alexa Abstract | Vestibular findings in autosomal recessive ataxia

The International Tinnitus Journal
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OBJECTIVE: This study aims to examine vestibular disorders in patients with recessive spinocerebellar ataxia.

DESIGN: A retrospective cross-sectional study was conducted. The patients underwent the following procedures: case history, ENT and vestibular evaluations.

STUDY SAMPLE: The tests were performed in 19 patients ranging from 6 to 63 years of age (mean age of 36.7).

RESULTS: Clinically, the patients commonly had symptoms of dizziness (57.8%), lack of coordination of movement with imbalance when walking (52.6%), and headaches (42.1%). In vestibular testing, alterations were predominantly evident under caloric testing (73.5%), rotational chair testing, and testing for gaze and optokinetic nystagmus (36.8%). The presence of alterations occurred under examination in 89.5% of these patients, 100% occurred in subjects with Friedreich's ataxia and 84.6% for subjects with indeterminate recessive spinocerebellar ataxia, with the majority occurring in those with central vestibular dysfunction, 57.9% of the examinations.

CONCLUSION: The most evident neurotological symptoms were dizziness, lack of coordination of movement, and imbalance when walking. Alterations in vestibular examinations occurred in 89.5% of patients, mostly in the caloric test, with a predominance of deficient central vestibular system dysfunction. This underscores the importance of the contribution of topodiagnostic labyrinthine evaluations for neurodegenerative diseases.

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Author(s): Bianca Simone Zeigelboim Helio Afonso Ghizoni Teive Hugo Amilton Santos de Carvalho Edna Marcia da Silva Abdulmassih Jair Mendes Marques and Rafaella Cristyne Cardoso


ataxia, spinocerebellar ataxias, spinocerebellar degenerations, vertigo, vestibular function tests

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