Ménière’s Disease

CT Imaging as a Diagnostic Tool for Ménière’s Disease

By Ngoc-Nhi Luu, M.D., Dr. med.

Ménière’s disease is an inner ear condition with symptoms including vertigo, hearing loss, and tinnitus, and may be associated with an accumulation of fluid in the inner ear, termed endolymphatic hydrops. Diagnosis of Ménière’s is entirely based on clinical characteristics, and to date, no classification has been established that can predict the onset or course of the disease. Patients with Ménière’s can have varying degrees of symptoms, so defining subtypes within the Ménière’s population may help establish a classification to improve diagnoses and treatments.

Our previous analysis of cadaveric ears of patients with Ménière’s revealed striking differences within the endolymphatic sac in the inner ear, which regulates endolymph fluid. We had found two different aberrations of the endolymphatic sac—its underdevelopment or its degeneration—among Ménière’s patients, suggesting that the loss of endolymphatic sac cell function and the possible impairment of endolymphatic fluid regulation may lead to Ménière’s. In addition, these two pathologies may be associated with differing clinical traits of the disease.

In our prior work, we examined sections of human cadaveric inner ears with Ménière’s and found differences in the angular trajectory of the vestibular aqueduct (ATVA), the bony canal in which the endolymphatic sac is located. These differences resembled either the trajectory of typical adults, or the trajectory of early developmental, fetal vestibular aqueducts. ATVA similar to other adults without Ménière’s were associated with late onset of the condition, whereas Ménière’s patients with “fetal” ATVA experienced early onset.

A 3D reconstruction of the endolymphatic space of a typical human adult inner ear. In Ménière’s disease patients, the anatomy of the endolymphatic sac differs, suggesting that the impairment of the sac’s function to regulate fluid may lead to Ménière’s. (LSC, lateral semicircular canal; PSC, posterior semicircular canal; SCC, superior semicircular canal.)

A 3D reconstruction of the endolymphatic space of a typical human adult inner ear. In Ménière’s disease patients, the anatomy of the endolymphatic sac differs, suggesting that the impairment of the sac’s function to regulate fluid may lead to Ménière’s. (LSC, lateral semicircular canal; PSC, posterior semicircular canal; SCC, superior semicircular canal.)

For our paper published in the journal Otology & Neurotology in April 2019, we hypothesized that this difference can be detected with a CT scan (computerized tomography) in patients with early or late onset Ménière’s.

We used a custom-made, open-source web application for angle measurements and applied this technique on high resolution CT imaging of patients with Ménière’s. Comparing the angle measurements of the ATVA, we confirmed the results of the cadaveric study. There was a strong correlation between late onset Ménière’s with a typical “adult” course of the vestibular aqueduct, while early onset Ménière’s was associated with a more straight, “fetal” course of the vestibular aqueduct.

As such our study aims to develop a radiographic screening tool, such as a CT scan of the inner ear, to classify different Ménière’s subtypes. It appears that early onset Ménière’s patients have a different anatomy of the vestibular aqueduct compared with late onset Ménière’s patients.

We want to better understand if these findings also correlate with additional clinical factors, such as specific symptoms or a positive family history for Ménière’s. Ultimately, this may help to further characterize different Ménière’s subtypes in order to better diagnose, predict the course of, and treat the condition.


Ngoc-Nhi Luu, M.D., Dr. med., is a postdoctoral fellow at Eaton-Peabody Laboratories, Massachusetts Eye and Ear, Harvard Medical School, and an ENT resident at University Hospital Zurich. Luu’s 2017 ERG grant was generously funded by The Estate of Howard F. Schum. Coauthors on the paper include Judith Kempfle, M.D. (a 2010 ERG scientist), Steven Rauch, M.D. (1990 ERG), and Joseph Nadol, M.D. (1976–77 ERG).

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Recording Electrical Responses to Improve the Diagnosis of Hearing Conditions

By Yishane Lee

Electrocochleography (ECochG) is a method to record electrical responses from the inner ear and the auditory nerve in the first 5 milliseconds after a sound stimulus, such as a click or tone burst. These stimuli can be adjusted for repetition rate and polarity, and recordings can also be taken from either the ear canal, eardrum, or through the eardrum. The main components of ECochG response are the summating potential (SP), from the sensory hair cells in the cochlea, and the action potential (AP) of auditory nerve fibers. In an October 2018 paper in the journal Canadian Audiologist, 2015 ERG scientist Wafaa Kaf, Ph.D., reviews the diagnostic applications for using ECochG for Ménière’s disease and cochlear synaptopathy, two conditions that can be difficult to pinpoint, especially early in the disease, and suggests how to improve the use of ECochG as a clinical tool.



