Learning Objectives:

A 38-year-old woman visited our clinic with her left progressive hearing loss for 2 months. She had characteristic blue sclera and experienced frequent fractures from minor trauma in her teens. She looked normal in her appearance and stature. She did not have family history of hearing loss. Her ear drum was clear and pure tone audiogram showed left side 40 dB air-bone gap conductive hearing loss. Her right hearing was normal. Temporal bone CT revealed nothing special. Exploratory tympanotomy was performed to find stapes fixation and decided to do the stapedotomy. However, profuse perilymph gush developed when perforating the foot plate. Piston wire prosthesis was placed with struggling effort. Lumbar drain was placed right after finishing the operation. Although she had immediate post-operative dizziness, hearing loss, and tinnitus for 3 days, her hearing gradually improved and air-bone gap was closed 2 months after the surgery. Her good hearing was maintained for the 6 months on the follow-up audiogram.