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Updated: Aug 19, 2026

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Progressive cone dystrophy and sensorineural hearing loss
J A Witters1, J De Zaeytijd, M Leys
1Department of Ophthalmology, Ghent University Hospital, Ghent, Belgium.
Abstract:
A 39-year old man presented 13 years ago with a history of progressive loss of vision and photophobia. A full ophthalmological and ENT work-up during several years of follow-up, including psychophysical as well as electrophysiological tests, revealed a progressive cone dystrophy in combination with sensorineural hearing loss. His younger sister presented with very similar features and underwent the same work-up. A novel syndrome of progressive cone dystrophy and sensorineural hearing loss is described in both siblings. Both also suffered from non-ocular disease possibly related to ciliary dysfunction. The condition is likely to be inherited as an autosomal recessive trait.
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