[Genetic deafness and Pendred syndrome]

The authors present two cases of genetic deafness in a brother and his sister. The girl has shown to suffer from a deafness belonging to Pendred's syndrome; in this case, the development of hearing symptomatology since her puberty is presented as an aggravated crisis of deafness and tinnitus coinciding with her menstruation, what suggests a possible endolymphatic hydrops depending of hormonal factors; the lack of knowledge about the ethyopatogenic mechanisms in this field generates therapeutic difficulties hard to solve.