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Almost 150 years ago, French physician Prosper Ménière first described the symptoms and probable cause of the inner ear disorder that bears his name. Unfortunately, despite decades of research, we’ve only made baby steps forward and Ménière’s still remains an idiopathic disease with controversial treatments and no cure, said Cliff Megerian, MD, Professor and Vice Chairman of Otolaryngology and Director of Otology and Neurotology at Case Western Reserve University Hospitals in Ohio.
With a prevalence rate of about 1,000 cases per 100,000 persons in the US population,1 this chronic disease typically starts between 20 and 50 years of age, increases in prevalence with age, affects men and women almost equally, and has unclear racial predispositions.2
Etiology
Since 1938, most otolaryngologists have recognized the causation of Ménière’s disease as endolymphatic hydrops, but this theory has now come into question, leaving the underlying cause(s) still unknown. Recent research indicates that Ménière’s disease probably has multiple etiologies, including autoimmune disorders, allergies, viruses, hereditary predisposition, and head injuries. The controversy is based on the fact that, upon autopsy, hydrops are not always found in persons with Ménière’s disease, yet they are commonly found in people who did not have Ménière’s type symptoms.3
Researchers are now looking less at hydrops and more specifically at the immunologic function of the endolymphatic sac, which is part of the immune system of the ear. Immune stimulation of the sac may disturb its fluid regulatory function or may cause hydrops to develop via independent mechanisms, such as the production of inflammatory mediators.4
Evidence also indicates that inhalant and food allergies may play a role in Ménière’s disease, causing dysfunction in the endolymphatic sac. Although more research is needed to substantiate that viruses are a possible cause, several investigators have found evidence of herpes simplex virus5,6 and varicella-zoster virus7 in the endolymphatic sac of patients with Ménière’s disease.
About one in three patients with Ménière’s disease has a first-degree relative with the disease; hereditary predisposition may be related to differences in anatomy of fluid channels within the ear or differences in immune response. Cases of post-traumatic Ménière’s disease are attributed to hydrodynamic changes caused by scarring from bleeding into the inner ear following a head injury or possibly ear surgery.4
Symptoms
The dilemma we face today is that once Ménière’s disease is diagnosed, we know how to control the vertigo, but we don’t know how to stop the decline in hearing. We need insight now as to the mechanism that causes hearing loss in this disease so that we can develop inhibitors of this process in the future.-Cliff Megerian, MD
Patients with Ménière’s disease usually experience an attack characterized by a tetrad of symptoms: tinnitus, aural fullness in one ear, fluctuating hearing loss, and episodic rotational vertigo that leads to imbalance, severe nausea, vomiting, and sweating. During an attack, patients commonly have visual dependence and nystagmus.
These symptoms are acute and vary in duration, from 20 minutes to two hours or longer. Multiple attacks may occur in clusters during short periods of time, or attacks might be as infrequent as once a year or less.
However, between attacks most patients are either asymptomatic or experience mild symptoms; their hearing loss may recover intermittently, but progressively worsens over time from an initial low-frequency sensorineural pattern to a flat loss or a peaked pattern.4
Depending on their intensity, these symptoms may be just a nuisance for patients or negatively affect their quality of life, making it impossible to perform normal activities of daily living. Vertigo tends to be the most debilitating symptom, especially since it can occur with little or no warning. Although an attack can be incapacitating, leaving patients exhausted, nauseated, and prone to falls, the disease itself is not fatal.
The disorder usually affects only one ear in the beginning, but some researchers state that after 15 years or more, roughly 50% of patients have bilateral Ménière’s disease;8 others suggest that the prevalence of bilaterality is closer to 17%.9
Diagnosis
The diagnosis of Ménière’s disease requires expert clinical judgment, not only because of its multifactorial etiology and broad differential diagnosis, but also because guidelines set forth by the American Academy of Otolaryngology Head and Neck Surgery (AAO-HNS) Committee on Hearing and Equilibrium are more sensitive and less specific in diagnosing the disease than Ménière’s original description of the disease.10
What seems like Ménière’s disease with unilateral hearing loss and vertigo is not always Ménière’s disease, said Dr. Megerian. Therefore, the AAO-HNS guidelines have categories like ‘probable’ as part of their diagnostic criteria. The only time that a diagnosis of Ménière’s disease is ‘certain’ is at post-mortem exam, forcing us to use indirect ways to ensure that something else is not masquerading as Ménière’s disease.
