Tehran University of Medical Sciences Auditory and Vestibular Research 2423-480X 0 0 2026 05 06 1532 1532 Özden Savaş Asst. Prof. M.D. Kıvanç Korkmaz M.D. Süleyman Men Prof. M.D. Enis Güneri Prof. M.D. 2025 12 29 2026 05 06 Background: Spontaneous intracranial hypotension is a recognized clinical entity caused by spinal cerebrospinal fluid leakage and is classically characterized by orthostatic headache. However, some patients initially present with audiovestibular symptoms that may mimic primary inner ear disorders and lead to diagnostic delay. Recognition of this atypical presentation is essential to avoid misdiagnosis and to facilitate timely neuroimaging and appropriate management. The Cases: Two patients with spontaneous intracranial hypotension are described, in whom tinnitus, sound sensitivity, aural fullness, and dizziness constituted the predominant initial manifestations. A cerebrospinal fluid–venous fistula at the level of the tenth thoracic vertebra was identified using computed tomography myelography, and symptoms resolved following transvenous embolization after unsuccessful epidural blood patch therapy. In another patient, no definite site of cerebrospinal fluid leakage could be localized, and conservative management was preferred. A review of the literature is also provided, focusing on the proposed pathophysiological relationship between reduced cerebrospinal fluid pressure and secondary endolymphatic hydrops. Conclusion: Spontaneous intracranial hypotension should be considered in the differential diagnosis of patients presenting with hydrops-like audiovestibular symptoms, even in the absence of headache. Early recognition and appropriate neuroimaging are essential for accurate diagnosis and optimal management. https://avr.tums.ac.ir/index.php/avr/article/view/1532