Fetal vestibular aqueduct orientation on CT not exclusive to Meniere's hypoplastic endotype — seen in BOR syndrome, trisomy 21, and other congenital anomalies

A fetal vestibular aqueduct angular trajectory (ATVA ≥140°) — the CT marker of the Meniere's disease hypoplastic endotype — also appears in congenital temporal bone anomalies without Meniere's. Found in 8 ears across 6 patients in a 98-patient retrospective cohort; clinical cont…

• Retrospective single-center study (Massachusetts Eye and Ear, 2016–2024): 98 patients >12 yrs with congenital temporal bone anomalies (190 ears); two blinded neuroradiologists measured ATVA independently with consensus adjudication.
• Fetal ATVA (≥140°) in 8 ears/6 patients; intermediate ATVA (121°–139°) in 19 ears/15 patients. Abnormal ATVA most prominent in branchio-oto-renal (BOR) syndrome (4/8 patients affected) and trisomy 21 (5/11 patients with unilateral abnormal ATVA); also observed in CHARGE, Apert syndrome, and Chiari I malformation. No patient carried a Meniere's disease diagnosis.
• Key limitation: retrospective, single-center design with small per-condition subgroups and no histologic validation of endolymphatic sac morphology; causal inference not possible.

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