Diagnosis is based on a combination of the right set of symptoms (sidebar 1) and begins with a history (sidebar 2) to primarily check for vertigo, and a physical that includes a battery of tests: caloric, Romberg, Fukuda marching step, Dix-Hallpike, Weber tuning fork, Rinne, electronystagmography (ENG), videonystagmography (VNG), auditory brain stem response (ABR), transtympanic electrocochleography (ECoG), and vestibular evoked myogenic potential (VEMP).
Additionally, an MRI and CT may be needed to detect abnormal masses, lesions or tumors, and dehiscent superior semicircular canals and widened cochlear and vestibular aqueducts, respectively.1
Allergy testing may be needed, as well as the following panel of blood tests:
- Thyroid-stimulating hormone (TSH), T4, and T3 to rule out hyperthyroidism and hypothyroidism.
- Glucose to rule out diabetes.
- Sedimentation rate and antinuclear antibody to rule out autoimmune disorders.
- Urine to rule out proteinuria and hematuria and indicators of otorenal syndrome.
- CBC count to rule out anemia and leukemia.
- Electrolyte levels to rule out salt/water imbalance.
- Fluorescent treponemal antibody (FTA-ABS) to rule out neurosyphilis and Lyme disease.1
The differential diagnosis of Ménière’s disease includes:
- Benign paroxysmal positional vertigo
- CNS causes of vertigo
- Inner ear
- Autoimmune disease
- Evaluation of dizziness
- Labyrinthitis
- Perilymphatic fistula
- Tinnitus
- Migraine-associated vertigo
- Thyroid, thyrotoxic storm following thyroidectomy.1
Otolaryngologists should also rule out alternative causes: congenital anomalies, high cholesterol or triglyceride levels, inner ear inflammation, microvascular compression syndromes, multiple sclerosis, otosclerosis, transient ischemic attacks and stroke, trauma, and viral infections.1
Treatment
Once the tests are complete and a patient meets the AAO-HNS diagnostic criteria for Ménière’s disease, there are numerous medical and surgical therapies available, said Michael Hoffer, MD, CDR MC USN, Director of the Spatial Orientation Center in the Department of Otolaryngology at the Naval Medical Center in San Diego.
Even though the AAO-HNS guidelines were established in 1995, they are still relevant because there hasn’t been a significant change in our understanding of the management and treatment of Ménière’s disease since then, said Dr. Megerian. Truth be told, the conservative treatment for Ménière’s disease hasn’t really changed much since the early 1900s.
All patients begin management of their disease through lifestyle and dietary changes to reduce the number of attacks. Physicians encourage their patients to avoid alcohol, caffeine, excessive fatigue, smoking, and stress, as well as to eat properly, get plenty of sleep, and remain physically active.
The Hydrops Diet, which involves reducing salt intake and taking a diuretic (triamterene/hydrochlorothiazide [Dyazide], amiloride/HCTZ [Moduretic], acetazolamide [Diamox]) for at least a three-month trial period, is intended to keep sodium concentrations in the inner ear from fluctuating, which causes the symptoms of hydrops. Strict adherence to a 1.5- to 2.0-gram salt diet stabilizes symptoms in most patients;2 some otolaryngologists refer patients to a dietician to help with this dietary challenge.
I also recommend that my patients drink six to eight glasses of water per day because it dilutes the sodium concentration and lowers their tendency to retain fluids, said Dr. Megerian.
Patients who present with acute, severe vertigo may be treated with vestibulosuppressants (meclizine [Antivert], lorazepam [Ativan], clonazepam [Klonapin], diazepam [Valium]) and antiemetics (promethazine [Phenergan], prochlorperazine [Compazine]), which are used on an as-needed basis, as they cause drowsiness and harm in the long term.1 Similar medications are also used in between attacks.
The majority of, if not all, ENTs agree that the treatment of Ménière’s disease begins with the Hydrops Diet and medications to mitigate or prevent symptoms, said Dr. Hoffer.
Almost 90 percent of patients are adequately managed and have resolution of their vertigo and stabilization of their hearing with this conservative treatment, added Dr. Megerian. Many well-respected otolaryngologists agree that Ménière’s disease is more a medical than a surgical disorder. Only 10 to 15 percent of patients must try further interventions, some more destructive than others, but this still represents a large number of patients.
The batting order, as to what treatments come next, is not only controversial, but dependent upon the ENT’s training, comfort level with specific therapies and geographic practice location.
