scholarly journals ACEMg Diet Supplement Modifies Progression of Hereditary Deafness

2016 ◽  
Vol 6 (1) ◽  
Author(s):  
Kari L. Green ◽  
Donald L. Swiderski ◽  
Diane M. Prieskorn ◽  
Susan J. DeRemer ◽  
Lisa A. Beyer ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Control Diet ◽  
Beta Carotene ◽  
Auditory Brainstem ◽  
Diet Group ◽  
Auditory Neuropathy ◽  
Childhood Deafness ◽  
Hearing Thresholds ◽  
Genetic Hearing Loss ◽  
Brainstem Response

Abstract Dietary supplements consisting of beta-carotene (precursor to vitamin A), vitamins C and E and the mineral magnesium (ACEMg) can be beneficial for reducing hearing loss due to aminoglycosides and overstimulation. This regimen also slowed progression of deafness for a boy with GJB2 (CONNEXIN 26) mutations. To assess the potential for treating GJB2 and other forms of hereditary hearing loss with ACEMg, we tested the influence of ACEMg on the cochlea and hearing of mouse models for two human mutations: GJB2, the leading cause of childhood deafness, and DIAPH3, a cause of auditory neuropathy. One group of mice modeling GJB2 (Gjb2-CKO) received ACEMg diet starting shortly after they were weaned (4 weeks) until 16 weeks of age. Another group of Gjb2-CKO mice received ACEMg in utero and after weaning. The ACEMg diet was given to mice modeling DIAPH3 (Diap3-Tg) after weaning (4 weeks) until 12 weeks of age. Control groups received food pellets without the ACEMg supplement. Hearing thresholds measured by auditory brainstem response were significantly better for Gjb2-CKO mice fed ACEMg than for the control diet group. In contrast, Diap3-Tg mice displayed worse thresholds than controls. These results indicate that ACEMg supplementation can influence the progression of genetic hearing loss.

scholarly journals 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

2019 ◽  
Vol 35 (2) ◽  
Author(s):  
Muhammad Azeem Aslam ◽  
Adeela Javed ◽  
Abdul Moiz
Keyword(s):  
Hearing Loss ◽  
Steady State ◽  
Auditory Brainstem Response ◽  
Auditory Brainstem ◽  
Steady State Response ◽  
State Response ◽  
Hearing Thresholds ◽  
Auditory Steady State Response ◽  
Brainstem Response ◽  
The Mean

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.

scholarly journals Analysis of Chronic Tinnitus in Noise-Induced Hearing Loss and Presbycusis

2021 ◽  
Vol 10 (8) ◽  
pp. 1779
Author(s):  
Hee Jin Kang ◽  
Dae Woong Kang ◽  
Sung Su Kim ◽  
Tong In Oh ◽  
Sang Hoon Kim ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Otoacoustic Emissions ◽  
Pure Tone Audiometry ◽  
Auditory Brainstem ◽  
Outer Hair Cells ◽  
Chronic Tinnitus ◽  
Noise Induced Hearing Loss ◽  
Distortion Product ◽  
Hearing Thresholds ◽  
Brainstem Response

The most frequent causes of tinnitus associated with hearing loss are noise-induced hearing loss and presbycusis. The mechanism of tinnitus is not yet clear, although several hypotheses have been suggested. Therefore, we aimed to analyze characteristics of chronic tinnitus between noise-induced hearing loss and presbycusis. Materials and Methods: This paper is a retrospective chart review and outpatient clinic-based study of 248 patients with chronic tinnitus from 2015 to 2020 with noise-induced or presbycusis. Pure tone audiometry (PTA), auditory brainstem response (ABR), distortion product otoacoustic emissions (DPOAE), transient evoked otoacoustic emissions (TEOAE), and tinnitograms were conducted. Results: PTA showed that hearing thresholds at all frequencies were higher in patients with noise-induced hearing loss than the presbycusis group. ABR tests showed that patients with presbycusis had longer wave I and III latencies (p < 0.05 each) than patients with noise-induced hearing loss. TEOAE tests showed lower values in patients with noise-induced hearing loss than presbycusis at 1.5, 2, 3, and 4 kHz (p < 0.05 each). DPOAE tests showed that response rates in both ears at 1.5, 2, and 3 kHz were significantly higher in patients with presbycusis than noise-induced hearing loss (p < 0.05 each). Discussion: This study showed that hearing thresholds were higher, the loudness of tinnitus was smaller, and the degree of damage to outer hair cells was lower in patients with presbycusis than with noise-induced hearing loss. Moreover, wave I and III latencies were more prolonged in patients with presbycusis despite their having lower hearing thresholds. These phenomena may reflect the effects of aging or degeneration of the central nervous system with age. Further studies are needed to evaluate the etiologies of tinnitus.

scholarly journals The prevalence of hearing loss in infants hospitalized in the neonatal intensive care units

