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At least 19 recordsLinked to original sources

Cochlear receptor (microphonic and summating potentials, otoacoustic emissions) and auditory pathway (auditory brain stem potentials) activity in auditory neuropathy.

OBJECTIVE: To define both auditory nerve and cochlear receptor functions in subjects with auditory neuropathy (AN). DESIGN: We tested 33 AN subjects (66 ears) and compared them with 21 healthy subjects (28 ears). In AN subjects, the average pure-tone (1, 2, and 4 kHz) threshold loss was 57 dB HL. Click stimuli were used to elicit transient evoked otoacoustic emissions (TEOAEs), cochlear microphonics (CMs), and auditory brain stem responses (ABRs). Both cochlear and ABR potentials were recorded from surface electrodes (vertex-ipsilateral mastoid) using averaging procedures. The amplitudes and latencies of CMs and ABRs and the amplitude of the TEOAEs were analyzed. RESULTS: CM amplitudes recorded from normal ears decreased as a function of subject age. CMs recorded from AN subjects fell within the normal age-adjusted range in 60% of the subjects and were >2 SEEs (standard error of estimate) above the age-adjusted normal regression in 40% of the subjects. TEOAEs were absent in 19 (30%) AN ears (bilaterally in eight, and unilaterally in three subjects) and were present in 44 ears. In AN subjects, correlations among CM amplitude, TEOAE amplitude, and pure-tone average thresholds were not significantly related. CM amplitudes were not significantly different whether TEOAEs or ABRs were present or absent. The ABR was present in 21% of AN subjects and consisted of a low-amplitude Wave V without a preceding Wave I. Measures of CM amplitude and PTA hearing loss were not significantly different in those AN ears with a preserved ABR compared with ears with absent ABRs. Summating potentials to transient click stimuli were of small amplitude (<0.1 microV) and detectable in approximately 50% of the AN and healthy control subjects limiting formal analysis of summating potentials. CONCLUSIONS: In a significant proportion of AN subjects, we found abnormalities of cochlear receptor function, including elevated CM amplitudes and absence of TEOAEs. These two abnormalities occurred independently of each other. A low amplitude Wave V of the ABR was found in approximately one-fifth of AN subjects, evidence that neural synchrony can be partially preserved in some subjects with this disorder.

Adolescent↗

Auditory pathway and auditory brainstem response in mice lacking NMDA receptor epsilon 1 and epsilon 4 subunits.

There is considerable evidence that the N-methyl-D-aspartate receptor (NMDAR) is a component of excitatory amino acid synapses in the ascending auditory pathway. The availability of mice that are defective in NMDAR epsilon 1 or NMDAR epsilon 4 subunit paves the way for investigations on the role of this receptor in auditory function. Non-radioactive in situ hybridization was used in the parent C57/6J wild strain to determine if these subunits are normally expressed in cochlear nucleus (CN) and superior olivary complex (SOC) and to confirm their absence in the respective mutant mice. Evoked auditory brainstem response (ABR) to normal acoustic stimulation was investigated to assess function. In situ hybridization revealed the expression of NMDAR epsilon 1 and epsilon 4 subunits mRNAs in major neuronal types in the CN and SOC of the wild type mice while epsilon 1 and epsilon 4 expression were absent in their respective mutant mice. The ABR threshold for the epsilon 1 mutant mice was similar to that of wild type mice however the threshold for the epsilon 4 mutant mice was significantly elevated. These results suggest a role for the NMDAR epsilon 4 in normal auditory functions while the NMDAR epsilon 1 may have a less critical function under normal conditions.

Animals↗

Auditory pathway and auditory activation of primary visual targets in the blind mole rat (Spalax ehrenbergi): I. 2-deoxyglucose study of subcortical centers.

