Arnold, that's the last straw!

The decision to wait some more came in mid-July.  That meant I only had a little over 4 months until I would go through another audiogram and MRI.  I thought all of this waiting would be so agonizing; that having Arnold just hanging over me would drive me crazy.  In fact, the waiting gave me time to accept that he really is there and to accept that my hearing is lost to me and would not ever return.  
For me, there was no epiphany moment where it just sank in and I got it.  It has been a slow process of living with the hearing loss and knowing that Arnold is just right there, hanging out between my ear and brain stem, pushing on those nerves, causing tons of tinnitus and distorting my hearing.  As I was able to talk about it more and make jokes about not being able to hear, somehow I slowly have been able to accept it.  
Many of you know this shirt that I have:

 

Greg bought this for me as way to relieve the tension and inject some humor.  I think it has helped quite a bit and, well, the kids love it.

I would like to interject here that most of my interaction with Dr. M's office is through e-mail with his amazing Practice Coordinator, Miss A.  She always answers my e-mails and is completely on top of everything.  She told me once I had decided to wait for the one year mark that she would be contacting me at the beginning of October to set up appointments.  On October 1 I received an e-mail from her telling me she had called my insurance to check for pre-authorization and had send the MRI request to PAMF, where I have been going for my MRIs.  Her professionalism, responsiveness and organization give me great confidence in this doctor's office.

My audiogram was on Thursday 11/8.  

The blue box is where my hearing was on my last audiogram.  As you can see, tones in my right ear took a dive.  Word recognition on this one was non-existent.   

If you have not done a hearing test lately, they certainly are different from when I was a kid and put those headphones on and listened for tones.  It is done in a sound room and they do lots of neat things like check that your ear drums are working properly and that conductivity through your bones works.  The main part of the test they plug both ears with an earphone in each ear.  The doctor closes the door to the sound room and then talks to you through the earphones, first in one ear and then the other.  This isolates the ears completely for each test.  

For this go around, she talked to me in the left ear when it came time to test in the right ear.  When the word recognition part came, she said she wanted to just try, so I said ok.  She switched over to the right ear and I listened and listened and waited for her to say something.  Then she said it was over.  My useful hearing in my right ear really is gone. 

One fun thing she did just on the right ear was to put a headset just behind my ear on the temporal bone.  She then played static in the left ear and tones in that headphone on the right side.  Because the bone could conduct it over to my left ear, I would hear the tones in my left ear!  It was so weird.

My MRI was scheduled for Friday 11/23.  Yup, the day after Thanksgiving.  As occupied as I was, it was still difficult to have to focus on confronting Arnold yet again and seeing what he had in store for me this time. 

Here is my first MRI from a year ago:

 Here is my latest scan, one year later:

You can see he has grown.  The official findings were:

Again identified within the right internal auditory canal is an enhancing expansile mass with dense associated enhancement measuring approximately 12 x 6 x 6 mm in maximal diameter, slightly enlarged as compared with prior exams.

My last MRI, 6 months ago, measured 8.3 x 4.8 x 5.8 mm.  So, according to my math, that was a 3.7mm hike in size!  That is a 44% increase?! and they think it is "slightly" enlarged?!  

Thankfully, I have copies of all my scans and software to view them.  I can even take my own measurements.  No, I am no radiologist, but given how different each reading has come back (let's see, the second one neglected the third measurement, the third one did not even bother with a decimal point accuracy...), I figured if I measured myself I would pick as close to the same points for measuring as three different radiologist would.  I found that from the first scan to the second it was slightly enlarged and the second scan to the third it also was slightly enlarged.  It has a rather steady growth pattern, which is expected for acoustic neuroma.

I was smart this time and went ahead and had scheduled an appointment with Dr. M back in October when I was setting up all these follow-up tests.  We met with him a week after my MRI.  He told us it's growing, it will continue to grow, might as well treat it now.  We talked about the different options, but then we pushed him to make a recommendation. He said given my age and that I have no useful hearing left, he recommends surgery, the translabyrinthine approach.

Now a little background on Dr. M.  He is a very well regarded neurosurgeon AND he is the director of the Gamma Knife program at UCSF (that's the radiation).  He treats with both approaches, I feel confident in his recommendation.   

