treatment

Shelley's Story: A Journey Toward an Acoustic Neuroma Treatment Plan

joanne — Wed, 05 May 2021 01:58:25 +0000

Shelley's Story: A Journey Toward an Acoustic Neuroma Treatment Plan joanne Tue, 05/04/2021 - 21:58

Shelley Lacroix is an Ottawa resident who explored treatment options available throughout the world.

Shelley Lacroix

My story began with a simple ear ache that would not go away. After trying an off-the-shelf antibiotic and, subsequently, a cortisone prescription to no success, I was referred to the ENT clinic at the hospital.

The doctor at the clinic told me that I had age-related hearing loss. I had a hard time accepting this. My hearing up to that point had not been an issue. My tests showed 60% hearing in one ear and 80% in the other with 100% speech recognition. On the off-chance that my condition was attributable to sudden hearing loss, my doctor treated me with four intratympanic dexamethasone injections in my middle ear. I assure you that was not a pleasant experience!

I continued to experience pressure and aural fullness in my ear. At times, it felt like something was pushing against my tympanic membrane. I was then prescribed a course of Prednisone which led to no change in my condition. At this point, another doctor in the clinic referred me for a contrast MRI. In spite of the doctor’s request, the technician refused to give me contrast. As a result, I had to return for a second MRI after the first one revealed there was indeed a problem!

My contrast MRI report indicated that I had an acoustic neuroma (AN) measuring approximately 2 x 1.8 x 1.4 cm, approximately the size of a pecan half. I was relieved to learn that it was benign. During a video-conference with an ENT surgeon and neurosurgeon, the ENT surgeon informed me that I was not a suitable candidate for middle fossa surgery because my acoustic neuroma was too big.

Alternatively, the neurosurgeon proposed translabyrinthine surgery which would result in complete hearing loss, a severed vestibular nerve and could lead to partial facial paralysis or numbness and the possibility of a permanent or temporary palsy (droopy eye and droopy lip). Who wants to gamble with that I thought?

I bought myself time by agreeing to accept a “wait and see” approach in monitoring the growth of my AN. I made it my personal goal to learn as much as I could on the topic of ANs and treatments available in Europe, Canada and the USA. I am glad that I did this as soon as possible as I discovered that my treatment options would become limited if I waited too long to address my condition.

I read journal articles from the Journal of Ontology and Neurotology including a study about Aspirin halting the growth of sporadic vestibular schwannomas. I came across a study from Harvard where stem cells had been injected in the cochlea of mice to restore cilia and hearing. Unfortunately, no human trials have been undertaken.

I also explored whether the palsy could be surgically treated and consulted two independent doctors. The otolaryngologist, specialist in head and neck surgery, told me about the problems related to droopy mouth surgery and facial paralysis, and recommended the CyberKnife as the best course of treatment. I also consulted an ophthalmologist who said it was impossible to correct a drooping eye and achieve a natural look.

After receiving this information, I proceeded to conduct extensive research to explore alternative treatments. Through my research, I found out that Munich, Germany, has the most current CyberKnife S7. According to Medical Device News Magazine, the S7 offers “advanced precision, real time artificial intelligence, motion tracking and delivers radiation treatment in as little as 15 minutes”. I contemplated flying to Munich for treatment but then...COVID made things very complicated to do that safely. Along the way, I learned that Canada doesn’t have a CyberKnife S7 (2020 generation) and my city has the CyberKnife VSI (2009 generation). Also, I discovered that Hamilton and Montreal have CyberKnife M6 (2012).

Determined to find a better solution, I explored various US options in California, Colorado, Florida, Connecticut, and Pennsylvania, among others. During this research, I discovered a link to the American Acoustic Neuroma Association (ANA) and read the information provided by this group. By this time, my husband engaged in my research and stumbled upon the Acoustic Neuroma Association of Canada (ANAC). What a revelation! I became a member and immediately received a phone call from Carole Humphries, Executive Director, who offered both research and evidence-based data. The publications answered most of my questions, but not all. Within 24 hours, Carole connected me with dozens of people across Canada who shared their experiences. This made me realize I was not the only one facing this rare tumour.

It was through ANAC that I learned about Gamma Knife. Carole indicated that Gamma Knife was an alternative to CyberKnife. It was then that I realized that I had been far too focused on CyberKnife.

From that point on, I channelled my energy into exploring the pros and cons of both CyberKnife and Gamma Knife. From the University of Virginia Medical School website, I learned that Gamma Knife is far less invasive than surgery, uses 100X less radiation than CyberKnife and has a radiologic accuracy better than 0.3 mm. The Rocky Mountain Gamma Knife Centre website in Colorado indicated that Gamma Knife has a radiological accuracy down to 0.15 and that Gamma Knife Perfexion is safer because of its tissue-sparing characteristics, and its headframe prevents patient movement during treatment. According to the Mayo Clinic website, Gamma Knife is 95-97% effective and requires only one session to treat an acoustic neuroma. I gained insights

from the Journal of Neurosurgery about long-term outcomes following Gamma Knife radiosurgery (GKRS). The low incidence of developing hydrocephalus after GKRS and the preservation of hearing 15 years post Gamma Knife radiosurgery confirmed that Gamma Knife was the right option for me.

According to the Orange County CyberKnife and Radiation Oncology Centre website in California, the CyberKnife requires multiple treatments, is 90% effective, and could damage the outer targeted area. The odds associated with Gamma Knife were in my favour.

After completing the bulk of my research, I requested that my family doctor make a referral to Toronto Western Hospital. I compiled all of the paperwork that she required for the referral (i.e., audiology testing, MRI reports, MRI CD, medical history) to facilitate my Dr.’s task and expedite the referral process to Dr. Zadeh, head of neurosurgery of the University Health Network.

Dr Zadeh offered me two treatment options during a telephone meeting: 1) Rectosigmoid surgery which would preserve my existing hearing in combination with Gamma Knife six months post surgery to treat the remaining tumour; or, 2) Gamma Knife. I opted for the least invasive surgery, and I am currently awaiting word from the Gamma Knife Clinic for the date of my treatment.

