Meniere’s Disease: Experts Discuss Diagnosis and Treatment

From its sometimes confusing overlap with migraine to limited evidence for systemic treatments, Ménière’s disease poses challenges to clinicians. Expert panelists offered their insights into managing the disorder at the Triological Society Combined Sections Meeting.

Yuri Agrawal, MD, associate professor of otolaryngology–head and neck surgery at Johns Hopkins School of Medicine in Baltimore, said that while an audiogram is the only testing required to make a diagnosis of Meniere’s, other tests could play a role in certain situations. Caloric testing, she noted, is the most sensitive in correctly identifying the affected ear, followed by cervical vestibular-evoked myogenic potential (cVEMP).

MRI and cVEMP, she said, could help with further management. “It might be useful, for example, when contemplating an ablative procedure for an index ear … to know what might be the
status of the contralateral ear,” she said. “If you do see evidence of hydrops in MRI of the contralateral ear, or altered cVEMP, that might suggest that an ablative therapy might be in store for the other ear, too, and may change your management accordingly.”

Other panelists said they typically only obtain an audiogram in suspected cases of Ménière’s, but, if further treatment is needed, they would be inclined to get an electronystagmogram (ENG) or other tests. Brian Blakley, MD, PhD, professor of otologic and neurotologic surgery at the University of Manitoba, said he tends to order MRI regularly to rule out acoustic neuroma.

Similarities with Vestibular Migraine

Hamid Djalilian, MD, professor of neurotology and skull base surgery at the University of California in Irvine, said his group started, approximately nine years ago, treating patients with Ménière’s disease as they do migraine patients. Researchers have repeatedly found similarities between patients with Ménière’s and those with vestibular migraine, in symptoms and test results, and have suggested that a link in physiology could account for this.

In a study of 37 patients with definite Ménière’s disease at his center, 49% of whom met the International Headache Society (IHS) criteria for migraine, the overlap with migraine was even greater than it seemed. One hundred percent of the patients with Ménière’s experienced three sensitivities associated with migraine, a family history of migraine, or two migraine-related symptoms  (Laryngoscope. 2016;126:163–168).

“There’s a population of Ménière’s disease that doesn’t qualify for the IHS criteria for migraine, but they have a lot of the migraine-related symptoms,” Dr. Djalilian said.

At his center, among a cohort of 25 Ménière’s patients with long-term follow-up who didn’t qualify for a vestibular migraine diagnosis but were treated with a migraine regimen, 92% experienced significant improvement in quality of life. “When you look at our success rate, it’s really on par with vestibular nerve section,” a procedure meant to eliminate vertigo attacks.

Dietary Modification

Mia Miller, MD, a neurotologist with the House Clinic in Los Angeles, said counseling on dietary matters with Ménière’s patients can be tricky. Research has found that those with possible disease are more compliant with salt restrictions than those with definite disease (possibly because they’re more motivated to prevent more serious disease), but both groups have been found to show improvement in some studies.

Ultimately, though, the quality of the evidence is low. “There are not a lot of studies that are large enough, or randomized, to evaluate the effectiveness of sodium restriction,” she said. While some guidelines recommend keeping sodium intake to a very low level at 1,000 mg per day, some clinicians advocate a “no salt added” approach so that patients at least avoid heavily salted foods.

One study found that fewer than half of patients had been given written dietary guidelines, and while patients said following low salt or caffeine diets was feasible, they had trouble naming appropriate foods within those diets (Otol Neurotol. 2013;34:1438–1443). “This shows how difficult dietary counseling can be,” Dr. Miller said. “It would be good to give written sodium intake guidelines so people at least know what you’re trying to counsel them to do.”

She added that some physicians refer to a nutritionist if a patient is confused about what they should be eating, and she refers to an allergist if she thinks it could help control symptoms.

Systemic Medications

Dr. Blakley said there is no clear path for using systemic medications in treating Ménière’s. He ticked off a series of drug types for which there is insufficient or a low level of evidence: There is not enough evidence on diuretics, studies on antivirals have primarily not suggested efficacy, and he has found no strong endorsements for allergy treatments.

