Objective To analyze the audiological presentations of acoustic neuroma(AN) patients, especially the atypical audiological characteristics, so as to provide reference for screening AN. Methods Clinical data of 397 patients with unilateral acoustic neuroma admitted to our department from August 2019 to April 2022 were retrospectively analyzed. All patients underwent pure tone audiometry(PTA), acoustic immittance(AI), speech discrimination score(SDS), distortion product otoacoustic emission(DPOAE), auditory brainstem response(ABR) and cranial enhanced MRI. Results There were 312 patients with typical presentations and 85 patients with atypical presentations, sudden sensorineural hearing loss patients accounted for 6.3%. There were 54 cases of normal hearing, who had large or super large tumors, and 99 patients with total hearing loss also had tumors of the internal auditory canal or medium. The speech recognition ability of 5 cases of total hearing loss did not disappear completely. There were 245 cases of compatibal type of DPOAE, 72 cases of cochlear type, and 79 cases of retrocochlear type. The ABR sensitivity was 98.0%, and 3 of 8 patients with normal ABR waveform had asymmetric hearing loss. Conclusion The atypical presentations accounted for 21.4%. AN could not be excluded in patients with sudden hearing loss, normal hearing, and normal ABR. DPOAE and ABR can be used as important screening methods for AN.