Cochleovestibular anomalies in children with cholesteatoma

To describe the cochleovestibular apparatus on computed tomography (CT) imaging in patients with cholesteatoma. We asked whether cochleovestibular anomalies coexist in individuals with cholesteatoma.

Randomized, controlled, prospective measurement.

A database search yielded 145 children with cholesteatoma: 31 met inclusion criteria by not having sensorineural hearing loss, not having an associated syndrome, and having digitally stored temporal bone CT imaging available. Prospective measurement of 31 individuals (62 ears) with unilateral cholesteatoma and 32 normally hearing nonsyndromic controls (64 ears) was performed by a neuroradiologist blinded to the study objective. Twenty-six temporal bone aspects on axial imaging were evaluated (16 measurement, 10 calculated from measurement).

The cholesteatoma group had a larger endolymphatic fossa and vestibular aqueduct, and there was a trend for the lateral semicircular canal vestibule to be smaller as compared with controls. Subgroup analysis revealed a gradient in prevalence of these findings being most common in the congenital cholesteatoma group, intermediate in the acquired cholesteatoma group, and least common in controls. There were no differences in measurements between ears with cholesteatoma and contralateral disease-free temporal bones.

Children with cholesteatoma have abnormal vestibular anatomy. The gradient in prevalence of these findings may suggest a relationship between congenital and acquired cholesteatoma. These may include a generalized temporal bone anomaly that predisposes to cholesteatoma formation, or a third variable such as genetic mutation may predispose to both anomalous cochleovestibular formation and cholesteatoma.