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The functional status of central neural pathways, in particular their susceptibility to plasticity and functional reorganization, may influence speech performance of deaf cochlear implant users. In this paper, we sought to determine how brain metabolic activity measured before implantation relates to cochlear implantation outcome, that is, speech(More)
In congenitally deaf children, chronological age is generally accepted as a critical factor that affects successful rehabilitation following cochlear implantation (CI). However, a wide variance among patients is known to exist regardless of the age at CI [Sarant, J.Z., Blamey, P.J., Dowell, R.C., Clark, G.M., Gibson, W.P., 2001. Variation in speech(More)
CONCLUSION KCNQ1/KCNE1 K(+) channels and P2Y(4) receptors are expressed in the apical membrane of rat strial marginal cells from postnatal day 1 (P1) and maintained throughout development. OBJECTIVES The purpose of the present study was to investigate the developmental expression of KCNQ1/KCNE1 K(+) channel and of P2Y(4), which is an important(More)
CONTEXT Enlargement of the vestibular aqueduct (EVA) is a commonly detected inner ear anomaly related to hearing loss and often associated with mutations of SLC26A4 encoding pendrin, a transmembrane exchanger of Cl(-), I(-), and HCO(3)(-). Here we describe the phenotypes of 27 Korean EVA subjects and their SLC26A4 genotypes determined by bidirectional(More)
OBJECTIVES This study was conducted to examine improvements of cognitive abilities after cochlear implantation in deaf children. We also examined the psychosocial factors that predict good outcomes of cochlear implantation. DESIGN A neuropsychological test battery was administered to 17 deaf children (mean age, 7 yr 2 mo) before receiving the cochlear(More)
OBJECTIVE To propose management options for cochlear implantation in chronic otitis media based on our experiences. MATERIAL AND METHODS A retrospective review of 418 cochlear implantations performed by the 2 senior authors between November 1988 and February 2004 was conducted. Nine patients who had chronic otitis media in the ear to be implanted were(More)
  Cerebellar Purkinje cells (PCs) are the sole output of the cerebellar cortex and function as key to a variety of learning-related behaviours by integrating multimodal afferent inputs. Intrinsic membrane excitability of neurons determines the input-output relationship, and therefore governs the functions of neural circuits. Cerebellar vermis consists of(More)
Identification of causative genes for hereditary nonsyndromic hearing loss (NSHL) is important to decide treatment modalities and to counsel the patients. Due to the genetic heterogeneity in sensorineural genetic disorders, the high-throughput method can be adapted for the efficient diagnosis. To this end, we designed a new diagnostic pipeline to screen all(More)
The genetic heterogeneity of sensorineural hearing loss is a major hurdle to the efficient discovery of disease-causing genes. We designed a multiphasic analysis of copy number variation (CNV), linkage, and single nucleotide variation (SNV) of whole exome sequencing (WES) data for the efficient discovery of mutations causing nonsyndromic hearing loss(More)
Speechreading is a visual communicative skill for perceiving speech. In this study, we tested the effects of speech experience and deafness on the speechreading neural network in normal hearing controls and in two groups of deaf patients who became deaf either before (prelingual deafness) or after (postlingual deafness) auditory language acquisition.(More)