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Common Variable Immunodeficiency (CVID) is the most frequent form of PIDs and is characterized by increased susceptibility to infections, autoimmune phaenomea and lymphomas. Besides these clinical manifestations, affected patients tend to develop progressive neurodegeneration, such as sensorineural hearing loss (SNHL), which we first described in CVID. Therefore we propose to study from a molecular/genetic and immunologic point of view more than 70 affected patients in order to define the prevalence of SNHL in CVID, correlate SNHL with B and T cell activations status, and try to identify, if it may exist, a genotype-phenotype correlation between SNHL and candidate genes (such as XBP1, Blimp1 and others)
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