My Acoustic Neuroma Journey

May 7 - 13 is Acoustic Neuroma Awareness Week so I thought I would write a blog sharing information about my journey with an Acoustic Neuroma. I realize that I have shared some of my journey, but not everyone has received a complete story and with Acoustic Neuroma Awareness Week, there is no time like the present to share.

An Acoustic Neuroma is a rare benign brain tumor that grows on the 8th cranial nerve (a nerve the comes off the brain stem). This nerve, known as the Vestibulocochlear Nerve has two divisions, the Vestibular Nerve responsible for balance (one of the 3 sensory systems for balance) and the Cochlear Nerve responsible for hearing. Interestingly, the Acoustic Neuroma is located on the Vestibular Nerve despite its name that gives the impression that it is located on the nerve for hearing. Technically, an Acoustic Neuroma is known as a Vestibular Schwannoma because the tumor is actually an overgrowth of Schwann cells (cells that are responsible for "insulating" a nerve to allow for a more rapid conduction of the impulses). The reason it is commonly called an Acoustic Neuroma is because the most common symptom is single-sided loss of hearing. The occurs because the Vestibular and Cochlear nerves enter the skull to the inner ear through the same hole (Internal Acoustic Meatus) and the tumor of the Vestibular Nerve puts pressure on the Cochlear Nerve, resulting in hearing loss.

Image from https://www.slideshare.net/sriloy/cranial-nerves-14327363

Other symptoms that could result include tinnitus (ringing the ear), feeling of fullness in the ear, loss of balance, dizziness/vertigo, facial numbness/tingling, headaches, facial weakness (very rare - the Facial Nerve which controls the muscles of facial expression is also located in close proximity), and taste issues (the Facial Nerve is also responsible for taste in the anterior 2/3 of the tongue). What is crazy is that everyone's symptoms are different and the symptoms don't necessarily correlate with the size of the tumor. Additionally, progression of symptoms do not necessarily indicate tumor growth.

My acoustic neuroma was discovered because of my single-sided loss of hearing. It was about nine years ago that I first started experiencing symptoms, but  remember it very vividly. At the time, I was working at CSUSB as the Fitness Coordinator, a part-time lecturer in the Kinesiology department, and the Assistant Coach for the Women's Cross Country team. I was sick quite frequently so I thought my hearing was impaired in that ear because of congestion. Fast forward 2-3 years and I was just starting my MS degree at CSU Fullerton. I was sitting in my Advanced Sport Psych class playing telephone as an once breaker exercise. When my classmate whispered into my left ear (the ear with the tumor), I couldn't understand what was said to me. I had no idea my hearing was that bad. It was a scary experience for sure, but of course I didn't do anything about it in the immediate future. Eventually I went to my GP and she thought I may have Eustachian Tube Dysfunction. It my mind, this made perfect sense. My mom and sister both had surgery for narrowed sinuses so, why not me? I started taking sinus meds, but they ended making me feel terrible and my hearing didn't change so I stopped taking them. I never followed up with an ENT, however. Fast-forward a couple years and now I was working on my PhD at USC. I had a transient vertigo episode and it freaked me out because my husband, Chris, had found information about Acoustic Neuromas and shared this information with me. I went to the health center, received a referral to an ENT and didn't take it any further. I let the busyness of life take priority. A year later, I had another momentary vertigo episode and went to the health center once again, was told I was likely dehydrated and that was causing my hearing loss. Luckily, I knew this was an absurd reason for my hearing loss and finally went to go see an ENT. 

In April 2016, I saw an Audiologist and was told I was legally deaf in my left ear. I could hear tones, though my abilities were reduced, but I had zero speech discrimination. This means I could hear sounds when someone spoke, but couldn't tell what was being said. This news was quite shocking to me. I knew my hearing was bad, but I didn't realize it was that terrible. Chris wasn't surprised however. I guess all of these years he would call me multiple times and I had no idea. All this time I thought I was hearing him right away, oooopppsss.

