Clinical characteristics in unilateral cochlear nerve canal stenosis

OBJECTIVE: To characterize the clinical features of patients with congenital hearing loss and unilateral cochlear nerve canal stenosis (CNCS). METHODS: A retrospective review of 12 patients with unilateral CNCS diagnosed between January 2018 and December 2019 at a tertiary referral hospital was performed. RESULTS: Of the 12 patients identified, there were 6 males and 6 females. All patients presented with hearing loss, with no other chief complaints. Two patients had accessory auricles. Eleven patients had a severe to profound sensorineural hearing loss on the affected side, while 1 patient had an isolated high-frequency hearing loss. Nine patients demonstrated atresia of the cochlear nerve canal (CNC), while three patients had a stenotic, but patent, CNC. CONCLUSION: Prompt radiologic diagnosis of patients with unilateral CNCS is important for patient counseling and appropriate rehabilitation.