Already a member? Sign in
What - Inspire is a place where you can connect with people who share your health concerns and find information and advice in groups sponsored by organizations you know and trust.
Why - As a member you can use Inspire to let friends and family know how you're doing, contact others who share your health concerns, receive personalized updates and information about participating in surveys and clinical trials, and more.
How - Joining Inspire is completely free and usually takes less than a minute. Join now!
I have a daughter recently diagnosed with NF2. Currently she is asymptomatic but her bilateral tumors are 2.0 and 2.2. My question is to anyone out there, would you reccomend surgery or gamma knife? Has anyone tried to reduce the size of the tumors before surgery?
Dizziness Surgery Cochlear implant Vertigo Weakness Valium Schwannomatosis Stress
Hello ttg
I'm suprised no one answered your questions in March - sorry I must have missed your post. I will tell you of my experience and hope it helps in your decision. I presented with a neuroma on my left acoustic nerve which was 2.2cm in the cisternal component - the part growing out the back of the skull, pressing into the brainstem - my only symptom was a tingling on my tongue and loss of taste. My hearing was normal which is not usual. I researched and decided to go with surgery to preserve my hearing but my surgeons told me this was not an option - translab surgery was the option to preserve my facial nerve which they did but I lost my hearing on that side. However, I felt that I had been pressured into a decision for the convenience of the surgeons. At my 2 year MRI I now have a shadow on my right acoustic nerve - my only hearing ear.
I would advise you to research very thoroughly and only make a decision when you are sure it is the best option with what you know - go to another surgeon if you feel you are not getting all the info or have any doubts.
I hope things are working out well for your daughter.
Santel- What other options do you think you might have had?
Thank you for your reply.
Tracy G.
Hi ttg;
Seems this was posted by you before I was a member here.
Re, the AN sizes, a Mid Fossa surgical procedure may be indicated if there is hearing to spare.
However, both ANs are in the range for GK. This is good, but GK has not proven to be as much a success in NF-2 pt. as those with a spontaneous, unilateral, one time, AN tumor.
Re, 'debulking', at the point your daughter is at, this making the tumors smaller by removing part and then irradiating, should not be necessary given the tumor sizes.
Would say you wan't to get these before there is hearing ( auditory nerve ) involvement as NF-2 related ANs have the tendency to mesh with and entangle the nerves, and actually grow inside them!
Re, "Middle Fossa surgery, you want someone well experienced in this particular form of surgery because it is a bit technical as compared to Translab or Sub Occiptital.
Best wishes always for your daughter's future with NF-2!
Russ
I have asked about radiation for this 'shadow' if it decides to be an neuroma - in W Australia we don't have a lot of choice with specialists - my ENT said 'his' radiation team wouldn't touch it, but I emailed a radiation professor in Sydney and his reply was that because it is my only hearing ear, he would be worried about the side affects and hearing loss sooner rather than waiting until that occurs naturally. So you see, a maybe wrong 1st choice leaves me with few alternatives now. The opinion I keep getting is 'you are going to lose your hearing with this thing eventually, so just let it happen and then we will try to save your facial nerve function' - I am 53 so it is not such a big deal for me and they are probably right but I wish I was more sure about the 1st decision.
Russcape has a lot of info on radiation and surgical tx - he lives with NF2 and does heaps of research. I would advise you to go with your gut feelings if you are not sure about anything. And you need to be confident about your Drs.
All the best.
Hi;
Re, radiation of an only hearing ear, as I understand things, sometimes the cochlear ciliary hair is damaged by radiation absorption.
Also, in some occassions, the radiation causes tissue to swell, thus blocking arterial blood flow to the cochlea. Another potential hit to the cochlea.
One might think, all is well because a cochlear implant can still be installed. Not necessarily so because the tumor intertwines nerves that area and can actually grow within them if one has NF-2. A Cochlear Implant requires a half way adequate auditory nerve to function well.
So; There's a dilemma here as one is still watch and wait following radiation, or, there may be progressive damage. Surgery can cause immediate damage to ANs interlaced between nerves also.
I believe given time, the usual outcome for NF-2 ( at this point in medical science ) is bilateral deafness often attenuated by an Auditory Brain Stem Implant.
I hope this sheds some light on decision making re, an only hearing ear. After a point, it seems prudent to leave well enough alone unless the AN is dangerously close to the brainstem or is affecting the facial nerve. One may keep remnant of their natural hearing for a while; Maybe a long while. This is where I'm at myself.
Russ
Russ - I knew the basics of that, but not why, so there, you have filled in the unknown for me - thanks.
I have one good ear at the moment so hope that stays the same for a long long time.
I am troubled the last couple of weeks by a pulling on my left eyelids - not like a tic, but pulling hard enough to make my vision jump - I can't read or use the computer when it happens - it may be abberant nerve growth from my previous surgery ? after 2.5 years - I hope so because the other option is tumor regrowth on my facial nerve. Only 4 weeks til my MRI.
