![]() 4 Thus, HRCT followed by MRI is necessary for the complete evaluation of cases of DIAC. 1 Rarely, DIAC can be associated with syndromes like Klippel-Feil syndrome and pontine tegmental cap dysplasia. 2 3 External, middle and inner ear abnormalities are occasionally seen with DIAC which are additionally responsible for the hearing loss and need consideration while planning for surgery. 1 It is important to differentiate IAC stenosis from DIAC on imaging because patients with IAC stenosis have dysplastic vestibulocochlear nerve and they usually benefit from electronic cochlear implantation, while cases with DIAC have aplastic/hypoplastic nerve and are usually not benefited from electronic cochlear implantation. Duplication of IAC usually occurs unilaterally and is often associated with the aplastic/hypoplastic vestibulocochlear nerve. 1 Imaging features of duplicated IAC and its association with other ear malformations are not well understood. Therefore, ossicular reconstruction of the left ear was advised for correcting conductive deafness, followed by plastic surgery consultation for correction of microtia.ĭuplication of IAC (DIAC) is a very rare anomaly with less than 50 cases reported in the literature. The absence of the cochlear nerve on the right side precluded the feasibility of cochlear implantation. Other bilateral inner ear structures and left VII/VIII nerve complexes were normal. MRI showed duplicated and narrow right IAC with non-visualisation of right cochlear nerve and normal facial nerve in the anterosuperior canal ( figure 2). For further evaluation of the VII/VIII nerve complexes, MRI was done on 3 Tesla (SIEMENS, Germany) using three-dimensional constructive interference in steady-state (CISS) images. Complete atlanto-occipital assimilation was seen ( figure 1G). Bilateral external ears were small and deformed however, external auditory canals were normal. Left internal auditory canal (IAC) was normal. ![]() Lesions in mucous membranes, an ear canal, and internal organs (e.g., stomach, intestines). Inner ear structures including cochlear turns, vestibule and semicircular canals were normal on both sides. Its called zoster sine herpete (ZSH) or internal shingles. Bilateral ear ossicles were dysplastic ( figure 1F). The two canals were separated by a bony septum ( figure 1E). The posteroinferior canal (<1 mm diameter) was terminating into the cochlea and vestibule ( figure 1C), while the anterosuperior compartment was continuous with the facial canal ( figure 1D). HRCT was performed on a 64-slice multidetector CT scanner (Philips Ingenuity, Philips Healthcare, Cleveland, Ohio, USA) which showed duplicated internal auditory canal (DIAC) on the right side, with a narrow posteroinferior and broader anterosuperior compartment.
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