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PubMed1994

Prepulse inhibition of the startle response in mice: relationship to hearing loss and auditory system plasticity.

Prepulse inhibition was used with C57BL/6J (C57) mice to assess behavioral-perceptual correlates of previously demonstrated physiological changes in the central auditory system associated with age-related hearing loss. Normal-hearing CBA/CaJ (CBA) mice and DBA/2J (DBA) mice, which exhibit extremely rapid hearing loss, were also tested. Tone prepulse stimuli (S1s) were presented 100 ms prior to a startle-evoking noise stimulus (S2), and a decrease in startle amplitude served as the measure of startle modification. As high-frequency hearing declined in C57 mice between 1 and 12 months of age, the efficacy of lower-frequency S1s was significantly enhanced. CBA mice exhibited no age-related changes in startle modification. DBA mice exhibited changes similar to those observed in C57s but at an accelerated rate. The enhanced behavioral saliency of low and middle frequencies in C57 and DBA mice appears to be a consequence of neural plasticity in the central auditory system.

Animals↗
PubMed1993

Wiener and Volterra analyses applied to the auditory system.

The application of a particular branch of non-linear system analysis, the functional series expansion or integral method, to the auditory system is reviewed. Both the Volterra and Wiener approach are discussed and an extension of the Wiener method from its traditional white-noise stimulus approach to that of Poisson distributed clicks is presented. This type of analysis has been applied to compound and single-unit responses from the auditory nerve, cochlear nucleus, auditory midbrain and medial geniculate body. Most studies have estimated only first-order Wiener kernels but in recent years second-order Wiener and Volterra kernels have been estimated, particularly with reference to dynamic non-linearities. A particular form of second-order analysis, the Spectro Temporal Receptive Field, offers an alternative to first-order cross-correlation when phase-lock is absent. The correlation method has revealed that neural synchronization is less affected by intensity changes and damage to the hair cells than is neural firing rate. Although the presence of the static cochlear non-linearity could be demonstrated on the basis of the intensity dependence of the first-order Wiener kernel, the identification of the exact form of the nonlinearity of the peripheral auditory system on basis of higher-order Wiener kernels has so far been inconclusive. However, successes of the method can be found in the description of the dynamic non-linearities and non-linear neural interactions.

Acoustic Stimulation↗
PubMed1993

[Acute sensory deafness originating from experimental double-membrane rupture].

Combined rupture of Reissner's membrane and the round window membrane (double-membrane rupture) as a cause of acute profound sensorineural hearing loss was investigated in guinea pigs. Action potentials (APs) of the cochlear nerve in response to tone pip stimuli (1, 2, 4, 8kHz) were recorded before and after rupture of Reissner's membrane at each turn of the cochlea. Then the round window was perforated to create leakage of the perilymph. Ninety minutes later, AP was again recorded. After these procedures, the animal was sacrificed for histological study to confirm the rupture of Reissner's membrane. The ears with double-membrane rupture showed a larger decrease in AP amplitude at all test frequencies than did control ears with round window rupture alone. The ears in which Reissner's membrane was ruptured at the second turn showed more a pronounced decrease in AP amplitude than those ruptured at the third and apical turns. It was concluded that double-membrane rupture could be a cause of sudden deafness, but that the disturbance of cochlear function depends on where Reissner's membrane ruptures.

Action Potentials↗
PubMed2005

Facial nerve function and hearing preservation after retrosigmoid excision of vestibular schwannoma: Christchurch Hospital experience with 97 patients.

BACKGROUND: Between January 1988 and December 2002, 97 patients underwent surgery for excision of vestibular schwannoma via the retrosigmoid approach at Christchurch Hospital. METHODS: A retrospective review was undertaken of the clinical notes with emphasis on facial nerve function and hearing preservation postoperatively. RESULTS: Of patients with small and medium-sized tumours, 81% had good facial nerve function at 1 year (House-Brackmann grade 1 and grade 2), 16% had moderate function (grade 3 and grade 4) and 3% had poor function (grade 5). Of patients with large tumours, 22% had good facial function (grade 1 and grade 2), 37% had moderate function (grade 3 and grade 4) and 41% had poor function (grade 5 and grade 6). Useful postoperative hearing was preserved in 21% of the 47 patients with tumours <3 cm and useful preoperative hearing. In the last 5 years the authors have been operating in conjunction with an ear, nose and throat surgeon (PAB) trained in base-of-skull surgery. Over this period, useful hearing was preserved in 32% of patients with small and medium-sized tumours and useful preoperative hearing. CONCLUSIONS: Tumour size was an important predictor of the postoperative facial and cochlear nerve function. The multidisciplinary approach to these tumours offers better results. These results compare well with other published series.

