Neurological involvement of the central nervous system in brucellosis is uncommon. Sensorineural hearing loss is a rare complication of neurobrucellosis, which has not attracted enough attention among known manifestations. A 50-year-old man presented with headache, deafness and deteriorated in cognitive functions. The presence of diffuse white matter changes on brain magnetic resonance imaging (MRI) and serum and cerebrospinal fluid findings led to the diagnosis of neurobrucellosis. The differential diagnosis of diffuse white matter diseases is constantly expanding. In the background of fever, deafness, demential symptoms, cognitive impairment and extensive white matter disease, neurobrucelosis should be considere