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Year : 2016  |  Volume : 22  |  Issue : 1  |  Page : 48-51

Congenital aberrant internal carotid artery in the middle ear: 10 years follow-up

1 Department of Otolaryngology, King Khalid General Hospital, Hafr Al-Baten, Kingdom of Saudi Arabia
2 Department of Otolaryngology, Head and Neck Surgery, Riyadh, Kingdom of Saudi Arabia
3 Department of Radiology, Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia

Date of Web Publication16-Feb-2016

Correspondence Address:
Ahmad Marai Aldhafeeri
Prince Sultan Military Medical City, Riyadh
Kingdom of Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-7749.176500

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An aberrant internal carotid artery (ICA) in the middle ear is a rare vascular anomaly causing objective tinnitus. The aberrant ICA (AICA) can mimic glomus tympanicum, dehiscent of jugular bulb, hemangioma, or cholesterol granuloma. The patient can exhibit nonspecific symptoms such as tinnitus, decrease hearing, and/or ear fullness. It is frequently diagnosed during surgery or biopsy, which may lead to massive bleeding. In this report, we present a 13-year-old girl referred from a primary health clinic with right retrotympanic mass in a routine examination. In her case, there is a history of tinnitus which increases with playing. The examination showed retrotympanic pulsatile red mass. The pure tone average discovered a mild conductive hearing loss on the same side. In addition, the images show a picture of an AICA. In this report, we also review the literature on this vascular anomaly, which may lead to serious complication if manipulated during surgery or other outpatient instrumentation.

Keywords: Aberrant internal carotid artery, Red mass middle ear, Tinnitus

How to cite this article:
Aldhafeeri AM, Alqahtani MM, Alhaidey AK. Congenital aberrant internal carotid artery in the middle ear: 10 years follow-up. Indian J Otol 2016;22:48-51

How to cite this URL:
Aldhafeeri AM, Alqahtani MM, Alhaidey AK. Congenital aberrant internal carotid artery in the middle ear: 10 years follow-up. Indian J Otol [serial online] 2016 [cited 2021 Jul 28];22:48-51. Available from: https://www.indianjotol.org/text.asp?2016/22/1/48/176500

  Introduction Top

An internal carotid artery (ICA) runs in petrous bone passing through the carotid canal. It is separated from the middle ear cleft by a thin bone. In a rare situation either congenital or acquired, an absence of this bone lamella leads to an aberrant course of the ICA through the middle ear. It is encountered more frequently among female than the male population.[1] If an aberrant ICA (AICA) is misdiagnosed, further instrumental intervention may lead to life-threatening complication.[1],[2],[3] It is difficult to provide a clinical diagnosis without images.[1],[2],[4] The differential diagnosis includes glomus tumor, high jugular bulb, hemangiomas, and cholesterol granuloma.[1],[3] In addition, a patient may have tinnitus, conductive hearing loss (CHL), and retrotympanic mass.[1],[2],[3] In this case report, we present a child with the congenital AICA with 10 years follow-up.

  Case Report Top

A 13-year-old girl was referred from school primary health care after the doctor suspected a mass behind the tympanic membrane during a routine examination. At the checkup, the patient was asymptomatic. The examination discovered right ear pulsatile retrotympanic red mass [Figure 1]. The rest of the ear, nose, and throat examination were unremarkable. The audiology examination showed right side mild CHL [Figure 2], and tympanometry was type A in both ears. The high-resolution computer tomography (CT) scan showed an absence of bone plate separating ICA from the middle ear with the posterolateral situation of the artery [Figure 3]. Diagnosis and potential complications during any possible intervention were explained to her parents. She was prescribed a yearly follow-up in the outpatient clinic.
Figure 1: Retrotympanic red mass

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Figure 2: Rinne's test conductive hearing loss, left side normal

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Figure 3: (a) Computed tomography scan axial show Rinne's test aberrant internal carotid artery (green arrow) with dilated inferior canaliculus (black arrow), (b) coronal computer tomography reconstruction confirms retrotympanic location

