The inner ear

2019 ◽  
pp. 117-161
Author(s):  
Rogan Corbridge ◽  
Nicholas Steventon
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Bone Fractures ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Noise Induced Hearing Loss ◽  
Hearing Rehabilitation ◽  
Age Related ◽  
Age Related Hearing Loss

Conditions affecting the inner ear are delineated. Sensorineural hearing loss and its aetiologies are explored in reference to age-related hearing loss, noise-induced hearing loss, syndromic problems, and auditory neuropathy spectrum disorder. Causes of vertigo related to peripheral vestibular dysfunction are discussed, including vestibular migraine. Management of temporal bone fractures and their sequelae are explored. Methods of hearing rehabilitation are described.

Auditory neuropathy spectrum disorder with Brown–Vialetto–Van Laere syndrome: challenges in hearing rehabilitation

2015 ◽  
Vol 129 (5) ◽  
pp. 504-508 ◽  
Author(s):  
R Chandran ◽  
M Alexander ◽  
P Naina ◽  
A Balraj
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Case Series ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Auditory Brainstem Responses ◽  
High Dose ◽  
Hearing Rehabilitation ◽  
Auditory Neuropathy Spectrum Disorder

AbstractBackground:Brown–Vialetto–Van Laere syndrome is a rare neurological disorder characterised by pontobulbar palsy and sensorineural hearing loss. Hearing rehabilitation continues to be a challenge because the exact lesion site is unknown.Case report:We examined the clinical and audiological profiles of a case series comprising four siblings with Brown–Vialetto–Van Laere syndrome who had decreased hearing and poor speech discrimination. Audiological investigations revealed normal otoacoustic emissions with absent auditory brainstem responses and middle-ear reflexes in sensorineural hearing loss, suggestive of auditory neuropathy spectrum disorder.Conclusion:The sensorineural hearing loss in Brown–Vialetto–Van Laere syndrome patients is a retrocochlear pathology resembling auditory neuropathy spectrum disorder, with the lesion being most probably of post-synaptic origin. Early cochlear implantation along with high-dose riboflavin represents a possible rehabilitation therapy. However, further research is needed to confirm this. This report emphasises the need for a thorough neurological evaluation of auditory neuropathy spectrum disorder patients.

scholarly journals Sex Differences in the Triad of Acquired Sensorineural Hearing Loss

2021 ◽  
Vol 22 (15) ◽  
pp. 8111
Author(s):  
Kuang-Hsu Lien ◽  
Chao-Hui Yang
Keyword(s):  
Hearing Loss ◽  
Sex Differences ◽  
Sexual Dimorphism ◽  
Sensorineural Hearing Loss ◽  
Hormone Replacement ◽  
Modern Society ◽  
Sensorineural Hearing ◽  
Drug Induced ◽  
Age Related ◽  
Age Related Hearing Loss

The triad of noise-generated, drug-induced, and age-related hearing loss is the major cause of acquired sensorineural hearing loss (ASNHL) in modern society. Although these three forms of hearing loss display similar underlying mechanisms, detailed studies have revealed the presence of sex differences in the auditory system both in human and animal models of ASNHL. However, the sexual dimorphism of hearing varies among noise-induced hearing loss (NIHL), ototoxicity, and age-related hearing loss (ARHL). Importantly, estrogen may play an essential role in modulating the pathophysiological mechanisms in the cochlea and several reports have shown that the effects of hormone replacement therapy on hearing loss are complex. This review will summarize the clinical features of sex differences in ASNHL, compare the animal investigations of cochlear sexual dimorphism in response to the three insults, and address how estrogen affects the auditory organ at molecular levels.

scholarly journals Noise-induced and age-related hearing loss:  new perspectives and potential therapies

F1000Research ◽  
2017 ◽  
Vol 6 ◽  
pp. 927 ◽  
Author(s):  
M Charles Liberman
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Sensorineural Hearing Loss ◽  
Hair Cells ◽  
Auditory Nerve ◽  
Cell Loss ◽  
Sensorineural Hearing ◽  
Hair Cell Loss ◽  
Age Related ◽  
Age Related Hearing Loss

The classic view of sensorineural hearing loss has been that the primary damage targets are hair cells and that auditory nerve loss is typically secondary to hair cell degeneration. Recent work has challenged that view. In noise-induced hearing loss, exposures causing only reversible threshold shifts (and no hair cell loss) nevertheless cause permanent loss of >50% of the synaptic connections between hair cells and the auditory nerve. Similarly, in age-related hearing loss, degeneration of cochlear synapses precedes both hair cell loss and threshold elevation. This primary neural degeneration has remained a “hidden hearing loss” for two reasons: 1) the neuronal cell bodies survive for years despite loss of synaptic connection with hair cells, and 2) the degeneration is selective for auditory nerve fibers with high thresholds. Although not required for threshold detection when quiet, these high-threshold fibers are critical for hearing in noisy environments. Research suggests that primary neural degeneration is an important contributor to the perceptual handicap in sensorineural hearing loss, and it may be key to the generation of tinnitus and other associated perceptual anomalies. In cases where the hair cells survive, neurotrophin therapies can elicit neurite outgrowth from surviving auditory neurons and re-establishment of their peripheral synapses; thus, treatments may be on the horizon.

scholarly journals Cortical Neurophysiologic Correlates of Auditory Threshold in Adults and Children With Normal Hearing and Auditory Neuropathy Spectrum Disorder

