Rare Disorders of the Vestibular Labyrinth: of Zebras, Chameleons and Wolves in Sheepʼs Clothing

The differential diagnosis of vertigo syndromes is a challenging issue, as many – and in particular – rare disorders of the vestibular labyrinth can hide behind the very common symptoms of “vertigo” and “dizziness”. The following article presents an overview of those rare disorders of the balance organ that are of special interest for the otorhinolaryngologist dealing with vertigo disorders. For a better orientation, these disorders are categorized as acute (AVS), episodic (EVS) and chronic vestibular syndromes (CVS) according to their clinical presentation. The main focus lies on EVS sorted by their duration and the presence/absence of triggering factors (seconds, no triggers: vestibular paroxysmia, Tumarkin attacks; seconds, sound and pressure induced: “third window” syndromes; seconds to minutes, positional: rare variants and differential diagnoses of benign paroxysmal positional vertigo; hours to days, spontaneous: intralabyrinthine schwannomas, endolymphatic sac tumors, autoimmune disorders of the inner ear). Furthermore, rare causes of AVS (inferior vestibular neuritis, otolith organ specific dysfunction, vascular labyrinthine disorders, acute bilateral vestibulopathy) and CVS (chronic bilateral vestibulopathy) are covered. In each case, special emphasis is laid on the decisive diagnostic test for the identification of the rare disease and “red flags” for potentially dangerous disorders (e. g. labyrinthine infarction/hemorrhage). Thus, this chapter may serve as a clinical companion for the otorhinolaryngologist aiding in the efficient diagnosis and treatment of rare disorders of the vestibular labyrinth.