Differential Ear Effects of Profound Unilateral Deafness on the Adult Human Central Auditory System

This study investigates the effects of profound acquired unilateral deafness on the adult human central auditory system by analyzing long-latency auditory evoked potentials (AEPs) with dipole source modeling methods. AEPs, elicited by clicks presented to the intact ear in 19 adult subjects with profound unilateral deafness and monaurally to each ear in eight adult normal-hearing controls, were recorded with a 31-channel system. The responses in the 70–210 ms time window, encompassing the N_1b/P₂ and Ta/Tb components of the AEPs, were modeled by a vertically and a laterally oriented dipole source in each hemisphere. Peak latencies and amplitudes of the major components of the dipole waveforms were measured in the hemispheres ipsilateral and contralateral to the stimulated ear. The normal-hearing subjects showed significant ipsilateral–contralateral latency and amplitude differences, with contralateral source activities that were typically larger and peaked earlier than the ipsilateral activities. In addition, the ipsilateral–contralateral amplitude differences from monaural presentation were similar for left and for right ear stimulation. For unilaterally deaf subjects, the previously reported reduction in ipsilateral–contralateral amplitude differences based on scalp waveforms was also observed in the dipole source waveforms. However, analysis of the source dipole activity demonstrated that the reduced inter-hemispheric amplitude differences were ear dependent. Specifically, these changes were found only in those subjects affected by profound left ear unilateral deafness.     

Of kittens and kids: altered cortical maturation following profound deafness and cochlear implant use

Profoundly deaf children who use a cochlear implant (CI) provide a unique opportunity to investigate the effects of auditory sensory deprivation on the maturing human central nervous system. Previous results suggest that children fitted with a CI show evidence of altered auditory cortical maturation, based on evoked potentials. This altered maturation was characterized by both latency delays and morphological changes in the cortical auditory evoked potentials (AEPs). Based on prolonged P(1) latencies compared to age-matched normal-hearing (NH) peers, these data suggested a delayed maturation nearly equivalent to the period of deafness. However, rates of maturation for this AEP peak were essentially the same in NH and CI children. This suggests that, given enough time, the AEPs of CI children would assume the characteristic morphology found in older NH teens and NH adults. However, the data also indicated a substantial alteration of the typical set of obligatory P(1)-N(1b)-P(2) peaks, specifically related to the absence of the N(1) potential. Recent analyses of more extensive sets of longitudinal and cross-sectional data indicate that even after many years of implant use, the AEPs of CI users in their late teens remain very different from those of their NH peers. The P(1) peak latency remains prolonged and P(1) amplitude remains much larger in CI users than in age-matched NH teens. These findings suggested that age-related changes in the P(1) peak are completed by 12 years of age. In addition, the normal N(1b) peak fails to emerge in virtually all of the CI children tested in our laboratory. A major new interpretation of the abnormal maturation of AEP waveforms in CI children is presented. It is based on direct evidence showing that a persistent immaturity of the superficial layer axons has persistent negative effects on the generation of the N(1b) and, consequently, on the morphology of the AEPs. A comparison of scalp-recorded AEPs from implanted children with local field potentials measured from the cortical surface in deaf white kittens suggests the effects of deafness and CI use are similar across these mammalian species. For both species, a period of profound deafness followed by CI stimulation reveals a substantial immaturity in cortical activation even after a period of electrical stimulation by the CI.     

Maturation of the mismatch negativity: effects of profound deafness and cochlear implant use

