Inner ear malformation (IEM) with associated sensoryneural hearing loss (SNHL) is a major cause of childhood disability. Computed tomography (CT) and magnetic resonance imaging (MRI) imaging play important and often complementary roles in diagnosing underlying structural abnormalities and surgical planning allows for direct visualization of the cochlear nerve and is the preferred imaging modality prior to cochlear implantation. CT is helpful to assess osseous anatomy and when evaluating children with mixed hearing loss or syndromic associations. When reviewing these cases, it is important for the radiologist to be familiar with the key imaging features. In this article, we will present the imaging findings associated with different inner ear malformations associated with congenital sensorineural hearing loss.
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