Objectives: To evaluate temporal bone CT findings in GJB2-related deafness (DFNB1) hearing loss and non-DFNB1 hearing loss children. Study Design: Case-control series. Subjects and Methods: Children with nonsyndromic hearing loss diagnosed as DFNB1 or non-DFNB1 after screening GJB2 allele variants and the large GJB6 deletion. Temporal bone CT images compared in a cohort of nine DFNB1 children with 10 non-DFNB1 children. Visual criteria and absolute measurements were compared against established normative values. Results: Visual inspection failed to identify two patients with abnormalities identified by using absolute measurements. Only one of nine DFNB1 children had an ear anomaly versus seven of 10 non-DFNB1 (odds ratio 16.33; 95% CI, 1.35, 197.78; P = 0.050). The non-DFNB1 group had a mean vestibule width that was significantly larger, and a mean lateral semicircular canal island width and vestibular aqueduct that were significantly smaller than the DFNB1 group. Conclusions: Visual inspection of temporal bone CT images alone may not identify all anomalies and should be used with absolute CT measurements. Abnormal temporal bone CT findings are significantly less likely in children with DFNB1 compared with non-DFNB1 children despite similar age and degree of hearing loss.
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