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3 While most hearing loss in older adults is sensorineural and due to presbycusis, cerumen impaction
4 nly external and middle ear defects but also sensorineural and vestibular phenotypes observed in thes
5 r knowledge, this is the first non-syndromic sensorineural autosomal deafness susceptibility gene to
6 ate the terminal differentiation of distinct sensorineural cells in which they exhibit selective spat
8 ed1-Notch2 signaling is known to pattern the sensorineural components of the inner ear, its role in m
11 ed a unique, inbred Bedouin kindred in which sensorineural deafness (SND) cosegregates with an infant
12 with a 21-kb deletion in chromosome Xq22 and sensorineural deafness along with dystonia, we character
13 ion of symptoms, including epilepsy, ataxia, sensorineural deafness and a renal tubulopathy designate
18 n is responsible for the familiar conditions sensorineural deafness and hypertrophic cardiomyopathy.
20 of mitochondrial RC dysfunction, congenital sensorineural deafness and progressive hepatic and renal
21 the inner ear, cause the autosomal dominant sensorineural deafness and vestibular disorder, DFNA9 (O
22 ome characterized by postlingual progressive sensorineural deafness as the first presenting symptom i
23 f these patients (73%) presented without the sensorineural deafness associated with Jervell and Lange
24 yndrome (JLNS) comprises profound congenital sensorineural deafness associated with syncopal episodes
26 before 12 months (2 before 6 months) (5/5), sensorineural deafness diagnosed soon after birth (5/5),
27 nice, and stitch, with recessively inherited sensorineural deafness due to novel mutations in the tra
28 aits of hypersensitivity to streptomycin and sensorineural deafness in a number of families from Chin
29 racterized by ovarian dysgenesis in females, sensorineural deafness in both males and females, and in
30 rative disorder characterized by progressive sensorineural deafness in combination with childhood amy
31 i, providing a molecular explanation for the sensorineural deafness in ectodermal dysplasia patients
32 nconventional myosin XVa are associated with sensorineural deafness in humans (DFNB3) and shaker (Myo
35 t Cx26 and Cx30 mutations that are linked to sensorineural deafness retained ionic coupling but were
38 d fever, rashes, arthralgia, conjunctivitis, sensorineural deafness, and an intense acute-phase respo
40 eration, seizures, cerebellar abnormalities, sensorineural deafness, and other multisystem features.
41 deficiency - such as in hypoparathyroidism, sensorineural deafness, and renal (HDR) syndrome - by OP
43 e are tissue specific--eg, optic neuropathy, sensorineural deafness, and type 2 diabetes mellitus.
44 isability, cortical malformations, coloboma, sensorineural deafness, and typical facial features.
45 ties, severe cerebellar hypoplasia, profound sensorineural deafness, and visual impairment due to sev
47 etabolic alkalosis associated with seizures, sensorineural deafness, ataxia, and developmental abnorm
48 cognized syndrome characterized by seizures, sensorineural deafness, ataxia, mental retardation, and
49 everal human diseases including nonsyndromic sensorineural deafness, Charcot-Marie-Tooth disease-5, a
50 hich is characterized by profound congenital sensorineural deafness, constant vestibular dysfunction
51 severe hyperinsulinism, profound congenital sensorineural deafness, enteropathy and renal tubular dy
52 utosomal recessive disorder characterized by sensorineural deafness, goiter, and impaired iodide orga
53 ed for MAF/Maf loss of function but includes sensorineural deafness, intellectual disability, seizure
54 sent in a pedigree with maternally inherited sensorineural deafness, levodopa-responsive parkinsonism
55 aracterized by CPEO, mitochondrial myopathy, sensorineural deafness, peripheral neuropathy, parkinson
56 Additional aspects of the phenotype include sensorineural deafness, reduced lifespan and decreased r
57 megaloblastic anemia, diabetes mellitus, and sensorineural deafness, responding in varying degrees to
58 ibes the association of profound, congenital sensorineural deafness, vestibular hypofunction and chil
59 Loss of Cx26 function causes nonsyndromic sensorineural deafness, without consequence in the epide
76 , Abnormal genitalia, Retardation of growth, sensorineural Deafness; LS), also called Noonan syndrome
80 drome type IC is a rare, autosomal recessive sensorineural disorder caused by mutations in the USH1C
81 ety of biologically active agents on colonic sensorineural function in vitro has been described, the
82 tomentosa (UT) would facilitate recovery of sensorineural functions following exposure to a damaging
84 showed several atypical features, including sensorineural hearing deficit, abnormal bleeding, and, m
85 improvement was defined as an improvement of sensorineural hearing from baseline, in at least one ear
87 lations) for the appropriate compensation of sensorineural hearing impairment across a range of frequ
88 nd January 2010 on screening for age-related sensorineural hearing impairment in adults aged 50 years
90 with a mild to severe, sloping, symmetrical, sensorineural hearing impairment were fitted with hearin
95 s (dRTA; OMIM #267300) often associated with sensorineural hearing impairment; however, mice with a k
96 articipants were 36 adults with symmetrical, sensorineural hearing loss (18 experienced hearing instr
97 fy mutations in Gipc3 underlying progressive sensorineural hearing loss (age-related hearing loss 5,
102 d-responsive, rapidly progressive, bilateral sensorineural hearing loss (autoimmune inner ear disease
103 ocus responsible for postlingual progressive sensorineural hearing loss (designated DFNA9) that maps
106 d whole-exome sequencing in individuals with sensorineural hearing loss (SNHL) and identified pathoge