2020 ◽  
pp. 1-15
Author(s):  
Garrett Cardon ◽  
Anu Sharma
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Normal Hearing ◽  
Auditory Neuropathy ◽  
Auditory Threshold ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Threshold Estimation ◽  
Auditory Thresholds ◽  
Auditory Neuropathy Spectrum Disorder

Purpose Auditory threshold estimation using the auditory brainstem response or auditory steady state response is limited in some populations (e.g., individuals with auditory neuropathy spectrum disorder [ANSD] or those who have difficulty remaining still during testing and cannot tolerate general anesthetic). However, cortical auditory evoked potentials (CAEPs) can be recorded in many such patients and have been employed in threshold approximation. Thus, we studied CAEP estimates of auditory thresholds in participants with normal hearing, sensorineural hearing loss, and ANSD. Method We recorded CAEPs at varying intensity levels to speech (i.e., /ba/) and tones (i.e., 1 kHz) to estimate auditory thresholds in normal-hearing adults ( n = 10) and children ( n = 10) and case studies of children with sensorineural hearing loss and ANSD. Results Results showed a pattern of CAEP amplitude decrease and latency increase as stimulus intensities declined until waveform components disappeared near auditory threshold levels. Overall, CAEP thresholds were within 10 dB HL of behavioral thresholds for both stimuli. Conclusions The above findings suggest that CAEPs may be clinically useful in estimating auditory threshold in populations for whom such a method does not currently exist. Physiologic threshold estimation in difficult-to-test clinical populations could lead to earlier intervention and improved outcomes.

Expression Profiling of MicroRNAs in the Inner Ear of Elderly People by Real-Time PCR Quantification

2017 ◽  
Vol 22 (3) ◽  
pp. 135-145 ◽  
Author(s):  
Kuwon Sekine ◽  
Tomohiro Matsumura ◽  
Toshihiro Takizawa ◽  
Yurika Kimura ◽  
Shiho Saito ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Real Time Pcr ◽  
Expression Profiling ◽  
Molecular Mechanisms ◽  
Elderly Subjects ◽  
Age Related ◽  
Inner Ear Development ◽  
Temporal Bones ◽  
Age Related Hearing Loss

The molecular mechanisms underlying age-related hearing loss are unknown, and currently, there is no treatment for this condition. Recent studies have shown that microRNAs (miRNAs) and age-related diseases are intimately linked, suggesting that some miRNAs may present attractive therapeutic targets. In this study, we obtained 8 human temporal bones from 8 elderly subjects at brain autopsy in order to investigate the expression profile of miRNAs in the inner ear with miRNA arrays. A mean of 478 different miRNAs were expressed in the samples, of which 348 were commonly expressed in all 8 samples. Of these, levels of 16 miRNAs significantly differed between young elderly and old elderly subjects. miRNAs, which play important roles in inner ear development, were detected in all samples, i.e., in both young and old elderly subjects, whether with or without hearing loss. Our results suggest that these miRNAs play important roles not only in development, but also in the maintenance of inner ear homeostasis.

Perceptual separation of sensorineural hearing loss and auditory neuropathy spectrum disorder

2015 ◽  
Vol 126 (6) ◽  
pp. 1420-1425 ◽  
Author(s):  
Shuo Wang ◽  
Ruijuan Dong ◽  
Dongxin Liu ◽  
Yuan Wang ◽  
Yitao Mao ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Perceptual Separation

scholarly journals Age-Related Hearing Loss Is Dominated by Damage to Inner Ear Sensory Cells, Not the Cellular Battery That Powers Them

2020 ◽  
Vol 40 (33) ◽  
pp. 6357-6366 ◽  
Author(s):  
Pei-zhe Wu ◽  
Jennifer T. O'Malley ◽  
Victor de Gruttola ◽  
M. Charles Liberman
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Sensory Cells ◽  
Age Related ◽  
Age Related Hearing Loss

FBXO3 Downregulation Attenuates Noise-induced Hearing Loss by Suppressing ATG10 Degradation and Activating Autophagy

2020 ◽  
Author(s):  
Fan Ye ◽  
Bi Lin ◽  
Lian Fang
Keyword(s):  
Hearing Loss ◽  
Noise Exposure ◽  
Underlying Mechanism ◽  
Noise Induced Hearing Loss ◽  
Age Related ◽  
Rat Cochlea ◽  
Model Treatment ◽  
Ear Injury ◽  
Age Related Hearing Loss

Abstract Noise induced hearing loss (NIHL) is a kind of hearing impairment, which is next to the age-related hearing loss. More and more evidences have verified that overproduction of reactive oxygen species is a common pathologic phenomenon of different inner ear injury including NIHL, and autophagy contributes to attenuate NIHL by reducing oxidative stress. However, the underlying mechanism by which noise exposure causes autophagy activation remains unclear. In this study, we found that NIHL was accompanied by autophagy in the rat cochlea. Furthermore, twelve common genes were found at the GEO datasets GSE85290 and GSE8342, and E3 ubiquitination ligase FBXO3 was confirmed significantly reduced in NIHL rat cochlea. Next, we demonstrated that FBXO3 can directly binding with autophagy-related protein 10 (ATG10), which is necessary for the initiation of autophagy, and mediate its degradation. In vivo animal model treatment with rapamycin, an autophagy activator, significantly reduced the NIHL. Based on these data, we confirmed that FBXO3 played an important role in autophagy caused by NIHL, may be a potential target of NIHL treatment.

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