Tyler R Halle1, Bruno P Soares2, N Wendell Todd3. 1. Department of Otolaryngology-Head & Neck Surgery, Emory University School of Medicine, 1365 Clifton Road, Atlanta, GA, 30322, USA. 2. Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, 1800 Orleans Street, Baltimore, MD, 21287, USA. 3. Department of Otolaryngology-Head & Neck Surgery, Emory University School of Medicine, 1365 Clifton Road, Atlanta, GA, 30322, USA. Electronic address: ntodd@emory.edu.
Abstract
OBJECTIVES/HYPOTHESIS: We aim to define the frequencies of anomalies of the inner ear, oval window, and round window ipsilateral to isolated non-syndromic unilateral aural atresia. METHODS AND MATERIALS: Retrospective case series. We reviewed high resolution computed tomography scans of the temporal bones of 70 children with isolated non-syndromic unilateral congenital aural atresia. Scans were reviewed according to the Jahrsdoerfer criteria and further evaluated for anomalies of the vestibule, semi-circular canals, cochlea, internal auditory canal and vestibulocochlear nerve. RESULTS: Inner ear dysplasia was seen in two of 70 atretic ears: one with a dysmorphic lateral semicircular canal and another with a large vestibule assimilating the lateral semicircular canal. Abnormalities of the oval window and round window ipsilateral to the atresia were identified in 21% (15) and 7% (5), respectively, of the atretic ears. Oval window and round window abnormalities were associated with disproportionately lower Jahrsdoerfer scores compared to aural atresia patients without these abnormalities (P < 0.001 and P = 0.04, respectively). CONCLUSION: Compared to studies that included syndromic or bilateral atresia cases, we found inner ear and oval window abnormalities less common in children with isolated non-syndromic unilateral aural atresia. However, round window anomalies seem to occur at about the same frequency.
OBJECTIVES/HYPOTHESIS: We aim to define the frequencies of anomalies of the inner ear, oval window, and round window ipsilateral to isolated non-syndromic unilateral aural atresia. METHODS AND MATERIALS: Retrospective case series. We reviewed high resolution computed tomography scans of the temporal bones of 70 children with isolated non-syndromic unilateral congenital aural atresia. Scans were reviewed according to the Jahrsdoerfer criteria and further evaluated for anomalies of the vestibule, semi-circular canals, cochlea, internal auditory canal and vestibulocochlear nerve. RESULTS: Inner ear dysplasia was seen in two of 70 atretic ears: one with a dysmorphic lateral semicircular canal and another with a large vestibule assimilating the lateral semicircular canal. Abnormalities of the oval window and round window ipsilateral to the atresia were identified in 21% (15) and 7% (5), respectively, of the atretic ears. Oval window and round window abnormalities were associated with disproportionately lower Jahrsdoerfer scores compared to aural atresiapatients without these abnormalities (P < 0.001 and P = 0.04, respectively). CONCLUSION: Compared to studies that included syndromic or bilateral atresia cases, we found inner ear and oval window abnormalities less common in children with isolated non-syndromic unilateral aural atresia. However, round window anomalies seem to occur at about the same frequency.
Authors: Jean-Paul Bryant; Vikram Chandrashekhar; Anthony J Cappadona; Pashayar P Lookian; Vibhu Chandrashekhar; Danielle R Donahue; Jeeva B Munasinghe; H Jeffrey Kim; Alexander O Vortmeyer; John D Heiss; Zhengping Zhuang; Jared S Rosenblum Journal: Front Neurol Date: 2021-07-15 Impact factor: 4.003