ライフサイエンスコーパス: deaf

1 posers to write and edit music while totally deaf.

2 ensuing adult transgenic mice are profoundly deaf.

3 ially improving hearing for children who are deaf.

4 nd hear speech in noise for children who are deaf.

5 uisition of spoken language in children born deaf.

6 iobp(Deltaex8/Deltaex8)) that are profoundly deaf.

7 no detectable ABR and by P30 these mice were deaf.

8 ced AF connectivity was observed in the tone deaf.

9 fness in humans, and OTOF knock-out mice are deaf.

10 lacking PCs owing to a mutation in Fgfr3 are deaf.

11 ecruited for visual and tactile input in the deaf.

12 ond and third rows of stereocilia and become deaf.

13 d auditory cortices in people born blind and deaf.

14 767Serfs*21 mutation (72.1%) were moderately deaf.

15 region of Tdrd1 containing the myeloid Nervy DEAF-1 (MYND) domain and the first two Tudor domains.

16 oteins with the N-terminal Myeloid Nervy and DEAF-1 (MYND)-type zinc finger of PHD2.

17 DEAF-1 activates the expression of Mtk and Drs promoter-

18 ification technology (MudPIT), we identified DEAF-1 as a candidate regulator.

19 A functional Deaf-1 binding site on the mouse 5-HT1A promoter was rec

20 X assays and footprinting data indicate that DEAF-1 binds to and activates Mtk and Drs regulatory DNA

21 In cell models Deaf-1 displays dual activity, repressing 5-HT1A autorec

22 We have disrupted the Lmo4 and Deaf-1 genes in mice to define their biological function

23 Deaf-1(-/-) mice indicate the importance of Deaf-1 in regulation of 5-HT1A gene expression and provi

24 the mouse 5-HT1A promoter was recognized by Deaf-1 in vitro and in vivo and mediated dual activity o

25 To address regulation by Deaf-1 in vivo, Deaf-1 knock-out mice bred to a C57BL/6

26 We propose that DEAF-1 is a regulator of Drosophila immunity.

27 To address regulation by Deaf-1 in vivo, Deaf-1 knock-out mice bred to a C57BL/6 background were

28 In the dorsal raphe, Deaf-1 knock-out mice displayed increased 5-HT1A mRNA, p

29 ro and in vivo and mediated dual activity of Deaf-1 on 5-HT1A gene transcription.

30 e Diptericin (Dpt) regulatory region confers DEAF-1 responsiveness to this normally DEAF-1-independen

31 The coexpression of DEAF-1 with Dorsal, Dif, and Relish results in the syner

32 expression and reduced raphe 5-HT content in Deaf-1(-/-) mice indicate the importance of Deaf-1 in re

33 Arabidopsis SAND (Sp100, AIRE-1, NucP41/75, DEAF-1) domain protein ULTRAPETALA1 (ULT1) functions as

34 deformed epidermal autoregulatory factor-1 (DEAF-1), also contributes to the immune response and is

35 ZMYND8 (zinc finger MYND (Myeloid, Nervy and DEAF-1)-type containing 8), a newly identified component

36 nfers DEAF-1 responsiveness to this normally DEAF-1-independent enhancer.

37 cells were reduced in the frontal cortex of Deaf-1-null mice, with no significant change in hippocam

38 hism (rs6295), which prevents the binding of Deaf-1/NUDR leading to dysregulation of the receptor, ha

39 8 (zinc finger and MYND [myeloid, Nervy, and DEAF-1] domain containing 8) as a novel DDR factor that

40 is: (1) rare in people who are congenitally deaf, (2) common in people with acquired deafness, and (

41 The study of congenitally deaf adult humans provides an opportunity to examine neu

42 ent using the Multiple Object Tracking task, Deaf adult native signers and hearing non-signers also p

43 first experiment using an enumeration task, Deaf adult native signers and hearing non-signers perfor

44 Deaf adults and children can be successfully (re)integra

45 s have restored hearing in more than 200 000 deaf adults and children to a level that allows most to

46 f hearing for nearly 200 thousand profoundly deaf adults and children.

