The West syndrome is a pediatric disease that involves muscular spasm, mental deficiency and epileptic encephalopathy. This disease tends to be noticed in the first year of life and has no etiology known. It is believed to be caused by different etiology factors as uterine infection, tuberous sclerosis, perinatal asphyxia, or post-born diseases. STUDY DESIGN: Observacional cohort with transversal cut. MATERIAL AND METHOD: In this study, ten West Syndrome patients were submitted to Auditory Brainstem Response (ABR) to evaluate the involvement of the hearing system. RESULTS: The abnormal results consisted in morphological alterations (case 7), increase of the interval I-V (cases 1, 2, 9), increase of the amplitude proportion I/V (case 8) and alteration of the thresholds. These findings suggest that the nervous system dysfunction results of the hypogenesis or degeneration of the nervous cells, as a result of the dysmyelination process. CONCLUSION: The authors recommend the use of ABR to evaluate the function of the nervous and hearing systems in patients with West Syndrome.
West syndrome; evoked auditory potentials; spasms; dysmyelination; pediatric group
