Congenital atresia of the ear.

Congenital atresia of the ear requires prompt diagnosis and an early assessment of hearing. In bilateral atresia, aural rehabilitation should be started early in life to avoid late sequelae of speech problems. In unilateral atresia, the need is not urgent if the child hears normally in the other ear. Polytomography and audiometric testing are the two most important parts of the patient's evaluation. Precise audiometric testing may be difficult, or even impossible, in these children. The indications for surgery are presented. The risk of injuring an abnormally placed facial nerve remains a deterrent to many otologic surgeons who would otherwise operate on atresia. Surgery for correction of atresia was performed on 20 ears in 18 patients. A method of fascia graft overlay in conjunction with a center-hole skin graft was used. In 14 or 17 ears where an attempt was made at hearing rehabilitation the average preoperative air conduction threshold was 59 db and the average postoperative air conduction threshold was 24 db. Two unique cases are discussed in detail. One is a primary cholesteatoma in association with atresia, and the other is a finding of primitive and embryonic subepithelial tissue in the mastoid air cell system.