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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2018; 76(03): 131-138
DOI: 10.1590/0004-282X20180001
Article

Otoneurological findings prevalent in hereditary ataxias

Alterações otoneurológicas prevalentes nas ataxias hereditárias
Bianca Simone Zeigelboim
1   Universidade Tuiuti do Paraná, Departamento de Otoneurologia, Curitiba, PR, Brasil
,
Hélio A. G. Teive
2   Universidade Federal do Paraná, Hospital de Clínicas, Serviço de Neurologia, Curitiba, PR, Brasil
,
Geslaine Janaína Barbosa Santos
3   Instituto Federal de Educação, Ciência e Tecnologia do Paraná, Curitiba PR, Brasil
,
Maria Izabel Rodrigues Severiano
3   Instituto Federal de Educação, Ciência e Tecnologia do Paraná, Curitiba PR, Brasil
,
Vinicius Ribas Fonseca
1   Universidade Tuiuti do Paraná, Departamento de Otoneurologia, Curitiba, PR, Brasil
,
João Henrique Faryniuk
1   Universidade Tuiuti do Paraná, Departamento de Otoneurologia, Curitiba, PR, Brasil
,
Jair Mendes Marques
1   Universidade Tuiuti do Paraná, Departamento de Otoneurologia, Curitiba, PR, Brasil
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ABSTRACT

Objective To describe and compare the vestibular findings most evident among the hereditary ataxias, as well as correlate their clinical features with the nervous structures affected in this disease.

Methods Seventy-five patients were evaluated and underwent a case history, otorhinolaryngological and vestibular assessments.

Results Clinically, the patients commonly had symptoms of gait disturbances (67.1%), dizziness (47.3%), dysarthria (46%) and dysphagia (36.8%). In vestibular testing, alterations were predominantly evident in caloric testing (79%), testing for saccadic dysmetria (51%) and rotational chair testing (47%). The presence of alterations occurred in 87% of these patients. A majority of the alterations were from central vestibular dysfunction (69.3%).

Conclusion This underscores the importance of the contribution of topodiagnostic labyrinthine evaluations for neurodegenerative diseases as, in most cases, the initial symptoms are otoneurological; and these evaluations should also be included in the selection of procedures to be performed in clinical and therapeutic monitoring.

RESUMO

Objetivo Descrever e comparar os achados vestibulares mais evidentes entre a ataxia hereditária, bem como correlacionar seus aspectos clínicos com o estudo das estruturas nervosas afetadas nesta doença.

Métodos 75 pacientes foram avaliados e submetidos aos seguintes procedimentos: anamnese, avaliação otorrinolaringológica e vestibular.

Resultados Clinicamente, os pacientes apresentaram sintomas de distúrbios da marcha (67,1%), tonturas (47,3%), disartria (46%) e disfagia (36,8%). No teste vestibular, as alterações foram predominantemente evidentes no teste calórico (79%), dismetria sacádicas (51%) e no teste rotatório (47%). A presença de alterações ocorreu em 87% dos pacientes. A maioria das alterações observadas foram da disfunção vestibular central (69,3%).

Conclusão O estudo ressalta a importância da contribuição da avaliação labiríntica no topodiagnóstico para doenças neurodegenerativas, uma vez que, na maioria dos casos, os sintomas iniciais são otoneurológicos, e essas avaliações também devem ser incluídas na seleção de procedimentos a serem realizados no monitoramento clínico e terapêutico.

Keywords

spinocerebellar ataxias - vestibular diseases - chronic disease

Palavras-chave

ataxias espinocerebelares - doenças vestibulares - doença crçnica


Publication History

Received: 27 July 2017

Accepted: 06 December 2017

Article published online:
28 August 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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