Search results for "Deaf"
showing 10 items of 104 documents
Amusic does not mean unmusical: Beat perception and synchronization ability despite pitch deafness
2013
Pitch deafness, the most commonly known form of congenital amusia, refers to a severe deficit in musical pitch processing (i.e., melody discrimination and recognition) that can leave time processing--including rhythm, metre, and "feeling the beat"--preserved. In Experiment 1, we show that by presenting musical excerpts in nonpitched drum timbres, rather than pitched piano tones, amusics show normal metre recognition. Experiment 2 reveals that body movement influences amusics' interpretation of the beat of an ambiguous drum rhythm. Experiment 3 and a subsequent exploratory study show an ability to synchronize movement to the beat of popular dance music and potential for improvement when give…
Phenotypical features of two patients diagnosed with PHARC syndrome and carriers of a new homozygous mutation in the ABHD12 gene.
2018
Abstract PHARC (Polyneuropathy, Hearing loss, Ataxia, Retinitis pigmentosa and Cataracts) (MIM# 612674 ) is an autosomal recessive neurodegenerative disease caused by mutations in the ABHD12 gene. We evaluated two Spanish siblings affected with pes cavus, sensorimotor neuropathy, hearing loss, retinitis pigmentosa and juvenile cataracts in whom the genetic test of ABHD12 revealed a novel homozygous frameshift mutation, c.211_223del (p.Arg71Tyrfs*26). The earliest clinical manifestation in these patients was a demyelinating neuropathy manifested with a Charcot-Marie-Tooth phenotype over three decades. Progressive hearing loss, cataracts and retinitis pigmentosa appeared after the age of 30. …
Phonological-Lexical Feedback during Early Abstract Encoding: The Case of Deaf Readers.
2016
In the masked priming technique, physical identity between prime and target enjoys an advantage over nominal identity in nonwords (GEDA-GEDA faster than geda-GEDA). However, nominal identity overrides physical identity in words (e.g., REAL-REAL similar to real-REAL). Here we tested whether the lack of an advantage of the physical identity condition for words was due to top-down feedback from phonological-lexical information. We examined this issue with deaf readers, as their phonological representations are not as fully developed as in hearing readers. Results revealed that physical identity enjoyed a processing advantage over nominal identity not only in nonwords but also in words (GEDA-GE…
Early use of phonological codes in deaf readers: An ERP study.
2017
Previous studies suggest that deaf readers use phonological information of words when it is explicitly demanded by the task itself. However, whether phonological encoding is automatic remains controversial. The present experiment examined whether adult congenitally deaf readers show evidence of automatic use of phonological information during visual word recognition. In an ERP masked priming lexical decision experiment, deaf participants responded to target words preceded by a pseudohomophone (koral - CORAL) or an orthographic control prime (toral - CORAL). Responses were faster for the pseudohomophone than for the orthographic control condition. The N250 and N400 amplitudes were reduced fo…
Musical familiarity in congenital amusia: Evidence from a gating paradigm
2013
Congenital amusia has been described as a lifelong deficit of music perception and production, notably including amusic individuals' difficulties to recognize a familiar tune without the aid of lyrics. The present study aimed to evaluate whether amusic individuals might have acquired long-term knowledge of familiar music, and to test for the minimal amount of acoustic information necessary to access this knowledge (if any) in amusia. Segments of familiar and unfamiliar instrumental musical pieces were presented with increasing duration (250, 500, 1000 msec etc.), and participants provided familiarity judgments for each segment. Results showed that amusic individuals succeeded in differentia…
Examining the contribution of motor movement and language dominance to increased left lateralization during sign generation in native signers
2016
Highlights • We tested hemispheric lateralization for language in deaf native signers. • Signers were more strongly left lateralized for overt than covert sign generation. • We found stronger left lateralization for BSL than for English production. • Stronger left lateralization for BSL is not driven by motoric activity alone. • Stronger left lateralization is not driven by language dominance.
A method for determining precise electrical hearing thresholds in cochlear implant users
2018
A psychoacoustic procedure designed for the precise assessment of perceptional threshold (T-level) in cochlear implant (CI) users is presented. The impact of this procedure on speech perception was investigated.Individual T-level measurements were obtained with the proposed procedure and three different speech processor fitting conditions were applied: (1) fitting familiar to the subject, T-levels unchanged, (2) T-level set to thresholds determined with the new procedure, (3) T-level set to thresholds determined with the new procedure, but T-level is decreased by 10 clinical units (CU). The impact of the different fitting conditions was measured by means of categorical loudness scaling (CLS…
Management of head and neck paragangliomas: review of 120 patients.
2008
Head and neck paragangliomas (PGL) are rare, mostly benign tumors. About 10% to 15% of PGL are caused by mutations in the succinate dehydrogenase genes B, C, or D and may appear multifocally.A retrospective review of 120 patients with 146 head and neck PGL, including 46 carotid body tumors (CBT), 13 vagal tumors, 55 jugulotympanic tumors (JTT), 25 tympanic tumors (TT) and 7 tumors in other locations are included. The internal carotid artery was preserved in 97.5% of CBT resections. Preservation of hearing was achieved in 92% of JTT and 88% of TT resections.According to our experience, the treatment of PGL must be individualized, taking into account the patient's age, medical condition, tumo…
Treatment of sudden sensorineural hearing loss with transtympanic injection of steroids as single therapy: a randomized clinical study.
2011
The aim of this study was to verify the efficacy and the safety of transtympanic dexamethasone to treat sudden sensorineural hearing loss as first and single drug method. Considering ethical implication of performing a mininvasive procedure on middle ear, we matched such proposed treatment with systemic prednisone administration that represents the widest adopted protocol. Randomized prospective study was conducted. The inclusion criterion was a sudden sensorineural hearing loss of at least 30 dB across three contiguous frequencies over a period of 24 h. Group A received transtympanic steroid injections; Group B received oral administration of steroids. 25 patients were treated with transty…
Intrafamilial variability of the deafness and goiter phenotype in Pendred syndrome caused by a T416P mutation in the SLC26A4 gene.
2004
AbstractPendred syndrome (PS) is the most common cause of syndromic deafness, accounting for more than 5% of all autosomal-recessive hearing loss cases. It is characterized by bilateral sensorineural hearing loss and by goiter with or without hypothyroidism. Mutations in the SLC26A4 gene cause both classical PS and deafness associated with an enlarged vestibular aqueduct without goiter.To investigate a possible genotype-phenotype correlation in PS, we performed a detailed clinical and genetic study in three adult German sibs with typical PS caused by a common homozygous SLC26A4 mutation, T416P. An audiological long-term follow-up of 23 yr showed that the mutation T416P is associated with a …