Isolated Congenital Round Window Atresia: Report of 2 Cases

Round window atresia (RWA) is an uncommon condition and can result in a conductive hearing loss. Two cases of nonsyndromal bilateral RWA in 2 members of the same family are reported. Both cases presented with a conductive hearing loss of 20 to 30 dB. High-resolution computed tomography scanning was used to diagnose the condition. The patients were rehabilitated with hearing aids. Review of the literature has shown disappointing results in hearing improvement with cochlear fenestration in an attempt to address this condition. Patients presenting with unexplained conductive hearing loss should be offered computed tomography scanning. The cases we report add to the literature to benefit future patients in preoperative counseling and better inform management.