Endolymphatic hydrops, or abnormal fluctuations in inner ear fluid, is believed to be the underlying cause of Ménière’s disease and its associated hearing and balance disorder. ECochG collects information about the SP/AP amplitude and area ratios that can be used to confirm a Ménière’s diagnosis, without relying solely on clinical symptoms.

Since the SP/AP amplitude ratio can vary among known Ménière’s patients, Kaf suggests including data about the SP/AP area ratio as well can help with diagnosing the disease. To further distinguish Ménière’s, Kaf suggests using ECochG AP latencies, and, building on her prior research, the effect of fast click rates on the auditory nerve latency and amplitude. Using the continuous loop averaging deconvolution technique, various properties of the SP and AP waveforms are easier to identify and parse. Results suggest that the functions of the cochlear nerve and/or cochlear synapses are damaged in Ménière’s. Earlier research that shows an abnormal acoustic reflex decay in about a quarter of Ménière’s patients, and a reduced number of synapses between inner hair cells and auditory nerve fibers, underscores the presence of nerve damage in Ménière’s.

Cochlear synaptopathy is a noise-induced or age-related dysfunction that is also causing reduced synapses between inner ear hair cells and auditory nerve fibers, resulting in tinnitus, hyperacusis, and difficulty hearing in noise despite normal hearing sensitivity. ECochG may help with its diagnosis, especially given that traditional audiograms and hearing tests have been found to miss this “hidden hearing loss.” The use of both the SP/AP amplitude and area ratios and specific auditory brainstem responses can help confirm this condition and distinguish it from Ménière’s disease.

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ECochG can also be used to help confirm the diagnosis of auditory neuropathy spectrum disorder, a problem with the way sound is transmitted between the inner ear and the brain, and other inner ear disorders. The technique can also be used to monitor ear responses, real-time, during surgeries such as a stapedectomy, endolymphatic shunt, and cochlear implantation, additional instances demonstrating how ECochG holds promise for expanded use in the clinic.

A 2015 ERG scientist funded by The Estate of Howard F. Schum, Wafaa Kaf, Ph.D., is a professor of audiology at Missouri State University.

We need your help supporting innovative hearing and balance science through our Emerging Research Grants program. Please make a contribution today.

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My Cautious Gratitude

By Lauren McGrath

Clockwise from left: Heather, her daughter, her husband, and son.

Clockwise from left: Heather, her daughter, her husband, and son.

Heather Mills never imagined her early adulthood would be interrupted by Ménière's disease, a chronic hearing and balance disorder without a cure. She was diagnosed at 21—just within the typical 20-to-50-year-old range of onset—after a slew of tests and follow-up visits with a specialist at the University of Minnesota.

Heather’s symptoms initially included a unilateral (in one ear) mild low-frequency hearing loss, tinnitus, and some ear pressure and pain. Within a few years, her hearing loss became bilateral and worsened from moderate to severe. She was regularly distressed by intense ear pressure, struggled with her balance, and experienced occasional bouts of vertigo. As Heather learned, Ménière's affects each patient differently. She considered herself fortunate not to face drop attacks (instances of falling to the ground without losing consciousness), one of the most terrifying symptoms associated with Ménière's disease.

Despite its prevalence Heather family, hearing loss—once her most debilitating Ménière's symptom—came as a surprise. Her father has lived with a unilateral hearing loss since childhood, while her mother and maternal grandmother both developed high-frequency sensorineural hearing loss in their late 40s. “It never occurred to me that it may one day affect me, too,” reflects Heather, who can recall her ability to hear whispers across her high school classrooms.

Though she followed her doctor’s directions to take diuretics and maintain a low-salt diet for her vestibular symptoms, Heather chose not to purchase hearing aids. Lacking amplification, Heather faced difficulty in her job as a legal project specialist, which required frequent verbal interaction with clients, lawyers, and vendors both on the phone and in person. She found herself increasingly dependent on a close friend and colleague who truly served as her ears by repeating information for her during and after meetings.

