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Stapedectomy for congenital fixation of the stapes
J L Dornhoffer1, J Helms, D H Hoehmann
1Department of Otolaryngology, Julius-Maximilians University, Wuerzburg, Germany.
Insights
Stapedectomy in children with congenital stapes fixation can be safe and effective, avoiding perilymph gusher complications. This surgery successfully closed air-bone gaps, improving hearing outcomes in pediatric patients.
Area of Science:
- Otolaryngology
- Pediatric Otology
- Neurosurgery
Background:
- Profuse cerebrospinal fluid leakage, or perilymph gusher, is a known risk during stapedectomy for congenital stapes fixation in children.
- The exact cause of perilymph gusher and its link to congenital stapes fixation remain unclear, leading many surgeons to avoid the procedure.
Purpose of the Study:
- To investigate the preoperative characteristics and postoperative hearing results in children who underwent stapedectomy for congenital stapes fixation.
- To evaluate the incidence of perilymph gusher and significant sensorineural hearing loss in this pediatric population.
Main Methods:
- A retrospective analysis of 10 pediatric cases undergoing stapedectomy for congenital stapes fixation.
- Review of literature case reports to identify risk factors and potential pathophysiologic mechanisms for perilymph gusher.
Main Results:
- No instances of perilymph gusher or significant sensorineural hearing loss were observed in the studied cases.
- Successful closure of the air-bone gap to within 20 dB was achieved in 9 out of 10 ears, with an average air-bone gap of 11.2 dB.
- Minor stapes superstructure abnormalities were noted in three cases.
Conclusions:
- Stapedectomy for congenital stapes fixation in children can yield favorable hearing results without the feared complication of perilymph gusher.
- Potential risk factors for perilymph gusher include male gender, internal auditory canal defects, and pre-existing sensorineural hearing loss.
Abstract:
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.