Audiometric assessment of pediatric patients with cystic fibrosis

J Cyst Fibros. 2018 May;17(3):383-390. doi: 10.1016/j.jcf.2017.10.007. Epub 2017 Dec 27.


Background: The purpose of this study was to evaluate hearing impairment in pediatric patients with cystic fibrosis (CF).

Methods: This is a retrospective analysis of the AudGen database generated by Children's Hospital of Philadelphia. Audiograms were analyzed for type of hearing loss (HL), pure-tone-average (PTA), laterality, and change in hearing over time. Medical charts were reviewed to identify factors that influence development and progression of hearing loss.

Results: 217 patients with CF were included in this study. 69 (31.8%) had hearing loss on initial audiogram. Chronic otitis media (OR: 2.4, 95% CI: 1.3-4.5, p<0.01), Eustachian tube dysfunction (OR: 2.4, 95% CI: 1.4-5.4, p<0.01), and otorrhea (OR: 6.3, 95% CI: 1.6-24.7, p<0.01) were positive predictors of HL. Children with a diagnosis of diabetes had more decline in hearing over time than those without diabetes (12.4±17.2dB worsening vs. -5.7±9.8dB improvement in PTA, p=0.014).

Conclusion: This is the largest comprehensive analysis of all types of hearing loss in pediatric patients with CF. Our data suggest that children with more severe sinus disease may be at lower risk for inflammatory middle ear disease and subsequent hearing loss. Patients who develop complications of CF such as diabetes should be monitored frequently, and the use of ototoxic drugs should be limited if possible.

Keywords: Audiogram; Conductive hearing loss; Cystic fibrosis; Hearing loss; Otitis media; Sensorineural hearing loss.

MeSH terms

  • Adolescent
  • Audiometry / methods*
  • Child
  • Child, Preschool
  • Cystic Fibrosis* / complications
  • Cystic Fibrosis* / epidemiology
  • Diabetes Mellitus / epidemiology
  • Disease Progression
  • Female
  • Hearing Loss* / diagnosis
  • Hearing Loss* / etiology
  • Hearing Loss* / prevention & control
  • Humans
  • Male
  • Retrospective Studies
  • Risk Factors
  • Sinusitis / epidemiology
  • United States / epidemiology