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Armin Mohi, Mahdy Ranjbar, Salvatore Grisanti, Henning Frenzel, Martin Rudolf; First description of a melanoma-associated paraneoplastic syndrome with retinopathy and cochleopathy. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):5349.
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© ARVO (1962-2015); The Authors (2016-present)
Melanoma-associated retinopathy (MAR) is a known paraneoplastic syndrome in some patients with cutaneous malignant melanoma. It is characterized by the presence of serum auto-antibodies (IgG) against retinal proteins, affecting retinal photoreceptor function and leading to retinal damage. While a reaction of melanoma-associated auto-antibodies against various neuronal proteins is already known, an association with symptoms of other sensory organs has not been published yet. In this case-report we are able to demonstrate, that a symptomatic cochleopathy due to paraneoplastic serum auto-antibodies can be present in patients with cutaneous malignant melanoma and MAR.
A 60 year old women with cutaneous malignant melanoma and visual affection was presented in our hospital for further diagnostics and treatment. She reported flickering photopsias and night-blindness. We were able to detect a reduction of visual acuity, a distinct loss of the visual field and reduced b-waves in the ERG. MAR-auto-antibodies were detected in an independent reference-centre. An advanced staging examination revealed a metastatic recurrence of the melanoma. Noteworthy was an early development of a tinnitus. We initiated an immune-suppressive therapy with prednisolone, performed weekly clinical examinations and took blood samples in a 14 days interval. The blood samples were used for indirect-immune-fluorescence on macaque retina and cochlea sections, to detect auto-antibodies.
We were able to observe a quick improvement of BCVA and field of view after one month of prednisolone treatment, while the b-waves in the ERG were still reduced. The tinnitus remained unchanged. A cerebral MRI showed no affection of the auditory pathway as an explanation for the tinnitus. The indirect-immune-fluorescence showed a specific pattern, staining the cell-bodies of photoreceptors and hair-cells. Under therapy this pattern showed a reduction of intensity. Controls with immune sera of healthy individuals showed no staining at all.
To our knowledge, we demonstrated for the first time the presence of specific auto-antibodies against hair cells of the cochlea which was associated with MAR. Together with the tinnitus it is the first description of symptomatic melanoma-associated cochleopathy (MAC).
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