Neuro-Ophthalmologic and Vestibular Outcomes in Patients With Unilateral Coronal Craniosynostosis

Unilateral coronal craniosynostosis (UCS) leads to significant craniofacial asymmetry and is associated with ocular anomalies. However, existing research on associated vestibular and neuro-ophthalmologic burdens is limited by small sample sizes. This study utilizes the largest cohort to date to quantify these associations.

A matched retrospective cohort study was conducted using Epic Cosmos data from January 2017 to March 2025. Patients with operatively repaired UCS (n=637) were matched 1:2 to control pediatric patients without craniosynostosis, based on age, sex, race, and ethnicity. Outcomes included amblyopia, strabismus, diplopia, unspecified visual field defects, Chiari malformation, and balance issues. Risk differences (RD) were derived from Poisson log-link regression.

Of 637 UCS patients (median age at repair: 8.3 mo), significant elevations in neuro-ophthalmologic and vestibular comorbidities were observed. UCS patients exhibited higher rates of strabismus (29.7% versus 1.5%; P<0.001) and amblyopia (22.4% versus 0.6%; P<0.001). Notably, UCS was associated with a significant vestibular burden, with balance disorders identified in 7.5% of patients compared with 2.1% of controls (RD=5.4%, P<0.001). Chiari malformation was also more prevalent in the UCS cohort (0.8% versus 0%, P=0.007). Time-to-event analysis confirmed higher cumulative risks for strabismus, amblyopia, and balance disorders in the UCS group (P<0.001).

Children with unilateral coronal craniosynostosis have an elevated risk of neuro-ophthalmologic and vestibular impairments, including a novel 5.4% risk difference in balance disorders. These findings support the need for comprehensive and early ophthalmologic and vestibular screening.