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This review examines 48 publications on its embryological basis, clinical presentation, imaging diagnosis, and management. The a-ICA results from agenesis of the first embryonic segment of the internal carotid artery, with collateralisation through the inferior tympanic artery. Presentation is often non-specific; hearing loss (47.7%) and pulsatile tinnitus (30.0%) are the most frequent symptoms. Otoscopy may show a pulsatile retrotympanic mass in the anteroinferior quadrant. High-resolution computed tomography is central to diagnosis; relevant features include absence of the normal vertical carotid canal and its exocranial opening, and enlargement of the inferior tympanic canaliculus. The a-ICA must be distinguished from the more common lateralised petrous ICA variant, which retains a normal proximal calibre and enters the anterior mesotympanum without involving the inferior tympanic canaliculus. In iatrogenic injury, immediate packing is followed by definitive treatment in most reported cases; endovascular approaches have fewer complications than surgical ligation. Preoperative recognition remains one of the main safeguards against severe haemorrhage and neurological sequelae.<\/jats:p>","DOI":"10.1007\/s00405-026-10500-9","type":"journal-article","created":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T01:40:20Z","timestamp":1785721220000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Aberrant ICA in the middle ear"],"prefix":"10.1007","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8723-0540","authenticated-orcid":false,"given":"Mugurel Constantin","family":"Rusu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,8,3]]},"reference":[{"key":"10500_CR1","doi-asserted-by":"publisher","first-page":"41","DOI":"10.5631\/jibirin.83.41","volume":"83","author":"A Kodama","year":"1990","unstructured":"Kodama A, Kawanishi K, Kimura M, Ishizuka Y (1990) Aberrant carotid artery in the middle ear. 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