The aetiology of Menière's disease (MD) remains obscure, but is likely to be multifactorial, one of the factors being a genetic predisposition. Forty-one families with more than one living member with MD were ascertained and affected and normal relations examined. Blood was collected and DNA extracted and stored. In these families the mode of inheritance is autosomal dominant, the penetrance of the mutation being about 60 per cent. Some of the family members exhibit a partial syndrome, vestibular symptoms predominating. Sporadic and familial cases exhibit the same clinical features. The striking finding is the phenomenon of anticipation, whereby with successive generations there is an earlier age of onset and a tendency to more severe manifestation. The inference, considering that the cells which regulate endolymph are of neuroectodermal origin, is that, like other neurodegenerative disorders which show anticipation, MD manifestation is likely to be related to trinucloetide expansion within a gene.
The technique, results, advantages, and complications of a combined translabyrinthine-transtentorial approach to the cerebellopontine angle are discussed. The method has been associated with a low morbidity and no operative mortality in 23 cases of acoustic nerve tumor and six with miscellaneous lesions.
KEY WORDScerebellopontine angle tumors tentorial approach translabyrinthine transj. Neurosurg. / Vohane 38 / March, 1973
The translabyrinthine route or an extension of it was used to remove 150 acoustic nerve tumors. The mortality was 2% and the morbidity low. Preservation of the facial nerve was achieved in 100% of the small and 80% of the medium-sized tumors, but in only 20% of the large growths. The complications encountered are discussed.
6 Wood R. The quest for a really child-safe closure system. Manufacturing Chemist 1973 ;44 :22-5. 7Carter SJ, reviser. Containers and closures. Cooper and Gunn's dispensing for pharmaceutical students. 12th ed. London: Pitman, 1979:13. 8 British Standards Institute. Test for mechanical strength. BS 1679 part 4. Appendix C.
Sera of 164 patients with classical Meniere's disease were screened for autoantibodies, immune complexes and HLA status. Increased thyroid microsomal antibodies were significant in those patients in the 40- to 60-year age range. Raised IgM complexes and C1q component of complement were significant in all age groups. Low levels of IgA complexes were a consistent finding and were significantly lower in the HLA Cw7 group. Patients with migraine had no increased incidence of immune disturbances.
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