Juvenile myoclonic epilepsy (JME) is hypothesized to originate from the dysfunction of thalamo-cortical circuit. We aimed to analyze any changes in auditory startle response in JME patients to determine the role of brainstem in JME. The responses of 18 JME patients to auditory simulation were recorded over the unilateral orbicularis oculi, masseter, sternocleidomastoid, and extremity muscles. Results were compared with those of 18 age and gender matched healthy volunteers. Total auditory startle response frequencies were similar between the two groups (31.1 +/- A 11.1 % vs. 33.7 +/- A 8.7 %, p = 0.400). Other parameters over each muscle were also similar. There were no impacts of antiepileptic drug use or disease duration. We may conclude that our findings may provide sufficient evidence for the lack of functional changes of the auditory startle response circuit even in longstanding cases of JME.