The effects of sensory hearing loss on cochlear filter times estimated from auditory brainstem response latencies

1998 ◽  
Vol 104 (4) ◽  
pp. 2280-2289 ◽  
Author(s):  
Manuel Don ◽  
Curtis W. Ponton ◽  
Jos J. Eggermont ◽  
Betty Kwong
2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Xi Gu ◽  
Daqi Wang ◽  
Zhijiao Xu ◽  
Jinghan Wang ◽  
Luo Guo ◽  
...  

Abstract Background Aging, noise, infection, and ototoxic drugs are the major causes of human acquired sensorineural hearing loss, but treatment options are limited. CRISPR/Cas9 technology has tremendous potential to become a new therapeutic modality for acquired non-inherited sensorineural hearing loss. Here, we develop CRISPR/Cas9 strategies to prevent aminoglycoside-induced deafness, a common type of acquired non-inherited sensorineural hearing loss, via disrupting the Htra2 gene in the inner ear which is involved in apoptosis but has not been investigated in cochlear hair cell protection. Results The results indicate that adeno-associated virus (AAV)-mediated delivery of CRISPR/SpCas9 system ameliorates neomycin-induced apoptosis, promotes hair cell survival, and significantly improves hearing function in neomycin-treated mice. The protective effect of the AAV–CRISPR/Cas9 system in vivo is sustained up to 8 weeks after neomycin exposure. For more efficient delivery of the whole CRISPR/Cas9 system, we also explore the AAV–CRISPR/SaCas9 system to prevent neomycin-induced deafness. The in vivo editing efficiency of the SaCas9 system is 1.73% on average. We observed significant improvement in auditory brainstem response thresholds in the injected ears compared with the non-injected ears. At 4 weeks after neomycin exposure, the protective effect of the AAV–CRISPR/SaCas9 system is still obvious, with the improvement in auditory brainstem response threshold up to 50 dB at 8 kHz. Conclusions These findings demonstrate the safe and effective prevention of aminoglycoside-induced deafness via Htra2 gene editing and support further development of the CRISPR/Cas9 technology in the treatment of non-inherited hearing loss as well as other non-inherited diseases.


2019 ◽  
Vol 35 (2) ◽  
Author(s):  
Muhammad Azeem Aslam ◽  
Adeela Javed ◽  
Abdul Moiz

Objectives: To compare the hearing thresholds obtained with auditory brainstem response (ABR) and auditory steady state response (ASSR) audiometry in children with hearing loss. Methods: Hearing thresholds were obtained by ABR and ASSR in children who presented with suspicion of deafness at Ear, nose & throat department of Al-Nafees Medical College Hospital Islamabad, between January to August 2018. The mean hearing thresholds obtained by two tests were compared within each category of severity of deafness. Time taken by both tests was also compared. Results: A total of 57 patients (114 ears) were included in the study. Among them 27 (47.4%) were male and 30 (52.6%) were female. The mean age of patients at presentation was 42 months (±30.9) with age range from one to 12 years. Mean hearing thresholds obtained by click ABR, chirp ABR, ASSR (1, 2, 4 kHz) & ASSR (0.5, 1, 2, 4 kHz) was 56.25 (±27.61), 58.88 (±27.44), 58.03 (±21.26) & 56.35 (±22.86) respectively. Mean thresholds were comparable between click ABR & ASSR (1, 2, 4 kHz) and between chirp ABR & ASSR (0.5, 1, 2, 4 kHz) in all degrees of hearing loss categories except in those patients with normal hearing thresholds. The mean time taken by clicks ABR, chirp ABR and ASSR were four minutes seven seconds, three minutes 15 seconds and 16 minutes and 7 seconds respectively. Conclusions: Hearing thresholds obtained by ABR and ASSR are comparable in all categories of severity of hearing loss. The time taken by ABR is less as compared to ASSR. How to cite this:Aslam MA, Javed A, Moiz A. Comparison of auditory brainstem response and auditory steady state response audiometry by evaluating the hearing thresholds obtained in children with different severity of hearing loss. Pak J Med Sci. 2019;35(2):---------.   doi: https://doi.org/10.12669/pjms.35.2.688 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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