Acoustic Neuroma: Treatment Planning

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1 Acoustic Neuroma: Treatment Planning Recorded on: June 12, 2012 Andrew J. Fishman, M.D. Otologist/Neurotologist and Skull Base Surgeon Northwestern Memorial Hospital Stacey Bock Please remember the opinions expressed on Patient Power are not necessarily the views of Northwestern Memorial Hospital, its medical staff or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That s how you ll get care that s most appropriate for you. Stacey s Story An acoustic neuroma is a tumor of the nerve that connects the ear to the brain. The tumor grows slowly and is benign, not cancerous, but it can damage other nerves as it grows in size. Coming up, an expert from Northwestern Memorial Hospital in Chicago explains strategies for treating this condition, and his patient shares her story. It s all next on Patient Power. Hello and welcome to Patient Power sponsored by Northwestern Memorial Hospital. I m Andrew Schorr. We re going to talk about a benign tumor that is in the brain, by the ear. It s called acoustic neuroma, and we re going to understand how variable it can be among people, how does it happen, how do you test for it, how do you see if it s growing, and when is surgery indicated and how is surgery done. And let s start by meeting someone who has gone through surgery for this. That s Stacey Bock. She s 55, lives in Orland Park, Illinois, about 35 minutes south of downtown Chicago. She has three children, two girls and a boy, and she s grown up really over the years, about 30 years now, has dealt with multiple sclerosis. But, Stacey, let s go back a couple of years. You would regularly go to your neurologist because of the MS and have MRIs just to follow that, correct? That s correct.

2 What was spotted two years ago? Two years ago they determined that there was a growth behind my ear on the right side of my head that appeared to be a possible acoustic neuroma. All right. Now, you have a dear friend, Linda, who is an audiologist, and so you had your hearing tested with Linda, right? Yes. I had had it tested probably two years prior to that MRI. Right. And your hearing was okay. Yes, it was. But then time goes on. You have a teenage daughter, right, and I understand you couldn t hear as well. Now, some might say if you can t hear a teenage daughter, so, well, that s okay. But you noticed that your hearing, you felt was deteriorating, right? Yes, I noticed that she was particularly hard to hear, but there were just some things like the TV. There seemed to be things I was missing, so it became more apparent to me that I had some hearing loss. So you go back to Linda, your friend who is the audiologist. Right. And the test showed what? 2

3 It showed significant hearing loss, and that kind of, as I understood it, was indicative of the tumor growing. And in fact you had another MRI and that showed growth in the tumor, right? Yes. All right. So then you say, well, what do you do about it? Eventually that brought you to Northwestern Memorial and the specialist we re going to meet in a minute, Dr. Andrew Fishman. But what ended up being discussed was surgery, and you decided in November of 2011 to go ahead and have surgery, right? That s exactly right. Now, why did you want to have surgery? Was it the concern that you had this tumor in your head was that growing even though it was benign, it was growing and you were just concerned about what could be next? Well, that was it in part because I think just something foreign in your brain is a little bit unnerving, and also the fact that it seemed like it was quite possible that I might need the surgery at some point down the line, and my thought was, I m 54 years old at the time, I m in relatively good health, I had good insurance, and why put it off? And so you had the surgery. Now, one of the things that can go along with that is losing your hearing, and you did, in your right ear. Yes. 3

