A 4-year-old, intact female Border Collie was presented for evaluation of hearing impairment. Clinical, neurological, otoscopic and magnetic resonance imaging examinations were carried out to determine the cause of hearing loss, but no remarkable change was found. Then, brainstem auditory-evoked response test was performed to assess hearing loss, and the dog had a bilateral sensorineural deafness was revealed. Since possible causes of acquired hearing loss were ruled out by several examinations and history taking, bilateral later-onset deafness was suspected to be genetic and not congenital. This report suggested the possibility that dogs had inherited later-onset sensorineural deafness.