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Postnatal development of auditory function in the chicken revealed by auditory brain-stem responses (ABRs).

Auditory evoked brain-stem responses (ABRs) were recorded from the surfaces of the brain of lightly anesthetized newborn (1-7 days old) and adult (7-9 weeks old) chickens as a measure of the development of auditory processing. One-day-old and older chickens showed a series of waves within 5 msec after the stimulus onset. This precocity of the ABR in chickens contrasts with the first appearance of the ABR in cats at 4 days of age. The ABR onset latency was shorter in adult chickens than in newborns. This indicates that developmental modifications of mechanical transmission in the external and middle ear or cytodifferentiation of the sensory hair cells of the basillar papilla and the neurons of the acoustic nerve continue postnatally. Within the complex wave form of the response, most of the inter-wave latencies decreased with maturation, indicating that development of the central auditory pathway also continues postnatally. One inter-wave latency (N1 to P3-4) was significantly shorter (P less than 0.05) in adults than in newborns for intense click stimuli, and even among newborns, this inter-wave latency was significantly shorter in 6- and 7-day-old specimens than in 1-3-day-old specimens. It seems likely that changes in the N1 to P3-4 inter-wave latency reflect changes in evoked activity of second order auditory neurons that are located in the nucleus angularis and nucleus magnocellularis, and that intensive developmental changes occur in these neurons during the first postnatal week. The ABR recorded in chickens is a reliable measure of functional activity in the auditory system which is reproducible between individuals and capable of demonstrating developmental changes in specific segments of the wave form.

Animals↗

The effects of DFMO on polyamine metabolism in the inner ear.

Difluoromethylornithine (DFMO) is a novel antineoplastic agent that was associated with an unexpected hearing loss in Phase II clinical trials. DFMO interferes with polyamine synthesis by inhibition of the enzyme ornithine decarboxylase (ODC). The objective of the current study was to establish a methodology to determine the effect of DFMO on polyamine levels and ODC activity in the cochlea. Guinea pigs received DFMO in their drinking water and were tested for auditory brainstem response threshold shifts. The organ of Corti, the lateral wall, and the acoustic nerve were assayed for both ODC activity and polyamine levels. In DFMO treated animals there was an inhibition of ODC activity in cochlear tissues as well as in intestinal mucosa. In addition, a significant depletion of cochlear polyamines was observed in the treatment animals. This study suggests that systemically administered DFMO inhibits ODC activity and interferes with polyamine synthesis in the cochlea.

Animals↗

Presence and distribution of three calcium binding proteins in projection neurons of the adult rat cochlear nucleus.

The presence and distribution of three cytoplasmic calcium binding proteins, calbindin, calretinin, and parvalbumin, have been investigated in the projection neurons of the cochlear nucleus complex in adult rats by using immunohistochemistry in free-floating slices. Identification of the individual cell types was carried out on the basis of their intranuclear localization, morphological characteristics, and (in the cases of pyramidal and bushy neurons) by retrograde labeling with rhodamine-dextran. The most important findings were confirmed by using confocal microscopy. The data obtained in these experiments are the first to demonstrate the presence of parvalbumin in pyramidal neurons and globular and spherical bushy cells of rat cochlear nucleus, whereas octopus and giant cells did not show positivity for parvalbumin. Calretinin was not present in either Purkinje-like cells or giant neurons. According to the double immunolabeling co-localization experiments, the pyramidal neurons, Purkinje-like cells, globular bushy cells, and octopus cells express two different calcium binding proteins in their cytoplasm (although in different combinations) whereas giant cells and spherical bushy cells contain solely calbindin and parvalbumin, respectively. The presence of calretinin in globular bushy cells provides a tool for distinguishing them from spherical bushy cells. The immunolabeling of the fibers and axonal endings of the acoustic nerve in the ventral part of the cochlear nucleus indicated that these structures are also parvalbumin positive. It is concluded that the heterogenous cell composition of the cochlear nucleus is accompanied by a rather complex expression pattern of the cytoplasmic calcium binding proteins.

Animals↗

Hexachlorobenzene, a dioxin-like compound, disrupts auditory function in rat.

