Intracochlear Hemorrhage: A Rare Cause of Sudden Sensorineural Hearing Loss

Abstract

Inner ear hemorrhage is an extremely rare cause of sudden sensorineural hearing loss with few cases reported in the literature. We report the case of a 30-year-old male who presented with a sudden left ear hearing loss, with no tinnitus nor vertigo. The audiogram revealed a profound left sensorineural hearing loss. An MRI of the brain and internal auditory canal was performed 3 weeks after and revealed an increased signal intensity on T1-weighted (T1W) and T2 fluid-attenuated inversion recovery (FLAIR) images in the left cochlea. No other abnormalities were found, in particular no enhancement after intravenous administration of gadolinium. The CISS 3D sequence showed a signal of discreetly lower intensity in the left cochlea compared to the right one. The diagnosis of intracochlear hemorrhage was made. No improvement of the hearing loss has been noted after medical treatment and hyperbaric oxygen therapy.

Bilateral intracochlear hemorrhage: A rare onset of chronic myelogenous leukemia

“…22 Today, inner ear MRI represents gold standard for diagnosis and should be considered when sudden sensorineural hearing loss is present along with suspected or confirmed hematological disease. 1,2 Chen et al examined the inner ears of 1252 patients with sudden sensorineural hearing loss with MRI. As many as 24 patients (1.9%) were diagnosed with intralabyrinthine hemorrhage.…”

“…Intracochlear hemorrhage is one possible cause and can be diagnosed with magnetic resonance imaging (MRI). 1,2 CML is nowadays successfully treated with tyrosine kinase inhibitors (TKIs) and the medication is generally well tolerated by the patients. Rare pulmonary complications have however been described, but seldom outside Asia.…”

“…Vertigo and severe hearing loss as debut of hematological diseases like chronic myelogenous leukemia (CML) is uncommon. Intracochlear hemorrhage is one possible cause and can be diagnosed with magnetic resonance imaging (MRI) 1,2 . CML is nowadays successfully treated with tyrosine kinase inhibitors (TKIs) and the medication is generally well tolerated by the patients.…”

High Signal Intensity of the Cochlear Modiolus on Unenhanced T1-Weighted Images in Classical Infratentorial Superficial Siderosis

“…12 According to previous studies, high SI in the otic labyrinth can be caused by methemoglobin in subacute-stage hematomas (intralabyrinthine hemorrhage) in patients with sudden hearing loss and vertigo. 12,14,15 However, the signal change can be seen not only in the CM but also in various regions of the otic labyrinth, including the cochlea, vestibule, and semicircular canals. 12,14,15 Weissman et al reported that the high SI of the otic labyrinth had decreased in follow-up scans of chronicstage intralabyrinthine hemorrhages, and speculated that that finding had resulted from hematoma turnover in the labyrinth, gradually diluting or clearing the blood.…”

“…12,14,15 However, the signal change can be seen not only in the CM but also in various regions of the otic labyrinth, including the cochlea, vestibule, and semicircular canals. 12,14,15 Weissman et al reported that the high SI of the otic labyrinth had decreased in follow-up scans of chronicstage intralabyrinthine hemorrhages, and speculated that that finding had resulted from hematoma turnover in the labyrinth, gradually diluting or clearing the blood. 12 In this study, the high SI localized in the CM in cases with i-SS (Cases 1 and 3) persisted for 8 months and 7.5 years, respectively.…”