Histopathological and ultrastructural analysis of vestibular endorgans in Meniere's disease reveals basement membrane pathology

Abstract

Abstract Background We report the systematic analysis of the ultrastructural and cytological histopathology of vestibular endorgans acquired from labyrinthectomy in Meniere's disease. Methods 17 subjects with intractable Meniere's disease and ipsilateral non-serviceable hearing presenting to the Neurotology Clinic from 1997 to 2006 who chose ablative labyrinthectomy (average age = 62 years; range 29–83 years) participated. The average duration of symptoms prior to surgery was 7 years (range 1–20 years). Results Nearly all vestibular endorgans demonstrated varying degrees of degeneration. A monolayer of epithelial cells occurred significantly more frequently in the horizontal cristae (12/13 = 92%) (p < 0.001), the superior cristae (5/5 = 100%) (p < 0.005), the posterior cristae (2/2) compared with the utricular maculae (4/17 = 24%). Basement membrane (BM) thickening was more common in all of the cristae ampullares (18 out of 20) than the utricular maculae. Although only four saccular maculae were obtained, 3 out of 4 exhibited BM thickening and monolayer degeneration. Monolayer degeneration was highly significantly correlated with the presence of BM thickening (p < 0.001). Other degenerative changes noted equally among the five vestibular endorgans which were not significantly correlated with BM thickening or monolayer degeneration included hair cell vacuolization and stereocilia loss, microvesicles in the supporting cells, and increased stromal intercellular spaces. Transmission electron microscopy demonstrated disorganization of the BM collagen-like fibrils, and normal ultrastructural morphology of the nerve terminals and myelinated fibers. Stromal fibroblasts and endothelial cells of stromal blood vessels demonstrated vacuolization, and stromal perivascular BMs were also thickened. Conclusion Systematic histopathological analysis of the vestibular endorgans from Meniere's disease demonstrated neuroepithelial degeneration which was highly correlated with an associated BM thickening. Other findings included hair cell and supporting cell microvessicles, increased intercellular clear spaces in the stroma, and endothelial cell vacuolization and stromal perivascular BM thickening.