In my practice, I commonly treat patients who have intractable Ménière’s disease with intratympanic gentamicin and endolymphatic sac decompression or shunt to control their vertigo, said Dr. Megerian.
Otolaryngologists are increasingly using office-based intratympanic perfusion11 to deliver high concentrations of medication directly to the inner ear cavity with minimal side effects. The aminoglycoside antibiotic, gentamicin, is most often used, even though it is an ototoxin. Usually four injections are given over a period of a month, but some otolaryngologists are successfully switching to a low-dose gentamicin protocol of only one or two shots per month.4 The procedure itself, done with either single or multiple injections, is low-risk and simple to perform, but is considered destructive, as it can leave patients with little or no balance function. Even though studies show an efficacy at about 80% to 90% for vertigo control, authors report substantial worsening of hearing in up to 25% of patients.12,13
Endolymphatic sac decompression involves removal of petrous bone from around the endolymphatic sac, whereas shunting requires the placement of a synthetic shunt to drain endolymph into the mastoid. Both procedures appear to reduce pressure, control vertigo, and stabilize hearing acuity equally.13
Even though this is a time-tested surgical operation, there is still some controversy due to a 1981 Danish study that suggested that outcomes from this procedure were no different than a sham procedure and were just a placebo effect, said Dr. Megerian. Almost two-thirds of my patients find that it controls their vertigo and I am very pleased with the results. The best thing is you don’t burn any bridges; if it isn’t successful, you can still use intratympanic gentamicin.
Other generally well-accepted therapies include vestibular nerve section and labyrinthectomy. In vestibular neurectomy, the diseased vestibular nerve is clipped where it leaves the inner ear and goes to the brain. This procedure often requires a neurosurgical approach (middle fossa or retrosigmoid performed through a small craniotomy) and is considered riskier than intratympanic gentamicin injections. Even though vertigo attacks are eliminated in about 95% to 98% of cases and hearing is preserved in the surgically treated ear about 95% of the time, patients are often reluctant to undergo this invasive treatment.13
There has been a dramatic reduction in the number of vestibular nerve sections or ‘Dandy operation’ as it is sometimes called, since the introduction of intratympanic gentamicin, said Dr. Megerian.
A labyrinthectomy is an old operation that is only recommended for a select group of patients who have lost all usable hearing on the affected side, failed other interventions or determined that vestibular nerve section is too dangerous for their situation, he continued.
This procedure involves ablation of the diseased inner ear organs. Because it can be performed through a basic mastoidectomy or transcanal approach and does not require entry into the cranial cavity, it is less complex than a vestibular nerve section. Labyrinthectomy has a high cure rate for controlling vertigo attacks, similar to vestibular nerve section.
Regardless of which of these two surgical therapies is done, patients who participate in vestibular rehabilitation afterwards demonstrate significant improvement in balance function,14 according to Dr. Hoffer.
Vestibular rehabilitation is a relatively new application for Ménière’s disease because general rehabilitation is usually only good for disorders that are constant, said Dr. Hoffer. Due to the fluctuating nature of Ménière’s disease and its unpredictability, vestibular rehabilitation would appear to have a limited role as a treatment option. As it turns out, it works well because of the underlying disequalibrium that is present in and frustrating for most patients with Ménière’s disease.
Even if you can stop the fluctuation of symptoms, patients will still have the unsteadiness; vestibular rehabilitation is physical therapy for the balance system, continued Dr. Hoffer. More hospitals are developing a balance therapy program where patients with all types of balance and hearing disorders can be evaluated.
Other Treatment Options
If patients don’t respond well to any of these treatments, then other, not-so-standard options may be considered. Some otolaryngologists are trying:
- Vasodilators/calcium channel blockers (verapamil), as they are used to treat migraine-associate vertigo.
- Antihistamines, such as betahistine (Serc), which is available only in Canada and Europe.
- Antiviral therapy, such as acyclovir.
- Immunologic therapy.
- Intratympanic and systemic steroids (dexamethasone, prednisone, methylprednisoline).
There is growing research regarding intratympanic steroids; it will be exciting to see their short and long-term benefits and where they ultimately fit in amongst the treatment options, said Dr. Megerian.