During my discussion with Dr Zadeh at Toronto Western Hospital, I was comforted to know that the hospital staff focuses on the quality of life of the patient and that their goals are congruent with those of the patient. I knew my goals and shared them with the surgeon in order to ensure quality of life. My goals were simple: preserve my hearing, preserve my facial nerves, and preserve my vestibular nerve. From my standpoint, any treatment satisfying these goals would allow me to live my life to the fullest.

My journey made me wonder how many people out there would have relied on one opinion and had the translabyrinthine surgery with its inherent risks, as proposed by the first surgeon. I was thankful that I connected with ANAC and was able to be guided in the right direction to a team of leading neurosurgeons and researchers in the area of acoustic neuromas and brain surgery. It is important for us to realize that perhaps surgeons recommend surgery because that is their specialty. I was so pleased to learn that Dr. Zadeh and her team do both microsurgery, and stereotactic radiation surgery based on the individual needs of the patient and the size of the neuroma. I was happy that I was given alternatives to my treatment plan.

To close off, I would like to leave you with this final thought. It is important to make an informed decision about any recommended medical treatment plan.

You, alone, control the journey and must research your condition and critically examine all the options available. It is critical to formulate precise questions so that the experts can fill in your knowledge gaps. Once you are confident with your understanding of your condition and possible treatments and their associated risks, you can then, if given a choice, select a treatment plan that satisfies your goals.

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The Current Landscape of Vestibular Schwannoma Therapy: Development of Novel Targeted Therapies

joanne — Tue, 04 May 2021 19:48:47 +0000

The Current Landscape of Vestibular Schwannoma Therapy: Development of Novel Targeted Therapies joanne Tue, 05/04/2021 - 15:48

May 2021

An acoustic neuroma, also known as a vestibular schwannoma, is a benign tumour that arises from Schwann cells of the vestibulocochlear nerve. In the majority of cases, these tumours occur sporadically, but the presence of bilateral tumours is pathognomonic for a genetic condition called neurofibromatosis type 2(NF2). The loss of the NF2 gene is linked to pathogenesis of acoustic neuromas, both in sporadic and genetic conditions. About 60% of sporadic unilateral acoustic neuromas have mutations in the NF2 gene. Mutations of the NF2 gene lead to deregulation of important pathways that regulate tumour growth such as RAS and mTORC1 pathway.

Regardless of the etiology, acoustic neuromas are typically located in the cerebellopontine angle (CPA) and lead to progressive hearing loss, vertigo, tinnitus, ataxia and sometimes facial nerve dysfunction.

The optimal treatment options for patients with acoustic neuromas is dependent on the size and growth of tumours, symptomology including ataxia and facial nerve dysfunction, and patient preference. Generally, the goal of treatment is to preserve neurological function as long as possible with tumour control. As such, small tumours with minimal symptomology is often treated with close observation and serial imaging.

Previous studies have identified that at the mean growth rate of these tumours is approximately 1-2 mm/year with up to 75% of tumours showing no further growth. Although observation can increase the risk of tumour progression and mass effect, it is a safe approach due to the minimal growth rate. Furthermore, studies have demonstrated that delaying surgical intervention appears to have no increased risk in mortality. However, when there is evidence of substantial growth with no compression on the brainstem, then radiotherapy or surgery is considered. When the tumour is compressing the brainstem with associated worsening of neurological symptoms, the preferred treatment is surgical resection.

Historically, the medical treatment options for vestibular schwannomas have been limited due to the tumours often benign and chronic nature. Bevacizumab, an intravenous chemotherapy, is a monoclonal antibody against the vascular endothelial growth factor (VEGF). The initial clinical trial was conducted in 10 patients with confirmed neurofibromatosis type 2 with evidence of tumour progression. The trial demonstrated 90% of patients had reduction in tumour volume and 57% patients had improvement in hearing. This landmark clinical trial led to the completion of a number of studies, which also reported decrease in tumour volume and improvement in hearing response in patients with neurofibromatosis type 2. Therefore, it is difficult to know whether the results will translate to majority of the sporadic vestibular schwannomas.

In theory, Everolimus is another promising treatment as it is an mTORC1 inhibitor. Activation of mTORC1 has been implicated in tumour growth. However, Phase II trials in NF2 patients have shown mixed results. One study showed no response to Everolimus on tumour growth or hearing improvement, while another study found a 66.5% reduction in tumour growth during Everolimus treatment. At this time, there is no strong data to support the use of Everolimus in management of vestibular schwannomas, and further long-term studies are needed to provide more robust evidence.

Vestibular schwannomas are a clinically important disease with an evolving knowledge base. Further work is needed to understand the biological alterations that are driving the tumour development, in order to better develop targeted therapies that will effectively treat this disease process.

Dr Gelareh Zadeh

Gelareh Zadeh, MD, PhD, FRCS(C), FAANS is an internationally renowned neurosurgeon and neuroscientist who also happens to be chair of ANAC’s Scientific Medical Advisory Committee. Dr. Zadeh recently became the first female chair of neurosurgery at University of Toronto, which is the largest department in Canada. She is also the medical director for the Krembil Brain Institute, TWH and head of surgical oncology at the University Health Network. In addition, Dr. Zadeh holds the Wilkins Family Brain Tumour Research Chair.

Her clinical practice focuses on skull base neuro-oncology, with a dedicated brain tumour clinic and many multidisciplinary clinics that she has established including skull base, pituitary, brain metastases, gamma knife and neurofibromatosis clinic.

Dr Suganth Suppiah

Suganth Suppiah, MD has since 2018 provided tremendous support to ANAC. He is the Senior Neurosurgery resident at University Health Network, University of Toronto, focusing on peripheral nerve surgery. He is currently completing his PhD under the supervision of Dr. Gelareh Zadeh on the molecular profile of peripheral nerve sheath tumours.

How Can Vestibular Rehabilitation Help Me?

joanne — Fri, 09 Apr 2021 20:54:00 +0000

How Can Vestibular Rehabilitation Help Me? joanne Fri, 04/09/2021 - 16:54

Vestibular Rehabilitation is an exercise-based approach for treating people with dizziness and balance disorders that originated in the 1940’s. Since then there have been many advances in the assessment and treatment approaches for inner ear disorders. The inner ear is housed deep in the mastoid process behind the ear and it cannot be viewed with the otoscope. The key to assessment of the inner ear is by watching the eyes through special goggles that the patient wears. Eye movements when the head is stationary or moving will indicate what conditions are or are not present.

