ABSTRACTThe inner ear is a critical sensory organ regulated by the immune system to maintain balance and respond to external stimuli. Immune cells like macrophages, lymphocytes, neutrophils, and natural killer cells play important roles in inner ear immunity. Inflammatory mediators, such as cytokines and chemokines, along with signaling pathways like NF‐κB, MAPK, and PI3K/AKT, regulate immune responses in the inner ear. Complement system activation and inflammatory vesicles further affect immune‐related inner ear pathology, causing damage to sensory epithelium, neurons, and blood vessels structurally. Many diseases involved in inner ear have been confirmed or speculated to be related to immune imbalance. Treatments for immune‐related inner ear diseases include corticosteroids, immunosuppressants, biological therapy agents, plasmapheresis, gene therapy, stem cell therapy, and surgical interventions like cochlear implantation. These treatments aim to regulate immune responses, reduce inflammation, and restore auditory and vestibular function. In conclusion, understanding the complex interaction between immunity and inner ear function is crucial for developing targeted therapeutic int
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