newly diagnosed with 2mm on L, what are odds of developing one on R

[75 bronco]

i am a 46 yr old male. the reason i ask is AN is scary enough but NF2 adds another level of fear. what are the odds of me developing a r sided AN or having one missed on the first MRI. thanks

[Jim Scott]

i am a 46 yr old male. the reason i ask is AN is scary enough but NF2 adds another level of fear. what are the odds of me developing a r sided AN or having one missed on the first MRI. thanks.

Very small.

NF2 is basically genetic.  If there is no family history of this condition, you're very likely 'safe'. 

I don't have a precise number but I would guess that having an AN on both sides is relatively rare, but not entirely unknown.  Unfortunately, one of our current posters has been diagnosed with that condition.  Still, the MRI doesn't miss much, unless the AN is ridiculously tiny or the doctor reading it is incompetent.  I wouldn't allow myself to become too anxious over these very slim possibilities.  I would recommend that you concentrate on dealing with what you have, not things you don't have. 

Jim

[tony]

The exact no sits between one in 40,000 and one in 25,000
of the "general" population (depending the research papers)
- so it is a rare condition
I think it is unlikely - but annual check ups for the next
5-10 years - may well put your mind at rest
best regards
Tony

[Cheryl R]

I have read that only 5% of AN's are NF2.      So your odds are 95% that you are not.    Rarely some of us do end up being in that unlucky 5%.                I was one of those.     My right sided AN was in 2001 and the left sided never showed up until 2003.           
Just keep watch.    It makes the whole AN experience just a little more challenging!         Don't worry ahead about it.     You deal with it if it ever does happen. 
                                                   Cheryl R
                                     

[75 bronco]

thanks for the replies. There is no family history, yet i am a worrier and a planner. i like to have all possible info when visiting the dr and look at my future. thanks again

[Kate B]

Bronco,

NF2 is rare and hereditary. NF2 inolves an abnormal chromosome 22. AN's do not.

Here are two links: 
http://www.hei.org/news/facts/nf2fact.htm

" Neurofibromatosis Type II (NF2) is a genetic disease (autosomal dominant) of the nervous system characterized by tumors (schwannomas) growing on both vestibular nerves that adversely affect the auditory and facial nerves. This disease occurs in approximately one in 40,000 live births in the U.S. Profound hearing loss and some facial paralysis result from the growth of bilateral tumors on these nerves. Because of the location of the tumors, their removal typically necessitates severing the auditory nerve."

http://www.emedicine.com/radio/topic475.htm

"Although it shares a name with neurofibromatosis type 1 (NF1; von Recklinghausen disease), the 2 diseases are separate entities. Neurofibromas are rarely found in NF2, which has led some to propose that the disease be termed schwannomatosis or MISME syndrome, the acronym for the following terms:


Multiple inherited schwannomas

Meningiomas

Ependymomas
The term MISME is becoming used widely as a mnemonic to remember the disease.

Although a presumed case of NF2 was first described in 1882 by Wishart, the disease was not separated from von Recklinghausen disease until 1987, when it was demonstrated that the 2 disorders arose from different chromosomes. The genetic defect responsible for NF2 is a deletion of a portion of chromosome 22, the same chromosomal abnormality found in spontaneous spinal schwannomas, indicating that a single location causes Schwann cell tumor growth."

When is your doctor appt? Choosing the right doctor  and treatment is paramount right now?  Your 2mm is very small.  What were your presenting symptoms?

Kindest Regards,
Kate

[jerseygirl]

Bronco,

I am not sure what precisely the odds are but they are very small, in single digits. However, many bilateral ANs grow at different speeds and because yours is so small, it is possible (I would like to emphasize that the chances of it are slim) that another one willl show up on the other side. I was watched by Neurofibromatosis clinic since 1988 (that's when I had my first AN surgery) and was told that overwhelming majority of ANs on the other side show up within the next few years, usually not more that 7. Unfortunately, we have posters that had another AN show up 20+ years later but that is exceedingly rare. So, keep having those MRIs!