Some of the inner ear conditions that cause dizziness or balance disorders include Unilateral Vestibular Hypofunction (UVH), Bilateral Vestibular Hypofunction (BVH), Benign Paroxysmal Positional Vertigo (BPPV), Meniere’s Disease, Perilymph Fistulas and Acoustic Neuromas. This slow-growing tumour deep within the inner ear along the acoustic nerve causes a gradual loss of function of the inner ear, usually on one side of the head only. When one inner ear is sending less information to the brain than the opposite ear sends, the result is conflicting signals that the brain is trying to sort out and which, in my opinion, is the cause of the sensation called ‘dizziness’.

Dizziness is not a diagnosis, but a symptom that something is wrong, and it alone does not describe what a person might be experiencing. The meaning of ‘dizziness’ can be different for each person. Some people say they are dizzy when they are feeling off balance, unsteady, light-headedness, spinning, nausea, woozy, rocking and many other descriptions. And dizziness can be from many different causes other than the inner ear, including visual loss, sensory loss, mixed sensory losses, disease processes, neurological, psychological, pharmaceutical, and musculoskeletal systems. The neck may also cause dizziness, and this is called cervico-genic dizziness.

Treatment of the inner ear involves several different approaches and is individualized depending on the assessment findings:

Adaptation which involves retraining of the eye-head coordination so that the vision will be stable when the head is moving.
Habituation to improve tolerance to head movements.
Balance retraining for fall prevention and overall improved function
Substitution exercises for the body to learn to rely on the intact sensory systems when one or more of the other ones are not functioning.
Neck rehabilitation if needed for cervico-genic dizziness
Maneuvers to treat BPPV
Education and reassurance

Pre-AN Diagnosis and Pre-AN Surgery

Some of the early signs of an AN include dizziness and imbalance for which the person might seek an assessment by a Physical Therapist or Vestibular Therapist. During the assessment, there may be indicators of AN that the therapist may bring to the attention of the physician. Vestibular treatment at this point may help with the brain to learn to compensate for the loss of function, to retrain balance control and to improve the eye-head coordination. Before surgery is indicated, the person may already have complete loss of the vestibular function on the one side, and their brain may have already compensated for the loss, meaning that they have minimal balance concerns and minimal to no dizziness. If there is partial compensation, then they may be having ongoing difficulties.

Post AN Surgery

Following the surgical removal of the AN, the level of symptoms will be determined by the level of loss they had before surgery. As mentioned above, if there is complete loss then the post-surgical symptoms will be minimized, and the person should be able to return to full function fairly easily. If the loss was not complete prior to the surgery, then they will be experiencing a sudden loss of function and will have more difficulties and will require more vestibular therapy.

Can I have more than one condition at the same time?

It has been both my professional and personal experience that people can have more than one condition happening at the same time. This makes the diagnosis more complicated but a systematic and thorough assessment would help discern what is involved. In my years of experience, I found that the neck is a key component for many people and some simple exercises and stretches can make a tremendous improvement for the cervicogenic dizziness. People can also have unilateral vestibular loss and BPPV which again can be seen with a complete assessment and both can be treated effectively. As well people may have weakness in their lower body or loss of sensation in the feet, which will affect their balance. Anxiety in any of these conditions will, in my opinion, magnify the symptoms they are feeling and may limit their willingness to participate in the rehabilitation process.

Who should I see?

After seeing your doctor to rule out any medical concerns that may be contributing to your symptoms, an assessment by a “Vestibular Therapist” would be of benefit. Vestibular therapists typically have a background in Physical Therapy, Occupational Therapy, Audiology, or medicine (ENT). There are various levels of training from one or two-day weekend courses to more intensive 5-day training in Canada and the US. As well, the level of prior experience or training the therapist has will vary. Some courses have a competency process of testing the practical skills and written knowledge and some don’t. There is no set standard of entry level practice for vestibular rehabilitation, but in my opinion, a vestibular assessment that does not use the goggles will be not be complete.

I wish everyone with AN to have a dizzy free and balanced life!

Robynne Smith,
B.Sc.P.T., B.Sc.Anat

About the Author

Robynne’s post-graduate training includes advanced course work in Vestibular Rehabilitation in Canada and the USA. Additionally, she has completed FallProof training, which provides her excellent fall prevention skills. She also has experience and training in orthopedics, neurology, paediatrics, arthritic conditions (including osteoporosis) and chronic pain.

Robynne who ironically was treated for an acoustic neuroma in 2011 has specialized in balance and dizziness concerns including: vestibular neuritis, BPPV, BPV, Vertigo, imbalance, unsteadiness, cervicogenic dizziness, Meniere’s, and other inner ear concerns.

Assisting clients in restoring their balance allows them to return to previous activities of daily living without dizziness and fear of falling. Robynne is now offering professional training on Vestibular Rehabilitation for allied health professionals through introductory to advanced level seminars, workshops and courses. You can email her at saskbalance@sasktel. netwww.saskbalance.com

Pat Greenwell’s Acoustic Neuroma Diary: Her AN Journey from 1997 to Now

joanne — Wed, 05 Jun 2019 21:48:27 +0000

Pat Greenwell’s Acoustic Neuroma Diary: Her AN Journey from 1997 to Now joanne Wed, 06/05/2019 - 17:48

Pat Greenwell's life became complicated for her while living in rural Alberta in 1997.

Pat Greenwell

Treatment for breast cancer and an increasing dizziness and loss of hearing in the right ear. Doctors blamed the chemo. We’ve all been there. Months of trying to convince doctors that the symptoms are not imaginary. No, trust me, Doctor, antibiotics, decongestants, anti- vertigos and valium don’t work.

Finally, a casual mention of dizziness to my breast cancer surgeon, dear Dr. Fraser, in Edmonton, who sent me off for an emergency CT scan and finally, within an hour, a diagnosis of a 3 cm brain tumour – an acoustic neuroma.

Shock for us both, especially my husband Ken, but I’d kind of suspected. The tumour had blocked the flow of fluid out of the brain so, in addition, I had hydrocephalus. An emergency procedure to install a Ventricular-peritoneal shunt was done the next day to prevent a stroke or aneurysm. Thank you, Dr. Max Findlay.

Now what?

