Hi; newly diagnosed intralabyrinthine VS, looking at options

[lryan42]

Hi everyone.

Just thought I'd say hi, leave a breadcrumb here, and introduce myself.  I'm Liam from Irvine (Southern California).  I'm in my early 40's and I've just learned that I'm the not-really-proud owner of a small (5 x 4 x 3mm) intralabyrinthine vestibular schwannoma apparently located inside the vestibule in my left ear.

In one sense, it's a tremendous relief to finally get a diagnosis that explains my symptoms.  For a couple of years my disequilibrium has been variously diagnosed as a vestibular neuritis and then later endolymphatic hydrops or vestibular migraines.  Frustrating.

On the other hand, it's a bummer to have a VS as you kind folks know all too well.

For me, this started as an incredibly vague sense of disequilibrium 3 or 4 years ago.  If you have balance issues you know how hard this can be to describe especially in the beginning.  It fluctuated for a couple of years and finally landed me at a balance clinic in Newport Beach where I underwent a complete workup and 6 months of vestibular rehabilitation which brought almost all my quantitative numbers back to "normal."  Early last year I started experiencing sudden hearing loss which responded well to prednisone.  The hearing loss led to my first (noncontrast) MRI last summer.  That MRI showed a small lesion inside the vestibule, but the radiologist and my ENT both thought it was most likely some type of scar tissue from the VN.  My follow-up contrast MRI earlier this week tells the full story..

So, I'm starting down the well-trod path to figure out how best to manage this condition.

My current primary symptoms:

• Unilateral hearing loss.  Since my last audiogram this is down across the board; now 35-45dB in the low frequencies and 60dbB at 6K and 8K.  Word discrimination 96% (75 dB)
• Pulsatile tinnitus with a background of whirring tinnitus.  It's like my own personal sonogram in my ear!  Not awesome.
• Disequilibrium.  I'm really used to this now; mostly annoying rather than bothersome.
• Sound-induced nystagmus.  Loud dog barks in an enclosed area make my eye jump.
• Aural fullness
• Slight, fleeting waves of pain

I've seen my local neurotologist (Dr. Shohett) who has pointed me at a couple folks to speak with:

• Dr. Chris Duma (Hoag)
• Dr. Derald Brackmann (House)
• Dr. Robert Jackler (Stanford)

I had previously seen Dr. Doherty (a neurotologist now at Keck and part of their AN service) and was planning to get back to see her, though I'm wondering if it makes sense to also pull in some of the other Keck folks I read so much about here.

My local ENT's sense is I should get treatment because my symptoms are worsening and the location of the VS is unusual.  While apparently small by comparison to others (5mm at it largest extent), it's inside the vestibule and there apparently isn't much room in there...the ENT said it may have completely filled the vestibule by this point.

The ENT said the choice between radiation and surgery is a very close call here.  He wasn't sure how well radiation would work given my VS's location and so he suggested I go talk to the above doctors to get their take.  He did tell me that in his opinion I'm very likely to completely lose my hearing on that side.  I hope that doesn't happen but my hearing in the right ear is excellent and if it ends up going that way I'll be bummed but ultimately OK with that.  I am an avid scuba diver and deeply hope that whatever treatment I find allows me to continue to dive.

Of course, like everyone my priorities are to fix this thing, preserve my hearing, not pick up new problems, and ideally reduce whatever symptoms I currently have.

I hope my approach makes sense.  If you guys think there are other approach(es) or specific doctors I should be looking at, please let me know.  I fortunate to be able to travel wherever is necessary and I have a job that will allow me to take whatever time I need to get this sorted out.

So again, hi.  I am grateful to have found this resource.

Liam

[jami]

Glad to hear about the diagnosis, that makes a huge difference, doesn't it?  Enjoy this resource, and i strongly recommend to everyone to check out the recording or transcript on the ANA site of the seminar "The Patient-Centered Approach to Acoustic Neuroma". It laid down the items i took months to understand (late nights with google) once i was diagnosed.

