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The purpose of this review is to review the evaluation and management of children with syndromic hearing loss. Specific syndromes and the impact of those syndromes on managing hearing loss will be discussed.Improved molecular testing has increased the ability to identify syndromes-associated hearing loss. Accurate diagnosis of syndromic hearing loss can guide discussions regarding prognosis and appropriate management options for the hearing impairment.A significant portion of childhood hearing loss is associated with a syndrome. Depending on the syndrome, surgical intervention including a bone-anchored hearing aid or cochlear implant may be helpful. In the future, targeted gene therapies may become a viable option for treating syndromic hearing loss.