Three patients with a middle ear malformation characterized by the absence of the oval window and complicated by facial nerve anomalies were treated by surgery. Hearing ability of all the patients was improved. A variety of facial nerve anomalies were observed. Fenestration of the vestibule above the facial nerve was performed in the case of 2 of the 3 patients. In the other patient, fenestration of the scala vestibuli below the facial nerve was performed. In the case of all the 3 patients, hearing ability improved after the surgery and remained stable for a long time. Surgery for this disease has been considered difficult because of a high risk of complications. Because all the 3 patients showed excellent long-term surgical results, we suggest that fenestration of the inner ear should be considered one of the main options for the management and treatment of an absent oval window associated with facial nerve anomalies.