title: [Otoneurologic symptomatology in hereditary cerebellar ataxia (Pierre Marie disease)].
abstract: Results of otoneurological examinations of 16 patients (from 6 families) with hereditary cerebellar ataxia have been analyzed. It has been found that all the patients exhibit vestibular function disturbances that manifest clinically by the truncal and supranuclear syndrome. Of other otoneurological signs the most frequent is dysphagia (in 10 out of the 16 patients) which is due not only to the dyscoordination of the glottal muscles, but also to a paresis of the epiglottis, this paresis also being a sign of supranuclear pathology. The absence of the otoneurological disturbances in practically healthy relatives of the patients (12 persons) requires further accumulation of the experimental material, since it can be a proof, that there is no pathological carrying of the gene of hereditary cerebellar ataxia (Pierre Marie's disease) and this may be used subsequently for medico-genetic consultations.
mesh_terms: D000293:Adolescent; D000328:Adult; D002524:Cerebellar Ataxia / Q000175:diagnosis / Q000235:genetics*; D002648:Child; D003680:Deglutition Disorders / Q000175:diagnosis; D005133:Eye Movements; D005260:Female; D006311:Hearing Disorders / Q000175:diagnosis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009759:Nystagmus, Pathologic / Q000175:diagnosis; D010160:Palate, Soft; D010243:Paralysis / Q000175:diagnosis; D013577:Syndrome; D014717:Vertigo / Q000175:diagnosis; D014724:Vestibular Function Tests
vernacular_title: Otonevrologicheskaia simptomatika pri nasledstvennoĭ mozzhechkovoĭ ataksii (bolezni P'era Mari).