Introduction: Down syndrome (DS) is the most common chromosomal anomaly; up to two thirds of children with DS may have some degree of hearing loss which may compromise language development. The objective of this review is to describe the prevalence of hearing loss in children with DS, its risk factors, and its association with language development. Method: Systematic Review of the Literature. We identified 69 articles through systematic searches in PubMed, ScienceDirect, and Scopus. Of these, 35 articles met our inclusion criteria. Results: The prevalence of hearing loss in children with DS varies between 34 and 36%. Conductive alterations associated with suppurative otitis media and a narrow auditory canal are the most frequent risk factors. Tympanostomy tube implantation is less effective in the treatment of otitis media and produces less satisfactory auditory outcomes than in the general population. Other types of implants, like Bone Anchored Hearing Aids and Cochlear Implants, produce benefits on quality of life and physical and social performance. However, studies are limited by small sample sizes. There is no evidence that a direct causal association exists between hearing loss and language development in children with DS. Conclusions: Auditory screening for children with DS should be carried out in the neonatal period, with appropriate followup and prompt diagnosis of ear infections throughout childhood, which would lead to appropriate treatments.
