Audiologic testing in children with Down Syndrome: Are current guidelines optimal?
Razan A Basonbul1, Evette A Ronner2, Anni Rong3
1Department of Otolaryngology, Harvard Medical School, Boston, MA, USA; Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, MA, USA; Department of Otolaryngology, Faculty of Medicine, King Abdulaziz University, Rabigh, Saudi Arabia.
Insights
Hearing loss is common in children with Down syndrome (DS). Optimal hearing screening for DS patients should occur before 6 months or after 10 months to ensure accurate results and avoid sedation.
Area of Science:
- Pediatric Audiology
- Genetics and Hearing Loss
- Developmental Pediatrics
Background:
- Down syndrome (DS) is a primary risk factor for childhood hearing loss.
- Established guidelines recommend close audiological monitoring for children with DS.
- Timely hearing testing is crucial for obtaining reliable data in this high-risk group.
Purpose of the Study:
- To report hearing outcomes in children with Down syndrome up to 8 years of age.
- To evaluate these outcomes against current hearing screening guidelines.
- To identify optimal timing for audiological assessments in young children with DS.
Main Methods:
- Retrospective analysis of audiometric data from children with DS (age ≤ 8 years).
- Data collected from a multidisciplinary DS clinic (January 2014 - June 2017).
- Key metrics included age at testing, test success rates, and hearing loss characteristics.
Main Results:
- 36% of DS patients failed newborn hearing screening; 9% had normal follow-up tests.
- Mild, conductive hearing loss was the most frequent diagnosis.
- Inconclusive test results were most common between 6-10 months of age.
Conclusions:
- Hearing impairment is highly prevalent in the pediatric DS population.
- To enhance test quality and minimize sedation, follow-up screenings should be scheduled before 6 months or after 10 months of age.
- Strategic timing of audiological evaluations is key for effective management in children with Down syndrome.
Introduction:
Down Syndrome (DS) is a Tier 1 risk factor for hearing loss. Guidelines exist to ensure close monitoring of children with DS for hearing loss. It is important to consider the timing of testing in order to obtain meaningful audiologic data in this high-risk population. The purpose of this study is to present hearing outcomes for children with DS during the first 8 years of life and to assess these outcomes in the context of current screening guidelines.
Methods:
Retrospective review of audiometric outcomes was conducted for children with DS age 8 or younger who presented to a multidisciplinary DS clinic between January 2014 to June 2017. Age at the time of testing, as well as test success rate and hearing loss type and severity were noted.
Results:
131 patients were included in the study, 52% of which were male. 36% of the patients failed their newborn hearing screening and only 9% of those subjects had normal hearing on subsequent testing. Most hearing loss identified was mild and conductive in nature. Inconclusive results were most likely to be obtained at 6-10 months of age.
Conclusion:
Hearing loss is common among children with DS. To optimize the quality of testing and avoid the need for sedation in followup testing, routine follow-up hearing screening should be performed either before 6 months of age or after 10 months of age.