Betahistine is an interesting case, he added. There is evidence that it might reduce the number of vertigo attacks, but the certainty of the evidence is low (Medwave. 2017;17:e7068). “It doesn’t have significant side effects; in fact, this is why many of us think that it’s a placebo drug,” Dr. Blakley said. “It started off as a dose of 4 mg, and they’re supposed to get 8, then 16, then 32,” and even 48 mg. “I don’t know of any physiologic drug that you could increase the dose by that much and still have no side effects. …. There are certainly a lot of people who just love that drug. And it’s cheap, it’s safe, so why not, if they like it?”

The ambiguity might be a benefit because it forces clinicians to tailor according to the situation, he said. “I think we have many treatments, all with not very good data,” he said. “The factory model, I think, does not work; there isn’t one step that leads to another, and then do this, and so forth. There is a lot of variety and I think that may be a good thing. It allows us to consider cost, safety, and patient preferences.”

Betahistine is unavailable in the U.S.—though it is available in Canada, where Dr. Blakley practices—and he was asked whether he though U.S. clinicians should push for its availability. He said he didn’t think it’s an “emergency.” Even though the case for it may not be strong, he said, “I think it may be reasonable. There are certainly a lot of people who would use it.”

Intratympanic Gentamicin

For inner ear treatments, intratympanic gentamicin has been shown to be effective, but there is no strong agreement on appropriate dosing, timing, and delivery method, said Soha Ghossaini, MD, an otologist-neurotologist at Ear, Nose, and Throat Associates of New York. “We have to remember that intratympanic gentamicin can potentially cause hearing loss in a good percentage of the patients, depending on the dose,” she said.

A key question is when to use the drug in patients who have good hearing. “The consensus is to use them in patients with impaired hearing function and patients with good contralateral vestibular function,” she said.

Some researchers have advocated for a “titration” protocol and not systematic weekly or monthly injections, with end points of symptom resolutions and signs of hearing loss, realizing that these signs will be delayed by two or three days after treatment (Clin Otolaryngol. 2015;40:682-690). Tests such as ENG, VEMPs, and head-impulse test (HIT) were not found to establish end points for treatment with gentamicin, Dr. Ghossaini noted.

Intratympanic steroid therapy, particularly dexamethasone, is becoming more popular for vertigo control. Even though some studies have found that gentamicin controls vertigo better, steroids come with fewer risks, she said. She added that patients might need repeated treatments and that adding oral betahistine to steroid treatment has been shown in one study to produce better vertigo control. She added that no improvement in hearing has been found after long-term follow up.

Surgical Options

Surgical procedures for Ménière’s have been found to have a range in efficacy, Dr. Miller said.

Researchers have found that overpressure devices reduce vertigo frequency, but the results on hearing loss have been mixed (Clin Otolaryngol. 2015;40:682–690; Clin Otolaryngol. 2015;40:197–207; Cochrane Database Syst Rev. 2015;CD008419). The official American Academy of Otolaryngology-Head and Neck Surgery position is that there is “some medical evidence” to support their use as a second-level therapy after medical treatment has failed.

Endolymphatic sac surgery isn’t supported by histopathologic studies for shunt placement, but has been found to bring vertigo relief for some patients. “Although we don’t necessarily understand the basis behind it, it can be effective,” Dr. Miller said.

Vestibular nerve section has been found to lead to complete vertigo resolution in 85% of patients, based on two approaches that were studied, Dr. Miller said. The number isn’t 100%, she said, likely because the literature mostly reports on cases of transection of the nerve and not on cases that also involve destruction of Scarpa’s ganglion, which would bring about a more complete effect.

Cochleosacculotomy can be 60% to 80% effective in eliminating vertigo, she said.

Transcanal labyrinthectomy is a shorter procedure than transmastoid labyrinthectomy and has lower morbidity, but also has a lower success rate. It involves a transcanal approach, so it can be more difficult to remove all of the vestibular end organs, Dr. Miller said. This, she said, might be a better approach for older or frail patients. 

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Thomas R. Collins is a freelance medical writer based in Florida.