Background: Sudden sensorineural hearing loss (SSNHL), defined as a hearing loss of ≥30 dB affecting at least three consecutive frequencies occurring over a 72-hour period, is commonly in audiologic and otolaryngologic practice. However, SSNHL with auditory neuropathy is rarely reported. We described the clinical characteristics of a patient with SSNHL and auditory neuropathy, together with its etiologic mechanism.
Case presentation: A 27-year-old woman was referred to our otological clinic with acute diminished hearing on the left ear, associated with persistent tinnitus, aural fullness and vertigo, all for a duration of 20 days. Audiological examination showed presence of otoacoustic emissions and cochlear microphone with absent ABR on the left ear, which was consistent with the criteria of auditory neuropathy. Magnetic resonance images of the brain and inner ear implicated demyelinating lesions in the auditory nerve. Forty months after onset, all the auditory retests were normal and symmetric T2-FLAIR signals of both auditory nerves were found in inner ear magnetic resonance images.
Conclusion: We showed that the SSNHL with auditory neuropathy could be caused by demyelination. The case also suggests that some SSNHL caused by demyelination is reversible.