Unusual presentation of an intracochlear schwannoma

In this report, the authors discuss the case of a 49-year-old male presenting with sudden onset moderately severe left-sided Sensorineural Hearing Loss (SNHL), tinnitus, left otalgia, and disequilibrium. Prior to referral to our facility, an MRI was obtained which demonstrated non-specific asymmetric enhancement of the left cochlea that was initially suspected to be labyrinthitis. Patient was treated with valacyclovir and a prednisone burst-taper prior to referral. Despite this treatment, his left SNHL worsened, and he was noted to have left-sided peripheral weakness on Videonystagmography (VNG). Upon referral to our facility, MRI demonstrated an Intra-Cochlear Schwannoma (ICS), with possible associated hemorrhage into the cochlea. After discussion of treatment options, repeat imaging in 6-month interval demonstrated stable persistence of the lesion with no growth. The patient is currently being followed with a “Wait-andScan” regimen. Sudden unilateral SNHL is a rare presenting symptom for ICS and this case highlights not only the rarity in presentation, but also the complexity in diagnosis due to the possibility of intracochlear hemorrhage complicating the imaging work-up. Keywords: Intra-labyrinthine schwannoma; vestibular schwannoma; sensorineural hearing loss; disequilibrium. Abbreviations: SNHL: Sensorineural Hearing Loss; VNG: Videonystagmography; ICS: Intracochlear Schwannoma; MRI: Magnetic Resonance Imaging; IAC: Internal Auditory Canal; ILS Intra-Labyrinthine Schwannomas; CT: Computed Tomography; SRT: Speech Reception Threshold.