Heather’s untreated hearing loss, combined with her constant fear of a sudden vertigo attack, fueled feelings of isolation. Unable to participate in conversations with friends, Heather stopped receiving invites to social outings. Challenges with work and friends began to affect Heather’s mental health. “I became depressed, lonely, and developed anxiety because of two unknowns: not knowing when my vertigo would strike again, and wondering how I’d continue to work to support my family.”

Heather noticed a sharp decline in her job performance when her helpful coworker—her ears—left the law firm. Part of Heather’s role required instructing staff on new software, and she was humiliated to find out that her trainees’ questions went unanswered because they’d not been heard. “This is when I began to lose confidence in myself,” Heather remembers.

Heather realized she had to address her hearing loss. In line with Hearing Health Foundation (HHF)’s findings in a 2017 survey of more than 2300 participants, cost is by far the largest barrier to a hearing aid purchase. Heather delayed taking action for so many years—11 to be exact—because her insurance provided no hearing healthcare coverage.

“All I can say now is I wish I had gotten hearing aids sooner!” exclaims Heather, who, with newfound confidence, no longer struggles in her daily professional communications and social life. Prior to pursuing treatment, her conversations had soured quickly when she constantly had to ask other parties to speak up, repeat themselves, or remind them of her hearing loss. Most painfully, communication without hearing aids often left Heather dismissed by a “nevermind” when she requested repetition. With her new devices, Heather felt her confidence restored.

Now in remission, Heather considers her life happy and her health stable. Hearing aids have somewhat alleviated her tinnitus, her ear pressure has subsided, and the vertigo spells are very rare. She’s sought treatment for her anxiety and depression. Heather credits her husband, Billy, with whom she has two young children, for his support during her more difficult years. Engaging in online Ménière's support groups has been a beneficial coping tool.

Heather is cautiously grateful for her current health, knowing the unpredictability of Ménière's could alter her circumstances at any time. She hopes for scientific advancements in Ménière's research that will one day uncover the causes, more reliable diagnostic procedures, and a cure.

Heather lives in Minnesota with her husband and children. She is a participant in HHF’s Faces of Hearing Loss campaign.

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Accomplishments by ERG Alumni

By Elizabeth Crofts

Progress Investigating Potential Causes and Treatments of Ménière’s Disease


Gail Ishiyama, M.D., a clinician-scientist who is a neurology associate professor at UCLA’s David Geffen School of Medicine, has been investigating balance disorders for nearly two decades and recently coauthored two studies on the topic. While not directly funded by HHF, Ishiyama is a 2016 Emerging Research Grants recipient and also received a Ménière’s Disease Grant in 2017.

Ishiyama and colleague’s December 2018 paper in the journal Brain Research investigated oxidative stress, which plays a large role in several inner ear diseases as well as in aging. Oxidative stress is an imbalance between the production of free radicals and antioxidant defenses. The gene responsible for reducing oxidative stress throughout the body is called nuclear factor (erythroid-derived 2)-like 2, or NRF2. Ishiyama’s study looked at the localization of NRF2 in the proteins in the cells of the human cochlea and vestibule. It was found that NRF2-immunoreactivity (IR) was localized in the organ of Corti of the cochlea. Additionally, it was observed that NRF2-IR decreases significantly in the cochlea of older individuals. The team postulates for future studies that modulation of NRF2 expression may protect from hearing loss that results from exposure to noise and ototoxic drugs.

In a January 2018 report in the journal Otology & Neurotology, Ishiyama and team researched endolymphatic hydrops (EH), a ballooning of the endolymphatic fluid system in the inner ear that is associated with Ménière’s disease. Symptoms include fluctuating hearing loss, as well as vertigo, tinnitus, and pressure in the ear.

For the study, patients with EH and vestibular schwannoma were tested to evaluate the clinical outcome of patients when EH is treated medically. Vestibular schwannoma, also known as acoustic neuroma, are benign tumors that grow in the vestibular system of the inner ear, which controls balance. Often when patients develop episodic vertigo spells and have a known diagnosis of vestibular schwannoma, surgeons recommend surgical intervention, as they attribute the symptoms to the vestibular schwannoma. However, a noninvasive treatment may hold promise. Through the use of high-resolution MRI scans, the researchers found that when EH coexists with vestibular schwannoma in a patient, and the patient also experiences vertigo spells, a medical treatment for EH—that is, the use of diuretics to relieve inner ear fluid buildup—may alleviate the vestibular symptoms.

