Because several cases of cerebral-spinal fluid leakage (perilymph gushers) have been reported during stapedectomy for congenitally fixed stapes in children, many otologists advise against surgical intervention. The pathophysiologic mechanism of the gusher and its association with a congenitally fixed stapes is, however, poorly understood. This study was designed to examine the pre-operative presentation and hearing results of 10 children undergoing stapedectomies for congenital fixation. No perilymph gushers of significant sensorineural hearing loss was encountered. All but one case resulted in closure of the air-bone gap and speech frequencies to within 20 dB. The average air-bone gap was 11.2 dB. Minor congenital abnormalities of the stapes suprastructure were seen in 3 cases. Review of case reports in the literature indicate that gushers in association with surgical manipulation of the congenitally-fixed stapes occur almost exclusively in males and that pathophysiology involves a defect in the internal auditory canal (as opposed to a patent cochlear aqueduct). A pre-existing sensorineural hearing loss may also be a risk factor for perilymph gusher.