Mondini dysplasia
| Classification according to ICD-10 | |
|---|---|
| Q 16.5 | Congenital malformation of the inner ear, anomaly: organ of Corti, membranous labyrinth |
| ICD-10 online (WHO version 2019) | |
The Mondini dysplasia or Mondini-deafness is a congenital malformation of the inner ear . The malformation is inherited as an autosomal dominant trait and can occur on one or both sides. The characteristic feature of the malformation is a cochlea consisting of only one and a half coils in contrast to the normally present two and a half coils. This malformation represents an inhibitory malformation from the seventh week of pregnancy. The consequence of the malformation is hearing loss of varying degrees up to complete deafness .
The deformity is named after the Italian anatomist Carlo Mondini (1729–1803), who first described it.
In addition to the reduced circumference of the coils of the cochlea, very different additional malformations of the inner ear can be found in Mondini's dysplasia, abnormal enlargements of labyrinth cavities , lack of semicircular canals , lack of the internal auditory canal , etc. Various proposals for a system of the different forms of malformation have not yet been implemented .
The use of a cochlear implant to correct the hearing loss must be clarified by examining the deficits using high-resolution computed tomography .
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- ↑ RJ Gorlin, HV Torielle, MM Cohen: Hereditary Hearing Loss and Its Syndromes. In: Oxford Monographs on Medical Genetics. No. 28, 1995. (online)
- ↑ Y. Zheng, PA Schachern, S. Cureoglu, C. Mutlu, H. Dijalilian, MM Paparella: The shortened cochlea: its classification and histopathologic features. In: Int J Pediatr Otorhinolaryngol. 2002 Mar 15; 63 (1), pp. 29-39.
- ↑ L. Sennaroglu, I. Saatci: A new classification for cochleovestibular malformations. In: Laryngoscope. 2002 Dec; 112 (12), pp. 2230-2241.
