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Hearing loss is prevalent in the Down syndrome population for a variety of reasons. Primarily, there are abnormalities present in the Eustachian tube, ossicular anomalies and remnant tissue in the middle ear cavity that contribute to the presence of conductive, sensorineural or mixed hearing loss in this population (Tedeshi, Roizen, Taylor, Murray, Curtis & Parikh, 2015). A common disease accompanied with middle ear infection is otitis media, which causes conductive hearing loss. Conductive hearing loss occurs when there is a disruption in the flow of sound waves in the outer and/or middle ear. In addition, it is hard to examine the tympanic membrane due to the narrowing of the external auditory canal (Tedeschi et al., 2015).

If hearing loss is a recurring problem in children with Down syndrome, there are various intervention strategies that can be implemented over the span of their life. These intervention strategies include both medical and surgical, such as cochlear implantation and the insertion of pressure equalization tubes. Amplification, such as hearing aids may be an option if medical or surgical intervention is not ideal. Both approaches have led to a decrease in hearing loss in this population. Although hearing loss can never be reversed, research has found the earlier intervention occurs, the more effective it is likely to be.  Early intervention is fundamental for children with Down syndrome accompanied with hearing loss. They are already at a cognitive disadvantage, so receiving therapeutic services from a young age is beneficial in multiple ways. According to the American Speech-Language Hearing Association, early intervention helps children stay on schedule with their speech and language development. If there is a hearing loss, speech may suffer, as there is a disruption in receptive language comprehension. Early intervention can also enhance the child’s awareness of their hearing loss and special communication services. In addition, early intervention allows for the documentation of the child’s progress, which can guide further intervention approaches, such as medical or surgical options. With a knowledgeable clinician, there are an abundance of benefits that children with Down syndrome can receive from early intervention services. (Shott et al., 2001).

In some scenarios, medical and surgical intervention, such as providing medication and the insertion of pressure equalization tubes are not adequate enough to subside the affects of hearing loss in children with Down syndrome. In severe cases, cochlear implantation may be suitable in order to diminish the affects of hearing loss. Cochlear implantation is implemented for severe to profound sensorineural hearing loss. Those implanted at a young age, as early as three months, receive greater benefits because language is developing at a similar rate as their peers. Since children with Down syndrome are prone to several disabilities, it is encouraged to have consistent audiology appointments and to possibly discuss cochlear implantation as early as six months.

References:
Shott, S. R., Joseph, A., & Heithaus, D. (2001). Hearing loss in children with Down syndrome. International Journal of Pediatric Otorhinolaryngology 61(3), 199-205. Retrieved March 20, 2016

Tedeschi, A. S., Roizen, N. J., Taylor, H. G., Murray, G., Curtis, C. A., & Parikh, A. S. (2015). The Prevalence of Congenital Hearing Loss in Neonates with Down Syndrome. The Journal of Pediatrics,166(1). Retrieved March 21, 2016.

-Ashley E., M.A, CF-SLP