scholarly journals A Porcine Congenital Single-Sided Deafness Model, Its Population Statistics and Degenerative Changes

2021 ◽  
Vol 9 ◽  
Author(s):  
Wei Ren ◽  
Cong Xu ◽  
Fan-jun Zheng ◽  
Ting-ting Lin ◽  
Peng Jin ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Middle Ear ◽  
Pathological Changes ◽  
Chi Square ◽  
Population Statistics ◽  
Fifth Generation ◽  
Brainstem Response ◽  
Single Sided Deafness ◽  
Chi Square Analysis

ObjectiveTo describe and study the population statistics, hearing phenotype, and pathological changes of a porcine congenital single-sided deafness (CSSD) pedigree.MethodsClick auditory brainstem response (ABR), full-frequency ABR, and distortion product otoacoustic emission (DPOAE) were used to assess the hearing phenotype of the strain. Tympanogram was used to assess the middle ear function since birth. Celloidin embedding–hematoxylin–eosin (CE-HE) stain and scanning electron microscopy (SEM) were used to study the pathological changes of cochlear microstructures. Chi-square analysis was used to analyze the relation between hearing loss and other phenotypes.ResultsThe mating mood of CSSD with CSSD was most efficient in breeding-targeted CSSD phenotype (47.62%), and the prevalence of CSSD reached 46.67% till the fifth generation, where 42.22% were bilateral hearing loss (BHL) and 9.00% were normal hearing (NH) individuals. Hearing loss was proved to have no relation with coat color (P = 0.0841 > 0.05) and gender (P = 0.4621 > 0.05) by chi-square analysis. The deaf side of CSSD offspring in the fifth generation had no relation with that of their maternal parent (P = 0.2387 > 0.05). All individuals in this strain exhibited congenital severe to profound sensorineural hearing loss with no malformation and dysfunction of the middle ear. The good hearing ear of CSSD stayed stable over age. The deaf side of CSSD and BHL presented cochlear and saccular degeneration, and the hair cell exhibited malformation since birth and degenerated from the apex to base turn through time. The pathology in BHL cochlea progressed more rapidly than CSSD and till P30, the hair cell had been totally gone. The stria vascularis (SV) was normal since birth and degenerated through time and finally exhibited disorganization of three layers of cells.ConclusionThis inbred porcine strain exhibited high and stable prevalence of CSSD, which highly resembled human non-syndromic CSSD disease. This porcine model could be used to further explore the etiology of CSSD and serve as an ideal tool for the studies of the effects of single-sided hearing deprivation on neural, cognitive, and behavioral developments and the benefits brought by CI in CSSD individuals.

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.

scholarly journals Effect of Phlorofucofuroeckol A and Dieckol Extracted from Ecklonia cava on Noise-induced Hearing Loss in a Mouse Model

Marine Drugs ◽  
2021 ◽  
Vol 19 (8) ◽  
pp. 443
Author(s):  
Hyunjun Woo ◽  
Min-Kyung Kim ◽  
Sohyeon Park ◽  
Seung-Hee Han ◽  
Hyeon-Cheol Shin ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Noise Exposure ◽  
Radical Scavenging ◽  
Threshold Shift ◽  
Ecklonia Cava ◽  
Control Group ◽  
Noise Induced Hearing Loss ◽  
Antioxidant Agents ◽  
Brainstem Response

One of the well-known causes of hearing loss is noise. Approximately 31.1% of Americans between the ages of 20 and 69 years (61.1 million people) have high-frequency hearing loss associated with noise exposure. In addition, recurrent noise exposure can accelerate age-related hearing loss. Phlorofucofuroeckol A (PFF-A) and dieckol, polyphenols extracted from the brown alga Ecklonia cava, are potent antioxidant agents. In this study, we investigated the effect of PFF-A and dieckol on the consequences of noise exposure in mice. In 1,1-diphenyl-2-picrylhydrazyl assay, dieckol and PFF-A both showed significant radical-scavenging activity. The mice were exposed to 115 dB SPL of noise one single time for 2 h. Auditory brainstem response(ABR) threshold shifts 4 h after 4 kHz noise exposure in mice that received dieckol were significantly lower than those in the saline with noise group. The high-PFF-A group showed a lower threshold shift at click and 16 kHz 1 day after noise exposure than the control group. The high-PFF-A group also showed higher hair cell survival than in the control at 3 days after exposure in the apical turn. These results suggest that noise-induced hair cell damage in cochlear and the ABR threshold shift can be alleviated by dieckol and PFF-A in the mouse. Derivatives of these compounds may be applied to individuals who are inevitably exposed to noise, contributing to the prevention of noise-induced hearing loss with a low probability of adverse effects.

