The Richards-Rundle syndrome (RRS) is characterized by hearing loss, mental deterioration, ataxia, primary hypogonadism and autosomal recessive transmission. In a sibship of six members we found two sisters with RRS together with baldness, impaired GH and PRL secretion after stimulation and different degrees of impaired insulin secretion. Cochleovestibular investigation of the sibship revealed in each subject more or less severe forms of bulbo-pontine cochleovestibular dysfunction. Three members of the same sibship had cutaneous signs of abortive forms of neurofibromatosis: the son of one of these subjects had a severe form of fully developed neurofibromatosis. Whether there is a pathogenetic linkage between the hereditary multisystemic degeneration (RRS), the dysembryopathy (neurofibromatosis) and the cochleovestibular dysfunction in this family is still not clear.

Acoustic Impedance Tests↗

PubMedDate not supplied

[Cystic parotid lesions in HIV infection].

The appearance of parotid lesions of cystic aspect with a HIV's infections has been described in the past by foreign Authors above all coming from USA and from Africa. Similar cases has been recorded in France but only among children. The Authors report a casuistry constituted by five male sex's and white race's subjects, everyone HIV positive, suffering from mono or bilateral benign lymphoepithelial cysts. Every patient under examination presented a positive history because of the use of intravenous drug. Four of the five subjects underwent a surgical treatment of parotidectomy. Echotomography, CT and RM scanning seem to be the more sensitive diagnostic techniques. The peculiar histopathologic aspects allow us to differentiate these lesions both from the classical benign lymphoepithelial cysts and from the Warthin's tumor. The increase of the signalling of these cases seems to be connected with the progressive diffusion of the infection and with major survival of the patients thanks to chemotherapy. The majority of the Authors is agreed to recommend the surgical treatment for the possible malignant degeneration of this kind of forms. A careful clinical and diagnostic analysis of every patient with parotid swelling, above all if at risk for HIV's infection, is strictly necessary.

Adult↗