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Original Article

Dominantly inherited low-frequency hearing loss

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Pages 165-172 | Published online: 07 Jul 2009
 

Abstract

Two families with dominantly inherited low-frequency hearing loss are descrtbed. The two families are different in mode of transmission and in audiological findings. On the basis of these two differences, it is claimed that two types of inherited low-frequency hearing loss can be distinguished. One type is the dominantly inherited low-frequency hearing loss with fully penetrant abnormal genes. The other is also most probably due to an autosomal dominant gene, but with incomplete penetrance and probably represents a malformation in the middle ear combined with a defect in the apical part of the cochlear mechanism.

Résumé

Nous rapportons le cas de deux familles présentant une atteinte héréditaire de l'audition pour les fréquences basses. Les deux familles sont différentes en ce qui concerne la transmission héréditaire et les résultats audiologiques, et cela nous permet de distinguer deux types d'atteinte de l'audition pour les fréquences basses: l'un est le résultat d'une anomalie dominante autosomique typique avec atteinte essentiellement cochléaire; l'autre est le résultat d'une anomalie autosomique non pénétrante avec une atteinte mixte de l'audition (malformation dans la transmission au niveau de l'oreillé moyenne et atteinte cochléaire apicale).

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