Korean Journal of Audiology  2013;17(3):152-155

doi:10.7874/kja.2013.17.3.152

Two Cases of Malleostapedotomy in Congenital Oval Window Atresia.

Sang Hyeon AHN 1 ; Da Hee KIM ; Jae Young CHOI ; Bo Gyung KIM

Affiliations

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Keywords

Conductive hearing loss; Aural atresia; Stapes surgery

Country

Republic of Korea

Language

English

Abstract

Congenital anomaly of the oval window with an abnormal facial nerve course is an uncommon embryological defect, which is related to the underdevelopment of second branchial arch derivatives. Some treatments for improving hearing levels are available; these include hearing aids, vestibulotomy, neo-oval window formation, and stapes surgeries, including incudostapedotomy and malleostapedotomy. However, surgery for congenital anomalies of the oval window has rarely been described, usually in very small series of patients. We describe two cases of congenital anomalies of the oval window with aberrant facial nerve courses. One was a 40-year-old male diagnosed with unilateral congenital oval window atresia; the other was a 10-year-old male diagnosed with bilateral congenital oval window atresia. We also describe the clinical manifestations and treatment outcomes of malleostapedotomy for congenital anomalies of the oval window with aberrant facial nerve courses.