Neonatal sound deprivation affects brain stem auditory nuclei

CBA/J mice deprived of airborne sound stimulation during postnatal development have smaller globular cells in the ventral cochlear nucleus and smaller neurons in the medial nucleus of the trapezoid body than do normal control mice. The sound deprivation in these mice is similar to that experienced by persons with pure congenital conductive hearing losses. Even more profound central neural changes were found in auditory nuclei in the brain stem of a congenitally sensorineural deaf human.     

Cochlear implantation: a review of the literature and the Nijmegen results

The field of cochlear implantation is developing rapidly. In subjects with bilateral profound deafness who gain no benefit from conventional hearing aids the aim of cochlear implantation is to provide a means for them to receive auditory sensations. Throughout the world, most cochlear implant centres are still continuing their research efforts to improve the results with this technique. Although it is still difficult to predict how an individual will perform with a cochlear implant, the success of cochlear implantation can no longer be denied. In this paper, we review some recent papers and reports, and the results of the various Nijmegen cochlear implant studies. Data about subject selection, examinations, surgery and the outcome are discussed. Our results were in good agreement with those of other authors. It can be concluded once again that cochlear implantation is an effective treatment for postlingually deaf adults and children, and for prelingually (congenital or acquired) deaf children with profound bilateral sensorineural deafness.     

Analysis of evoked otoacoustic emissions in patients with high frequency cochlear hearing loss

Evoked otoacoustic emissions (EOAE) and auditory brainstem evoked response (ABR) were tested in patients with high frequency cochlear hearing loss and subjects with normal hearing. Results showed that: 1. In the normal group, the main component of EOAE varied between 0.5-5kHz. 2. There was a close correlation between the pure-tone audiogram figure and EOAE spectrum. 3. The I/O function curves of EOAE displayed non-linear characteristics and a trend of saturation with higher stimulus levels in the normal group. In the patient group the growth rates of EOAE and ABR were remarkably higher than those in the normal group with high stimulus levels. It suggests that the abnormal EOAE are probably relevant to the recruitment phenomenon in cochlear hearing loss.     

Otoacoustic emissions in full-term newborns at risk for hearing loss

Distortion product otoacoustic emissions (DPOEs) and click-evoked otoacoustic emissions (CEOEs) characteristics of the normal newborn population have been previously reported in the literature. There is little information about DPOE evaluations in the newborn population at risk for hearing loss. The authors now report the DPOE and/or CEOE data from six full-term subjects at risk for hearing loss or with highly suspected hearing loss. These subjects were less than 1 year of age and at risk for hearing loss secondary to a history of hereditary hearing loss, meningitis, hyperbilirubinemia, and ototoxic drug exposure. Audiometric evaluation included auditory brainstem responses (ABR), behavioral observation audiometry, and tympanometry. The CEOEs and DPOEs were found to be decreased or absent in the subjects with suspected hearing loss secondary to cochlear pathology; they were found to be normal in a subject with a suspected central hearing loss. This study's data suggest that otoacoustic emissions when combined with ABR can provide a frequency-specific evaluation of cochlear function and help determine the anatomic site of a pathologic lesion.     

Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging

PURPOSE: To introduce aplasia or hypoplasia of the vestibulocochlear nerve (VCN) as a possible cause of hearing loss and to identify the magnetic resonance (MR) imaging characteristics of this entity. MATERIALS AND METHODS: In seven patients with congenital deafness or unexplained sensorineural hearing loss, MR imaging enabled diagnosis of aplasia or hypoplasia of the VCN. Axial (0.7-mm) three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) images and parasagittal reconstruction images perpendicular on the course of the VCN were obtained. Twenty normal inner ears were also studied; their findings were compared with those of the patients. RESULTS: The facial nerve and inferior and superior vestibular and cochlear branches of the VCN were identified on the MR images in the 20 normal inner ears. Aplasia of the VCN was detected in two patients with normal labyrinths but with a severe stenosis of the internal auditory canal. A common VCN with absence of the cochlear branch was found bilaterally in two patients with a congenital malformation of the labyrinth. A common VCN with absence or hypoplasia of the cochlear branch was found in three patients with normal internal auditory canals and labyrinths. CONCLUSION: Submillimetric gradient-echo images (eg, 3DFT-CISS) should always be used to exclude aplasia or hypoplasia of the cochlear branch of the VCN in all cochlear implant candidates and patients with congenital deafness. This entity, which can occur with or without associated labyrinthine malformation, should be confirmed in two planes.     

