Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 10 3-4 2011 12 15 54 7 Samira Yadegari M.D, Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran. Askar Ghorbani M.D, Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran Mitra Ansari Dezfouli School of Biology, University College of Science, University of Tehran, Tehran, Iran Shahriar Nafissi M.D, Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran 2015 10 17 Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurological disorder. We report our finding about four patients clinically and electrophysiologically diagnosed as BVVLS and denoted their clinical features with comparison to previous reports. The first symptom was bilateral hearing loss and the onset of other cranial nerves involvement varied between 0-15 years. Our patients represented some rare features like upper motor neuron signs in one patient and two cases interestingly developed some clinical improvement. This is the first report of BVVLS from Iran. Our patients which represent Caucasian population had generally similar features like previously reported cases. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/843 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/843/249