Capt Bridget McMullen
Inner ear malformations are fairly rare. They account for approximately 20% of the known causes of congenital deafness. A Mondini Malformation of the inner ear occurs when the cochlea does not develop fully. This aplasia will result in hearing loss of varying degree and may be associated with further inner ear abnormalities.
A discussion of etiology, pathophysiology and embryology of a Mondini Malformation will be presented. We will discuss a typical patient with a Mondini Malformation, including imaging studies of a normal cochlea and a cochlea with a Mondini malformation. We will also discuss management and prognosis of inner ear abnormalities.
This information is important for all clinical audiologists. Although this is a rare disorder, it is common enough for audiologists to encounter during their career. It is important to have good ENT support for diagnosis. It is also helpful to understand inner ear formation and long-term prognosis of inner ear abnormalities when counseling families.