Can You Hear Me Now? Understanding Meniere’s Disease with Habib Rizk, MD

Meniere's disease is an inner ear disease named after the physician who actually discovered it. The patients usually present with fluctuation of hearing loss, ringing in the ears, and episodes of dizziness characterized by a spinning sensation or an illusion of movement sensation that we call vertigo.

Dr. Habib Rizk is an Associate Professor in the Department of Otolaryngology, Head and Neck Surgery. Dr. Rizk completed his medical degree and residency training at St. Joseph University in Beirut, Lebanon, and went on to complete a fellowship at MUSC. Dr. Rizk is on the Board of Directors of the American Balance Society, a member of the Equilibrium Committee of the American Academy of Otolaryngology, as well as a representative of the Academy in a joint task force of the American Academy of Neurology to investigate quality improvement measures in neurotology. His research focuses on hearing-related and dizziness-related research.

Read The Transcript

[00:00:05] Lorette Lynch-Reichert: Hello, everyone, and welcome to our June Science Never Sleeps podcast. Today, our guest is Dr. Habib Rizk, Associate Professor in the Department of Otolaryngology, Head and Neck Surgery. Dr. Rizk completed his medical degree and residency training at St. Joseph University in Beirut, Lebanon, and went on to complete a fellowship at MUSC.

Dr. Rizk is on the board of directors of the American Balance Society, a member of the Equilibrium Committee of the American Academy of Otolaryngology, as well as a representative of the Academy in a joint task force of the American Academy of Neurology to investigate quality improvement measures in neurotology.
His research focuses on hearing-related and dizziness-related research. Welcome, Dr. Rizk.

[00:00:55] Habib Rizk, MD: Thank you, Loretta.

[00:00:56] Lynch-Reichert: Dr. Rizk, today we are discussing Meniere's disease. Would you explain to our audience what it is and what are the symptoms of Meniere's disease?
Rizk: So, Meniere's disease is a benign inner ear disease named after the physician who actually discovered it. The patients usually present with fluctuation of hearing loss, ringing in the ears, and episodes of dizziness characterized by a spinning sensation or an illusion of movement sensation that we call vertigo.

[00:01:29] Lynch-Reichert: Vertigo, just like the movie.

[00:01:31] Rizk: Exactly.

[00:01:33] Lynch-Reichert: Is there a genetic component to this disease?

[00:01:36] Rizk: Meniere's patients, we now know they fall into multiple phenotypes, one of them is the genetic familial phenotype. Another one is the migraine phenotype. There's a higher propensity of migraine in Meniere's patients. And it seems like there's a subgroup of Meniere's that comes with a migraine, and it seems like the migraine affects the pathology. A third group would be the head trauma or any type of trauma, including surgical trauma after a middle ear or an inner ear surgery. The fourth group is a group of patients who tend to have more autoimmune disorders. And more recent literature about Meniere's is postulating that Meniere's is a type of autoinflammatory disorder in the same range as periodic fever and those other rheumatological diseases. And finally, the biggest group is the idiopathic group where we believe there's a dysregulation in one of the water transport mechanisms within the cell.
Is it because of a genetic malformation or a mutation due to a viral illness, we don't know, but there's something that happens at that level that deregulates the fluid and electrolyte balance.

[00:02:47] Lynch-Reichert: I'm going to ask a question related to all of this because you indicated that women may have a higher incidence of Meniere's. And if I'm correct, I understand migraines are more prevalent in women, and some autoimmune, like lupus. Do you think there’s a hormonal component to any of this?

[00:03:08] Rizk: That's an excellent question. The hormonal component is more seen in the migraine or vestibular migraine pathologies, which are the migraine dizziness pathologies. There's not a clear-cut cause where hormones are the cause of the Meniere's disease onset, but some patients may have episodes triggered by hormonal fluctuation. So, even if hormones may not be the necessary problem to start with, it might end up affecting the frequency and the nature of the episode, especially if there's a migraine associated with it.

[00:03:45] Lynch-Reichert: And I'm going to just say, this is very layperson science I'm speaking of now, but when you think about young women, when they're having their menstrual cycle, they take a water pill. I wonder if there's been any research or any kind of taking a look at, okay, if you're on your menstrual cycle and you're having Meniere's and you take a water pill for your menstrual cycle, if that decreases the Meniere's or anything like that. It would be interesting to consider.

