Previous studies have shown that patients with acoustic neuroma (AN) sometimes present with sudden sensorineural hearing loss (SSNHL) as an initial symptom. The purpose of this research was to investigate the clinical characteristics, diagnosis and treatment of AN in patients diagnosed as SSNHL initially. A total of 10 patients presented with SSNHL as the initial symptom and diagnosed as AN by magnetic resonance imaging (MRI) finally were enrolled in this study. Patient demographics, associated complaints (mostly tinnitus and vertigo), severity of hearing loss, audiogram configurations, auditory brainstem response (ABR) and MRI examination were reviewed and analyzed. Treatment outcome and management protocols were also included in this study. These patients exhibited varying severity of hearing loss and a variety of audiogram configurations. All patients showed an abnormal ABR. According to the Koos grading standard, there were 5 grade I (intracanalicular) tumors, 3 grade II tumors and 2 grade III tumors. The treatment outcome revealed that 2 patients exhibited recovery of the average hearing of impaired frequency by more than 15 dB, and 6 patients showed no recovery. Four patients were referred to undergo surgical treatment after being diagnosed with AN, 1 patient accepted stereotactic radiation therapy, and the remaining 5 patients were taken “waiting and scan” strategy. The findings of this study show that hearing recovery after corticosteroid treatment for SSNHL does not exclude the presence of AN and all patients diagnosed as SSNHL initially should undergo MRI and ABR to prevent misdiagnosis and delays in potential treatment.