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Aim: To synthesize published evidence regarding hearing impairment diagnosis and interventions in infants with or at high risk for cerebral palsy in the first year after birth. Method: Nine databases were searched for MeSH terms up to February 2020. Included studies were published in English, enrolled infants with or at high risk for cerebral palsy, and addressed hearing evaluation/rehabilitation within the first year after birth. Quality of evidence was evaluated using RTI Item Bank and QUADAS-2. Results: Eighteen articles met inclusion criteria. Quality of the evidence ranged from low to high, revealing variability in diagnostic assessment methodologies and adherence to diagnostic schedules. Concerns for bias included lack of recognition of cerebral palsy effects and etiologies on functional hearing assessment methods and results. Two interventions (hearing aid and cochlear implantation) were identified; however, reported use was inconsistent. Interpretation: Hearing screening in infants with or at high risk for cerebral palsy requires evaluation of the entire auditory pathway preferentially using comprehensive electrophysiological panels of assessments. For infants with perinatal neural insults, pediatric neurologists are uniquely positioned to recommend adherence to systematic surveillance and comprehensive audiology assessments, regardless of comorbidities and motor impairments.

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