[lryan42]

Glad to hear about the diagnosis, that makes a huge difference, doesn't it?  Enjoy this resource, and i strongly recommend to everyone to check out the recording or transcript on the ANA site of the seminar "The Patient-Centered Approach to Acoustic Neuroma". It laid down the items i took months to understand (late nights with google) once i was diagnosed.

Thanks very much.  That video was excellent and was just as you described: it captured and humanized and explained far better in 1 hour than my prior 20+ hours of research.

[v357139]

Liam,
Your approach seems pretty sound.  Here are a few suggestions.  If you want the best answers on radiosurgery, talk to a radiosurgeon, not a regular surgeon, and vice versa.   Surgeons tend to want to operate, and radiosurgeons tend to want to radiate.  There will not be a medical answer for your choice, it will be a personal choice based on your evaluation of the pros and cons.  I had surgery and am very happy with the result.   Dr Chang is supposed to be tops for radiosurgery.

On scuba diving, I and others who have had surgery will tell you that sometimes we can feel it when a change in the weather is coming, and I think it has to do with barometric pressure.  Personally, I would not want to try scuba diving since my surgery, not that I ever did.  I think this would be a good question to ask the doctors - is it advisable for me to scuba dive after treatment?  Their answers may help you decide which way to go.

I think you are looking at hearing the right way.  No one wants to lose it, but many of us have.  For me, it has been a very minor thing.
All the best,
Rich

[lryan42]

Just some more info after lots and lots of reading and talking with a half dozen different neurotologists about my specific case.

One thing that's become very clear is that everybody's tumor is different and you really cannot make any judgements about how to proceed until you've had a number of conversations.

At this point I've talked to all of the usual suspects and have been terribly impressed with pretty much everyone.  I've had consults with Friedman at UCSD, Brackmann and House, Jackler at Stanford, Moliterno at Yale, Doherty at Keck, and Shohet, Djillian, and Duma here in Orange County.

With one exception everyone agrees that my tumor cannot be treated by any means without guaranteeing total deafness in that ear.  It's because of the wacky location inside dude vestibule - way too close to the cochlea for GK or CK, and the surgical fix would basically be a labyrinthectomy.  So, that stinks.

That said,  my symptoms are stable for now and I've purchased a he hearing aid (I went with the Eargo) which has been a huge improvement.  Even with "just" moderate hearing loss (60dB at 2K and above; 40dB below 2K) the impact was pretty striking.  Life with the hearing aid is way better.   If I do need treatment at some point I'd absolutely get a cochlear implant on that side.

So that's about it. I'm on the wait and scan plan with the next MRI in 6 months or so.  More details later.

Good luck, all.

Liam

[v357139]

Liam,
Sounds very sensible.  Best of luck.  Here's to hoping for no grow...
Rich

[lryan42]

Another update -

My hearing on the LHS is markedly worse.  Got an audiogram and we're down to the 50-70db level across the board.  Word discrimination is down to 64%.  Balance is a little worse - though that fluctuates.  Tinnitus is louder and is a crappy combination of pulsatile "whoosh whoosh" along with a high pitched whine and occasional low tones.

Talking to my ENT locally we've agreed to just wait until December for the next MRI.  I'm still getting helpful results from the hearing aide, and it's not like knowing that the tumor has grown will change anything.

I've reached out to Dr. Stankovic about her recent research into medical approaches to VS management but haven't heard back from her (though her lab did reach out and offer to enroll me in their double blind aspirin clinical study - however that requires travel to MA).  After talking with my PCP I am considering just starting aspirin a couple times a week, though I understand that the very latest meta studies on aspirin for VS growth have been pretty discouraging.  It's hard not to grasp for straws, though.


This has all been a helpful reminder that nobody is promised tomorrow, and we should live in the present we have right now: worrying about what the future will look like doesn't help anything.  Easier said than done.

More updates to this thread as they are relevant.

[jami]

GREAT words and attitude "This has all been a helpful reminder that nobody is promised tomorrow, and we should live in the present we have right now: worrying about what the future will look like doesn't help anything.  Easier said than done."