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At the age of 19, the girl complained of right ear tinnitus and hearing loss. The tinnitus was pulsatile during a routine physical activity. Otoscopy; however, did not discover any change in the size of retrotympanic mass. Pure tone audiogram showed almost same mild CHL. Magnetic resonance arteriography (MRA) discovered an elongated course of aberrant right ICA [Figure 4]. The patient's last visit to the clinic was at the age of 23 years, and there were no changes in her clinical picture. At the moment, she is married and a mother of a 10-month-old girl, with an uneventful perinatal period.
Figure 4: Elongate lateralize horizontal segment of internal carotid artery

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There are several pathophysiologies proposed for congenital or acquired mechanisms as a cause of the AICA in the middle ear. Acquired causes include chronic ear infection, cholesteatoma and thrombophlebitis, and granulomatous disease.[5],[6] Congenital etiology of AICA in the middle ear has three hypothesis: (1) An absence of bony lamella along vertical part leads to lateral displacement of the artery;[7] (2) stapedial artery is not involute and remains in middle ear pull on the ICA, bringing it into abnormal place.[5],[6],[7] Approximately, 30% of cases of the AICA are associated with stapedial artery;[7] and (3) the most prevalent hypothesis focuses on an embryogenic malformation of first and second branchial arches.[3] Lasjaunias and Santoyo-Vazquez hypothesis argues that cervical portion of ICA involutes [Figure 5], and consequently the compensatory ascending pharyngeal artery increases the flow and enlarges inferior tympanic artery through the caroticotympanic vessels into a horizontal portion of the petrous segment (C2).[2],[3],[5],[8]
Figure 5: Inferior tymanic artery and caroticotympanic artery compensating the absence of vertical portion of internal carotid artery

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  Discussion Top

The AICA is a rare vascular anomaly, usually manifested with nonspecific symptoms, such as pulsatile tinnitus, CHL, and retrotympanic mass. However, these symptoms can be associated with a variety of differential diagnosis, for instance, glomus tumor, high jugular bulb, hemangiomas, and vascular anomaly. This significantly complicates a clinical diagnosis of the AICA. There are many cases of an asymptomatic AICA in the literature, usually diagnosed during the middle ear surgery.[2],[8] Furthermore, the AICA may cause cholesteatoma by obstructing the eustachian tube.[9]

CT scan is a reliable way to diagnose an AICA, and further assessment may be made by MRA.[10],[11] Conventional angiography is unnecessary, thereby, avoiding possible neurological complication that occur in 2.6% of the cases with 0.3% permanent damage.[7] CT scan is effective in detecting bony lamella of carotid canal and relation of the ICA to the middle ear cavity. Feature of AICA in CT scan include: (a) ICA run adjacent to jugular bulb in a posterior position and a reduced diameter, (b) deficient bony plate along the tympanic segment of the ICA, (c) agenesis or hypoplastic vertical segment of the carotid canal, and (d) distention of the inferior tympanic canaliculus.[2],[10],[12] In addition, MRA can be used for a definitive diagnosis and an excellent assessment of intra and extracranial circulation.[10] Findings of MRA include a lateral and superior location of a carotid gene in the middle ear on the anteroposterior projection and the posterior and superior location of this structure on the lateral projection.[13] If an AICA becomes complicated by incidental external trauma, iatrogenic manipulation, infection, local disease, or tumors, in these cases other modalities of investigation can be implemented mainly angiography. Otherwise, CT scan is enough for a diagnosis and conservative follow-up of an uncomplicated AICA.

