Perilymphatic gusher in osteogenesis imperfecta

Perilymphatic gusher is a rare event during stapes surgery that can have dramatic consequences on hearing outcomes. While gusher has been reported in X-linked deafness and otosclerosis, this is the first report of perilymphatic gusher during stapedotomy in osteogenesis imperfecta. A 15-year-old female, with no hearing or vestibular symptoms aside from mixed hearing loss, underwent endoscopic stapedotomy when a perilymphatic gusher was unexpectedly encountered intraoperatively. Prompt insertion of the stapes prosthesis and packing with fascial and cartilage graft were used to manage the gusher. At 7-month follow-up, the patient reported improved hearing and denied dizziness; audiogram showed complete closure of air-bone gap. Preoperative computed tomography scan did not reveal obvious radiologic signs to predict the gusher. Perilymphatic gusher should thus be recognized by the otologic surgeon as a possible complication during stapes surgery in osteogenesis imperfecta despite normal imaging findings. Patients and their families should be counseled of this risk appropriately.