WHAT IS MÉNIÈRE’S DISEASE?
Ménière’s disease is a chronic vestibular (inner ear) disorder with often debilitating symptoms and no known cure. Ménière’s disease affects both hearing and balance. It is named for the French doctor Prosper Ménière who, in 1861, first identified and described the symptoms of this medical condition.
The disorder can be caused by a buildup of a fluid called endolymph in the compartments of the inner ear, called the labyrinth. The labyrinth contains the organs of balance (the semicircular canals and otolithic organs) and hearing (the cochlea).
The labyrinth has two sections: the bony labyrinth and the membranous labyrinth, the second of which is filled with endolymph which, 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.
This excess fluid–beyond the normal amount of fluid that exists in the cochlea–affects both balance and hearing. The cause of the fluid buildup is not yet completely understood. Some researchers believe it is related to the same blood vessel constrictions that lead to migraine headaches; others say it might be due to autoimmune conditions, viral infections, allergic reactions, or head trauma. Ménière’s appears to have a hereditary component, so there may be a gene mutation connected to the regulation of endolymph fluid. Further research is critical.
Sources: David S. Haynes, M.D., FACS, Member, HHF's Ménière’s Disease Advisory Board; National Institute on Deafness and Other Communication Disorders (NIDCD)
Dizziness as a result of an attack may cause unsteadiness and feelings like the world is moving or spinning.
Nausea and vomiting occur when one experiences severe dizziness.
Hearing loss is typically unilateral (in one ear) and fluctuating. Patients often also report sensitivity to sound.
Tinnitus or ringing/buzzing in the ears may result in the absence of sound.
Diarrhea is possible during attacks, making it important to stay hydrated.
Migraines are common during flare-ups.
Uncontrolled eye movements can result because the inner ear affects balance and, in turn, eye movement. This may be a jerky eye movement in one or both eyes, side to side, up and down, or in a circular motion.
Cold sweats can result from vertigo.
Fatigue is common and may increase one’s risk of falling prey to an attack.
Extreme mood changes, including strong feelings of anxiety, fear, and anger are common. It’s unknown if anxiety contributes to and causes episodes or if anxiety is a by-product of the disease, occurring after attacks.
Content is adapted from repurposed with permission of the author and originally appeared on activebeat.com.
“I received my diagnosis at 21, but did not get hearing aids until last year. Going through my 20s I worried about vertigo and not being able to hear conversations when socializing. I eventually stopped going out with friends, and they stopped inviting me. I became depressed, lonely, and developed anxiety for fear of two unknowns: not knowing when my vertigo would strike again, and wondering how I’d continue to work to support my family.”
- Heather (left) with her children and husband
AVAILABLE TREATMENT OPTIONS
A cure for Ménière's disease does not yet exist, but lifestyle modifications and treatments have been found to help patients. Symptoms are sometimes lessened in patients by reducing the body’s retention of fluids through dietary changes (e.g. eliminating or reducing salt, caffeine and/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 some patients.
Medical therapy is directed at treating the underlying disorder and controlling the symptoms. The primary method of treating the underlying hydrops is to implement a low-sodium diet and diuretics to reduce fluid retention and, as a result, inner ear fluid pressure. A variety of vestibular suppressants, ranging from antihistamines to benzodiazepines, are utilized to control patients’ acute symptoms. Some patients are well controlled on medical therapy and require no surgical intervention.
With the intervention intratympanic steroids, a steroid solution is placed directly into the middle ear, allowing for passive perfusion into the inner ear via the round window. Intratympanic steroids are often offered to patients with episodic vertigo, sensorineural hearing loss, and other classic symptoms of Ménière’s disease that do not respond well to medical management. Many patients often prefer intratympanic steroids on a frequent basis as opposed to taking daily diuretics and/or sedating vestibular suppressed intermittently. Intratympanic steroids are associated with fewer side effects than systemic steroids.
The Meniett Device was approved by the FDA in 1999 and requires a tympanostomy tube to be placed in the ear drum. A self-administered pressure device, the Meniett is inserted into the ear canal and applies intermittent, alternating air pressure pulses to the middle ear in order to reduce fluid pressure causing dizziness. While having early promise as a conservative treatment option, it is now used less and less frequently due to other treatment modalities.
Endolymphatic sac surgery is done in the operating room under general anesthesia and is classified as a conservative procedure because it retains the function of the inner ear. By decompressing the endolymphatic sac, there is a chance to decompress the functional component of the inner ear, reducing symptoms. The success rate is around 80% according to “Endolymphatic Sac Surgery for Ménière's Disease – Current Opinion and Literature Review,” published in International Archives of Otorhinolaryngology in 2017.
Another option is intratympanic gentamicin. Gentamicin is an antibiotic with a known side effect of hearing loss and a capacity to destroy the vestibular system. It is delivered specifically to the middle ear with the intent to reduce the function of the vestibular system in that ear. It is titrated so hearing can be preserved while vestibular function is reduced or eliminated.
A vestibular nerve section requires surgically opening the skull. The balance portion of the 8th (auditory-vestibular) nerve is cut, sparing the hearing portion. This procedure is performed less due to the advent of transtympanic steroids and gentamicin therapy. Terri L. Rocco, whose story was highlighted in the Spring 2017 issue of Hearing Health magazine, underwent this surgery. Read more about Terri.
Labyrinthectomy is the surgical removal of the inner ear and is definitive in eliminating vestibular (inner ear) function. It remains a standard for treating Ménière’s disease patients with poor hearing but is generally only used after other treatment options have failed.
Sources: David S. Haynes, M.D., FACS, Member, HHF's Ménière’s Disease Advisory Board
The information on this page is not a substitute for professional medical advice. Hearing Health Foundation advises individuals seeking a Ménière’s disease diagnosis or treatment to see a doctor.