Ménière’s Disease: A Diagnosis of Exclusion with Controversial Therapies

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.