I read everything I could on what my choices were, learned to live without driving, learned to live with worsening symptoms, wore a medic alert bracelet so that the shunt which goes to the abdomen doesn’t get interfered with... tumour is too large to consider anything but surgery.

Okay, Dr. Findlay, pretend I’m your mother, what do you recommend? Edmonton, a higher chance of facial paralysis (21 years ago) vs Toronto, Dr. Findlay’s mentor, Dr. Charles Tator.

No contest.

Off to Toronto for three weeks...

Appointments arranged by Dr. Tator’s secretary, Maria Vespa, giving info about hotels and transportation between hospitals for Ken and me, two traumatized people. A week of appointments and tests with Dr. Rutka (ENT) to see if I had salvageable hearing in the right ear.

Nope.

Oct 6, 1997...

14 hours of surgery at Toronto Western Hospital in the midst of a nursing shortage. I was warned in ICU to keep a bedpan handy because calling a nurse would not often work. My roommate was a teenage girl who had had numerous brain surgeries and her mom slept on the floor by her bed —a cozy threesome. Ken stayed close, took care of me, taught me to walk again when I was wobbly. A constant stream of young doctors all who had been inside my brain, learning at the hands of the master, my hero, Dr Tator.

A week of recovery, then back to Alberta. Bald, clumsy, hard to see with an eyepatch... not answering the door... Two weeks of temporary facial paralysis which felt like a lifetime. Running to the mirror every morning to see if I can smile yet and to see if my eye will close by itself. Pins and needles on my face and the traumatized facial nerves recovered.

Lots of naps, lots of hovering family, friends taking me for walks and I finally became myself again.

2019: fast forward to age 76......

Retirement to Powell River on the BC coast in 2005.

Daily bike rides with hubby, singing in a local choir, volunteering, good health most of the time. I still find it hard to walk in a straight line, and don’t balance well on one leg. Teary right eye and runny nose -- a minor inconvenience handled with tissues.

I take very good care of my left ear, try to avoid loud places and crowded events, and wear an earring from ANAC in my right ear which says, “out of order”. (The grandkids love that!) We are bird watchers and while I don’t know what direction the song is coming from, I can still love the song.

Gratitude is part of my life! Life is good!

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To Judy: Further Insights in the Conservative Management of a VS

joanne — Sun, 28 Apr 2019 21:29:02 +0000

To Judy: Further Insights in the Conservative Management of a VS joanne Sun, 04/28/2019 - 17:29

Dr. John Rutka is a fellow of Royal College of Physicians and Surgeons of Canada and a professor of Otolaryngology-Head and Neck Surgery at the University of Toronto. He sent the following letter to Judy Haust, President of ANAC.

Dear Judy,

I have known you for many years personally but became your treating physician when you were diagnosed on a MRI scan to have a vestibular schwannoma (VS). I can only imagine what went through your mind when you found you had a benign brain tumour that was responsible for your left sided hearing loss.

You seemed somewhat surprised I didn’t more forcibly recommend an active treatment option at the time such as stereotactic radiation or microsurgical removal. I know that this is what I may have thought if I was in your position.

Instead I recommended that as your tumour was relatively small, we could continue to watch it with serial intracranial MRI scans. If there was no significant growth, we could continue with this management. If your tumour did grow however then some form of active intervention would be required. Since we met to professionally discuss your management it has now been almost 6 years and I am pleased to report that everything has been stable with regard to the tumour size.

As you know we have had quite considerable experience watching individuals with VS’s over the years with Conservative Management (or the Wait and Scan Approach). While we still don’t know why some tumours grow and others do not (in fact up to 15% might decrease in size naturally) we certainly know that your quality of life is best if no active intervention is required as we are still not able to improve upon your lost hearing or the balance function despite our best intentions. As they say, sometimes less is better in medicine.

When we met I advised you that our 10 year longitudinal prospective study in patients (average age of 58 years at entry) with tumours < 1.5 cm in size within the cerebellopontine (CP) angle had demonstrated the vast majority of tumours continued to grow slowly (92% < 2mm/year in cross sectional diameter), the average growth rates of tumours within the internal auditory canal was remarkably 0mm/year (the presence of a tumour localized to the IAC seemed to demonstrate indolent behavior for the most part in this age group) and that 95% of patients with IAC tumours did not require active intervention. The most important stats from this study were that over 10 years 60% of patients did not go on to receive active treatment and in the 40% that did there was no harm by waiting compared to our normal surgical and stereotactic radiation controls. Moreover, a tumour usually declared whether it was growing within a five-year timeframe.¹

Just wanted to let you know we have continued to offer a trial of conservative management to most patients with vestibular schwannomas < 2cm in size within the CP angle taking into account age, presence of other health co-morbidities and whether hearing is serviceable (and associated with favorable prognostic findings for hearing preservation surgery). Our findings have paralleled further studies regarding the conservative management of VS’s from the UK, Denmark, Netherlands, US and even China specifically. ^2,3,4

I don’t know whether I ever told you the story that in April 2005, I had presented our then seven- year longitudinal findings at the time to the North American Skull Base Society who were meeting in Toronto. The findings are captured in the editorial I wrote shortly afterwards for the journal, Clinical Otolaryngology, entitled “What Would You Do if You Had a Small Vestibular Schwannoma? An Apocryphal Tale.” ⁵ I had presented the case of an intracanalicular VS in someone who was 50 years old (my age at the time) where hearing preservation was not an issue.

I polled the audience to see what they would recommend as the treatment before my lecture began. Most were surgeons and it came to no surprise that almost all recommended microsurgical removal although there were a few who felt stereotactic treatment was reasonable. Only a couple of people in the audience raised their hands to show support (myself included) for an initial trial of conservative management at the time.

After presenting our seven-year results (70% continued to be managed conservatively; 30% had required some form of active treatment) I asked the same question showing the same imaging study but said “this time the patient is you”. Remarkably well over 90% of the audience now seemed comfortable following their tumour with serial imaging initially. Interesting how things can change?