47 rtex to visual speech in the same profoundly deaf adults both before and 6 mo after implantation.

48 d the strength of the visual illusion in the deaf adults in line with the interpretation that the ill

49 In addition, deaf adults perceived bimodal stimuli differently; in co

50 Here we enrolled 15 deaf and 15 hearing adults into an functional MRI experi

51 The zebrafish pinball wizard (pwi) mutant is deaf and blind.

52 19P) of TRPML3 (mucolipin 3), are profoundly deaf and display vestibular and pigmentation deficiencie

53 n in the left inferior frontal gyrus in both deaf and dyslexic adults when contrasted with hearing no

54 The data indicate greater activation in the deaf and dyslexic groups than in the hearing non-dyslexi

55 elated Cib2 mutation, and show that both are deaf and exhibit no mechanotransduction in auditory hair

56 lay the foundation for the clinical care of deaf and hard-of-hearing persons in the future.

57 They are deaf and have vestibular dysfunction but do not develop

58 Varitint-waddler (Va and Va(J)) mice are deaf and have vestibular impairment, with inner ear defe

59 gray (GMV) and white (WMV) matter volume in deaf and hearing native users of ASL, as well as deaf an

60 and hearing native users of ASL, as well as deaf and hearing native users of English.

61 l cortices (STC) we collected fMRI data from deaf and hearing participants (male and female), who eit

62 s with meaningless gestures by pre-lingually Deaf and hearing participants.

63 Both deaf and hearing signers exhibited an increased volume o

64 Both deaf and hearing subjects performed the task visually, i

65 We thus questioned whether the deaf and immature human auditory system is able to integ

66 In the absence of FGF20, mice are deaf and lateral compartment cells remain undifferentiat

67 urtship vocalizations emitted by chronically deaf and normal hearing adult male mice.

68 ted deletion of Tmc1 (Tmc1(Delta) mice) were deaf and those with a deletion of Tmc2 (Tmc2(Delta) mice

69 mechanotransduction, we discovered a line of deaf and uncoordinated zebrafish with defective hair-cel

70 also described for further investigating the deaf and vestibular mutants identified in the primary sc

71 r principal neurons from the MSO of hearing, deaf, and deaf cats with cochlear implants.

72 any of their best compositions while totally deaf, and Georg Friedrich Handel and Frederick Delius st

73 humans, mice, and zebrafish; individuals are deaf, and stereocilia are disorganized.

74 mc1 pD569N heterozygote mice were profoundly deaf, and there was substantial loss of outer hair cells

75 erior visual motion detection ability in the deaf animal.

76 aphically organized, "isofrequency" bands in deaf animals over the ages examined, P18-P70.

77 Late-deaf animals showed small-scale changes in projections f

78 matic terminals was significantly smaller in deaf animals when compared with hearing animals.

79 A1 that differ between early- and late-onset deaf animals, suggesting that potential crossmodal activ

80 ures we examined were similar in hearing and deaf animals.

81 are congenitally hypothyroid and profoundly deaf as a consequence when the condition is untreated.

82 difference in STM span, hearing speakers and deaf ASL users have comparable working memory resources

83 both baringo and nice mutants are profoundly deaf at the age of 4 weeks.

84 ntified reliably in the vast majority of the deaf, at the single subject level, despite the absence o

85 nsive and auditory-responsive neurons in the deaf auditory cortex formed two distinct populations tha

86 tion are abolished when a specific region of deaf auditory cortex, the dorsal zone (DZ), is deactivat

87 altered cross-modal organization observed in deaf auditory cortex.

88 r electrical stimulation of the congenitally deaf auditory system via cochlear implants would restore

89 y heterogeneous group of autosomal recessive deaf-blinding disorders.

90 the motor protein myosin VIIA (MYO7A) cause deaf-blindness (Usher syndrome type 1B, USH1B) and nonsy

91 CDH23 null alleles cause deaf-blindness (Usher syndrome type 1D; USH1D), whereas

92 Usher syndrome type 1B is a combined deaf-blindness condition caused by mutations in the MYO7

93 Mutations in the MYO7A gene cause a deaf-blindness disorder, known as Usher syndrome 1B.

94 er syndrome (USH) is the most common form of deaf-blindness in humans.

