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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 that segregates with postlingual progressive sensorineural autosomal dominant hearing loss (HL) in 20
6 hlear development.SIGNIFICANCE STATEMENT The sensorineural components of the cochlea include hair cel
7 ed1-Notch2 signaling is known to pattern the sensorineural components of the inner ear, its role in m
9 ion of symptoms, including epilepsy, ataxia, sensorineural deafness and a renal tubulopathy designate
13 n is responsible for the familiar conditions sensorineural deafness and hypertrophic cardiomyopathy.
16 of mitochondrial RC dysfunction, congenital sensorineural deafness and progressive hepatic and renal
17 the inner ear, cause the autosomal dominant sensorineural deafness and vestibular disorder, DFNA9 (O
18 f these patients (73%) presented without the sensorineural deafness associated with Jervell and Lange
20 before 12 months (2 before 6 months) (5/5), sensorineural deafness diagnosed soon after birth (5/5),
21 nice, and stitch, with recessively inherited sensorineural deafness due to novel mutations in the tra
22 racterized by ovarian dysgenesis in females, sensorineural deafness in both males and females, and in
23 rative disorder characterized by progressive sensorineural deafness in combination with childhood amy
24 i, providing a molecular explanation for the sensorineural deafness in ectodermal dysplasia patients
25 nconventional myosin XVa are associated with sensorineural deafness in humans (DFNB3) and shaker (Myo
27 t Cx26 and Cx30 mutations that are linked to sensorineural deafness retained ionic coupling but were
29 iency with agranulocytosis, lymphopenia, and sensorineural deafness that requires hematopoietic stem
30 d fever, rashes, arthralgia, conjunctivitis, sensorineural deafness, and an intense acute-phase respo
32 osynthesis pathway enzyme, develop SRNS with sensorineural deafness, and demonstrated the beneficial
33 eration, seizures, cerebellar abnormalities, sensorineural deafness, and other multisystem features.
34 deficiency - such as in hypoparathyroidism, sensorineural deafness, and renal (HDR) syndrome - by OP
36 e are tissue specific--eg, optic neuropathy, sensorineural deafness, and type 2 diabetes mellitus.
37 isability, cortical malformations, coloboma, sensorineural deafness, and typical facial features.
38 ties, severe cerebellar hypoplasia, profound sensorineural deafness, and visual impairment due to sev
40 etabolic alkalosis associated with seizures, sensorineural deafness, ataxia, and developmental abnorm
41 cognized syndrome characterized by seizures, sensorineural deafness, ataxia, mental retardation, and
42 ous DKC1 p.Glu206Lys developed cataracts and sensorineural deafness, but nephrotic syndrome in only o
43 everal human diseases including nonsyndromic sensorineural deafness, Charcot-Marie-Tooth disease-5, a
44 pe comprising nephrotic syndrome, cataracts, sensorineural deafness, enterocolitis, and early lethali
45 utosomal recessive disorder characterized by sensorineural deafness, goiter, and impaired iodide orga
46 dactyly, structural brain abnormalities with sensorineural deafness, hypothyroidism, and frequent inf
47 dactyly, structural brain abnormalities with sensorineural deafness, hypothyroidism, and frequent inf
48 ed for MAF/Maf loss of function but includes sensorineural deafness, intellectual disability, seizure
49 sent in a pedigree with maternally inherited sensorineural deafness, levodopa-responsive parkinsonism
50 aracterized by CPEO, mitochondrial myopathy, sensorineural deafness, peripheral neuropathy, parkinson
51 Additional aspects of the phenotype include sensorineural deafness, reduced lifespan and decreased r
52 on, and seizures), a condition that includes sensorineural deafness, shortened terminal phalanges wit
53 seizures, developmental delay, microcephaly, sensorineural deafness, spastic quadriparesis and progre
54 ibes the association of profound, congenital sensorineural deafness, vestibular hypofunction and chil
55 Loss of Cx26 function causes nonsyndromic sensorineural deafness, without consequence in the epide
72 , Abnormal genitalia, Retardation of growth, sensorineural Deafness; LS), also called Noonan syndrome
75 drome type IC is a rare, autosomal recessive sensorineural disorder caused by mutations in the USH1C
76 ety of biologically active agents on colonic sensorineural function in vitro has been described, the
77 tomentosa (UT) would facilitate recovery of sensorineural functions following exposure to a damaging
79 showed several atypical features, including sensorineural hearing deficit, abnormal bleeding, and, m
80 improvement was defined as an improvement of sensorineural hearing from baseline, in at least one ear
82 lations) for the appropriate compensation of sensorineural hearing impairment across a range of frequ
83 nd January 2010 on screening for age-related sensorineural hearing impairment in adults aged 50 years
85 with a mild to severe, sloping, symmetrical, sensorineural hearing impairment were fitted with hearin
90 s (dRTA; OMIM #267300) often associated with sensorineural hearing impairment; however, mice with a k
91 articipants were 36 adults with symmetrical, sensorineural hearing loss (18 experienced hearing instr
92 fy mutations in Gipc3 underlying progressive sensorineural hearing loss (age-related hearing loss 5,
97 d-responsive, rapidly progressive, bilateral sensorineural hearing loss (autoimmune inner ear disease
98 ia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) syndrome is caused by
102 known about the effects of mild-to-moderate sensorineural hearing loss (MMHL) during development.
