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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2018; 76(03): 170-176
DOI: 10.1590/0004-282X20180008
Article

The importance of central auditory evaluation in Friedreich's ataxia

A importância da avaliação auditiva central na ataxia de Friedreich
Bianca Simone Zeigelboim
1   Univesidade Tuiuti do Paraná, Departament of Otoneurology, Curitiba – Paraná State, Brazil
,
Hélio A. G. Teive
2   Universidade Federal do Paraná, Hospital de Clínicas, Neurology Service, Curitiba – Paraná State, Brazil
,
Michèlli Rodrigues da Rosa
1   Univesidade Tuiuti do Paraná, Departament of Otoneurology, Curitiba – Paraná State, Brazil
,
Jéssica Spricigo Malisky
1   Univesidade Tuiuti do Paraná, Departament of Otoneurology, Curitiba – Paraná State, Brazil
,
Vinicius Ribas Fonseca
1   Univesidade Tuiuti do Paraná, Departament of Otoneurology, Curitiba – Paraná State, Brazil
,
Jair Mendes Marques
1   Univesidade Tuiuti do Paraná, Departament of Otoneurology, Curitiba – Paraná State, Brazil
,
Paulo Breno Liberalesso
3   Hospital Pequeno Príncipe, Departament of Neurology, Curitiba – Paraná State, Brasil
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ABSTRACT

Objective To assess central auditory function in Friedreich's ataxia.

Methods A cross-sectional, retrospective study was carried out. Thirty patients underwent the anamnesis, otorhinolaryngology examination, pure tone audiometry, acoustic immittance measures and brainstem auditory evoked potential (BAEP) assessments.

Results The observed alterations were: 43.3% in the pure tone audiometry, bilateral in 36.7%; 56.6% in the BAEP test, bilateral in 50%; and 46.6% in the acoustic immittance test. There was a significant difference (p < 0.05) in the comparison between the tests performed.

Conclusion In the audiological screening, there was a prevalence of the descending audiometric configuration at the frequency of 4kHz, and absence of the acoustic reflex at the same frequency. In the BAEP test, there was a prevalence of an increase of the latencies in waves I, III and V, and in the intervals of interpeaks I-III, I-V and III-V. In 13.3% of the patients, wave V was absent, and all waves were absent in 3.3% of patients.

RESUMO

Objetivo Avaliar a função auditiva central na ataxia de Friedreich (AFRD).

Métodos Foi realizado um estudo retrospectivo de corte transversal. 30 pacientes realizaram anamnese, avaliações otorrinolaringológica, audiológica, imitanciométrica e do potencial evocado auditivo de tronco encefálico (PEATE).

Resultados As alterações observadas foram: 43,3% no exame audiométrico sendo 36,7% dos casos, bilateralmente; 56,6% na avaliação do PEATE com 50% dos casos, bilateralmente e 46,6% no exame imitanciométrico. Houve diferença significativa (p < 0,05) na comparação entre os exames realizados.

Conclusão No exame audiológico, ocorreu uma preponderância maior da configuração audiométrica descendente a partir da freqüência de 4kHz e ausência do reflexo acústico na mesma frequência. No exame do PEATE, houve prevalência do aumento das latências nas ondas I, III e V, e nos intervalos dos interpicos I-III, I-V e III-V. Em 13,3% dos casos, a onda V estava ausente, e em 3,3% dos casos, todas as ondas estavam ausentes.

Keywords

spinocerebellar degenerations - hearing disorders - Friedreich ataxia - evoked potentials, auditory - ataxic gait

Palavras-chave

degenerações espinocerebelares - transtornos da audição - ataxia de Friedreich - potenciais evocados auditivos - marcha atáxica


Publication History

Received: 19 September 2017

Accepted: 14 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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