My understanding of the situation is that all ANs, even sporadic, involve chromosome 22. In sporadic AN the genetic defect is confined to the tumor cells. In NF2 the same genetic defect is present in ALL cells in the body and you have 50% of passing it to your children. In NF2 somatic mosaic, the defect is in some cells but not the others, making it more difficult to pass the condition to the children but also making it more difficult to estimate the chances of your passing it to your children. Having said that, even NF2 is a very variable conditon and nobody can predict with any certainty if, when and where a person with NF2 will develop the tumors. The medical knowledge is not there yet.

One of the biggest problems with bilateral ANs is complete deafness eventually rendering people in their prime years unable to work and have a social life. Single sided deafness is bad enough but in my experience it stops me from very few things that requre bilateral hearing. I really advise all newbies with small tumors to save their hearing on that side because you simply don't know what is going to happen to you in the future, be it another AN, age-related hearing loss or something else that might make you completely deaf. It is possible now ( but not guaranteed) for small tumors and serviceable hearing. Make sure you doctor knows it is your priority #1 and shop around for the best doctor to save your hearing. It is absolutely worth a try.

Another thing to realise is that medicine goes at a very fast pace. There is now a genetic test for NF2 (not 100% accurate, unfortunately), MRIs with contrast which means more tumors are found when they are small and surgical innovations which means smaller incisions, less bleeding, very possible facial nerve preservation, possible hearing nerve preservation if the tumor is small, etc. Every five years the medicine makes a big leap forward. So, if you wait and watch for those five years and your hearing is still serviceable, chances are that doctors will be able to save it. On the other hand, if you choose to do something soon, it is still possible to save it.

Best of luck to you and please keep us posted!

               Eve

[jerseygirl]

I should really add that 50% of all NF2s are spontaneous mutations meaning that there is no family history of the disease.

               Eve

[ppearl214]

Hi Bronco.... and welcome.

Wikipedia also notes regarding NF2 and the correllation of chromosome 22... this link may also help, besides what folks here have shared.

http://en.wikipedia.org/wiki/Neurofibromatosis_type_2

Again, welcome.
Phyl

[jerseygirl]

What a great article, Phyl! Thank you very much! I certainly learned from it. I wonder who wrote it for the Wikpedia. It talks about genetics of NF2 as well as endoscopic techniques for AN removal. Some surgeons don't even want to admit they exist! I wish endoscopic version existed in NYC, was covered by my insuracnce and I did not have to travel to California to get it.

                                     Eve

[75 bronco]

thank you to everyone for the replys on a Sunday afternoon! My symptoms are tunnitis and pressure. adiogram indicates mild hearing loss. yes, i am scheduled to see Dr. Mitchell Schaber in a few weeks for a long term plan. he is in nashville and seems to be who most seehere in Tn. does anyone have any feedback on him and his ideas? Bill

[sgerrard]

Bill, is your AN really 2mm? Small doesn't mean no symptoms, but it does mean you should have lots of options, including watch and wait.

I took a look at that article on NF2, it did seem pretty good. The discussion tab says that the article is a translation from the German Wikipedia, and has a link to the German version. So Eve, if you can read German, maybe you can figure out the author there.

I have to take exception to this sentence, though: "In NF II Acoustic neuromas usually affect young people, whereas, in sporadic forms of Acoustic neuromas, the appearance of the tumour is limited to the elderly."

Since most of us are in the "sporadic form" category, I don't think it is fair to characterize us all as "elderly". 

Steve

[Kaybo]

Bill~
Hi!  Lori67 lives in Nashville (I think) & would be a GREAT one to talk to!!
K

[jerseygirl]

Steve,

I already figured out who wrote the article. If you click on tab "discusssion", the author is andy.we. By clicking on this name, I found out that the author is Andreas Werle, he is a German trained doctor. I wonder now if endoscopic An removal is more accepted in Europe and who does it.

I agree with you, Steve: we are all young people no matter how old!

             Eve

[75 bronco]

Eve- what procedures are available now to remove a tumore of this size and save hearing?
Sgerrard- yep, i looked at the mri report. it says " a tiny area of enhancement of 2mm in the left internal auditory canal",  "nothing abnormal on the r side".
thanks again everybody.