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Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH) was confirmed by dominant -SP/AP of the electrocochleogram (ECochG). When thalidomide and prednisolone were prescribed for the treatment of MMM, symptoms of RP -- including the inner ear dysfunction -- were ameliorated. Isosorbide, one of the osmotic diuretics commonly used for the treatment of Meniere's disease (MD) in Japan, was also effective in keeping her free from inner ear dysfunction. This is the first report to confirm the existence of EH in a patient with RP with audio-vestibular manifestations. We suppose that an immunological imbalance due to MMM, in conjunction with a specific immunogenetic background, may have played a role in the pathogenesis of RP and the formation of EH in this patient.

Structure and function of the marginal cell in the stria vascularis were studied by freeze-fracture, cytochemistry and immunohistochemistry with special regard to the ion transport of potassium. Freeze-fracture showed that marginal cells were connected by tight junctions beneath the scala media K(+)-NPPase cytochemistry showed that Na+, K(+)-ATPase was abundant on the basolateral infoldings of the marginal cell. Immunohistochemistry of a rat Isk protein, which has a property of a potassium channel, revealed that the rat Isk protein was localized at the endolymphatic surface of the marginal cell. These findings supported the 'one-pump' theory (Offner et al. Hear Res 1987; 29: 117-24).