2020 ◽  
Author(s):  
Nasrin Gohari ◽  
Farhad Farahani ◽  
Soraya Gharebaghy ◽  
Sanaz Alaei ◽  
Shahla Ahmadi ◽  
...  
Keyword(s):  
Intensive Care Unit ◽  
Hearing Loss ◽  
Intensive Care ◽  
Birth Weight ◽  
Neonatal Intensive Care Unit ◽  
Auditory Brainstem Response ◽  
Neonatal Intensive Care ◽  
Auditory Brainstem ◽  
Auditory Neuropathy ◽  
Brainstem Response

Background and Aim: Studies have shown that several factors affect the hearing loss of infants hospitalized in the neonatal intensive care unit (NICU). These factors include hyperbilirubine­mia, low birth weight, asphyxia, and prematurity. This study aimed to investigate the prevalence of hearing loss and its risk factors in NICU infants. Methods: This is a cross-sectional study. Sam­ples were 159 infants admitted to the NICU in Fatemieh and Shahid Beheshti hospitals in Ham­adan, Iran, who were selected using a conveni­ence sampling method. Their hearing was scree­ned using transient evoked otoacoustic emission (TEOAE) and automated auditory brainstem res­ponse (AABR) tests; in case of failure in these tests, auditory brainstem response (ABR) was performed. Results: Of 159 infants, 9 (5.66%) were iden­tified with different types of hearing loss where 4 (2.51%) had sensory-neural hearing loss, one (0.62%) had auditory neuropathy and 4 (2.51%) had conductive hearing loss. There was a statis­tically significant relationship between hearing loss and birth weight < 1500 g, hyperbilirubine­mia, antibiotic therapy, family history of hearing loss, asphyxia and Apgar score < 5. Conclusion: Prevalence of hearing loss in NICU infants is noticeable so hearing assessment after discharge is necessary. Due to the presence of auditory neuropathy, simultaneous use of TEOAE and AABR tests in these infants is recommended. Keywords: Infant hearing screening; neonatal intensive care unit; auditory brainstem response

scholarly journals Optimizing parameters for using the parallel auditory brainstem response (pABR) to quickly estimate hearing thresholds

2021 ◽  
Author(s):  
Melissa J Polonenko ◽  
Ross K Maddox
Keyword(s):  
Hearing Loss ◽  
Auditory Brainstem Response ◽  
Presentation Rate ◽  
Auditory Brainstem ◽  
Single Frequency ◽  
Time Saving ◽  
Recording Time ◽  
Hearing Thresholds ◽  
Wide Range ◽  
Brainstem Response

Timely assessments are critical to providing early intervention and better hearing and spoken language outcomes for children with hearing loss. To facilitate faster diagnostic hearing assessments in infants, we developed the parallel auditory brainstem response (pABR), which presents randomly timed trains of tone pips at five frequencies to each ear simultaneously. We have shown that the pABR yields high-quality waveforms that are similar to the standard, single-frequency serial ABR but in a fraction of the recording time. While well-documented for standard ABRs, it is yet unknown how presentation rate and level interact to affect responses collected in parallel to random tone pip stimuli. Therefore, in this study we determined the optimal range of parameters for the pABR by recording responses across a range of six presentation rates, each at a low and high stimulus level. We show that a wide range of rates yields robust responses in under 15 minutes, but 40 Hz is the optimal singular presentation rate. Extending the analysis window to include later components of the response offers further time-saving advantages for the temporally broader responses to low frequency tone pips. Perceptual thresholds that subtly change across rate allow for a testing paradigm that easily transitions between rates, which may be useful for quickly estimating thresholds for different configurations of hearing loss. These optimized parameters facilitate expediency and effectiveness of the pABR to estimate hearing thresholds in a clinical setting.

scholarly journals Neuropati Auditori

2012 ◽  
Vol 1 (1) ◽  
Author(s):  
Sukri Rahman ◽  
Rossy Rosalinda
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Inner Ear ◽  
Signal Transmission ◽  
Correct Diagnosis ◽  
Auditory Brainstem ◽  
Auditory Neuropathy ◽  
Management Approach ◽  
Profound Hearing Loss ◽  
Brainstem Response

