Endolymphatic hydrops (EH) results from increased endolymph volume in the inner ear, which leads to episodic vertigo, ear fullness, and sensorineural hearing loss. In these patients, Vestibular Evoked Myogenic Potential (VEMP) examination has high sensitivity and specificity for diagnosis. These patients have also been suggested to have frequency-specific changes in otoacoustic emissions (OAE). The aim of this study is to evaluate the result of the OAE examination in patients with altered VEMP and clinical history compatible with EH. The medical records and exams of 107 patients with previous diagnosis of EH were retrospectively analyzed. The results of OAE were studied for their validation in the diagnosis of patients with EH. The results showed that 98.1% of the evaluated patients presented at least one OAE frequency with no response in low or high, with a 95% confidence interval of 95.6% to 100%. When comparing the cases with normal and altered audiometry, there was a significant difference regarding the left side (p=0.041), right (p<0.001) and left (p=0.001), low (p=0.006) and high (p<0.001). Overall, a trend towards significant difference was observed (p=0.077). It is concluded that there is a significant alteration in the OAE of patients with EH, showing that such exam has the potential for diagnosis and follow-up.