Progressive familial intrahepatic cholestasis, sometimes described as Byler disease, is a lethal liver disease and its inheritance is autosomal recessive. There is a previous report on the occasional association between this disease and sensorineural hearing loss without any audiological findings. We report here two siblings, an 18-year-old female and a 16-year-old male, suffering from Byler disease and hearing loss. Pure tone, Bekesy and speech audiometries and auditory brain stem response examination were performed. Audiometric data showed hearing characteristics of cochlear origin, high-frequency loss and progressiveness. This sensorineural hearing loss possibly results from a genetic mutation. The mechanism of cochlear disorder in patients with Byler disease is unknown, however, a novel gene responsible for deafness might be found to be related to Byler disease.