Head and Neck ImagingCharge syndrome
acronym to describe the nonrandom association of coloboma of the eye (C), congenital heart defect (H), atresia or stenosis of the nasal choanae (A), retardation of growth, development and/or central nervous system anomalies (R), genital hypoplasia (G) and ear anomalies and/or deafness (E). The 'E' traditionally refers to the mixed hearing loss (conductive and sensorineural) noted in these patients, and to the external ear malformations. On CT, a hypoplastic middle ear cavity, with ossicular malformations and an absent oval window is seen; cochlear abnormalities range from subtle deficiencies of the modiolus (see modiolus anomalies) to Mondinis malformation; the vestibule is often hypo- or dysplastic and the semicircular canals are absent.
Fig.1.
RH
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Axial CT images through left temporal bone show abnormal ossicles, possibly fixed to the middle ear walls (arrows) and a dysplastic vestibule (arrowhead, a) with absence of the semicircular canals; the cochlea shows incomplete partition (arrowhead, b), and there is no oval window. The contralateral ear displayed similar abnormalities.
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Charge syndrome, Fig.1 (a) | | Charge syndrome, Fig.1 (b) | |