It is important for otolaryngologists to recognize Chiari malformations as part of the differential diagnosis of balance disorders because patients may initially be seen with symptoms referable to the vestibular system, including ataxia, nystagmus, or vertigo. The objective of this paper is to review the signs, symptoms, and vestibular test findings of a series of patients with Chiari 1 malformation. Six patients were identified by retrospective chart review with a diagnosis of Chiari malformation. Each patient had a complete otoneurologic examination and vestibular function testing. The results indicated that patients fell into two different vestibular test result profiles. First, patients with advanced symptoms demonstrated oculomotor dysfunction, central vestibular nystagmus, abnormal vestibular visual interaction, and abnormal tilt suppression of postrotatory nystagmus. On the other hand, a number of patients were identified with incidentally noted Chiari malformation on magnetic resonance imaging scan who had a vestibular test profile consistent with peripheral vestibulopathy without signs and symptoms of central nervous system dysfunction. Guidelines are provided to help determine the extent of the group of symptoms attributable to an incidentally discovered Chiari malformation.