Objective: To identify vestibular alterations in patients with sporadic ataxia (SA). Materials and methods: A retrospective, cross-sectional study was carried out. Eleven patients, four females and seven males, 35–58 years of age (mean age, 46.6 ± 8 years) were evaluated, and they underwent the following procedures: anamnesis, otorhinolaryngological assessment to exclude any kind of middle ear impairments that may hinder exam results, and vestibular evaluation to verify the existence of vestibular disorders, kind and site of the lesion. Significance criterion by comparing the result of the vestibular screening (analysing normal and altered results) was p < .05. Results: Clinically, patients evidenced greater occurrence of gait imbalance (81.8%), dysarthria (63.8%) and dysphagia (54.5%). Dizziness was reported in 45.4% of the cases. In the vestibular testing, prevalence of alterations occurred in the caloric test (54.5%), in the rotation test (45.5%), and in the search for gaze and optokinetic nystagmus (27.3%, each). The presence of testing alteration was observed in 73% of the patients, central vestibular disorder was the greatest occurrence in 64% of the exams. Conclusions: Vestibular function disorder occurred in 73% of the patients, detected in the caloric test, central vestibular system disorder, deficit kind, was prevalent. The high number of alterations, diagnosed in the test, point to the relevance of the labyrinth screening in this rare kind of ataxia, and evidence the importance of patients’ early diagnosis and follow-up.