[00:04:15] Rizk: It's an interesting question. I don't think we ever looked at it specifically in that population, because Meniere's is a rare disease. And one of the main problems of getting to a cure or to a medication that works is that, as I said, Meniere's seems to be a heterogeneous disease.

[00:04:31] Lynch-Reichert: Mm-hm.

[00:04:32] Rizk: So, even though the endpoint manifestation is the same, there might be several mechanisms working. And that's why certain medications are working on a group of patients and not the others.

[00:04:42] Lynch-Reichert: And that's why it's so important to do the research as well.

[00:04:43] Rizk: Correct.

[00:04:44] Lynch-Reichert: And speaking of research, I understand a recent clinical trial with Meniere's failed in phase III but is now being done on tinnitus. Can you talk a little more about that?

[00:04:54] Rizk: Yes, this is a trial that was based on the fact that steroids seem to help in certain category of patients to reduce the vertigo attacks of Meniere's. And that company, Otonomy, did that trial, phase I, II, and III, using a gel that allows a timed release of steroids, which would allow us to inject the middle ear, and that steroid would be delivered over a period of several days to weeks into the inner ear. The idea was great. Unfortunately, the results did not show a greater efficacy of that steroid than placebo. And now that same company is trying to see if that works for tinnitus, because along the way some of the secondary outcomes that were collected, tinnitus seems to have responded in a greater-than-chance ratio.

So we're going to study it systematically, or at least that industry is sponsoring the study to study it systematically in tinnitus patients. And that study is slated to start in July 2021. And MUSC is a site of that.

[00:06:00] Lynch-Reichert: Wonderful. What you said kind of leads me to the question. Is there any relationship between Meniere's or at least those symptoms? They sound a little similar, sometimes, to a stroke or a TIA. Do you ever speak with your colleagues about stuff like that?

[00:06: 16] Rizk: Well, as I said initially, that Meniere's is a benign inner ear disorder, but anybody who presents to the emergency room with a severe vertigo attack, one of the most important aspects of evaluation is to rule out a stroke, because some strokes can present as a vertigo attack. However, once you have the stereotypical manifestation of hearing loss, ringing, coming at the time of the attack of vertigo, that paints a picture. We might not be able to diagnose it at the first time, but then as we follow the patient and as we observe the hearing loss presenting on the audiogram, which is the hearing test, we can definitively diagnose Meniere's disease.

[00:07:04] Lynch-Reichert: And then after you define it, what do you do?

[00:07:08] Rizk: So there are many aspects to treating Meniere's disease, but there's no cure. So, the approach for Meniere's disease is gradual, it's related to the frequency of the episodes, to the amount of hearing loss that we encounter in those patients, and to the level of disruption of their quality of life.

And, as you know, this also varies from patient to patient. Some patients are fine with one episode a week, where others, one episode every two weeks can wreck their quality of life, they need to call out. It can lead to significant disability and loss of productivity. So the first step of treating Meniere's usually is reducing the salt in the diet. So that's kind of a general recommendation. It's not based on hard evidence but based on empirical evidence, meaning with habit and with clinical experience over the past 50 years people have reported improvement with that. And we ask the patients to aim for 1500 milligrams per day sodium intake, which is even less than what your cardiologist would recommend for high blood pressure.

[00:08:15] Lynch-Reichert: Mm-hm.

[00:08:16] Rizk: It is tough. And I tell patients, I'm a little bit more permissive, and I tell patients it's okay to be at 1600 or 1700 milligrams, but avoid bad fluctuations. Like don't go one day at one gram and one day at four grams of sodium. The next step would be, there are two options, and they are reported as options by the most recent guidelines of the American Academy of Otolaryngology, is a diuretic called triamterene hydrochlorothiazide. It's a combination pill of two diuretics. And the other option would be betahistine. Betahistine is a product that is not available in the U.S. unless you get it from a compounding pharmacy. It was never cleared by the FDA, but it's a safe drug. And it works by increasing blood flow into the inner ear. Those two medications are listed as options because the level of evidence is not very, very high. It does work on some people. And on others it doesn't. And so far we haven't been able to narrow down which patient from the get-go will respond. So we don't have a profile of the patient who will respond to this drug or not. We do try them on a lot of patients, and some of them it works and some it doesn't.