95 syndrome (USH) is the most common inherited deaf-blindness with the majority of USH causative genes

96 Mutations in PCDH15 cause Usher Syndrome (deaf-blindness) and recessive deafness.

97 fects, olfactory dysfunction, growth delays, deaf-blindness, balance disorders and congenital heart m

98 (USH) is the most common cause of inherited deaf-blindness, manifested as USH1, USH2 and USH3 clinic

99 rome (USH) is the leading cause of inherited deaf-blindness, with type 2 (USH2) being the most common

100 ons for research on myosin-7a and hereditary deaf-blindness.

101 sher syndrome, the most common form of human deaf-blindness.

102 her syndrome is the leading cause of genetic deaf-blindness.

103 ead to non-syndromic deafness, blindness and deaf-blindness.

104 Usher syndrome is the major cause of deaf/blindness in the world.

105 tocadherin-15, a product of the gene for the deaf/blindness Usher syndrome type 1F/DFNB23 locus.

106 We asked two questions regarding how the deaf brain in humans adapts to sensory deprivation: (1)

107 These Ush1 knockout mice are deaf but do not recapitulate vision defects before 10 mo

108 d develop progressive hearing loss, becoming deaf by 8 months of age.

109 lants (CIs) partially restore hearing to the deaf by directly stimulating the inner ear.

110 estore hearing cues in the severe-profoundly deaf by electrically stimulating spiral ganglion neurons

111 revealed that S1P(2) receptor-null mice were deaf by one month of age.

112 cell defects by postnatal day 2 (P2) and are deaf by P21-P25.

113 e hearing loss at the age of 4 weeks and are deaf by the age of 8 weeks, whereas both baringo and nic

114 ements indicate that these mice are severely deaf by the third week of life.

115 rticipants.SIGNIFICANCE STATEMENT Those born deaf can offer unique insights into neuroplasticity, in

116 mplete sensory deprivation, the congenitally deaf cat.

117 higher-order auditory fields in congenitally deaf cats (CDCs).

118 ical areas in adult hearing and congenitally deaf cats (CDCs): the primary auditory field A1, two sec

119 ity to low-rate pulse trains in congenitally deaf cats compared with acutely deafened cats.

120 We stimulated congenitally deaf cats for 3 months with a six-channel cochlear impla

121 Three and 6-month-old congenitally deaf cats received unilateral cochlear implants and were

122 l neurons from the MSO of hearing, deaf, and deaf cats with cochlear implants.

123 ere significantly larger than the boutons in deaf cats, although not as large as in the hearing cats,

124 Additionally, in a subset of early- and late-deaf cats, area 17 and the lateral posterior nucleus (LP

125 In early-deaf cats, ipsilateral neuronal labeling in visual and s

126 best ITDs were more variable in congenitally deaf cats, leading to poorer ITD coding within the natur

127 In late-deaf cats, projections from the anterior auditory field

128 In deaf cats, substantially reduced induced responses were

129 Specifically, in early-deaf cats, the anterior auditory field (AAF) is unrespon

130 und to be significantly larger than those of deaf cats.

131 hlear implants in adult hearing controls and deaf cats.

132 ed in cross-modal (visual) reorganization in deaf cats.

133 ield (but not in primary auditory cortex) of deaf cats.

134 elopment that were not pruned postnatally in deaf cats.

135 ith less marked differences observed in late-deaf cats.

136 ochlear implants provide sound perception to deaf children and can mitigate, to varying extents, the

137 ecome a standard clinical procedure for born-deaf children and its success has led over the years to

138 Research on deaf children and sign language acquisition can broaden

139 r example, after cochlear implantation, some deaf children develop spoken language skills approaching

140 early childhood cochlear-implant, profoundly deaf children do not develop intact, high-level, auditor

141 normal hearing children and in congenitally deaf children fitted with cochlear implants.

142 ncomplementary matings that can produce only deaf children has increased by a factor of more than fiv

143 Participants include deaf children whose first exposure to ASL was delayed up

144 limitations of implantation in congenitally deaf children.

