scholarly journals Gene therapy for hereditary hearing loss by SLC26A4 mutations in mice reveals distinct functional roles of pendrin in normal hearing

Theranostics ◽  
2019 ◽  
Vol 9 (24) ◽  
pp. 7184-7199 ◽  
Author(s):  
Min-A Kim ◽  
Sung Huhn Kim ◽  
Nari Ryu ◽  
Ji-Hyun Ma ◽  
Ye-Ri Kim ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Hearing Loss ◽  
Normal Hearing ◽  
Hereditary Hearing Loss ◽  
Functional Roles

Gene Therapy for Hereditary Hearing Loss

2013 ◽  
Vol 17 (1) ◽  
pp. 5
Author(s):  
Devin S. McDougald ◽  
Lisa L. Cunningham ◽  
Wade W. Chien
Keyword(s):  
Gene Therapy ◽  
Hearing Loss ◽  
Hereditary Hearing Loss

When Hearing Does Not Mean Understanding: On the Neural Processing of Syntactically Complex Sentences by Listeners With Hearing Loss

2021 ◽  
pp. 1-13
Author(s):  
Margreet Vogelzang ◽  
Christiane M. Thiel ◽  
Stephanie Rosemann ◽  
Jochem W. Rieger ◽  
Esther Ruigendijk
Keyword(s):  
Hearing Loss ◽  
Sentence Processing ◽  
Secondary Task ◽  
Sentence Comprehension ◽  
Inferior Frontal Gyrus ◽  
Normal Hearing ◽  
Cognitive Demands ◽  
Sentence Complexity ◽  
Complex Sentences ◽  
And Task

Purpose Adults with mild-to-moderate age-related hearing loss typically exhibit issues with speech understanding, but their processing of syntactically complex sentences is not well understood. We test the hypothesis that listeners with hearing loss' difficulties with comprehension and processing of syntactically complex sentences are due to the processing of degraded input interfering with the successful processing of complex sentences. Method We performed a neuroimaging study with a sentence comprehension task, varying sentence complexity (through subject–object order and verb–arguments order) and cognitive demands (presence or absence of a secondary task) within subjects. Groups of older subjects with hearing loss ( n = 20) and age-matched normal-hearing controls ( n = 20) were tested. Results The comprehension data show effects of syntactic complexity and hearing ability, with normal-hearing controls outperforming listeners with hearing loss, seemingly more so on syntactically complex sentences. The secondary task did not influence off-line comprehension. The imaging data show effects of group, sentence complexity, and task, with listeners with hearing loss showing decreased activation in typical speech processing areas, such as the inferior frontal gyrus and superior temporal gyrus. No interactions between group, sentence complexity, and task were found in the neuroimaging data. Conclusions The results suggest that listeners with hearing loss process speech differently from their normal-hearing peers, possibly due to the increased demands of processing degraded auditory input. Increased cognitive demands by means of a secondary visual shape processing task influence neural sentence processing, but no evidence was found that it does so in a different way for listeners with hearing loss and normal-hearing listeners.

An Evaluation of the Relationship between Retinal Nerve Fiber Thickness, Cochlear Nerve Thickness, the Level of Tinnitus, and Hearing Loss in Unilateral Tinnitus Patients

2021 ◽  
pp. 1-8
Author(s):  
Mustafa Avcu ◽  
Mehmet Metin ◽  
Raşit Kılıç ◽  
Muhammed Alpaslan
Keyword(s):  
Hearing Loss ◽  
Nerve Fiber ◽  
Normal Hearing ◽  
Cochlear Nerve ◽  
Significant Difference ◽  
The Relationship ◽  
Fiber Thickness ◽  
Moderate Hearing Loss ◽  
Group 1 ◽  
Retinal Nerve Fiber

