CLINICAL MANIFESTATIONS OF CHARGE ASSOCIATION

The clinical literature regarding CHARGE Association is mostly retrosp ective in nature and deals largely with non-auditory issues related to the care and management of these patients with multisystem involvemen ts. In this paper, we describe the clinical findings in 24 patients ev aluated in the Division of Audiology and Electrophysiology at the Univ ersity of Michigan Medical Center from 1983 to 1993. We report on the clinical manifestations of CHARGE Association in these patients with p articular attention paid to their audiologic status. We discuss the re lationships between auditory, ear, and craniofacial anomalies. Our rev iew of these previously unreported cases suggests the following: (1) a variety of audiologic outcomes is possible, however, if a sensorineur al or mixed hearing loss exists, it tends to be severe in degree; (2) progressive hearing loss does not appear to occur, but recurring otiti s media is a probable confounding factor in the early identification o f hearing loss; (3) congenital unresolved facial weakness may serve as a reliable predictor of sensorineural hearing loss; and (4) amplifica tion use may be poor due to a number of factors. We hope to offer guid ance to the professionals from assorted disciplines who participate in the care of these children.