The blind mole rat Spalax ehrenbergi is a subterranean rodent that shows striking behavioral, structural, and physiological adaptations to fossorial life including highly degenerated eyes and optic nerves and a behavioral audiogram that indicates high specialization for low-frequency hearing. A 2-deoxyglucose functional mapping of acoustically activated structures, in conjunction with Nissl/Klüver-Barrera-stained material, revealed a typical mammalian auditory pathway with some indications for specialized low-frequency hearing such as a poorly differentiated lateral nucleus and a well-developed medial nucleus in the superior olive complex. The most striking finding was a marked 2-deoxyglucose labeling of the dorsal lateral geniculate body and of cortical regions that correspond to visual areas in sighted rodents. The results render the blind mole rat a good model system for studying natural neural plasticity and intermodal compensation. In this report, we confine ourselves to the subcortical levels. The cortical level will be dealt comprehensively in a following paper.

Acoustic Stimulation↗

Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy.

Auditory neuropathy is a particular type of hearing impairment in which neural transmission of the auditory signal is impaired, while cochlear outer hair cells remain functional. Here we report on DFNB59, a newly identified gene on chromosome 2q31.1-q31.3 mutated in four families segregating autosomal recessive auditory neuropathy. DFNB59 encodes pejvakin, a 352-residue protein. Pejvakin is a paralog of DFNA5, a protein of unknown function also involved in deafness. By immunohistofluorescence, pejvakin is detected in the cell bodies of neurons of the afferent auditory pathway. Furthermore, Dfnb59 knock-in mice, homozygous for the R183W variant identified in one DFNB59 family, show abnormal auditory brainstem responses indicative of neuronal dysfunction along the auditory pathway. Unlike previously described sensorineural deafness genes, all of which underlie cochlear cell pathologies, DFNB59 is the first human gene implicated in nonsyndromic deafness due to a neuronal defect.

Amino Acid Sequence↗

Expression of the c-fos transcription factor in the rat auditory pathway following postnatal auditory deprivation.

As an animal model for inborn hearing loss rat pups were reared in a sound-proof chamber from birth until age 21 days. In addition, pinnae were bilaterally sutured closed to reduce any influence of ambient sound. At the end of the sound deprivation, outer ear channels were reopened. Since previous studies failed to show any difference in the number or morphology of neurons in the auditory pathway in bilaterally sound-deprived animals, expression of c-fos protein was used as a functional marker to map trans-synaptic information transfer in the auditory pathway with cellular resolution. At day 21 sound-deprived rats and untreated controls were stimulated with pure tones of 8kHz for 5min at different sound pressure levels. Acoustic stimulation induced c-fos in both parts of the cochlear nucleus, superior olivary complex and inferior colliculus. Compared to untreated rats, deprivation reduced the number of c-fos labeled neurons in the dorsal and ventral part of the cochlear nucleus and inferior colliculus by 58% and 30%, respectively, following low sound pressure levels (90dB). In contrast, high sound pressure levels (120dB) increased the number of c-fos labeled neurons in these areas and evoked only minor differences in the number of labeled neurons in both untreated and sound deprived rats.

Acoustic Stimulation↗

Deafness in Cockayne's syndrome: morphological, morphometric, and quantitative study of the auditory pathway.

The auditory pathway of a 17-year-old deaf patient with Cockayne's syndrome was examined histologically. The cochlea showed marked atrophy of the spiral ganglion and attenuation of the cochlear division of the eighth cranial nerve. By means of the Computer Image Analyzer, the total number of neurons in the ventral cochlear nucleus was found to be reduced from 30,440 to 18,821. The mean diameter of the neurons in the ventral cochlear nucleus, medial dorsal olivary nucleus, and inferior colliculus was smaller than in a control patient, whereas in the medial geniculate nucleus and anterior transverse gyrus of Heschl, the neuronal size approximated the norm. The changes in the first three auditory relay nuclei were considered to represent transsynaptic atrophy caused by degeneration of the spiral ganglion and, possibly, the cochlear neuroepithelium. This histological report verifies that deafness in Cockayne's syndrome is largely sensorineural and that degeneration of spiral ganglion in humans can lead to a chain of trans-synaptic degeneration in the ventral cochlear nucleus, medial dorsal olivary nucleus, and inferior colliculus.