Inputs to a physiologically characterized region of the inferior colliculus of the young adult CBA mouse

Presbycusis is a sensory perceptual disorder involving loss of high-pitch hearing and reduced ability to process biologically relevant acoustic signals in noisy environments. The present investigation is part of an ongoing series of studies aimed at discerning the neural bases of presbycusis. The purpose of the present experiment was to delineate the inputs to a functionally characterized region of the dorsomedial inferior colliculus (IC, auditory midbrain) in young, adult CBA mice. Focal, iontophoretic injections of horseradish peroxidase were made in the 18-24 kHz region of dorsomedial IC of the CBA strain following physiological mapping experiments. Serial sections were reacted with diaminobenzidine or tetramethylbenzidine, counterstained and examined for retrogradely labeled cell bodies. Input projections were observed contralaterally from: all three divisions of cochlear nucleus; intermediate and dorsal nuclei of the lateral lemniscus (LL); and the central nucleus, external nucleus and dorsal cortex of the IC. Input projections were observed ipsilaterally from: the medial and lateral superior olivary nuclei; the superior paraolivary nucleus; the dorsolateral and anterolateral periolivary nuclei; the dorsal and ventral divisions of the ventral nucleus of LL; the dorsal and intermediate nuclei of LL; the central nucleus, external nucleus and dorsal cortex of the IC outside the injection site; and small projections from central gray and the medial geniculate body. These findings in young, adult mice with normal hearing can now serve as a baseline for similar experiments being conducted in mice of older ages and with varying degrees of hearing loss to discover neural changes that may cause age-related hearing disorders.     

Afferent projection patterns in the auditory brainstem in normal and congenitally deaf white cats

Cochlear implantation in congenitally deaf children is developing to a successful medical tool. Little is known, however, on morphology and pathophysiology of the central auditory system in these auditory deprived children. One form of congenital hearing loss, that seen in the deaf white cat, was investigated to see if there are differences in the afferent pathways from the cochlear nuclei to the inferior colliculus. The retrogradely transported fluorescent tracer diamidino yellow (DY) was injected into different parts of the central nucleus of the inferior colliculus (ICC) of normal cats and deaf white cats. It was found that the main afferent projection patterns in deaf white cats were unchanged in spite of congenital auditory deprivation; minor differences were seen.     

Afferent influences on the development of the brain stem auditory nuclei of the chicken: otocyst ablation

The effects of embryonic deafferentation on the morphological development of the avian cochlear nuclei, n. angularis (NA) and n. magnocellularis (NM), were investigated. The right otocyst was surgically removed from chick embryos at 55 to 60 hours of incubation and the subsequent development of total volume, neuron number, and neuron cross-sectional area were studied with quantitative methods in animals sacrificed at 2-day intervals between embryonic days 9 and 19 and at 28 days posthatching. The development of NA and NM is severely affected by otocyst ablation. Between embryonic days 9 and 19, a large group of NA neurons in the medioventral portion of the nucleus on the operated side moves to an ectopic ventromedial position, while the remainder of this nucleus stays in its normal dorsolateral position. Beginning about day 13 of incubation, the normal increase in the volume of NA and the size of its neurons becomes progressively retarded and 40% of its neurons are lost. The growth of NM is also retarded after day 11 of incubation and the growth of mean neuron size is retarded after day 15. There is a 30% loss of neurons in NM which begins after embryonic day 11. The results indicate the primary cochlear fibers make a critical contribution to the growth and maintenance of their target neurons. The absence of this facilitative influence following otocyst ablation becomes apparent just at the time synapses would normally be formed between the the primary auditory afferents and the brain stem auditory neurons. The abnormal movement of neurons in nucleus angularis to an ectopic position after otocyst ablation suggests that primary auditory afferents may serve to stabilize the position of their target cells within the developing brain.     

