Dominant hereditary conductive hearing loss due to an ossified stapedius tendon.
Familial conductive deafness is rare. This report confirms the existence of a lineage with congenital conductive hearing loss in 3 generations. The results of otologic evaluations, including pure-tone audiometry, tympanometry, acoustic reflex test, and liquid test, in 14 patients in this family were consistent with the findings of ossicular fixation in 10 patients. Tympanotomies performed in both ears of 4 patients indicated stapes ankylosis caused by ossification of the stapedius tendon. The patients gained normal hearing levels using a simple surgical procedure.
Hara
A
A
Department of Otolaryngology, Institute of Clinical Medicine, University of Tsukuba, Japan.
Ase
Y
Y
Kusakari
J
J
Kurosaki
Y
Y
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006314:Hearing Loss, Conductive / Q000209:etiology* / Q000235:genetics* / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D009999:Ossification, Heterotopic / Q000150:complications* / Q000601:surgery; D010375:Pedigree; D013198:Stapedius; D013710:Tendons / Q000473:pathology* / Q000601:surgery