- and then later the other one appeared? Or do these typically both appear at the same time at the same rate of growth? (Not that anything about my case has ever been “typical”)

I guess I am needing an ANA support group hug… as I sit here at my laptop mulling over the possibilities of the recent symptoms: new “balance regression”, new “tinnitus” in the good ear, new “sense of fullness” in the GOOD ear. (the quoted words I am all too familiar hearing- on this forum.)

DHM

[lholl36233]

Sending you a big hug from Millbury, MA.

[Cheryl R]

DHM,     How scary for you.  My first surgery was in 2001 and not a big one at all but in the IAC.     2003 is when the one showed up on the other side.    Very small and just kept watch till 2008 when it was 1.4 and had the surgery as was near deaf with it.    In the IAC also.  Not much in way of symptoms for quite a while and then think was tinnitis and maybe some balance and the slow loss of hearing.                   I have had a bit of hyperacusis at times. Not near what you have.   Why you do is unusual but this whole AN business is unusual for many.                   I was 49 with my first surgery.                I have no family history I think.         I had old parents and my dad had some possible with hearing problems but he was old,diabetic with little care like now and some what I think was TIAs.    Also both legs off and in a wc.     He died when I was 21.
   We had a guy as a patient when I was working who had severe hyperaccusis and the nurses thought he had psychological issues.           I am not so sure as had an idea of how it might be bothering him as was when I had had the first AN.   Everything had to be done a certain way for him and I understood but no one one else did.  
All I can say is the next MRI will show if there is one  and make sure it is a good one to show the IAC well.     I so hope you do not have NF2 and there is a chance it is not.                                   I keep watch on my daughters and granddaughters balance and know my daughters think I am nuts for some of how I get nervous over potential for them.                  
                                         Big hug and wish I had the answer for you!              Cheryl R          

[jerseygirl]

Hi, DHM,

Hugs, hugs and more hugs! I would not worry just yet: worsened balance and increased tinnitus or tinnitus in a good ear 2 year after surgery given your tumor size is actually normal. Your balance and tinnitus will fluctuate years after surgery and that by itself does not mean there is a tumor. You would be surprised at the everyday, common things that can affect these, in fact, ANY change will. That means change in sleep, both time and quality, change in exercise habits (a different routine or interruption of a current one for any reason, even for a day). After my first surgery 21 years ago, I cannot tell you how many times I got worse before I got better but my MRIs were stable. The weird thing is that I finally felt normal only after the tumor started growing again. Go figure! I would get that overdue MRI to put your mind at ease but remind yourself that the chance of having another tumor is slim.

I was counseled in a Neurofibromatosis clinic and here is what I was told. Even if you have bilateral ANs at the time of diagnosis, they can be (and frequently are) of  different size. That means one tumor either appeared later and/or grows slower. If you have unilateral AN and are destined to have another, most of them will appear within the first 7 years of the removal of the first. THe risk increases with young age (being in one's twenties) and being female (estrogen, pregnancy  and HRT). After 7 years are passed, the risk is progressively smaller overall but there are a couple of people on this board who developed tumors after 20 years, so the risk is always there and cannot be excluded. This is one reason to have periodic MRIs for the rest of your life.

As far as NF2 mutations in the tumor, the problem with testing for these is that many, if not most ANs, unilateral or bilateral have them. The difference is where in the body these mutations exist, which is not obvious. Unilateral ANs contain them only within the tumor tissue. NF2 somatic mosaics contain NF2 mutations in some parts of the body but not all. Full blown NF2 case contain NF2 mutations everywhere, including blood, so they are easily found. Since tumor testing after removal is not common and there is no statistics, it is hard to say what all AN mutations are but I read a study where they found NF2 mutations in unilateral ANs. 

As I said, I would not worry just yet. Get an MRI and chances are that it will show absolutely nothing. Keep us posted and best of luck!

                  Eve

[jerseygirl]

Hi DHM,

I was rereading your post and realized that I completely missed that your hearing in a good ear is significantly worse. That changes things a bit but not in terms of NF2. Unfortunately, having AN removed on one side does not preclude you from having other "common" maladies like ear wax, ear infections and the like. It is still a greater possibility than another AN. You need to preserve as much functional hearing as you have, so act fast here. Call an ENT and demand to be seen as soon as possible, otherwise your remaining hearing can be lost forever. If seeing an ENT soon is not a possibility either because he/she is not available or it is weekend, go to an emergency room and explain your situation. THey might even do an MRI on an emergency basis so by the end of today you will know where you stand. I understand that it is hard to advocate for yourself so take somebody with you. Good luck and keep us posted.   


                Eve

[marie]

My original AN (left side) was diagnosed and removed in December, 1967 

In 1993 an AN was discovered on the right side.

