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Background and Objectives Acute low-tone hearing loss (ALHL) is gaining attention as anindependent disease identity with close association with endolymphatic hydrops and early stageMeniere’s disease (MD). This study aims to compare patients of ALHL with patients exhibitinglow-tone hearing loss and ear fullness without vertigo in various audio-vestibular assessmentsand in progression to overt MD. Subjects and Method A total of 249 patients with low-tone hearing loss with ear fullnesswithout vertigo was enrolled in this study. Of these patients, 58 patients met criteria for ALHL,which was defined as having an average hearing loss of ≥30 dB at 125, 250, and 500 Hz and ≤20dB at 2, 4, and 8 kHz. Demographics, electrocochleography (ECoG) abnormality, rate of hearingimprovement, vestibular functions, and progression to MD were analyzed. Results An average low-tone hearing loss of ALHL patients was 42.8 dB, which recoveredto 18.9 dB following a combined treatment of diuretics and oral steroid therapy. The hearingrecovery rate of this group was 87.9% and the ECoG abnormality ratio was 42.5%. Also, 15.5%of ALHL patients eventually progressed to MD. Conclusion This study described demographics and characteristics of ALHL, demonstratinga successful response to the combined treatment of diuretics and oral steroid. Also, this reportdemonstrated a close relationship between the degree of low-tone hearing loss and ECoG abnormalityand observed the progression to MD in ALHL patients. These data can be usefullyapplied in clinical setting to explain clinical outcomes of ALHL.