4 But you have some confidence now and we re going to meet your doctor in a minute that the tumor was taken out. Yes. That was the whole reason to do it. The sacrifice of the hearing, while it s a pretty big deal it seemed like the lesser of the two evils. Right. Well, thank goodness we ve got two ears, right? Two eyes and two ears. Exactly. Acoustic Neuroma Signs and Symptoms We re going to be back to you in a couple of minutes, but let s meet your doctor, and that is Dr. Andrew Fishman. Dr. Fishman is what s called an otologist/neurotologist and a cranial base surgeon at Northwestern Memorial, and in fact Stacey s doctor. First of all, Dr. Fishman, help us understand what is an acoustic neuroma. You know, you hear of tumor and you think well, cancer, but that s not what this is. But still, it may need intervention, we ll talk about the different kinds, but what is an acoustic neuroma? An acoustic neuroma is a benign tumor that originates from the coating of the nerve, from the hearing nerve, and it typically originates from one of the branches which is responsible for balance. So another term that is used for acoustic neuroma is vestibular schwannoma. You know, for practical purposes those terms are interchangeable. The hearing nerve which goes from the brain to the cochlea and the balance canal system branches off into one component that is going for the hearing part and another component which has two branches which go to the balance canal. And it s usually in about 90 percent or greater of cases one of those balance nerves which grows the tumor. And it is a benign tumor. Now, do we have any idea what causes this? Why in some people and not in others? 4

5 We don t. There s some speculations, and we hear often in the media about cell phones and all of that but the answer is not in on that, and it s really speculative. But there are two main categories or types of acoustic neuroma. One is what we use the word sporadic, which simply means it just happens, and it happens to be one of the most common tumors of the central nervous system. There are patients that have multiple or an acoustic neuroma on both sides, and they have a genetic-mediated process called neurofibromatosis type 2. But that s a whole other subject matter, and the decision-making for treatment of those patients is quite complex, while in Stacey s case she has what we call sporadic or single acoustic neuroma. All right. We don t know how it happens, but in her case she was being followed for MS with a MRI, so they spot it, and then she begins to have some hearing loss. So what s the tip-off to an acoustic neuroma? Would hearing loss be it? I mean, not everybody has a brain MRI, so how would you know if you even had it? Well, there are a few early signs and symptoms of acoustic neuroma. The most common is hearing loss because the balance and hearing nerve are very close together. And one of the other symptoms can be some dizziness, some vertigo, or some balance issues. Now, in Stacey s case it was incidentally found before there were any real substantial hearing loss symptoms, and that s actually a fairly common scenario given today s climate of frequently having MRIs for other problems. Now, what about headache? Would that come up? It would not be a classic feature of a small- to medium-size acoustic neuroma. However, if an acoustic neuroma grows to a quite large size then it could compress the brain and cause some swelling and even cause some backing up of the spinal fluid and a term we call hydrocephalus. Now, that would be in more advanced or larger presentations, and there could be some headache involved in that. But for a small- to medium-size tumor it s typically painless. Ringing in your ears? 5

6 Yes. That is another symptom that could be indicative of an acoustic neuroma. So we frequently see patients that pass through the clinic with ringing in their ears, and if there s ringing in a single ear then that should be considered a potential sign and a work-up is indicated in that case. Testing and Treatment Let s talk about the work-up. So Stacey was seeing her friend Linda, an audiologist, and testing her hearing and confirming the hearing loss, and she was also having MRIs. Are those the tests that are usually brought to bear? Yes. The MRI is the gold standard imaging study to detect the presence of an acoustic neuroma. It s usually done as an outpatient. It involves an injection of an intravenous contrast agent, and it s as close to 100 percent accurate as possible. The hearing test is very important because the level of the hearing is a major determinant in the decision-making of the treatment plan. Yes, we re going to talk about that in depth, but I understand that the situation can be very variable. Help us just at the 10,000-foot level first. What are the variables to consider? Well, you know, the first thing we need to take into consideration are the broad categories of treatment options. There are three main treatment options for any given patient at any given time, the first being observation, or to simply repeat the imaging. Because we know it s a benign process and it can be slow growing, it s reasonable in many instances to observe with serial imaging at a set time interval, usually a number of months to a year or so. We have, one, observation. Another treatment option is surgical removal, and there are a variety of approaches which we can get into a little bit later. And then the third is stereotactic radiation. Now, in order to determine a specific treatment plan for a given patient, we look at some of the symptom-specific factors like hearing and dizziness or vertigo. We also look at the tumor-specific factors, which include size, or change in size, as well as location along the nerve, for example, how close to the brain stem or how close to the cochlea the tumor is. And then we look at patientspecific factors which include the age of the patient and also risk for surgery. But ultimately there 6

