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Background And Purpose: Although the otic capsule is fully formed at birth, the vestibular aqueduct (VA) continues to mature postnatally. Failure of this maturation-VA hypoplasia-identifies a subgroup of Menière's disease (MD) patients and can be detected on CT by measuring the VA's angular trajectory (ATVA). However, the age at which ATVA stabilizes and hypoplasia can be reliably diagnosed remains unclear. We therefore defined the normal timeline of ATVA development to establish age-specific CT norms for distinguishing physiologic VA development from fetal/hypoplastic arrest.
Materials And Methods: We retrospectively reviewed temporal bone and head CTs in 159 children (318 ears) aged 0-16 years without otologic abnormality. Two head & neck radiologists measured ATVA and retrolabyrinthine bone (RL) thickness-previously established surrogates of VA hypoplasia-on axial reformatted images. Inter-reader reliability was assessed by intraclass correlation coefficient (ICC). We modeled ATVA versus age using generalized additive mixed models. First-derivative analysis of the age spline identified when ATVA change plateaued. Eighty-and 95% prediction intervals determined ages at which ATVA reliably fell below clinical thresholds (140°, 130°, 120°).
Results: Inter-reader agreement was excellent (ICC = 0.92 ATVA; 0.88, RL thickness). Mean ATVA declined from 135° (±7.5°) in infants to 98° (±6.0°) in adolescents. By 80% prediction interval, the ATVA upper limit fell below 140° by ∼1.8 years, 130° by ∼3.1 years, and 120° by ∼10.4 years. The 95% interval excluded ATVA ≥140° by ∼3.0 years and ≥130° by ∼10.3 years, but not >120° before age 16 years. No ears above age 8 years fell in the fetal category (≥140°), and none above age 12 years in the intermediate category (121°-139°). First-derivative analysis showed ATVA change plateaued at ∼5.0 years. RL thickness ≥1.2 mm universally corresponded to mature ATVA (≤120°).
Conclusions: ATVA transitions from fetal (≥140°) to mature (≤120°) trajectory over the first decade, stabilizing by ∼5 years. ATVA >120° before ∼10 years reflects normal development; after ∼12 years, it indicates adult-persistent hypoplasia. RL thickness ≥1.2 mm serves as a practical surrogate for mature VA orientation. These benchmarks empower radiologists to differentiate normal maturation from MD-associated VA hypoplasia, enabling early risk stratification and management.
Abbreviations: VA = vestibular aqueduct; ATVA = angular trajectory of the vestibular aqueduct; RL = retrolabyrinthine bone; MD = Menière's disease; ED = endolymphatic duct; ES = endolymphatic sac.
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http://dx.doi.org/10.3174/ajnr.A8933 | DOI Listing |
Eur J Pediatr
September 2025
Department of Otolaryngology-Head and Neck Surgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, 230001, Anhui, China.
Unlabelled: Enlarged vestibular aqueduct (EVA) is one of the most common inner ear malformations (IEMs) leading to hearing loss in children. Although its genetic and clinical characteristics have been studied, its manifestations in completely deaf children in China, especially those with or without incomplete partition type II (IP-II), are not yet fully understood. We conducted a comprehensive analysis of 123 pediatric EVA children with complete hearing loss.
View Article and Find Full Text PDFJ Med Case Rep
August 2025
Otolaryngology Head and Neck Surgery, Jiujiang University Affiliated Hospital, Jiujiang, Jiangxi, The People's Republic of China.
Background: Branchio-oto-renal syndrome is a rare autosomal dominant disorder characterized by branchial arch anomalies, hearing loss, and renal dysplasia. Its diagnosis remains challenging due to clinical heterogeneity and overlapping features with other syndromes. This case report aims to enhance awareness of branchio-oto-renal syndrome and highlight multidisciplinary management strategies.
View Article and Find Full Text PDFLaryngoscope Investig Otolaryngol
August 2025
Objective: To clinically validate manual measurement of cochlear length from pre-operative image of cochlea with post-operative image of cochlear implant (CI) electrode.
Methods: Temporal bone computer tomography (CT) scans of 23 ears were available for this pilot study. The inner ear was three-dimensionally (3D) segmented, and cochlea length was manually measured for preferred angular depth depending on inner ear anatomical types for CI electrode length selection.
Diagn Cytopathol
October 2025
Department of Pathology and Laboratory Medicine, University of North Carolina Medical Center, Chapel Hill, North Carolina, USA.
Endolymphatic sac tumors are uncommon, slow growing, and locally aggressive neoplasms that arise within the endolymphatic sac, most commonly the intraosseous portion located in the temporal bone. There are only three previous cytology reports, and no prior FNAs have been reported. We present a case of a metastatic sporadic endolymphatic sac tumor, a phenomenon that has yet to be described on FNA and has only been previously reported once in cerebrospinal fluid cytology.
View Article and Find Full Text PDFJ Vestib Res
August 2025
Otology & Neurotology Group CTS495, Instituto de Investigación Biosanitaria, ibs.GRANADA, Granada, Spain.
PurposeTo estimate the prevalence of endolymphatic sac hypoplasia (EShp)-a proposed specific finding in Ménière's disease (MD) that defines an endophenotype characterized by bilateral involvement, male predominance, temporal bone abnormalities, and familial clustering-in individuals without MD, to assess its specificity for the condition.MethodsWe analyzed 956 temporal bone CT scans from individuals without MD to assess the prevalence of EShp using the Angular Trajectory of the Vestibular Aqueduct (ATVA) marker. ATVA distribution, reproducibility, and associations with clinical variables were also evaluated.
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