104 d whole-exome sequencing in individuals with sensorineural hearing loss (SNHL) and identified pathoge
106 Hippel-Lindau disease and cause irreversible sensorineural hearing loss (SNHL) and vestibulopathy.
108 c.325A>T (p.I109F) in a child with profound sensorineural hearing loss (SNHL) associated with incomp
111 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
120 ngenital cytomegalovirus (CMV) infection and sensorineural hearing loss (SNHL) was first described al
122 s a rare recessive disorder characterized by sensorineural hearing loss (SNHL), amelogenesis imperfec
123 terized by vascularizing keratitis, profound sensorineural hearing loss (SNHL), and progressive eryth
124 rated that noise exposure, a common cause of sensorineural hearing loss (SNHL), leads to cognitive im
131 -five percent of people with VS present with sensorineural hearing loss (SNHL); the mechanism of this
133 anging from highly restricted pili torti and sensorineural hearing loss (the Bjornstad syndrome) to p
134 tic (venous thrombosis 25%, lymphedema 11%), sensorineural hearing loss 76%, miscarriage 33%, and hyp
137 otogl with morpholinos in zebrafish leads to sensorineural hearing loss and anatomical changes in the
139 292 had no polyneuropathy or ataxia, and the sensorineural hearing loss and cataract were attributed
140 a good animal model to evaluate the role of sensorineural hearing loss and central inhibition in aud
141 ion in multiple tissues, suggesting that the sensorineural hearing loss and characteristic brain malf
142 ilitate early detection and intervention for sensorineural hearing loss and developmental delay, wher
143 nia, facial dysmorphology, ocular anomalies, sensorineural hearing loss and developmental delay.
144 dered in patients presenting with congenital sensorineural hearing loss and disorders of cornificatio
146 erturn a long-standing dogma in the study of sensorineural hearing loss and highlight the importance
147 ty of hearing-loss disorders, such as sudden sensorineural hearing loss and Meniere's disease that ar
149 autosomal recessive disorder associated with sensorineural hearing loss and pili torti, is caused by
150 d that an absence of Np65 causes early-onset sensorineural hearing loss and prevented the normal syna
151 ant mice, absence of Np65 causes early-onset sensorineural hearing loss and prevents normal neurotran
152 disorder characterized by moderate to severe sensorineural hearing loss and progressive retinitis pig
153 pmental disorder and bilateral non-syndromic sensorineural hearing loss and provide further data supp
154 racterized by progressive pontobulbar palsy, sensorineural hearing loss and respiratory insufficiency
155 syndrome type 2, characterized by congenital sensorineural hearing loss and retinitis pigmentosa (RP)
156 ree individuals with non-syndromic bilateral sensorineural hearing loss and vestibular areflexia.
157 ) gene, causing the adult-onset, progressive sensorineural hearing loss and vestibular disorder at th
158 e etiologic for the late-onset, progressive, sensorineural hearing loss and vestibular dysfunction kn
159 an follow-up of 24 months, the proportion of sensorineural hearing loss and/or neurologic sequelae we
160 e received a diagnosis of bilateral profound sensorineural hearing loss at neonatal hearing screening
161 obtained from 49 participants with moderate sensorineural hearing loss before fitting and after 3 we
162 gs to more clinically relevant conditions of sensorineural hearing loss by examining the role of frac
163 that this variant may modify the severity of sensorineural hearing loss caused by a variety of factor
165 oximately 20-30% of patients with congenital sensorineural hearing loss demonstrate radiographic abno
166 nalysis of dominantly inherited, progressive sensorineural hearing loss DFNA41 in a six-generation ki
167 oding connexin 26 (cx26) have been linked to sensorineural hearing loss either alone or as part of a
168 elin plasticity and how this could relate to sensorineural hearing loss following peripheral impairme
172 either common (hearing loss) or distinctive (sensorineural hearing loss in a child), they are importa
173 t, progressive, high-frequency, nonsyndromic sensorineural hearing loss in a large, multigenerational
174 te OPG at high levels and lack of OPG causes sensorineural hearing loss in addition to the previously
178 channel-like gene 1 (TMC1) cause progressive sensorineural hearing loss in humans and Beethoven (Tmc1
181 GED1 loss, and a diversity of conductive and sensorineural hearing loss in nearly half of AGS patient
182 53G > A), of NLRP3 causes autosomal-dominant sensorineural hearing loss in two unrelated families.
183 oss in which the cochleae are not damaged or sensorineural hearing loss in which both cochleae are re
188 e not precipitated by cold exposure and that sensorineural hearing loss is frequently also present.
