Semicircular canal dysplasia is relatively common of the labyrinthine anomalies. About 40% of patients with a malformed cochlea will have associated lateral semicircular canal (SCC) dysplasia.1 The other two common labyrinthine anomalies include SCC aplasia and SCC dehiscence.
Lateral SCC dysplasia results from disordered embryological development in the sixth week of gestation. During this time, a bud forming the SCC will form a semicircular evagination from the vestibular anlage with the central part of the pocked-shaped protrusion which adheres centrally leaving a peripheral semicircular tube. SCC dysplasia results when failed central adhesion occurs. The lateral SCC is more often deformed in this process over the posterior or superior SCC because it forms earlier in embryogenesis.
The typical appearance of SCC dysplasia is a broad, short and cystic space confluent with the vestibule.
- 1. Jackler RK, Luxford WM, House WF: Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 97(Suppl 40):2, 1987.
Inner ear pathology
inner ear anatomy
- enlarged vestibular aqueduct
- semicircular canal dysplasia
- classification of cochlear anomalies
- infection and inflammation
- trauma and miscellaneous