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.

In this report, we present a case of rapid otic capsule obliteration within an exceedingly short timeframe in the setting of Chronic Suppurative Otitis Media (CSOM) in an immunocompromised pediatric patient with Down Syndrome. Following maximal therapy for a right sided cholesteatoma, the patient developed a multi-drug resistant infection that cause CSOM, which within 6 weeks progressed to complete obliteration of the right cochlea and otic capsule. The possibility of congenital temporal bone microscopic dehiscence allowing infection propagation cannot be excluded. Nonetheless, this case highlights the importance of appreciating how quickly chronic middle ear disease can progress to involve the labyrinth and cause intracranial complications, even with adequate concurrent medical therapy in the form of antibiotics and surgical therapy. A greater awareness as physicians should be made on management of refractory chronic middle ear disease to better treat their potential complications, which is made apparent in this case report. Keywords: Otic capsule; Chronic suppurative otitis media; Otic capsule; Tympanomastoidectomy; Multi-drug resistance. Abbreviations: CSOM: Chronic Suppurative Otitis Media; MDR: Multi-Drug Resistant; CT: Computed Tomography; MRI: Magnetic Resonance Imaging; ID: Infectious Disease.