Background: In this study, optic coherence tomography (OCT) examination was performed to check whether there was any interaction between ophthalmic axonal structures in unilateral tinnitus patients, and the relationship between optic nerve thickness and cochlear nerve thickness was evaluated. Objective: The aim of the study was to evaluate the relatioship between hearing loss, tinnitus, and nerve thicknesses. Study Design: Prospective study. Setting: Tertiary referral university hospital. Patients: The study included 88 patients with unilateral tinnitus, for which no organic cause could be found in physical examination, psychiatric evaluation, or with imaging methods. Study groups were formed of the tinnitus side and control groups were formed of the healthy side as follows: Group 1 (Non-tinnitus side normal hearing values – n = 30), Group 2 (non-tinnitus side minimal hearing loss – n = 27), Group 3 (non-tinnitus side moderate hearing loss – n = 31), Group 4 (tinnitus side normal hearing values – n = 25), Group 5 (tinnitus side minimal hearing loss – n = 25), and Group 6 (tinnitus side moderate hearing loss – n = 38). Intervention: Retinal nerve fiber layer (RNFL) thickness was evaluated with OCT, and the cochlear nerve cross-sectional area was evaluated with MRI. Main Outcome Measures: RNFL measurements were taken with OCT from the subfoveal area (RNFL-SF) and 1.5 mm temporal to the fovea (RNFL-T µm) and nasal (RNFL-N µm) sectors. On MRI, 3 measurements were taken along the nerve from the cerebellopontine angle as far as the internal auditory canal, and the mean value of these 3 measurements was calculated. Results: When the groups were evaluated in respect of cochlear nerve thickness, a significant difference was seen between Group 1 and both the groups with hearing loss and the tinnitus groups. In the subgroup analysis, a statistically significant difference was determined between Group 1 and Groups 3, 4, 5, and 6 (p = 0.013, p = 0.003, p < 0.001, and p < 0.001, respectively). When the groups were evaluated in respect of the RNFL-SF (µm), RNFL-T (µm), and RNFL-N (µm) values, the differences were determined to be statistically significant (p < 0.001 for all). In the correlation analysis, a negative correlation was determined between hearing loss and cochlear nerve diameter (r: −0.184, p = 0.014), and RNFL-N (r: −0.272, p < 0.001) and between tinnitus and cochlear nerve diameter (r: −0.536, p < 0.001), and RNFL-T (r: −0.222, p < 0.009). Conclusion: The study results clearly showed a relationship between cochlear nerve fiber thickness and hearing loss and the severity of tinnitus in cases with unilateral tinnitus and that there could be neurodegenerative factors in the disease etiology. A similar relationship seen with the RNFL supports the study hypothesis.

Genetic etiology of hereditary hearing loss in the Gulf Cooperation Council countries

2021 ◽  
Author(s):  
Abdullah Al Mutery ◽  
Mona Mahfood ◽  
Jihen Chouchen ◽  
Abdelaziz Tlili
Keyword(s):  
Hearing Loss ◽  
Gulf Cooperation Council ◽  
Genetic Etiology ◽  
Hereditary Hearing Loss

scholarly journals Susceptibility to Residual Inhibition Is Associated With Hearing Loss and Tinnitus Chronicity

2021 ◽  
Vol 25 ◽  
pp. 233121652098630
Author(s):  
S. Hu ◽  
L. Anschuetz ◽  
D. A. Hall ◽  
M. Caversaccio ◽  
W. Wimmer
Keyword(s):  
Hearing Loss ◽  
Narrow Band ◽  
Acoustic Stimulation ◽  
Normal Hearing ◽  
Short Term ◽  
Factors Associated ◽  
Band Noise ◽  
Comparator Group ◽  
Residual Inhibition ◽  
Subject Comparison

Residual inhibition, that is, the temporary suppression of tinnitus loudness after acoustic stimulation, is a frequently observed phenomenon that may have prognostic value for clinical applications. However, it is unclear in which subjects residual inhibition is more likely and how stable the effect of inhibition is over multiple repetitions. The primary aim of this work was to evaluate the effect of hearing loss and tinnitus chronicity on residual inhibition susceptibility. The secondary aim was to investigate the short-term repeatability of residual inhibition. Residual inhibition was assessed in 74 tinnitus subjects with 60-second narrow-band noise stimuli in 10 consecutive trials. The subjects were assigned to groups according to their depth of suppression (substantial residual inhibition vs. comparator group). In addition, a categorization in normal hearing and hearing loss groups, related to the degree of hearing loss at the frequency corresponding to the tinnitus pitch, was made. Logistic regression was used to identify factors associated with susceptibility to residual inhibition. Repeatability of residual inhibition was assessed using mixed-effects ordinal regression including poststimulus time and repetitions as factors. Tinnitus chronicity was not associated with residual inhibition for subjects with hearing loss, while a statistically significant negative association between tinnitus chronicity and residual inhibition susceptibility was observed in normal hearing subjects (odds ratio: 0.63; p = .0076). Moreover, repeated states of suppression can be stably induced, reinforcing the use of residual inhibition for within-subject comparison studies.

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