Learning Objectives:

We report a case of endolymphatic hydrops with an ossicular anomaly, in which a hearing test showed fluctuating mixed hearing loss. A 42-year-old man with hearing impairment had experienced varying ear symptoms on his right side since elementary school. Evaluation by computed tomography showed an ossicular anomaly, and magnetic resonance imaging revealed endolymphatic hydrops in the symptomatic ear. Ossiculoplasty or stapes surgery is considered in patients with conductive hearing loss; however, the existence of endolymphatic hydrops is a risk factor for surgical complications. Preoperative magnetic resonance imaging examination may be beneficial when evaluating inner ear conditions such as ossicular anomalies, especially in cases accompanied by fluctuating hearing loss.