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Revista CEFAC

Print version ISSN 1516-1846On-line version ISSN 1982-0216

Abstract

ZEIGELBOIM, Bianca Simone et al. Spinocerebellar ataxia type 6: a case report. Rev. CEFAC [online]. 2014, vol.16, n.5, pp.1650-1654. ISSN 1982-0216.  https://doi.org/10.1590/1982-0216201412513.

The aim of this study was to investigate the vestibulocochlear alterations observed in a case of spinocerebellar ataxia type 6. The case was referred from the Hospital das Clinicas to the Otoneurology Laboratory of an educational institution and was subjected to the following procedures: anamnesis, otologic examination, as well as audiological and vestibular assessments. The case shows a 57-year-old female patient with a genetic diagnosis of spinocerebellar ataxia type 6 who presented unsteadiness of gait with tendency to fall to the left, dysarthria, and dysphonia. The audiological assessment presented sloping audiometric configuration from 4.0 kHz and tympanogram type “A” with the presence of acoustic reflexes bilaterally. Observed during the survey of positional vertigo in the vestibular assessment were the presence of oblique and vertical downbeat nystagmus, spontaneous and semispontaneous with multiple core features (absence of latency, paroxysm, fatigue and vertigo), abolished optokineticnystagmus and hyporeflexia in the caloric test. We found labyrinthic alterations that indicate central vestibular system disorders and lend credence to the importance of this evaluation. The existence of a possible relationship between the findings and vestibular symptoms displayed by the patient indicated the relevance of the labyrinthine evaluation for this type of ataxia once the presence of vertical downbeat nystagmus proved to be frequent in this type of pathology.

Keywords : Spinocerebellar Degenerations; Ataxia; Vestibular Function Tests; Electronystagmography; Nystagmus; Optokinetic.

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