A retrospective analysis was performed on the clinical data of 16 cases (32 ears) from 2012 to 2017 that underwent temporal bone high-resolution computed tomography (HRCT), audiology, and cochlear implantation (CI) at Second Affiliated Hospital of Dalian Medical University for LVAS (predisposing factors, diagnosis, surgical cases, preimplantation audiological examination, and prelingual and post-language deafness).

Among the 16 patients who underwent HRCT, 11 had prelingual deafness, 2 had a family history of genetic diseases, 13 also underwent an MRI of the inner ear and brain, 9 underwent acoustic impedance tests, 6 underwent the otoacoustic emission tests, 15 underwent auditory brainstem response and audio steady-state response tests, and 6 underwent pure tone audiometry. Of the 32 ears, 1 had moderate-to-severe acoustic deafness, 1 had severe acoustic deafness, and the remaining had severe sensorineural hearing loss. The patients were treated with CI, following which 1 had dizziness, and 1 had facial paralysis.

LVAS with progressive, fluctuating hearing loss has an autosomal recessive inheritance. Early diagnosis can be made using HRCT and audiological examination. The preventive and treatment measures of LVAS currently include genetic screening for prenatal and postnatal deafness and CI.