Hexachlorobenzene (HCB) is a dioxin-like compound widely distributed in the environment. In this study, we investigated the effects of HCB on the cochlea. Conscious free-moving rats were given HCB per os daily for 4 weeks at doses of 0.16, 4 or 16 mg/kg in olive oil, whereas the control group received olive oil only. The effects of HCB were evaluated at various time intervals, by measuring auditory nerve acoustic thresholds and plasma thyroid hormone concentration by radioimmunoassay. Histological evaluation involved surface preparation and scanning electron microscopy observations of cochlear hair cells. At a dose of 0.16 mg/kg, HCB induced no loss of acoustic sensitivity, whereas at 4 mg/kg, it induced cochlear sensitivity deficits at the mid-frequencies (2-16 kHz) with complete recovery once treatment was stopped. At a dose of 16 mg/kg, permanent threshold shifts were observed at all frequencies tested (from 1 to 32 kHz). Morphological studies showed no cochlear hair cell loss or alteration of stereocilia. HCB treatment reduced circulating thyroxine concentrations. Thyroidectomy had no effect on cochlear sensitivity in control animals. Thus, HCB is a potent oto-toxicant, and its ototoxicity may be independent of its thyroidal effects.

Animals↗

Relationship between NRT measurements and behavioral levels in children with the Nucleus 24 cochlear implant may change over time: preliminary report.

OBJECTIVE: Response from spiral ganglion cells to electrical stimulation via the Nucleus 24 cochlear implant can be measured using the neural response telemetry system. The purpose of this study was to assess, in children, the correlation between the neural response threshold and the behavioral levels used for cochlear implant programming process. METHODS: The neural response telemetry test was administered to 23 children (mean age at implantation: 4 years) with the Nucleus 24 cochlear implant. Four intra-cochlear electrodes (electrodes 5, 10, 15 and 20) were tested. The neural response threshold at 3, 6, 9 and 12 months post-implantation was compared with the behavioral threshold and the maximum comfort level estimated during the same periods: a Pearson's correlation test was performed for each tested electrode. RESULTS: On apical electrodes, the correlation with the behavioral threshold remained significant from 3 to 12 months post-implantation (r ranging from 0.696 to 0.909, P<0.05), and the correlation with the maximum comfort level was also significant throughout the study period, except on electrode 15 at 9 months (tendency to significance). On basal and intermediate electrodes, statistical correlations were found only at some points of time; nonetheless, at 12 months post-implantation, a significant correlation with behavioral levels could be clearly demonstrated both on electrode 15 (r=0.914--0.778, P<0.05) and on electrode 10 (r=0.845--0.720, P<0.05). CONCLUSIONS: This preliminary study suggests that the correlation between the neural response threshold and behavioral levels may improve from the base towards the apex of the cochlea. However, a significant correlation can be demonstrated for all tested electrodes at 12 months post-implantation. During the first months post-implantation care must be exercised when interpreting neural response telemetry measurements: a positive test does not necessarily mean that the stimulus delivered to the acoustic nerve will be centrally processed with the result of an auditory perception.

Action Potentials↗

Cochlear implantation in a child with osteogenesis imperfecta.

Osteogenesis imperfecta (OI) is a hereditary disease of connective tissue and affects bone, dentine, sclera, joint, tendon, blood vessels, heart valves, and skin. Approximately 50% of the adult patients with OI have associated hearing impairment. To date, only three cases of cochlear implantation in adults with OI have been reported, but none in children. We present a case of cochlear implantation in a congenitally deaf 6-year-old boy with OI. The Nucleus 24 Contour device was successfully implanted using the suprameatal approach (SMA). At 6 months post-initial stimulation there was no evidence of non-acoustic nerve excitation (i.e. facial twitching) or discomfort, and significant progress in auditory abilities was manifested by open set word identification.

Audiometry↗

How well do we understand the cochlea?

As sensory cells, hair cells within the mammalian inner ear convert sounds into receptor potentials when their projecting stereocilia are deflected. The organ of Corti of the cochlea contains two types of hair cell, inner and outer hair cells, which differ in function. It has been appreciated for over two decades that although inner hair cells act as the primary receptor cell for the auditory system, the outer hair cells can also act as motor cells. Outer hair cells respond to variation in potential, and change length at rates unequalled by other motile cells. The forces generated by outer hair cells are capable of altering the delicate mechanics of the cochlear partition, increasing hearing sensitivity and frequency selectivity. The discovery of such hair-cell motility has modified the view of the cochlea as a simple frequency analyser into one where it is an active non-linear filter that allows only the prominent features of acoustic signals to be transmitted to the acoustic nerve by the inner hair cells. In this view, such frequency selectivity arises through the suppression of adjacent frequencies, a mechanical effect equivalent to lateral inhibition in neural structures. These processes are explained by the interplay between the hydrodynamic interactions among different parts of the cochlear partition and the effective non-linear behaviour of the cell motor.

