The aim of this work is to summarize the main advances on the pathophysiology, diagnosis, and treatment of Meniere's disease (MD). Different immune responses to biotic stimuli may trigger MD, with subgroups identified based on cytokine and genetic profile, suggesting potential benefits from immune therapy, including antiallergic medication. Genetic and epigenetic research, along with imaging studies, reveal the complexity of MD, involving inflammation, immunity, and metabolic processes. Advanced imaging techniques define specific temporal bone features and endolymphatic hydrops, while machine learning models enhance diagnostic accuracy through clinical and laboratory data analysis. Differentiating MD from vestibular migraine remains challenging due to overlapping symptoms, but combining vestibular tests, audiological assessments, and biomarkers like cytokines and chemokines shows promise. Pharmacological treatments such as betahistine or corticosteroids show varying effectiveness and require further research according to immune subgroups. Surgical options like endolymphatic sac decompression, semicircular canal occlusion and labyrinthectomy are restricted to intractable cases. Research into MD aims to improve diagnosis and treatment through genetic, immunological, and advanced imaging studies. Current treatments include pharmacological, intratympanic, and surgical interventions, but current research supports a personalized approach based on clinical and molecular re-definition of patient subgroups.