Usher syndrome is a genetic disorder featured by combined visual impairment and hearing loss.  Currently, the genetic basis remains unknown in 20-30% of patients.  In this article, we described the identification of CEP78 as a new gene that leads to a mild Usher syndrome featured by juvenile or adult-onset cone-rod dystrophy and sensorineural hearing loss when mutated.  Our results add additional link between ciliopathy and Usher protein network in photoreceptor cells and inner ear hair cells. (http://jmg.bmj.com/content/early/2016/09/21/jmedgenet-2016-104166 )