An Acoustic Neuroma is diagnosed with a MRI. I had an appointment with my Otolaryngologist, Dr. Elina Kari, and she told me she wanted me to get an MRI with contrast. She explained that they could do an MRI without contrast; however, if anything came up on the MRI, they would have to do a second MRI with contrast. She told me that I might as well just do it as contrast; I knew at that point that she thought I had an Acoustic Neuroma. It was at this point that I decided to tell my family that there was a chance I had a tumor and that this was likely causing my hearing loss. I figured it was best to prepare my family for the worse while we awaited the MRI results. 

On May 5, 2016, I received a phone call from Dr. Kari, they found a 2 cm tumor (about the size of a boulder marble...the big marbles that we used to try to knock our marbles). No wonder it felt like I had a marble stuck in my ear all these years, haha. The tumor was located in my internal acoustic meatus and was "spilling out" towards my brainstem similar to the image below. 

Image from https://makemehear.com/adults/acousticneuroma

Dr. Kari then took the time to address my treatment options. The first option was watch-and-wait meaning I would get scans every 6 months or so to monitor the growth of my tumor. Since this was my first MRI, they had no idea how quickly my tumor was growing and we wouldn't know until I had a scan at another date. I decided against this option, after discussing with Dr. Kari for two primary reasons. First, given my age, I would eventually need to get it treated. My tumor was already considered to be moderate in size and if the tumor grew too much, it could put pressure on my brainstem and become life-threatening. My second reason to opt out of watch and wait was that if I eventually chose surgery, my outcomes would likely be better since 1) I was young and 2) I didn't have any dizziness/balance symptoms. When these symptoms would appear was completely unknown (if they ever would), so with watch-and-wait I risked getting worse and also risked having worse outcomes following surgery in terms of long term balance. 

My second-option was gamma knife, a form of radiation treatment. After discussing with Dr. Kari, her and I both came to the conclusion that this wasn't the best option for me. She expressed that gamma knife has positive outcomes in terms of recovery, but she wasn't comfortable with this as my treatment given my age. There were no current long term outcome studies investigating the long-term effects of gamma knife. However, there is research that suggests radiation could eventually result in healthy cells becoming cancerous (rare but a possibility). I was familiar with this because of my reading about Angiosarcoma with Tory was diagnosed. Secondary Angiosarcoma of the Breast occurred in Breast Cancer Survivors that were treated with radiation. Thus, given my discussion with Dr. Kari, I decided to not go forward with gamma knife. 

My final option was surgical removal of the tumor. I would have a team of surgeons that were part of the USC Acoustic Neuroma Center that would open my skull and remove the tumor, essentially, my final option was brain surgery. There are multiple approaches to the surgery and since I was already deaf in my left ear, they would use the Translabyrinthine approach. This approach would completely destroy my hearing; however, it had a reduced risk for post-operative headaches and facial weakness. Facial weakness looks like you had a stroke, impairing your ability to fully smile and close your eye. This is ofter transient and will recover within a year, but in some cases additional therapy or surgery is required to restore the function of the Facial Nerve. While the risk for facial weakness was still a possibility, the risk was dramatically reduced. 

On June 7, 2016, I had surgery to remove my Acoustic Neuroma. The surgery would last 3-4 hours. This was a really fortunate scenario as the USC Acoustic Neuroma Center is a team of world-renowned surgeons that perform the greatest number of AN surgeries in a given year (out of University hospitals) that have perfected the art of the surgery to limit surgery time and post-op hospitalization. At other hospitals, this surgery could take 12+ hours. I was thus very thankful that I would only be in surgery for 3-4 and I am sure my family was very relieved as well, probably moreso than me!