I did not decide then and there in the appointment to go ahead with surgery and schedule it.  It still took a couple of weeks to come to that decision.  So, yes, I am going to have surgery and I hope to say good-bye to Arnold forever.  

As Arnold moves in...my hearing moves out.

Watching and waiting turned out to not be as agonizing as I thought it would be.  By the time I had my meeting with Dr. L, the Otolaryngologist at UCSF, it was mid march and four months had passed since my diagnosis.  With just two months left to reach the 6 month mark, it was not all that much waiting.
I had my next MRI on Tuesday May 22.  The results came in and the radiologist deemed it "unchanged".  Here are her exact words:

Findings: ...Again identified in the right internal auditory canal is an enhancing mass of 8.3mm transverse and 4.8mm AP, unchanged, consistent with an intracanalicular acoustic neuroma.

Impression: Stable 8.3 x 4.8 mm enhancing mass in right internal auditory canal consistent with an intracanalicular acoustic neuroma.

Remember my first scan measured: 8.3 x 4.5 x 6.1 mm, so of course the first question to pop into my head was, "What happened to the third measurement?"  Next I thought, "well, 4.5mm to 4.8mm looks like growth to me. "
To answer the first question, I e-mailed back to the ENT at PAMF, Dr. S, and asked him about that third dimension.  He was able to have the radiologist re-read the scan and she provided:

Addendum: The craniocaudal dimension of the right intracanicular acoustic neuroma is 5.8 mm

So, now I have a dimension, and it is smaller than the initial reading.  Immediately this tells me, two different radiologist I am going to get two different measurements. 
I sent a copy of my scans off to Dr. M's (neurosurgeon at UCSF) office and waited to see what he had to say.

In the mean time, I had another audiogram done on June 5th.  I very deliberately scheduled my audiogram to be done by the same doctor, Dr. K. 
In a nutshell, she said while I seem to be able to hear the tones about the same in the right ear as 6 months ago, my Word Recognition Score (WRS) has dropped to 0.

Just for fun, here is my first audiogram.  I circled where you can see my right side hearing is worse (the line is lower) than my left side hearing:

 

Here is my audiogram from June.  Though slightly lower, it is not considered a significant difference.

It took another month to hear back from Dr. M's office.  His letter said: 

There is no change in the size of a contrast-enhancing mass within the internal auditory canal.  I also have a copy of your audiogram from [6/5/2012] which shows that your word recognition is now a 0%.  This is not useful hearing.

From my point of view either document growth or loss of useful hearing are both prompts to consider treatment.

Again, Dr. M was very concise, one of the factors we were waiting for happened, my hearing is no longer considered useful.  Thus, he recommends treatment.  Again, I felt this was an answer, I should do something about Arnold, I need to consider radiation or surgery.
By now it was the middle of the summer.  When I think of summer as a kid, I remember what a fun and wonderful time summer was.  So now with my own kids, summers are meant to be fun and are the time to do and be with them as much as possible.  There was no way I was ready to think about doing anything about Arnold at this point.  
Then, come the middle of August, school would start up again and I remember what a very busy time that was last year.  I knew it would be somewhat easier with her going into first grade, no half days to start off the year.  But it would be busy with her birthday, then Halloween (my favorite holiday), then Thanksgiving and the Christmas holidays.  I knew I would not want to do anything during all of this.  So, I figured I might as well wait and get another audiogram and MRI in November, when it will have been a whole year.

An Otolaryngologist, Radiation Therapist and Neurosurgeon walk into a bar...

So now that I knew I had an acoustic neuroma, my next step was to see a specialist in this field, an Otolaryngologist.  They are ENT's that have specialized in the surgical aspect of treating diseases of the ear, nose and throat.  I think.

My first stop was to meet with Dr. B at Stanford Medical Center.  I went alone and in all I would say the meeting went well.  I felt comfortable with him.  He seemed very knowledgeable and very patient in explaining the nuances of acoustic neuroma, the factors that play into a decision of what to do about it and the various surgical approaches to treating acoustic neuroma.  He referred me to his colleague, Dr. G, that works with the CyberKnife program at Stanford and could give me more information about radiation treatment.

I was so completely overwhelmed with information and the fact that there was no clear path of when or how to treat Arnold that I think my brain once again shut down.  I had no idea what to do next.  

