Patients with end-stage renal disease (ESRD) have a higher risk of suffering sudden sensorineural hearing loss (SSNHL), but little is known about this population. To investigate the clinical characteristics, treatment and recovery of SSNHL patients with ESRD. Records of 32 SSNHL patients with ESRD were reviewed, including clinical characteristics and hearing recovery. Patients were divided into intratympanic steroid (ITS) group and oral steroid (OS) group, and hearing recovery was compared between two groups. Twenty-six patients (81.3%) exhibited tinnitus, and 18 patients (56.3%) suffered vertigo. Mean pure-tone threshold at the initial presentation was 73.2 ± 19.4 dB, and the audiogram configuration was ascending in 9.4%, descending in 9.4%, flat in 34.4% and profound in 46.9% cases. At 3-month follow-up, percentages of patients in complete recovery, partial recovery, slight recovery, and no improvement were 18.8%, 31.3%, 21.9% and 28.1%, respectively. Furthermore, the overall recovery rate, complete recovery rate and hearing improvement were significantly higher in the ITS group than those in the OS group. SSNHL patient with ESRD often suffered a severe hearing loss with a high rate of accompanying tinnitus and vertigo. ITS may provide better audiological results for SSNHL with ESRD than OS.