I was full of anxiety and urgency when diagnosed and waiting 2 moths for surgery to be scheduled felt impossible when it was first underway, but I relaxed along the way. Am thankful I had that opportunityl

Liam - I am almost 1 year past surgery and 4-5 months past CK (had to do both) and life is different, but still so much better than some folks manage through. I have traveled 90K airlines miles this year so far for business, and I get much more tired and sometimes these massive headaches, but I also plan more rests, go to bed earlier, and it all works out in the end.

As a side note - spend the weekend between Long Beach and Santa Barbara and you guys had amazing weather! Live your weather (not the traffic...)

[v357139]

Sounds like a great plan.  Keep us posted.

[lryan42]

Update:

From August of 2019 to now, my symptoms have worsened moderately.  I haven't had another audiogram since 2019, but subjectively my hearing is much worse.  I can barely hear very loud noises - everything else I can't discern.  My balance fluctuates but the "bads" are worse.  I still have the loud pulsatile tinnitis but also have a lot of high-frequency "e-crickets" again.  I've sort of settled into the grind of the whole thing - trying to stay positive and not dwell on things I can't control.

All that said, I'm optimistic because I have a plan for this - cochlear implant.  My doctors more-or-less agree that when I get to the point where my hearing is gone, it's a green-light for the surgery to remove it.  There's a lot of hopeful consensus that removing the tumor and installing a CI would  likely improve the tinnitis a lot (maybe end it) and give me a good shot at restoring hearing.  There's also a good chance my balance will improve with the tumor removed. 

Now, with covid on the way out (fingers crossed), I'm starting to think more seriously about getting this done.

There have been a number of studies since 2019 demonstrating the dramatic QOL improvements of CIs for the SSD so I'm really hopeful insurance will play ball here.

What a crazy 4 years this has been!

[lryan42]

Update:

I recently had another MRI which showed a stable VS (well, that's what the UCSD and radiologist say - my local neurotologist thinks it's grown very slightly).  I also had a hearing test (my first since 12/2019) which showed nearly complete deafness.  My two "best" scores are a 95dB at 8kHz and a 100dB at 6kHz - otherwise it's all 110dB and worse.  The audiologist who administered the test was super concerned about how severely my hearing had deteriorated until I explained that this was entirely expected, due to the peculiar location of my particular VS.

It's a little strange to say, but I'm so grateful I'm finally SSD!  This milestone means I can get out of the "watch and wait...while your hearing gets worse and your tinnitus gets worse" purgatory I've been in since I first got diagnosed back in 2019.  Ever since my hearing started fading in 2019 the plan has been to wait for me to become SSD and then remove the tumor and install a cochlear implant.  And now, here we are.

I'm doing the CI eligibility testing in a couple of weeks to appease the insurance company, then I'm planning on getting the surgery in early 2022.  Exciting!

[Mia_M]

Hello Liam,

Just wanted to say I appreciate your thoughtful, detailed updates, and hope you'll keep at those. I'm newly diagnosed as of last month — this is actually my first post here — but I feel already like I've consumed enough research to present a thesis  . Mine's a small one, a little bigger than yours, so its W&W until at least late summer after the next MRI.

If/when I get to that fork in the road, my current feeling is that I'll take the CK route, no microsurgery. I'm early 60s, so I look at time a little differently than I did in my 40s, when I still felt like I had forever and a day to heal from anything that might come my way. But we never know what might compel us to redraw our hard lines in the sand either. So we'll see. I've also been reading about various drug trials, one interesting one in particular involving mifepristone, aka RU-486. Weird! Keeping an eye on that one, for sure, since aspirin doesn't seem to have panned out.

Looking forward to hearing how you fare with your surgery, which I believe you have planned in 2022. Wishing you ALL the best at every step, Liam.

[donjehle]

Thank you, Mia_M for your first post!  I can tell that you have been very thoughtful about it and have studied your options.  Please continue to share as you continue on your journey.  Everyone's journey is a bit different, but we all gain as we join hands (figuratively) and support one another.  I know you have a lot you could contribute.