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Hearing loss in children with Down syndrome
N J Roizen1, C Wolters, T Nicol
1Department of Pediatrics, University of Chicago Pritzker School of Medicine, Illinois.
Insights
Children with Down syndrome often have hearing loss. Auditory brain-stem responses revealed significant hearing deficits in most young children with Down syndrome, highlighting the need for early auditory evaluation.
Area of Science:
- Pediatric Audiology
- Developmental Pediatrics
- Genetics
Background:
- Children with Down syndrome exhibit a high incidence of hearing deficits.
- Conventional hearing tests are challenging for young children (< 3.5 years) with Down syndrome due to cooperation issues.
Purpose of the Study:
- To assess auditory brain-stem responses in infants and young children with Down syndrome.
- To determine the prevalence and type of hearing loss in this population.
Main Methods:
- Auditory brain-stem responses were evaluated in 47 unselected patients aged 2 months to 3.5 years.
- Hearing loss was categorized by type (conductive, mixed, sensorineural) and degree (mild, moderate, severe to profound).
Main Results:
- 34% of children had normal hearing, while 28% had unilateral and 38% had bilateral hearing loss.
- Conductive loss was found in 19 ears, mixed in 14, and sensorineural in 16.
- No association was found between language quotients and the degree of hearing loss.
Conclusions:
- A significant prevalence of hearing loss exists in young children with Down syndrome.
- Early evaluation of auditory brain-stem responses within the first 6 months of life is recommended for children with Down syndrome.
Abstract:
Conventional behavioral testing of hearing shows that children with Down syndrome have a high prevalence of hearing deficits. However, most young (< 3.5 years of age) children with Down syndrome are unable to cooperate in this type of testing. We evaluated auditory brain-stem responses of 47 unselected patients 2 months to 3 1/2 years of age. Thirty-four percent had normal hearing, 28% had unilateral loss, and 38% had bilateral loss. Type of loss was conductive in 19 ears, mixed in 14, and sensorineural in 16. Degree of loss was mild in 33 ears, moderate in 13, and severe to profound in 3. Language quotients and degree of hearing loss were compared and an association was not found. Because of the high prevalence of hearing loss in children with Down syndrome, we recommend evaluation of auditory brain-stem responses in the first 6 months of life.