Animals↗

Effect of SOD1 overexpression on age- and noise-related hearing loss.

Reactive oxygen species (ROS) have been implicated in hearing loss associated with aging and noise exposure. Superoxide dismutases (SODs) form a first line of defense against damage mediated by the superoxide anion, the most common ROS. Absence of Cu/Zn SOD (SOD1) has been shown to potentiate hearing loss related to noise exposure and age. Conversely, overexpression of SOD1 may be hypothesized to afford a protection from age- and noise-related hearing loss. This hypothesis may be tested using a transgenic mouse model carrying the human SOD1 gene. Contrary to expectations, here, we report that no protection against age-related hearing loss was observed in mice up to 7 months of age or from noise-induced hearing loss when 8 week old mice were exposed to broadband noise (4-45 kHz, 110 dB for 1 h). Mitochondrial DNA deletion, an index of aging, was elevated in the acoustic nerve of transgenic mice compared to nontransgenic littermates. The results indicate the complexity of oxidative metabolism in the cochlea is greater than previously hypothesized.

Age Factors↗

Cochlear implantation in a patient with grand mal epilepsy.

A case is reported in which a Nucleus 22 channel intracochlear implant was used to treat a deaf Hungarian woman (aged 37 years) with a 34-year history of grand mal (GM) epilepsy maintained on carbamazepine-diazepam combination therapy who had not benefited from conventional hearing aids. Pre-operative electrical stimulation of the acoustic nerve, however, exhibited a good nerve function with no evidence of abnormal waveforms in the electroencephalogram (EEG). Successful intracochlear insertion of the 22 electrode resulted in a 40 dB hearing improvement at frequencies 250-2000 Hz in the implanted ear with no signs of pathologic wave activity at either the previously recognized epileptic focus (fronto-precentral region) or indeed, in other regions of the brain at use of the implant. We conclude that intracochlear implantation per se is not a hazardous intervention in patients with fronto-precentral epileptic foci.

Adult↗

Meningioma of the internal auditory canal with extension into the vestibule.

Meningiomas account for approximately 18 to 19 per cent of all brain tumours. Although they can arise in numerous locations, meningiomas of the internal auditory canal (IAC) are rare. Most tumours that originate in the IAC are schwannomas of the VIIIth cranial nerve (acoustic neuromas). We report a case of a meningioma which appears to originate from the IAC and extends into the vestibule. The clinical findings and the radiographical features of meningiomas of the IAC are similar to those of acoustic neuromas. Pre-operative differentiation between acoustic neuromas and meningiomas of the IAC may be difficult.

Diagnosis, Differential↗

Immunocytochemistry of glycine in small neurons of the granule cell areas of the guinea pig dorsal cochlear nucleus: a post-embedding ultrastructural study.

The axon terminals of the acoustic nerve contact different part of the cochlear nucleus including granule cell areas. Little is known of the cell composition and neural circuits of granule cell areas present in the fusiform and upper polymorphic layers of the dorsal cochlear nucleus in the guinea pig. The present ultrastructural immunocytochemical study exploits the technique of post-embedding immunogold and silver intensification to reveal the characteristics of small neurons in granule cell areas. Few neurons (Golgi-stellate cells) use glycine as inhibitory neurotransmitter which is present in symmetric synaptic boutons with pleomorphic and flat vesicles. In contrast, most neurons (granule and unipolar brush cells) are not glycine-positive, and presumably not excitatory. Most of the large axons (mossy fibres) in granule areas are probably excitatory (glycine-negative and storing round synaptic vesicles) and contact unipolar brush cells forming large synapses or granule cell dendrites by small synapses. A few large glycinergic boutons (inhibitory) also contact unipolar brush cells. The excitatory circuit of mossy fibre-unipolar brush and granule cells may be inhibited by the glycinergic terminals from the few glycinergic cells (Golgi-stellate neurons) present within the granule cell areas. The latter are not contacted by large mossy-like glycine terminals.

Animals↗

Brainstem auditory evoked responses in rats with experimental chronic renal failure.

1. Chronic renal failure was induced in rats by five-sixths nephrectomy. Brainstem auditory evoked response (BAER) was recorded after 3 months. 2. In the uraemic rats latency of the first wave was delayed, while the interpeak I-V latency was similar to that of the controls. 3. These results suggest a delayed neural conduction along the acoustic nerve or cochlear changes in uraemic rats.

Animals↗