Adolescent↗
PubMed2002

Acoustic trauma induces reemergence of the growth- and plasticity-associated protein GAP-43 in the rat auditory brainstem.

We explored the consequences of unilateral acoustic trauma to intracochlear and central nervous system structures in rats. An acoustic trauma, induced by applying click stimuli of 130 dB (sound pressure level; SPL) for 30 minutes, resulted in an instant and permanent threshold shift of 95.92 +/- 1.08 dB (SEM) in the affected ear. We observed, as a consequence, a structural deterioration of the organ of Corti. Deprivation-dependent changes of neurons of the auditory brainstem were determined using antibodies against neurofilament and the growth-associated protein GAP-43 and compared with those following cochleotomy, studied earlier. By 231 days posttrauma, spiral ganglion cell bodies and their processes were almost entirely lost from all cochlear regions with destroyed organ of Corti. In the lateral superior olive (LSO) ipsilateral to the trauma, cell bodies of lateral olivocochlear neurons turned transiently GAP-43 positive within the first 1.5 years posttrauma. The time course of emergence and disappearance of this population of neurons was similar to that found after cochleotomy. Additionally, after noise trauma, principal cells in contralateral LSO and in medial superior olive (MSO) on both sides of the brainstem developed an expression of GAP-43 that began 3 and 16 days posttrauma, respectively, and lasted for at least 1 year. Such cells were rarely observed after cochleotomy. An unequivocal rise in GAP-43 immunoreactivity was also found in the neuropil of the inferior colliculus and the ventral cochlear nucleus, both preferentially on the acoustically damaged side. We conclude that the degree and specific cause of sudden unilateral deafness entail specific patterns of plasticity responses in the auditory brainstem, possibly to prevent the neural network dedicated to locate sounds in the environment from delivering erroneous signals centralward.

Acoustic Stimulation↗
PubMed1998

Differential expression of the metabotropic glutamate receptor mGluR1alpha by neurons and axons in the cochlear nucleus: in situ hybridization and immunohistochemistry.

mGluR1alpha is a metabotropic glutamate receptor involved in synaptic modifiability. A differential expression in specific neuronal types could reflect their different connections and response properties in central auditory processing. Using in situ hybridization and immunohistochemistry, we studied mGluR1alpha receptor expression throughout the cochlear nucleus. Robust labeling occurred in the dorsal cochlear nucleus and small cell shell, with less in the ventral cochlear nucleus. Among the most intensely labeled were the granule cells of the small cell shell. In the dorsal cochlear nucleus, most cell types expressed message and receptor protein, except granule cells. High levels of receptor were expressed by corn cells and cartwheel cells. The terminal dendrites and synaptic spines of cartwheel and fusiform cells contained receptor protein in the molecular layer, where they could synapse with parallel fibers. Fusiform dendrites also expressed mRNA for mGluR1alpha. The basal dendrites of fusiform cells contained receptor protein in the region where they receive cochlear nerve synapses. Immunostaining of terminal axons was prominent in the molecular layer and the small cell shell, where they were associated with synaptic nests, structures thought to provide long-term changes in excitability. Differential expression levels may reflect different functional requirements of specific cell types, including inhibitory interneurons, like corn cells and cartwheel cells, and excitatory interneurons, like granule cells in the small cell shell, which may participate in local circuits involved in modulatory or gating functions, such as stimulus enhancement or suppression. In presynaptic axons, mGluR1alpha may relate to the long-term signaling requirements of their modulatory functions.

Animals↗
PubMed2001

Anomalous facial nerve canal with cochlear malformations.