Alpha-pompilidotoxin (alpha-PMTX), a novel neurotoxin from the venom of a solitary wasp, facilitates transmission in the crustacean neuromuscular synapse

The volume of cochlear nucleus and nucleolus, neural populations were determined in rats of three groups aged (1, 2-3, 22-28 months). The study data indicated that there is no difference between the volume of cochlear nucleus in the aged and adult animals (P > 0.05). Compared with the other two groups, the aged animals show a reduction of volume of necleolus of cochlear nucleus neurons (P < 0.01), a loss of neurons (spherical cell and multipolar cell) (P < 0.01). On the other hand, it shows an increase in number for the glial cells in the aged animals (48.48%, P < 0.01). Quantitative results of the cell of cochlear nucleus were analysed. We suggest that central changes also play an important role in presbycusis.     

Cochlear neuronal loss are determined by use of a neurofilament protein antibody in cases of bilateral profound deafness

The temporal bones of two patients with profound bilateral deafness from infancy were studied immunohistochemically, using a neurofilament protein antibody to detect the cochlear neuronal elements. One patient exhibited Mondini dysplasia of the inner ear, with the organ of Corti almost completely deteriorated. The other patient is the first reported case involving complete aplasia of the organ of Corti in all turns. In both cases, the immunohistochemical staining clearly revealed a severe reduction in the number of afferent neurons, such as dendrites, spiral ganglion cells and cochlear axons. The number of efferent spiral bundles in the osseous spiral lamina and intraganglionic portion also decreased in parallel with the reduction in the number of cochlear afferent neurons. Our results are inconsistent with previously reported cases of presbycusis and acquired deafness induced by the measles virus, in which efferent neurons were preserved while afferent neurons degenerated. The loss of both the efferent and afferent neurons might be characteristics of congenital deafness.     

Transient CD15 expression reflects stages of differentiation and maturation in the human subcortical central auditory pathway

The expression of the terminal saccharide determinant CD15 (3[a1-3]-fucosyl-N-acetyl-lactosamine) was evaluated in the central auditory system of the human developing brain by using monoclonal antibodies against this epitope. CD15 immunoreactivity was first observed in the ventral cochlear nucleus at 10 weeks of gestation, whereas the dorsal cochlear nucleus became positive from 13 weeks of gestation. In both nuclei, the intensity of immunoreactivity increased until 16 weeks of gestation and lasted until 25 weeks of gestation. In the inferior colliculi, CD15 was poorly expressed in the central nucleus from 13 to 23 weeks of gestation and later with moderate levels until birth. Within the medial geniculate nucleus, a biphasic pattern of expression was observed with peaks around 14-17 and 21-24 weeks of gestation. Heterogeneous expression in the medial geniculate nucleus, which was associated either with neurons or the neuropil, allowed distinction of subnuclei. In many of the auditory pathway structures (e.g., ventral cochlear nucleus and central nucleus of the inferior colliculus), a heterogeneous pattern of CD15 expression in the form of repeating parallel bands, possibly related to tonotopic organization, became transiently apparent around 23 weeks of gestation, whereas in the magnocellular part of the medial geniculate nucleus, a striking modular or compartmental arrangement of immunoreactive structures (which could also be associated with tonotopic organization) was also noted at about 23 weeks of gestation. We propose that the initiation of CD15 expression in each nucleus heralds the appearance of functional contacts and that high levels of neuropil labeling are related to the formation of nonstabilized synaptic contacts. Thus, transient CD15 expression in the central auditory system is possibly correlated with phases of functional plasticity in this pathway.     

