General
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• Also called endolymphatic hydrops
• Idiopathic disorder of inner ear associated with episodic attacks of vertigo, fluctuating sensorineural hearing loss, tinnitus and sensation of aural fullness
• Incidence varies from 7.5 per 100K in France to 157 per 100K in England
• 60% women, peaks in 40’s to 60’s, but wide age range
• Rarely occurs in children (J Laryngol Otol 2006;120:343)
• Associated with HLA B8/DR3
• May be due to accumulation of endolymph in membranous labyrinth, perhaps due to inadequate absorption by endolymphatic sac

Treatment
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• Dietary modification, intermittent dehydration, diuretics, vasodilators in increase microcirculation of ear
• 60-80% improve
• Surgery includes shunting and decompression of endolymphatic sac (Laryngoscope 2005;115:1454), labyrinthectomy, sectioning of vestibular nerve

Micro description
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• Initially involves cochlear duct and saccule
• Later entire endolymphatic system with dilation, rupture and collapse of membranous labyrinth with possible fistula
• May have severe atrophic changes with loss of cochlear neurons

Micro images
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Section through cochlea

Additional references
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• National Institutes of Health
• Wikipedia
• eMedicine

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