145 concept formation are at particular risk in deaf children.

146 that account for as many as 2% of profoundly deaf children; however, the underlying cause for its dos

147 Retinas of deaf circler (dfcr) mice, which possess mutant Ush1c, we

148 Moreover, the 'deaf-circler' dfcr mutant form of harmonin, which does n

149 ation of nonsensory cells that remain in the deaf cochlea.

150 Sign languages used by deaf communities around the world possess the same struc

151 disorders occur at rates 2-3 times higher in deaf compared with hearing children.

152 icant differences between normal-hearing and deaf controls.

153 sensitivity and prevented hair cell death in deaf Cx30-/- mice.

154 t of binaural processing in children who are deaf despite early access to bilateral auditory input by

155 mate transporter-3 (VGLUT3) are congenitally deaf due to loss of glutamate release at the inner hair

156 port that mice lacking VGLUT3 are profoundly deaf due to the absence of glutamate release from hair c

157 ccess with which electrical stimulation of a deaf ear can mimic acoustic stimulation of a normal-hear

158 articularly challenging to treat because the deaf ear can no longer be stimulated by acoustic means.

159 Subjects typically describe tinnitus in the deaf ear on the side of the surgery that can be modulate

160 1 of these, and was initially positive in 1 deaf ear, becoming negative at followup.

161 echanisms that regulate neuronal survival in deaf ears.

162 Va mice are deaf, exhibit circling behavior due to vestibular defect

163 ASL, and 16 hearing subjects who grew up in deaf families and were native ASL signers.

164 ing role of the mouth allows these seemingly deaf frogs to communicate effectively without a middle e

165 We tested children who had been deaf from birth and who subsequently received cochlear i

166 Here we demonstrate that in those deaf from birth the left and the right STC have altered

167 data revealed that in participants who were deaf from birth, STC showed increased activation during

168 ion of the auditory cortex develop in people deaf from birth?

169 ocessing when auditory processes are absent (deaf group) or impaired (dyslexic group).

170 sensory experience revealed less GMV in the deaf groups combined (compared with hearing groups combi

171 nstitutively expressing GFP (H9 Cre-LoxP) in deaf guinea pig cochleae that were pre-conditioned to re

172 knock-out mice (Atoh1(CKO)) are behaviorally deaf, have diminished auditory brainstem evoked response

173 In the deaf Heschl's gyrus, signal change was greater for somat

174 Therefore, cross-modal plasticity in the deaf higher-order auditory cortex had limited effects on

175 owever, both the auditory responsiveness of "deaf" higher-order fields and interactions between the r

176 man primary auditory cortex, in congenitally deaf humans by measuring the fMRI signal change in respo

177 We conclude that in deaf humans the high-level auditory cortex switches its

178 and functional imaging) in a group of early deaf humans.

179 erties of dELL and a dELL-associated factor (dEaf) in a living organism.

180 in the deaf is complicated by the fact that deaf individuals also differ in their language experienc

181 aphy and psychophysics were assessed in tone-deaf individuals and matched controls.

182 We found that sign in deaf individuals and speech in hearing individuals activ

183 aring developmental dyslexics and profoundly deaf individuals both have difficulties processing the i

184 These deaf individuals develop their own gestures, called home

185 Nevertheless, some profoundly deaf individuals do learn to read at age-appropriate lev

186 These deaf individuals have had no contact with any convention

187 d an analysis of nearly 5000 marriages among deaf individuals in America collected during the 19(th)

188 Although the majority of deaf individuals in the United States are born to hearin

189 natomical and functional changes observed in deaf individuals is not only sensory, but also cognitive

190 common view, the "unused" auditory cortex of deaf individuals is reorganized to a compensatory sensor

191 suggest that during human motion processing, deaf individuals may engage specialized neural systems t

192 ditory feedback in vocal production, how can deaf individuals produce intelligible speech?