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A 2016 ERG scientist funded by The Estate of Howard F. Schum, Gail Ishiyama, M.D., is an associate professor of neurology at UCLA’s David Geffen School of Medicine. She also received a 2017 Ménière’s Disease Grant.

New Insights Into Aging Effects on Speech Recognition

Age-related changes in perceptual organization have received less attention than other potential sources of decline in hearing ability. Perceptual organization is the process by which the auditory system interprets acoustic input from multiple sources, and creates an auditory scene. In daily life this is essential, because speech communication occurs in environments in which background sounds fluctuate and can mask the intended message.

Perceptual organization includes three interrelated auditory processes: glimpsing, speech segregation, and phonemic restoration. Glimpsing is the process of identifying recognizable fragments of speech and connecting them across gaps to create a coherent stream. Speech segregation refers to the process where the glimpses (speech fragments) are separated from background speech, to focus on a single target when the background includes multiple talkers. Phonemic restoration refers to the process of filling in missing information using prior knowledge of language, conversational context, and acoustic cues.

A July 2018 study in The Journal of the Acoustical Society of America by William J. Bologna, Au.D., Ph.D., Kenneth I. Vaden, Jr., Ph.D., Jayne B. Ahlstrom, M.S., and Judy R. Dubno, Ph.D., investigated these three components of perceptual organization to determine the extent to which their declines may be the source of increased difficulty in speech recognition with age. Younger and older adults with typical hearing listened to sentences interrupted with either silence or envelope-modulated noise, presented in quiet or with a competing talker.

As expected, older adults performed more poorly than younger adults across all speech conditions. The interaction between age and the duration of glimpses indicated that, compared with younger adults, older adults were less able to make efficient use of limited speech information to recognize keywords. There was an apparent decline in glimpsing, where interruptions in speech had a larger effect on the older adult group.

Older adults saw a greater improvement in speech recognition when envelope modulations were partially restored, leading to better continuity. This demonstrated that with age comes a poorer ability to resolve temporal distortions in the envelope. In speech segregation, the decline in performance with a competing talker was expected to be greater for older adults than younger adults, but this was not supported by the data.

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A 2015 Emerging Research Grants scientist, Kenneth I. Vaden, Jr., Ph.D., is a research assistant professor in the department of otolaryngology–head and neck surgery at the Medical University of South Carolina.

A 1986–88 ERG scientist, Judy R. Dubno, Ph.D., is a member of HHF’s Board of Directors. The study’s lead author, William Bologna, Au.D., Ph.D., is a postdoctoral research fellow at the National Center for Rehabilitative Auditory Research in Portland, Oregon.

A 2018 HHF intern, Author Elizabeth Crofts is a junior at Boston University studying biomedical engineering. For our continually updated list of published papers by ERG alumni, see hhf.org/erg-alumni.


We need your help supporting innovative hearing and balance science through our Emerging Research Grants program. Please make a contribution today.

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Understanding a Pressure Relief Valve in the Inner Ear

By Ian Swinburne, Ph.D.

The inner ear senses sound to order to hear as well as sensing head movements in order to balance. Sounds or body movements create waves in the fluid within the ear. Specialized cells called hair cells, because of their thin hairlike projections, are submerged within this fluid. Hair cells bend in response to these waves, with channels that open in response to the bending. The makeup of the ear’s internal fluid is critical because as it flows through these channels its contents encode the information that becomes a biochemical and then a neural signal. The endolymphatic sac of the inner ear is thought to have important roles in stabilizing this fluid that is necessary for sensing sound and balance.

This study helps unravel how a valve in the inner ear's endolymphatic sac acts to relieve fluid pressure, one key to understanding disorders affected by pressure abnormalities such as Ménière’s disease.

This study helps unravel how a valve in the inner ear's endolymphatic sac acts to relieve fluid pressure, one key to understanding disorders affected by pressure abnormalities such as Ménière’s disease.

While imaging transparent zebrafish, my team and I found a pressure-sensitive relief valve in the endolymphatic sac that periodically opens to release excess fluid, thus preventing the tearing of tissue. In our paper published in the journal eLife June 19, 2018, we describe how the relief valve is composed of physical barriers that open in response to pressure. The barriers consist of cells adhering to one another and thin overlapping cell projections that are continuously remodeling and periodically separating in response to pressure.

