ObjectiveThe objetive of our study was to assess the most frequent otorhinolaryngological manifestations in patients with Down syndrome, and to propose diagnostic and management guidelines to improve their quality of life. MethodsPatients with Down’s syndrome referred to the ENT Department of two spanish Hospitals during a 4-year period were retrospectively reviewed. Data of the following variables were collected: main symptoms, diagnosis, comorbidities, surgical procedures, and complications. ResultsThirty patients with Down’s syndrome were included in our study. The most frequent reasons for referral were hearing loss and newborns from the Hearing Impairment Screening Program. Otitis media with effusion, adenoid hypertrophy and obstructive sleep apnea were the most common diagnosis. Five patients underwent head and neck surgical procedures without complications. ConclusionsHearing loss secondary to chronic otitis media with effusion and upper airway obstruction are frequent pathologies in patients with Down syndrome.