The use of cochlear implants to restore auditory sensation in deaf children is increasing, with a trend toward earlier implantation. However, little is known about how auditory deprivation and subsequent cochlear implant use affect the maturing human central auditory system. Our previous studies have demonstrated that the obligatory auditory evoked potentials (AEPs) of implanted children are very different from those of normal-hearing children. Unlike the obligatory potentials, which primarily reflect neural responses to stimulus onset, the mismatch negativity (MMN) provides a neurophysiological measure of auditory short-term memory and discrimination processes. The purpose of this investigation is to review our studies of the effects of auditory deprivation due to profound deafness and cochlear implant use on the maturation of the MMN in children, placed in the context of overall age-related changes in the AEPs. The development and application of a statistical technique to assess the MMN in individuals is also reviewed. Results show that although the morphology of the obligatory AEPs is substantially altered by the absence of a normal N(1) peak, the MMN is robustly present in a group of implanted children who have good spoken language perception through their device. Differences exist in the scalp distribution of the MMN between implanted and normal-hearing children. Specifically, the MMN appears to be more symmetrical in amplitude over both hemispheres, whereas it is initially much larger over the contralateral hemisphere in normal-hearing children. These findings suggest that, compared to N(1), the MMN is a better measure of basic auditory processes necessary for the development of spoken language perception skills in profoundly deaf children and adults who use a cochlear implant.     

Sensorineural hearing loss during development: morphological and physiological response of the cochlea and auditory brainstem

We have investigated the effects of sensorineural hearing loss on the cochlea and central auditory system of profoundly deafened cats. Seventeen adult cats were used: four had normal hearing; 12 were deafened neonatally for periods of < 2.5 years (five bilaterally, seven unilaterally); and one animal had a long-term (approximately 8 years) profound bilateral hearing loss. Bipolar scala tympani stimulating electrodes were bilaterally implanted in each animal, and electrically evoked auditory brainstem responses (EABRs) were recorded in an acute study to evaluate the basic physiologic response properties of the deafened auditory pathway. The cochleae and cochlear nuclei (CN) of each animal were examined with light microscopy. Spiral ganglion cell density in neonatally deafened cochleae was 17% of normal, and only 1.5% of normal in the long-term deaf animal. There was a 46% reduction in total CN volume in neonatally deafened animals compared to normal, and a 60% reduction in the long-term deaf animal. Neural density in the anteroventral CN of bilaterally deafened animals was 37% higher than normal; 44% higher in the long-term deaf animal. Significantly, however, we saw no evidence of a loss of neurones within the anteroventral CN in any deafened animal. There was a significant increase in EABR threshold and wave IV latency in the deafened animals, and a significant decrease in response amplitude and input/output function gradient. Again, these changes were more extensive in the long-term deaf animal. These data show that a sensorineural hearing loss can evoke significant morphological and physiological changes within the cochlea and auditory brainstem, and these changes become greater with duration of deafness. It remains to be seen whether these changes can be reversed following the introduction of afferent activity via chronic electrical stimulation of the auditory nerve.     

A perceptual evaluation of the speech of adventitiously deaf adult males

The present study investigated the effects of adventitious deafness on perceptual judgments of segmental and suprasegmental speech production skills. Subjects were 25 adventitiously deaf and 10 normal-hearing adult male speakers. Twelve subjects were classified as aidable profound (AP) and 13 subjects were classified as unaidable profound (UP). Each subject was judged by 15 listeners, first on seven speech and voice variables and, second, on whether they were adventitiously deaf or normal-hearing and, if adventitiously deaf, if speech rehabilitation was warranted. Both the AP and UP adventitiously deaf subjects were judged significantly different from each other and from normal-hearing subjects on all seven variables. They were, in hierarchical order: intonation, pitch, rate, nasality, vowel duration, articulation, and intensity. Aural rehabilitation was judged to be warranted in 49% of the speakers identified as being adventitiously deaf, specifically 26% of the AP speakers and 74% of the UP speakers. This finding indicates that auditory information is a necessary component for maintaining accurate speech and voice production abilities following onset of profound hearing loss after the acquisition of an adult phonological system.     

N1 action potentials in humans: influence of simultaneous contralateral stimulation

Nl action potentials, elicited by low-level, 4.0-kHz filtered clicks, were recorded from the ear canals of normal-hearing adults. Responses were recorded at two sensation levels in the presence and absence of a pure tone delivered to the contralateral ear. Waveforms were analysed for changes in amplitude and latency. A significant reduction in the amplitude of Nl was observed following the introduction of the contralateral tone at both FC levels. A concomitant change in latency was not observed. For all subjects, ipsilateral and contralateral signals were below the intensities which resulted in acoustic reflexes and crossover of contralateral signals. These data suggest that the Nl amplitude reduction observed in this study represents inhibitory activity of the efferent auditory pathway.     