7 are a couple of choices that can be pursued by any given patient, and then we factor that into the final plan as well. All right. So that argues for the patient who has been diagnosed with acoustic neuroma to be in touch with a specialist such as yourself where all the options, broad experience are on the table, and then some of it is, I think, what you call the factor of time is watching, too. Because, as you said, often these are very slow growing, right? Yes. And are there some people where this just it got to a certain point? Does it ever just sort of level off, if you will. Yes, you have an acoustic neuroma, but, no, your life is not really being affected, and just go on with your life? Well, that actually intersects with the factor of age because if we take a patient of more advanced age there is a lower probability that in their lifetime that even some continued slow growth of the tumor could potentially cause them no harm at all. But if we take patients of younger age, then we look at the situation and say, well, if we let this grow throughout next 30, 40 years eventually it s going to cause some ill effects from compression of the brain, so in that case it argues for either surgery or some means of either stopping it or treating the tumor. Right. We re going to talk about that after the break, but just taking what you just said and applying it to Stacey. So Stacey at the time was 54 years old, we hope, Stacey, you re going to live to be a hundred, so we give you a lot more years, and there was growth going on and hearing loss going on, so you ll explain to us how as these tumors get bigger the surgery maybe gets more intricate and where you d rather do it earlier than later. And, Stacey, I know that was your option, right, Stacey? Yeah. Okay. We re going to talk more about that as we learn in depth now the various treatment strategies and surgery and surgical approaches for acoustic neuroma. It s all coming your way as we continue Patient Power right after this. Welcome back to Patient Power. Andrew Schorr here with another Andrew, Dr. Andrew Fishman, who is an otologist/neurotologist at Northwestern Memorial Hospital. He s a cranial base surgeon, and he helps people who have acoustic neuroma, people like Stacey Bock from Orland Park in 7

8 Illinois who had surgery in the fall of 2011, and now as we record this program in 2012 she is doing well. Dr. Fishman, so acoustic neuroma, first of all, is a tumor. People hear that and they think, oh, my god, I have a brain tumor, and they think of a cancerous brain tumor, and, as you know, that diagnosis is often a very tough one. So, first of all, how do you doctors know that it s not malignant? Is it just looking at it in the MRI you can tell, and where it is? Yeah. The acoustic neuroma has a very typical appearance on the MRI. It has a smooth outline. The location of it, generally the appearance of it signifies that it s a benign process. Hearing Preservation vs. Hearing Sacrifice Surgery All right. So still there s the question of do you cut it out, do you radiate it. So talk to us about that. So in Stacey s case her hearing had deteriorated, and the concern was as you alluded to a minute ago that if the tumor grows, and hers was growing, that there could be more complications down the road, and that would argue for surgery earlier which I would think if the tumor is smaller might be less complex. But take us through that. Right. When we look at the hearing level we sort of put patients into different categories. There are some patients where the hearing is still very good, and if the hearing is still at a very high level then there are some approaches that can be used to try to preserve the hearing that is still there. They differ technically from the approaches that are used when the hearing is deteriorating or poor. The broad category of those different terms is typically referred to hearing preservation versus hearing sacrifice surgery. Now, there are some technical advantages to using the translabyrinthine approach, which is the approach that we used on Stacey because the hearing was poor. And what that does is it is an approach which goes directly through the balance canal system and allows us to reach the facial nerve, which is a nerve that gives motor function or movement to the face, on two ends. It allows us to access it on both sides of the tumor. And the advantage of that is that we can very easily control and peel the tumor off of that nerve directly with full visibility. So that s why we chose this particular approach for Stacey because the hearing preservation was less of a concern because of the degree of the hearing at the time. 8

