In Meniere disease, pressure and volume changes in the labyrinthine endolymph affect inner ear function. The etiology of endolymphatic fluid buildup is unknown. Risk factors include a family history of Meniere disease, preexisting autoimmune disorders, allergies, trauma to the head or ear, and, very rarely, syphilis. Peak incidence is between ages 20 and 50.
Meniere syndrome refers to the triad of vertigo, tinnitus, and hearing loss not caused by endolymphatic fluid buildup (eg, congenital anomalies).
Symptoms and Signs of Meniere Disease
Patients with Meniere disease have sudden attacks of vertigo that usually last for 20 minutes to 12 hours; rarely, the attacks last up to 24 hours. Usually, nausea and vomiting are also present Other symptoms include diaphoresis, diarrhea, and gait unsteadiness.
Tinnitus in the affected ear may be constant or intermittent buzzing, ringing, roaring, whistling, or hissing; it is not related to position or motion.
Hearing impairment, typically affecting low frequencies, may follow the attacks. Before and during an episode, most patients sense fullness or pressure and hyperacusis (sensitivity to loud sounds) in the affected ear. In most patients, only one ear is affected.
During the early stages, symptoms remit between episodes; symptom-free periods may last > 1 year. As the disease progresses, however, hearing impairment fluctuates but eventually persists and gradually worsens, and tinnitus may be constant, even between attacks.
Diagnosis of Meniere Disease
Clinical evaluation
Audiogram and gadolinium-enhanced MRI to rule out other causes
The diagnosis of Meniere disease is made clinically. The simultaneous combination of fluctuating low-frequency sensorineural hearing loss, episodic vertigo, ipsilateral fluctuating aural fullness, and tinnitus is characteristic. Similar symptoms can result from vestibular migraine, vestibular neuronitis, viral labyrinthitis, a cerebellopontine angle tumor (eg, vestibular schwannoma), or a brain stem stroke. Although bilateral Meniere disease can occur, bilateral symptoms increase the likelihood of an alternate diagnosis (eg, vestibular migraine). Vestibular migraine (also known as migrainous vertigo) is characterized by episodes of vertigo in patients who have a history of migraines or with other features of migraines, such as headache, photophobia and phonophobia, or visual aura; there is no loss of hearing.
Between attacks, the examination may be entirely normal. But during an acute attack, the patient has nystagmus and falls to the affected side. Also, in long-standing or refractory cases with associated labyrinthine hypofunction, the Fukuda stepping test (marching in place with eyes closed, previously known as the Unterberger test) causes the patient to turn toward the affected ear, consistent with a unilateral labyrinthine lesion.
The Halmagyi head thrust maneuver, or head impulse test, is another technique that is used to check for unilateral labyrinthine dysfunction. In the Halmagyi maneuver, the examiner has the patient visually fixate on a target straight ahead (eg, the examiner's nose). Then while observing the patient's eyes, the examiner rapidly rotates the patient's head 15 to 30° to one sides.
When the head is rotated to one side, vestibular function on that side is normal if the patient's eyes remain fixated on the target. When vestibular function is impaired, the vestibulo-ocular reflex is absent and the patient's eyes do not remain fixated on the target but instead transiently follow the head rotation and then quickly and voluntarily return back to the target (called delayed catch-up saccades).
Patients with symptoms suggesting Meniere disease should have an audiogram and an MRI (with gadolinium enhancement) of the central nervous system with attention to the internal auditory canals to exclude other causes. Audiogram typically shows a low-frequency sensorineural hearing loss in the affected ear that fluctuates between tests. The Rinne test and the Weber test may indicate sensorineural hearing loss.
Treatment of Meniere Disease
Symptom relief with antiemetics, antihistamines, or benzodiazepines
Diuretics and low-salt diet
Rarely vestibular ablation by medications or surgery
Meniere disease tends to be self-limited. Treatment of an acute attack is aimed at symptom relief and done in a staged fashion; the least invasive measures are done first, and then if the measures are ineffective, ablative procedures are sometimes done.
A low-salt (<
Although more invasive, endolymphatic sac decompression relieves vertigo in most patients, spares vestibular function, and poses minimal risk of hearing loss. Thus this procedure is still classified as a vestibular-sparing treatment.
Ablative surgery is reserved for patients with frequent, severely debilitating episodes who are unresponsive to less invasive modalities. Vestibular neurectomy (an intracranial procedure) relieves vertigo in about 95% of patients and usually preserves hearing. A surgical labyrinthectomy is done only if preexisting hearing loss is profound.
Unfortunately, there is no known way to prevent the natural progression of hearing loss. Most patients sustain moderate to severe sensorineural hearing loss in the affected ear within 10 to 15 years.
Key Points
Meniere disease typically causes vertigo with nausea and vomiting, unilateral tinnitus, and chronic, progressive hearing loss.
Testing is with audiogram, and MRI is done to rule out other disorders.
Antiemetics and antihistamines can help relieve symptoms; some clinicians also use oral or transtympanic corticosteroids or migraine preventive medications (eg, tricyclic antidepressants or SNRIs).