Because I am an anatomy nerd, I can't skip over the surgical approach. The surgeons entered my skull just below the tip of my ear (mastoid process) as this is where the Facial Nerve exits the skull. They then drilled through my skull, following the Facial Nerve, to make sure it was protected. As a result of this path, the inner ear is essentially destroyed. To enter the brain, they then cut through the protective covering (dura mater) to visualize the tumor and where my nerve comes off the brain stem. The tumor was then removed often resulting in the removal of the Vestibular Nerve. The tumor was then removed often resulting in the removal of the Vestibular Nerve. This is an extremely delicate process as the nerves are very tiny and everything is in very close proximity. I had a hard time isolating these nerves when I was working on a cadaver....I can't imagine a live human being!  In order to close everything up, they took fat from my abdomen to pack in my skull (yes, I am officially a fat head) to reduce the risk of a CSF leak. A titanium plate was placed in my skull and the skin was closed over the plate, leaving me with a gnarly scar that looked like a mini shark bite. 

Abdomen Scar for Fat Graft 

Shark Bite..I mean surgery scar

The guaranteed side effects of the surgery with vertigo/dizziness, loss of balance (essentially needed to relearn my balance to walk), and complete loss of hearing. Potential side-effects/complications were facial numbness/tingling, facial weakness, headaches and alterations in taste. I was incredibly fortunate in that besides the guaranteed side-effects, I had no facial weakness/numbness issues and only had a transient alteration in taste (food tasted incredibly salty!!!). I am so thankful to my surgeons and God for allowing for a complete removal of the tumor without any unexpected complications!

The first 12-24 hours of recovery in the hospital were TERRIBLE. If I opened my eyes, I swore everything, I mean everything was spinning. This made me immediately sick. I have never been so sick in my entire life...it was such a hard experience. My dear husband was so amazing through it all. He just sat by my side, held my hand, and watched me sleep for hours. When I got sick, he was there to help, and this is a job I wouldn't wish on anyone. I can't imagine my recovery without him. It was at this stage that I had no idea how I was going to wake up the next morning and complete my physical therapy. I couldn't open my eyes, let alone sit up and I was supposed to walk the next day. It seemed like a very daunting task. I couldn't even roll over in bed without getting sick, any sort of head movement resulted in the worse feeling of the "spins." This was all occurring because the surgery damages/removes the vestibular organs on the surgical side, resulting in my brain needing to learn to adapt to half of a vestibular system. 

The next morning came and sure enough, I had PT. My Physical Therapist was the amazing Dr. Libby  Krause and she was accompanied by one of my former students who was completely her neuro inpatient rotation as part of her DPT. Both were so unbelievably amazing. They got me up and walking! I was able to walk 50 feet with assistance and felt incredibly accomplished. It was a totally weird feeling and I probably looked like a druken sailor, but I did it! During my walking experience, I didn't necessarily have distinct vertigo/dizziness. It was a different feeling. I now use the term "wonky head" to describe this feeling. It's like you aren't a part of your body and the world is moving fast but you are moving in slow motion. I still experience this feeling from time to time, so I ever say I am symptomatic or dizzy, this is how I feel. 

I was only in the hospital for 3 days. I was admitted on Tuesday and released on Thursday! I was so excited to go home. It was so amazing to see and feel how quickly my brain was adapting. When I got home, I had work to do. Walking was still difficult, especially at dusk. During this time, there was enough light that my brain wanted to use my eyes for balance, bit not enough so my impaired vestibular system was trying to take up the slack. I also would stumble or drunk walk when I would turn my head during walking. There was no way I could drive because the head turns made my dizzy and if I was pulled over there was no way I could pass a field sobriety test, believe me I would try walking in a straight line at home and it was an immediate failure haha. 

With time and vestibular therapy, my balance improved and my frequency of wonky head decreased. Every month, around the anniversary of my surgery, I tried to do something "big." I think my favorite memories are the Lake Gregory 5K two-months post-op, my first mountain bike ride, and my first time back on skis. I look forward to many more adventures! 

Two-months post-op after my first 5K back!