9 Let me see if I ve got that right. So her hearing was going downhill anyway, and so by agreeing with her to go ahead and cut out the tumor in the most efficient way to give you the highest degree of success, then you knew that that would affect hearing totally in that ear, but it would give you the best chance of getting all the tumor. Is that right? Exactly. And the recurrence rate for a translabyrinthine removal is also the lowest because the cells where the tumor originates are situated somewhere laterally out in the outer part of the internal auditory canal, and through the translabyrinthine route we have the fullest view of the internal auditory canal to remove all those originator cells. And just so our audience understands, we re talking about microsurgery, right? Yes. I mean, these are tiny, tiny areas, so when you re doing this you re looking in a microscope, and the movements you re making are just tiny. Essentially, yes. We re working under a high-power microscope and using little pick instruments that look like needles and small little spatulas that are about a millimeter or two in size. And we also use little micro drills to remove the bone that range in size from a half millimeter to four or five millimeters in diameter. Enlarged Tumors Wow. Now, the concern would be, and again you alluded to this earlier, if you wait longer how does somebody go downhill, if you will? If you wait longer, not only is the surgery more difficult but what would be the symptoms that someone might be experiencing if this tumor continues to grow? That s a very good question. The hearing loss and the changes in hearing are not always in direct correlation to the size of the tumor. So what I mean by that is that you can have a relatively small 9

10 tumor with a relatively sudden drop and poor hearing, or you can have a tumor that gradually enlarges and doesn t reduce the hearing as much. There s not a direct correlation. So the main concern is that as the tumor enlarges we have more work to carefully remove it from the facial nerve, the nerve that gives movement to the face. And as it enlarges even further it then starts to lean up against the brain stem, and all the fine blood vessels that are in that area which also control other functions like movement of arms and legs. Wow. So this would argue for if in fact this is diagnosed in someone that early and sort of an ongoing dialogue with a specialist such as yourself makes sense so that you intervene in the best possible way at the best possible time. Multidisciplinary Approach to Treatment Exactly. You know, the team that typically takes care of a patient with an acoustic neuroma is multidisciplinary. We have surgeons like myself who are neurotologist cranial base surgeons. I collaborate with vascular neurosurgeons. We also collaborate with radiation oncology specialists. And then there are audiologists that are involved in the testing, and there are even physical therapists and physiatrists involved in the rehabilitation afterwards because there may be some balance and dizziness issues that will take some time to resolve. It sounds to me like the team that s required is not on every street corner. You re of course at one of the major medical centers in the country, and that s a good thing for this condition. Yes, I would agree with that. All right. So help me understand. You mentioned about radiation for a minute. So sometimes would a patient have radiation as well as surgery, or maybe some radiation might be done at some point and surgery later, or how do those come together? You know, that s also a very good question. Now, it is used actually in all the ways that you mentioned. There are patients that have very large tumors, and it is determined that the risk of total removal would put some of the critical structures at undo risk, and those are decisions that 10

11 are often made intraoperatively, at the time of removal. In those cases we typically observe any residual tumor, and then if we note growth of those residual segments of tumor, especially if they ve been in the location of critical structures like the brain stem or the facial nerve, then some focal stereotactic radiation can be used to try to slow or stop the growth. There are other patients, and this we would tend to favor in patients of more advanced age, where just stopping or slowing the growth of the tumor is desirable, and no surgery is utilized, but instead delivering a dose of radiation to the tumor can be effective. What I m hearing is again individualized care for what s right for that patient and their specific situation. Absolutely. Every factor has to be looked at. You know, not just the age, not just the hearing, but the occupation, the lifestyle, all of these factors play in, and it can be a quite complex process to come up with an exact treatment plan for a patient. And often patients can be presented with a few options, with a balance between the advantages and the disadvantages of those, and they ultimately make the decision by comparison of options. Now, you mentioned about this, did I get it right, translabyrinthine approach? Yes. Did I get it right? Yes. And you said it has very good statistics as far as negating or lowering the need for later surgery. Yes. Recurrence 11