In my further review for this letter I have also looked again at the world literature and find that the conservative management option continues to be recommended certainly in the Northern European countries and less grudgingly now in the US. Unintended benefits from conservative management also interestingly demonstrated what seemed to be a significant cost saving for publicly funded health care systems (i.e., Canada) where the money saved could be reinvested in other programs. From our calculations it would take up to an 80% failure rate before the upfront costs of conservative management would exceed those of microsurgical removal for example.⁶

While not everyone should be considered a candidate for a trial of conservative management, I think that when your tumour is relatively small and hearing preservation not of concern then this option should at least be considered. The sine qua non however is that you need to be followed with interval scans possibly for many years to come.

When I look back, I think the advice I gave you at the time was right advice for the moment. I hope that things will continue as they have and perhaps maybe your tumour will start to get a little smaller as the years go by.

Will have my office make the arrangements for your next MRI in August-September 2019. Wishing you the very best as always.

Sincerely, John

References

Hajioff D, Raut VV, Walsh RM et al. Conservative Management of Vestibular Schwannomas: Third Review of a 10 Year Prospective Study. Clinical Otolaryngology 2008; 33:255-59.
Patel J, Vasan R, van Loveren H, Downes K et al. The Changing Face of Acoustic Neuroma Management in the USA: Analysis of the 1998 and 2008 Patient Surveys from the Acoustic Neuroma Association. Brit J Neurosurgery 2014; 28:20-24.
Stangerup SE, Caye-Thomasen P. Epidemiology and Natural History of Vestibular Schwannomas. Otolaryngology Clinical N Am 2012; 45: 257-268.
Martin T, Tzifa C, Kowalski C, Holder RL et al. Conservative Versus Primary Surgical Treatment of Acoustic Neuromas: A Comparison of Rates of Facial Nerve and Hearing Preservation. Clinical Otolaryngology 2008; 33: 228-235.
Rutka J, What Would You Do if You Have a Small Vestibular Schwannoma? An Apocryphal Tale. Clinical Otolaryngology 2008; 33:236-238.
Verma S, Anthony R, Tsai V, Taplin M et al. Evaluation of Cost Effectiveness for Conservative and Active Management Strategies for Acoustic Neuroma. Clinical Otolaryngology 2009; 34; 438-446.

Dr John Rutka

Dr. John Rutka is a fellow of Royal College of Physicians and Surgeons of Canada and a professor of Otolaryngology-Head and Neck Surgery at the University of Toronto. His active staff appointment is at the University Health Network where he has a subspecialty interest in otology/neurotology. He is on consultant staff at Sinai Health Systems, St Michael's Hospital, the Dryden District Regional Health Centre and the Meno Ya Win, Sioux Lookout District Hospital.

Dr. Rutka has been involved in the active management of patients with VS's over the past three decades in conjunction with his neurosurgical and radiation colleagues at the University Health Network. John has been recognized for his work and has been awarded the Sir William Osler Clinician Scientist Award previously.

Dr. Rutka is the chief mentor for the the Peter and Melanie Munk Foundation Fellowship in Otology/Neurotology and is the co-director of the UHN Center for Advanced Hearing and Balance Testing and the Hertz Multidisciplinary Neurotology Clinic. Dr. Rutka lives in Toronto with his wife Marilena.

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Dear Dr. Rutka: Judy’s Response to the Doctor About Her Treatment

joanne — Sun, 28 Apr 2019 21:24:17 +0000

Dear Dr. Rutka: Judy’s Response to the Doctor About Her Treatment joanne Sun, 04/28/2019 - 17:24

In her reply to Dr. John Rutka, Judy Haust follows up with the results of her ongoing treatment.

Dear Dr. Rutka,

John, you are noted amongst your colleagues for the comprehensive letters you send to your patients, painstakingly explaining their diagnoses and the various treatment options available to them. I was so grateful to receive one of those letters going on six years ago! At my follow up appointment with you in early 2014 to discuss the initial MRI results, you pointed out that the good news was that my tumour was benign (the detected mass in my brain being the bad news!) and what a huge relief that was for me! Also, because my tumour was relatively small, I considered myself fortunate to have options in terms of which treatment to choose.

Judy Haust and Dr John Rutka

Nevertheless, as many AN patients in this dubious position soon realize, having options is somewhat akin to sitting under the legendary Sword of Damocles (to borrow a metaphor used by your fellow UHN neurosurgeon, Dr. Michael Tymianski, at the 2016 ANAC Symposium): with our great fortune comes great responsibility and risk! Regardless of which option we choose, we are never guaranteed a positive outcome, nor will our quality of life necessarily improve. As you mentioned in your recent letter to me, “Sometimes less is better in medicine.”

Navigating the world of Acoustic Neuromas is an on-going journey of discovery. In the early stages, I felt very much like a pinball bouncing from one so-called “no brainer” decision to another... first choosing the translabyrinthine approach, then retrosigmoid approach and, eventually, Gamma Knife. (You may remember that I wrote my full story within an article entitled, June Kudos, in the Summer 2016 ANAC newsletter.)

As you know, during the first two years after diagnosis, my tumour grew quite rapidly by approximately one centimetre (.5 x 1.1 to 2.1 x 1.4). To avoid having my tumour make the decision for me if it were to grow much larger, I finally decided on Gamma Knife surgery and was booked for an appointment at TWH at the end of June 2016.

Miraculously, the planning MRI just prior to that surgery showed that the tumour had stopped growing! Upon my asking if a reprieve would be possible, you granted me a “stay of execution”, so long I was comfortable with that decision. Is a banana bent? My radiosurgery was cancelled, and I felt as if an elephant had suddenly stepped off my chest! Since that time, my tumour has been stable, as determined by the semi-annual MRI scans you order. I can't help but think of the proverb, "Good things come to those who wait." Obviously, this approach isn't for everyone but, so far, patience has worked well for me.

Your recent letter reinforces this resolve to continue to embrace the Wait and Scan approach of conservative management. Who knew that my rapidly growing tumour would stop growing two years later! I’d forgotten your story from April 2005 at the North American Skull Base Society meeting in Toronto where, at the beginning of your presentation, you polled the (mostly) surgeons about what they would recommend for patients with small ANs... traditional surgery, of course, according to the majority. Then, after presenting them with the (then) seven-year longitudinal findings of your ten-year prospective study of patients presenting with small tumours, you polled them again, saying “this time the patient is you”. Fascinating outcome! That story warrants repeated telling.