The unexpected discovery of a physical relief valve in the ear emphasizes the need for further study into how organs control fluid pressure, volume, flow, and ion homeostasis (balance of ions) in development and disease. It suggests a new mechanism underlying several hearing and balance disorders characterized by pressure abnormalities, including Ménière’s disease.

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Here is a time-lapse video of the endolymphatic sac, with the sac labeled “pressure relief valve” at 0:40.

2017 Ménière’s Disease Grants scientist Ian A. Swinburne, Ph.D., is conducting research at Harvard Medical School. He was also a 2013 Emerging Research Grants recipient.

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Improving Diagnostic Test for Ménière’s Disease

By Wafaa Kaf, Ph.D., and Carol Stoll

Electrocochleography (ECochG) is a commonly used assessment of the auditory system, specifically the inner ear and the hearing nerve. ECochG is most often elicited by a brief acoustic stimulus, known as a “click,” at a relatively low repetition rate. It measures two key responses: summating potential (SP) and action potential (AP), which assist in the diagnosis of Ménière’s disease, an inner ear and balance disorder. Previous research has established that individuals with Ménière’s disease are likely to have abnormally large SPs and a large SP/AP ratio. Though click ECochG has great potential to detect Ménière’s disease, it lacks sensitivity, or the ability to correctly identify those with the disease. Only 69% of those with Ménière’s disease are correctly diagnosed, while 31% of those with the disease have normal ECochG results. This lack of accuracy prevents its use as a definitive diagnostic tool. Hearing Health Foundation 2015 Emerging Research Grants recipient, Wafaa Kaf, Ph.D., is researching the use of a novel analysis technique called Continuous Loop Averaging Deconvolution (CLAD) to best improve the sensitivity of ECochG to high click rate for diagnosing Ménière’s disease. Findings were recently published in Ear and Hearing 2017.


In a recently published paper in Frontiers in Neuroscience, Kaf’s research team shares its findings on the effects of altering the parameters of the acoustic stimulus on ECochG responses to quantify the effect of stimulus rate and duration of the elicited stimuli. Kaf and her research team obtained SP measurements to 500Hz and 2000Hz tone bursts that varied in duration and repetition rate from 20 adult females with normal hearing. CCLAD was used to interpret the tracings elicited by the differing stimuli of tone bursts.

They found that SP amplitude was significantly larger when using the highest stimulus repetition rate. It is believed that the high stimulus repetition rates minimize the neural contributions and mostly reflect hair cell responses, the target of ECochG. In addition, longer duration stimuli is believed to better reflect hair cell involvement while shorter stimuli may be useful in eliciting responses reflective of neural contributions. Lastly, 2000Hz tone bursts produced larger SP amplitude as compared to 500Hz tone bursts. Therefore, 2000Hz tone bursts with a high repetition rate and long duration can be used as parameters to minimize neural contributions to SP measures whereas short duration stimuli can be used if one wishes to asses neural activity.  

The data that Kaf’s team published is a critical initial advancement towards ultimately understanding the SP measurement in diseased ears. Their findings not only provide normative data for tone burst ECochG across stimulus frequencies, stimulus rates, and stimulus durations, but also help others better understand how to improve sensitivity of ECochG for early diagnosis of Ménière’s disease.  

Wafaa Kaf, Ph.D., is a 2015 Emerging Research Grants recipient. Her grant was generously funded by The Estate of Howard F. Schum.


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HHF Launches Faces of Hearing Loss Campaign

Think of someone you know who has hearing loss. Who do you see?

You envision a relative, but you are not thinking of your 4-year-old niece. A neighbor comes to mind, but not the 32-year-old who lives across the street.

This is a trick question. Hearing loss—and related conditions like tinnitus, Ménière's disease, and hyperacusis—can affect anyone, anywhere. Hearing loss is your 4-year-old niece, your 32-year-old neighbor, your colleague in her mid-20s. Hearing loss affects every age, race, ethnicity, and gender.

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No one is immune from developing a hearing and balance disorder—and hearing loss has no single face. To refute common misconceptions that it only affects older adults, HHF has collected images of individuals living with a hearing condition to capture the diversity of its impact across the country. These are HHF’s “Faces of Hearing Loss.”