So, what does my future hold....continued growth and learning. I go in for a scan after the baby is born to check to see if there is any regrowth.  If this MRI comes out clear, I won't have to return for a scan for 4 more year. I am getting used to my single-sided deafness (not much different than before). I also had a secondary surgery for an implant for a bone-anchored hearing aid (looks like a bluetooth). This helps a lot in loud environments. I am also continuing to learn to live with my "new normal." My balance will never return to 100%, but it will continue to improve. Additionally, while my wonky head will reduce in frequency, stress, exhaustion, sickness, or particular environments/situations will trigger my symptoms and I will have to used my strategies to help me improve. Really, the majority of my recovery will be about acceptance, learning, and growth. 

I know this blog has been long, but I wanted to end with a honest reveal of my feelings and thoughts through this process. Thank you so much for taking the time to read.

What I Struggle With

• I get frustrated when I can't do things that use to come easy. I.e. lunges have become challenging to perform. I believe God blessed me with physical abilities and it gets hard when the things that used to be easy are now very challenging. 
• The most difficult part of recovery has been that I have an "invisible" impairment. I look totally normal on the outside, and for the most part, I feel good; however, on my bad days I look no different. People (except my hubby) can't tell when I have these "bad days" or when I don't feel well and I don't really mention it when I don't feel great. Therefore, I go on with daily life feeling not completely understood.
• "Bad days" come and go and this is hard to accept some time. Other than my hearing loss, I felt normal before surgery. Surgery actually makes you "worse"....it saves your life though. It is learning to accept these challenges as my new normal and learning to celebrate the small victories. 
• Large, social situations, while always something overwhelming for me before, have become that much harder due to my hearing loss. I have a hard time following conversations and engaging and by the end of the "party" I am exhausted from working so hard to focus. 
• Ironically, research, specifically data processing, is one of my triggers for my wonky head. This has been a hard adjustment because it is something I need to do as part of my PhD, but really takes a lot out of me.

What I am Thankful For

• I feel so incredibly blessed that I had no facial weakness. I have met so many AN patients through my support group that have experienced this complication. I don't know why I was so blessed, but I am incredibly thankful. 
• Sleep is absolutely amazing with my single-sided deafness! I just sleep with my good ear down, put my deaf ear up, and it is like nothing else exists in the world!
• I am so incredibly thankful for the amazing team of surgeons, therapists, and support at the USC Acoustic Neuroma Center. Treatment at this facility essentially feel into my lap and people from all over the country come to get treated by this time. I really was so blessed by my experiences.
• I am thankful for the small victories. While at times I get frustrated when things get hard, I had learned to find joy in the things I loved that we routine. I celebrated after my first bike ride, I celebrate after a successful hike. I get to celebrate the ordinary!

What I Learned

• I can no longer cry out of my left eye. Apparently the surgery affects the tear ducts...and yes I found this out during a breakdown, which was scary, but funny all at the same time. 
• I have witnessed the importance of PT. Inpatient and Outpatient PT was part of my treatment plan and it is amazing what it has done for me. I can't imagine my recovery process without my therapists!
• I really have been given a whole new perspective on life. When you go through a "health scare" it really shows you what is important in life. 
• While I have always known it, I learned how truly amazing my husband is. I cannot even imagine going through all of this without him. 
• I learned to fully trust God. If you know me, I am a worrier. My life verse is "Cast all your anxiety on Him for He cares for you." I have always struggled with letting go and letting God and I can honestly say this experience was the first time in my life that I was not "worried." Yes, I was scared, but it was different. I truly just let if go and believed God would care for me. 

What I Fear

• Not being able to hear my baby cry in the middle of the night. 
• Not knowing where my child is at when they cry for help. When you only have one functioning ear, you can't isolate where noise comes from. 
• Losing my hearing in my other ear and not being able to hear the voices of my loved ones. 

What I Look Forward To

• Continued growth as I adapt to my new normal
• Trying new/old things as I continue to recover
• I truly believe that God does not give you more than you can handle. I also believe He uses all things for His good. I look forward to using my experiences to help others. I think I had this tumor for a reason and that I need to use these experiences to give back and support others.