Dr. B had indicated that I would not be recovering my hearing.  This was the first time I had been definitively presented with this outcome.  At this point I could not accept or understand that the hearing I had lost in my right ear was gone forever.  

Dr. B also said that my age was a huge factor.  I am rather young to be diagnosed with one of these.  In my lifetime I will have to do something active about it.  

At this point Greg stepped in and insisted from now on he would be going to the doctor appointments with me.  I think what coherent information I was able to relay to him really hit him how serious this diagnosis is.  Where as I was reeling in the unacceptable truths of what was happening to me, he was realizing I would need all the support I could get from him and someone with a more objective view to help me wade through this process.

So, we scheduled a meeting with Dr. G to discuss radiation treatment.  She reviewed my scans and basically said I would be a good candidate for radiation treatment.  When we left Greg said he felt like he had just sat through a sales pitch.  We had no direction on what to do, just two different doctors at Stanford offering to treat it in their own expertise.

More uncertain than ever, I decided to just not do anything for awhile.  The holidays were upon us, I wanted to enjoy them.

Sometime around the new year, my oldest brother, Kent, called me up for some reason or another.  While we had seen each other quite a bit over the holidays, we had not actually sat and talked about Arnold and what progress I had made.  So over this phone call I told him about my doctors visits and how those went.  

Now, you must know, my brother works for UCSF Medical Center in Technology Assessment.  We also recently had a very bad experience with Stanford Hospital.  So while we were talking he started digging around on the internet, quoting me UCSF's rankings on various lists "Best of" lists.  UCSF is very well ranked where Stanford was not even on the list.  

He was adamant that I should be going to a health care team that would be working together to advise me on a course of action, not just selling me their services.   He said he would ask around and find out who I should talk to at UCSF.  

I am forever thankful for that phone call.  He lit a fire under me and pushed me to expect and demand more from my doctors.  It was the push I needed to move my treatment forward.

In the mean time, my sister-in-law, who is very well connected, had been reaching out to her network of professionals on my behalf.  She said at UCSF, the team of Dr. L and Dr. M were who her colleagues recommended I go and see.  Kent also soon got back to me and seconded that same team is who I should contact and meet.

So the process now was that I could not just call up Dr. M and Dr. L's office and ask for an appointment.  I had to ask my original ENT doctor, Dr. S, to refer me to them. He did push back a little and wanted to refer me to the "other" team at UCSF but I insisted these were the doctors I wanted to see and he sent the referral.  

Holy smokes are these guys in high demand.  The first person to contact me was someone from Dr. L's office.  I scheduled my appointment with him for March.  It was January at the time.  Good thing these things are slow growing!

Dr. M's office contacted me next and he was easier to schedule.  I met with him in February.  There were many striking similarities between that visit and my visit with Dr. B.  The assistant came in first to chat with us and get my scans.  She lefts for awhile, then retured with Dr. M.  They did a few tests on me to see if there were other symptoms besides hearing loss.  They confirmed it looks like an acoustic neuroma and here are your options.  We did not even have to ask him what he recommended we do.  He up front offered, at this stage of they recommend to watch and wait.  Get a scan in May and if it grows or my hearing gets worse we think about doing something.

That was the answer we were looking for.  A doctor to definitively say, "this is what we recommend you do."  Not a doctor that says, "well, you could do this or this or this...it's up to you."

So, even before meeting with Dr. L, we knew we would be watching and waiting and getting a fresh series of scans and tests in May.  What a relief to have a plan of action, if only for the next three months.

What do you do about someone like Arnold

As defined on the Mayo Clinic website: 

Acoustic neuroma is a noncancerous (benign) and usually slow-growing tumor that develops on the main nerve leading from your inner ear to your brain.

Other excellent references for information about acoustic neuroma are the Acoustic Neuroma Association website and the Stanford Medical Center website.  The important things to note are that is it non-cancerous, slow-growing and is located in a rather unfortunate spot.  These factors translate into some important points:

1. Well, it's not cancer.
2. Slow-growing so there is no rush to decide or do anything about it.
3. It is growing, so at some point in my lifetime I will have to do something about it.
4. It's location makes treatment rather tricky.

The treatment options are bucketed into three simple paths:

1. Watch and Wait
2. Radiation
3. Surgery

Watch and wait is pretty straight forward, every 6 months to a year re-scan and get an audiogram.  If the tumor grows or hearing declines think about doing one of the other options. 

