scholarly journals Differential Expression of KCNQ4 in Inner Hair Cells and Sensory Neurons Is the Basis of Progressive High-Frequency Hearing Loss

2005 ◽  
Vol 25 (40) ◽  
pp. 9285-9293 ◽  
Author(s):  
K. W. Beisel
Author(s):  
Prathamesh Thangaraj Nadar Ponniah ◽  
Shahar Taiber ◽  
Michal Caspi ◽  
Tal Koffler-Brill ◽  
Amiel A. Dror ◽  
...  

AbstractStriatin, a subunit of the serine/threonine phosphatase PP2A, is a core member of the conserved striatin-interacting phosphatase and kinase (STRIPAK) complexes. The protein is expressed in the cell junctions between epithelial cells, which play a role in maintaining cell-cell junctional integrity. Since adhesion is crucial for the function of the mammalian inner ear, we examined the localization and function of striatin in the mouse cochlea. Our results show that in neonatal mice, striatin is specifically expressed in the cell-cell junctions of the inner hair cells, the receptor cells in the mammalian cochlea. Auditory brainstem response measurements of striatin-deficient mice indicated a progressive, high-frequency hearing loss, suggesting that striatin is essential for normal hearing. Moreover, scanning electron micrographs of the organ of Corti revealed a moderate degeneration of the outer hair cells in the middle and basal regions, concordant with the high-frequency hearing loss. Importantly, striatin-deficient mice show aberrant ribbon synapse maturation that may lead to the observed auditory impairment. Together, these results suggest a novel function for striatin in the mammalian auditory system.


Author(s):  
L. Sivasankari ◽  
Lalitha Subramanian

<p class="abstract"><strong>Background:</strong> Various medications have been associated with ototoxicity. Platinum containing chemotherapeutic agents are associated with cochleotoxicity, characterized by high frequency hearing loss. Cisplatin and related agents are absorbed by the cochlear hair cells, resulting in ototoxicity through the production of reactive oxygen species.</p><p class="abstract"><strong>Methods:</strong> About 67 patients, irrespective of the type of cancer, fit to undergo chemotherapy were considered for study after meticulous examination. Audiograms were taken prior to chemotherapy, at the end of each cycle of chemotherapy, and follow-up audiograms at 3 months and 6 months after completion of chemotherapy.  </p><p class="abstract"><strong>Results:</strong> Among 37% of the patients with normal hearing, 10% of the patients developed sensorineural hearing loss after treatment. Among 63% of the patients with prior mild sensorineural hearing loss, 11.8% developed worsening of hearing after completion of treatment.</p><p class="abstract"><strong>Conclusions:</strong> Audiologic monitoring is important in patients undergoing cisplatin chemotherapy and post- chemotherapy auditory monitoring is essential to rehabilitate the patients with Sensorineural hearing loss.</p>


1968 ◽  
Vol 11 (1) ◽  
pp. 204-218 ◽  
Author(s):  
Elizabeth Dodds ◽  
Earl Harford

Persons with a high frequency hearing loss are difficult cases for whom to find suitable amplification. We have experienced some success with this problem in our Hearing Clinics using a specially designed earmold with a hearing aid. Thirty-five cases with high frequency hearing losses were selected from our clinical files for analysis of test results using standard, vented, and open earpieces. A statistical analysis of test results revealed that PB scores in sound field, using an average conversational intensity level (70 dB SPL), were enhanced when utilizing any one of the three earmolds. This result was due undoubtedly to increased sensitivity provided by the hearing aid. Only the open earmold used with a CROS hearing aid resulted in a significant improvement in discrimination when compared with the group’s unaided PB score under earphones or when comparing inter-earmold scores. These findings suggest that the inclusion of the open earmold with a CROS aid in the audiologist’s armamentarium should increase his flexibility in selecting hearing aids for persons with a high frequency hearing loss.


Author(s):  
Wei Gong ◽  
Liangliang Zhao ◽  
Ling Li ◽  
Thais C. Morata ◽  
Wei Qiu ◽  
...  