12 But let s talk about that. Generally when you do surgery how often is it that you have to do something again later, whether it s a year later or two years or months later? I mean, is that something people need to be prepared for? It s uncommon. It can happen, but it s not typical. The translabyrinthine approach, especially if it s utilized for a small to medium tumor is typically curative, with the recurrence rates in the single digits. Now, there are some other approaches. One is typically referred to as a retrosigmoid. It also is a modification of an older approach, which is termed suboccipital, and those approaches go not through the ear itself but behind that area by making a formal craniotomy or opening into the skull and then taking the tumor out from that avenue. The recurrence rates in those areas are slightly higher because of a less complete opening of the internal canal where the nerves transmit. All right. So if somebody is talking to a surgeon such as yourself part of it is what approach do you favor that can give me the best chance of you being able to take out all the tumor and hopefully then I can go on with my life, right? Right. However, we do have to factor in that with some of these other approaches we can scoot around the inner ear and save some of the hearing in certain patients if it s a reasonable thing to do. Now, there s the retrosigmoid approach. There s also a retrolabyrinthine approach that goes around the back of the inner ear, but that s limited for tumors in a very specific location. And then there s another approach called a middle cranial fossa approach where we open up a craniotomy above the ear and take the tumor out from the top. But each of these approaches is for a very specific size, shape and location of the tumor. Okay. Stacey, I m sure as you listen to this, as I do, you say Dr. Fishman is really a super specialist in this area, isn t he? He knows what he s talking about, that s for sure. Closing Thoughts 12

13 Stacey, let me ask you, so you decided to go ahead with concern that if you didn t there would be like another shoe that would drop, some other effect on your life. So for someone who is listening, who is saying, I ve been diagnosed with acoustic neuroma, what would you say, not necessarily whether to have surgery or not but as far as getting expertise to make a decision? What would you urge them to do? Well, I think the first thing I would urge them to do is not panic, because I think you hear acoustic neuroma and you see someone and they I guess the long and short of it is that it is brain surgery, which I think initially sounds very frightening and involved. And not to diminish it because it is frightening and it is involved, and certainly worth the effort and whatever. You know, initially I was really kind of nervous about it and whatever, and I checked on YouTube and saw disturbing videos with removals and all that, but I do think it s worth whoever would be diagnosed with this to get, you know one opinion or two and do what you feel most comfortable with. And keep in mind that if you re in good hands and if it s a surgeon that s respected and knows what he s doing then you re in a good place. And follow his lead, I guess would be my suggestion. Well said, and, Dr. Fishman, just about second and maybe even third opinions, you have no problem. If somebody sees you first and then talks to others or comes to you for a second opinion, you re fine with that, right? Oh, absolutely. Actually, I think Stacey did come to me originally as a second opinion. Mm-hmm. And often patients ask us for names of people to go for additional opinions and we always encourage that to get as much information as possible because this is a very complex decisionmaking plan, and one of the huge advantages of going through this process, especially with additional opinions, is to educate yourself and get as much information as possible. 13

14 Well, hopefully we have done that today. Dr. Andrew Fishman, neurotologist and cranial base surgeon, Northwestern Memorial Hospital, thank you for helping us understand better acoustic neuroma. Thanks for being with us, sir. You re welcome, and thank you. And Stacey, right, thank you. So we wish you all the best. We hope that your multiple sclerosis stays in a very low, relapsing-remitting way and you just go on with your life, and that you can enjoy hearing all the things you can with your left ear and with some confidence that that whole tumor has been removed. And maybe you ve got some weddings to dance at. I do, I do. Yeah, there you go. So have fun with that, Stacey. All the best to you. Thanks. I appreciate it. All right. Interesting topic, acoustic neuroma. And as we said and as the doctor said it s important for you, the patient, to get informed and so you can make an informed decision with a specialist such as Dr. Fishman or whoever you choose. I m Andrew Schorr. Thank you for joining us. Remember, knowledge can be the best medicine of all. Please remember the opinions expressed on Patient Power are not necessarily the views of Northwestern Memorial Hospital, its medical staff or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That s how you ll get care that s most appropriate for you. 14

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