Authors
WE Nance, R Setleff, A McLeod, A Sweeney, C Cooper, F McConnell
Publication date
1971/3/1
Journal
Birth defects original article series
Volume
7
Issue
4
Pages
64-69
Description
An X-linked syndrome characterized in males by profound mixed deafness, vestibular abnormalities, congenital fixation of the stapes and perilymphatic otorrhea on attempted stapedectomy has been documented in a large Caucasian kindred. Female heterozygotes have similar but milder audiologic abnormalities. The syndrome of congenital fixation of the stapes with perilymphatic gusher may be a relatively common form of X-linked deafness and is an important clinical entity because affected males may be significantly benefited by sound amplification.
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WE Nance, R Setleff, A McLeod, A Sweeney, C Cooper… - Birth defects original article series, 1971