DUEN-LII HSIEHYI-HO YOUNG2023-02-182023-02-182006-04-0110163190https://scholars.lib.ntu.edu.tw/handle/123456789/628417Rapidly progressive sensorineural hearing loss is a specific type of sudden deafness characterized by a progressive stepwise process over a period of few hours to months with the mechanism attributable to autoimmune reactivity. The hearing organs are always involved and usually the vestibular organs as well. Recently, we have encountered a 33-year-old woman with rapidly progressive sensorineural hearing loss bilaterally. A battery of audiovestibular function tests e.g. audiometry, distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), caloric test, and vestibular evoked myogenic potential (VEMP) test were conducted throughout the course. The sequence of audiovestibular dysfunction was absent DPOAE initially, followed by total deafness and absent ABR, and finally absent VEMPs and caloric areflexia, indicating that the cochlea is vulnerable earlier than the vestibule. Hence, this case demonstrates the sequence of degeneration in the inner ear, that is, rapid progressive hearing loss followed by vestibular loss, similar to that seen in the aging process (degeneration) in the inner ear.Autoimmune sensorineural hearing loss | Idiopathic bilateral sensorineural hearing loss | Rapid progressive hearing loss | Sudden deafnessjournal article2-s2.0-33745083954https://api.elsevier.com/content/abstract/scopus_id/33745083954