A 22-year-old woman presented with severe mixed hearing loss and a flow of
cerebrospinal fluid in the middle ear during stapes surgery (stapes gusher)
. HRCT of the temporal bones showed characteristic abnormalities of the inn
er ear (bulbous dilatation of the lateral portion of the internal acoustic
meatus with incomplete separation from the cochlea, and widening of the fir
st part of the facial nerve canal) described in X-linked progressive mixed
deafness with stapes gusher. The evaluation of the patient's family reveale
d a sister with the same clinical history and identical HRCT findings, and
11 normal male relatives. This is the first report with typical findings of
this entity that affects only female members of a family, suggesting anoth
er type of inheritance. (C) 1998 Elsevier Science Ireland Ltd. All rights r
eserved.