scholarly journals Wideband acoustic immittance for assessing middle ear functioning for preterm neonates in the neonatal intensive care unit

2017 ◽  
Vol 64 (1) ◽  
Author(s):  
Nandel Gouws ◽  
De Wet Swanepoel ◽  
Leigh Biagio De Jager
Keyword(s):  
Intensive Care Unit ◽  
At Risk ◽  
Hearing Loss ◽  
Intensive Care ◽  
Neonatal Intensive Care Unit ◽  
Middle Ear ◽  
Diagnostic Tool ◽  
Neonatal Intensive Care ◽  
Preterm Neonates ◽  
Brainstem Response

Background: The primary aim of newborn hearing screening is to detect permanent hearing loss. Because otoacoustic emissions (OAEs) and automated auditory brainstem response (AABR) are sensitive to hearing loss, they are often used as screening tools. On the other hand, false-positive results are most often because of transient outer- and middle ear conditions. Wideband acoustic immittance (WAI), which includes physical measures known as reflectance and absorbance, has shown potential for accurate assessment of middle ear function in young infants.Objective: The main objective of this study was to determine the feasibility of WAI as a diagnostic tool for assessing middle ear functioning in preterm neonates in the neonatal intensive care unit (NICU) designed for premature and ill neonates. A further objective was to indicate the difference between the reflectance values of tones and click stimuli.Method: Fifty-six at-risk neonates (30 male and 26 female), with a mean age at testing of 35.6 weeks (range: 32–37 weeks) and a standard deviation of 1.6 from three private hospitals, who passed both the distortion product otoacoustic emission (DPOAE) and AABR tests, were evaluated prior to discharge from the NICU. Neonates who presented with abnormal DPOAE and AABR results were excluded from the study. WAI was measured by using chirp and tone stimuli. In addition to reflectance, the reflectance area index (RAI) values were calculated.Results: Both tone and chirp stimuli indicated high-power reflectance values below a frequency of 1.5 kHz. Median reflectance reached a minimum of 0.67 at 1 kHz – 2 kHz but increased to 0.7 below 1 kHz and 0.72 above 2 kHz for the tone stimuli. For chirp stimuli, the median reflectance reached a minimum of 0.51 at 1 kHz – 2 kHz but increased to 0.68 below 1 kHz and decreased to 0.5 above 2 kHz. A comparison between the present study and previous studies on WAI indicated a substantial variability across all frequency ranges.Conclusion: These WAI measurements conducted on at-risk preterm NICU neonates (mean age at testing: 35.6 weeks, range: 32–37 weeks) identified WAI patterns not previously reported in the literature. High reflective values were obtained across all frequency ranges. The age of the neonates when tested might have influenced the results. The neonates included in the present study were very young preterm neonates compared to the ages of neonates in previous studies. WAI measured in at-risk preterm neonates in the NICU was variable with environmental and internal noise influences. Transient conditions affecting the sound-conduction pathway might have influenced the results. Additional research is required to investigate WAI testing in ears with and without middle ear dysfunction. The findings of the current study imply that in preterm neonates it was not possible to determine the feasibility of WAI as a diagnostic tool to differentiate between ears with and without middle ear pathology.

scholarly journals N-Methyl-D-Aspartate Receptors Involvement in the Gentamicin-Induced Hearing Loss and Pathological Changes of Ribbon Synapse in the Mouse Cochlear Inner Hair Cells

