Otology & Neurotology 36:e134Ye135 Ó 2014, Otology & Neurotology, Inc.
Imaging Case of the Month
Sudden Sensorineural Hearing Loss Associated With Intralabyrinthine Hemorrhage Sergio Santino Cervantes and David M. Barrs Mayo Clinic Arizona, Phoenix, Arizona, U.S.A.
A 71-year-old man with a history of chronic myelomonocytic leukemia (CMML) presented with sudden-onset complete unilateral hearing loss, tinnitus, and vertigo. The
initial audiogram revealed left-sided moderate-severe sensorineural hearing loss (SNHL). Despite a 14-day 60-mg prednisone taper, the patient’s symptoms persisted, and an ensuing
FIG. 1. Magnetic resonance image. A, Axial postcontrast T1W image with fat saturation showing slight labyrinthine hyperintensity (arrow). B, Axial FIESTA image shows symmetric labyrinthine signal. C, Axial T2W image demonstrating symmetric labyrinthine uptake. D, Axial MRI T2 FLAIR image demonstrates left labyrinthine increased signal consistent with ILH (arrow).
audiogram confirmed a profound left SNHL. He denied any personal history or family history of otologic disease, autoimmune disease, bleeding disorders, or otologic or neurologic surgery.
Address correspondence and reprint requests to David M. Barrs, M.D., 1. 5777 E. Mayo Blvd, Phoenix, AZ 85054, U.S.A.; E-mail: [email protected] The authors disclose no conflicts of interest.
e134
Copyright © 2015 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
SUDDEN SNHL ASSOCIATED WITH ILH
e135
FIG. 2. A, Coronal MRI T1W image demonstrating left labyrinthine hyperintensity (arrow). B, Coronal MRI postcontrast T1W image showing no significant change in the left labyrinth.
Physical examination was unremarkable. Videonystagmography and vestibular-evoked myogenic potentials revealed a severe loss of vestibular function with incomplete compensation. The only abnormal laboratory value was leukocytosis at 13.5, consistent with his CMML diagnosis. His medication regimen did include aspirin 81 mg. Magnetic resonance imaging (MRI) is an established diagnostic modality in the evaluation of a patient with sudden SNHL. Although a distinct pathology can only be identified in 10% of the patients, the differential diagnosis includes infection, trauma, neoplastic, immunologic, toxic, vascular compromise, and neurologic and metabolic causes. An MRI of the brain and internal auditory canals was obtained 2 weeks after completing prednisone. Although an outside facility reported the scan as normal, radiologic review noted increased signal intensity on T1-weighted (T1W) and T2 fluid-attenuated inversion recovery (FLAIR) images in the vestibule and lateral and superior semicircular canals (Figs. 1, 2). Plain T2-weighted (T2W) images were symmetric to the normal labyrinth. No enhancement was appreciated or change after fat suppression techniques. A diagnosis of subacute/chronic intralabyrinthine hemorrhage (ILH) was rendered. At the latest audiogram, the patient’s hearing loss remained profound. Improvements in MRI allow for better characterization of more subtle inner ear pathologies such as ILH (1). Normally, perilymph and endolymph are isointense to cerebrospinal fluid on all MRI sequences. Fat, proteinaceous materials, and methemoglobinemia will all cause hyperintensity on T1W, but fat can be distinguished with suppression. Intralabyrinthine hemorrhage is characterized on MRI by a high signal intensity on T1W and T2 FLAIR imaging yet will not enhance with gadolinium. Plain T2W images are not helpful because signal intensity can be variable. Three-dimensional FLAIR technique is sensitive in detecting high concentrations of protein. Berrettini et al. (2) and Yoshida et al. (3) used three-dimensional FLAIR to evaluate
more than 70 patients with sudden SNHL and found that greater than 50% showed labyrinthine signal accumulation. Berrettini hypothesizes that this technique, in conjunction with T1W and T2W series, can differentiate ILH from acute inflammation and disruption of the blood-labyrinth barrier (2). Labyrinthine schwannomas differ from ILH on MRI by marked enhancement postcontrast and, if ruled out, a second scan can be avoided. If tumor cannot be definitively excluded, follow-up imaging should be delayed for a minimum of 6 months to allow evacuation of lingering erythrocyte degradation products. Yoshida et al. (3) felt that a high labyrinthine signal intensity correlated with worse hearing outcome. Berrettini, however, found no such hearing correlation but did associate vertigo with a high signal intensity. In summary, labyrinthine hemorrhage can readily be diagnosed with advancing MRI techniques. Intralabyrinthine hemorrhage has been linked to CMML, sickle cell disease, systemic lupus erythematosus, endolymphatic sac tumors, Von HippelYLindau syndrome, and the use of anticoagulants. Although retrocochlear lesions must be ruled out, modern MRI scans can also detect the presence of ILH. Three D-FLAIR imaging is most sensitive to ILH, yet T1W imaging is necessary to confirm hemorrhage. Imaging may be important for hearing prognosis if future research can correlate MRI findings with hearing outcomes.
REFERENCES 1. Sabatini PR, Kurtz JW. Radiology quiz case 1. Intralabyrinthine hemorrhage. Arch Otolaryngol Head Neck Surg 2009;135:612Y4. 2. Berrettini S, Seccia V, Fortunato S. Analysis of the 3-dimensional fluidattenuated inversion-recovery (3D-FLAIR) sequence in idiopathic sudden sensorineural hearing loss. JAMA Otolaryngol Head Neck Surg 2013; 139:456Y64. 3. Yoshida T, Sugiura M, Naganawa S. Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging findings and prognosis in sudden sensorineural hearing loss. Laryngoscope 2008;118:1433Y7.
