Mondini dysplasia

The Mondini dysplasia or Mondini - deafness is a congenital malformation of the inner ear. The malformation is inherited as an autosomal dominant trait and may be unilateral or bilateral. The characteristic feature of the malformation is made ​​up only of one and a half turns of the cochlea, in contrast to the normally existing two and a half turns. This malformation is a malformation inhibition of the seventh pregnancy week shows a result of the malformation is a variable degree of hearing loss to total deafness.

The deformity is named after the Italian anatomist Carlo Mondini (1729-1803) named, who described it for the first time.

In addition to the reduced scope of the turns of the screw very different additional malformations of the inner ear abnormal extensions of labyrinth cavities, absence of the semicircular canals, the absence of the internal auditory canal can be found at the Mondini dysplasia, etc Various proposals in a classification of different malformation forms have not been enforced.

The use of a cochlear implant to correct the hearing loss should be resolved by examination of the deficits by high-resolution computed tomography.

Swell

• Ear disease
• Deafness
• Hearing
• Malformation