2018 ◽  
Vol 2018 ◽  
pp. 1-16 ◽  
Author(s):  
Juan Hong ◽  
Yan Chen ◽  
Yanping Zhang ◽  
Jieying Li ◽  
Liujie Ren ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Auditory Brainstem ◽  
Ribbon Synapse ◽  
Synaptic Ribbons ◽  
Pathological Changes ◽  
Inner Hair Cell ◽  
Ribbon Synapses ◽  
Brainstem Response ◽  
Mature Mouse ◽  
Response Thresholds

Cochlear inner hair cell (IHC) ribbon synapses play an important role in sound encoding and neurotransmitter release. Previous reports show that both noise and aminoglycoside exposures lead to reduced numbers and morphologic changes of synaptic ribbons. In this work, we determined the distribution of N-methyl-D-aspartate receptors (NMDARs) and their role in the gentamicin-induced pathological changes of cochlear IHC ribbon synaptic elements. In normal mature mouse cochleae, the majority of NMDARs were distributed on the modiolar side of IHCs and close to the IHC nuclei region, while most of synaptic ribbons and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) were located on neural terminals closer to the IHC basal poles. After gentamicin exposure, the NMDARs increased and moved towards the IHC basal poles. At the same time, synaptic ribbons and AMPARs moved toward the IHC bundle poles on the afferent dendrites. The number of ribbon synapse decreased, and this was accompanied by increased auditory brainstem response thresholds and reduced wave I amplitudes. NMDAR antagonist MK801 treatment reduced the gentamicin-induced hearing loss and the pathological changes of IHC ribbon synapse, suggesting that NMDARs were involved in gentamicin-induced ototoxicity by regulating the number and distribution of IHC ribbon synapses.

Reduced BDNF Expression in Auditory Cortex Contributed to Neonatal Pain Induced Hearing Impairment and Dendritic Pruning Deficiency in Mice

2021 ◽  
Author(s):  
Nanqi Li ◽  
Bing Chen ◽  
Gaogan Jia ◽  
Rui Xu ◽  
Ying Xia ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Auditory Cortex ◽  
Hair Cell ◽  
Hearing Impairment ◽  
Dendritic Spine ◽  
Bdnf Level ◽  
Cochlear Function ◽  
Neonatal Pain ◽  
Brainstem Response ◽  
Spine Pruning

Abstract Hearing loss in children is common especially in NICU with consequences of worse outcomes in speech, language, education, social functioning, cognitive abilities, and quality of life. Whether neonatal pain is link to increase risks for hearing loss remains to be explored. Here, we implemented Complete Freund's adjuvant (CFA) plantar injection and needle prick model to mimic neonatal pain in NICU during critical period of hearing development. Auditory brainstem response (ABR) test was used to determine the hearing threshold at 4w and 8w postnatal. Sufentanil and Oxycodone were used as analgesic to treat neonatal pain. Hair cell and ribbon synapse stanning were performed to detect cochlear function. Golgi-cox staining and BDNF immunofluorescence of auditory cortex were performed to determine dendritic spine pruning in auditory cortex. The dendritic pruning related protein CaMKII and Rac1/2 level were detected by western blot. We found that CFA induced neonatal pain and ABR threshold increased at 4w and 8w postnatal and the impairment were attenuated after analgesic administration. Neither the inner hair cell (IHC) nor the synapse of CFA mice was damaged in cochlear. CFA mice showed increased dendritic spine density at auditory cortex and reduced BDNF level. Furthermore, Rac1/2 and CaMKII might contributed to the disrupt dendritic spine pruning. Our study suggested that neonatal pain could induced hearing impairment in adulthood ascribed to the reduced BDNF level and AC dendritic spine pruning deficiency, optimal analgesic in early-life could beneficial for hearing development.

