Neuro-otological Examinations in Patients With Spinocerebellar Degeneration

Background: Spinocerebellar-degeneration （SCD） has been reported to show a variety of abnormal neurootological findings on electronystagmography （ENG） rather in a late stage of disease developing process, but not in an earlier stage.
Purpose: We have analysed ENG findings derived from 79 patients confirmed with SCD, in order to assess a diagnostic value of ENG and determine which manifestations are helpful in diagnosing SCD.
Results: We observed a high incidence of saccadic pursuit, severely impaired optokinetic nystagmus, and impaired visual suppression; observations that likely to be useful in diagnosing SCD. For each abnormal finding, we compared the data among patients with different types of SCD （olivopontocerebellar cortical atrophy, late cerebellar cortical atrophy, Shy-Drager syndrome, and hereditary SCD）. We also studied the relationship between MRI findings and ocular abnormalities in these patients with SCD. In typical cases, the abnormal neuro-otological findings are effective in an earlier diagnosis of SCD.
Conclusion: In our present study, neuro-otological examinations are demonstrated to be useful for evaluating patients with SCD.