title: [Otospongiosis has a genetic disease its early detection its medical management (author's transl)].
abstract: Progress in new audiometric techniques, chiefly in impedance-audiometry, and better knowledge of the innermost mechanism of the otospongiotic disease, have led the authors to plan prevention of the otospongiotic disease, which appears to be a genetic deafness with an autosomal dominant inheritance and about 40% penetrance of genes. The bases of this prevention are: first, the early detection of stapedial fixation by means of systematic audiometric investigations, particularly elicitation of the on-off effect by impedance-audiometry; second, the treatment of oto-spongiotic children with very low doses of sodium fluoride without any risk of stunting the growth. The authors have been applying these means for four years to families of stapedectomized otosclerotic patients, but they think that it would be advisable to extend this type of prevention to the systematic detection of otospongiosis in school-children by means of audiometry investigations, including the elicitation of the stapedius reflex in order to detect a possible on-off effect. Moreover, the prescription of a fluoride treatment to otospongiotic mothers for prevention in their new-borns (exactly as in dental prevention), would allow us, with the support of parents and public authorities, to eradicate this severe disease in the near future. Twenty years ago, otospongiosis still seemed incurable, but at present this disease can be very easily controlled by a medical treatment based on enzymogenesis regulators, associated with surgery if needed.
mesh_terms: D000328:Adult; D000818:Animals; D002648:Child; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010040:Otosclerosis / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics* / Q000601:surgery; D010375:Pedigree; D011247:Pregnancy; D012969:Sodium Fluoride / Q000008:administration & dosage / Q000627:therapeutic use; D013198:Stapedius / Q000502:physiology; D013997:Time Factors; D014357:Trypsin / Q000378:metabolism
chemical_list: D012969:Sodium Fluoride; D014357:Trypsin
vernacular_title: L'otospongiose, maladie familiale. Sa détection précoce. Son traitement médical.