Mondini dysplasia, also known as Mondini malformation and Mondini defect, is an

abnormality of the inner ear that is associated with sensorineural hearing loss.

This deformity was first described in 1791 by Carlo Mondini after examining the inner ear of a
deaf boy. The Mondini dysplasia describes a cochlea with incomplete partitioning and a reduced
number of turns, an enlarged vestibular aqueduct and a dilated vestibule. A normal cochlea has
two and a half turns, a cochlea with Mondini dysplasia has one and a half turns; the basal turns
being normally formed with a dilated or cystic apical turn to the cochlear. The hearing loss can
deteriorate over time either gradually or in a step-wise fashion, or may be profound from
birth."case history".

Hearing loss associated with Mondini dysplasia may first become manifest in childhood or early
adult life. Some children may pass newborn hearing screen to lose hearing in infancy but others
present with a hearing loss at birth. Hearing loss is often progressive and because of the
associated widened vestibular aqueduct may progress in a step-wise fashion associated with
minor head trauma. Vestibular function is also often affected. While the hearing loss is
sensorineural a conductive element may exist probably because of the third window effect of the
widened vestibular aqueduct. The Mondini dysplasia can occur in cases of Pendred Syndrome
and Branchio-oto-renal syndrome and in other syndromes, but can occur in non-syndromic
deafness.