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Classical Meniere Disease Attack – Symptoms – Prognosis – Treatment – Hearing Loss
Meniere Disease Symptoms
Periodic recurrent vertigo associated with decreased hearing, noise in the ear and sensation of fullness in the ear are the symptoms of Meniere disease. Typically, the attack starts with tinnitus, followed by sensation of fullness in the ear and then by an incomplete transient hearing loss. The vertigo comes within minutes, rapidly escalates in severity, and then gradually subsides over the course of half an hour to a few hours. For the first couple of years tinnitus and clogged ear sensation may occur independently of the vertigo.
The attacks are accompanied by nausea, vomiting, lack of balance and jerky eye movements (nystagmus). The fast phase of nystagmus is usually directed towards the diseased side in the beginning of the attack and switches to the opposite direction later on. Nystagmus lasts for a few hours. Head motion does make the symptoms worse but, unlike BPPV, in Meniere disease vertigo and nausea don’t go away completely during immobility. Mild dizziness and imbalance might persist for a few days following the attack.
Meniere disease has a close relationship with vestibular migraine and they may be inherited together. Moreover, some patients experience migraine aura and headache during Meniere disease attacks. Vestibular migraine does not cause loss of hearing but sensation clogged ears and tinnitus may occur. Even though these disorders are somewhat related, these are two distinct diagnostic entities.
Meniere Disease Natural Course
Meniere disease prevalence ranges from 20 to 200 per 100,000 equally affecting men and women.
Vertigo attacks eventually stop after about 10 to 15 years leaving an incomplete but permanent hearing loss and noise in the ear (tinnitus). Low frequency hearing loss develops first and later the whole spectrum gets affected.
Later in the disease course, some patients may experience sudden falls without vertigo. They feel as if they are violently pushed. There is no loss of consciousness. These attacks are very brief.
While Meniere disease initially affects only one ear, in about half of the cases it eventually spreads to the second one.
Cause of Meniere Disease
Vestibular (balance) canal systems and cochlear (hearing) systems are interconnected.
Meniere disease is caused by an increased pressure in the vestibulocochlear system; possibly due to lack of balance between production and absorption of the liquid filling the canal system (endolymph). High pressure initially irritates and eventually kills the sensor cells (hair cells). A potential mechanism periodic exacerbations (vertigo attacks) is an electrolyte imbalance within the vestibulocochlear system caused by loss of the barrier between endolymph and perilymph. Perilymph is rich with sodium while endolymph is rich with potassium. Transient increase of potassium level may be responsible for the attacks and possibly eventual demise of the hair cells essential for the sound recognition.
What mechanism is responsible for the endolymph production-resorption imbalance and/or some other cause of the syndrome is not known. It is possible that Meniere disease is caused by some autoimmune process. There is also some statistical association of Meniere disease and migraine.
Treatment of Meniere Disease
There are no treatment options available that are able to stop or delay the disease progression.
Symptoms of vertigo can be suppressed by “vestibular suppressants”, such as Meclizine or Valium on as needed basis.
In severe Meniere disease a local injection of chemicals (Gentamicin) will stop the vertogo attacks in most patients by destroying vestibular hair cells (sensor cells in the balance system). Killing vestibular hair cells translates into a complete loss of vestibular function on the same side.
Gentamicin is less toxic for the hearing hair cells; so no significant hearing loss due to this procedure is expected.
On the other hand, this treatment is not going to prevent tinnitus and ear fullness sensation during the attacks and beyond. It will not prevent the inevitable gradual hearing loss due to underlying disease process either.
Use of surgical procedures, low salt diet (in US) and Betahistine (in Europe) ale all lacking any proof of effectiveness in well controlled studies.