Bilateral symmetrical maldevelopment of external and middle-ear cleft with pharyngeal and soft palate defects.
An unusual combination of first and second arch anomlies is presented. This has resulted in absent middle and external ears with gross naso- and oropharyngeal defects. A brief reference is made to the embryology of this region to explain the anatomical and functional defects. Vascular abnormality during the peri-gestational period is suggested as a possible cause and a conservative line of treatment has been followed with good result.
D001299:Audiometry; D001844:Bone Conduction; D001934:Branchial Region; D002648:Child; D003638:Deafness / Q000209:etiology; D004431:Ear, External / Q000002:abnormalities*; D004432:Ear, Middle / Q000002:abnormalities*; D005260:Female; D006801:Humans; D010159:Palate / Q000002:abnormalities*; D010614:Pharynx / Q000002:abnormalities*