High incidence of Meniere-like symptoms in relatives of Meniere patients in the areas of Oulu University Hospital and Kainuu Central Hospital in Finland

Diagnostic criteria for Menière's disease

“…Familial MD is observed in 8%-9% of sporadic cases in populations of European descent [22,23]. It has been described in Caucasians from United Kingdom [24], Brazil [25], Sweden [26], Finland [23,27], Germany [28] and Spain [23]. Although most families described have an autosomal dominant pattern of inheritance, familial MD shows genetic heterogeneity, and mitochondrial and recessive inheritance patterns are also observed in some families [22].…”

Variable expressivity and genetic heterogeneity involving DPT and SEMA3D genes in autosomal dominant familial Meniere’s disease

“…[27][28][29] Third, several relatives in these families presented a partial syndrome with different types of SNHL, including sudden hearing loss (III: 2 in family 2) or pantonal SNHL (II: 2 in family 2) and no vestibular symptoms. These findings observed across different families with MD 12,14,15 suggests that (1) different genes can be involved in the development of the partial or complete phenotype or (2) the interaction of environmental or epigenetic factors can also determine the differences in expressivity within the phenotype. Our findings have started to define two candidate genes associated with FMD and support the hypothesis of genetic heterogeneity in FMD.…”

“…13 Most of the cases of MD are considered sporadic, but familial clustering has been reported. 12,[14][15][16] Familial MD (FMD) is observed in 8-10% of sporadic cases, 17 and FAM136A and DTNA genes have been found in a single family with incomplete penetrance. 18 Although most cases present an autosomal dominant (AD) pattern of inheritance, different patterns have been observed, 14,15 suggesting a genetic heterogeneity in FMD.…”

Genetics of presbycusis and presbystasis

“…The causes underlying Ménière's disease remain unknown, but familial association has recently been reported in two large independent cohorts in Spain 38 and Finland. 39 Most of these families showed an autosomal dominant mode of inheritance; however, recessive or mitochondrial transmission is also observed, therefore suggesting genetic heterogeneity. 8,[31][32][33] Animal/experimental data Animal studies available in this field are also scant.…”