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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
Insights
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.
Objectives:
To characterize otologic management of two patient groups, those with the CHARGE association and those not strictly labeled as CHARGE but with several features of the disorder (CHARGE-like), in order to determine: (1) the clinical validity and utility of managing CHARGE-like children in a similar manner to patients with the strictly defined 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 children's hospital.
Patients:
37 children, 22 in the CHARGE group and 15 in the CHARGE-like group.
Interventions:
Otorhinolaryngologic and audiologic management.
Main Outcome Measures:
Otorhinolaryngologic and audiologic 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 assessed in the absence of otitis media. Among the 32 patients with bilateral 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 (4/37, 11%), 36/37 (97%) patients required surgical management of otitis media. Three patients (3/37, 8%) exhibited radiographic evidence of inner ear deformity. Facial nerve dysfunction was noted in the records of 14/37 (38%) patients. No statistically significant difference was found when CHARGE and CHARGE-like patients were compared for degree of hearing 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 statistically different degrees (P = 0.3125) or types (P = 0.1515) of hearing loss from patients without auricular anomalies. The presence of facial nerve anomaly correlated significantly (P = 0.0021) with profound hearing loss.
Conclusions:
Children who are CHARGE-like may be may be considered equivalent in terms of otologic and audiologic management to children strictly defined as CHARGE patients. These children all require otologic care due to the high prevalence of middle ear disease and the underlying permanent hearing loss that is both stable and aidable. The degree of hearing loss cannot be predicted by external ear morphology, but may be predicted by facial nerve palsy.
