Objective: To evaluate the development of hearing in a child with cochlear nerve deficiency (CND).Methodology: Cochlear nerve deficiency (CND) is believed to be associated with profound hearing loss and severely elevated auditory brainstem response (ABR).A child with CND participated in the study.The audiology tests were measured to evaluate the state of hearing.The computed tomography (CT) and magnetic resonance images (MRI) was performed to detect the anatomy structure of auditory.