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Published on: January 23, 2017
Identification of hearing loss in pediatric patients with Down syndrome
Albert H Park1, Matt A Wilson, Paul T Stevens
1Division of Otolaryngology-Head and Neck Surgery, University of Utah, Salt Lake City, Utah, USA. pcapark@ihc.com
Insights
Children with Down syndrome (DS) have a high rate of hearing loss, including sensorineural hearing loss (SNHL), and often miss recommended diagnosis timelines. Early detection and intervention are crucial for these children.
Area of Science:
- Pediatric Audiology
- Genetics and Hearing Impairment
- Down Syndrome Research
Background:
- Children with Down syndrome (DS) exhibit a higher prevalence of hearing loss compared to the general pediatric population.
- Early identification and management of hearing deficits are critical for developmental outcomes in infants with DS.
Purpose of the Study:
- To investigate the types of hearing loss, incidence of loss to follow-up, and diagnostic timeliness of sensorineural hearing loss (SNHL) in children with Down syndrome.
- To assess adherence to the Joint Committee on Infant Hearing's 90-day diagnostic recommendation for SNHL in this population.
Main Methods:
- A retrospective case series design was employed, analyzing data from a statewide database.
- Chart review of 332 children with Down syndrome born in Utah between 2002 and 2006, identified through newborn hearing screening and birth defect registries.
Main Results:
- 26.2% of infants with DS did not pass their newborn hearing screening (NBS).
- Conductive hearing loss was prevalent (37.9% of those who failed NBS), often due to otitis media.
- Sensorineural hearing loss (SNHL) and mixed hearing loss (MHL) were identified in 5 and 3 infants, respectively. The average time to diagnose SNHL was 485 days, exceeding the recommended 90-day window.
Conclusions:
- Children with Down syndrome have a significant incidence of conductive, mixed, and sensorineural hearing loss, along with a higher rate of lost to follow-up.
- Diagnostic delays for SNHL in infants with DS are common, falling short of established guidelines.
- Increased vigilance and improved follow-up protocols are necessary for managing hearing loss in children with Down syndrome.
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.
