Superior semicircular canal dehiscence syndrome. Embryological and surgical consideration

Presenting the first case of superior semicircular canal dehiscence syndrome in the Spanish literature and to establish, using embryological studies, the period in wich superior semicircular canal dehiscence originates. 52 embryos and foetuses, from 6 mm to foetal maturity, were studied. The case of a patient suffering from superior semicircular canal dehiscence syndrome is presented. The superior semicircular canal and the intracranial space are communicated through bony lacunae, in the period between the 24th and 28th week of foetal development, but this communication is discontinued in the 30th week. Permeability of these lacunae, later in life, could result in the development of superior semicircular canal dehiscence syndrome. The clinical results of the surgical repair of this abnormal communication, in this particular case, using a middle fossa approach and a transmastoid approach is presented. Superior Semicircular Canal Dehiscence Syndrome could be due to an abnormality in foetal development and its genesis, therefore, could be prenatal. Surgical repair via transmastoid approach is a reasonable alternative treatment to the middle fossa approach.