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.