title: Otoneurological Abnormalities in Patients with Friedreich's Ataxia.
abstract: IntroductionFriedreich's ataxia is a neurodegenerative disease and progressive by nature. It has autosomal recessive inheritance and early onset in most cases. Nystagmus and hearing loss (in some cases) make up some of the common symptoms seen in this disorder.ObjectiveThe objective of this study is to examine vestibular disorders in patients with Friedreich ataxia.MethodsWe 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.ResultsClinically, 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).ConclusionThe 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.
authors.affiliation: Department of Communication Disorders, Universidade Tuiuti do Paraná, Curitiba, Brazil.
authors.forename: Bianca Simone
authors.initials: BS
authors.lastname: Zeigelboim
authors.affiliation: Department of Otorhinolaringology, Hospital da Cruz Vermelha, Curitiba, Brazil.
authors.forename: Juliana Cristina
authors.initials: JC
authors.lastname: Mesti
authors.affiliation: Department of Otorhinolaringology, Hospital da Cruz Vermelha, Curitiba, Brazil.
authors.forename: Vinicius Ribas
authors.initials: VR
authors.lastname: Fonseca
authors.affiliation: Department of Communication Disorders, Universidade Tuiuti do Paraná, Curitiba, Brazil.
authors.forename: João Henrique
authors.initials: JH
authors.lastname: Faryniuk
authors.affiliation: Department of Communication Disorders, Universidade Tuiuti do Paraná, Curitiba, Brazil.
authors.forename: Jair Mendes
authors.initials: JM
authors.lastname: Marques
authors.affiliation: Department of Communication Disorders, Universidade Tuiuti do Paraná, Curitiba, Brazil.
authors.forename: Rafaella Cardosa
authors.initials: RC
authors.lastname: Cardoso
authors.affiliation: Department of Internal Medicine, Hospital das Clínicas, Curitiba, Brazil.
authors.forename: Hélio Afonso Ghizoni
authors.initials: HA
authors.lastname: Teive
keywords: ataxia; spinocerebellar ataxias; spinocerebellar degenerations