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International Archives of Otorhinolaryngology

Print version ISSN 1809-9777On-line version ISSN 1809-4864

Abstract

ZEIGELBOIM, Bianca Simone et al. Otoneurological Abnormalities in Patients with Friedreich's Ataxia. Int. Arch. Otorhinolaryngol. [online]. 2017, vol.21, n.1, pp.79-85. ISSN 1809-4864.  https://doi.org/10.1055/s-0036-1572529.

Introduction

Friedreich's ataxia is a neurodegenerative disease and progressive by nature. It has autosomal recessive inheritance and early onset inmost cases. Nystagmus and hearing loss (in some cases) make up some of the common symptoms seen in this disorder.

Objective

The objective of this study is to examine vestibular disorders in patientswith Friedreich ataxia.

Methods

We conducted a retrospective cross-sectional study. We evaluated 30 patients with ages ranging from six to 72 years (mean age of 38.6 ( 14.7). The patients underwent the following procedures: anamnesis, ENT, and vestibular evaluations.

Results

Clinically, the patients commonly had symptoms of incoordination of movement (66.7%), gait disturbances (56.7%), and dizziness (50%). In vestibular testing, alterations were predominantly evident under caloric testing (73.4%), gaze nystagmus testing (50.1%), rotational chair testing (36.7%), and optokinetic nystagmus testing (33.4%). The presence of alterations occurred under examination in 90% of subjects, with the majority occurring in those with central vestibular dysfunction (70% of the examinations).

Conclusion

The most evident neurotological symptoms were incoordination of movement, gait disturbances, and dizziness. Alterations in vestibular examinations occurred in 90% of patients, mostly in the caloric test, with a predominance of deficient central vestibular system dysfunction.

Keywords : spinocerebellar ataxias; ataxia; spinocerebellar degenerations.

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