Mondini cochlea in Pendred's syndrome. A histological study

Abstract

In the past the Mondini malformation of the cochlea has been described histologically on the basis of findings in one temporal bone from a patient with confirmed Pendred's syndrome. The present study presents the histological examination of six temporal bones from 5 patients with confirmed Pendred syndrome. The characteristic Mondini cochlea was found in all preparations. It is concluded that the inner ear malformation in Pendred's syndrome is more in accordance with Mondini's original description than in other syndromes in which a Mondini-like cochlea has been described.