Ménière's disease
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idiopathic endolymphatic hydrops (too much endolymph)
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incidence rate: 0.5-2 cases/1000 people - onset usually in 40's and 50's
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diagnosed by excluding other causes
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variations in the nature of the symptoms is the rule rather than the exception
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symptoms include (survivor's story):
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intermittent spells (including periods of remission) of severe vertigo and nystagmus accompanied by nausea, vomiting, sweating and all the symptoms normally associated with extreme motion sickness
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fluctuating hearing impairment and tinnitus - may be a low frequency or mixed low and high frequency ("Pike's Peak") pattern. A documented fluctuating hearing loss, especially in the low frequencies, is very helpful in making the diagnosis.
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a sensation of pressure or fullness in the involved ear that cannot be relieved by swallowing
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rapid onset in attacks, with the severe vertigo usually lasting for hours, followed by auditory symptoms and unsteadiness that can last for several days
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after the initial attacks, the hearing usually returns to normal but, ultimately, there is a progressive permanent hearing loss - in 50% of patients, disease becomes bilateral
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occasionally, the patient will present with only the vertigo or only the auditory symptoms
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thought to be due to distention of the membranous labyrinth (endolymphatic hydrops)
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full blown attacks of Ménières are probably due to a break in the membrane separating perilymph and endolymph - potassium rich endolymph then bathes the vestibular nerve, leading to a depolarization block and transient loss of function
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theories of causes (remember, Ménière's is idiopathic!) include electrolyte imbalance, autoimmune disease, allergies, vasomotor reactions, trauma, metabolic disorders, infection (eg, viral, syphilitic), or hereditary factors
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attacks typically begin when patients are in their 40s to 50s - extremely rare in children
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therapy includes:
- short term: antiemetics and sedatives
- long term: diuretics, with concurrent restriction of dietary sodium, caffeine, and alcohol
- vasodilators, steroids, and other anti-inflammatory drugs may be helpful
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surgical procedures for patients who fail medical management include:
- endolymphatic sac procedures
- use of ototoxic antibiotics to ablate the affected vestibular system
- labyrinthectomy
- vestibular nerve section