Positron emission tomography of auditory sensation in deaf patients and patients with cochlear implants

The present study investigated the function of the auditory cortices in severely hearing-impaired or deaf patients and cochlear implant patients before and after auditory stimulation. Positron emission computed tomography (PET), which can detect brain activity by providing quantitative measurements of the metabolic rates of oxygen and glucose, was used. In patients with residual hearing, the activity of the auditory cortex measured by PET was almost normal. Among the totally deaf patients, the longer the duration of deafness, the lower the brain activity in the auditory cortex measured by PET. Patients who had been deaf for a long period showed remarkably reduced metabolic rates in the auditory cortices. However, following implantation of the cochlear device, the metabolic activity returned to near-normal levels. These findings suggest that activation of the speech comprehension mechanism of the higher brain system can be initiated by sound signals from the implant devices.     

Pediatric and psychiatric comorbidity. Part I: The future of consultation-liaison psychiatry

This study was conducted to investigate the influence of high-frequency aircraft noise on the function of the auditory system of school-age children. A total of 228 students attending a school near an airport (school A) and 151 students attending a school far from an airport (school B) were analyzed. Audiometry and brainstem auditory evoked potential (BAEP) detection were performed in all subjects to evaluate cochlear and retrocochlear function. The results of audiometry indicated that hearing ability was significantly worse in the children of school A, which was located under the flight paths. The values of pure tone average, high pure tone average, and threshold at 4 kHz were all higher in children who were frequently exposed to aircraft noise. There was no consistent difference in BAEP latencies between the two schools. These results indicate that central transmission is not affected in children who have been exposed to aircraft noise for several years. The results of the present study showed a significant association between aircraft noise exposure and prevalence of noise-induced hearing loss. Although damage to peripheral cochlear organs was confirmed in school-age children, involvement of the central auditory pathway could not be verified.     

Transitory evoked and distortion products of otoacoustic emissions in absent auditory evoked potentials

Transient click-evoked otoacoustic emissions (TEOAE) and distortion-product otoacoustic emissions (DPOAE) are produced by an active biomechanical process in the cochlea, presumably related to outer hair-cell activity. Although it is generally accepted that in most cases of hearing loss with absent auditory evoked potentials neither TEOAE nor DPOAE can be found, some cases with such a constellation have been described. Here we report another four cases of children with severe to profound hearing loss where we discovered reproducible TEOAE and DPAOE, whereas auditory evoked potentials were missing. TEOAE and DPOAE recordings in these cases indicate substantially preserved outer hair-cell function independent of profound pre-sensineural hearing loss. Since the incidence of children with preserved otoacoustic emissions together with impairment of synaptic or postsynaptic function of the first neuron is not known, the unconditioned use of TEOAE nor DPOAE as a screening instrument must be seriously questioned. Secondly, in conjunction with subjective audiometry and brain-stem-evoked potentials, emission recordings is an indispensable measurement prior to cochlear implantation and use of high-power hearing aids.     

Deafness-induced plasticity in the mature central auditory system

Studies in rats and guinea pigs indicate that local changes in inhibitory transmitters may underlie deafness-induced plastic changes in electrophysiological responsiveness of cells of the mature central auditory system. Following 21 days of bilateral deafness there is an increase in evoked Fos-immunoreactive neurones in the central nucleus of the inferior colliculus (CIC) to contralateral cochlear electrical stimulation, compared with normal or 1-day deafened animals. Deafness is also associated with a dramatic reduction in the population of CIC neurones that respond with suppression of activity to electrical stimulation. Moreover, in vivo microdialysis reveals a marked decrease in gamma-aminobutyric acid (GABA) release from the CIC cells in deafened animals. The results may have general implications for the mediation of central nervous system plasticity induced by deafferentation of sensory input.     

Electrical stimulation of the auditory nerve. I. Correlation of physiological responses with cochlear status

