[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.

[Moya-moya disease in a child with multiple malformations].

Moya-Moya disease is an obstruction of the internal carotids and of the afferent and efferent channels of Willis's polygon, which causes a collateral circulation, responsible for the typical angiographic image of a "puff of smoke" (Moya-Moya, in Japanese). Its etiology is unknown, and it might be congenital or acquired. It has been associated with congenital diseases such as Neurofibromatosis and disease of the falciform cells, among others.