![]() ![]() Most cases are sporadic, late-onset (3rd-4th decade of life) and present monaurally. Meniere’s disease (MD (MIM 156000)) is a chronic disease, with an incidence of about 1 in 2000 in Caucasians ( Morrison et al., 2009), characterised by episodes of vertigo, low-to-middle-frequency sensorineural hearing loss (SNHL), tinnitus and aural fullness ( Lopez-Escamez et al., 2015 Lopez-Escamez et al., 2017). ![]() Indeed, noise-induced hearing loss (NIHL) and age-related hearing loss (ARHL) may result from acute and chronic cellular stress (respectively) that eventually overwhelm the homeostatic repair ( Ohlemiller, 2006). The wide-spread, and multi-factorial nature of hearing disorders exposes complex vulnerabilities on the level of the specialised mechanosensory processes, which support the ear’s function throughout life, including extensive mechanisms of auditory homeostasis. It includes infections ( Cohen et al., 2014), ototoxic drugs ( Laurell, 2019), noise trauma ( Le et al., 2017), ageing ( Davis et al., 2016) and genetic factors ( Korver et al., 2017). The aetiology of HL and inner ear disorders is diverse. Hearing loss (HL) affects about 5% of the population worldwide ( World Health Organization, 2020). ![]() These results have important implications for the human condition, and notably, we note that DTNA is expressed in equivalent cells of the mammalian inner ear. Their specific locations suggest that they form part of support cell contacts, thereby helping to maintain the integrity of the hemolymph-neuron diffusion barrier, which is equivalent to a blood-brain barrier. Dyb and another component of the dystrophin-glycoprotein complex (DGC), Dystrophin ( Dys), are expressed in support cells within JO. We show that mutation, or knockdown, of the DTNA orthologue in Drosophila, Dystrobrevin ( Dyb), results in defective proprioception and impaired function of Johnston’s Organ (JO), the fly’s equivalent of the inner ear. Evidence from epidemiology and Whole Exome Sequencing (WES) suggests a genetic susceptibility involving multiple genes, including α-Dystrobrevin ( DTNA). Meniere’s disease (MD) is an inner ear disorder characterised by recurrent vertigo attacks associated with sensorineural hearing loss and tinnitus. 3Ear Institute, University College London, London, United Kingdom.2Division of Functional Genetics and Development, The Royal Dick School of Veterinary Sciences, The Roslin Institute, The University of Edinburgh, Edinburgh, United Kingdom.1Biomedical Sciences: Centre for Discovery Brain Sciences, Edinburgh Medical School, University of Edinburgh, Edinburgh, United Kingdom. ![]()
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