Careful follow-up during early childhood of children with Turner syndrome is necessary to detect middle ear disease and prevent sequelae. However, long-term periodic review is mandatory even after resolution of middle ear disease to detect sensorineural hearing loss.
CT is an excellent technique for depicting the cochlear modiolus. Results suggest that all ears with a large vestibular aqueduct have associated cochlear modiolar deficiencies. Thus, a large vestibular aqueduct may be only occasionally, if ever, an isolated developmental anomaly of the inner ear.
Lipoblastoma is a rare benign tumour arising from embryonic white fat. The tumours occur primarily in infancy and early childhood and usually arise from the limbs and the trunk, but neck involvement is rare. We report three cases of head and neck lipoblastoma. In all cases, imaging showed a well-delineated, fat-containing tumour. After surgical resection, the outcome of these patients was uneventful.
We reviewed the CT examinations of the temporal bone, performed with 1-mm-thick contiguous sections, of seven patients with the CHARGE association. We found abnormalities of the incus and stapes, with ossicular chain fixation, absence of the stapedius muscle and oval window, hypoplasia or dysplasia of the vestibule and absence of the semicircular canals in all ears. The pyramidal eminence and tympanic sinus were absent and there were anomalies of the cochlea in 13 of 14 ears. Absence of the semicircular canals is the most specific change in patients with the CHARGE association.
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