193 Congenitally deaf individuals receive little or no auditory input, an

194 ree data on 311 contemporary marriages among deaf individuals that were comparable to those collected

195 We investigated sign language that enables deaf individuals to communicate through hand movements w

196 we focus on homesign, gestures developed by deaf individuals who cannot acquire spoken language and

197 means of communication among a population of deaf individuals who could not acquire the surrounding s

198 s who lack conventional language for number (deaf individuals who do not have access to a usable mode

199 restored hearing in hundreds of thousands of deaf individuals worldwide.

200 or research areas: syntactic competence in d/Deaf individuals, and language documentation.

201 In congenitally profoundly deaf individuals, auditory speech processing is essentia

202 t underlie reading achievement in profoundly deaf individuals.

203 d anxiety disorders occur at higher rates in deaf individuals.

204 uditory-based) representations of speech for deaf individuals.

205 ture neural prostheses to restore hearing to deaf individuals.SIGNIFICANCE STATEMENT The question of

206 ly improves hearing thresholds in the mature deaf inner ear after delivery to nonsensory cells throug

207 ce of the brain's structural variance in the deaf is complicated by the fact that deaf individuals al

208 However, PCDH15-CD2-deficient mice are deaf, lack kinociliary links and have abnormally polariz

209 A deaf lip-reading interpreter and a hearing American sign

210 first time, to elicit sound sensations in a deaf listener using an electrode implanted in his inner

211 of each syllable type emitted by hearing and deaf males in the presence of a female.

212 nal repair in the organ of Corti of a mature deaf mammal.

213 In these deaf mice, we found responses to noxious noise, which da

214 lation of SGNs restored auditory activity in deaf mice.

215 the pathogenesis processes in the cochlea of deaf mice.

216 ense phenotypic assortative mating among the deaf might have greatly accelerated the normally slow re

217 es anyway - advanced acoustic defences for a deaf moth.

218 ction of wild-type VGLUT3 in the genetically deaf mouse cochlea results in significantly improved hea

219 In the deaf mutant quivering mouse, the localization of Nav1.6

220 Deaf native users of American Sign Language (ASL) were p

221 studies on deafness have been conducted with deaf native users of ASL (deaf signers).

222 wer areas of anatomical differences than did deaf native users of ASL (each compared with their heari

223 rience and language experience revealed that deaf native users of English had fewer areas of anatomic

224 Pre-lingually deaf, native signers of ASL participated in the fMRI stu

225 Children who are born deaf never have this bimodal experience.

226 ined the gesture systems that three isolated deaf Nicaraguans (ages 14-23 years) have developed for u

227 A sample of young-adolescent deaf observers performed with higher accuracy than heari

228 for a general visual processing advantage in deaf observers rather than a face-specific effect.

229 Tshrhyt/hyt mutant mice remained profoundly deaf on P24 and although thresholds improved by approxim

230 with reference to studies of people who are deaf or bilingual.

231 Hearing people with signing deaf parents are bilingual in sign and speech: languages

232 tle or no auditory input, and when raised by deaf parents, they acquire sign as their native and prim

233 ngless gestures (both relative to rest), the Deaf participants, but not the hearing, showed greater r

234 ith the same sign language experience as the deaf participants, did not activate the STCs.

235 Deaf participants, who used American Sign Language, acti

236 tivated to a greater extent by dyslexic than deaf participants.

237 d altered functions of left and right STC in deaf participants.SIGNIFICANCE STATEMENT Those born deaf

238 g the appropriate regenerative therapy for a deaf patient.

239 [i.e., auditory midbrain implant (AMI)] for deaf patients who cannot benefit from cochlear implants

240 Thus, in deaf patients with GJB6 deletion as well as in the previ

241 ery of auditory function for some profoundly deaf patients, potential biological therapies must exten

242 guage has emerged among three generations of deaf people and their families in a Bedouin community in

243 contradictory views - that sign languages of deaf people are "just gestures," or that sign languages

244 w sign languages have emerged recently among deaf people brought together in a community, offering in

245 ther the permanent auditory deprivation that deaf people experience leads to the enhanced visual proc

246 Worldwide, more than 300,000 deaf people have been fitted with a cochlear implant; it

247 functioning auditory periphery of profoundly-deaf people to electrically stimulate their auditory ner

248 Many profoundly deaf people wearing cochlear implants (CIs) still face c

249 Deaf people who use cochlear implants show surprisingly

250 Here, we show that tone-deaf people, with impaired sound perception and producti

251 neurons and recreate auditory sensations in deaf people.

