There still is inadequate literature regarding the surgical indications and postoperative outcomes of cochlear implantation (CI) in patients with Branchio-oto-renal syndrome; therefore, the aim of this study was to investigate whether CI surgery improved the hearing and speech performance of children with BOR syndrome-related severe hearing loss. Clinical manifestations, genetic results,audiological features, intraoperative findings and post-CI outcomes of four children were ascertained and analysed. All patients had bilateral profound HI before CI). The surgery of one patient with aural atresia and complex ear deformity was rather challenging, but the outcome after CI was satisfactory. However,auditory and speech performance of other three patients wasn’t good enough, which was probably related to cochlear nerve deficiency.