The purpose of the present study was to evaluate evoked potential and single fibre responses to biphasic current pulses in animals with varying degrees of cochlear pathology, and to correlate any differences in the physiological response with status of the auditory nerve. Six cats, whose cochleae ranged from normal to a severe neural loss (< 5% spiral ganglion survival), were used. Morphology of the electrically evoked auditory brainstem response (EABR) was similar across all animals, although electrophonic responses were only observed from the normal animal. In animals with extensive neural pathology, EABR thresholds were elevated and response amplitudes throughout the dynamic range were moderately reduced. Analysis of single VIIIth nerve fibre responses were based on 207 neurons. Spontaneous discharge rates among fibres depended on hearing status, with the majority of fibres recorded from deafened animals exhibiting little or no spontaneous activity. Electrical stimulation produced a monotonic increase in discharge rate, and a systematic reduction in response latency and temporal jitter as a function of stimulus intensity for all fibres examined. Short-duration current pulses elicited a highly synchronous response (latency < 0.7 ms), with a less well synchronized response sometimes present (0.7-1.1 ms). There were, however, a number of significant differences between responses from normal and deafened cochleae. Electrophonic activity was only present in recordings from the normal animal, while mean threshold, dynamic range and latency of the direct electrical response varied with cochlear pathology. Differences in the ability of fibres to follow high stimulation rates were also observed; while neurons from the normal cochlea were capable of 100% entrainment at high rates (600-800 pulses per second (pps)), fibres recorded from deafened animals were often not capable of such entrainment at rates above 400 pps. Finally, a number of fibres in deafened animals showed evidence of 'bursting', in which responses rapidly alternated between high entrainment and periods of complete inactivity. This bursting pattern was presumably associated with degenerating auditory nerve fibres, since it was not recorded from the normal animal. The present study has shown that the pathological response of the cochlea following a sensorineural hearing loss can lead to a number of significant changes in the patterns of neural activity evoked via electrical stimulation. Knowledge of the extent of these changes have important implications for the clinical application of cochlear implants.     

Glycine immunoreactivity and receptor binding in the cochlear nucleus of C57BL/6J and CBA/CaJ mice: effects of cochlear impairment and aging

Glycinergic neurons in the cochlear nucleus (CN) of C57BL/6J (C57) and CBA/CaJ (CBA) mice were studied by using immunocytochemical and receptor-binding techniques. Adult C57 mice exhibit progressive cochlear pathology as they age, whereas aging CBA mice retain good hearing. In the CN of old C57 mice (18 months) with severe hearing loss, the number of glycine-immunoreactive neurons decreased significantly. The number (Bmax) of strychnine-sensitive glycine receptors (GlyR) decreased significantly in the dorsal CN of old C57 mice. Significant effects were not observed in the CN of middle-aged C57 mice (with less-severe hearing loss) or in very old CBA mice (which do not exhibit severe hearing loss). The data suggest that the combination of severe hearing loss and old age results in deficits in one or more inhibitory glycinergic circuits in the CN.     

Glutamate-induced Co2+ uptake in rat auditory brainstem neurons reveals developmental changes in Ca2+ permeability of glutamate receptors

Ca2+ influx through glutamate receptors (GluRs) is thought to play a crucial part in developmental processes and neuronal plasticity. Here we have examined the spatiotemporal distribution of Ca2+-permeable GluRs in auditory brainstem neurons of the rat from birth to adulthood, using the cobalt-staining technique of Pruss and collaborators. In slices of young adult rats, 1 mM glutamate evoked intense cobalt uptake in subsets of neurons in the ventral cochlear nuclei, the medial nucleus of the trapezoid body, the lateral and the medial superior olive, and the lateral lemniscal nuclei. Neurones in the central nucleus of the inferior colliculus, and thalamic auditory nuclei appear to express few, if any, Ca2+-permeable GluRs. Thus, in adults, Ca2+-permeable GluRs are present in neurons of almost all main relay stations of the auditory brainstem. During development, cobalt-stained cells first appeared at about hearing onset (at postnatal day 12 [P12]). At P16, staining levels were highest and the pattern of distribution was already adult-like. The staining intensity slightly declined during the fourth postnatal week. In contrast, Ca2+-permeable receptors were detected in the external cortex of the inferior colliculus as early as P4. Our results show that auditory neurons, characterized by a high temporal precision in neuronal activity, display Ca2+-permeable GluRs. Because Ca2+ permeability appears at about the onset of hearing and is highest during the following 2 weeks, Ca2+ influx through GluRs is likely to be implicated in remodelling processes occurring during this ontogenetic period.