Objective: The increased endolymph volume affects a shift in the organ of Corti and basilar membrane in ears with endolymphatic hydrops (EH), which might affect distortion-product otoacoustic emissions (DPOAE) by altering the operating point of the outer hair cells. We investigated how changes in DPOAE are related to the distribution site of EH.Study Design: Prospective study.Methods: Among 403 patients with hearing or vestibular symptoms who underwent contrast-enhanced magnetic resonance imaging (MRI) for the diagnosis of EH and subsequent DPOAE testing, subjects whose hearing levels on pure tone audiometry were ≤35 dB at all frequencies were included in this study. In patients with EH on MRI, the presence and amplitude of DPOAE were evaluated between groups with hearing levels of ≤25 dB at all frequencies versus hearing levels of >25 dB at one or more frequencies.Results: There were no differences in the distribution of EH between groups. The amplitude of DPOAE had no clear correlation with the presence of EH. However, in both groups, there was a significantly higher probability of the presence of a DPOAE response from 1001 to 6006 Hz in cases with EH in the cochlea.Conclusion: Among patients whose hearing levels were ≤35 dB at all frequencies, better responses on DPOAE testing were found in subjects with EH in the cochlea.