ISMJ

Background: Brown-Vialetto-Van Laere syndrome is a neurological devastating condition classified as one of motor neuron disorders. In spite of rarity, it has a widespread distribution. It mostly presented with progressive pontobulbar palsy and bilateral deafness. Diagnosis is based on clinical and electrophysiological findings. Methods: We reviewed related articles by search in pub med, Ovid, Cochrane library and Scopus database. The used key words included motor neuron disorders, Brown-Vialetto-Van laere Syndrome, progressive pontobulbar palsy and sensorineural deafness. Results: The search culminated in 351 articles which 25 were correlated with the subject of our study. The results are presented in epidemiology, pathogenesis, clinical features, diagnosis and differential diagnosis subtitles. Conclusion: In this study Brown-Vialetto-Van Laere syndrome is explained regarding laboratory and clinical findings in previously reported cases. Similar syndromes based on discriminating features were also described.