Participants shared their picture, current age, state of residence, type of hearing condition, and the age at onset or diagnosis. Among the tens of millions of Americans with hearing loss are an 11-year-old boy in Oregon, an 80-year-old woman living in Washington, and a 47-year-old man in North Dakota. These individuals may never meet, but “Faces of Hearing Loss” connects them through their shared experiences.

If you or a loved one has hearing loss, please consider participating in “Faces of Hearing Loss” by completing this brief form, sending in picture, and answering a few basic questions. If you are the parent of a child under 18, you may sign a release form on their behalf.

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Cellular Changes and Ménière’s Disease Symptoms

By Carol Stoll

Ménière’s disease is characterized by fluctuating hearing loss, vertigo, tinnitus, and ear fullness, but the causes of these symptoms are not well understood. Past research has suggested that a damaged blood labyrinthine barrier (BLB) in the inner ear may be involved in the pathophysiology of inner ear disorders. Hearing Health Foundation (HHF)’s 2016 Emerging Research Grants (ERG) recipient Gail Ishiyama, M.D., was the first to test this proposition by using electron microscopy to analyze the BLB in both typical and Ménière’s disease patients. Ishiyama’s research was fully funded by HHF and was recently published in Nature publishing group, Scientific Reports.

The BLB in a Meniere’s disease capillary. a) Capillary located in the stroma of the macula utricle from a Meniere’s subject (55-year-old-male). The lumen (lu) of the capillary is narrow, vascular endothelial cells (vec) are swollen and the cytoplasm is vacuolated (pink asterisks). b. Diagram showing the alterations in the swollen vec, microvacuoles are also abundant (v). Abbreviations, rbc: red blood cells, tj: tight junctions, m: mitochondria, n: cell nucleus, pp: pericyte process; pvbm: perivascular basement membrane. Bar is 2 microns.

The BLB in a Meniere’s disease capillary. a) Capillary located in the stroma of the macula utricle from a Meniere’s subject (55-year-old-male). The lumen (lu) of the capillary is narrow, vascular endothelial cells (vec) are swollen and the cytoplasm is vacuolated (pink asterisks). b. Diagram showing the alterations in the swollen vec, microvacuoles are also abundant (v). Abbreviations, rbc: red blood cells, tj: tight junctions, m: mitochondria, n: cell nucleus, pp: pericyte process; pvbm: perivascular basement membrane. Bar is 2 microns.

The BLB is composed of a network of vascular endothelial cells (VECs) that line all capillaries in the inner ear organs to separate the vasculature (blood vessels) from the inner ear fluids. A critical function of the BLB is to maintain proper composition and levels of inner ear fluid via selective permeability. However, the inner ear fluid space in patients with Ménière’s has been shown to be ballooned out due to excess fluid. Additionally, the group had identified permeability changes in magnetic resonance imaging studies of Meniere’s patients, which may be an indication of BLB malfunction.

Ishiyama’s research team used transmission electron microscopy (TEM) to investigate the fine cellular structure of the BLB in the utricle, a balance-regulating organ of the inner ear. Two utricles were taken by autopsy from individuals with no vestibular or auditory disease. Five utricles were surgically extracted from patients with severe stage IV Ménière’s disease with profound hearing loss and intractable recurrent vertigo spells, who were undergoing surgery as curative treatment.

Microscopic examination revealed significant structural differences of the BLB within the utricle between individuals with and without Ménière’s disease. In the normal utricle samples, the VECs of the BLB contained numerous mitochondria and very few fluid-containing organelles called vesicles and vacuoles. The cells were connected by tight junctions to form a smooth, continuous lining, and were surrounded by a uniform membrane.

However, samples with confirmed Ménière’s disease showed varying degrees of structural changes within the VECs; while the VECs remained connected by tight junctions, an increased number of vesicles and vacuoles was found, which may cause swelling and degeneration of other organelles. In the most severe case, there was complete VEC necrosis, or cell death, and a severe thickening of the basal membrane surrounding the VECs.