108 Hippel-Lindau disease and cause irreversible sensorineural hearing loss (SNHL) and vestibulopathy.
112 Behavioral studies in humans suggest that sensorineural hearing loss (SNHL) decreases sensitivity
113 e to four decades, the incidence of acquired sensorineural hearing loss (SNHL) in children living in
115 Lassa virus (LASV)-infected patients develop sensorineural hearing loss (SNHL) in the late stages of
119 ngenital cytomegalovirus (CMV) infection and sensorineural hearing loss (SNHL) was first described al
121 s a rare recessive disorder characterized by sensorineural hearing loss (SNHL), amelogenesis imperfec
122 terized by vascularizing keratitis, profound sensorineural hearing loss (SNHL), and progressive eryth
127 -five percent of people with VS present with sensorineural hearing loss (SNHL); the mechanism of this
129 anging from highly restricted pili torti and sensorineural hearing loss (the Bjornstad syndrome) to p
130 tic (venous thrombosis 25%, lymphedema 11%), sensorineural hearing loss 76%, miscarriage 33%, and hyp
133 otogl with morpholinos in zebrafish leads to sensorineural hearing loss and anatomical changes in the
135 292 had no polyneuropathy or ataxia, and the sensorineural hearing loss and cataract were attributed
136 a good animal model to evaluate the role of sensorineural hearing loss and central inhibition in aud
137 ion in multiple tissues, suggesting that the sensorineural hearing loss and characteristic brain malf
138 ilitate early detection and intervention for sensorineural hearing loss and developmental delay, wher
139 nia, facial dysmorphology, ocular anomalies, sensorineural hearing loss and developmental delay.
140 dered in patients presenting with congenital sensorineural hearing loss and disorders of cornificatio
143 erturn a long-standing dogma in the study of sensorineural hearing loss and highlight the importance
144 ty of hearing-loss disorders, such as sudden sensorineural hearing loss and Meniere's disease that ar
146 autosomal recessive disorder associated with sensorineural hearing loss and pili torti, is caused by
147 d that an absence of Np65 causes early-onset sensorineural hearing loss and prevented the normal syna
148 ant mice, absence of Np65 causes early-onset sensorineural hearing loss and prevents normal neurotran
149 disorder characterized by moderate to severe sensorineural hearing loss and progressive retinitis pig
150 disorder characterized by moderate to severe sensorineural hearing loss and progressive retinitis pig
151 racterized by progressive pontobulbar palsy, sensorineural hearing loss and respiratory insufficiency
152 der characterized by the association between sensorineural hearing loss and thyroid swelling or goitr
153 ) gene, causing the adult-onset, progressive sensorineural hearing loss and vestibular disorder at th
154 e etiologic for the late-onset, progressive, sensorineural hearing loss and vestibular dysfunction kn
155 members inherited pili torti and prelingual sensorineural hearing loss as autosomal recessive traits
157 obtained from 49 participants with moderate sensorineural hearing loss before fitting and after 3 we
158 gs to more clinically relevant conditions of sensorineural hearing loss by examining the role of frac
159 that this variant may modify the severity of sensorineural hearing loss caused by a variety of factor
162 oximately 20-30% of patients with congenital sensorineural hearing loss demonstrate radiographic abno
163 nalysis of dominantly inherited, progressive sensorineural hearing loss DFNA41 in a six-generation ki
164 oding connexin 26 (cx26) have been linked to sensorineural hearing loss either alone or as part of a
165 elin plasticity and how this could relate to sensorineural hearing loss following peripheral impairme
169 either common (hearing loss) or distinctive (sensorineural hearing loss in a child), they are importa
170 t, progressive, high-frequency, nonsyndromic sensorineural hearing loss in a large, multigenerational
171 te OPG at high levels and lack of OPG causes sensorineural hearing loss in addition to the previously
175 channel-like gene 1 (TMC1) cause progressive sensorineural hearing loss in humans and Beethoven (Tmc1
178 GED1 loss, and a diversity of conductive and sensorineural hearing loss in nearly half of AGS patient
179 53G > A), of NLRP3 causes autosomal-dominant sensorineural hearing loss in two unrelated families.
180 oss in which the cochleae are not damaged or sensorineural hearing loss in which both cochleae are re
185 e not precipitated by cold exposure and that sensorineural hearing loss is frequently also present.
189 sly unknown autosomal-recessive nonsyndromic sensorineural hearing loss locus (DFNB91) to chromosome
192 more likely than controls to have bilateral sensorineural hearing loss of 40 dB or more (unmatched 1
193 s a higher risk of adverse neonatal outcome (sensorineural hearing loss or neurological deficits).