N1 action potentials in humans. Influence of simultaneous contralateral stimulation

N1 action potentials, elicited by low-level, 4.0-kHz filtered clicks, were recorded from the ear canals of normal-hearing adults. Responses were recorded at two sensation levels in the presence and absence of a pure tone delivered to the contralateral ear. Waveforms were analysed for changes in amplitude and latency. A significant reduction in the amplitude of N1 was observed following the introduction of the contralateral tone at both FC levels. A concomitant change in latency was not observed. For all subjects, ipsilateral and contralateral signals were below the intensities which resulted in acoustic reflexes and crossover of contralateral signals. These data suggest that the N1 amplitude reduction observed in this study represents inhibitory activity of the efferent auditory pathway.     

Functional localization of auditory cortical fields of human: click-train stimulation

Averaged auditory evoked potentials (AEPs) to bilaterally presented 100 Hz click trains were recorded from multiple sites simultaneously within Heschl's gyrus (HG) and on the posterolateral surface of the superior temporal gyrus (STG) in epilepsy-surgery patients. Three auditory fields were identified based on AEP waveforms and their distribution. Primary (core) auditory cortex was localized to posteromedial HG. Here the AEP was characterized by a robust polyphasic low-frequency field potential having a short onset latency and on which was superimposed a smaller frequency-following response to the click train. Core AEPs exhibited the lowest response threshold and highest response amplitude at one HG site with threshold rising and amplitude declining systematically on either side of it. The AEPs recorded anterolateral to the core, if present, were typically of low amplitude, with little or no evidence of short-latency waves or the frequency-following response that characterized core AEPs. We suggest that this area is part of a lateral auditory belt system. Robust AEPs, with waveforms demonstrably different from those of the core or lateral belt, were localized to the posterolateral surface of the STG and conform to previously described field PLST.     

Congenital and Prolonged Adult-Onset Deafness Cause Distinct Degradations in Neural ITD Coding with Bilateral Cochlear Implants

Bilateral cochlear implant (CI) users perform poorly on tasks involving interaural time differences (ITD), which are critical for sound localization and speech reception in noise by normal-hearing listeners. ITD perception with bilateral CI is influenced by age at onset of deafness and duration of deafness. We previously showed that ITD coding in the auditory midbrain is degraded in congenitally deaf white cats (DWC) compared to acutely deafened cats (ADC) with normal auditory development (Hancock et al., J. Neurosci, 30:14068). To determine the relative importance of early onset of deafness and prolonged duration of deafness for abnormal ITD coding in DWC, we recorded from single units in the inferior colliculus of cats deafened as adults 6 months prior to experimentation (long-term deafened cats, LTDC) and compared neural ITD coding between the three deafness models. The incidence of ITD-sensitive neurons was similar in both groups with normal auditory development (LTDC and ADC), but significantly diminished in DWC. In contrast, both groups that experienced prolonged deafness (LTDC and DWC) had broad distributions of best ITDs around the midline, unlike the more focused distributions biased toward contralateral-leading ITDs present in both ADC and normal-hearing animals. The lack of contralateral bias in LTDC and DWC results in reduced sensitivity to changes in ITD within the natural range. The finding that early onset of deafness more severely degrades neural ITD coding than prolonged duration of deafness argues for the importance of fitting deaf children with sound processors that provide reliable ITD cues at an early age.     