The documentation of the cellular changes in the utricle of Ménière’s patients was the first of its kind and has important implications for future treatments. Ishiyama’s study concluded that the alteration and degeneration of the BLB likely contributes to fluid changes in the inner ear organs that regulate hearing and balance, thus causing the Ménière’s symptoms. Further scientific understanding of the specific cellular and molecular components affected by Ménière’s can lead to the development of new drug therapies that target the BLB to decrease vascular damage in the inner ear.

Gail Ishiyama, M.D., is a 2016 Emerging Research Grants recipient. Her grant was generously funded by The Estate of Howard F. Schum.


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Sounds like Meniere's Disease

By Haley Walker

Haley getting hearing aid mold impressions

Haley getting hearing aid mold impressions

“Looks like you have Ménière’s disease,” my doctor said. My heart skipped a beat. What does that mean? What on earth is that? Over the next couple of months I had various hearing tests done, and met with an ear, nose, and throat doctor. It was a lot to take in.

At first I was just glad to have an answer to my problems. A name, a label, an explanation—and to know I am not going crazy. But after that I began to feel worried. Ultimately my diagnosis meant progressive hearing loss; not only did I need hearing aids for moderate hearing loss, my hearing could get worse.

Ménière’s also means I have to follow a low-salt diet. I can’t eat more than 1.5 grams a day of sodium. That’s about a third of a teaspoon. No fast food, no processed food, no added salt.

Ménière’s disease is a disorder that causes abnormal fluid retention in the inner ear, leading to balance problems, hearing loss, and tinnitus (ringing in the ears). Typically, it only affects one ear but lucky me—I have it in both. My doctor was very surprised and said it's quite rare, but as I researched the condition I found a lot of people have it in both ears. That made me feel a bit better.

The truth is it took over a year to finally get this diagnosis of Ménière’s. If I really think about it, I started having disabling dizzy spells that caused vomiting and nausea when I was in high school, at age 16 or 17. (I am 20 now.) My family and I wrote them off as anxiety attacks and dealt with them as they came.

I remember trying to walk home up a hill behind the school one day and literally falling on my face because I couldn’t walk straight. Mmmmm dirt… yummy. I stumbled home and laid down on the floor in our living room crying. I couldn’t get a grip on myself. What is happening to me? Anxiety definitely played a part, but I now know there was a more pieces to the puzzle.

I am now treating Ménière’s by following my low-sodium diet, wearing my hearing aids, and taking a diuretic—a medication that helps to control the abnormal fluid retention in my ears. (This is why limiting salt also helps—salt makes you retain water.) It was incredible when I first got my hearing aids. Everything I had been missing I could suddenly hear! I now can enjoy the little things like the birds singing outside my window in the morning. When the tinnitus gets really bad I put on background white noise, like the sound of the ocean or rain falling. And when the dizzy spells hit, I do the only thing I really can, sit or lay down, and close my eyes waiting for it to pass.

Haley and her hearing aid

Haley and her hearing aid

If you or someone you love has been diagnosed with Ménière’s, don’t worry—it's not the end of the world! You learn to cope and manage your flare-ups, and hearing aids are amazing. I cried tears of joy the first time I listened to music after I got them.

The important thing to remember is that you aren’t alone! There are others out there with Ménière’s. Join a group on Facebook or start your own. Talking to others who understand what it's like and what you are going through helps so much. Look at celebrities, like Katie Leclerc, who are dealing with it every day and rocking it.

And lastly, take care of yourself. On bad days, pace yourself and do what you need to do to feel better. Always remember, “This too shall pass.”

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10 Signs and Symptoms of Ménière's Disease

By Catherine Roberts

According to the Mayo Clinic, Ménière's Disease affects the inner ear and causes bouts of vertigo due to fluid that fills the tubes of your inner ear. On top of the dizziness and nausea, flare ups can also cause some loss of hearing in one or both ears and a constant ringing sound. You might not be able to drive, work, or do regular daily activities at times, though this chronic condition varies greatly from person to person. Unfortunately, the disease is not curable, but there are many treatment options that successfully help manage the disease.

Keep an eye out for these ten signs and symptoms of Ménière's Disease, and see your doctor if you experience any of them…

  1. Dizziness
    Dizziness can be caused by a lot of different diseases and infections, but it’s one of the main symptoms associated with Ménière's disease. Your inner ear is responsible for your balance and since the disease attacks the tubes in your inner ear, dizziness is bound to occur when experiencing a flare up of the disease. If you experience dizziness as a result of an attack of Ménière's disease, it may cause you to unsteady on your feet and feel like the world is moving unsteadily around you. In severe cases, dizziness can make you fall over or trip while walking, making it dangerous to be driving, working, or even simply walking.