Dr. Brackmann said it was probably NF2  but I have never been tested for same.
Marie

[moe]

DHM,
I am not NF2 (in fact I had to google it, because I'm uncertain as to what it means), but am interested in your new symptoms.
You have every reason to be freaking out, but still try to take some deep breaths, meditate, and just get the MRI asap.
If it does show something that proves that you have NF2, you can always get your daughter tested with a blood test. And you will be on top of whatever it is....
I can only imagine those feelings of "OMG now what?" You've been through so much and sounds like you are doing sooo great, from the symposium.
Hopefully it is something that is not as severe as you fear it is.
Hang in there, just take one day at a time, until it is "figured out."
Prayers going your way.
Maureen

[Brendalu]

DHM
 I am so sorry you are having to go through this.  I , too, had a fairly large AN, lost my hearing in my right side and never regained my balance. I had my routine MRI's and filed for disability.  I think it was about a year ago I got my MRI's out, all of them and hung them for my windows to compare.  At first I thought I had them backwards.  I followed the progression and discovered a multitude of sin.  I made a quick call to my PCP and she got me in that day.  I hauled all of my films with me.  After confirming what I was seeing, she told me that she thought the ENT and the neurosurgeon had already discussed it with me and since it isn't her expertise and I hadn't asked about it, she felt that I had decided what i was going to do and was doing it!  I went to the ENT and the neurosurgeon for explanations and was told by both that because the tumor on the left side was so small and because I would be having MRI's annually they could watch it without terrorizing me.  Needless to say I don't see either doctor any longer.  Fortunately, I haven't lost my hearing in my "good" ear, my balance hasn't been good in so long I don't know what good balance is, so I can't tell if it is getting worse.  I am learning to sign.  I am very sensitive to loud noise. 
I am keeping you in my prayers and sending many good thoughts your way.
Hugs,
Brenda

[mimoore]

Oh DMH here is a big cyber hug for you <squeeze>.
I do not have NF2 but certainly want to be here for you, please keep me posted.
Knowledge is power. You are a strong woman and you will get through this. When is the MRI scheduled?
Take a deep breath, a hot bath and enjoy a glass of wine.
We are all here for you!
Michelle

[4cm in Pacific Northwest]

When is the MRI scheduled?
Take a deep breath, a hot bath and enjoy a glass of wine.

Thanks. The ongoing Saga of the MRI.... My doctor and I e-mailed about this in May ... Then he delegated the paper work to an assistant ....(He delegates my case often  ) ...Somehow  they lost me in the paperwork and sent the MRI paperwork to the wrong place thinking I would fly back to CA when I wanted to do it in OR... sooh I am still playing phone and e-mail tag... here we are almost September.

I wish I could enjoy wine. When the facial nerve and taste buds came back- my taste for apples and grapes did not. Sadly I live right Pinot Noir wine country and can no longer stand the taste of the stuff- so lattes for me it is. (Cheers  )

So yesterday I decided to have a "MONKs day" at home and requested a day of silence so I could go without earplug. I also only had 6 oz of coffee. Last night I slept over 10 hours... this morning I can hear (Hyperacsus remains) and the high pitch tinnitus in The good ear stopped. Divine Intervention? Coincidence? Removal of chemical and audio stimulants plus some sleep? I also think I may have had too much pressure in the ear by wearing an earplug too much (overuse of)

SO today I am better ... but this has come and gone before - as it did with the AN side -before the tumor was detected in 2007.

I will call an ENT and audiologist in the morning for a testing appointment and exam. Plus hound my "Lead" doctor, my neurotologist in CA, who I actually quite like, but I will admit he has too much on his plate... It drives me crazy when his staff loose me amidst the students, the interns, and all the other zillion people he is responsible for... as: doctor, professor, department chair, associate dean, Dad of 4  teenagers...I understand why he has to delegate ... but it drives me crazy. 

It scared the cajeebies out of me when I could not hear on Saturday morning... even though this has improved ... It is a reminder how we must take care of the one ear we may have left. I think I will follow Brendulu and get registered for signing classes.

My little one I will continue to observe as she has ENT issues...

Thanks folks- today I feel a little better. (Your understanding helps)

I will post once I get the results of MRI, audiology exam...

I do not want another AN tuma... but this is always a possibility for us- isn't it?

DHM

[Jackie]

Say, DHM,

I'd be interested in learning to sign also! Maybe we could do that together??? Let me know what you think.
Jackie

[moe]

DHM,
I'm glad to see that you feel a wee bit better. Amazing what rest relaxation, sleep, and decrease noise can do to help you feel better.
Like Michelle said, you are a strong, well informed, educated woman and are on top of things! So let us know how the appts go.
How frustrating for  you to have to be playing doctor tag, though. I can empathize there.
I had brain surgery at Madigan in Tacoma, follow up eye surgeries in San Antonio, and nerve surgery at UW in Seattle! My records are spewed around everywhere-hopefully consolidated in the military record, but I should probably check it out You got me thinking on that one......
All I have is my original MRI copy.
Life in the military........
Hang in there-Maureen

[ppearl214]

Oh, my dazy hazy mazy!!!!!!!!!!!!!      

You and I and others know that the only true way to know is via MRI with contrast... and I see you are doing what you can on that end.

Lattes work... so, does a good glass of Merlot?  Shiraz? 

So, other than lattes or harrassment from me.. how about this?

HUGGLEZZZZZZZZZZZ!!!!!!!!!!      
will that work for now?

xoxoxo
Pearly Whites

[yardtick]

Thinking of you 

[Tumbleweed]

Wishing you the best, DHM. I hope everything turns out okay for you. Please keep us posted on your test results.

Best wishes,
TW