Adolescent↗

Early phase of spatial mismatch negativity is localized to a posterior "where" auditory pathway.

The auditory mismatch negativity (MMN) is an event-related potential that reflects early processing of changes in acoustic stimulus features. Although the MMN has been well characterized by previous work, the number, roles, and anatomical locations of its cortical generators remain unresolved. Here, we report that the MMN elicited by occasional deviations in sound location is comprised of two temporally and anatomically distinct phases: an early phase with a generator posterior to auditory cortex and contralateral to the deviant stimulus, and a later phase with generators that are more frontal and bilaterally symmetric. The posterior location of the early-phase generator suggests the engagement of neurons within a putative "where" pathway for processing spatial auditory information.

Acoustic Stimulation↗

Frequency-specific maturation of the eighth nerve and brain-stem auditory pathway: evidence from derived auditory brain-stem responses (ABRs).

Previous studies of human auditory development using frequency-specific auditory brain-stem responses (ABRs) have reported that maturation for both peak and interpeak latencies occurs earlier for responses generated by low-frequency stimuli. In two of these studies, low-frequency ABRs presumed to originate from apical locations in the cochlea were likely dominated by activity from higher frequency regions closer to the base. In the present study, the high-pass noise-masking technique was used to generate derived ABRs that represent activity from isolated place specific regions along the basilar membrane. Analysis of auditory brain-stem maturation based on I-V interpeak latency differences with adult means revealed a frequency-specific pattern of development. Developmental changes occurred faster and mature function was attained earlier for ABRs from the mid-center-frequency (CF) derived conditions than from either the highest or lowest CF derived conditions. The differential maturation of mid-CF derived ABRs may reflect the delayed effects of the pattern of development that occurs in the cochlea.

Auditory Pathways↗

The consequences of deafness and chronic intracochlear electrical stimulation on the central auditory pathways.

1. Auditory deprivation can result in significant morphological and physiological changes within the central auditory nervous system. These changes are generally more pronounced when the onset of deafness occurs early in development, as is the case with congenitally deaf children. 2. A review of the auditory plasticity literature suggests that some of these deafness-induced changes may be preventable or partially reversible by electrical stimulation of the auditory nerve. 3. With the increasing application of cochlear implants in congenitally deaf children, it is important to evaluate the consequences of introducing electrical stimulation to the developing auditory nervous system.

Animals↗

[New method for the clinical study of the auditory pathway in the brainstem and cerebral primary and secondary auditory cortex using averaged auditory brain mapping for 15 seconds after sound stimulation].

A practical new method for the clinical examination of the auditory pathway from the ear to the brain is presented. Averaging of 4000 stimuli produces a graphic image of evoked potentials in the brainstem and both cerebral hemispheres. We report the results of examination with this new method in 60 normal ears of 30 healthy young people to determine the normal pattern of cerebral processing of evoked auditory signals 5 to 15 milliseconds after stimulating the ear. It is concluded that the examination is useful for studying auditory signal processing in the brain. It also demonstrated that the primary and secondary auditory cortexes are not the destination of the auditory pathway, but relay stations.

Adolescent↗

Effects of auditory pathway anatomy and deafness characteristics? (1): On electrically evoked auditory brainstem responses.

The purpose of this study was to distinguish the effects of different parameters on latencies of wave IIIe, wave Ve, and interpeak interval IIIe-Ve of electrical auditory brainstem responses (EABRs). EABRs were recorded from all the intra-cochlear electrodes in eight adult HiRes90K((R)) cochlear implant users. The relationship between latencies and stimulation sites in the cochlea was characterized to assess activity along the auditory pathway. Audiograms before implantation, psychophysics at first fitting and duration of deafness were used to describe the influence of deafness on latencies. A decreasing baso-apical latency gradient was found for waves IIIe and Ve, while the interpeak interval IIIe-Ve remained the same along the electrode array. Electrical stimulation enabling to stimulate various parts of the cochlea at the same time, this could indicate an anatomical way of compensating for the delay the acoustic wave takes to reach the cochlea apex in a non-implanted ear. However, psychophysical levels were also found to increase at the cochlear base showing that the latency gradient could result from an increasing gradient of neural degeneration toward the base. Correlations of EABR latencies with psychophysics, audiometric data and duration of deafness show that factors linked to deafness have indeed an influence on EABR latencies. The possible explanations for the latency shift observed, whether they are anatomical and/or pathological, are exposed.