252 s have provided hearing to more than 120,000 deaf people.

253 ially reorganize for face processing in born-deaf people.

254 sure waves observed during the developmental deaf period.

255 of many HCs with stereocilia these mice are deaf, possibly owing to HC and OC patterning defects.

256 marriage partners that included at least one deaf proband, who were ascertained by complete selection

257 maging (fMRI) to examine brain activation in deaf readers (N = 21), comparing proficient (N = 11) and

258 Proficient deaf readers activated left inferior frontal gyrus and l

259 e question of what differentiates proficient deaf readers from less-proficient readers is poorly unde

260 l, our results support the idea that skilled deaf readers have a stronger connection between the orth

261 Twenty congenitally deaf readers made lexical decisions to target words and

262 been proposed that poor reading abilities in deaf readers might be related to weak connections betwee

263 edback modulating orthographic processing in deaf readers.

264 We show that these mice are deaf secondary to rapid loss of initially well-formed ou

265 ings from the cortical surface of profoundly deaf signer during awake craniotomy.

266 A rare case of a deaf signer undergoing awake craniotomy has revealed tha

267 the question of whether observations made in deaf signers can be generalized.

268 As expected, deaf signers engaged left-hemisphere perisylvian languag

269 Data from deaf signers shows a non-uniform response to different c

270 We tested two cohorts of deaf signers who acquired an emerging sign language in N

271 tations, this visuo-motor content can affect deaf signers' linguistic and cognitive processing.

272 een conducted with deaf native users of ASL (deaf signers).

273 the processing of non-linguistic actions in deaf signers.

274 1965 to 1997, and retinal examinations from deaf students born between 1985 and 1994, applying the W

275 ords, 148 from disability records, and 15 in deaf students.

276 tement: Successful restoration of hearing in deaf subjects by means of a cochlear implant requires a

277 Compared with both hearing groups, deaf subjects exhibited a significant increase in the am

278 Although performing the visual task in deaf subjects induced an increase in functional connecti

279 Intriguingly, we show that some deaf subjects perform faster than controls.

280 were based on MRI data from 25 congenitally deaf subjects who were native users of American Sign Lan

281 FC is retained to varying degrees among the deaf subjects, it may serve to predict the potential for

282 sk robustly activated the auditory cortex in deaf subjects, peaking in the posterior-lateral part of

283 gyrus, was activated to a greater extent by deaf than dyslexic participants, whereas the superior po

284 isual responses in Heschl's gyrus, larger in deaf than hearing, were smaller than those elicited by s

285 peripheral visual localization is better in deaf than in normal hearing animals, and that this enhan

286 histicated acoustic protection despite being deaf themselves and hence unable to respond to bat attac

287 how similar FC structure in the congenitally deaf throughout the auditory cortex, including in the la

288 duplication during Salmonella evolution, is deaf to SgrS because of a nonproductive G-U pair in the

289 een speakers with normal hearing and between deaf users of American Sign Language, but laughter rarel

290 For deaf users of ASL specifically, WMV differences resided

291 The deaf-waddler isoform of PMCA2, operating at 30% efficacy

292 mutant allele with mice heterozygous for the deaf-waddler mutant allele, we found severe hair bundle

293 ABR) showed that mtl and bsd homozygotes are deaf, whereas heterozygous and wildtype littermates have

294 g myosin IIIa and myosin IIIb are profoundly deaf, whereas Myo3a-cKO Myo3b(-/-) mice lacking myosin I

295 Some of these cats were raised deaf, whereas others were chronically implanted with coc

296 Lcc/Lcc mice are completely deaf, whereas Ysb/Ysb mice are severely hearing impaired

297 The deaf white cat, a proven model of congenital deafness, w

298 ring until experimentation, and congenitally deaf white cats, which received no auditory inputs until

299 Slc26a4-null mice are profoundly deaf, with severe inner ear malformations and degenerati

300 osthetics, such as cochlear implants for the deaf, with very high spatial resolution.