Radiation and surgery each have their own special circumstances and nuances.  There are different approaches to each and different risks involved.  Simply put, choosing to eradicate Arnold with either of these approaches is not an easy decision.  ANA has a pretty good overview of the treatment options.

Radiation
The thought of radiation itself scares me.  Most likely a completely irrational fear, but my sense of radiation is if all else fails, then you radiate.  So, I admit that my research on radiation is minimal.  Here is what I think I know: the general idea for treating with radiation is to aim a bunch of non-damaging radiation beams from all around your head at the tumor.  Where the beams intersect is where the tumor is and it gets hit with a high, lethal dose of radiation.  The rest of the tissue that the radiation passes through is not as damaged.  This treatment is either done over a few days or a few weeks depending on where you go.
The technology that does this is either the Gamma Knife, which delivers beams all over your head all at once, or the CyberKnife, which moves around your head zapping beams at your tumor from all different angles.
The way radiation works is it messes with the DNA of your cells so the cell can no longer replicate.  You can see why this is a great idea for cancer cells; cancer cells are quickly replicating cells.  If their DNA is corrupt and they can no longer replicate, the threat of the cancer is diminished.  
For acoustic neuroma, after radiation treatment, what tends to happen is the tumor actually grows for awhile.  The radiated spot has now been damaged, so your body responds.  It takes about 3 years to know how effective the radiation treatment is.
Could the radiation cause the tumor to become cancerous?  It's the same percentage as if you did nothing at all.
Outcomes from radiation are actually very good and very encouraging.  After the initial swelling, most people experience the tumor shrinking back to the original size or even smaller than it was pre-radiation treatment.
One of my biggest reserves is they only have outcome data out to about 20 years.  In 20 years I will not even be 60 yet.  I plan on living way past 60, I really would not like some crazy thing to creep up from having radiation treatment now.
In a nutshell, a contact of mine, Dr. C, put it very succinctly:

The positive sides of radiation are: no intracranial surgery, it's a one time visit with no hospitalization, generally your facial nerve (which controls that half of your facial movement) will remain normal. 

The negatives are: it does not make the tumor go away, it just prevents growth.  you are committed to annual mri's to make sure it's not growing.  also, if you are young, there is the possibility that radiation increases your risk for other malignancies (small risk but real).

Surgery
I believe it is called microsurgery.  This is the well known traditional method of go in and cut out what is wrong.  While the theory of surgery is easier to grasp and accept, you have to remember this tumor is basically growing within a bone in my skull; not within my skull, not next to it, but right inside one of the hardest bones in the body.  It is an extremely inconvenient location.
There are three different approaches to surgically removing an Arnold: translabyrinthine, retrosigmoid/sub-occipital and middle fossa.  
When I had some hearing left, the retrosigmoid approach was recommended as this one is the best chance at preserving what hearing is left.  This approach requires drilling into your skull to where your brain is, pushing your brain aside so the neurosurgeon can get to where the tumor is.  
Now that I have no useful hearing left, the translabyrinthine approach is the recommended approach.  This one requires about 2+ hours of drilling through the temporal bone, the hardest bone in the body, to get to the tumor.  They remove certain hearing organs so they guarantee deafness in that ear.
The middle fossa approach I do not really know about since it was never an approach recommended for my case.
Again, Dr. C summed it up very well:

surgery postives: the tumor is removed and you don't have to worry about it for the rest of your life. 

negatives: risks of intracranial surgery including meningitis (1% risk), risk to your facial nerve (small risk in low 1-2% range but still more than with radiation).

I think the hardest thing to focus on when thinking about treatment is that hearing is not the top priority.  While that is the obvious symptom, I have to somehow get over the fact that my hearing is lost to me and focus on the important thing: this tumor is growing and it is growing straight for my brain stem.  Yes, this thing can kill me given enough time.  So I need to think about treatment to save my life.  The loss of hearing is just a minor issue.