Evidence shows that a single injection of steroids may be ineffective, as the steroids do not remain in the ear for long; however, multiple injections per year or those done on an as-needed basis appear more effective and to have longer-lasting results.4 Some researchers are mixing steroids with hyaluronic acid, because of its viscosity, to see if they would remain in the ear longer. Transtympanic perfusion using steroids is considered a nondestructive procedure and success rates near 90% have been reported.13
Finally, the Meniett device is designed to reduce vertigo by delivering pulses of pressure to the inner ear via a tympanostomy tube; some patients have symptomatic relief when the device is used on a daily basis.
Future Promises
Since it’s so common, Ménière’s disease has been highly researched and fascinated our profession for years, yet that ‘golden ring’ of how to prevent endolymphatic hydrops from occurring still eludes us, said Dr. Hoffer.
The disease has certainly captured the hearts and minds of otolaryngologists because of its many controversies. This is good, as it forces us to keep an open mind about alternative therapies and not rely on what we learned 20 to 30 years ago or how we were trained.
The dilemma we face today is that once Ménière’s disease is diagnosed, we know how to control the vertigo, but we don’t know how to stop the decline in hearing, said Dr. Megerian. We need insight now as to the mechanism that causes hearing loss in this disease so that we can develop inhibitors of this process in the future. Unfortunately, we are nowhere close to doing this.
One of the exciting things I see in the future of otolaryngology is being able to administer pharmaceutical agents, through a tympanostomy tube, that either protect or preserve hearing, continued Dr. Megerian. I think in the next five to 10 years, new imaging protocols with high-powered MRI scans will allow us to home in on the inner ear and see the anatomical changes to confirm a diagnosis of Ménière’s disease in living patients with certainty.
The disease has certainly captured the hearts and minds of otolaryngologists because of its many controversies. This is good, as it forces us to keep an open mind about alternative therapies and not rely on what we learned 20 to 30 years ago or how we were trained.-Michael Hoffer, MD
AAO-HNS Definitions of Ménière’s Disease
Possible
- Episodic vertigo of the Ménière’s type (>20 minutes, associated with horizontal rotatory nystagmus) without documented hearing loss, or
- Sensorineural hearing loss, fluctuating or fixed, with disequilibrium, but without definitive episodes
- Other causes excluded
Probable
- One definitive episode of vertigo
- Audiometrically documented hearing loss on at least one occasion
- Tinnitus or aural fullness in the treated ear
- Other causes excluded
Definite
- Two or more definitive spontaneous episodes of vertigo 20 minutes or longer
- Audiometrically documented hearing loss on at least one occasion
- Tinnitus or aural fullness in the treated ear
- Other cases excluded
Certain
- Definite Ménière’s disease, plus histopathologic confirmation
AAO-HNS Functional Level Scale
Regarding my current state of overall function, not just during attacks (check the ONE that best applies):
- My dizziness has no effect on my activities at all.
- When I am dizzy I have to stop what I am doing for a while, but it soon passes and I can resume activities. I continue to work, drive, and engage in any activity I choose without restriction. I have not changed any plans or activities to accommodate my dizziness.
- When I am dizzy, I have to stop what I am doing for a while, but it does pass and I can resume activities. I continue to work, drive, and engage in most activities I choose, but I have had to change some plans and make some allowance for my dizziness.
- I am able to work, drive, travel, take care of a family, or engage in most essential activities, but I must exert a great deal of effort to do so. I must constantly make adjustments in my activities and budget my energies. I am barely making it.
- I am unable to work, drive, or take care of a family. I am unable to do most of the active things that I used to. Even essential activities must be limited. I am disabled.
- I have been disabled for 1 year or longer and/or I receive compensation (money) because of my dizziness or balance problem.
References
- Li JC. Inner Ear, Meniere Disease, Medical Treatment. January 17, 2007. Retrieved November 10, 2007 from eMedicine from WebMD website: www.emedicine.com/ent/TOPIC232.htm .
[Context Link] - Hain, TC. Epidemiology of Ménière’s Disease. September 16, 2003. Retrieved November 10, 2007 from Dizziness and Balance website: www.dizziness-and-balance.com/disorders/menieres/men_epi.html .
[Context Link] - Rauch SD, Merchant SN, Thedinger BA. Ménière’s syndrome and endolymphatic hydrops. Double-blind temporal bone study. Ann Oto Rhinol Laryngol 1989;98(11):873-83.
[Context Link] - Hain TC. Ménière’s Disease. April 8, 2007. Retrieved November 10, 2007 from Dizziness and Balance website: www.dizziness-and-balance.com/disorders/menieres/menieres.html .
