Auditory Brainstem Response Testing in Neurodiagnosis: Structure versus Function

2012 ◽  
Vol 23 (04) ◽  
pp. 269-275 ◽  
Author(s):  
Judy L. Peterein ◽  
J. Gail Neely
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Auditory Brainstem Response ◽  
Accurate Determination ◽  
Auditory Brainstem ◽  
Structural Abnormality ◽  
Patient Counseling ◽  
Cochlear Hearing Loss ◽  
Brainstem Response ◽  
Magnetic Resonance Imaging Mri

This case report describes a 44-yr-old female referred by an outside facility who presented with progressive hearing loss in her left ear. Magnetic resonance imaging (MRI) results were normal, but a battery of audiological tests suggested neural hearing loss in the left ear. Following diagnosis of left neural hearing loss, the patient was successfully fit with a hearing aid on the left ear.This case report underlines the importance of using a battery of medical, radiologic, and audiological tests in the accurate determination of hearing loss site of lesion. Obvious retrocochlear dysfunction was revealed via auditory brainstem response (ABR) testing. MRI did not reveal underlying structural abnormality. Without the addition of the ABR to the diagnostic test battery, a cochlear hearing loss site of lesion would most likely have been diagnosed. Accurate diagnosis of hearing loss site of lesion is critical for patient counseling and treatment as well as for patient follow-up and monitoring.

scholarly journals Efficiency of Melatonin as a Sedative for Auditory Brainstem Response in Children

2020 ◽  
Vol 10 (2) ◽  
pp. 50-54
Author(s):  
Anass Chaouki ◽  
Zineb El Krimi ◽  
Amine Mkhatri ◽  
Oukessou Youssef ◽  
Sami Rouadi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
General Anesthesia ◽  
Auditory Brainstem Response ◽  
Auditory Brainstem ◽  
Psychomotor Development ◽  
Testing Method ◽  
Significant Difference ◽  
Brainstem Response ◽  
Pediatric Audiology ◽  
Magnetic Resonance Imaging Mri

Introduction—Although auditory brainstem response (ABR) testing is among the most frequently used investigations in pediatric audiology and it often requires sedation or general anesthesia. In recent years, melatonin has been successfully used as an alternative way of inducing sleep, particularly in children undergoing magnetic resonance imaging (MRI) or electroencephalography (EEG). Purpose—To assess the effectiveness of orally administered melatonin as an alternative to sedation or general anesthesia during ABR testing. Method—In total, 33 children with suspected hearing loss underwent ABR tests in melatonin-induced sleep. Each patient received an initial dose of 5 mg, which was re-administered in case of failure to obtain sleep. Click-induced ABR tests were performed on both ears. Results—ABR tests were successfully performed in 72.7% of the patients. The average total length of time needed to obtain sleep and complete the ABR testing was 45 min. There was no significant difference between the patients who completed the examination and those who did not in terms of age or psychomotor development. There was a statistically significant association between receiving a maintenance dose and successful completion of the test (p < 0.001). There was also a significant connection between the degree of hearing loss and the success rate of the ABR tests (p < 0.001). Conclusions—Melatonin-induced sleep is a good and safer alternative to anesthesia to perform ABR testing in young children. It is easily administered, tolerated by the patients, and accepted by parents.

scholarly journals Prevention of acquired sensorineural hearing loss in mice by in vivo Htra2 gene editing

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Xi Gu ◽  
Daqi Wang ◽  
Zhijiao Xu ◽  
Jinghan Wang ◽  
Luo Guo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Protective Effect ◽  
Hair Cell ◽  
Sensorineural Hearing Loss ◽  
Auditory Brainstem Response ◽  
Gene Editing ◽  
Auditory Brainstem ◽  
Sensorineural Hearing ◽  
Brainstem Response

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.

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