Fehlbildungen des Innenohrs – Diagnostik in CT, MRT und DVT

Angeborene Fehlbildungen des Innenohrs sind die häufigste Ursache eines Hörverlusts bei Kindern – und für eine Bildgebung des Felsenbeins im Kindesalter. In der Bildgebung ist das meist betroffene membranöse Labyrinth nicht abgrenzbar, bei bis zu 20% der Patienten zeigen sich jedoch Veränderungen der Innenohrstrukturen oder des Hörnervs. Für die optimale Patientenversorgung ist dann die korrekte Klassifizierung der Fehlbildung entscheidend.

Abstract

Congenital malformations are the most common cause of hearing loss in children and the most common cause of temporal bone imaging in childhood. In the vast majority of cases, the malformation involves the membranous labyrinth and cannot be delineated on clinical imaging. However, in up to 20% of patients, abnormalities in the inner ear structures or auditory nerve can be delineated on imaging. Correct classification of the malformation is critical for optimal patient management. In this review, common malformations of the inner ear are described according to the currently valid classification. Their typical image-morphological characteristics are shown, as well as their clinical manifestation and therapeutic options.

Schlüsselwörter

Fehlbildungen Innenohr - sensorineuraler Hörverlust - CT - MRT

Key words

Congenital malformations of the inner ear - sensorineural hearing loss - CT - MRI

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