The Effects of Congenital Deafness on Auditory Nerve Synapses: Type I and Type II Multipolar Cells in the Anteroventral Cochlear Nucleus of Cats

Sensory deprivation has been shown to exert detrimental effects on the structure and function of central sensory systems. Congenital deafness represents an extreme form of auditory deprivation, and in the adult white cat, synapses between auditory nerve endings and resident cells of the anteroventral cochlear nucleus exhibited abnormal structure. Endbulbs of Held were reduced in branching and displayed striking hypertrophy of their postsynaptic densities. So-called modified endbulbs showed no change in branching complexity but did exhibit hypertrophy of their postsynaptic densities. These differential pre- and postsynaptic effects prompted the question of how deafness might affect other primary endings and synapses. Thus, we studied type I and type II multipolar cells that receive bouton endings from auditory nerve fibers. Type I multipolar cells project to the contralateral inferior colliculus and have relatively few axosomatic endings; type II multipolar cells project to the contralateral cochlear nucleus and have many axosomatic endings. Compared with normal-hearing cats, bouton endings of congenitally deaf cats were smaller but there was no difference in synaptic vesicle density or size of postsynaptic densities. These data reveal that different classes of primary endings and second-order neurons exhibit different degrees of synaptic anomalies to deafness.     

Role of the efferent medial olivocochlear system in contralateral masking and binaural interactions: an electrophysiological study in guinea pigs.

Contralateral broadband noise (BBN) elevates ipsilateral auditory thresholds (central masking) and reduces the amplitude of ipsilateral brainstem auditory evoked potentials (BAEPs). Binaural interactions are complex psychophysical phenomena, but binaural interaction components are easily extracted from BAEPs to monaural versus binaural click stimulation. However, contralateral, or binaural, acoustical stimulation is known to activate simultaneously the crossed and uncrossed medial olivocochlear (MOC) efferent systems and decrease activity in both cochleas. Particularly, contralateral BBN stimulation suppresses in part ipsilateral peripheral activity. What is the role of such contralaterally induced peripheral suppression in the overall changes in central BAEPs observed during contralateral masking or binaural stimulation? Compound action potentials (CAPs) of the auditory nerve and BAEPs were recorded simultaneously in awake guinea pigs from electrodes chronically implanted on the round window of the cochlea and the surface of the brain. Peripheral and central measures of contralateral masking and binaural interactions were obtained from responses to monaural or binaural clicks, with or without contralateral BBN, recorded before, during, and after the reversible blockade of the MOC function following a single intramuscular injection of gentamicin. Contralateral BBN effectively reduced the amplitudes of CAP and of all BAEP peaks. CAP to ipsilateral click did not, however, change significantly from monaural to binaural click stimulation; still, normal binaural interaction components developed in the BAEPs. When the medial efferent function was blocked by gentamicin, the normal contralateral BBN suppression of CAP and of the earliest BAEP peak was lost; however, the later BAEP peaks were suppressed by contralateral BBN as before gentamicin, and the central binaural interaction components were unchanged. In these experimental conditions, the MOC efferent system seems to play little role in centrally recorded contralateral masking and binaural interactions.     

Comparison between simultaneously recorded auditory-evoked magnetic fields and potentials elicited by ipsilateral, contralateral and binaural tone burst stimulation

Both auditory-evoked magnetic fields (AEMF) and auditory-evoked potentials (AEP) mainly consist of three peaks with latencies of about 50, 100 and 160 ms. Comparison of responses to ipsilateral, contralateral and binaural stimulation yields no significant amplitude or latency differences of the AEP peaks whereas the simultaneously recorded AEMF peaks exhibit a 10 ms shorter latency and an approximately 38% greater amplitude for contralateral versus ipsilateral stimulation. This fact can be due to differences in the strength, location (especially the depth) and the direction of the dipole source, and a decision cannot be made considering the data recorded from just one position. Another finding is that binaural stimulation reduces the peak amplitudes by approximately 25% compared with contralateral stimulation. This result indicates some kind of interference between the ipsilateral and contralateral pathways ('binaural interaction').     