Finally we meet: my first MRI

It took 11 days from my appointment with Dr. S, the ENT, to finally get my first MRI.  I wish I could say getting my first MRI was exciting or emotional or something intriguing.  For me, it was rather uneventful. And by now, I have had three MRI's and they all went about the same:

• Undress in the changing cubicle
• Put dressing gown on with opening at the back
• Put valuables in a locker
• Check no earrings are in
• Go into room when called
• Put key on cabinet
• Earplugs in 
• Lay down on machine
• (this is important) Be sure to bolster knees up.  (Last time I didn't and my back was killing me half way through!!)
• They put that cage around your head
• Close eyes as they slide you into the machine

So you do not have to close your eyes for the MRI.  I just do that because I am a little bit claustrophobic.  It has never been debilitating, but why try to freak myself out?  I have peaked while in there and, well, either I stare at that cage thing around my head or at the white tunnel I am in.  So, I mostly just close my eyes, hold my head still and attempt to sleep.  Thus far I have not been able to fall asleep.

The machine is very loud.  They run a number of scans and each one seems to have a different pattern of sounds, all loud, hence the ear plugs.  The person in the booth does talk to you some, telling you how long each scan will take.  I have not figure out if they expect a response or not.  Am I supposed to talk?  can the hear me?  or maybe give a thumbs up?  I do have ear plugs in and I've lost the hearing in my right ear, so really it's quite silly to try to talk to me.  

Once the scan is over, the anxiety is still there.  When will it be read? When will they tell me the result?  This first scan of course I was very anxious and did not know how long I would have to wait.  Dr. W had said she would be able to get the results of the scan the day I had it.  However, I had to wait a whole day before I found out.  

The scan took place on Tuesday November 22, 2011.  All day I had been waiting to hear and when I finally received a call from my doctor, I missed it.  I called back and left a message but was unable to reach him.  

We had plans in San Francisco on Wednesday night to meet up with family for a big pre-Thanksgiving dinner.  I remember we were in the restaurant, just settling in when my phone rang.  It was the general number for PAMF, so it was one of my doctors calling.  Dr. S had called to tell me that they read my results and that I do have an acoustic neuroma.  There are three treatment options, watch and wait, radiation or surgery.  He would refer me to a colleague at Stanford that specializes in this area.

So again, I did not have high emotion here.  I was not overcome with grief or depression.  I also was not completely dumbfounded and lost.  It was so matter of fact and, well, that explains the hearing loss.  I now have confirmation.  So now what?  It seemed like every step of the way thus far just opened more questions and uncertainty instead of coming to an answer or conclusion.  

My doctor, Dr. W, bless her heart also called that evening to check in with me.  I will have to dedicate a post about her for sure.  So her call, knowing she cared enough to review my results and check in with me gave me such comfort.  It made me feel like she is *my* doctor that cares what is happening to me and is going to watch out for me.  Even though this is not her specialty, I have someone I can talk to that will listen and help me however she can.  

 

MRI with and without contrast: Intracanalicular enhancing mass compatible with an intracanalicular acoustic neuroma, measuring 8.3 x 4.5 x 6.1 mm 

Tracking a phantom

After our first chance encounter, I did not really think about Arnold all that much. With two young kids and a part-time job I keep pretty busy.  Time passes quickly and things like whether the hearing in one ear is slightly worse than in the other ear just don't make the priority.

So about two months later, near the end of October 2011, I scheduled an appointment to visit my doctor, Dr. W, for a number of physical ailments, including asking about my hearing.  Apparently once you pass 30, stuff starts to not work as well as it used to.  I was having muscle spasms along with recurring aches and pains.  I figured I had better check into those to be sure I was not causing any permanent damage.

At the end of my appointment I mentioned my hearing loss.  She checked my ears to be sure they were free and clear.  She said she would request a hearing test for me.  She also said it is a little concerning that I only have hearing loss in one ear.  If the loss were in both ears, she would chalk it up to old age and playing my music too loud.  But, since it was asymmetrical hearing loss, she thought there was a possibility of a tumor causing the loss.

I went straight away up to Audiology and scheduled my hearing test.  They could get me in at the beginning of December.  That was the best they could do and it seemed there was no urgency, so I scheduled it.

About a week later, while working on my computer at my daughter's dance class, I decided to do a little hearing test of my own.  I played music and listened to it through my ear bud headphones.  I left the right ear bud out and listened only with the left ear bud.  Then I put that ear bud into my right ear and could tell there was a significant difference in the sound.  I also did the same with the right ear bud to rule out a difference in hardware.  The music was distorted and much fainter in my right ear.