Adolescent↗

[Integrated methods for assessing auditory nerve-auditory pathway integrity].

OBJECTIVE: To evaluate the feasibility and value of integrated methods to assess auditory pathway integrity. METHOD: Twenty-four cases of bilateral profoundly-deafened individuals who were considered as the candidates of cochlear implantation were included in this study. Auditory pathway integrity from these candidates of cochlear implantation were assessed with the integrated methods established by our team, which consist of 5 categories including 1. audiological test; 2. radiological imaging study; 3. ear-canal electric audiometry; 4. response to sound in daily life; and 5. speech development. RESULT: Twenty-three candidates who meet the criteria of auditory nerve-auditory pathway integrity received cochlear implantation with improved hearing and speech development postoperatively. The remainder one of the 24 candidates was diagnosed as bilateral absence of auditory nerve. The fault of cochlear implantation was avoided. CONCLUSION: The integrated methods for assessing auditory pathway integrity is feasible and valuable. Auditory nerve-auditory pathway integrity should be considered and included as one of the most important criteria for cochlear implantation candidate.

Acoustic Stimulation↗

Time course of axonal myelination in the human brainstem auditory pathway.

Structures in the human brainstem auditory pathway, from the proximal end of the cochlear nerve to the inferior colliculus, undergo myelination between the 26th and 29th fetal weeks. By the 26th week of gestation, axons in the cochlear nerve and brainstem pathways have acquired linear arrays of oligodendrocytes, and faint myelin sheaths can be distinguished. By the 29th week, definitive myelination is present in all auditory pathways, including the proximal end of the cochlear nerve, trapezoid body, lateral lemniscus, dorsal commissure of the lemniscus, commissure of the inferior colliculus and brachium of the inferior colliculus. Subsequent to the 29th gestational week, density of myelination increases in all pathways until at least 1 year postnatal age. The time of onset of myelination coincides with the onset of acousticomotor reflexes and brainstem auditory evoked responses, processes which depend on rapid, synchronized conduction of auditory impulses in the cochlear nerve and brainstem. The cotemporality in appearance of myelin, reflex responses, and evoked responses supports the idea that the 26th to 28th gestational weeks are a critical period in the onset of human central auditory function. The subsequent increase in myelin density is likely to be a factor in the steady decrease in ABR wave III-V latencies observed during the perinatal period.

Axons↗

Threshold sensitivity of the auditory pathway: effects of worsening signal-to-noise ratio in the auditory nerve.

Perfusions of scala tympani with high potassium solutions increase spontaneous activity of auditory nerve fibres without affecting their threshold sensitivity. In these circumstances, however, the signal-to-noise ratio of the auditory nerve response to a given stimuli is worsened. Recordings from cochlear nucleus neurones during such perfusions indicate that this worsening is critical for threshold sensitivity at higher levels in the auditory pathway.

Acoustic Stimulation↗

Effects of aircraft noise on hearing and auditory pathway function of airport employees.

