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INTRODUCTION Obtaining vestibular-evoked myogenic potentials (VEMP) by means of the vestibulocollic reflex is a readily available technique that provides an image of vestibular functioning and is useful for evaluating the pathologies that involve compromise of the anatomical pathway of the reflex. Although normal patterns do exist, responses vary at(More)
Charcot-Marie-Tooth disease type 4C (CMT4C) is a hereditary neuropathy with prominent unsteadiness. The objective of the current study is to determine whether the imbalance in CMT4C is caused only by reduced proprioceptive input or if vestibular nerve involvement is an additional factor. We selected 10 CMT4C patients and 10 age-matched and sex-matched(More)
Toll-like receptors trigger the innate immune response by activating various cell types such us macrophages and lymphocytes. We genotyped SNV of TLR3, TRL7, TLR8 and TLR10 in 863 Spanish and 150 Italian patients with Meniere’s disease (MD) and 1,013 controls by using Taqman assays. Real-Time qPCR was used to measure the expression level of TLR10 in(More)
HYPOTHESIS Immune response may influence hearing outcome in Ménière's disease (MD). BACKGROUND Major histocompatibility complex class I chain-related A (MICA) encodes a highly polymorphic stress-inducible protein, which interacts with NKGD2 receptor on the surface of NK, γδ T cells and T CD8 lymphocytes. We investigated the association of MICA gene with(More)
OBJECTIVE To analyze the associations of HLA-DRB1* and DQB1* Class II alleles in patients with bilateral Méniére's disease (MD). PATIENTS AND METHODS Eighty patients from two ethnically defined groups with definite bilateral MD, according to the diagnostic scale of the American Academy of Otolaryngology-Head and Neck Surgery, were compared with normal(More)
BACKGROUND Autoimmunity appears to be associated with the pathophysiology of Meniere's disease (MD), an inner ear disorder characterized by episodes of vertigo associated with hearing loss and tinnitus. However, the prevalence of autoimmune diseases (AD) in patients with MD has not been studied in individuals with uni or bilateral sensorineural hearing loss(More)
OBJECTIVE To investigate the sequence and correlation of symptoms of Ménière's disease (MD) depending on their order of manifestation. METHODS Descriptive, longitudinal study of the symptoms in 237 tertiary hospital patients who had been diagnosed with definite MD according to the criteria of the American Academy of Otolaryngology. Patients were followed(More)
PURPOSE Usher syndrome type I (USH1) is an autosomal recessive disorder characterized by severe-profound sensorineural hearing loss, retinitis pigmentosa, and vestibular areflexia. To date, five USH1 genes have been identified. One of these genes is Usher syndrome 1C (USH1C), which encodes a protein, harmonin, containing PDZ domains. The aim of the present(More)
BACKGROUND Autoimmune diseases with elevated circulating autoantibodies drive tissue damage and the onset of disease. The Fcγ receptors bind IgG subtypes modulating the clearance of circulating immune complexes (CIC). The inner ear damage in Ménière's disease (MD) could be mediated by an immune response driven by CIC. We examined single-nucleotide(More)
Usher syndrome (USH) is an autosomal recessive hereditary disorder characterised by congenital sensorineural hearing loss and gradual visual impairment secondary to retinitis pigmentosa (RP). The disorder is clinically and genetically heterogeneous. With regard to Usher type I (USH1), several subtypes have been described, the most frequent being USH1B(More)