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Table 1: Diagnostic Criteria for Meniere's Disease

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Table 1: Diagnostic Criteria for Meniere's Disease Table 1: Diagnostic criteria for Meniere’s disease. A) Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours. B) Low- to medium frequency hearing loss in one ear during or after one of the episodes of vertigo C) Fluctuating aural symptoms in the affected ear D) Not better accounted for by another vestibular diagnosis Familial MD patients were included only if, at least, one other relative (first or second degree) fulfilled all the criteria of definite or probable MD (Lopez-Escamez et al. 2015). Table 2: Clinical information of familial MD patients without variants in OTOG gene. Code Sex Laterality Ear Age of Relatives Headache Autoimmune affected onset with MD disease F15 Male Unilateral Left 18 Brother Tension No Headache F16 Female Unilateral Right 34 Grandmother No No F17 Male Unilateral Left 33 Sister and No No son F18 Female Unilateral Left 50 Uncle No — F19 Female Bilateral Both 69 Nephew Tension No Headache F20 Female Bilateral Both 34 Father MA No F21 Female Bilateral Both 20 Sister and No — brother F22 Female Unilateral Right 20 Mother and MO No sister-in-law F23 Female Bilateral Both 16 Mother MA Hypothyroidism F24 Female Unilateral Right 42 Mother No No F25 Female Unilateral Right 31 Mother and No No cousin F26 Male Unilateral Left 54 Father and No No brother F27 Male Unilateral Right 25 Mother No No F28 Female Unilateral Left 19 Mother Tension No headache F29 Female Bilateral Both 40 Daughter Tension Type 1 Headache Diabetes F30 Female Unilateral Right 42 Aunt No No F31 Female Bilateral Both 28 Mother No No F32 Male Bilateral Both 31 Daughter Tension No headache F33 Male Bilateral Both 49 Brother No No F34 Female Unilateral Right 40 Brother No No F35 Female Bilateral Both 42 Sister No No F36 Female Bilateral Both 62 Father Tension No headache F37 Female Bilateral Both 46 Twin sister No No F38 Male Unilateral Right 70 Sister No No F39 Female Unilateral Right 56 Father and No No Uncle F40 Female Unilateral Left 54 Sisters Migraine No F41 Male Unilateral Right 42 Mother No No F42 Female Unilateral Left 29 Mother No No F43 Female Bilateral Both 14 Father and No No Aunt F44 Female Bilateral Both 15 Father No No F45 Male Bilateral Both 60 Brother MA No F46 Female Unilateral Left 20 Mother Yes Arthritis MA, migraine with aura; MO, migraine without aura. Table 3: Clinical information of sporadic MD patients. Code Sex Laterality Ear Age of Headache Autoimmune affected onset disease S1 Female Unilateral Right 28 Tension headache No S2 Female Unilateral Right 28 Tension headache Hypothyroidism S3 Male Unilateral Right 33 Tension headache No S4 Male Bilateral Both 16 No No S5 Female Unilateral Right 27 No No S6 Female Unilateral Right 20 MA Hypothyroidism S7 Female Unilateral Left 14 Tension headache No S8 Male Bilateral Both 35 No Psoriasis S9 Female Unilateral Left 28 No No S10 Male Bilateral Both 35 No Behçet syndrome S11 Female Unilateral Left 31 No No S12 Female Unilateral Left 30 No No S13 Female Unilateral Right 27 MO No S14 Male Bilateral Both 29 No No S15 Male Bilateral Both 27 No Psoriasis S16 Female Bilateral