Acoustic
Neuroma Diagnosis
Acoustic
Neuroma Diagnosis
Back to: Something's Wrong
My work for Boeing is vital to the certification of a new airplane, and the schedule was in serious jeopardy before the SPEEA strike. Now, the pressure is on for me to work massive overtime to try to recover the lost time. Needless to say I’m deeply distracted and find it difficult to work even 40 regular hours a week, much less OT.
I find myself occasionally wracked with paralysing fear. Fictional "disease of the week" movies have it all wrong. Patients don’t cry out of "why me" self-pity, they cry because of fear. I don’t personally have the weepy episodes, but I do find myself hyperventilating at quiet times when I can’t avoid thinking about what’s happening to me. My body wants to fight or flee, but this isn’t a threat that I can fight with adrenaline.
My family is a real help. I try to remember the slogan from the SPEEA strike: together, we can bear it.
Now here's the weird part: In October, I dropped by my sister's office after work so we could have dinner together. I saw Dave, an aquaintance of mine and one of my sister's coworkers, while I was waiting for her. I can't remember why, but I was in a lousy mood and didn't feel like talking to this guy. Over dinner, my sister told me her coworker had a brain tumor and was waiting for surgery. I felt awful! Brain tumor? Gee, that means he's going to die, right?
So when I tell my sister in March that my ENT is sending me to get an MRI because I may have an Acoustic Neuroma, she replies, "I think that's what Dave had, and he's doing fine." No way!
Dave went to the doctor with a progressive case of facial numbness. He was diagnosed with a large acoustic neuroma, and was immediately referred to a radiosurgeon. Dave and his wife were OK with the radiosurgery, but while he was waiting for treatment, he met family friend and Seattle celebrity, TV achor Kathi Goertzen. Kathi went to the Seattle Ear clinic in 1998 to try to determine the cause of a progressive hearing loss. The MRI's showed a tumor around her 8th cranial nerve - it turned out to be a meningioma mimicking an acoustic neuroma. Here's an article about her surgery. (Meningiomas are much more difficult to remove than acoustic neuromas).
Kathi regained almost all of her hearing, and was very pleased with Dr. Mangham at Seattle Ear. Dave decided to go to Seattle Ear for a second opinion. Dr. Mangham is a fairly quiet, introverted person. He was very open about the risks and potential complications of surgery, but as you would expect from any microsurgeon, he was not upbeat about radiosurgery.
Dave was impressed with Dr. Mangham's candor and experience, but he was dismayed by how little Dr. Mangham and the radiosurgeon agreed. He sought a third opinion, and got almost exactly the same story from another source. He was told that [in the recent past,] radiosurgery had a success rate which was worse than surgery for everything except hearing, and even that tended to fail with time. There have been some recent improvements, but no one knows how well they work in the long term.
Dave looked on the internet, and quickly found that radiosurgery was still controversial for AN, especially for large tumors. This was hardly the story he'd gotten from the radiosurgeon. Dave lost confidence in the radiosurgeon, who he felt hadn't given him the whole story. He decided to go with Dr. Mangham. While he waited for surgery, Dave's condition progressed until one half of his face was totally numb. His hearing was essentially unaffected.
The day after surgery, Dave's numbness was gone. For a few weeks after surgery, he said he had difficulty concentrating for long periods of time. He found himself sleeping for several hours, awake for a several hours, and so on, all around the clock. A few weeks after surgery, he had facial weakness for four days. And then he started getting back to normal. He went back to work during the fifth week after surgery, but he could only work two days because he got tired so quickly.
The next week he worked three days. Eight weeks after surgery he was able to work full time, and felt he had 80-90% of his energy back. Five months later, he's doing fine at his fairly intense job, although occasionally he finds he gets tired a little more easily than he used to. Dr. Mangham told him it could take another 6-18 months to get the last 5-10% of his stamina back.
Since Dave had near perfect hearing before surgery, he was disappointed that after surgery he couldn't make out enough words to be able to use the AN ear with the telephone.