When I learned several years ago that you sat on ANAC’s Medical Advisory Committee, I delved further into what the association had to offer, soon becoming a member and attending the amazingly helpful June 2016 ANAC Symposium. I also started participating in the invaluable Toronto Chapter group support meetings. It wasn’t long before I decided to become even more actively involved by accepting an invitation to join ANAC’s Board of Directors. I’ve never looked back and continue to be so appreciative of your guidance and care. I look forward to continuing this journey of discovery together!

Respectfully, and with many thanks,

Judy

Trekking to the 2018 Symposium: Elizabeth’s Pathway to Managing Her AN

joanne — Tue, 05 Mar 2019 22:17:50 +0000

Trekking to the 2018 Symposium: Elizabeth’s Pathway to Managing Her AN joanne Tue, 03/05/2019 - 17:17

Elizabeth Ewashkiw from Belleville, Ontario has been working hard to minimize her symptoms with the help of a vestibular therapist.

Elizabeth Ewashkiw

As an active, 74-year-old retired kindergarten teacher, I consider myself an engaged member of the community. Medically, I have keratoconus which is a progressive eye disease in which the normally round cornea thins and begins to bulge into a cone-like shape. This cone shape deflects light as it enters the eye on its way to the light-sensitive retina causing distorted vision, for which I have had three corneal transplants over the past 40-plus years culminating in an unsuccessful trabeculectomy for glaucoma. This has left me with no vision in one eye and a scleral lens in the other.

Having gradually adjusted to the lack of depth perception and minor balance issues, I was therefore not surprised when I seemed to develop vertigo a few years ago. I was careful getting into boats (we cottage on an island, so this is significant), started holding hand rails when possible, and got on with life.

In January 2018 after my flight home from BC, I experienced sudden onset deafness in one ear and secured an appointment with our ENT in Belleville. Also, after the flight to Europe in May 2018, I had balance issues and needed to take my husband's arm for the whole trip. The vertigo gradually settled down somewhat after we were home again.

After waiting for an appointment, waiting for an audiogram, waiting to have my ear cleaned out, taking time to cottage in the summer, and having an MRI, I was eventually diagnosed at the end of August 2018 with a small acoustic neuroma. It was not until November, when I had a second audiogram, that I was referred to Dr. Joseph Chen at Sunnybrook Health Sciences in Toronto.

My way of coping with the unknown is to learn all I can, so I turned to the search engines on the Internet looking for recognized national sites about acoustic neuromas. I soon learned that there was an acoustic neuroma association in Canada and, lo and behold, they would be holding a national symposium in Toronto at the end of September.

Living two hours away, I registered for the live webcast of the 2018 Symposium and sent a note. I immediately received a phone call from Carole Humphries, whose knowledgeable, warm and supportive manner, was exactly what I needed. Carole encouraged me to come to Toronto to experience the conference in person. The networking around our table, the fourteen pages of notes I took, the fact that the room was full of people with my symptoms, including some who had had surgery, plus the presenters making themselves available for brief chats during our refreshment breaks made this an invaluable day. I was able to introduce myself to Dr. Chen as a probable new patient and he immediately told me to discard the cane, use walking poles, and start walking, looking left and right continuously.

Elizabeth walking as part of her rehabilitation

I gathered later that this was to help my brain adapt to receiving balance information from my one set of circular canals, rather than the usual two.

The conference also gave me the opportunity to speak to a vestibular therapist whose expertise I realized I needed.

Arriving home in full-blown overload, I emailed my physiotherapist and the manager/friend of the clinic I had used in the past. I learned that my present therapist was also qualified in vestibular therapy. I started immediately, as I continued the twice-a-week free VON seniors' exercise classes, which contain a balance component. Homework for the therapy became mandatory as I practised walking a straight line, gradually moving my arms, and eventually I expect to be able to change elevation and focus (by bending knees, raising heels, rotating shoulders, etc.). This is only one of the multitude of exercises I have learned in recent months. If I neglect to maintain them, my balance is compromised. The rewards are built in. If I work at it, I experience success. If not, . . .? I get right back to practising!

By mid November 2018, I had an appointment with Dr. Chen, who reassured me that my small (0.6 x 1.5 cm) neuroma only needs monitoring every couple of years with an MRI, which I can have done in my home town of Belleville.

I have "graduated" from vestibular therapy but am most welcome to return when I deem it necessary and will continue to participate in two VON one-hour classes per week.

I had also learned about the CROS hearing aids when at the ANAC conference. Messages from the deaf ear are transmitted wirelessly to the functioning ear, from which the info is sent to the brain. When home, I went to learn about hearing aids. After another audiogram I asked what would be appropriate for me. The answer was the CROS. These I received on December 4. I certainly hear more with them (they look just like any other hearing aids, i.e. inconspicuous), but I do get caught out by the lack of directionality. If in a crowded room I hear a voice calling my name, I must rotate, checking people's facial expressions, to tell who has spoken to me! Similarly, around a table of unfamiliar people at a meeting, I don't automatically know who spoke. It all just adds another layer to "paying attention" to make it all work.

Presently I don't have to explain to people that I have balance issues or hearing in one ear only because I've learned how to compensate as much as possible. This is my new normal and it's working for me.

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What is a Vestibular Migraine?

admin — Tue, 18 Dec 2018 22:50:32 +0000

What is a Vestibular Migraine? admin Tue, 12/18/2018 - 17:50

Claire Sissons / Reviewed by Elaine K. Luo, MD

Many people who experience migraines also have feelings of dizziness or vertigo. When this happens repeatedly, it is known as vestibular migraine. The causes of vestibular migraine are not always clear but relate to the inner ear, nerves, and blood vessels. The vestibular system of the inner ear and brain controls balance and how people understand the space they are in. When this is affected, someone may experience feelings of vertigo, unsteadiness, or dizziness, which can be triggered by movement.

Vestibular migraine is diagnosed when the vestibular system is repeatedly affected, in episodes lasting for minutes or hours, in someone who has a history of migraine. The sensation may be experienced alongside other migraine symptoms, such as an intense headache or nausea, or on its own. Around 40 percent of the people who suffer from migraine also have vestibular symptoms.

Symptoms

Key symptoms of vestibular migraine are dizziness, vertigo, and difficulties with balance, but symptoms can also include: neck pain, discomfort turning, bending down, or looking up, feeling of pressure in the head or the ear, tinnitus, partial or complete loss of vision and visual disturbances, such as flashing lights, spots, or blurring.

