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Otologic management in children with the CHARGE association
U K Shah1, L A Ohlms, M W Neault
1Division of Pediatric Otolaryngology, Children's Hospital of Philadelphia, PA 19104-4399, USA. shah@email.chop.edu
International Journal of Pediatric Otorhinolaryngology
|September 2, 1998
Summary
Children with CHARGE-like conditions require similar otologic and audiologic care as those with CHARGE association. Hearing loss is common, stable, and treatable with hearing aids, with facial nerve palsy predicting severity.
Area of Science:
- Otolaryngology
- Audiology
- Genetics
Background:
- CHARGE association is a complex genetic disorder with significant otologic manifestations.
- Distinguishing CHARGE-like phenotypes is crucial for appropriate management.
- Otologic issues, including hearing loss and middle ear disease, are prevalent in these patient groups.
Purpose of the Study:
- To compare otologic and audiologic management between CHARGE association and CHARGE-like patients.
- To assess the progression and prognosis of hearing loss in these children.
- To identify predictors of hearing loss severity.
Main Methods:
- A case series design was employed.
- 37 children (22 CHARGE, 15 CHARGE-like) were evaluated at a tertiary care children's hospital.
- Otorhinolaryngologic and audiologic evaluations were conducted.
Main Results:
- 86% of patients had bilateral hearing loss, 14% unilateral; 97% with bilateral loss were successfully fitted with hearing aids.
- 97% required surgical management for otitis media; 11% had middle ear anomalies.
- Facial nerve dysfunction was present in 38% and significantly correlated with profound hearing loss (P=0.0021).
- No significant differences in hearing loss or anomalies were found between CHARGE and CHARGE-like groups.
Conclusions:
- CHARGE-like children can be managed similarly to CHARGE association patients regarding otologic and audiologic care.
- High prevalence of middle ear disease and stable, aidable hearing loss necessitates consistent otologic care.
- Facial nerve palsy is a predictor of hearing loss severity, whereas external ear morphology is not.
