OTOLOGIC MANAGEMENT IN CHILDREN WITH THE CHARGE ASSOCIATION

Objectives: To characterize otologic management of two patient groups, those with the CHARGE association and those not strictly labeled as C HARGE but with several features of the disorder (CHARGE-like), in orde r to determine: (1) the clinical validity and utility of managing CHAR GE-like children in a similar manner to patients with the strictly def ined CHARGE association, (2) the progression and prognosis of hearing loss and (3) the identification of factors that may predict the degree of hearing loss. Design: Case series. Setting. Tertiary care urban ch ildren's hospital. Patients: 37 children, 22 in the CHARGE group and 1 5 in the CHARGE-like group. Interventions: Otorhinolaryngologic and au diologic management. Main outcome measures: Otorhinolaryngologic and a udio logic evaluation. Results: All patients required otologic and/or audiologic care. Bilateral hearing loss was found in 32 patients (86%) and unilateral hearing loss in five patients (14%) when hearing was a ssessed in the absence of otitis media. Among the 32 patients with bil ateral hearing loss, 31 (97%) were able to be fit with useful hearing aids. External ear anomalies were present in 25/37 (68%) patients, and middle ear and ossicular anomalies were identified in four cases (4/3 7, 11%). 36/37 (97%) patients required surgical management of otitis m edia. Three patients (3/37, 8%) exhibited radiographic evidence of inn er ear deformity. Facial nerve dysfunction was noted in the records of 14/37 (38%) patients. No statistically significant difference was fou nd when CHARGE and CHARGE-Iike patients were compared for degree of he aring loss (P = 0.5964), type of hearing loss (P = 0.2657), worsening of hearing level (P = 0.7908), or anomalies of the external ear (P = 0 .6921), ossicles (P = 0.7908), inner ear (P = 0.7908) or facial nerve (P = 0.6409). Patients with external ear anomalies did not exhibit sta tistically different degrees (P = 0.3125) or types (P = 0.1515) of hea ring loss from patients without auricular anomalies. The presence of f acial nerve anomaly correlated significantly (P = 0.0021) with profoun d hearing loss. Conclusions: Children who are CHARGE-like may be may b e considered equivalent in terms of otologic and audiologic management to children strictly defined as CHARGE patients. These children all r equire otologic care due to the high prevalence of middle ear disease and the underlying permanent hearing loss that is both stable and aida ble. The degree of hearing loss cannot be predicted by external ear mo rphology, but may be predicted by facial nerve palsy. (C) 1998 Elsevie r Science Ireland Ltd. All rights reserved.