The effects of aircraft noise on hearing and auditory pathway function were studied in 112 airport employees, both by audiometry and brainstem auditory evoked potentials (BAEPs) to evaluate cochlear function and to verify the possibility of retrocochlear involvement. Employees were divided into five groups according to their daily jobs. Group A was made up of 23 maintenance workers, Group B of 20 firemen, Group C of 24 policemen, Group D of 34 airline ground staff, and Group E of 14 civil servants. The typical audiogram pattern of noise-induced hearing loss (NIHL) was a dip at 3 or 4 kHz and moderate hearing loss in the frequency range of 6 to 8 kHz. The results of audiograms in this study revealed the prevalence rate of high-frequency loss in all employees was 41.9%. The incidences of NIHL were highest in the groups of maintenance workers (65.2%) and firemen (55.0%), who are almost continuously exposed to aircraft noise. As for the BAEPs, both click threshold and latencies showed that the impairment was most severe in the groups of maintenance workers and firemen. There was prolongation in central conduction time, shown mainly in intervals of I-V and III-V. This suggests that involvement of the central auditory pathway, especially between the pons and midbrain, is present. In summary, the degree of auditory damage coincided with job patterns. Furthermore, damage of both peripheral cochlear organs and the central auditory pathway by high-frequency aircraft noise exposure was confirmed.

Adult↗

Regeneration of the central auditory pathway in adult rats.

Regeneration of the auditory pathway in the central nervous system, which has not been reported previously, was investigated in adult rats. The ventral cochlear tract (VCT) in the medulla oblongata of adult rats was completely transected unilaterally by a ventral approach through an opening in the basioccipital bone. After postoperative intervals of 1 day to 3 months, the cochlear tract was examined by an anterograde tracing method using wheat germ aggulutinin-conjugated horseradish peroxidase (WGA-HRP) injected unilaterally in the ventral cochlear nucleus (VCN) ipsilateral to the lesion. In this simple transection experiment no regeneration was observed. However, when embryo brainstem tissue was transplanted on the transected site, regeneration occurred in about 30% of the rats examined. These results indicate the occurrence of obvious regeneration in the central auditory pathway, which was previously thought not to be restored once damaged, and they raise hope for future treatment of humans.

Animals↗

Hypoxia-induced ABR change and heat shock protein expression in the pontine auditory pathway of young rabbits.

The auditory brainstem response (ABR) was compared with the immunohistochemical expression of heat shock protein (HSP-72) and microtubule-associated protein 2 (MAP-2) of the brainstem auditory pathway in young rabbits subjected to hypoxic stress. Severe hypoxia for 2 h produced significant prolongation and decreased amplitude of the later component of ABR. HSP-72 expression was distinctly increased in the cochlear nucleus, but there was less induction in the inferior colliculus under severe hypoxia. MAP-2 immunostaining of neuropiles in the inferior collicular nucleus was decreased slightly after severe-long hypoxia, but cytoplasmic staining did not change. The present ABR change, which was produced by brainstem hypoxia-ischemia and acidosis, may be due to the neural cytoarchitectural derangement and less induction of stress proteins in the upper brainstem.

Animals↗

Distribution of Fos-like immunoreactivity in the auditory pathway evoked by bipolar electrical brainstem stimulation.

OBJECTIVE: The auditory brainstem implant (ABI) represents a new modality for the treatment of patients deafened as a result of complete excision of a bilateral VIIIth nerve tumor. However, little work has been done on the effect of the ABI on the mammalian auditory pathway. The aim of this study was to demonstrate the effect of the ABI using Fos-like immunoreactivity. MATERIAL AND METHODS: A bipolar electrode was implanted in the dorsal cochlear nucleus of bilaterally deafened Sprague-Dawley rats, and electrical stimulation was presented at an intensity four times that of threshold. RESULTS: Fos-like immunoreactivity was induced in the neurons of various auditory brainstem nuclei and observed in the low-to-middle frequency area. In the ipsilateral dorsal cochlear nucleus, ipsilateral posterior ventral cochlear nucleus and bilateral inferior colliculus, Fos-like immunoreactive neurons were observed as a distinct banding pattern. CONCLUSIONS: This study showed that Fos-like immunoreactivity was observed in the restricted area of the primary brainstem auditory pathway with the appropriate tonotopicity. These results indicate that the ABI can provide auditory information suitable for speech recognition.

Animals↗