Andrew Chin / C. Flanigan / Walter McBride (Getty Images)
En español | Rocker Huey Lewis, 67, had to put his career on hold recently when he was diagnosed with Meniere’s disease, a condition of the inner ear that can cause permanent hearing loss as well as balance issues.
Lewis is one of the approximately 615,000 in the U.S. who have the condition, according to the National Institutes of Health. In his case, it caused him to lose his pitch. “I can’t hear music well enough to sing. The lower frequencies distort violently, making it impossible to find pitch,” the singer said, announcing the cancellation of all his 2018 tour dates.
Meniere’s, named for the French physician Prosper Ménière, who discovered it in the 1860s, isn’t new. However, with more than 45,000 new cases diagnosed each year, and celebrities such as Lewis, actress and singer Kristin Chenoweth, and singer Ryan Adams among the high-profile people who have revealed their struggles with the disease, it has become better known in recent years. There is no known cause or cure for the condition, but it is treatable. Here’s what to know.
A Meniere’s diagnosis requires all four major symptoms.
Because a cause hasn’t been identified, the disease is defined by its four major symptoms. The first is a sudden, spontaneous attack of prolonged vertigo — violent rotary spinning and dizziness — occurring from 20 minutes to eight hours at a time. The second is a fluctuating hearing loss of lower frequencies just before, during or right after the vertigo attack. The third is roaring tinnitus — ear ringing, or the perception of sound in the absence of sound — that rises with the attack and then abates. The fourth symptom is a sensation of the affected ear’s canal filling up.
"If you don’t have that spectrum of symptoms, then you don’t meet the criteria,” says Gregory Basura, M.D., assistant professor in the Department of Otolaryngology–Head and Neck Surgery at the University of Michigan.
Meniere’s symptoms mimic those of other conditions, so it’s important to see a specialist to get a proper diagnosis.
“A lot of providers might not truly know the nuance of the disease,” Basura says. “You want to go to a specialist who’s familiar with Meniere’s disease. I see a lot of people who are diagnosed with Meniere’s who don’t actually have it.”
Basura sees many patients complaining of vertigo, one of the trademarks of Meniere’s. But vertigo is also a symptom of many other conditions, including benign situational vertigo, crystals in the ear and vestibular migraines. So it’s important to see an audiologist or otolaryngologist — a doctor specializing in conditions of the ear — who may administer specific tests such as an audiogram to measure sustained hearing loss or an electrocochleogram to measure fluid pressure in the ear, both of which can help narrow the cause of symptoms.
The disease can come on at any age, and symptoms abate and reappear randomly.
Basura says that most people who are diagnosed with the disease are between the ages of 40 and 60 but that symptoms can appear at any point. And while Huey Lewis has been around loud noise as a rock singer for a number of years, Basura says noise exposure has little to do with a diagnosis. In fact, “there’s no predisposing thing” to the condition.
After symptoms do appear, they can come and go, with some people experiencing attacks several times per week and some experiencing them only once a month. The disease is incurable, but it is possible to undergo treatment to prevent attacks and further hearing loss.
Meniere’s treatments depend on how much permanent damage has occurred to hearing.
When a patient is first diagnosed with Meniere’s and still has usable hearing, the first treatment plan is often a strict low-sodium diet. Because a trigger for attacks is thought to be the rising levels of fluid in the inner ear, a low-sodium intake, sometimes paired with a diuretic, keeps fluid levels below the threshold thought to trigger symptoms or an attack.
Often, strictly keeping to a low-sodium diet is enough for patients to fend off future attacks. “There are a lot of people that when they go on sodium restriction, and they’re hypervigilant, they do well,” Basura says.
If a patient is strict about sodium intake and symptoms persist, further treatments can include a Meniett device — a small tube placed in the inner ear that delivers ultrasonic pulses to help offset pressure buildup — as well as steroid and antibiotic injections.
For patients with no hearing remaining in an affected ear, a last resort is often a transmastoid labyrinthectomy — a surgical procedure to "literally drill out the inner ear," according to Basura. Patients requiring this level of treatment need postsurgical physical therapy to train their brains to compensate for the loss of balance perception within the ear.
There’s no cure yet, but research shows promise.
Treatments in development center on individualized gene therapy. “We’re getting more sophisticated with our genetic analysis and our understanding of disease and inner ear stuff, so I think I’m optimistic,” Basura says.
But while a cure is not imminent, there is more understanding about diagnosis and treatments. Basura is the chair of a team writing "Clinical Practice Guideline: Meniere’s Disease," which will provide action items to doctors around the world to help guide a diagnosis and provide treatments for patients. The guidelines, endorsed by the Academy of Otolaryngology–Head and Neck Surgery, will be released next summer.