Case series: Hearing loss in neuromyelitis optica spectrum disorders.

ABSTRACT

BACKGROUND: Aquaporin 4 (AQP4)- and myelin oligodendrocyte glycoprotein (MOG)-associated neuromyelitis optica spectrum disorders (NMOSD) are thought to primarily affect the central nervous system (CNS). However, emerging evidence suggests that there are extra-CNS manifestations of NMOSD, including myopathies, gastrointestinal dysfunction, renal involvement and adverse pregnancy outcomes.1 METHODS: Three patients who reported hearing loss during a NMOSD relapse were identified through a retrospective case review. RESULTS: In this article, we discuss two AQP4-IgG positive NMOSD cases, each presenting with conductive and sensorineural hearing loss, and a case of MOG-IgG-associated NMOSD presenting with sensorineural hearing loss. CONCLUSION: Hearing loss may be present as a relapse in patients with NMOSD. Early recognition and timely treatment are essential to prevent irreversible hearing loss.