CHARGE association refers to a constellation of congenital malformations of unknown etiology that includes ocular Coloboma, Heart defects, choanal Atresia, Retarded growth/development, Genital hypoplasia, and Ear anomalies and/or hearing loss. This paper discusses audiologic results in a set of fraternal twins with a diagnosis of CHARGE. Both infants exhibit external ear defects and audiologic patterns characteristic of CHARGE and demonstrate the variable and often asymmetric findings associated with this disorder. Early identification and evaluation are imperative for appropriate audiologic management of individuals with a diagnosis of CHARGE and for persons with CHARGE-like anomalies.