ABSTRACT
Congenital anomalies of the middle ear associated to anomalies of the inner ear are rarely described. Our case displays a malformation due to an association of congenital absence of the round window and aplasia of the semi-circular canals, causing conductive hearing loss. First diagnosis was a congenital atresia isolated of the round window, related to a previous surgical exploration. We tried a novel technique designed to restore the function of the round window. Labyrinthine anomalies prevented a good outcome. This kind of malformative association could be due to the complexity of early genetic control of ear development. Attentive analysis of imaging should be performed before middle ear surgery to determine the presence of inner ear anomalies which are quite frequent and which would limit its usefulness.