194 otitis media (OM), including high-frequency sensorineural hearing loss or vertigo, is not uncommon.
195 regating an autosomal dominant, progressive, sensorineural hearing loss phenotype that has been linke
196 man with a history of progressive bilateral sensorineural hearing loss presented to a neuro-ophthalm
197 trial involving 250 patients with unilateral sensorineural hearing loss presenting within 14 days of
198 mbrane abnormalities may be one aetiology of sensorineural hearing loss primarily affecting the mid-f
199 year-old female has intellectual disability, sensorineural hearing loss requiring bilateral cochlear
201 transcriptional co-activator EYA4 gene cause sensorineural hearing loss that can occur in association
202 describe 2 unrelated pedigrees with MTP and sensorineural hearing loss that segregate with a DIAPH1
207 utosomal dominant, nonsyndromic, progressive sensorineural hearing loss with vestibular pathology.
208 sease erythrokeratoderma variabilis (EKV) or sensorineural hearing loss with/without peripheral neuro
211 sequential probands referred for congenital sensorineural hearing loss, 22 (42%) were found to have
212 who presented with osteoporosis, cataracts, sensorineural hearing loss, and mild learning defects.
213 s much to learn about pathogenesis of sudden sensorineural hearing loss, and more clinical trials are
214 wo thirds of respondents were male, most had sensorineural hearing loss, and most were older than 50;
215 al-recessive inheritance, severe to profound sensorineural hearing loss, and partial agenesis of the
216 nction, cachetic dwarfism, photosensitivity, sensorineural hearing loss, and retinal degradation.
217 llar ataxia, early-onset cerebellar atrophy, sensorineural hearing loss, and the distinctive associat
218 etely penetrant, predominantly low-frequency sensorineural hearing loss, and the Fgfr3(P244R) mice sh
219 spontaneous attacks of vertigo, fluctuating sensorineural hearing loss, aural fullness, and tinnitus
220 cause of neurological problems, particularly sensorineural hearing loss, but data on long-term sequel
221 vide significant benefit for a wide range of sensorineural hearing loss, but no carefully controlled,
222 cterized by pigmentary retinal degeneration, sensorineural hearing loss, childhood obesity, non-insul
223 disability, hypotonia, spasticity, seizures, sensorineural hearing loss, cortical visual impairment,
224 plantation, the current therapy for profound sensorineural hearing loss, depends on a functional nerv
225 rophy, neuropathy, myopathy, cardiomyopathy, sensorineural hearing loss, diabetes mellitus, and other
226 results in vertebral compression fractures, sensorineural hearing loss, eye defects, and heart defec
227 bers show a bilateral, sloping, progressive, sensorineural hearing loss, first evident at 6000 and 80
229 cted in two unrelated persons with increased sensorineural hearing loss, in the other caused by a mut
230 coustic trauma, one of the leading causes of sensorineural hearing loss, induces sensory hair cell da
231 sible causes of hearing loss, such as sudden sensorineural hearing loss, is important to maximize the
232 syndrome that includes the subacute onset of sensorineural hearing loss, often accompanied by vertigo
233 verlapping clinical features, manifesting as sensorineural hearing loss, often associated with vertig
234 racterized by mental retardation, hypotonia, sensorineural hearing loss, optic atrophy, and other fea
235 autosomal dominant disorder characterized by sensorineural hearing loss, palmoplantar keratoderma, kn
236 iginally identified in Persian families with sensorineural hearing loss, regulates peroxisomal dynami
237 ic TBS patients by displaying high-frequency sensorineural hearing loss, renal cystic hypoplasia and
238 cochlea, caused by macular degeneration and sensorineural hearing loss, respectively, affect a growi
240 of hair cells or auditory neurons results in sensorineural hearing loss, the consequence of supportin
241 chronic disorder of the inner ear defined by sensorineural hearing loss, tinnitus and episodic vertig
242 ting from minutes to hours, with fluctuating sensorineural hearing loss, tinnitus, and aural pressure
243 rized by fever, chronic meningitis, uveitis, sensorineural hearing loss, urticarial skin rash, and a
244 essive disorder characterized by progressive sensorineural hearing loss, vestibular dysfunction, and
283 siblings presented with autosomal recessive sensorineural hearing loss: two had high-frequency loss,
284 Patients with ATP6B1 mutations also have sensorineural hearing loss; consistent with this finding
288 ng loss in older adults, is a multifactorial sensorineural loss that frequently includes a component
289 518Dfs*2) in an Italian pedigree affected by sensorineural mild-to-moderate HHL but also showing a va
290 ast, children with SLI showed no evidence of sensorineural modulation with attention, despite behavio
291 ns of mice have different predispositions to sensorineural pathway activation, we quantified expressi
292 These findings expand our understanding of sensorineural plasticity in adult vestibular organs and
293 ta3 is expressed in a distinct population of sensorineural precursor cells within the otic epithelium
294 the effects of selective attention on early sensorineural processing may give rise to the diverse se
295 omal dominant, fully penetrant, nonsyndromic sensorineural progressive hearing loss in a large Costa
297 ping children showed an amplification of the sensorineural response to attended as compared to unatte
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