An auditory profile of sclerosteosis

2014 ◽  
Vol 128 (4) ◽  
pp. 336-344 ◽  
Author(s):  
J M Potgieter ◽  
D W Swanepoel ◽  
B M Heinze ◽  
L M Hofmeyr ◽  
A A S Burger ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Bone Growth ◽  
Round Window ◽  
Internal Auditory Canal ◽  
Auditory Brainstem ◽  
Oval Window ◽  
Speech Discrimination ◽  
Cross Sectional ◽  
Brainstem Response

AbstractObjective:To characterise auditory involvement secondary to excessive craniotubular bone growth in individuals with sclerosteosis in South Africa.Methods:This cross-sectional study assessed the auditory profile of 10 participants with sclerosteosis. An auditory test battery was used and results for each ear were recorded using descriptive and comparative analyses.Results:All participants presented with bilateral, mixed hearing losses. Of the 20 ears, hearing loss was moderate in 5 per cent (n = 1), severe in 55 per cent (n = 11) and profound in 40 per cent (n = 8). Air–bone gaps were smaller in older participants, although the difference was not statistically significant (p > 0.05). Computed tomography scans indicated pervasive abnormalities of the external auditory canal, tympanic membrane, middle-ear space, ossicles, oval window, round window and internal auditory canal. Narrowed internal auditory canals corresponded to poor speech discrimination, indicative of retrocochlear pathology and absent auditory brainstem response waves.Conclusion:Progressive abnormal bone formation in sclerosteosis involves the middle ear, the round and oval windows of the cochlea, and the internal auditory canal. The condition compromises conductive, sensory and neural auditory pathways, which results in moderate to profound, mixed hearing loss.

scholarly journals Hidden hearing loss is associated with loss of ribbon synapses of cochlea inner hair cells

2021 ◽  
Author(s):  
Feng Song ◽  
Bin Gan ◽  
Na Wang ◽  
Zhe Wang ◽  
An-ting Xu
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Noise Exposure ◽  
Repeated Exposure ◽  
Ribbon Synapse ◽  
Intensity Noise ◽  
Auditory Function ◽  
Ribbon Synapses ◽  
Brainstem Response ◽  
Hidden Hearing Loss

This study aimed to observe the changes in the cochlea ribbon synapses after repeated exposure to moderate-to-high intensity noise. Guinea pigs received 95 dB SPL white noise exposure 4 hours a day for consecutive 7 days (we regarded it a medium-term and moderate-intensity noise, or MTMI noise). Animals were divided into 4 groups: Control, 1DPN (1-day post noise), 1WPN (1-week post noise), and 1MPN (1-month post noise). Auditory function analysis by ABR and CAP recordings, as well as ribbon synapse morphological analyses by immunohistochemistry (Ctbp2 and PSD95 staining) were performed one day, one week, and one month after noise exposure. After MTMI noise exposure, the amplitudes of auditory brainstem response (ABR) I and III waves were suppressed. The compound action potential (CAP) threshold was elevated, and CAP amplitude was reduced in the 1DPN group. No apparent changes in hair cell shape, arrangement or number were observed, but the number of ribbon synapse was reduced. The 1WPN and 1MPN groups showed that part of ABR and CAP changes recovered, as well as the synapse number. The defects in cochlea auditory function and synapse changes were observed mainly in the high-frequency region. Together, repeated exposure in MTMI noise can cause hidden hearing loss, which is partially reversible after leaving the noise environment; and MTMI noise induced hidden hearing loss is associated with inner hair cell ribbon synapses.

Epidemiology, Haemato-biochemical and Pathological Changes Related to Field Outbreaks of PPR in Small Ruminants in Odisha

2021 ◽  
Author(s):  
P.K. Rath ◽  
S.K. Panda ◽  
B.P. Mishra ◽  
R. Mishra ◽  
D.K. Karna
Keyword(s):  
Small Ruminants ◽  
Pathological Changes ◽  
Chi Square ◽  
Tissue Samples ◽  
Helminthic Infection ◽  
Epidemiological Risk ◽  
Intracytoplasmic Inclusion Bodies ◽  
Polymerase Chain ◽  
Chi Square Analysis ◽  
Poor Management

