ライフサイエンスコーパス: progressive hearing loss

1 aC mice degenerate over time, giving rise to progressive hearing loss.

2 revealing new molecular pathways involved in progressive hearing loss.

3 o thrive, thrombocytopenia, photophobia, and progressive hearing loss.

4 and sensitivity and give rise to late-onset progressive hearing loss.

5 natal Tmc1(Bth/+) mice substantially reduced progressive hearing loss.

6 ototoxic drugs or aging diseases, leading to progressive hearing loss.

7 embrane organelle connectomics may result in progressive hearing loss.

8 t degenerated as hearing matured, leading to progressive hearing loss.

9 four mouse strains with different levels of progressive hearing loss.

10 esponse, two had stable hearing, and one had progressive hearing loss.

11 about the genetic basis for its early onset, progressive hearing loss.

12 n an Iranian pedigree that is afflicted with progressive hearing loss.

13 causes outer hair cell defects, and leads to progressive hearing loss.

14 up of patients with pretreatment intractable progressive hearing loss.

15 e mutations in DSPP are also associated with progressive hearing loss.

16 dent elevated thresholds characteristic of a progressive hearing loss.

17 asement membrane and usually associated with progressive hearing loss.

18 ve degeneration, which results in congenital progressive hearing loss.

19 ring in Beethoven mice, a model of dominant, progressive hearing loss.

20 of Caprin1 in mice leads to an early onset, progressive hearing loss.

21 wer regions of stereocilia, and they develop progressive hearing loss.

22 Notably, Yars(DeltaNLS) mice also develop progressive hearing loss.

23 o disrupted retinal vascular development and progressive hearing loss accompanied by sensory hair cel

24 nately found in outer hair cells, leads to a progressive hearing loss after 1 month, suggesting that

25 Loss of gata3 in inner hair cells causes progressive hearing loss and accounts for at least some

26 Ankrd24KO/KO mice show progressive hearing loss and diminished recovery of audi

27 ntion, but also in predicting late-onset and progressive hearing loss and identifying individuals who

28 ncating mutations causing autosomal dominant progressive hearing loss and missense variants causing a

29 However, the progressive hearing loss and morphological defects of ha

30 s mellitus and optic atrophy and may include progressive hearing loss and other neurological symptoms

31 use low-frequency nonsyndromic hearing loss, progressive hearing loss, and isolated optic atrophy ass

32 P2RX2-null mice developed severe progressive hearing loss, and their early exposure to co

33 zygous Gfi1(Cre) mice exhibit an early onset progressive hearing loss as compared with their wild-typ

34 Dusp1 gene knock-out caused premature progressive hearing loss, as confirmed by auditory evoke

35 Csb(m/m) and Csa(-/-) mice manifested progressive hearing loss, as measured by an increase in

36 Progressive hearing loss associated with aging may resul

37 The KO-TgAC1 mice displayed delayed onset progressive hearing loss associated with deterioration o

38 onse test showed that mice lacking CRMP1 had progressive hearing loss at high frequencies.

39 noelectrical transducer current, and develop progressive hearing loss, becoming deaf by 8 months of a

40 Affected individuals experience progressive hearing loss beginning in the 2d-4th decades

41 mmonly used in hearing research: early-onset progressive hearing loss (C57BL/6J and C57BL/6NTac) and

42 The progressive hearing loss correlated with the accumulatio

43 Q4, which, when mutated, causes the dominant progressive hearing loss DFNA2.

44 man K(+) channel hKv7.4 lead to post-lingual progressive hearing loss (DFNA2), which affects world-wi

45 elayed onset autosomal dominant nonsyndromic progressive hearing loss, DFNA20/26.

46 missense allele linked to autosomal dominant progressive hearing loss (DFNA22), provide an allelic sp

47 profound congenital deafness (DFNB7/B11) and progressive hearing loss (DFNA36), respectively, caused

48 eotide substitution that causes the dominant progressive hearing loss, DFNA36.

49 Dominantly inherited progressive hearing loss DFNA38 is caused by heterozygos

50 er, they suffer increased mortality and show progressive hearing loss during adulthood despite compen

51 DBA/2J (DBA) mice exhibit progressive hearing loss, evident for high frequencies (

52 ns between these mutations and the causes of progressive hearing loss have been elusive.

53 es of mutant genes responsible for inherited progressive hearing loss have suggested possible mechani

54 fully penetrant, nonsyndromic sensorineural progressive hearing loss in a large Costa Rican kindred

55 NA20, a gene causing dominant, nonsyndromic, progressive hearing loss in a three-generation Midwester

56 Using a mutant mouse model (Beethoven) for progressive hearing loss in humans (DFNA36), which harbo

57 Recently, dominant-progressive hearing loss in humans and mice was linked t

58 a missense change in LOXHD1, a gene causing progressive hearing loss in humans, as the sole variant

59 Here we show that progressive hearing loss in the ethylnitrosourea-generat

60 ied a mutation in the Usp53 gene that causes progressive hearing loss in the mambo mouse line.

61 age of 30 years (range, 14 to 79 years) and progressive hearing loss in the target ear (median basel

62 cipants with NF2 (median age, 23 years) with progressive hearing loss in the target ear (median basel

63 e in DFNA2, an autosomal dominant version of progressive hearing loss, involves degeneration of hair

64 Age-related hearing loss is a progressive hearing loss involving environmental and gen

65 In an extended Israeli family, nonsyndromic progressive hearing loss is caused by three different re

66 Progressive hearing loss is common in the human populati

67 Progressive hearing loss is very common in the populatio

68 a, which has been implicated in nonsyndromic progressive hearing loss, is localized at stereocilia ti

69 As is seen in humans, the mice had progressive hearing loss leading to profound deafness.

70 "F-actin") assembly, which is disrupted by a progressive hearing loss mutation (p.D1647G, "jordan").

71 Late-onset and progressive hearing losses occur following congenital CM

72 , which has been identified in a pre-lingual progressive hearing loss patient in Taiwan, yielded cell

73 se that these events conspire to produce the progressive hearing loss phenotype in Tg-mtTFB1 mice.

74 career as a composer and pianist, including progressive hearing loss, recurring gastrointestinal com

75 non-syndromic, sensorineural, bilateral and progressive hearing loss, segregating as an autosomal do

76 A306T) homozygous mice display delayed onset progressive hearing loss similar to human DFNB8 patients

77 es in gata3(fl/fl) otof-cre(+/-) mice reveal progressive hearing loss that becomes profound by 6-7 mo

78 ps8L2 null-mutant mice exhibit a late-onset, progressive hearing loss that is directly linked to a gr

79 haracterized typically by bilateral, rapidly progressive hearing loss that responds therapeutically t

80 ltransferase TFB1M (Tg-mtTFB1 mice) exhibits progressive hearing loss that we proposed models aspects

81 nse mutation (G321S) that has been linked to progressive hearing loss to further examine the inhibito

82 af, stdf ) leading to recessive, early-onset progressive hearing loss was detected and exome sequenci

83 ent Clrn2(-/-) mice, used here as a model of progressive hearing loss, we report synaptic auditory ab

84 7a(WT/Sh1) mouse model of autosomal dominant progressive hearing loss, which may model MYO7A-associat

85 Gene mutations that cause nonsyndromic progressive hearing loss with early onset may provide in

86 All participants showed progressive hearing loss within 3 months prior to the st