Clinical Applicability
Inner ear malformations have been studied and classified in great detail
by various authors in the past. The consensus is that the timing and
severity of insult determine the morphology of the malformation and the
hearing. However, cases with severe IEMs as in our patients with normal
hearing are rare to be found in literature pointing to the fact that
certain other factors may play a more important role than the dimensions
of the cochlea alone on the ultimate hearing status of the patient.
Also, one must keep in mind that patients with a radiologically severely
abnormal cochlea may have serviceable or even a remarkably good hearing.