I read everything on the USCF website and UWMC. I was mostly concerned with diagnosis at this point, not treatment. I read the ANA website, including the 1991 NIH concensus statement. Since I still wasn't diagnosed, I also read about Meniere's Disease and Labyrinthitis, and came to the conclusion the ENT was correct - I probably didn't have these conditions. I read about astrocytomas and gliomas and all sorts of other tumors.
The news looked bad: the most probable diagnosis from my symptoms was an acoustic neuroma. If it wasn't a neuroma, my ENT had suggested there where some unusual infections and inflamations which could be the cause, although they were less likely than AN. On my own, I determined that really evil and probably fatal brain cancers were also unlikely, but just as probable as infections.
On April Fool's day, I crashed the Napa Valley wedding of my friend and former coworker, John Klym, with the full knowledge and support of his charming bride Gayle. We were all happy, and I kept my mouth shut about my problems. Brain tumors are not for single people. If you're single, don't get one! Your life flashes before your eyes and the only thought that really stands out is, "Why am I facing this alone?"
A few days later I went to see Bruce Springsteen in Tacoma, and my hearing had gotten so bad that an industrial-strength earplug in my right ear gives me equal hearing on both sides.
I went in for my MRI on a Saturday knowing there was no real opportunity for good news, only varying degrees of bad news. My ENT will be on vacation, so I'm not really sure who will be calling me with the diagnosis.
The MRI technician placed a folded washcloth over my eyes before I went into the tube. She explained, "People usually don't want to see the inside of the MRI tube." After 40 minutes of scanning * and a gadolinium injection, she unloaded me. As I slid out of the tube, I wiggled my face until the washcloth slid off, just so I could see what it was hiding. I was astonished how close the inside walls of the tube were to my face - perhaps only two inches away. I could see how people with no claustrophobia at all could go whacko without the washcloth. I'm fairly slender - how the heck do they fit big people into these machines?
The technicians at these places are schooled to have poker faces. They never reveal anything, but you can kind of tell how bad the news is by how much effort they put into being neutral. I did not get a good feeling about the results.
* source
ChoicesThe pressure is mounting at work. It's been three weeks since the SPEEA strike ended, and management wants us to start pressing our noses more firmly against the grindstone. Certfication documents are due. Analyses are running late. Risks taken by program management are turning into roadblocks which threaten to delay a billion dollar program. My work is on the critical path and I'm not working any overtime. Why?My co-worker George Bates catches me off-guard when he asks, "What are the chances you're going to have to have something done about your hearing problem?" Pretty good. "How long do you think you'll be out?" I tell him maybe a month, knowing that 8 weeks is more likely. Crap, if it turns out to be cancer, they might never see me at work again.
Wednesday is nerve-wracking. I'm a wreck. I'm supposed to call the Everett Clinic this afternoon for a diagnosis, but I have a fat certification document to finish. If the news isn't good, I'm going to take the next two days off, and I want the document ready for my coworkers to review before I go. I keep putting off calling the clinic, and I imagine trying to explain my agitated state to my lead.
I decide to call at 1 PM. At 12:30, my GP's office calls me. It is always bad news when the doctor's office calls you. I make an appointment for 4:30, and sit at my desk pale and sweaty. Somehow I manage to finish my certification document and put it on George's desk.
Lead: Why are you so nervous today? Me: I'm worried about a phone call. Lead: What could possibly be so upsetting about a phone call? Me: Because there's a small chance it's a call from the guy in the black bathrobe, and I've never had to deal with that possibility before. I've had the same GP, Dr. Brown, for perhaps 7 of the last ten years. He's a good guy, not much older than me. He comes into the room looking at his shoes. The news is clearly not good, but there really wasn't really any chance of good news. He says, "Well, it looks like you have something called an acoustic neuroma." I jumped up and shook his hand! He said,"I've never had anybody be happy to find out they had a brain tumor before." I said, "Thank God I'm not malignant!"