Both 33 MO Hughes syndrome S17 Female Bilateral Both 24 MA No S18 Male Unilateral Left 35 No No S19 Female Unilateral Left 31 MA No S20 Female Bilateral Both 23 MO Hypothyroidism S21 Female Bilateral Both 35 No No S22 Female Bilateral Both 22 MO Autoimmune arthralgia S23 Male Unilateral Left 35 No No S24 Female Unilateral Left 31 No No S25 Female Unilateral Right 33 No No S26 Female Unilateral Right 32 No No S27 Male Bilateral Both 21 Tension headache No S28 Female Bilateral Both 20 No No S29 Female Bilateral Both 24 No No S30 Male Bilateral Both 22 Migraine No S31 Female Unilateral Right 21 No No S32 Female Bilateral Both 27 Tension headache No S33 Male Unilateral Right 30 No No S34 Male Unilateral Left 25 No No S35 Female Bilateral Both 25 MA No S36 Male Bilateral Both 17 No Psoriasis MA, migraine with aura; MO, migraine without aura. Table 4: List of sensorineural hearing loss genes selected for analysis, chromosome position, phenotypes and references. Gene Position Phenotype Reference https://www.ncbi.nlm.nih.gov ESPN chr1:6,484,848-6,521,430 DFNB36 /pubmed/15930085 https://www.ncbi.nlm.nih.gov IFNLR1 chr1:24,480,647-24,514,449 DFNA2C /pubmed/29453195 https://www.ncbi.nlm.nih.gov GJB3 chr1:35,246,790-35,251,970 DFNA2B /pubmed/9843210 https://www.ncbi.nlm.nih.gov KCNQ4 chr1:41,249,684-41,306,124 DFNA2A /pubmed/10369879 https://www.ncbi.nlm.nih.gov BSND chr1:55,464,606-55,476,556 DFNB73 /pubmed/19646679 https://www.ncbi.nlm.nih.gov ROR1 chr1:64,239,690-64,647,181 DFNB108 /pubmed/27162350 https://www.ncbi.nlm.nih.gov CDC14A chr1:100,810,584-100,985,833 DFNB32 /pubmed/27259055 https://www.ncbi.nlm.nih.gov COL11A1 chr1:103,342,023-103,574,052 DFNA37 /pubmed/30245514 https://www.ncbi.nlm.nih.gov GPSM2 chr1:109,417,972-109,477,167 DFNB82 /pubmed/20602914 https://www.ncbi.nlm.nih.gov LMX1A chr1:165,171,104-165,325,952 DFNA7 /pubmed/29754270 https://www.ncbi.nlm.nih.gov NLRP3 chr1:247,579,458-247,612,410 DFNA34 /pubmed/28847925 https://www.ncbi.nlm.nih.gov OTOF chr2:26,680,071-26,781,566 DFNB9 /pubmed/10903124 https://www.ncbi.nlm.nih.gov PNPT1 chr2:55,861,198-55,921,045 DFNB70 /pubmed/23084290 https://www.ncbi.nlm.nih.gov ELMOD3 chr2:85,581,517-85,618,875 DFNB88 /pubmed/24039609 https://www.ncbi.nlm.nih.gov PJVK chr2:179,316,163-179,326,117 DFNB59 /pubmed/16804542 https://www.ncbi.nlm.nih.gov TMIE chr3:46,742,823-46,752,413 DFNB6 /pubmed/12145746 https://www.ncbi.nlm.nih.gov ILDR1 chr3:121,706,170-121,741,127 DFNB42 /pubmed/21255762 https://www.ncbi.nlm.nih.gov MCM2 chr3:127,317,066-127,341,279 DFNA70 /pubmed/26196677 https://www.ncbi.nlm.nih.gov CCDC50 chr3:191,046,866-191,116,459 DFNA344 /pubmed/17503326 https://www.ncbi.nlm.nih.gov WFS1 chr4:6,271,576-6,304,992 DFNA6 /pubmed/10424813 https://www.ncbi.nlm.nih.gov GRXCR1 chr4:42,895,283-43,032,675 DFNB25 /pubmed/20137778 https://www.ncbi.nlm.nih.gov REST chr4:57,774,042-57,802,010 DFNA27 /pubmed/29961578 https://www.ncbi.nlm.nih.gov GAB1 chr4:144,257,915-144,395,721 DFNB26 /pubmed/29408807 https://www.ncbi.nlm.nih.gov MARVELD2 chr5:68,710,939-68,740,157 DFNB49 /pubmed/15538632 https://www.ncbi.nlm.nih.gov BDP1 chr5:70,751,442-70,863,649 DFNB112 /pubmed/24312468 https://www.ncbi.nlm.nih.gov PPIP5K2 chr5:102,455,853-102,548,500 DFNB100 /pubmed/15538632 https://www.ncbi.nlm.nih.gov