192 sly unknown autosomal-recessive nonsyndromic sensorineural hearing loss locus (DFNB91) to chromosome
195 more likely than controls to have bilateral sensorineural hearing loss of 40 dB or more (unmatched 1
196 GNIFICANCE STATEMENT People with age-related sensorineural hearing loss often struggle to follow spee
197 s a higher risk of adverse neonatal outcome (sensorineural hearing loss or neurological deficits).
198 otitis media (OM), including high-frequency sensorineural hearing loss or vertigo, is not uncommon.
199 regating an autosomal dominant, progressive, sensorineural hearing loss phenotype that has been linke
200 man with a history of progressive bilateral sensorineural hearing loss presented to a neuro-ophthalm
201 trial involving 250 patients with unilateral sensorineural hearing loss presenting within 14 days of
202 year-old female has intellectual disability, sensorineural hearing loss requiring bilateral cochlear
204 transcriptional co-activator EYA4 gene cause sensorineural hearing loss that can occur in association
205 describe 2 unrelated pedigrees with MTP and sensorineural hearing loss that segregate with a DIAPH1
206 ific algorithms that predict the presence of sensorineural hearing loss using only miRNA expression p
208 sease erythrokeratoderma variabilis (EKV) or sensorineural hearing loss with/without peripheral neuro
211 oms can help predict which infants will have sensorineural hearing loss, among asymptomatic cCMV ther
212 lities, insulin-dependent diabetes mellitus, sensorineural hearing loss, and mild intellectual disabi
213 who presented with osteoporosis, cataracts, sensorineural hearing loss, and mild learning defects.
214 s much to learn about pathogenesis of sudden sensorineural hearing loss, and more clinical trials are
215 wo thirds of respondents were male, most had sensorineural hearing loss, and most were older than 50;
216 al-recessive inheritance, severe to profound sensorineural hearing loss, and partial agenesis of the
217 nction, cachetic dwarfism, photosensitivity, sensorineural hearing loss, and retinal degradation.
218 llar ataxia, early-onset cerebellar atrophy, sensorineural hearing loss, and the distinctive associat
219 etely penetrant, predominantly low-frequency sensorineural hearing loss, and the Fgfr3(P244R) mice sh
220 spontaneous attacks of vertigo, fluctuating sensorineural hearing loss, aural fullness, and tinnitus
221 cause of neurological problems, particularly sensorineural hearing loss, but data on long-term sequel
222 vide significant benefit for a wide range of sensorineural hearing loss, but no carefully controlled,
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 rg syndrome (MTS), which is characterised by sensorineural hearing loss, dystonia and blindness.
226 results in vertebral compression fractures, sensorineural hearing loss, eye defects, and heart defec
228 cted in two unrelated persons with increased sensorineural hearing loss, in the other caused by a mut
229 coustic trauma, one of the leading causes of sensorineural hearing loss, induces sensory hair cell da
230 evere form of spondylo-epiphyseal dysplasia, sensorineural hearing loss, intellectual disability and
231 sible causes of hearing loss, such as sudden sensorineural hearing loss, is important to maximize the
232 , developmental delay, brain dysmyelination, sensorineural hearing loss, nystagmus, progressive chole
233 syndrome that includes the subacute onset of sensorineural hearing loss, often accompanied by vertigo
234 verlapping clinical features, manifesting as sensorineural hearing loss, often associated with vertig
235 racterized by mental retardation, hypotonia, sensorineural hearing loss, optic atrophy, and other fea
236 autosomal dominant disorder characterized by sensorineural hearing loss, palmoplantar keratoderma, kn
237 iginally identified in Persian families with sensorineural hearing loss, regulates peroxisomal dynami
238 ic TBS patients by displaying high-frequency sensorineural hearing loss, renal cystic hypoplasia and
239 cochlea, caused by macular degeneration and sensorineural hearing loss, respectively, affect a growi
241 of hair cells or auditory neurons results in sensorineural hearing loss, the consequence of supportin
242 chronic disorder of the inner ear defined by sensorineural hearing loss, tinnitus and episodic vertig
243 ting from minutes to hours, with fluctuating sensorineural hearing loss, tinnitus, and aural pressure
244 rized by fever, chronic meningitis, uveitis, sensorineural hearing loss, urticarial skin rash, and a
245 essive disorder characterized by progressive sensorineural hearing loss, vestibular dysfunction, and
283 diagnosis and treatment for individuals with sensorineural hearing loss.SIGNIFICANCE STATEMENT Differ
284 siblings presented with autosomal recessive sensorineural hearing loss: two had high-frequency loss,
290 ng loss in older adults, is a multifactorial sensorineural loss that frequently includes a component
291 518Dfs*2) in an Italian pedigree affected by sensorineural mild-to-moderate HHL but also showing a va
292 ast, children with SLI showed no evidence of sensorineural modulation with attention, despite behavio
293 ns of mice have different predispositions to sensorineural pathway activation, we quantified expressi
294 These findings expand our understanding of sensorineural plasticity in adult vestibular organs and
295 ta3 is expressed in a distinct population of sensorineural precursor cells within the otic epithelium
296 the effects of selective attention on early sensorineural processing may give rise to the diverse se
297 ping children showed an amplification of the sensorineural response to attended as compared to unatte