Auditory memory span in Mandarin-speaking preschoolers with congenital hearing loss: Impact of task structure

2019 ◽  
Vol 33 (sup1) ◽  
pp. 75-95 ◽  
Author(s):  
Ming Lo ◽  
Yi-Xiu Lin ◽  
Shiou-Yuan Chen ◽  
Pei-Hua Chen ◽  
Cho-Yuan Lin
Keyword(s):  
Hearing Loss ◽  
Memory Span ◽  
Auditory Memory ◽  
Task Structure ◽  
Congenital Hearing Loss

Auditory Sequential Organization Among Children With and Without a Hearing Loss

1999 ◽  
Vol 42 (3) ◽  
pp. 553-567 ◽  
Author(s):  
Benoît Jutras ◽  
Jean-Pierre Gagné
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Memory Span ◽  
Normal Hearing ◽  
Auditory Memory ◽  
Sensorineural Hearing ◽  
Verbal Stimuli ◽  
Acoustic Stimuli ◽  
Sequential Organization ◽  
Significant Difference

The present investigation examined the ability of children with and without a hearing loss to correctly reproduce sequences of acoustic stimuli that varied in number, temporal spacing, and type. Forty-eight children took part in the investigation. They were divided into four groups: two groups of 6- and 7-year-old children, 12 with normal hearing and 12 with a sensorineural hearing loss; and two groups of 9- and 10-year-old children, 12 with normal hearing and 12 with a sensorineural hearing loss. All of the children completed auditory temporal sequencing tasks with verbal (/ba/ and /da/) and nonverbal (a 1-kHz pure tone and a wide band noise) acoustic stimuli. For the 6- and 7-year-old children, the results revealed a significant difference between the children with a hearing loss and their peers with normal hearing for immediate recall of verbal sequences. There were no significant differences in performance between the children with a hearing loss and their peers with normal hearing on the nonverbal sequencing tasks or on the nonverbal and verbal memory span tasks. For the 9- and 10-year-old children, the results did not show any significant differences in performance between the two groups of children for the reproduction of sequences containing more than two verbal or nonverbal elements nor for the auditory memory span task when the sequences consisted of verbal stimuli. For the recall of two verbal stimuli with a variable interstimulus interval (ISI) duration, the results showed that the children with a hearing loss experienced more difficulty than the children with normal hearing. Overall, the results indicated that on the auditory sequential organization tasks, the poorer performance of the children with a hearing loss is likely attributable to auditory perceptual processing deficits rather than to poorer short-term memory capabilities. Also, an analysis of the data revealed that the older children obtained significantly better results than the younger children on auditory sequential organization tasks.

Short-Term Auditory Memory, Oral Perception, and Experimental Sound Learning

1969 ◽  
Vol 12 (1) ◽  
pp. 185-192 ◽  
Author(s):  
John L. Locke
Keyword(s):  
Memory Span ◽  
Auditory Memory ◽  
Span Task ◽  
Short Term ◽  
Learning Framework ◽  
Sound Learning ◽  
Oral Perception

Ten children with high scores on an auditory memory span task were significantly better at imitating three non-English phones than 10 children with low auditory memory span scores. An additional 10 children with high scores on an oral stereognosis task were significantly better at imitating two of the three phones than 10 children with low oral stereognosis scores. Auditory memory span and oral stereognosis appear to be important subskills in the learning of new articulations, perhaps explaining their appearance in the literature as “etiologies” of disordered articulation. Although articulation development and the experimental acquisition of non-English phones have certain obvious differences, they seem to share some common processes, suggesting that the sound learning framework may be an efficacious technique for revealing otherwise inaccessible information.

scholarly journals A novel case of concurrent occurrence of demyelinating-polyneuropathy-causing PMP22 duplication and SOX10 gene mutation producing severe hypertrophic neuropathy

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nozomu Matsuda ◽  
Koushi Ootsuki ◽  
Shunsuke Kobayashi ◽  
Ayaka Nemoto ◽  
Hitoshi Kubo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Neuromuscular Disorders ◽  
Whole Body ◽  
Congenital Hearing Loss ◽  
Severe Phenotype ◽  
Demyelinating Neuropathy ◽  
Magnetic Resonance Neurography ◽  
Peripheral Myelin Protein 22 ◽  
Hypertrophic Neuropathy

