British singer Jessie J (born Jessica Ellen Cornish) spent her Christmas Eve in the hospital. The 32-year-old “Domino” singer was diagnosed with Ménière’s disease after she complained of difficulty hearing and dizziness.
In an Instagram Stories video, Jessie said “I woke up … felt like I was completely deaf in my right ear, couldn’t walk in a straight line…. I got told I had Ménière’s syndrome. I know that a lot of people suffer with it, and I’ve actually had a lot of people reach out to me and give me great advice … I’ve just been laying low in silence.”
Jessie went on to quip that she had to watch the first episode of “The Queen’s Gambit” four times, with her finger in her ear because it sounded like “someone crawled in [my ear] and turned a hairdryer on.”
On Christmas day, Jessie said she was glad that she had seen a doctor early and “got put on the right medicine, and I feel much better today.”
What Is Ménière’s Disease?
Ménière’s disease is a disorder of the inner ear that causes severe vertigo, tinnitus, hearing loss, and a feeling of fullness or congestion in the ear. Ménière’s disease usually affects only one ear.
Attacks of dizziness may come on suddenly or after a short period of tinnitus or muffled hearing. Some people will have single attacks of dizziness separated by long periods of time. Others may experience many attacks closer together over a number of days. Some people with Ménière’s disease have vertigo so extreme that they lose their balance and fall. These episodes are called “drop attacks.”
Ménière’s disease can develop at any age, but it is more likely to happen to adults ages 40 to 60. The National Institute on Deafness and Other Communication Disorders (NIDCD) estimates that approximately 615,000 individuals in the U.S. are currently diagnosed with Ménière’s disease and that 45,500 cases are newly diagnosed each year.
Potential Etiologies
The underlying cause of Ménière’s disease is unknown, although it probably results from a combination of environmental and genetic factors. Possible causes that have been studied include viral infections, trauma to the middle ear, middle ear infection (otitis media), head injury, a genetic predisposition, syphilis, allergies, abnormal immune system response, migraines, and noise pollution. Several autoimmune diseases are associated with Ménière’s disease, namely rheumatoid arthritis, systemic lupus erythematosus, and ankylosing spondylitis.
The symptoms of Ménière’s disease are caused by the buildup of fluid in the compartments of the inner ear, called the labyrinth. The labyrinth contains the organs of balance (the semicircular canals and otolith organs) and of hearing (the cochlea). It has two sections: the bony labyrinth and the membranous labyrinth. The membranous labyrinth is filled with a fluid called endolymph that, in the balance organs, stimulates receptors as the body moves. The receptors then send signals to the brain about the body’s position and movement. In the cochlea, fluid is compressed in response to sound vibrations, which stimulates sensory cells that send signals to the brain.
In Ménière’s disease, the endolymph buildup in the labyrinth interferes with the normal balance and hearing signals between the inner ear and the brain. This abnormality causes vertigo and other symptoms of Ménière’s disease.
Treatments
At the present time, there is no cure for Ménière’s disease, but lifestyle modifications and some treatments can help affected people cope with the symptoms. The symptoms of the disease are often controlled successfully by reducing the body’s retention of fluids through dietary changes (such as a low-salt or salt-free diet and no caffeine or alcohol). Medications such as antihistamines, anticholinergics, and diuretics may lower endolymphatic pressure by reducing the amount of endolymphatic fluid. Eliminating tobacco use and reducing stress levels may also help lessen the severity of symptoms.
Symptoms such as dizziness, vertigo, and associated nausea and vomiting may respond to certain medications. The FDA has approved a device for Ménière’s disease called a Meniett device. The device fits into the outer ear and delivers intermittent air pressure pulses to the middle ear (pressure pulse treatment). The air pressure pulses appear to act on endolymph fluid to prevent dizziness. According to a 2005 study, “the device is portable and self-administered, requiring a 5-min three-times daily application for an indefinite period. Placement of a tympanostomy tube in the eardrum of the affected ear is necessary. Patient acceptance has been high. Long-term success in control of vertigo is over 80%. Although hearing loss is not improved, Meniett device usage does not adversely affect balance, as do most forms of surgical therapy.”
Different surgical procedures are an option for people with persistent, debilitating vertigo. Labyrinthectomy (removal of the inner ear sense organ) can effectively control vertigo but sacrifices hearing and is reserved for patients with nonfunctional hearing in the affected ear. Vestibular neurectomy, selectively severing a nerve from the affected inner ear organ, usually controls the vertigo while preserving hearing but carries surgical risks. Recently, the administration of the ototoxic antibiotic gentamicin directly into the middle ear space has gained popularity worldwide for the control of vertigo associated with Ménière’s disease.
Prognosis
Scientists estimate that six out of 10 people either get better on their own or can control their vertigo with diet, drugs, or devices. However, a small group of people with Ménière’s disease will get relief only by undergoing surgery.
The course of Ménière’s disease can vary widely among affected people. The condition is often most bothersome during the early stages. During progression to later stages, vertigo spells often disappear. Acute attacks are typically replaced by constant imbalance and progressive hearing loss.
The long-term outlook for people with Ménière’s disease also varies. Periods of remission punctuated by exacerbations of symptoms are typical. Some people have minimal symptoms, whereas others have severe attacks. Episodes may occur as infrequently as once or twice a year or they may occur on a regular basis. The pattern of exacerbation and remission makes evaluation of prognosis difficult. In general, the condition tends to spontaneously stabilize over time, and it is said to “burn out.” The spontaneous remission rate is high, with over 50% experiencing this within 2 years, and over 70% after 8 years. However, most people are left with poor balance and poor hearing. Those whose condition does not stabilize are generally well-managed with medications, but surgical treatment is necessary in about 5%-10% of cases.
Sources: NIDCD, Genetic and Rare Diseases Information Center, StatPearls: Meniere’s Disease, Ménière’s Society
Michele R. Berman, MD, and Mark S. Boguski, MD, PhD, are a wife and husband team of physicians who have trained and taught at some of the top medical schools in the country, including Harvard, Johns Hopkins, and Washington University in St. Louis. Their mission is both a journalistic and educational one: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.
Last Updated January 05, 2021