SLC22A4 chr5:131,630,136-131,679,899 DFNB60 /pubmed/27023905 Gene Position Phenotype Reference https://www.ncbi.nlm.nih.gov DIAPH1 chr5:140,894,583-140,998,622 DFNA1 /pubmed/9360932 https://www.ncbi.nlm.nih.gov GRXCR2 chr5:145,239,296-145,252,531 DFNB101 /pubmed/24619944 https://www.ncbi.nlm.nih.gov POU4F3 chr5:145,718,587-145,720,083 DFNA15 /pubmed/18228599 https://www.ncbi.nlm.nih.gov SERPINB6 chr6:2,948,393-2,972,399 DFNB91 /pubmed/20451170 https://www.ncbi.nlm.nih.gov DCDC2 chr6:24,171,983-24,383,520 DFNB66 /pubmed/25601850 https://www.ncbi.nlm.nih.gov RIPOR2 chr6:24,797,601-25,042,238 DFNB104 /pubmed/24958875 https://www.ncbi.nlm.nih.gov COL11A2 chr6:33,130,458-33,160,276 DFNA13 /pubmed/10581026 https://www.ncbi.nlm.nih.gov LHFPL5 chr6:35,773,070-35,801,651 DFNB67 /pubmed/16752389 https://www.ncbi.nlm.nih.gov CLIC5 chr6:45,866,188-46,048,132 DFNB103 /pubmed/17021174 https://www.ncbi.nlm.nih.gov MYO6 chr6:76,458,909-76,629,254 DFNA22 /pubmed/11468689 https://www.ncbi.nlm.nih.gov CD164 chr6:109,687,717-109,703,762 DFNA66 /pubmed/26197441 https://www.ncbi.nlm.nih.gov EYA4 chr6:133,561,736-133,853,258 DFNA10 /pubmed/17567890 https://www.ncbi.nlm.nih.gov GSDME chr7:24,737,972-24,809,244 DFNA5 /pubmed/9771715 https://www.ncbi.nlm.nih.gov PDE1C chr7:31,790,793-32,338,941 DFNA74 /pubmed/29860631 https://www.ncbi.nlm.nih.gov ADCY1 chr7:45,613,739-45,762,715 DFNB44 /pubmed/15583425 https://www.ncbi.nlm.nih.gov HGF chr7:81,328,322-81,399,754 DFNB39 /pubmed/19576567 https://www.ncbi.nlm.nih.gov TRRAP chr7:98,475,556-98,610,866 DFNA /pubmed/31231791 https://www.ncbi.nlm.nih.gov SLC26A5 chr7:102,993,177-103,086,624 DFNB61 /pubmed/12719379 https://www.ncbi.nlm.nih.gov SLC26A4 chr7:107,301,080-107,358,254 DFNB4 /pubmed/16570074 https://www.ncbi.nlm.nih.gov MET chr7:116,312,444-116,438,440 DFNB97 /pubmed/25941349 https://www.ncbi.nlm.nih.gov MIRN96 chr7:129,414,532-129,414,609 DFNA50 /pubmed/19363479 https://www.ncbi.nlm.nih.gov ESRP1 chr8:95,653,302-95,719,694 DFNB109 /pubmed/29107558 https://www.ncbi.nlm.nih.gov GRHL2 chr8:102,504,660-102,681,954 DFNA28 /pubmed/12393799 https://www.ncbi.nlm.nih.gov TJP2 chr9:71,736,209-71,870,124 DFNA51 /pubmed/20602916 https://www.ncbi.nlm.nih.gov TMC1 chr9:75,136,717-75,451,267 DFNB7 /pubmed/18616530 https://www.ncbi.nlm.nih.gov WHRN chr9:117,164,360-117,267,736 DFNB31 /pubmed/12833159 https://www.ncbi.nlm.nih.gov TNC chr9:117,782,805-117,880,536 DFNA56 /pubmed/23936043 https://www.ncbi.nlm.nih.gov TPRN chr9:140,086,069-140,098,645 DFNB79 /pubmed/20170898 https://www.ncbi.nlm.nih.gov MYO3A chr10:26,223,002-26,501,465 DFNB30 /pubmed/12032315 Gene Position Phenotype Reference https://www.ncbi.nlm.nih.gov PCDH15 chr10:55,562,531-57,387,702 DFNB23 /pubmed/14570705 https://www.ncbi.nlm.nih.gov CDH23 chr10:73,156,691-73,575,704 DFNB12 /pubmed/11090341 https://www.ncbi.nlm.nih.gov C10orf105 chr10:73,471,458-73,497,581 DFNB12 /pubmed/11090341 https://www.ncbi.nlm.nih.gov PDZD7 chr10:102,767,440-102,790,914 DFNB57 /pubmed/26849169 https://www.ncbi.nlm.nih.gov EPS8L2 chr11:694,438-727,727 DFNB106 /pubmed/26282398 https://www.ncbi.nlm.nih.gov USH1C chr11:17,515,442-17,565,963 DFNB18A /pubmed/12107438 https://www.ncbi.nlm.nih.gov OTOG chr11:17,568,920-17,668,697
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