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S. M. Ronan, A. Aizman, R. E. Hackel, S. J. Saxe; Multiple Evanescent White Dot Syndrome in a Patient With Multifocal Choroiditis and Unilateral Hearing Loss. Invest. Ophthalmol. Vis. Sci. 2008;49(13):944.
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The White Dot Syndromes are a group of rare inflammatory disorders of the retina, retinal pigment epithelium and choroid that include Multiple Evanescent White Dot Syndrome, Multifocal Choroiditis, Acute Posterior Multifocal Placoid Pigment Epitheliopathy, Serpiginous Choroidopathy, Birdshot Retinochoroidopathy and Punctate Inner Choroidopathy. Here we report a unique case of a patient presenting with Multiple Evanescent White Dot Syndrome evidence for previous Multifocal Choroiditis with a recent history of unilateral hearing loss.
A thorough review of the White Dot Syndromes and their relationship to hearing loss was conducted. The patient records together with ancillary studies to include fluorescein angiography (FA), Humphrey visual fields, indocyanine green angiography (ICG) and audiograms were also compiled and reviewed.
An otherwise healthy 40 year old woman presenting with decreased visual acuity, multiple outer retinal white lesions in the macula and paramacular area, a granular appearance of the fovea and an enlarged blind spot. FA and ICG demonstrated findings consistent with a diagnosis of Multiple Evanescent White Dot Syndrome. There was also evidence on exam and a history consistent with previous Multifocal Choroiditis with midperipheral "punched out" chorioretinal scars in a curvilinear pattern. In addition, the patient was found to have a recent history of profound and persistent unilateral sensorineural hearing loss document by audiography.
There have been rare reports of sensorineural hearing loss associated with the White Dote Syndromes. Specifically, both Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Birdshot Retinochoroidopathy have been reported with sensorineural hearing loss. To our knowledge this is the first report of an immune-competent patient with unilateral hearing loss, evidence of Multifocal Choroiditis and a subsequent diagnosis of Multiple Evanescent White Dot Syndrome. The finding of these three rare conditions in a single otherwise healthy patient could be explained by a common underlying susceptibility to a post-infectious inflammatory state or autoimmune process. Due to the rarity of such cases, no definitive conclusions may be drawn, but require ongoing consideration and further study.
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