Sensorineural hearing loss in OPA1-linked disorders

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Sensorineural hearing loss in OPA1-linked disorders.

Auteur Leruez, Stéphanie [1], Milea, Dan [2], Defoort-Dhellemmes, Sabine [3], Colin, E. [4], Crochet, M. [5], Procaccio, Vincent [6], Ferré, Marc [7], Lamblin, J. [8], Drouin-Garraud, V. [9], Vincent-Delorme, C. [10], Lenaers, Guy [11], Hamel, Christian [12], Blanchet, Catherine [13], Juul, G. [14], Larsen, M. [15], Verny, Christophe [16], Reynier, Pascal [17], Amati-Bonneau, Patrizia [18], Bon...

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Reply: Sensorineural hearing loss in OPA1-linked disorders

Sir, Autosomal dominant optic atrophy (DOA) is the most common inherited optic neuropathy seen in clinical practice, and in 460% of cases, the underlying genetic defect is a pathogenic mutation within the OPA1 gene (Ferre et al., 2009; Yu-Wai-Man et al., 2010a). The pathological hallmark of this disorder is the preferential loss of retinal ganglion cells and patients typically present with bila...

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LETTER TO THE EDITOR Sensorineural hearing loss in OPA1-linked disorders

1 Département d’Ophtalmologie, CHU Angers, Université d’Angers, 49100, Angers, France 2 CNRS UMR 6214, 49100, Angers, France 3 INSERM UMR 1083, 49100, Angers, France 4 LUNAM Université, Université d’Angers, 49100, Angers, France 5 Faculty of Health Sciences, University of Copenhagen, DK-1165 Copenhagen, Denmark 6 Département d’Ophtalmologie, CHU Lille, Université de Lille, 59037 Cedex, Lille, F...

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Studying VEMP in Sudden Sensorineural Hearing Loss

Introduction: Sudden sensorineural hearing loss (SSNHL) has a prevalence of 10 in 100,000. Viral infections, vascular obstruction and rupture of the intracochlear membranes are supposed to be its most common etiologies. About 40% of patients experience vertigo or imbalance. The VEMP (vestibular evoked myogenic potentials) test is a known approach for detailed study of the labyrinth. The advant...

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Pseudoexfoliation Syndrome and Sensorineural Hearing Loss

Introduction: Pseudoexfoliation syndrome (PXS) occurs due to the deposition of extracellular fibrillar materials on the anterior chamber of the eye. This syndrome has been considered to be part of a systemic disease with the potential involvement of the inner ear called sensoroneural hearing loss (SNHL).  In this study, we aimed on evaluating SNHL within PXS patients in Iran to compare them wi...

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ژورنال

عنوان ژورنال: Brain

سال: 2013

ISSN: 1460-2156,0006-8950

DOI: 10.1093/brain/aws340