These symptoms may appear alongside a headache but can also appear on their own.

Causes, Triggers and Diagnosis

The causes of migraine are not completely understood. They are likely to relate to an unusual electrical charge in the neurons that sets off the brain's pain receptors. Triggers may vary from person to person. Keeping a record of factors leading up to a vestibular migraine can aid a diagnosis and help avoid an episode.

Common triggers include: stress and anxiety, food or drink, such as caffeine, alcohol, or dairy products, lack of sleep or too much sleep, bright artificial lights, and hormonal changes.

Treatment and Living with a Vestibular Migraine

Eating a healthful diet; getting the same amount of sleep each night; trying to reduce stress; exercising regularly; and avoiding any food or drink that can be a trigger help manage the condition. During a migraine episode, many people will find that lying down in a dark room or sleeping can help. Taking over-the-counter pain or nausea-relief medication at the first sign of migraine may reduce the severity of the episode.

Vestibular rehabilitation may help with regular or particularly bad episodes. This treatment can include exercises to stabilize the gaze and improve the ability of the eyes to track movement. It can also incorporate tasks to improve balance and hand-eye coordination.

Lifestyle changes, consulting a specialist, preventative medication and avoiding triggers can contribute to reducing the number of vestibular migraine episodes for many people. Medication is also available to help if vestibular migraine is severe and happens regularly enough to interfere with a person's life.

Elaine K. Luo, MD is a board-certified internal medicine physician who graduated from Tufts University School of Medicine in Boston, Massachusetts. She has experience in utilization management and has worked as a hospitalist and an outpatient primary care provider.

treatment

What a Difference a Day Makes! Ruth's AN Journey

joanne — Wed, 05 Dec 2018 22:08:08 +0000

What a Difference a Day Makes! Ruth's AN Journey joanne Wed, 12/05/2018 - 17:08

Ruth Toskas was in her 40s when she was diagnosed with a large acoustic neuroma.

Ruth Toskas

What a difference a day makes! I have heard this little saying many times in my young 49 years of age and I can certainly hum the tune as Dinah Washington croons these words in my head, but I didn’t realize the impact these few little words and meaning would have on me, until this year. More to come on this later in my story . . .

I awoke one night in September 2016 with a loud ringing in my ear and that’s where it all started. I won’t bore you with all the details of my various doctors’ appointments, hearing tests and ultimately the confirming MRI, but fast forward to February 2017, a Valentine’s Day that I will never forget, where I was diagnosed with a left-sided acoustic neuroma. I couldn’t believe the words I heard from the ENT that I have a “rare brain tumour”. I have two young daughters and they were my first initial worry and how this news and outcome would affect my family. I was devastated. I tried to carry on every day as normal as possible but at the end of the working day and when my family went to sleep, that’s when I would really think about it and I admit, I cried myself to sleep many nights.

My husband, Demetrios, and I spent a lot of time researching what an acoustic neuroma is and by the time I initially saw a neurosurgeon in my home town of Hamilton, Ontario, I was prepared with a list of questions. Unfortunately, my appointment did not go as well as I had hoped, and I was left with more questions. I did however find out that my tumour was on the larger side, being almost 3 cm, and that my brainstem was being compressed.

I continued reading and researching everything I could about my AN. I reached out to close family and friends and those in the know in professional circles. I had decided that I also wanted to explore other options such as advanced radiation techniques. After a couple of meetings, including a much appreciated one by a close connection, my quest eventually led me to Dr. G. Zadeh’s name at Toronto Western. I politely insisted for a referral from my family doctor who gracefully indulged my request to see Dr. Zadeh. I had also found the Acoustic Neuroma Association website and sent a quick email. I immediately received a call from Carole Humphries and felt, for the first time, that I was not the only person in the world dealing with this kind of tumour.

I met with Dr. Zadeh and knew immediately she was the doctor I was looking for. Dr. Zadeh exuded competence and confidence in a humble way and she understood our concerns and addressed them in a graceful, straight-forward, sincere manner. Dr. Zadeh‘s wealth of experience and knowledge and her calmness was exactly what I needed. I knew surgery, as scary as it seemed, was probably going to be the road I needed to take but, in the meantime, I still had a couple of appointments booked to explore radiation options. Ultimately, after weighing all the options and because of the size and location of the tumour, radiation was not the right choice for me. After meeting with Dr. Zadeh again, it was agreed that surgery was the best option for me. I waited for the date and surgery was scheduled for February 2018.

Almost a year to the day that I was diagnosed, the surgery day was upon me. For almost a year, I had tried to stay as positive as I could but there were days that the worry, stress and anxiety got the better of me. Although he hid it from me, I knew it took a toll on my husband, but he remained supportive and reassuring. I did, however, surprise myself that I was relatively calm the morning of surgery. I know that had to do with the fact that I was very confident in my neurosurgeon, Dr. Zadeh, and her very skilled surgical team, plus I had done everything I could do to prepare myself and be ready for this day. With my husband by my side, I was prepped to go and then rolled into the operating room.

The surgery was very successful. It was a long, almost 10-hour, surgery. When they woke me up in the operating room, I was told to smile and wink and told all looked good. I was taken to the ICU to be monitored. I had many tubes and wires hooked up and then of course the nausea set in. Needless to say, it was an incredibly rough night.

Well, back to the beginning of my story and what did I mean by that little phrase? The next morning, after only a couple hours of sleep, I felt different. Sure, there was the expected recovery, the risk of brain fluid leakage, which did occur, and other risks which did not. What I really mean by that is it was a new day, surgery was now done and all that worry, stress and anxiety I had leading up to the surgery, was now behind me. So yes, what a difference a day makes.

I lost my hearing on my left side completely and the tinnitus is still as annoying as ever. There is also some residual tumour which we will keep an eye on with periodic MRIs and follow ups. These of course are things that I expected and was prepared for, so I feel so very grateful.

I am still adjusting to a world of single-sided deafness and the fatigue that still hits me after I have taken on too much is still a gentle reminder of what I have been through.

