Patients with benign recurrent intrahepatic cholestasis type 1 (BRIC type 1) or progressive intrahepatic cholestasis type 1 (PEIC type 1) caused by mutations in ATP8B1 also present with progressive hearing loss, as observed in the ATP8B1 mutant mouse model [7]. Here, ATP8B1 is linked to benign recurrent intrahepatic cholestasis.