Abstract Background Hereditary motor and sensory neuropathy, also referred to as Charcot–Marie–Tooth disease (CMT), is most often caused by a duplication of the peripheral myelin protein 22 (PMP22) gene. This duplication causes CMT type 1A (CMT1A). CMT1A rarely occurs in combination with other hereditary neuromuscular disorders. However, such rare genetic coincidences produce a severe phenotype and have been reported in terms of “double trouble” overlapping syndrome. Waardenburg syndrome (WS) is the most common form of a hereditary syndromic deafness. It is primarily characterized by pigmentation anomalies and classified into four major phenotypes. A mutation in the SRY sex determining region Y-box 10 (SOX10) gene causes WS type 2 or 4 and peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, WS, and Hirschsprung disease. We describe a 11-year-old boy with extreme hypertrophic neuropathy because of a combination of CMT1A and WS type 2. This is the first published case on the co-occurrence of CMT1A and WS type 2. Case presentation The 11-year-old boy presented with motor developmental delay and a deterioration in unstable walking at 6 years of age. In addition, he had congenital hearing loss and heterochromia iridis. The neurological examination revealed weakness in the distal limbs with pes cavus. He was diagnosed with CMT1A by the fluorescence in situ hybridization method. His paternal pedigree had a history of CMT1A. However, no family member had congenital hearing loss. His clinical manifestation was apparently severe than those of his relatives with CMT1A. In addition, a whole-body magnetic resonance neurography revealed an extreme enlargement of his systemic cranial and spinal nerves. Subsequently, a genetic analysis revealed a heterozygous frameshift mutation c.876delT (p.F292Lfs*19) in the SOX10 gene. He was eventually diagnosed with WS type 2. Conclusions We described a patient with a genetically confirmed overlapping diagnoses of CMT1A and WS type 2. The double trouble with the genes created a significant impact on the peripheral nerves system. Severe phenotype in the proband can be attributed to the cumulative effect of mutations in both PMP22 and SOX10 genes, responsible for demyelinating neuropathy.

Evaluation of DNA damages in congenital hearing loss patients

2021 ◽  
Vol 822 ◽  
pp. 111744
Author(s):  
Özge Çağlar ◽  
Hayal Çobanoğlu ◽  
Atilla Uslu ◽  
Akın Çayır
Keyword(s):  
Hearing Loss ◽  
Congenital Hearing Loss ◽  
Dna Damages

scholarly journals Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Next Generation Sequencing ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Transverse Diameter ◽  
Congenital Hearing Loss ◽  
Canal Stenosis ◽  
Ear Anomalies ◽  
Generation Sequencing

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient’s hearing was rehabilitated with bilateral cochlear implantation.

Mild matters: parental insights into the conundrums of managing mild congenital hearing loss

2021 ◽  
pp. 1-7
Author(s):  
Jing Jing Lin ◽  
Lynn Gillam ◽  
Libby Smith ◽  
Peter Carew ◽  
Alison King ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Congenital Hearing Loss

CRISPR/Cas9-mediated genome editing of splicing mutation causing congenital hearing loss

Gene ◽  
2019 ◽  
Vol 703 ◽  
pp. 83-90 ◽  
Author(s):  
Nari Ryu ◽  
Min-A Kim ◽  
Deok-Gyun Choi ◽  
Ye-Ri Kim ◽  
Jong Kyung Sonn ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Genome Editing ◽  
Splicing Mutation ◽  
Congenital Hearing Loss

Congenital Viral Induced Hearing Loss

1995 ◽  
Vol 112 (5) ◽  
pp. P192-P192
Author(s):  
Melvin Strauss
Keyword(s):  
Hearing Loss ◽  
Experimental Studies ◽  
Congenital Hearing Loss ◽  
Educational Objectives ◽  
Congenital Cytomegalovirus

Educational objectives: To understand the nature and magnitude of congenital hearing loss with a specific understanding of viral causes of congenital hearing loss and to be knowledgeable of all aspects of congenital cytomegalovirus labyrinthitis including efforts at prevention and experimental studies.

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