Delayed endolymphatic hydrops as a clinical entity

Delayed endolymphatic hydrops (DEH) is a clinical entity that can be differentiated from Ménière's disease and is typically observed in patients who have been suffering from longstanding unilateral profound inner-ear hearing loss. DEH probably is caused by delayed atrophy or fibrous obliteration of the endolymphatic resorptive system of the membranous labyrinth. The time that elapses between the occurrence of hearing loss and the onset of DEH can range from 1 to 74 years. The most common cause of hearing loss preceding DEH is juvenile-onset unilateral profound deafness (early childhood unilateral profound sensorineural hearing loss of unknown etiology), followed by labyrinthitis from various causes and physical and acoustic traumas to the inner ear. Two types of DEH exist: the ipsilateral type, in which the ear with profound hearing loss suffers progressive endolymphatic hydrops, and the contralateral type, in which the formation of progressive endolymphatic hydrops takes place in the ear opposite to the previously deafened ear. The incidence of the ipsilateral type is higher than that of the contralateral type, and the contralateral type is more common in older patients. When recurrent episodic vertigo cannot be remedied through conservative treatment, labyrinthectomy and vestibular neurectomy on the deaf ear are curative for ipsilateral DEH. However, no such surgical treatment is available for the contralateral type.     

T complex hemispheric asymmetries: effects of stimulus intensity

The T complex component of the human auditory evoked potential (AEP) is thought to be produced in auditory cortex, on the posterior lateral surface of the temporal lobe. Recorded over temporal scalp, it consists of an 80-90 ms positive peak, Ta, and a 120-140 negative peak, Tb. As part of an effort to develop the clinical usefulness of the T complex in assessing auditory cortical function, we studied the effects of change in monaural stimulus intensity (20-80 dB SL) on T complex latency, amplitude, and hemispheric differences in normal adults. Ta and Tb peak latencies decreased as stimulus intensity increased. These latency changes were not dependent on ear or hemisphere. Right hemisphere Ta latency was shorter with contralateral than with ipsilateral stimulation; while left hemisphere Ta latency was not dependent on the ear stimulated. Tb latency was shorter over the left hemisphere, and over the contralateral hemisphere. Ta-b amplitude increased as stimulus intensity increased. This amplitude change was not dependent on ear or hemisphere. Ta-b amplitudes were larger over the right hemisphere and over the contralateral hemisphere. Hemispheric asymmetries were not significantly affected by stimulus intensity.     

Auditory brain stem response in newborn infants--masking effect on ipsi- and contralateral recording

Ipsilateral and contralateral auditory brain stem responses (ABR) were recorded in 10 full-term neonates. We investigated the effect of the masking level on the peak latency and amplitude on ipsi- and contralateral recordings. Clicks were presented at 85 dB HL to the ipsilateral ear and the masking white noise was presented at 75, 65, 55, 45 and 0 dB HL on the contralateral one, respectively. Masking had no significant effect on the ipsi- and contralateral recording in regard to latency and amplitude except for wave CVI (contralateral wave VI). In addition, ABR was recorded in an infant with total unilateral hearing loss. Crossover responses on both sides were observed with-out contralateral masking, but these responses were completely eliminated when 45 dB HL contralateral masking masked the 85 dB HL clicks to the dead ear. Therefore, it is suggested that such crossover responses will contribute to the ipsi- and contralaterally recorded ABR waveform when an ABR recording is carried out without contralateral masking. Our results indicate that contralateral masking is necessary and should be used in cases of unilateral hearing loss.     

Cochlear Implantation in a Profoundly Deaf Patient Who also Suffers from Multiple Sclerosis — A Case Study

Outcomes of cochlear implantation in individuals with known central nervous system conditions are varied. Long-standing deafness is also thought to correlate negatively with auditory performance in post-linguistically deaf adult implant users.

We present a case study of cochlear implantation in a post-lingual adult having bilateral profound hearing loss for over 30 years in addition to multiple sclerosis unrelated to his deafness. Assessment of benefit in terms of speech-perception ability and quality of life reveal that long-term auditory deprivation and co-incidental multiple sclerosis are not a contraindication for cochlear implantation.     

