J Korean Soc Radiol.  2009 Jul;61(1):9-15. 10.3348/jksr.2009.61.1.9.

CT and MR Imagings of Semicircular Canal Aplasia

• Shin CH
• Hong HS
• Yi BH
• Cha JG
• Park SJ
• Kim DH
• Lee HK
• Kim SC

• KMID: 1471213
• DOI: http://doi.org/10.3348/jksr.2009.61.1.9

Abstract

PURPOSE: To evaluate the clinical, CT and MR imaging findings of semicircular canal (SCC) aplasia and to evaluate if a correlation exists between these findings and the associated anomalies or syndromes.
MATERIALS AND METHODS
This study retrospectively reviewed the CT and MRI findings of five patients with SCC aplasia. The CT and MR findings were analyzed for SCC, direction of facial nerve canal, cochlea, vestibule, oval or round window, middle ear ossicles, and internal auditory canal (IAC).
RESULTS
The subjects included three boys and two girls ranging in age from one to 120 months (mean age; 51 months). Four of the subjects had the CHARGE syndrome, and one had the Goldenhar syndrome. Moreover, four subjects had sensorineural hearing loss and one had combined hearing loss. The course of the facial nerve canal was abnormal in all five cases. Moreover, trapped cochlea and dysplastic modiolus were each observed in one case. Four subjects had atresia of the oval window; whereas ankylosis of the ossicles was present in three subjects. IAC stenosis was present in one patient with the CHARGE syndrome.
CONCLUSION
The aberrant course of the facial nerve canal, atresia of the oval window, and abnormal ossicles were frequently associated in patients with SCC aplasia. In addition, the Goldenhar and CHARGE syndromes were also commonly associated syndromes.

MeSH Terms

Ankylosis
CHARGE Syndrome
Cochlea
Constriction, Pathologic
Deafness
Ear, Inner
Ear, Middle
Facial Nerve
Goldenhar Syndrome
Hearing Loss
Hearing Loss, Sensorineural
Humans
Retrospective Studies
Semicircular Canals
Tomography, X-Ray Computed