[00:09:34] Lynch-Reichert: Can I ask you a quick question?

[00:09:35] Rizk: Yes.

[00:09:36] Lynch-Reichert: And this may be a little ignorant but I'm going to ask it anyhow. So this is an inner ear situation and it has a lot to do with electrolytes and kind of a water imbalance, if you will. But when you talked about lowering your sodium intake, do any high blood pressure medicines affect or offer any benefit, or is this really totally unrelated to high blood pressure, it's more a water and electrolyte imbalance?

[00:10:04] Rizk: That's actually an excellent question. There are some reports of association of cardiovascular morbidities with Meniere's, especially when it presents at the later stage in life, because Meniere's can manifest in two peaks, one in young adults and one after the age of 65. The majority of patients we see are in the fourth or fifth decade of life. So there have been reports of higher incidence of high blood pressure with it, but it's not a direct correlation to the disease. The water pill, I'm talking about, the triamterene hydrochlorothiazide, can be used as a high blood pressure medication, but it's not a very effective high blood pressure medicine, it's a soft one. So it's not like, usually, the first line of treatment for high blood pressure.

[00:10:47] Lynch-Reichert: Okay. Which also leads me to believe or to ask, is there a category of people that this affects more than others? You said it could affect the young or the old. Are there sex, gender differences? Are there race differences? Who do you see most coming into the clinic?

[00:11:08] Rizk: So, the general incidence that is reported for Meniere's disease, in the U.S., in the Western countries, is 190 patients per 100,000 people. So it's about two in a thousand, almost the same as MS. It has higher prevalence in female patients. There's no racial predisposition, which speaks to the heterogeneity of the disease, the multiple factors that could lead to that. And as I said previously, some of those factors can be familiar or genetics, but they're not, like, the most important ones.

[00:11:45] Lynch-Reichert: Okay. So, let's speak about the research related to this disease. And I wanted to let our audience know, I recently understood that Otolaryngology as a department has one of the best research profiles in the country for across the ear, nose, and throat spectrum. So, I know you guys are very proud of the research work that's being done in your department. So, tell me what kind of research is being done in regard to this disease.

[00:12:12] Rizk: Yeah, thank you, we are pretty fortunate to have a supportive department for research. And for Meniere's disease, we currently have two foundation grants from the American Hearing Research Foundation and Cures Within Reach Foundation to study the effect of a migraine medication called Venlafaxine in the treatment of Meniere's disease. And this is based on the primary hypothesis that this is a water-electrolyte problem. And that specific migraine drug, Venlafaxine, one of its side effects is that it can cause retention of water in the cells. And the research was supported partly because of a big incidence of comorbid migraine with Meniere's, but also because of that side effect profile of the drug. We've currently enrolled 20 patients. None has dropped out. We're currently ongoing, aiming to reach 40 patients. And we'll see the results of that.

[00:13:10] Lynch-Reichert: That's exciting. First of all, how can folks learn more about this particular research? How long will the clinical trial go on? And when do you expect to get some analysis of the data?

[00:13:24] Rizk: So, we're currently expecting to continue enrollment till the end of this year.

[00:13:28] Lynch-Reichert: Okay.

[00:13:29] Rizk: And we might ask for an extension. In order to learn more, I usually give out my email for patients who are interested in knowing more about that research. My email is RizkH@MUSC.edu.

[00:13:42] Lynch-Reichert: Wonderful. I hope that everybody takes advantage of this. This is one of the reasons why we do Science Never Sleeps here at the Medical University, to encourage our public to be aware of the research that goes on, to understand how it actually benefits the translation of the research into the clinical care that you offer, and to help them to engage with our scientific community. So, delighted to have you so generously offer your email.

When you are able to treat successfully a patient with Meniere's, and they have significant hearing loss, cochlear implants or are there other options for them to regain some of their hearing?