Altered Cortical Activity in Prelingually Deafened Cochlear Implant Users Following Long Periods of Auditory Deprivation

Auditory stimulation during childhood is critical for the development of the auditory cortex in humans and with that for hearing in adulthood. Age-related changes in morphology and peak latencies of the cortical auditory evoked potential (CAEP) have led to the use of this cortical response as a biomarker of auditory cortical maturation including studies of cortical development after deafness and subsequent cochlear implantation. To date, it is unknown whether prelingually deaf adults, with early onset deafness (before the age of 2 years) and who received a cochlear implant (CI) only during adulthood, would display absent or aberrant CAEP waveforms as predicted from CAEP studies in late implanted prelingually deaf children. In the current study, CAEP waveforms were recorded in response to electric stimuli in prelingually deaf adults, who received their CI after the age of 21 years. Waveform morphology and peak latencies were compared to the CAEP responses obtained in postlingually deaf adults, who became deaf after the age of 16. Unexpectedly, typical CAEP waveforms with adult-like P1-N1-P2 morphology could be recorded in the prelingually deaf adult CI users. On visual inspection, waveform morphology was comparable to the CAEP waveforms recorded in the postlingually deaf CI users. Interestingly, however, latencies of the N1 peak were significantly shorter and amplitudes were significantly larger in the prelingual group than in the postlingual group. The presence of the CAEP together with an early and large N1 peak might represent activation of the more innate and less complex components of the auditory cortex of the prelingually deaf CI user, whereas the CAEP in postlingually deaf CI users might reflect activation of the mature neural network still present in these patients. The CAEPs may therefore be helpful in the assessment of developmental state of the auditory cortex.     

Changes in [14C]-2-deoxyglucose uptake in the auditory pathway of hamsters previously exposed to intense sound

The current study evaluated changes in [14C]-2-deoxyglucose (2-DG) uptake along the auditory pathways of hamsters that were exposed unilaterally to intense sound. The measurement of the acoustically evoked auditory brainstem responses indicated that intense sound exposure caused asymmetrical hearing loss. The 2-DG results revealed some changes in metabolic activity in exposed animals, as compared to unexposed animals. Significant decreases in 2-DG uptake were found in the ipsilateral anteroventral and posteroventral cochlear nucleus, with respect to the exposed left ears. Exposed animals also showed significant increases in the ipsilateral nucleus of the lateral lemniscus, central nucleus of inferior colliculus and medial geniculate body. No significant changes in uptake were observed in the ipsilateral dorsal cochlear nucleus, superior olivary complex, auditory cortex and any contralateral structures. The mechanisms for the observed changes in 2-DG uptake are discussed.     

Hereditary unilateral deaf-mutism as a variable manifestation of bilateral deaf-mutism or hearing loss]

A total of 2,519 patients with bilateral severe congenital deafness (deaf-mutism) from Central Switzerland were recorded during the period between 1834 to 1979. More recently, 31 persons with unilateral severe deafness were examined. By constructing genealogical charts going back to the 17th century, at least ten of these unilaterally deaf persons were shown to be related to patients with bilateral deafness (pedigrees I-IV). Some of them exhibited a more or less marked Klein- Waardenburg syndrome. The unilateral deafness of those patients was inherited and not acquired. Congenital unilateral deafness may be understood to be the result of the broad variability of the expressivity of acoustic defects.     

单侧耳蜗性聋患者对侧耳耳声发射的表现

目的 :探讨致聋因素在致单耳发病时对对侧耳潜在的影响。方法 :利用OtodynamicILO96耳声发射分析仪 ,对 35例单侧耳蜗性聋患者 (分为 5组 :发病 2个月以内的突发性聋 9例为S1组 ,发病 1年以上的突发性聋 6例为S2 组 ,梅尼埃病 13例分为M1、M2 组 ,不明机理的耳蜗性聋 7例为UNK组 )对侧耳瞬态诱发性耳声发射 (TEOAE)的反应幅值、反应重复率及频带的重复率 ,畸变产物耳声发射 (DPOAE)曲线特点及 1～ 5kHz反应幅值进行分析比较。结果 :S1组、M2 组TEOAE的反应幅值、反应重复性、频带重复性明显低于正常值 ,DPOAE幅值明显降低 ;S2 组、M1组、UNK组TEOAE和DPOAE正常。结论 :在不同致聋因素中 ,有的对单耳产生损伤作用 ,有的对双耳产生损伤作用。