Hi Santel;
I guess the next MRI will tell the tale re, regrowth or not. Let's hope not!!
I hope the weekend is nice for you in some way : )
Russ
Hello Russ- Thank you for your reply. Currently, the only symptoms my daughter is having is some balance in the dark. Otherwise, no hearing loss. What is your story with NF2? We know several young people who are currently in the PTC299 trial study here in the States. They have had really good results. Significant tumor shrinkage. This may be an option for my daughter to try to postpone hearing loss. We are being recommended to NOT use radiation of any kind. Radiation can cause scaring, nerves to meld together, etc. So, we can't go that route. I will ask about the midfossa approach. Thank you for your reply. I really appreciate it. Are you a member of the NF2 Crew?
Tracy
I just went to a symposium on NF2 and schwannomatosis. The doctors talked about using gamma knife to remove acoustic tumors. They recommended not to use it for children under the age of 12. It has a slight chance of causing the tumor to grow back malignant. Even though the chance is slight, who want to take a chance? I also talked to many people who went to the Ear House Clinic in California with great results. They were able to remove the tumor and save the hearing. Take a look at the website earhouseclinic.com and it might be able to help in your decision. I have also heard good things about the trail you are talking about also. Let's hope some day there is a cure.
Hi Tracy;
No, I'm no longer a Crew member. There were some personal problems with a member a few years back and that absolved into quite a bit of upset.
Re, my NF-2 story; I first showed symptoms before the disease was separated form Von Recklinghausen's Disease ( NF-1 ) at 19.
First op was a Translab removal in 1977.
I coasted along feeling quite well for a few years.
In 1993, I again regained symptoms. They were chronic dizziness and a minor hearing loss.
Experts misdiagnosed me in 1994 as having Menieres Disease when the new ANs showed clearly on MRI. I went misdiagnosed and was treated for Menieres for 6 years.
In 2000, I switched to Mayo Clinic. The NF-2 Dx was confirmed and watching was recommended as my hearing was getting quite a bit worse by then. NF-2 ANs interweave in and around nerves.
Today, I'm nearly deaf but still have the ANs. Drs. don't wish to treat for fear of breaking the auditory nerve.
I'm a cochlear implant candidate, but this would probably be of little value with the tumors still in. There are 2 on the right side about 1.00 cm each.
I've 10, very small spinal tumors and a small meningioma and facial nerve AN which appear, then disappear on subsequent MRIs.
Have been hospitalized 3 xs with intractable, true vertigo and have also suffered 3 total, sudden hearing losses, attenuated by steroid.
I'm 59, single, and live in Northern Iowa in 2009.
Thanks for asking about me.
Best wishes to you!
Russ
My God you have been so lucky with this disease! I know you don't believe it, but I wish it could be that light with my daughter. Question- Your first translab- did they debulk or totally remove the tumor? How large was it? How has your diet and stress level been over the years, (if you do not mind me asking). Also, have you seen improvement in this since 1977? Obviously in education but also in treatment? I appreciate your responses. you seem to be quite educated with this. Do you remember having any balance issues before age 19, especially at night?
Should we keep steroids on hand if my daughter should have a sudden hearing loss? As of yet, no hearing loss- but she did mention that when she was walking in the dark in Costa Rica (she went there on a turtle study program) that her friends noticed her zigzagging some. Another question- the midfossa approach- can it help save the facial nerve also or is translab the only way to go with that? I realize that you are not a neruosurgeon- didn't know if you had studied it that much. also, do you take any supplements?
Thanks so much for your reply!!!
Tracy G.
Dear Russ- One more question- Did your meningioma, facial AN and spinal tumors appear with your VSs? Or did they all come much later. Dr. Giovanni at HEI told someone who told me that by the age of 25-30, you have pretty much the tumor burden that you are going to have. Would you agree with that?
Tracy G.
Hi Tracy;
I would agree from what I've read, the greatest tumor burden is usually seen early. However, this is not always the case.
Yes, the meningioma and facial nerve tumors appeared with the second AN(s), but, the small meningiomas and facial nerve AN appear, then disappear from year to year in MRI. They are considered a 'non issue' by the Neurotologist.
The 1977 Translab of a 1.5 cm AN was a total removal. There were some facial nerve extraction problems and bleeding problems which extended the length of the surgery. I have no facial weakness evident now.
Re, keeping steroid on hand, I guess that would be a Dr's call, but, I've been told a SHL continuing for 24 hours and not treated with steroid will mean deaf forever that side. On an emergency basis, some facilities will employ an in pt. 'steroid perfusion', as perfected at Shea Clinic, Chicago.
My stress level screeched out of sight with the NF-2 Dx!! I have to force myself to eat a balanced diet now and take Valium for the oscillopsia of having bilateral vestibulopathy ( loss both sides ) and anxiety/stress. Before that, and even after Translab, I lived fairly normally.
I recall no balance issues before 19 day, or night.
Best wishes with your daughter's health, Tracy!
Russ
Add to the discussion