My husband has been my biggest advocate and supporter during this journey. I can’t thank him enough and am blessed to have him by my side. We are also very proud of our young daughters as they handled this with such grace and positivity. We are all adjusting to the new us. As many have said before me, the love and support from family and great friends is truly a blessing. I have also made some new friends at the AN support group throughout this journey and they too have similar stories to share. They have given me great advice and insight with their journeys which

was appreciated, as it has helped me understand my journey and recovery. I am honoured to also be there to lend an ear (well at least my one good functioning ear) to newly diagnosed patients and hope sharing my story with them can help in some small way.

If there is one thought I can share with anyone going through this, it is this: Stay positive and surround yourself with support -- you will get through this and you are much stronger than you think you are!

Current and Future Treatment Modalities For Acoustic Schwannomas

admin — Tue, 31 Jul 2018 21:28:12 +0000

Current and Future Treatment Modalities For Acoustic Schwannomas admin Tue, 07/31/2018 - 17:28

Acoustic schwannomas (also known as acoustic neuromas or vestibular schwannomas) are a relatively rare non-cancerous tumour that arises from the vestibulocochlear nerve. These tumours grow slowly due to the over proliferation of Schwann cells, which normally wrap around nerves to provide support and insulation. Most commonly, patients present with hearing loss, sensation of fullness in the ear or ringing in the ears. In addition, the vestibulocochlear nerve runs through the cerebellopontine angle alongside important cranial nerves, blood vessels and the brainstem. Large tumours can compress these nearby structures and cause additional symptoms, such as facial paralysis and balance problems or unsteadiness. Once the preliminary diagnostic procedures including hearing tests (audiometry) and computed tomography (CT) scans are completed, the gold standard for diagnosis of acoustic schwannomas is magnetic resonance imaging (MRI) scans, which help determine the location and the size of the tumours.

Treatment options for acoustic schwannomas can range from clinical observation to surgery or radiotherapy. The treatment for acoustic schwannomas depends on the size of the tumour, patient’s general health and preferences. For some small acoustic schwannomas with minimal symptoms clinical observation is acceptable, especially in elderly patients or patients with medical comorbidities where the risks of therapy may be greater. Surgery can often remove the tumour completely, through a suboccipital, translabyrinthine or middle fossa approaches. The goal of surgery is the removal of the tumour and the preservation of the facial nerve, and to preserve hearing when possible.

Another option is radiosurgery, where focused radiation is applied to the tumour to reduce the size or limit the growth of the tumour. It often takes months before the effects of radiosurgery are evident on the tumour. For tumours that are less than 3 cm in size, both surgery and radiosurgery are an option, with similar efficacy. For larger tumours, surgery is the only option. The risks of treatment include hearing loss, ringing in the ears, facial weakness/numbness, balance problems and treatment failure (continued growth in the case of radiosurgery).

To date, there are no effective medical treatment options established for the treatment of acoustic schwannomas. The limited understanding of the molecular drivers of tumour development, in part, is a reason for the dearth of drug development. Our laboratory is focused on uncovering the genetic alterations that lead to the growth of acoustic schwannomas.

We published a study in Nature Genetics (2016) characterizing the genomic landscape of sporadic schwannomas, which identified mutations in NF2, ARID1A, ARID1B and DDR11. Most notably, we identified a novel genomic rearrangement resulting in a fusion gene SH3PX2DA-HTRA1 in a subset of schwannomas that was demonstrated to increase the growth of these tumours. Experiments in our lab showed that MEK inhibitors, a class of targeted therapies, is a potential therapeutic option for the subset of schwannomas with the gene fusion. This study highlights the possibility of developing targeted therapies for acoustic schwannomas based on preclinical studies focused on understanding the tumour biology.

Clinical trials studying the efficacy of a subset of targeted medical therapies are in their infancy. Bevacizumab has been demonstrated to prolong tumour stability and prolong time to hearing loss in two clinical trials2. Based on these trials, the Congress of Neurological Surgeons (CNS) have recommended the use of bevacizumab in patients without surgical options. In another study, a receptor tyrosine kinase inhibitor, acting on epidermal growth factor receptor, demonstrated significant reduction in tumour volume in 23.5% of patients, with a median response time of 4.5 months3. However, not all clinical trials have demonstrated positive results4. There are currently four clinical trials studying targeted medical therapies in acoustic schwannomas.

In an era of precision medicine, the most difficult step is translating bench-side laboratory research into clinical practice. The ongoing basic scientific research has identified potential molecular pathways and targeted therapies that could prove to be beneficial in treating acoustic schwannomas. However, current data from clinical trials has demonstrated mixed results with these targeted therapies. More work is needed to characterize the biological subgroups of acoustic schwannomas that are driven by different genomic alterations. One possible explanation for the mixed results in the clinical trials is that only a subset of tumours with specific genomic alterations respond to the specific targeted therapies. Further concerted effort is required from both biologists and clinicians to develop targeted therapies for treatment of acoustic schwannomas.

References:

Agnihotri, S. et al. The genomic landscape of schwannoma. Nat. Genet. 48, 1339–1348 (2016).
Blakeley, J. O. et al. Efficacy and Biomarker Study of Bevacizumab for Hearing Loss Resulting From

Neurofibromatosis Type 2–Associated Vestibular Schwannomas. J. Clin. Oncol. 34, 1669–1675 (2016).
Karajannis, M. A. et al. Phase II trial of lapatinib in adult and pediatric patients with neurofibromatosis

type 2 and progressive vestibular schwannomas. Neuro-Oncol. 14, 1163–1170 (2012).
Plotkin, S. R. et al. Erlotinib for progressive vestibular schwannoma in neurofibromatosis 2 patients.

Otol. Neurotol. Off. Publ. Am. Otol. Soc. Am. Neurotol. Soc. Eur. Acad. Otol. Neurotol. 31, 1135–1143 (2010).

Dr Zadeh

Gelareh Zadeh, MD, PhD, FRCS(C), FAANS; Wilkins Family Chair in Brain Tumour Research, Co-Director Krembil Brain Institute, Associate Professor, University of Toronto & Scientific Medical Chair, ANAC.

Dr Radovanovic

Ivan Radovanovic, MD, PhD, FMH;Scientist, Toronto Western Research Institute, UHN & Assistant Professor of Surgery, University of Toronto.

Dr Suppiah

Suganth Suppiah, MD, PhD Candidate; Resident Physician, Department of Neurosurgery, University of Toronto

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