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Giesemann AM et al. – These cases demonstrate gradual changes in the two most severe IEMs. Clarification of terms was necessary and, based on these findings, authors propose defining otocyst deformity as a cystic structure in place of the inner ear, with the cochlea, IAC and carotid canal absent. This condition needs to be differentiated from the common cavity described by Edward Cook. A clear definition of inner ear malformations is essential if outcomes following cochlear implantation are to be compared.


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