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Unilateral auditory neuropathy spectrum disorder (U-ANSD) is characterized by abnormal audiological findings such as abnormal or absent auditory brainstem response with normal otoacoustic emissions in one ear. Some cases with U-ANSD might have cochlear nerve agenesis or dysplasia. In this case report, we represent a case with U-ANSD and cochlear agenesis.doi:10.6084/m9.figshare.7066286 fatcat:2taihcennjhh7oljbtrcpnakae