Disorders of the inner-ear balance organs and their pathways

Allison S Young, Sally M Rosengren, Miriam S Welgampola
Handbook of Clinical Neurology 2018, 159: 385-401
Disorders of the inner-ear balance organs can be grouped by their manner of presentation into acute, episodic, or chronic vestibular syndromes. A sudden unilateral vestibular injury produces severe vertigo, nausea, and imbalance lasting days, known as the acute vestibular syndrome (AVS). A bedside head impulse and oculomotor examination helps separate vestibular neuritis, the more common and innocuous cause of AVS, from stroke. Benign positional vertigo, a common cause of episodic positional vertigo, occurs when otoconia overlying the otolith membrane falls into the semicircular canals, producing brief spells of spinning vertigo triggered by head movement. Benign positional vertigo is diagnosed by a positional test, which triggers paroxysmal positional nystagmus in the plane of the affected semicircular canal. Episodic spontaneous vertigo caused by vestibular migraine and Ménière's disease can sometimes prove hard to separate. Typically, Ménière's disease is associated with spinning vertigo lasting hours, aural fullness, tinnitus, and fluctuating hearing loss while VM can produce spinning, rocking, or tilting sensations and light-headedness lasting minutes to days, sometimes but not always associated with migraine headaches or photophobia. Injury to both vestibular end-organs results in ataxia and oscillopsia rather than vertigo. Head impulse testing, dynamic visual acuity, and matted Romberg tests are abnormal while conventional neurologic assessments are normal. A defect in the bony roof overlying the superior semicircular canal produces vertigo and oscillopsia provoked by loud sound and pressure (when coughing or sneezing). Three-dimensional temporal bone computed tomography scan and vestibular evoked myogenic potential testing help confirm the diagnosis of superior canal dehiscence. Collectively, these clinical syndromes account for a large proportion of dizzy and unbalanced patients.

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