Here's the radiologist's report:
MRI BRAIN:
Clinical Hx: Unilateral hearing loss, sudden onset.TECHNIQUE: Saggital T1W, axial T2 and FLAIR weighted, and T1 post gadolinium administration images in coronal and/or axial plane are obtained of the brain. 15 cc Gadolinium.
FINDINGS: There is a mass at the left cerebellopontine angle which has intermediate T1 signal, heterogeneously prolonged T2 signal, and diffuse fairly uniform enhancement with Gadolinium adminstration. This enhancement extends into the internal auditory canal. This mass measures approximately 2.75 x 2.75 cm in the axial dimension and extends over a vertical distance of approximately 2 cm. There is a mass effect on the brachium pontis and fourth ventricle. Statisically most likely this would be an acoustic neuroma with left seventh nerve, fifth nerve neuromas and meningioma included in the differential diagnosis but less likely. Note of abnormal enhancement extending to the lamina cribrosa laterally within the internal auditory canal is noted. Otherwise the ventricles are not enlarged nor displaced. No focal structural brain abnormality or other evidence of abnormal enhancement is appreciated. No other mass lesions are identified.
CONCLUSION: 1. 2.5 X 2.5 X 2.0 CM HOMOGENEOUSLY ENHANCING LEFT CP ANGLE MASS MOST CONSISTANT WITH AN ACOUSTIC NEUROMA EXERTING MASS EFFECT ON THE LEFT BRACHIUM PONTIS AND FOURTH VENTRICLE.
2.75 cm?! That's not at all what I was expecting. That's bigger than a golf ball!
"I've got an appointment for you to see a radiotherapist at Virginia Mason in seven weeks." Radiation? Seven weeks? I was rapidly losing my hearing and I had unilateral headaches six hours a day. This was an improvement from the 8-12 hour a day headaches I'd had the week before, but was still intolerable. I was buying jumbo economy size bottles of ibuprofin. There was something funny about a supposedly slow-growing tumor causing changes in symptoms over weeks instead of months.
I said, "Doc, I haven't done much reading on treatment yet, but I know that radiation for AN is controverisal, is more risky for big tumors, and most medical centers would NOT recommend it for an otherwise healthy 35 year-old. And I know there are specialists in treating AN in Los Angeles and San Francisco and even right here in Seattle. I just can't see handling this in-network when there are people who treat these tumors on a weekly basis."
On the SANG website I had stumbled across the statement: You want a guy who treats these tumors at least once a month, and preferably once a week. There are roughly 3000 cases on AN diagnosed every year in the U.S., and my HMO covers perhaps a few hundred thousand people in the Northwest. They're probably only going to see a few acoustic neuromas a year, maybe four or five in a bad year. I wanted a team of specialists who did AN for breakfast.
My GP telephoned the radiotherapist, and returned to tell me, "The radiotherapist says he knows of a microsurgeon at Virginia Mason who performs AN surgeries, but he's not aware of any specialists in the Seattle area." Whoo-boy, that destroyed my confidence right there. This guy was either ignorant or being misleading, and I didn't want anything to do with him after that.
Dr. Brown offered to refer me to any doctor I wanted, but he wanted me to choose by Friday morning. Two days to pick a doctor! Aargh!
I left the doctor's office euphoric that I didn't have cancer. The euphoria lasted just about 6 hours, until the reality of my disease set in. I had a brain tumor. A real god-damned brain tumor. The stuff of Reader's Digest stories and Movie-of-The-Week weepies.
Look, Ma! It's My Brain!I called Dave back. "I bet my AN is bigger than yours was!" Nope - Dave had a 3+ cm AN.At Dave's suggestion, I called the MRI office, and arranged to buy copies of my MRI's and my medical records.
I didn't want to end up blindly following Dave to the same surgeon, but then I found Dr. Mangham's entry on the Seattle Acoustic Neuroma Group (SANG) website: Seattle Ear - Dr. Manham - performs about 1 AN surgery a week. Wow!
