OBJECTIVE: To describe a rare case of pneumolabyrinth (PNL) and pneumocephalus (PNC) due to otogenic meningitis in a patient with superior canal dehiscence (SCD) resulting in profound sensorineural hearing loss (SNHL), semicircular canals impairment but preservation of SCD-related enhanced otolith function. PATIENT: A 65-year-old woman with otogenic meningitis. INTERVENTION: Temporal bone high-resolution computed tomography (CT) scans, brain-magnetic resonance imaging, audiometry, bedside examination, video-head impulse test, and vestibular-evoked myogenic potentials (VEMPs). MAIN OUTCOME MEASURES: Enhanced otolith function despite canal and cochlear loss. RESULTS: The patient developed right profound SNHL and acute labyrinthitis. Imaging showed middle ear inflammatory tissue, right PNL and PNC despite lack of bony fractures. Bilateral SCD and tegmen dehiscence were detected. The patient underwent mastoidectomy, drainage of effusion, and surgical repair of tegmen dehiscence. Exploratory tympanotomy was uneventful. She was treated with intravenous antibiotics and dexamethasone for 3 weeks with improvement of general condition. At 3-weeks follow-up, right profound SNHL persisted with global hypofunction for ipsilateral semicircular canals and selective impairment for left superior canal activity at video-head impulse test. Surprisingly, both cervical and ocular-VEMPs exhibited bilaterally abnormal amplitudes and reduced thresholds, consistently with preserved SCD-related macular hypersensitivity to sounds even on the affected side. CONCLUSIONS: This case report exhibits a unique clinical scenario as it offers interesting insights concerning PNL aetiology despite lack of either bony fractures or barotrauma and PNC likely conveyed intracranially by SCD. Moreover, it provides an unusual pattern of functional dissociation among inner-ear receptors showing enhanced otolith function despite severe labyrinthine damage.