Oval window atresia: A novel surgical approach and pathognomonic radiological finding

Abstract Objectives The facial nerve usually occupies the oval window area in patients with oval window atresia. During exploration, if the facial nerve is discovered to lie in the oval window area, this is usually regarded as a contraindication for further surgical intervention. The aim of the present paper is to demonstrate the preoperative pathognomonic radiological sign and describe a new surgical approach for this difficult situation. Methods 3 patients and 4 ears were operated due to conductive hearing loss by the same surgeon in a tertiary referral center. Their clinical presentation, radiological findings, surgical findings and final outcomes were evaluated and correlated. Results Surgical findings were identical in all 4 ears: facial nerve was running over the oval window and tympanic portion was completely dehiscent. Incus long arm was medially displaced due to abnormal development of the stapes suprastructure. In each ear a successful vestibulotomy and teflon piston placement was achieved. Preoperative mean air-bone gap of 47.5 dB was improved to 21.5 dB. There were no complications. Conclusion Oval window atresia is a rare middle ear anomaly usually regarded as a contraindication for surgical intervention. In this study we present a novel surgical approach with succesful results. However the best approach is to inform the family by showing the nerve on tomography, showing the operation video, informing the family about the sensorineural hearing loss and letting the family choose the treatment option.