Background: Odisha experiencing sporadic outbreaks of Peste des petits ruminants (PPR) throughout the year. There is a scarcity of available literature on PPR in Odisha till today. This is the first ever detail investigative approach in the state undertaken with an objective to corelate the epidemiological risk factors, haemato-biochemical and pathological changes in natural field outbreaks occurring in eight different districts. Methods: Fourteen field outbreaks of PPR were evaluated clinically as well as epidemiologically and confirmed through polymerase chain reaction (PCR). Blood, serum, faecal and tissue samples were collected to observe haemato-biochemical and pathomorphological changes to asses disease severity. Result: Present study concluded an overall mortality rate of 46.81%. Chi-square analysis revealed significant highest prevalence among 7-12 months (46.13%) age, Ganjam breed (45.51%) and females (80.49%). Frequent migration among the border areas along with poor management and helminthic infection was major precipitating factor. There was polycythemia along with neutrophilia and lymphopenia. Significant increase in alanine transaminase (ALT), aspartate aminotransferase (AST), K+ and Ca+2 along with creatinine, urea and blood urea nitrogen (BUN) BUN was observed in affected flocks. Antero-ventral consolidation of lungs, syncytia and presence of both eosinophilic intranuclear and intracytoplasmic inclusion bodies were major pathological changes.

The Effects of Age at Cleft Palate Repair on Middle Ear Function and Hearing Level

2018 ◽  
Vol 55 (5) ◽  
pp. 753-757 ◽  
Author(s):  
Qun Lou ◽  
Hongping Zhu ◽  
Yi Luo ◽  
Zhibo Zhou ◽  
Lian Ma ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cleft Palate ◽  
Middle Ear ◽  
Hearing Threshold ◽  
Hearing Level ◽  
Chi Square ◽  
Group I ◽  
Cleft Palate Repair ◽  
Palate Repair ◽  
Medical Histories

Objective: To investigate the age effects of cleft palate repair on middle ear function and hearing level in patients who underwent cleft palate repair at different ages by audiologic examination. Methods: Medical histories were gathered in detail, and audiologic tests (ie, tympanometry and pure tone hearing threshold) were conducted in 126 patients after palatoplasty. The patients were divided into the following 4 groups according to their ages when they underwent cleft palate repair: group I (0-3 years, 73 patients), group II (4-7 years, 29 patients), group III (8-11 years, 16 patients), and group IV (12 years and older, 8 patients). The data regarding tympanograms, hearing levels, and the average hearing thresholds of each group were analyzed using chi-square tests. Results: The prevalence of middle ear dysfunction and hearing loss in the patients who underwent palatoplasty before 3 years old (27.4% and 2.0% respectively) was significantly lower than that in patients who underwent palatopalsty at 12 years or older (75.0% and 43.7%, respectively). Linear-by-linear association revealed that the prevalences of middle ear dysfunction and hearing loss among the 4 groups were significantly different ( P < .05). Conclusions: The prevalence of middle ear dysfunction and hearing loss tended to increase with advancing age at the time of cleft palate repair. From an audiologist’s perspective, palatoplasty at an early age is very beneficial in helping children with cleft palates acquire better middle ear function and hearing level.

Effects of Conductive Hearing Loss on Auditory Brainstem Response

1982 ◽  
Vol 91 (3) ◽  
pp. 304-309 ◽  
Author(s):  
Therese J. McGee ◽  
Jack D. Clemis
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Auditory Brainstem Response ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Auditory Brainstem ◽  
Middle Ear Effusion ◽  
Brainstem Response ◽  
Conductive Hearing ◽  
Ear Effusion

The purpose of this paper is not to propose that auditory brainstem response (ABR) be utilized for the assessment of conductive losses, but to define the effects of conductive hearing loss on the ABR when such a complication occurs. Conductive losses attenuate cochlear stimulation. Since wave V latency is inversely related to stimulus intensity, the magnitude of the conductive loss should be a predictor of the wave V latency delay. In this study, ABR wave V latencies from patients with known conductive losses due to canal occlusion, middle ear effusion, ossicular fixation and chain interruption were compared with latency values calculated from the magnitude of the loss. In those patients with occlusion of the external auditory canal and middle ear effusion, the shift of the wave V latency-intensity function correlated well with the air-bone gap. This correlation was poor for patients with ossicular chain disorders. In mixed hearing losses, the increased wave V latency due to the conductive component may totally mask an increase in latency caused by a retrocochlear component.

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