I would not say I panicked, but it definitely raised my concern level.  This hearing loss is real and I think it is getting worse.   As one of our five major senses, it is not something I am prepared to lose.  I want to get it fixed as soon as possible.  So, I emailed Dr. W and asked if there was anything she could do to get my hearing test moved up. Fantastically responsive on e-mail, she responded quickly and said someone would be calling me shortly and she had asked them to get me in within the week.

The very next Tuesday I had a hearing test with Dr. K.  She found mild to moderate asymmetrical high frequency sensorineural hearing loss in my right ear.  And, my WRS (Word Recognition Score) was really low, only 30%.  She referred me to their ENT department.

So, now I know I have hearing loss in my right ear.  I have an audiogram to prove it.  How do we fix this?  How do I get my hearing back?  What will the ENT say? Will it keep getting worse and worse?  Can I make it get better?  What did I do to make this happen?  So many questions and such uncertainty filled my head and there was nothing to do.  I just had to wait and meet the ENT and see what he had to say.

That Friday I met with Dr. S.  He confirmed hearing loss and suggested we could attempt to treat it with steroids.  Sometimes if they catch the hearing loss early enough, treating with steroids can help, but that was a shot in the dark and I did not like the sound of that.  He was not telling me why I had hearing loss nor that there was a definite plan of action or cure for what was happening.  Seemed like all he could tell me was, "Yup, you've lost hearing in your right ear."  Well, duh?!

He did recommend an MRI.  He touched on Acoustic Neuromas, but said he did not expect to find anything on the MRI.  I guess that was meant to comfort me, but I did not find it comforting.  I wanted to know why I was losing my hearing.  I wanted to attribute it to something real and physical.  I could not accept that it was just going away for no reason at all.  At least if it was an acoustic neuroma that is something tangible and concrete.  Yes, it will complicate everything, but at least the hearing loss will no longer be a mystery.

At this point I knew no more than when I came into the appointment.  I was so confused and frustrated, the last thing I wanted to do was try to explain to someone else that I knew absolutely nothing.  I could not bring myself to call my mom or even my husband.  What was there to say?  

All I could say was there was more waiting.  First waiting for my audiogram.  Then waiting for the appointment with the ENT.  Now waiting for my MRI.  Waiting for what?  Some sort of phantom to finally appear?

A Chance Meeting

The first time I noticed Arnold I didn't even know it was him. It was over a phone call in the airport. 

Ok, actually it was a voicemail, but we were in the airport!  We were on our annual trip to New Jersey to see Greg's family.  We visit once a year sometime during the summer.  That particular year (2011) we were going in early August for Greg's cousin's wedding.

Dad had dropped us off and he had a new headset for his cell phone. He had not tried it out yet, but he was excited to have it and would be able to use it when he was waiting for our return flight. 

Dad had called and left a message on my cell phone. We were busy going through security so I did not know he had called. When I saw that I had a voice-mail from him, I was concerned there was something wrong since he had just dropped us off. So, as we were moving along the people mover I attempted to listen to his message.
 
If you don't remember what it is like in the airport, it's actually quite noisy. There are lots of things going on and all kinds of announcements every few minutes. When I was trying to listen to Dad's message on my right ear, I could hardly understand what he was saying.  I had the volume on my phone turned all the way up, too.  I had a flash of concern.  Greg had been saying for months now that "[I] just don't hear very well," as though if I just tried harder I could overcome such an ailment.  Now, however, I was afraid maybe there was something to these accusations.
 
We reached the end of the people mover where our gate was and the kids found something fun with which to play. With the kids thus entertained, Greg sent me back the other direction to get food.  So I hopped back onto the people mover. I decided to try to listen to Dad's message again since it was quieter, no announcements. I listened on my left ear and heard it with no problem. He was calling to test out his new headset, so it was nothing urgent. 

This raised some suspicion in my mind that something might be wrong.  But, then again, when I had tried to listen to the message the first time, it was very noisy with someone announcing something over the PA system.  I did not have much time to explore what was going on at the airport, so left it alone.

That evening after settling in at Greg's parent's house, I decided to try to listen to Dad's message again on both ears.  In the quiet of the guest room, I could hear the message fine in both ears.  There was a very slight difference from left to right, but it was so little I decided not to worry about it.  Next time I talked to my doctor maybe I would mention it.