A survey was administered to 385 noise-exposed workers from an auto parts factory and 1268 non-noise-exposed health department employees in China. Individual 8 h A-weighted equivalent sound levels (LAeq,8h), earplug personal attenuation ratings (PARs), and pure-tone audiometric tests were performed. The average LAeq,8h of noise-exposed workers was 87 dB (A) with a mean PAR of 7 dB. The prevalence of high-frequency hearing loss was 65% for noise-exposed workers and 33% for the non-noise-exposed employees. The use of earplugs had no observable effect on the prevalence of high-frequency hearing loss of the study participants (OR 0.964, 95% CI 0.925–1.005, p = 0.085). No significant relationship between the effectiveness offered by earplug use and high-frequency hearing thresholds at 3, 4, and 6 kHz was found (t = −1.54, p = 0.125). The mandatory requirement of earplug use without individualized training on how to wear HPDs correctly had no detectable effect on the prevention of hearing loss at the auto parts factory. The hearing conservation program at the surveyed factory was not effective. Periodic hearing tests, earplug fit testing, expanding the offer of different types of hearing protection, and employee education about the importance of protecting their hearing were recommended to the occupational health and safety program.


1998 ◽  
Vol 79 (5) ◽  
pp. 2603-2614 ◽  
Author(s):  
Stephanie Carlson ◽  
James F. Willott

Carlson, Stephanie and James F. Willott. Caudal pontine reticular formation of C57BL/6J mice: responses to startle stimuli, inhibition by tones, and plasticity. J. Neurophysiol. 79: 2603–2614, 1998. C57BL/6J (C57) mice were used to examine relationships between the behavioral acoustic startle response (ASR) and the responses of neurons in the caudal pontine reticular formation (PnC) in three contexts: 1) responses evoked by basic startle stimuli; 2) the prepulse inhibition (PPI) paradigm; and 3) the effects of high-frequency hearing loss and concomitant neural plasticity that occurs in middle-aged C57 mice. 1) Responses (evoked action potentials) of PnC neurons closely paralleled the ASR with respect to latency, threshold, and responses to rapidly presented stimuli. 2) “Neural PPI” (inhibition of responses evoked by a startle stimulus when preceded by a tone prepulse) was observed in all PnC neurons studied. 3) In PnC neurons of 6-mo-old mice with high-frequency (>20 kHz) hearing loss, neural PPI was enhanced with 12- and 4-kHz prepulses, as it is behaviorally. These are frequencies that have become “overrepresented” in the central auditory system of 6-mo-old C57 mice. Thus neural plasticity in the auditory system, induced by high-frequency hearing loss, is correlated with increased salience of the inhibiting tones in both behavioral and neural PPI paradigms.


PLoS ONE ◽  
2016 ◽  
Vol 11 (3) ◽  
pp. e0151467 ◽  
Author(s):  
Abhijit Dandapat ◽  
Benjamin J. Perrin ◽  
Christine Cabelka ◽  
Maria Razzoli ◽  
James M. Ervasti ◽  
...  

2005 ◽  
Vol 16 (09) ◽  
pp. 653-661
Author(s):  
Francis Kuk ◽  
Denise Keenan ◽  
Chi-Chuen Lau ◽  
Nick Dinulescu ◽  
Richard Cortez ◽  
...  

The present study compared differences in subjective and objective performance in completely-in-the-canal (CIC) hearing aids with conventional uniform 1.5 mm parallel vents and another with a reverse horn vent where the diameter increased from 1.5 mm on the lateral faceplate to 3 mm on the medial opening of the hearing aid. Nine hearing-impaired persons with a high-frequency hearing loss participated. The test battery included unaided in situ thresholds, amount of available gain before feedback, speech in quiet, speech in noise (HINT), subjective ratings of hollowness and tolerance, objective measures of the occlusion effect, and real-ear aided response. Results showed less available gain before feedback but less occlusion effect for subjective ratings and objective measures with the reverse horn vent. This type of vent design may be useful to increase the effective vent diameter of custom (including CIC) hearing aids.


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