BACKGROUND AND PURPOSE: Anteromedial "migration" of the first segment of the facial nerve canal has been previously identified in a patient with a non-Mondini-type cochlear malformation. In this study, several patients with the same facial nerve canal anomaly were reviewed to assess for the association and type of cochlear malformation. METHODS: CT scans of the temporal bone of 15 patients with anteromedial migration of the first segment of the facial nerve canal were collected from routine departmental examinations. In seven patients, the anomalous course was bilateral, for a total of 22 cases. The migration was graded relative to normal as either mild/moderate or pronounced. The cochlea in each of these cases was examined for the presence and size of the basilar, second, and apical turns. The turns were either absent, small, normal, or enlarged. The CT scans of five patients with eight Mondini malformations were examined for comparison. RESULTS: The degree of the facial nerve migration was pronounced in nine cases and mild/moderate in 13. All 22 of these cases had associated cochlear abnormalities of the non-Mondini variety. These included common cavity anomalies with lack of definition between the cochlea and vestibule (five cases), cochleae with enlarged basilar turns and absent second or third turns (five cases), and cochleae with small or normal basilar turns with small or absent second or third turns (12 cases). None of the patients with Mondini-type cochlear malformations had anteromedial migration of the facial nerve canal. CONCLUSION: Anteromedial migration of the facial nerve canal occurs in association with some cochlear malformations. It did not occur in association with the Mondini malformations. A cochlea with a Mondini malformation, being similar in size to a normal cochlea, may physically prohibit such a deviation in course.

Abnormalities, Multiple↗
PubMed2006

Sensorineural hearing loss with brainstem auditory evoked responses changes in homozygote and heterozygote sickle cell patients in Guadeloupe (France).

This prospective study involved 79 homozygote and heterozygote sickle cell anaemia patients (16 to 50 years old) and a control group of 40 people.All patients underwent ENT, audiological and brainstem auditory evoked responses (BSER) examinations in order to evaluate the incidence of sensorineural hearing loss (SNHL), to identify the changes at the level of the cochlear nerve and the central pathways, and to determine the most vulnerable group, in order to intervene with early prevention and rehabilitation for this condition.A hearing loss of greater than 20 dB at two or more frequencies was found in 36 (45.57 per cent) sickle cell patients (19 (47.22 per cent) HbSC patients and 17 (43.59 per cent) HbSS patients) and three (7.5 per cent) members of the control group. Homozygote and heterozygote patients, as well as both sexes, were equally affected. Bilateral hearing loss occurred in 19 (52.78 per cent) patients, unilateral right-sided hearing loss in five (13.89 per cent) patients and unilateral left-sided hearing loss in 12 (33.33 per cent) patients. Brainstem auditory evoked potential demonstrated a prolonged I-V (III-V) interpeak latency in 13 (25.35 per cent) sickle cell patients (11 men (eight with HbSS) and two women). The hearing loss in HbSS patients was neural in nature and of earlier onset; the hearing loss in HbSC patients was usually cochlear in nature and of later onset. Despite high medical standards and 100 per cent social security cover, the high incidence of SNHL in our sickle cell affected patients (the majority with the Benin haplotype) was probably due to their specific haematological profile and to the original geographical distribution of the disease in the tropics. Our results highlight the necessity for early and regular hearing assessment of sickle cell patients, including BSER examination, especially in male patients with SNHL.

Adolescent↗
PubMed2001

Cochlear implantation in children with large vestibular aqueduct syndrome and a review of the syndrome.

OBJECTIVE: Children with Large Vestibular Aqueduct Syndrome (LVAS) frequently develop speech and language skills prior to deterioration of their hearing. Operations designed to halt the progression of hearing loss have largely failed so the question of Cochlear Implantation in these children has arisen. It had been suggested that there would be technical difficulties in implanting these patients and, therefore, there had been an initial reluctance to proceed to implantation. The aim of the present paper is to assess surgical and functional outcomes in implanted children with LVAS and review the related literature. MATERIAL AND METHODS: From the 170 children assessed by MRI in the Nottingham Paediatric Cochlear Implant Programme, seven (4%) were identified as having LVAS. Four of these children were implanted and had at least 12 months follow up. Two of the children are on the waiting list for implantation and one child was not implanted because of absence of the cochlear nerve. Operative findings, complications and outcome measures were recorded. The auditory skills of the children were assessed before implantation and 1 year following implantation. A literature search was done to identify other series with experience in implanting children with LVAS. RESULTS: Full insertion of the electrode array was achieved in all our cases. After cochleostomy two patients experienced a mild CSF leak that was easily controlled by the muscle graft. On the first day post-operation two patients were nauseous and one had an episode of vomiting, however, all were discharged within 24 h of surgery. Initial outcome measures at 12 months post-implantation were encouraging showing significant progress in children's auditory skills. CONCLUSIONS: The results of the present study and the review of the literature suggest that LVAS is not a contraindication to implantation as initial concerns about severe perilymph leaks and surgical complications have proved to be unfounded. The post-operative progress of these children in listening skills also suggest that these children are suitable for cochlear implantation

Auditory Threshold↗
PubMed2004

Cloning and developmental expression of Shaker potassium channels in the cochlea of the chicken.

