Hearing loss in children represents a relevant topic, which needs an increasing attention by clinicians and researchers. Unfortunately, most cases of hearing loss still remain idiopathic (most frequently reported causes are genetic, infectious, toxic). An early diagnosis is crucial, as if not properly recognized, hearing disorders may impact negatively on children development and on quality of life. Literature data show that also children with mild-moderate, or even monolateral, hearing loss may present learning or verbal language disorders, if not adequately managed. The diagnostic work-up of hearing loss in children, which starts with universal neonatal hearing screening at birth, is complex, has to define hearing threshold, and, when possible, its etiology, often by multidisciplinary approach. At the same time, the audiological follow-up of those affected by moderate-severe hearing loss must be tight, as it is necessary to verify constantly: (i) hearing threshold levels, (ii) adequateness of hearing aids fitting, and (iii) language development. This review focuses specifically on the diagnostic work-up and the rehabilitative features of moderate-severe hearing loss in children
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