Identification of hearing loss in pediatric patients with Down syndrome

Otolaryngol Head Neck Surg. 2012 Jan;146(1):135-40. doi: 10.1177/0194599811425156. Epub 2011 Oct 10.


Objective: To determine the type of hearing loss, incidence of the lost to follow-up rate, and the time to diagnose sensorineural hearing loss (SNHL) in children with Down syndrome (DS) identified from a statewide database.

Study design: Case series with chart review.

Setting: Pediatric referral center.

Subjects and methods: Three hundred forty-four patients with DS born in Utah between January 2002 and December 2006 were identified using the Utah Department of Health's Newborn Hearing Screening database and birth defects registry.

Results: Three hundred thirty-two patients were included in the study. Eighty-seven infants (26.2%) did not pass their newborn hearing screening (NBS). Thirty-three of these children (37.9%) had a conductive hearing loss attributed to serous otitis media. Five infants had SNHL; 3 children were diagnosed with a mixed hearing loss (MHL). The average time to diagnose a sensorineural hearing loss was 485 ± 601 days. One child who passed his NBS was subsequently found to have an SNHL. More than 43% of the newborns with DS who passed their NBS developed a conductive hearing loss requiring insertion of ventilation tubes. Eighty-four percent of newborns with DS who did not undergo NBS did not have any apparent subsequent audiologic testing.

Conclusion: Patients with DS present with a relatively high incidence of conductive hearing loss, MHL, and SNHL and a higher lost to follow-up rate compared to patients without DS. The authors were not able to diagnose SNHL within the 90-day period recommended by the Joint Committee on Infant Hearing.

Publication types

  • Comparative Study
  • Multicenter Study

MeSH terms

  • Audiometry / methods*
  • Disease Progression
  • Down Syndrome / complications*
  • Down Syndrome / epidemiology
  • Evoked Potentials, Auditory, Brain Stem / physiology*
  • Female
  • Follow-Up Studies
  • Hearing Loss, Conductive / diagnosis*
  • Hearing Loss, Conductive / epidemiology
  • Hearing Loss, Conductive / etiology
  • Hearing Loss, Sensorineural / diagnosis*
  • Hearing Loss, Sensorineural / epidemiology
  • Hearing Loss, Sensorineural / etiology
  • Hearing Tests / methods*
  • Humans
  • Incidence
  • Infant, Newborn
  • Male
  • Otoacoustic Emissions, Spontaneous / physiology*
  • Prevalence
  • Prognosis
  • Registries / statistics & numerical data
  • Retrospective Studies
  • Time Factors
  • Utah / epidemiology