Mondini and Michel deformities of the inner ear: a case report

Abstract

Congenital hearing loss is an important disease in otology. Approximately 20 percent of congenital sensorineural hearing loss have radiological bone malformations. Inner ear anomalies, resulting in congenital sensorineural hearing loss, observation frequency are classified according to Schuknecht: Mondini, Scheibe, Michel and Alexander. These malformations are rare and occur due to stoppage of development of inner ear, in the first trimester of pregnancy. In our case, eigthyears- old patient referred us with congenital hearing loss at right ear and effusion at left ear following common cold. After evaluation of the patient, we determined the Michel aplasia and Mondini dysplasia together. A case study with diagnosis and management together with relevant literature study is presented in this article.

Keywords

• michel aplasia
• mondini dysplasia
• congenital hearing loss