Eur J Radiol. 2025 Nov 17;194:112545. doi: 10.1016/j.ejrad.2025.112545. Online ahead of print.
ABSTRACT
OBJECTIVE: To characterize craniofacial, temporal-bone, vertebral, and systemic anomalies in oculo-auriculo-vertebral (OAV) spectrum using high-resolution computed tomography (HRCT) and to examine associations with clinical severity by the Tasse Objective Scoring System.
METHODS: We performed a retrospective study (2015-2024) at a national tertiary center including 223 clinically diagnosed OAV patients; 217 had bilateral temporal-bone HRCT suitable for analysis. HRCT assessed external auditory canal (EAC), ossicular, and intratemporal facial-nerve anatomy; inner-ear/vestibulocochlear-nerve abnormalities were evaluated in a subset. Vertebral anomalies were CT-confirmed when coverage was available; renal and cardiac findings were extracted from clinical records. Statistics included chi-square or Fisher tests with Cramér’s V, Cochran-Armitage trend tests across Tasse grades, and Spearman correlation for vertebral anomaly counts (two-sided α = 0.05).
RESULTS: Mean age was 7.6 ± 4.2 years; 55.2 % were male. In the HRCT subset, EAC stenosis/atresia and ossicular abnormalities were frequent and increased with Tasse severity (EAC: 48.4 %→59.8 %→82.8 %, p = 0.0078; ossicles: 40.3 %→49.6 %→82.8 %, p < 0.001), as did aberrant intratemporal facial-nerve course (24.2 %/27.4 %/53.3 %, p = 0.010). Inner-ear malformations were identified in 14.3 % and vestibular/vestibulocochlear-nerve anomalies in 42.9 % of those specifically evaluated. CT-confirmed vertebral anomalies occurred in 29.1 % overall; segmentation defects showed a strong grade-wise increase (p < 0.001) and the cumulative vertebral anomaly count correlated with Tasse severity (Spearman ρ = 0.41, p < 0.001). Renal anomalies were present in 16.6 % and rose across grades (p = 0.044; trend p < 0.001), whereas cardiac anomalies occurred in 14.8 % with no significant between-grade difference (p = 0.19).
CONCLUSION: Pairing HRCT phenotyping with Tasse severity stratification provides clinically actionable information for operative planning (canaloplasty/ossiculoplasty/device candidacy) and prioritizes systemic surveillance (spine and renal screening) in OAV spectrum. This integrated approach supports coordinated multidisciplinary care and offers a framework for future standardized screening and outcome-oriented research.
PMID:41289623 | DOI:10.1016/j.ejrad.2025.112545
