Epidemiology of Severe Hearing Impairment in a Population-Based Cerebral Palsy Cohort

BACKGROUND: Comorbidities including hearing impairment occur commonly in individuals with cerebral palsy (CP). METHODS: Hearing impairment was assessed in a registry-derived population-based sample of children with CP. RESULTS: Hearing impairment was documented in 12.7% (27 of 212) with less than a quarter of these (or 2.7% overall [6 of 212]) having a severe hearing loss of greater than 70 dB bi-aurally. Those with severe hearing impairment were more likely to be nonambulatory (Gross Motor Function Classification System IV/V; 100% versus 34.0% in the registry; P < 0.001) and have a spastic quadriplegic or dyskinetic CP variant (100% versus 42%, P = 0.001). Severe neonatal hyperbilirubinemia necessitating exchange transfusion demonstrated a striking association with hearing impairment in the context of CP in spite of small observed numbers; three of three had severe hearing loss, accounting for 50% of cases of severe hearing loss. CONCLUSIONS: These results suggest a specific CP context and antecedent conditions that necessitate a heightened vigilance for the detection and remediation of hearing impairment.