On the Seattle Ear website, I found the following news: Seattle Ear has never had a case of facial paralysis for tumors 3 cm and smaller. I like that! And useful hearing is preserved in 15% of patients with tumors big enough (like mine) to cause brainstem compression. Now that's no so good. But on the Seattle Ear website, Dr. Mangham is quoted as saying something like, "Even when the odds are poor, we owe it to our patients to try to save their hearing." Best of all, I was able to schedule an appointment with Dr. Mangham the following Tuesday! And if I wanted it, there was an opening for surgery in 5 weeks.
I called the House Ear Clinic in Los Angeles, and eventually got a return phone call from a Dr. Luxford. What can the most renowned AN center in the world do for me? Dr. Luxford said, essentially, with a 2.8 cm neuroma there's no point in even making the effort to try and save your hearing - we'd remove it using a trans-labyrinthine approach. What did he think of my HMO proposing radiosurgery? "They want you to accept radiosurgery because they think it's cheaper."
What does he think of the AN surgeons in Seattle? "Well, there's a Dr. Mangham who trained here at House." After a little thinking, he also vaguely remembered the surgeon at Virginia Mason.
But I didn't want to go with a surgeon who wasn't even going to bother to attempt to save my hearing. Dr. Luxford is a commanding individual. Since I didn't want argue with him, I just failed to return his calls instead of saying, "No." I really got the feeling that since the trans-labyrinthine procedure was developed by Dr. House, House Ear Clinic has fixated on it and won't consider suboccipital. Sort of like the "not invented here" policy commonly seen in the aerospace and computer industries.
( Other problems with the trans-lab approach: It destroys so much of the inner ear that you won't qualify for current or future techniques that might restore your hearing. )
UCSF looked good, but the phone number on their website is wrong, and in the brief time I had I was unable to get connected to a good number.
I called the office of the microsurgeon at Virginia Mason, and was offered an appointment seven weeks later. What is with this seven weeks crap? I declined.
I phoned one of the smaller radiosurgerical sites back east, (I forget which one, I had not yet discovered SIUH or JHC) and was told "Mail us your MRI's, we'll look at them. We can get you an appointment in a few weeks if you'd like." Now that's more like it.
I decided to take the appointment with Dr. Mangham, and spend the weekend researching where I wanted to go for a second opinion. I called Dr. Brown and he put in the referral to Seattle Ear.
I picked up the MRI's. It's interesting to see your own brain. Heck, I'm one of the few engineers at Boeing who can actually prove he's GOT one! The tumor is an ugly lump, jammed into my inner ear canal and pushing my brainstem off-center.I stopped to talk with one of technicians at the imaging center, and was astonished to find she had once worked with Dr. Mangham doing imaging at a downtown hospital for Seattle Ear. She said he performed a LOT of AN surgeries, and really seemed to be on top of things. This was how Dave found Dr. Mangham - a friend of his used to work with Seattle Ear.
I made a number of signs, which explained what had happened to me. The advantage of the signs is that they get the patient out of having to do the "So what have you got?" explanation over and over again. I dropped by my desk late in the day, when most everybody would have gone home, and posted copies over my desk.
A few of my coworkers were still at work, and I got to explain what happened in person. One of the guys quietly paged my lead while I was distracted, and he showed up as well. Boeing may be a screwy place to work, but my coworkers are damned fine people. I got more offers for help than I knew what to do with.
My ENT, Dr. Abson, called on Monday. He was concerned that my diagnosis had been handled properly while he was out of town. I said, yes, Dr. Brown had me come in to get the news. Dr. Abson then said, "Good! Now as for treatment, I think you should consider seeing Dr. Mangham at Seattle Ear, or maybe a Dr. Duckert at the University of Washington." I told him I had an appointment with Dr. Mangham the next day, and he replied, "You're in good hands. Dr. Mangham is the best."
Back to: Something’s wrongBut I still wondered about other treatment options...
Forward to: Decisions
