Less Common Neuro-otologic Disorders

Abstract

A systematic approach to the history and examination allows the physician to diagnose the most common vestibular disorders of the brain or inner ear. However, some less common disorders require a specific familiarity so that they are not misdiagnosed as one of the more common disorders,treated inappropriately, or misattributed to a psychogenic etiology. This article describes four of the less common disorders that can present with a primary problem of dizziness and imbalance: (1) mal de débarquement syndrome, (2) bilateral vestibulopathy, (3) cerebellar ataxia, and (4) vestibular schwannomas (ie, acoustic neuromas). Associated clinical features of mal de débarquement syndrome have recently been investigated to clarify the spectrum of the syndrome. The combination of cerebellar ataxia, neuropathy, and vestibular areflexia (bilateral vestibulopathy) has been summated into a new syndrome. Further refinement of ocular motor features of cerebellar ataxia can narrow genetic testing requirements. Vestibular schwannomas remain an uncommon etiology for isolated dizziness; recent imaging studies have helped quantify the low yield of screening MRI protocols for the evaluation of undefined dizziness. A working knowledge of these less common disorders will help the physician make the diagnosis efficiently by gathering key elements of the history and fine-tuning diagnostic testing.