CT and MR Imagings of Semicircular Canal Aplasia

Shin, Chung Hee; Hong, Hyun Sook; Yi, Beom Ha; Gyu, Jang; Park, Seong Jin; Kim, Dae Ho; Lee, Hae Kyung; Kim, Shi Chan

doi:10.3348/jksr.2009.61.1.9

Cited by 9 publications

(9 citation statements)

References 12 publications

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“…In this condition, too, the cochlea may be normal or deformed. An abnormal course of the facial nerve, atresia of the oval window, and abnormal ossicles are frequently seen in children with aplasia of the canals (16). Absence of all semicircular ducts is known to occur frequently in patients with CHARGE syndrome (a combination of coloboma, heart anomalies, choanal atresia, retardation of growth and development, and genital and ear anomalies) (16,17).…”

Section: Malformations Of the Vestibule And Semicircularmentioning

confidence: 98%

“…Absence of all semicircular ducts is known to occur frequently in patients with CHARGE syndrome (a combination of coloboma, heart anomalies, choanal atresia, retardation of growth and development, and genital and ear anomalies) (16,17). Isolated aplasia of the posterior semicircular duct has been described in patients with Waardenburg syndrome and Alagille syndrome (16).…”

Section: Malformations Of the Vestibule And Semicircularmentioning

confidence: 99%

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CT and MR Imaging of the Inner Ear and Brain in Children with Congenital Sensorineural Hearing Loss

Joshi¹,

Navlekar²,

Kishore³

et al. 2012

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Imaging plays an important role in the evaluation of congenital sensorineural hearing loss. In children who are candidates for cochlear implantation surgery, it provides vital preoperative information about the inner ear, the vestibulocochlear nerve, and the brain. High-resolution computed tomography (CT) and magnetic resonance (MR) imaging provide excellent delineation of the intricate anatomy of the inner ear: CT depicts the minute details of osseous structures, and MR imaging allows visualization of the fluid-filled spaces and the vestibulocochlear nerve. Together, these complementary modalities can aid decision making about the best management strategy by facilitating the identification and characterization of inner ear malformations and any associated neurologic abnormalities. It is important that the radiologist be familiar with the key imaging features when interpreting CT and MR images obtained in this patient group. A broad spectrum of inner ear malformations have been described and linked to developmental insults at different stages of embryogenesis, and various systems have been proposed for classifying them. In this article, these malformations are described by using classification systems used by otolaryngologists for ease of interpretation. The relevant normal anatomy and development of the inner ear are briefly surveyed, standard imaging protocols for studying the inner ear are reviewed, and the imaging appearances of frequently observed inner ear malformations are described and illustrated. The impact of the identification of these malformations and commonly associated brain abnormalities on clinical management and prognosis also is discussed.

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Section: Malformations Of the Vestibule And Semicircularmentioning

confidence: 98%

Section: Malformations Of the Vestibule And Semicircularmentioning

confidence: 99%

CT and MR Imaging of the Inner Ear and Brain in Children with Congenital Sensorineural Hearing Loss

Joshi¹,

Navlekar²,

Kishore³

et al. 2012

RadioGraphics

161

134

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“… 1 Concomitant abnormalities of the cochlea may be seen depending on the embryological stage at which the arrest occurred 25 Furthermore abnormal semi-circular canals in isolation or associated with other labyrinthine malformations are usually seen in the setting of various syndromes. 26 …”

Section: Inner Ear Malformationsmentioning

confidence: 99%

Radiological diagnosis of the inner ear malformations in children with sensorineural hearing loss

et al. 2019

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Malformations in either the inner ear, vestibulocochlear nerve (VIIIth) or auditory cortex of the brain can lead to congenital sensorineural hearing loss (SNHL). In most cases the underlying disorders involve the membranous labyrinth at a microscopic level and therefore radiological examinations are entirely normal. In a significant proportion however (up to 20%), there are abnormalities visualized in the inner ear and/or the VIIIth nerve; the type of abnormality is relevant for the surgical planning of a cochlear implant. Imaging and the accurate radiological identification of the affected inner ear structures therefore plays an integral role in the clinical evaluation of sensorineural hearing loss. In this pictorial review, we describe the main malformations of the inner ear in view of recent classifications and briefly explore the surgical implications.

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“…Otras malformaciones afectan al vestíbulo y a los CSC, la más frecuente cursa con un vestíbulo globular agrandado y una dilatación del CSC lateral, el último en desarrollarse ( fig. 1, fig.1 B 1 ) 4,7 . La aplasia de los CSC es menos común que la displasia.…”

Section: Malformaciones Congénitas Del Oído Internounclassified

“…En el síndrome CHARGE (Coloboma, Heart anomalies, Atresia coanas, Retraso mental, hipoplasia Genital, Ear abnormalities) ( fig. 1, B 2 ) todos los CSC pueden estar ausentes 7,8 .…”

Section: Malformaciones Congénitas Del Oído Internounclassified

Diagnóstico por imagen: malformaciones congénitas y lesiones adquiridas del oído interno

Pont

Mazón

Montesinos

et al. 2015

Acta Otorrinolaringológica Española

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CT and MR Imagings of Semicircular Canal Aplasia

Cited by 9 publications

References 12 publications

CT and MR Imaging of the Inner Ear and Brain in Children with Congenital Sensorineural Hearing Loss

CT and MR Imaging of the Inner Ear and Brain in Children with Congenital Sensorineural Hearing Loss

Radiological diagnosis of the inner ear malformations in children with sensorineural hearing loss

Diagnóstico por imagen: malformaciones congénitas y lesiones adquiridas del oído interno

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