Stapedectomy for congenital fixation of the stapes.

Because several cases of profuse cerebrospinal 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 perilymph gusher and its association with congenital fixation of the stapes is, however, poorly understood. This retrospective study was designed to examine the preoperative presentation and hearing results of children undergoing stapedectomy for congenital fixation of the stapes. Stapedectomy was performed on 10 ears for this condition. No perilymph gushers or significant sensorineural hearing loss were encountered. All but one case resulted in closure of the air-bone gap at the speech frequencies to within 20 dB, with the average air-bone gap being 11.2 dB. Minor congenital abnormalities of the stapes superstructure were seen in three cases. A review of case reports in the literature would seem to indicate that the perilymph gusher in association with surgical manipulation of the congenitally fixed stapes occurs almost exclusively in males, and that the 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.