Introduction  Spinocerebellar ataxia (SCA) is part of a genetic and clinical heterogeneous group of neurodegenerative diseases characterized by progressive cerebellar ataxia. Objective  To describe the results of audiological and electrophysiological hearing evaluations in patients with sporadic ataxia (SA). Methods  A retrospective cross-sectional study was carried out with 11 patients submitted to the following procedures: anamnesis, otorhinolaryngological evaluation, tonal and vocal audiometry, acoustic immittance and brainstem auditory evoked potential (BAEP) tests. Results  The patients presented with a prevalence of gait imbalance, of dysarthria, and of dysphagia; in the audiometric and BAEPs, four patients presented with alterations; in the acoustic immittance test, five patients presented with alterations, predominantly bilateral. Conclusion  The most evident alterations in the audiological evaluation were the prevalence of the descending audiometric configuration between the frequencies of 2 and 4 kHz and the absence of the acoustic reflex between the frequencies of 3 and 4 kHz bilaterally. In the electrophysiological evaluation, the patients presented changes with a prevalence of increased I, III and V wave latencies and the interval in the interpeak I-III, I-V and III-V. In the present study, it was observed that auditory complaints did not have a significant prevalence in this type of ataxia, which does not occur in some types of autosomal recessive and dominant ataxia.

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.