Clinical manifestations of CHARGE Association

The clinical literature regarding CHARGE Association is mostly retrospective in nature and deals largely with non-auditory issues related to the care and management of these patients with multisystem involvements. In this paper, we describe the clinical findings in 24 patients evaluated in the Division of Audiology and Electrophysiology at the University of Michigan Medical Center from 1983 to 1993. We report on the clinical manifestations of CHARGE Association in these patients with particular attention paid to their audiologic status. We discuss the relationships between auditory, ear, and craniofacial anomalies. Our review of these previously unreported cases suggests the following: (1) a variety of audiologic outcomes is possible, however, if a sensorineural or mixed hearing loss exists, it tends to be severe in degree; (2) progressive hearing loss does not appear to occur, but recurring otitis media is a probable confounding factor in the early identification of hearing loss; (3) congenital unresolved facial weakness may serve as a reliable predictor of sensorineural hearing loss; and (4) amplification use may be poor due to a number of factors. We hope to offer guidance to the professionals from assorted disciplines who participate in the care of these children.