  2. Nausea
    Nausea is another common symptom. Feeling sick to your stomach and actually vomiting can easily occur when you’re experiencing severe dizziness as well. When attacks of the disease come on, symptoms of vertigo are felt for as little as half an hour to as long as a full 24 hours. Though these attacks are seemingly short-lived, nausea and other symptoms can be felt strongly during the whole flare up. Although a symptom like nausea is extremely inconvenient and unpleasant to deal with, there are over-the-counter medications for motion sickness and prescription anti-nausea medications that your doctor can provide that could greatly help manage the attack. There are also some antibiotics that can be injected into the ear to help relieve symptoms if necessary.

  3. Hearing Loss
    Though usually temporary and in one ear, permanent hearing loss happen to some over time. Many patients describe the hearing loss as if their ear is plugged—sound can seem distorted, fuzzy, far away, or even tinny. Sensitivity to sound is also common, and finding a way to successfully remove the fluid helps improve any hearing problems the patient suffers from.

  4. Ringing In the Ear (Tinnitus)
    According to the Mayo Clinic, Ménière's disease can cause tinnitus, unfortunate sounds in the ear described as ringing, hissing, roaring, buzzing or whistling. Since the disease affects people in different ways and with varying symptoms, some patients may experience tinnitus without vertigo and hearing loss, or a combination of several symptoms. While not painful, any sort of foreign sound like ringing in the ear can be really difficult to cope with.

  5. Uncontrolled Eye Movements
    Some people who have the disease experience a sort of jerky eye movement in one or both eyes, side to side, up and down, or in a circular motion. It varies depending on the patient and each attack. The inner ear directly affects balance, and in turn, the movement of your eyes. Tests are done to check the response of your eyes when your inner ear balance is changed.

  6. Diarrhea
    Those who experience diarrhea might be more likely to also be experiencing vertigo during the same attack. To make matters worse, diarrhea can continue to occur after flare ups, not just during them. This makes it very important for you to stay hydrated when you’re struggling to cope with these symptoms of Ménière's disease. Since vertigo can cause you to throw up, the combination of vomiting and diarrhea will make you extremely dehydrated, and cause other health problems if not corrected. Along with diarrhea, abdominal pain and other gastrointestinal discomfort is possible.

  7. Cold Sweats
    When in the middle of an attack, people who experience vertigo symptoms—nausea, dizziness, and even vomiting—could also develop cold sweats, adding another uncomfortable symptom to deal with. Since cold sweats are typically a result of vertigo in the case of Ménière's disease, vertigo medication could eliminate or greatly reduce this and other vertigo symptoms. Depending on how severe your episodes are, your doctor may prescribe an anti-nausea medication to reduce the harmful symptoms of the attacks.

  8. Feelings of Fatigue
    It’s thought that those with Ménière's disease are more sensitive or susceptible to fatigue, increasing the risk of falling prey to an attack. Some patients have also shown that fatigue could be what causes flare ups, so people with the disease should adjust their lifestyle as needed to reduce the risk of becoming too tired. Work on sleeping well and not over-working or over-extending your capabilities.

  9. Extreme Mood Changes
    People with Ménière's disease have claimed feeling a variety of changes in their mood both during episodes and in-between them, from anger and irritability to anxiety and fear. These mood swings and feelings of instability can be caused by many things, so they aren’t necessarily from Ménière's disease. It’s also worth it to note that mood changes and roller coaster-type feelings aren’t necessarily a physical reaction from the disease—the Mayo Clinic explains that it’s currently unknown if anxiety contributes to and causes episodes or if anxiety is a by-product of the disease, occurring after attacks.

  10. Migraines
    A sign of Ménière's disease that’s easy to overlook are severe headaches, specifically migraines. There are so many dietary and lifestyle causes of migraines that it’s no wonder this sign can be overlooked. That said, once you really think about the disease and what it attacks, it’s not so hard to see the connection. It’s thought that migraines can cause damage to the inner ear, and those with Ménière's disease are more likely to get migraines during flare ups.

Excerpts of this article was repurposed with permission of the author and originally appeared on activebeat.com January 23, 2016.


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