Objective: The clinical symptoms, audiological and imaging features of unilateral cochlear nerve canal stenosis were reported. Method: The clinical data of 12 patients with unilateral cochlear nerve canal stenosis diagnosed in Beijing Friendship Hospital from January 2018 to December 2019 were analyzed retrospectively, including age of onset, clinical symptoms, signs, audiology, HRCT and MRI of temporal bone and so on. Results: Twelve patients (6 males and 6 females) were identified with unilateral cochlear nerve canal stenosis. All patients presented with hearing loss and no other complaints. Among them, 2 patients were accompanied with accessory ears and the rest had no positive signs. 11 patients had severe sensorineural hearing loss, 1 patient had normal low-frequency hearing and high-frequency hearing loss. Imaging showed that 9 patients were suspected atresia of the cochlear nerve canal, 3 patients were narrow. Conclusion: For patients with unilateral stenosis of cochlear canal, early diagnosis and intervention should be carried out as early as possible, and the healthy ears should be protected.