Otologic management in children with the CHARGE association

Udayan K. Shah, Laurie A. Ohlms, Marilyn W. Neault, Karen D. Willson, William F. McGuirt, Nedda Hobbs, Dwight T. Jones, Trevor J.I. McGill, Gerald B. Healy

Research output: Contribution to journalArticle

18 Scopus citations

Abstract

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 audio logic evaluation. Results: All patients required otologic and/or audiologic care. Bilateral heating 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.

Original languageEnglish (US)
Pages (from-to)139-147
Number of pages9
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume44
Issue number2
DOIs
StatePublished - Jul 10 1998
Externally publishedYes

Keywords

  • CHARGE association
  • Cranio-facial syndromes
  • Facial nerve palsy
  • Hearing loss

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Otorhinolaryngology

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    Shah, U. K., Ohlms, L. A., Neault, M. W., Willson, K. D., McGuirt, W. F., Hobbs, N., Jones, D. T., McGill, T. J. I., & Healy, G. B. (1998). Otologic management in children with the CHARGE association. International Journal of Pediatric Otorhinolaryngology, 44(2), 139-147. https://doi.org/10.1016/S0165-5876(98)00064-0