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Mnire disease

Dpt. Aamir Memon


Mnire disease

(Idiopathic endolymphatic hydrops)

Mnire disease is a chronic disorder of the semicircular canals and labyrinths of the inner ear. It is named after Dr. Prosper Mnire, who first reported the syndrome in a young girl in 1861. This disease is associated with severe attacks of vertigo (sense of spinning or disequilibrium), often accompanied by nausea. The cause of Mnire disease is unknown, but it appears related to an overproduction of endolymph in the inner ear. Elevation in antidiuretic hormone may be involved in some cases. Occurrences of Mnire disease may follow middle-ear infection or head trauma or may be associated with systemic illness such as thyroid disease. The condition may also show a genetic predisposition. Typically, the disorder is unilateral (only one ear is affected).

Physiology: The inner ear is composed of several structures, including the bony labyrinth, which forms the semicircular ducts leading to the ampulla, the vestibule, and the snailshaped cochlea. The bony labyrinth is filled with a sodium-rich fluid called perilymph. Inside the bony labyrinth, the membranous labyrinth floats in the perilymph. The membranous labyrinth is filled with a potassium-rich fluid called. Together the endolymph and perilymph, separated by the membranous labyrinth, help to conduct sound vibrations. Furthermore, nerve endings from the vestibulocochlear nerve terminate in the ampulla, an enlarged space where the semicircular canals converge. These nerve projections are suspended in endolymph and move like seaweed in water whenever the head changes position. Signals from the vestibulocochlear nerve coordinate with the eyes and general proprioceptors throughout the body to help orient us in space.


Most people diagnosed with Mnire disease are in their 20s to 50s, although it has been seen in children and older people. It is estimated that about 625,000 people in the United States have been diagnosed with Mnire disease, and about 45,000 new diagnoses are made each year. Men and women are affected about equally, and no specific racial or genetic predilection has been identified.


The exact causes or sequence of events that lead to Mnire disease are not well understood. Several theories have been developed and are being intensively researched, but this disease is still largely a mystery. Most researchers agree, however, that it has to do with the accumulation of excess fluid in the endolymph inside the membranous labyrinth. When no other cause can be found for this process, idiopathic endolymphatic hydrops, a synonym for Mnire disease, is identified. Possible causes for the accumulation of excess endolymph include: o Rupture of the membranous labyrinth that allows the perilymph and endolymph to mix, o Autoimmune activity o Viral infection o Pressure from a tiny blood vessel wrapping around the vestibulocochlear nerve.

Signs and Symptoms: Mnire disease has four classic symptoms, all of which appear intermittently and in any combination. It usually affects only one ear, but it can progress to affect the other ear as well. Onset of an episode is typically fast and unpredictable, and Any given Mnire attack can last 20 minutes to 24 hours. Hearing loss o Typically involves low-frequency sound. o It is worst during flares but eventually becomes permanent. o A person with a long history of this disorder may eventually become totally deaf. Tinnitus. o This is an umbrella term for any unexplained ringing, whistling, or pounding noise in the ear. o It can feel loud enough to interfere with the ability to sleep or concentrate. o People describe tinnitus as sounding like a million crickets or like the whine of a jet engine. A sense of fullness in the middle ear. o Many people with Mnire disease report that during flares they feel their ear is full, similar to the sensation of ascending or descending quickly, or coming in for a landing on an airplane. o This is not relieved by yawning, as normal ear pressure is. o Physical examinations of people with Mnire disease dont show a measurable increase in middle ear pressure, even during acute episodes. Rotational vertigo. o This may be the most disabling symptom of Mnire disease. o During an episode the person perceives that the world is spinning or the floor is sloping. o Nystagmus (an abnormal rhythmic oscillation of the eyes) is observed as well. Nausea and vomiting are common results. o Unlike the short-term vertigo that anyone gets when they spin or go on a Tilt-A-Whirl ride, this version lasts for several minutes or hours and is aggravated by any movement of the head. Nausea, vomiting, hypotension, and sweating often occur with attacks.

Diagnosis: Mnire disease is diagnosed when no other cause for the dysfunction (like MS, a neuroma on the acoustic nerve) can be found; when a person has at least two spontaneous episodes of vertigo and a feeling of fullness that last longer than 20 minutes; and when that person also has documented hearing loss. Tests of vestibular function, including balance testing, and tests of nystagmus eye movements may help confirm the diagnosis.

Treatment: Because Mnire disease is an idiopathic condition, treatment options focus on symptomatic control rather than trying to correct an identified problem. o Symptoms may decrease if the patient lies down or sits still, making no sudden movements. Many people are counselled to avoid aggravating foods and habits that raise blood pressure and increase fluid retention; ultimately increasing the risk of attack. Early recommendations Avoid monosodium glutamate, limitcaffeine and alcohol, and quit smoking Medications to manage vertigo and nausea may be prescribed. Treatments to reduce fluid volume, including diuretics and a low-salt diet, are suggested. Antihistamines and steroid hormones have been used with varying degrees of success. Surgical placement of a shunt to drain excess endolymph may be performed. Prescription of vestibulotoxic drugs, including systemic administration of streptomycin or intratympanic delivery of streptomycin and gentamicin, is used for severe cases. Surgical placement of a shunt to drain excess endolymph may be performed. . Complications: Mnire disease may progress to unilateral nerve deafness.