[Context Link] - Arnold W, Niedermeyer HP. Herpes simplex virus antibodies in the perilymph of patients with Ménière’s disease. Arch Otolaryngol Head Neck Surg 1997;123(1):53-6.
[Context Link] - Vrabec JT. Herpes simplex virus and Ménière’s disease. Laryngoscope 2003;113(9):1431-8.
[Context Link] - Yazawa Y, Suzuki M, Hanamitsu M, et al. Detection of viral DNA in the endolymphatic sac in Ménière’s disease by in situ hybridization. ORL J Otorhinolaryngol Relat Spec 2003;65(3):162-8.
[Context Link] - Stahle J, Friberg U, Svedberg A. Long-term progression of Ménière’s disease. Acta Otolaryngol Suppl 1991: 485:75-83.
[Context Link] - Silverstein H, Rosenberg SI. Surgical Techniques of the Temporal Bone and Skull Base. Philadelphia: Lea and Febinger, 1992.
[Context Link] - Stapleton E, Mills R. Clinical diagnosis of Ménière’s disease: how useful are the American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium guidelines? J Laryngol Otol 2007;Oct 12:1-7.
[Context Link] - Silverstein H, Lewis WB, Jacson LE, et al. Changing trends in the surgical treatment of Ménière’s disease: results of a 10-year survey. Ear Nose Throat J 2003;82(3):185-7, 191-4.
[Context Link] - Martin E, Perez N. Hearing loss after intratympanic gentamicin therapy for unilateral Ménière’s disease. Otol Neurotol 2003;24(5):800-6.
[Context Link] - Li, JC. Inner Ear, Meniere Disease, Surgical Treatment. January 17, 2007. Retrieved November 10, 2007 from eMedicine from WebMD website; www.emedicine.com/ent/TOPIC233.htm .
[Context Link] - Gottshall KR, Hoffer ME, Moore RJ, Balough BJ. The role of vestibular rehabilitation in the treatment of Ménière’s disease. Otolaryngol Head Neck Surg 2005;133(3):326-8.
[Context Link]
Laryngoscope Highlights
Flap Reconstruction in Conjunction with Intraoperative Radiation Therapy
For patients with advanced or recurrent head and neck cancer, major resections combined with complex reconstructions are frequently necessary to obtain locoregional control and restore form and function. These patients may benefit from intraoperative radiation therapy (IORT) because it allows high doses of radiation to be delivered directly to the tumor bed while avoiding adjacent tissues. IORT is especially useful for patients who have received radiation previously or who have tumors near radiation-intolerant tissues. The higher doses, however, raise concerns regarding wound healing, flap survival, and functional outcome. Michael D. Most, MD, and associates present 22 cases (in 21 patients) of head and neck cancer in which IORT was used in conjunction with immediate flap reconstruction.
The investigators performed a retrospective chart review of all cases involving ablative head and neck surgery, IORT, and immediate flap reconstruction in one medical center over a seven-year period. Wound healing was graded excellent, satisfactory, or poor by the reconstructive surgeon. All but one of the patients had received prior treatment and presented with locoregionally recurrent disease requiring salvage therapy.
In total, 25 flap reconstructions were performed in the 21 patients at the time of IORT. Of these, 11 were free tissue transfers and 14 were pedicled tissue transfers. All flaps were inset directly into the field of IORT. None of the patients developed any flap sequelae or wound complications. Wound healing was described as excellent (uncomplicated) in 17 cases, satisfactory in two cases, and poor in three cases. All patients in the group with poor healing required surgical revisions and were considered to have major complications.
The researchers point out a number of advantages of IORT over traditional fractionated external beam radiation therapy (EBRT). Tumor margins can be assessed at the time of surgery, and the surgeon and radiation oncologist can accurately define the borders of the tumor bed. Shielding can be used to protect sensitive organs adjacent to the radiation field, thus increasing the allowable radiation dose. IORT can also be used in conjunction with adjuvant EBRT to lower the postoperative radiation dose required. These factors all contribute to minimizing complications of radiation therapy while providing the highest possible dose.
The investigators observed that morbidity of perioperative radiotherapy was significantly decreased after the introduction of the deltopectoral and pectoralis major pedicled flaps for head and neck reconstruction. Microvascular reconstructive techniques have expanded reconstructive options, with concomitant improvement of functional and aesthetic results. The investigators feel that head and neck flap reconstruction can be performed in conjunction with IORT with the expectation of good wound healing in most cases.
(Laryngoscope 2008;118:69-74)
©2008 The Triological Society