Abstract
Donnai-Barrow syndrome (DBS) is a rare autosomal recessive hereditary disorder that affects a variety of body systems. One of the most common symptoms in DBS patients is severe bilateral sensorineural hearing loss. The objective of this report is to highlight the performance of such patients after receiving cochlear implants as a management of their hearing loss. We reviewed the medical records of two cousins diagnosed with DBS before and after cochlear implantation, with a particular focus on their auditory and language performance. After receiving the cochlear implant, both patients showed substantial progress in auditory and speech perception, as well as their intelligence quotients, allowing them to join mainstream schools. In conclusion, our findings showed that cochlear implantation can be considered an ideal approach for the management of DBS patients who suffer from bilateral sensorineural hearing loss.