Otology & Neurotology, Vol. 36, No. 8, 2014
Copyright © 2015 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
Imaging Case of the Month
Sudden Sensorineural Hearing Loss Associated With Intralabyrinthine Hemorrhage Sergio Santino Cervantes and David M. Barrs Mayo Clinic Arizona, Phoenix, Arizona, U.S.A.
A 71-year-old man with a history of chronic myelomonocytic leukemia (CMML) presented with sudden-onset complete unilateral hearing loss, tinnitus, and vertigo. The
initial audiogram revealed left-sided moderate-severe sensorineural hearing loss (SNHL). Despite a 14-day 60-mg prednisone taper, the patient’s symptoms persisted, and an ensuing
FIG. 1. Magnetic resonance image. A, Axial postcontrast T1W image with fat saturation showing slight labyrinthine hyperintensity (arrow). B, Axial FIESTA image shows symmetric labyrinthine signal. C, Axial T2W image demonstrating symmetric labyrinthine uptake. D, Axial MRI T2 FLAIR image demonstrates left labyrinthine increased signal consistent with ILH (arrow).
audiogram confirmed a profound left SNHL. He denied any personal history or family history of otologic disease, autoimmune disease, bleeding disorders, or otologic or neurologic surgery.
Address correspondence and reprint requests to David M. Barrs, M.D., 1. 5777 E. Mayo Blvd, Phoenix, AZ 85054, U.S.A.; E-mail: [email protected] The authors disclose no conflicts of interest.
e134
Copyright © 2015 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
SUDDEN SNHL ASSOCIATED WITH ILH
e135
FIG. 2. A, Coronal MRI T1W image demonstrating left labyrinthine hyperintensity (arrow). B, Coronal MRI postcontrast T1W image showing no significant change in the left labyrinth.
Physical examination was unremarkable. Videonystagmography and vestibular-evoked myogenic potentials revealed a severe loss of vestibular function with incomplete compensation. The only abnormal laboratory value was leukocytosis at 13.5, consistent with his CMML diagnosis. His medication regimen did include aspirin 81 mg. Magnetic resonance imaging (MRI) is an established diagnostic modality in the evaluation of a patient with sudden SNHL. Although a distinct pathology can only be identified in 10% of the patients, the differential diagnosis includes infection, trauma, neoplastic, immunologic, toxic, vascular compromise, and neurologic and metabolic causes. An MRI of the brain and internal auditory canals was obtained 2 weeks after completing prednisone. Although an outside facility reported the scan as normal, radiologic review noted increased signal intensity on T1-weighted (T1W) and T2 fluid-attenuated inversion recovery (FLAIR) images in the vestibule and lateral and superior semicircular canals (Figs. 1, 2). Plain T2-weighted (T2W) images were symmetric to the normal labyrinth. No enhancement was appreciated or change after fat suppression techniques. A diagnosis of subacute/chronic intralabyrinthine hemorrhage (ILH) was rendered. At the latest audiogram, the patient’s hearing loss remained profound. Improvements in MRI allow for better characterization of more subtle inner ear pathologies such as ILH (1). Normally, perilymph and endolymph are isointense to cerebrospinal fluid on all MRI sequences. Fat, proteinaceous materials, and methemoglobinemia will all cause hyperintensity on T1W, but fat can be distinguished with suppression. Intralabyrinthine hemorrhage is characterized on MRI by a high signal intensity on T1W and T2 FLAIR imaging yet will not enhance with gadolinium. Plain T2W images are not helpful because signal intensity can be variable. Three-dimensional FLAIR technique is sensitive in detecting high concentrations of protein. Berrettini et al. (2) and Yoshida et al. (3) used three-dimensional FLAIR to evaluate
more than 70 patients with sudden SNHL and found that greater than 50% showed labyrinthine signal accumulation. Berrettini hypothesizes that this technique, in conjunction with T1W and T2W series, can differentiate ILH from acute inflammation and disruption of the blood-labyrinth barrier (2). Labyrinthine schwannomas differ from ILH on MRI by marked enhancement postcontrast and, if ruled out, a second scan can be avoided. If tumor cannot be definitively excluded, follow-up imaging should be delayed for a minimum of 6 months to allow evacuation of lingering erythrocyte degradation products. Yoshida et al. (3) felt that a high labyrinthine signal intensity correlated with worse hearing outcome. Berrettini, however, found no such hearing correlation but did associate vertigo with a high signal intensity. In summary, labyrinthine hemorrhage can readily be diagnosed with advancing MRI techniques. Intralabyrinthine hemorrhage has been linked to CMML, sickle cell disease, systemic lupus erythematosus, endolymphatic sac tumors, Von HippelYLindau syndrome, and the use of anticoagulants. Although retrocochlear lesions must be ruled out, modern MRI scans can also detect the presence of ILH. Three D-FLAIR imaging is most sensitive to ILH, yet T1W imaging is necessary to confirm hemorrhage. Imaging may be important for hearing prognosis if future research can correlate MRI findings with hearing outcomes.
REFERENCES 1. Sabatini PR, Kurtz JW. Radiology quiz case 1. Intralabyrinthine hemorrhage. Arch Otolaryngol Head Neck Surg 2009;135:612Y4. 2. Berrettini S, Seccia V, Fortunato S. Analysis of the 3-dimensional fluidattenuated inversion-recovery (3D-FLAIR) sequence in idiopathic sudden sensorineural hearing loss. JAMA Otolaryngol Head Neck Surg 2013; 139:456Y64. 3. Yoshida T, Sugiura M, Naganawa S. Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging findings and prognosis in sudden sensorineural hearing loss. Laryngoscope 2008;118:1433Y7.
Otology & Neurotology, Vol. 36, No. 8, 2014
Copyright © 2015 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
