Sensorineural Hearing Loss in Seropositive Neuromyelitis Optica Spectrum Disorder and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder

Background. Acute sensorineural hearing loss (SNHL) is a rare development in the central nervous system (CNS) demyelinating diseases such as aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods. We retrospectively reviewed consecutive patients with seropositive NMOSD or MOGAD in the CNS Inflammatory and Demyelinating Disease Registry at Samsung Medical Center from January 2015 to December 2020. After the medical chart review, the demographic data and the results of brain magnetic resonance imaging (MRI) and audiometry of patients with hearing loss were collected. Results. Five patients (NMOSD, n=3; MOGAD, n=2) were diagnosed with SNHL, two developed SNHL before the first core clinical symptom, and another two patients who underwent brain MRI at the timing of hearing loss showed lesions. Only three patients received high-dose steroids; however, hearing loss did not improve in any patients. Conclusion. SNHL was observed in a small number of patients with seropositive NMOSD and MOGAD; however, it could be underrecognized. Further large cohort prospective studies are helpful to elucidate the clinical implication of SNHL in NMOSD and MOGAD.