[00:14:23] Rizk: Definitely. I mean, again, we were talking earlier about how this spectrum of treatment, as the disease progresses, and in some people it doesn't. In some people it's a very benign form, very infrequent attacks. But if you have somebody who has the unfortunate type where it's kind of very frequent attacks, one after the other, and the hearing does not recover, we try, initially, steroid injections in the drum. That's also an option that is geared toward reducing the vertigo attacks but also hopefully lifting the hearing back to as close as possible to baseline. If that doesn't work, sometimes we induce the hearing loss by injecting gentamicin in the ear, because those are the patients who are so miserable from their vertigo attacks. They're basically needing to go to the ED or having to need antiemetics, one every other day. They are at risk of losing their job. And this is affecting their everyday life. Then we make the decision with the patient that, okay, we need to control the vertigo. So we inject something called gentamicin which is toxic to the balance portion of the ear.

[00:15:25] Lynch-Reichert: Okay.

[00:15:26] Rizk: It has less incidence of toxicity to the hearing, but it has a propensity to cause hearing loss. Traditionally, we only inject it in patients who already have had hearing loss, but the newer guidelines, because of its effectiveness, because of the severe quality of life, if all the conservative measures have been exhausted, we can consider a shot of gentamicin to see if it can control the vertigo. And in those cases, in those cases where the disease is severe and patients end up losing their hearing whether because of Meniere's or because of our intervention, then we have to offer rehabilitative strategies. And those strategies can range from a hearing aid, if the hearing loss is mild, to a cochlear implant if there's profound deafness. And in the most extreme cases where we need to do a labyrinthectomy, which is a surgical destruction of the ear to stop the vertigo attacks, then a cochlear implant would be the only solution to treat those patients, those patients' hearing loss.

[00:16:30] Lynch-Reichert: You know, what you’re suggesting to me really makes me understand how very serious this disease is and how much it will complicate one's life if it's not well-controlled and looked into. I wonder, for medical students getting into the world right now, is this a disease that they know anything about? And I ask that question because, say your internal medicine or general practitioner has a patient and the patient presents with some of these symptoms. Would they know to think about this or would they have to go through a whole...roll of factors to consider whether this is the case or not?

[00:17:13] Rizk: I think it's often misdiagnosed.

[00:17:15] Lynch-Reichert: Mm.

[00:17:16] Rizk: And sometimes, again, understanding the constellation of symptoms and how they present on and off is a cardinal manifestation of the disease. So, sometimes we get patients referred because they have tinnitus, but then when you explore with the patient the symptom, then the tinnitus is not coming with the vertigo and they're not time-locked. But I'd rather that a patient get sent and not have Meniere's disease than a patient is misdiagnosed and not seen for several months or until his disease evolved.

[00:17:50] Lynch-Reichert: Absolutely. But that's the wonderful thing about the Medical University of South Carolina as an academic health center. Our patients have access to folks like you and they have fabulous general practitioners, family medicine, internal medicine, that are probably very aware of the Department of Otolaryngology and understand the options that are available for their patients, especially if they can't figure out what's going on with them. So, that's the beauty of who we are and what we do. And I want to thank you so much for your passion in the work that you do. It's very clear that this is an issue that you strongly believe it and want to resolve, and I love that, that's wonderful. Are there any other research opportunities going on when it comes to Meniere's disease? Across the country, not just at MUSC.

[00:18:35] Rizk: So, there are some industry-sponsored trials. There's one that is not slated to begin before a few months called the Sound Trial. We were a site of phase II, and they're going to start phase III, I believe, at the beginning of 2022. And it's a trial that is investigating the efficacy of a drug called Ebselen, which is an antioxidant. And preliminary results from phase I and phase II were encouraging, so phase III has been approved by the FDA.

[00:19:10] Lynch-Reichert: Wonderful. That would be great. I would love to see some real action going on with that. For people that are suffering like that, it sounds miserable, so I love the fact that we will offer them an opportunity, hopefully, to check out that clinical trial.

Dr. Rizk, thank you so much for this wonderful discussion on a disease that is so challenging and all the work you do and your team in Otolaryngology trying to make a difference in Meniere's disease, a disease I'd never heard of. And I'm so proud that we're able to offer this information to our audience. And I hope that our listeners will take advantage of Dr. Rizk's email address. If you have any issues or know of anyone who has an issue, please look him up.

And thank you to our devoted listeners for your continued support and interest in MUSC research. To consider participating in a clinical trial at MUSC or to learn more about our research, visit our Facebook page, MUSC Research, or go to our website, research.musc.edu.

Until next time, stay healthy.

[00:20:15] Rizk: Thank you.