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Ménière’s Disease and Other Causes of Episodic Vertigo 

Chapter:
Ménière’s Disease and Other Causes of Episodic Vertigo
Author(s):

Yuri Agrawal

and Lloyd B. Minor

DOI:
10.1093/med/9780199608997.003.0022

Ménière’s syndrome is an inner ear disorder characterized by spontaneous attacks of vertigo, fluctuating low-frequency sensorineural hearing loss, aural fullness and tinnitus. When the syndrome is idiopathic and cannot be attributed to any other cause (e.g. syphilis, immune-mediated inner ear disease, surgical trauma), it is referred to as Ménière’s disease.(1) Ménière’s syndrome exhibits a relapsing-remitting pattern, with episodic attacks terminated by periods of restitution to normal auditory and vestibular function. Additionally, the natural history of Ménière’s syndrome is such that auditory and vestibular function typically decline over time. (2) Prosper Ménière first described this constellation of symptoms in 1861, and given the co-occurrence of auditory and vestibular phenomena he proposed that the pathologic locus was the labyrinth.(3) Subsequent investigations have corroborated his hypothesis: post-mortem temporal bone analyses of individuals with Ménière’s syndrome demonstrated histopathologic abnormalities in the labyrinth. Additionally, physiologic tests of labyrinthine function were also found to be abnormal in these patients. In this chapter we will review the clinical and pathophysiological features of Ménière’s disease that distinguish it from other disease processes in the differential diagnosis of vertigo and imbalance. We will begin by describing some key clinical characteristics of Ménière’s disease. We will then outline the central pathologic hypothesis behind Ménière’s disease – endolymphatic hydrops – insofar as this informs our understanding of physiologic and radiographic tests as well as management strategies in Ménière’s disease. We will explore the physiologic effects of Ménière’s disease on vestibular function, as measured by caloric, head impulse and vestibular-evoked myogenic potential (VEMP) testing. Finally, we will review management strategies for the treatment of Ménière’s disease.

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