[A report of two familial cases of Michel syndrome (bilateral agenesis of the inner ear)].

In two siblings, wearing conventional hearing aid, presenting profound but not total congenital deafness, with no particular antecedents, the imaging destined to confirm the indication of a cochlear implant revealed a total bilateral agenesis of the inner ear. In one of the children, this imaging was confirmed by an exploration of the middle ear performed during a tonsillectomy that was otherwise necessary. We will summarize the literature concerning Michel's Disease, exceptional in its princeps form, and we will discuss the manner of action of conventional devices in these cases which are a priori without any sensorial element.