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BACKGROUND Recently, Hydrops MRI has unexpectedly identified endolymphatic hydrops (EH) in patients presenting with profound sensorineural hearing loss (SNHL), highlighting a discrepancy that prompted further investigation. OBJECTIVE This study aimed to elucidate the mechanism of profound SNHL in ears with EH. METHODS Six (3%) of 184 patients with positive EH on Hydrops MRI presented with profound SNHL. Three of 6 patients had been previously diagnosed as Meniere's disease, and 4 patients were compromised hosts. All patients underwent an inner ear test battery followed by Hydrops MRI. RESULTS The prevalence of EH at the cochlea and vestibule was identified in 67% and 42% of the ears, respectively. Three (50%) out of 6 patients experienced a short interval of 1-2 months between the onset of profound SNHL in both ears, which was associated with superimposed infection. In contrast, another three patients (50%) had a longer interval of 5-8 years, attributed to delayed EH. CONCLUSION Profound SNHL in ears with EH suggests two potential mechanisms: a short interval (1-2 months) between onset in both ears points to a pre-existing hydrops ear further compromised by a superimposed infection, whereas a longer interval (5-8 years) indicates the development of delayed EH.