Acute low-tone sensorineural hearing loss (ALHL) is a special type of sudden sensorineural hearing loss, which arises growing concern in clinical practice in recent years. Its clinical manifestations are sudden drop of low-frequency hearing without obvious causes, normal hearing of high-frequency area, no vertigo seizures or spontaneous eye shake, and normal auditory brainstem response (ABR). The disease seems to be related to endolymphatic hydrops, topical ischemic injury, immune factors and so on. Glucocorticoid treatment is very effective with good prognosis. Progression and recurrence of ALHL may be associated with Meniere’s disease.