We performed a retrospective study of the medical records of 35 patients with autoimmune sensorineural hearing loss (parameters: audiogram documenting a sensorineural hearing deficit in one or both ears and a positive result on Western blot assay with 68-kd band for antibodies to inner ear antigens) and examined precontrast and postcontrast T1-weighted axial and coronal MRI scans of the inner ear. 9 - 11 To our knowledge, the frequency of such enhancement has not been studied in a larger population of patients diagnosed as having autoimmune hearing loss confirmed by Western blot assay, nor has it been determined whether enhancement correlates with improvement of auditory function after a course of therapy. The total number of patients within each of these reports ranged from 6 to 12 patients, not all of whom had a positive result on Western blot assay for antibodies to the 68-kd inner ear antigen. 7 - 13 Some authors have described incidental cases of enhancement of inner ear structures on postcontrast MRI studies in patients diagnosed with autoimmune sensorineural hearing loss. Investigators have shown this modality to be excellent for evaluating the inner ear for signs of inflammation and neoplasm, especially when comparing precontrast and postcontrast T1-weighted studies using gadolinium–diethylenetriamine pentaacetic acid. Magnetic resonance imaging (MRI) has also been used to evaluate the inner ear for signs of autoimmune cochleopathy. 1 - 3 However, the conclusions of completed studies are controversial, and many authors do not accept the autoimmune model of hearing loss or the tests ordered to diagnose it. Among these, the presence of 68,000-d (68-kd) serum antibodies to an inner ear antigen that is detectable on Western blot assay has been shown to correlate with autoimmune disease activity and to predict response to treatment in patients with sensorineural hearing loss. Many clinical tests and examinations have been proposed to aid in the diagnosis of this disorder. However, that finding was thought to represent postoperative inflammatory change.Ĭonclusion No correlation was found between the presence of antibodies to inner ear antigen in patients with hearing loss and cochlear enhancement on MRI scans.ĪUTOIMMUNE sensorineural hearing loss has been recognized as a cause of sensorineural hearing loss. Results One patient demonstrated +2 cochlear enhancement. Main Outcome Measures Frequency and intensity of cochlear enhancement on MRI scans. Patients Thirty-five patients with autoimmune sensorineural hearing loss defined by audiograms documenting a sensorineural hearing deficit in one or both ears and the presence of an anti–inner ear antibody (68-kd band) in serum samples who underwent precontrast and postcontrast T1-weighted axial and coronal MRI scans of the inner ear, which were performed concurrently with the hearing loss. Study Design Retrospective case review with reexamination of MRI scans. Objective To evaluate magnetic resonance imaging (MRI) scans for enhancement of inner ear structures of patients with sensorineural hearing loss and documented antibodies to the 68-kd inner ear antigen.
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