During stapedotomy for otosclerosis, the surgeon opens the footplate and there is profuse, uncontrolled flow of perilymph that wells up repeatedly. Which underlying condition is classically associated with this 'perilymph gusher'?

• A Congenital stapes fixation due to X-linked mixed deafness (DFNX2) ✓
• B Far-advanced otosclerosis with obliterative footplate disease
• C Oval window fistula from previous trauma
• D Mondini dysplasia limited to the cochlear apex

Explanation

Perilymph gusher results from a wide or absent cribriform area at the fundus of the internal acoustic meatus allowing cerebrospinal fluid to track into the vestibule, and it is classically linked with congenital stapes fixation in X-linked stapes gusher syndrome (DFNX2, formerly DFN3). Management is immediate sealing of the oval window with tissue. Obliterative otosclerosis causes a thick footplate, not a gusher.

Reference: Scott-Brown's Essential Otorhinolaryngology, Head and Neck Surgery ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.