title: Otologic management in children with the CHARGE association.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Case series.
  
  
  METHODS
  Tertiary care urban children's hospital.
  
  
  METHODS
  37 children, 22 in the CHARGE group and 15 in the CHARGE-like group.
  
  
  METHODS
  Otorhinolaryngologic and audiologic management.
  
  
  METHODS
  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.
authors.affiliation: Division of Pediatric Otolaryngology, Children's Hospital of Philadelphia, PA 19104-4399, USA. shah@email.chop.edu
authors.forename: U K
authors.initials: UK
authors.lastname: Shah
authors.forename: L A
authors.initials: LA
authors.lastname: Ohlms
authors.forename: M W
authors.initials: MW
authors.lastname: Neault
authors.forename: K D
authors.initials: KD
authors.lastname: Willson
authors.forename: W F
authors.initials: WF
authors.lastname: McGuirt
authors.forename: N
authors.initials: N
authors.lastname: Hobbs
authors.forename: D T
authors.initials: DT
authors.lastname: Jones
authors.forename: T J
authors.initials: TJ
authors.lastname: McGill
authors.forename: G B
authors.initials: GB
authors.lastname: Healy
mesh_terms: D000015:Abnormalities, Multiple; D000293:Adolescent; D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D019465:Craniofacial Abnormalities / Q000150:complications*; D004432:Ear, Middle / Q000000981:diagnostic imaging; D005158:Facial Paralysis / Q000150:complications*; D005260:Female; D006311:Hearing Disorders / Q000150:complications* / Q000175:diagnosis; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D011859:Radiography; D013577:Syndrome