Division of General Neurology and Ataxia Unit, Department of Neurology, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
Department of Neurology, Universidade de Campinas, Campinas, SP, Brazil.
Cerebellum. 2019 Dec;18(6):1011-1016. doi: 10.1007/s12311-019-01042-4.
Cerebellar ataxias are a clinically heterogeneous group of neurological disorders. Besides the cerebellum, several forms of hereditary ataxias or non-genetic ataxias also affect other areas of the brain. Some forms of cerebellar ataxias may have cochlear and vestibular involvement and may present with deafness and symptoms or signs of vestibulopathy (dizziness, nystagmus and diplopia). Recognizing otoneurological symptoms in patients with cerebellar ataxias is mandatory, since these signs may guide a specific diagnosis, and clinicians may provide a suitable therapeutic approach. In this review, we describe and discuss the most common forms of cerebellar ataxias associated with deafness and vestibulopathy.
小脑共济失调是一组临床异质性的神经退行性疾病。除了小脑外，几种遗传性共济失调或非遗传性共济失调也会影响大脑的其他区域。一些小脑共济失调可能伴有耳蜗和前庭受累，并可能出现耳聋和前庭病的症状或体征（头晕、眼球震颤和复视）。在小脑共济失调患者中识别出耳神经病学症状是强制性的，因为这些体征可能会指导特定的诊断，临床医生可能会提供合适的治疗方法。在这篇综述中，我们描述和讨论了与耳聋和前庭病相关的最常见的小脑共济失调类型。
