Mondini dysplasia

A person with Mondini dysplasia has a cochlea that is incomplete.

Mondini dysplasia, also known as Mondini malformation and Mondini defect, is an abnormality in the inner ear that can be the cause of hearing loss.

A normal cochlea has two and a half turns, a cochlea with Mondini dysplasia has two, one and a half, one, or no turns (or increments).

MRI scanning of the inner ear usually shows widened or large vestibular aqueducts with enlarged endolymphatic sacs and may show abnormalities of the cochleae known as Mondini dysplasia.