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Updated: Mar 16, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Hearing loss in Down Syndrome revisited - 15 years later
Vairavan Manickam1, Gordon S Shott2, Dorsey Heithaus3
1Department of Otolaryngology-Head & Neck Surgery, Geisinger Medical Center, Danville, PA, USA.
Insights
Children with Down syndrome (DS) continue to experience ear disease and hearing loss as they age. While most have functional hearing, ongoing monitoring of their otologic and audiologic status is crucial.
Area of Science:
- Pediatric Otolaryngology
- Genetics and Hearing Impairment
- Down Syndrome Research
Background:
- Down syndrome (DS) is associated with a high incidence of otologic issues, including chronic otitis media and hearing loss.
- Previous studies have highlighted the prevalence of these conditions in young children with DS.
- Longitudinal data on the progression of ear disease and hearing status in adolescents with DS is limited.
Purpose of the Study:
- To re-evaluate the audiologic status of young children with DS previously studied.
- To determine the incidence of pressure equalization tube (PET) placement and tympanic membrane (TM) perforations in this cohort.
- To understand the natural history of ear disease and hearing loss in Down syndrome patients as they age.
Main Methods:
- Retrospective chart review of 57 children with DS, previously part of a 5-year longitudinal study.
- Analysis of updated audiologic data for 54 participants.
- Review of audiograms, age at testing, PET placement history, and TM status.
Main Results:
- Ages ranged from 14 to 18 years (mean 16.34).
- 88.8% required PET placement (mean 3.5 procedures); 30% after age 6.
- Normal hearing was found in 44% (right ear) and 38% (left ear). Functional hearing (≤30 dB loss) was present in 83.3%.
- Sensorineural/mixed hearing loss occurred in 11% (right) and 9% (left). TM perforation rate was 17%.
Conclusions:
- Chronic otitis media and the need for PETs persist into adolescence for individuals with DS.
- Despite 83.3% having functional hearing, overall hearing levels declined with age.
- TM perforations occurred in 17%, with no cholesteatomas identified. Continued otologic and audiologic surveillance is recommended.
Objective:
In 2001, the senior authors published a study investigating hearing loss in young children (ages 11 months to 3.8 years) with Down Syndrome (DS). We re-visit this same study population to review current audiologic status, the incidence of pressure equalization tube (PET) placement(s), and rate of tympanic membrane (TM) perforations. We aim to better understand the natural history of ear disease and hearing loss in DS and assess potential complications.
Methods:
This retrospective chart review included 57 children with DS who previously completed in 2006, a 5 year, longitudinal study investigating otolaryngologic problems in DS. Updated audiologic data was available for 54. Audiograms, age of ear specific testing, PET placement(s), and tympanic membrane(TM) descriptions were reviewed.
Results:
Ages ranged from 14 to 18 years (mean 16.34 years). PET placement occurred in 88.8%, with mean of 3.5 procedures. 30% of PET's were placed after age 6. Ear specific testing was obtained in 92.5% (mean age 4.54 years). Normal hearing was present in 44% (right ear) and 38% (left ear). "Functional" hearing levels, defined as normal or mild hearing loss and speech reception threshold ≤ 30 dB, occurred in 83.3%. Sensorineural/mixed hearing loss was present in 11% (right ear) and 9% (left ear). TM perforations rate was 17%. No cholesteatomas were found.
Conclusion:
Chronic otitis media and indications for PET's persist as children with DS age. Although functional hearing occurred in 83.3%, there was an overall decrease in hearing levels as the children aged. Tympanic membrane perforations occurred in 17%. Continued surveillance of otologic and audiologic status in patients with Down syndrome is recommended.