Once an AICA is diagnosed, most of the authors suggest that the surgeon should conduct a conservative management, particularly in the cases of uncomplicated AICA. Ruggles and Reed (1972) advocate in favor of surgery to relieve symptoms and to prevent possible destruction of the middle ear structures and formation of an aneurysm. The authors recommend separating the ICA from middle ear with fascia graft and then compressing it into the defect by bone graft.[14] However, this procedure may potentially compromise blood flow to the brain.[8] Glasscock et al. suggest to handle carefully the malleus that touch the AICA.[15]

  Conclusion Top

The AICA is a rare vascular anomaly that can be misdiagnosed as other vascular tumors. Biopsy or any surgical exploration should be avoided not to have devastating consequence. Therefore, careful evaluation of the possible middle ear vascular lesion should always be done by CT scan of temporal bone. MRA can be used when the case is complicated with pseudoaneurysm or if further evaluation is necessary. In conclusion, observation is the best approach for patients with uncomplicated AICA.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Surmelioglu O, Tarkan O, Ozdemir S, Özşahinoğlu C. An aberrant carotid artery; which is underlying cause of unilateral objective tinnitus. Int Adv Otol 2014;10:84-6. Available from: http://www.advancedotology.org/sayilar/81/buyuk/84-6.pdf.  Back to cited text no. 1
Roh KT, Kang H. Aberrant internal carotid artery in the middle ear. J Korean Soc Radiol 2014;71:169-72.  Back to cited text no. 2
Endo K, Maruyama Y, Tsukatani T, Furukawa M. Aberrant internal carotid artery as a cause of objective pulsatile tinnitus. Auris Nasus Larynx 2006;33:447-50.  Back to cited text no. 3
Saylam G, Tulgar M, Saatci I, Korkmaz H. Iatrogenic carotid artery pseudoaneurysm presenting with conductive hearing loss. Am J Otolaryngol 2009;30:141-4.  Back to cited text no. 4
Hunt JT, Andrews TM. Management of aberrant internal carotid artery injuries in children. Am J Otolaryngol 2000;21:50-4.  Back to cited text no. 5
Henriksen SD, Kindt MW, Pedersen CB, Nepper-Rasmussen HJ. Pseudoaneurysm of a lateral internal carotid artery in the middle ear. Int J Pediatr Otorhinolaryngol 2000;52:163-7.  Back to cited text no. 6
Merán Gil JL, Masgoret Palau E, Avilés Jurado FJ, Domènech Vadillo E, Figuerola Massana E, Martínez Novoa MD, et al. Abnormal internal carotid artery in the middle ear and severe otorrhagia after myringotomy. Acta Otorrinolaringol Esp 2008;59:142-4.  Back to cited text no. 7
Sauvaget E, Paris J, Kici S, Kania R, Guichard JP, Chapot R, et al. Aberrant internal carotid artery in the temporal bone: Imaging findings and management. Arch Otolaryngol Head Neck Surg 2006;132:86-91.  Back to cited text no. 8
Costa SS, Silva MN, Rosito LP, Selaimen FA. One case, two lessons: An aberrant internal carotid artery causing acquired cholesteatoma. Braz J Otorhinolaryngol 2014;80:453-4.  Back to cited text no. 9
Nicolay S, De Foer B, Bernaerts A, Dinther JV Parizel PM. Aberrant internal carotid artery presenting as a retrotympanic vascular mass. Acta Radiol Short Repv 2014;3:PMC4271713.  Back to cited text no. 10
Roll JD, Urban MA, Larson TC 3rd, Gailloud P, Jacob P, Harnsberger HR. Bilateral aberrant internal carotid arteries with bilateral persistent stapedial arteries and bilateral duplicated internal carotid arteries. AJNR Am J Neuroradiol 2003;24:762-5.  Back to cited text no. 11
Toros SZ, Karaca CT, Noseri H, Naiboglu B, Kalaycik C, Egeli E. Bilateral aberrant internal carotid arteries: A case report presenting with pulsatile middle ear discharge. Int J Pediatr Otorhinolaryngol 2010;74:97-8.  Back to cited text no. 12
Davis WL, Harnsberger HR. MR angiography of an aberrant internal carotid artery. AJNR Am J Neuroradiol 1991;12:1225.  Back to cited text no. 13
Ruggles RL, Reed RC. Treatment of aberrant carotid arteries in the middle ear: A report of two cases. Laryngoscope 1972;82:1199-205.  Back to cited text no. 14
Glasscock ME 3rd, Dickins JR, Jackson CG, Wiet RJ. Vascular anomalies of the middle ear. Laryngoscope 1980;90:77-88.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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