This study aims to assess the diagnostic value of the acoustically evoked short latency negative response (ASNR) during the auditory brainstem response (ABR) test for enlarged vestibular aqueduct (EVA). The ABR test was performed on 175 subjects with severe and profound hearing loss from July 2008 to August 2011. Patients were submitted to high-resolution computed tomography scans for the temporal bone of the inner ear, and were diagnosed with EVA (EVA group; n = 24 cases, 46 ears), no inner ear deformity (no deformity group; n = 136 cases, 272 ears), or other inner ear deformity (other deformities group; n = 15 cases, 29 ears). The prevalence of ASNR was 26/46 ears (56.52 %) in the EVA group, 10/272 ears (3.67 %) in the no deformity group, and 3/29 ears (10.34 %) in the other deformities group. The rate of ASNR in the EVA group was higher than that in other groups (p < 0.05). The rate of ASNR is positively correlated with EVA. Therefore, the recording of ASNRs could be a valuable method for discovering EVA.