Abstrak Latar belakang: Neuropati auditori merupakan suatu gangguan pendengaran yang jarang terjadi dengan prevalensi yang belum diketahui secara pasti dan membutuhkan identifikasi dan diagnosis secara dini. Tujuan: Untuk menjelaskan gambaran audiologi dan elektrofisiologi neuropati auditori sehingga dapat menentukan terapi dan intervensi yang efektif. Tinjauan Pustaka: Neuropati auditori merupakan bagian dari tuli sensorineural, dimana suara dapat masuk hingga telinga dalam, tetapi transmisi sinyal dari telinga dalam ke otak terganggu pada jaras tertentu. Kelainan ini dapat mengenai semua umur mulai dari bayi hingga dewasa. Pasien dengan neuropati auditori dapat memiliki derajat pendengaran yang normal atau mengalami penurunan dari ringan hingga tuli sangat berat, tetapi selalu memiliki kemampuan persepsi bicara yang buruk. Neuropati auditori ditandai dengan hasil abnormal pada brainstem evoked response audiometry (BERA), tetapi otoacoustic emission (OAE) yang normal. Kelainan ini membutuhkan pendekatan manajemen yang berbeda untuk masalah pendengaran dan komunikasi dibandingkan tuli perifer lainnya. Kesimpulan: Evaluasi klinis dan audiologi yang akurat dibutuhkan pada neuropati auditori, dan pada akhirnya, diagnosis yang tepat memberikan strategi terapi dan rehabilitasi yang lebih baik. Kata kunci: Neuropati auditori, BERA, OAE, persepsi bicara Abstract Background: Auditory neuropathy is a rare hearing disorder which is the prevalence not well established and need an early identification and diagnosis. Purpose: To describe the audiological and electrophysiological features of auditory neuropathy in order to determine the effective treatment and intervention. Literature Review: Auditory neuropathy is a kind of sensorineural hearing loss, in which sounds enter the inner ear normally, but the signal transmission from the inner ear to the brain is impaired in some ways. It can affect people of all ages from infant to adult. Patients with auditory neuropathy may have normal hearing or hearing loss ranging from mild to profound hearing loss, but they always have poor speech perception abilities. Auditory neuropathy is characterized by the abnormal result of the auditory brainstem response (BERA), but in the presence of preserved otoacoustic emissions (OAE). It requires a different management approach to the auditory and communication problems that used for usual peripheral hearing losses. Conclusion: An accurate clinical and audiological evaluation are needed in auditory neuropathy, and finally, a correct diagnosis allow better treatment and rehabilitative strategies. Keywords: Auditory neuropathy, BERA, OAE, speech perception

scholarly journals Algorithm of hearing monitoring of children with congenital heart disease

2019 ◽  
Vol 10 (2) ◽  
pp. 129-135
Author(s):  
Ekaterina S. Garbaruk ◽  
Alice A. Nnomzoo ◽  
Pavel V. Pavlov ◽  
Oksana C. Gorkina
Keyword(s):  
Congenital Heart Disease ◽  
Hearing Loss ◽  
Heart Disease ◽  
Congenital Heart ◽  
Late Onset ◽  
Auditory Brainstem ◽  
Auditory Neuropathy ◽  
Auditory Function ◽  
Brainstem Response

Congenital heart disease (CHD) is one of the most common types of birth defect. Often newborn hearing screening is not performed for infants with congenital heart disease because of serious health problems at birth. Hearing loss in children with CHD may have late onset due to different stages of CHD treatment. The monitoring of the auditory function is required for early identification of hearing loss in children with CHD, however algorithm of follow-up assessment is not currently defined. Objective – estimation of prevalence, types and time of hearing loss onset in children with congenital heart disease and development a follow-up assessment for CHD children. Results. 148 children with congenital heart disease have been evaluated with auditory brainstem response testing, otoacoustic emissions, impedancemetry, behavioral hearing tests. The patients ranged in age from 7 days to 6 years. 55 children had hearing loss: 28 children with conductive hearing loss and 27 children with sensorineural hearing loss, 2 of them with auditory neuropathy. 6 children had late onset sensorineural hearing loss. The age of delayed hearing loss identification ranged from 4 months to 5 years. Conclusion. Children with CHD require long-term monitoring of the auditory function; an algorithm of follow-up assessment for children with congenital heart disease is proposed. Audiological assessment in children with CHD should include auditory brainstem response registration for auditory neuropathy identification.

scholarly journals Auditory Brainstem Response Abnormalities in Autistic Children

1982 ◽  
Vol 9 (4) ◽  
pp. 429-433 ◽  
Author(s):  
M.J. Taylor ◽  
B. Rosenblatt ◽  
L. Linschoten
Keyword(s):  
Hearing Loss ◽  
Auditory Brainstem ◽  
Auditory Brainstem Responses ◽  
National Society ◽  
Autistic Children ◽  
Profound Hearing Loss ◽  
Hearing Thresholds ◽  
High Incidence ◽  
Early Portion ◽  
Brainstem Response

SUMMARY:In an attempt to clarify issues of brainstem dysfunction and hearing thresholds in autistic children, we studied the Auditory Brainstem Responses (ABRs) in 32 children who clearly fit within the criteria of autism established by the National Society for Autistic Children (1977). ABRs were recorded between Cz and ipsilateral ear in response to click stimuli. Interwave latencies and auditory threshold in each ear were determined. Of the 32 children, 11 had moderate hearing loss (8 bilaterally) and 3 had severe to profound hearing loss, all bilaterally. 8 of the 14 with hearing loss also had associated features (e.g., perinatal encephalopathy). The I-III andI-Vinterwave latencies were significantly longer in the autistic children compared to normal control children; the increased conduction times were found mainly in the early portion of the auditory brainstem pathway. These data confirm some earlier reports of ABR abnormalities in autistic children and are concordant with some theories of the etiological basis of autism. The high incidence of hearing loss in these children is significant and routine ABR testing is recommended.

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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