Signal coding by the receptor and neuronal cells of the auditory system involves various ion channels that modulate a sound stimulus. The genes that encode a number of these ion channels and their accessory subunits are presently unknown for channels found in the sensory epithelium and cochlear nerve. Among these genes are those that encode delayed rectifier and transient type potassium channels found in both the sensory cells and the ganglion. Here, we report the cloning and developmental expression of Shaker family members that include cKv1.2, cKv1.3, cKv1.5, and the Shaker-related cGMP-gated potassium channel cKCNA10. Clones were obtained by screening a chicken embryonic cochlea cDNA library using, as a probe, a mixture of two DNA fragments of cKv1.2 and cKv1.3 obtained by the reverse transcription polymerase chain reaction (RT-PCR). Sequence analysis revealed chicken homologues of Kv1.2, Kv1.3, Kv1.5 and cGMP-gated potassium channels with a deduced amino acid homology of 96-98%, 82-84%, 67-71% and 67-79% to correspondent mammalian homologues. During development of chicken inner ear, RT-PCR studies show expression of cKv1.2, cKv1.3 and cKv1.5 as early as Embryonic Day (ED) 3, while cKCNA10 was detected at low levels beginning on ED6 and was highly expressed by ED9. Additionally, analysis of expression in different parts of the cochlea showed that these genes were co-expressed in different regions of the cochlea, including the cochlear ganglion, sensory epithelium, lagena, and tegmentum. This expression pattern suggests the potential for the formation of heteromeric channels from the corresponding alpha-subunits in these various tissues.

Aging↗
PubMed1978

The technical aspects of cochlear implantation.

The historical development of attempts to provide some hearing ability, in totally deaf patients, by electrical stimulation of the cochlear nerve is reviewed to the point at which single channel intracochlear electrodes were tried in patients. The results obtained with this type of prosthesis are described and discussed in terms of the possible physiological mechanisms involved. Results obtained from promontory stimulation are also presented as a means of patient selection and are compared with the results from intracochlear electrodes. Based on these findings, the approaches for the development of multichannel intracochlear electrodes and for a single channel electrode with speech processing are described. The problems involved with materials, coding, electrode isolation and transmission are considered and the current results in these areas are detailed. Such results imply that the long-term prospects of developing a prosthesis that could provide adequate speech intelligibility are good.

Animals↗
PubMed1986

Pathogenesis of labyrinthitis associated with Haemophilus influenzae type b meningitis in infant rats.

The pathogenesis of labyrinthitis associated with bacterial meningitis was studied by histopathologic examination of inner ears of 114 rats with Haemophilus influenzae type b meningitis produced by ip inoculation of one of 13 clinical isolates. Findings consisted of inflammation of the perilymphatic spaces of the cochlea and semicircular canals with sparing of the endolymphatic space, cochlear nerve fibers, and middle ear. The degree of inflammation peaked at 48 hr after inoculation, then declined by 96 to 144 hr. No interstrain differences in type or degree of pathology were observed. Immunofluorescent staining of cochleae from 15 animals demonstrated that bacteria were present in areas of inflammation and also in the endolymphatic space and organ of Corti. One isolate displayed a tendency to accumulate in the perilymphatic spaces in larger numbers than those seen with three other isolates. These findings suggest that, in this model, inflammation reaches the inner ear by spreading from the subarachnoid space. Bacterial invasion of the organ of Corti may be one mechanism by which deafness occurs in bacterial meningitis.

Animals↗
PubMed2002

Simultaneous measurement of glutamate and dopamine release from isolated guinea pig cochlea.

Glutamate is proved to be a neurotransmitter in the mammalian cochlea, transmitting signals between the inner hair cells and the afferent cochlear nerve terminals. The transmission in this synapse is modulated by the lateral olivocochlear efferent fibers by releasing dopamine and other neurotransmitters. This study undertakes to measure simultaneously the release of dopamine and glutamate from isolated guinea pig cochleae. We combined the in vitro microvolume superfusion method, that uses liquid scintillation analysis, to measure [3H]dopamine with high pressure liquid chromatography (HPLC) to determine the glutamate content of the superfusate at rest and during stimulation. The release of both neurotransmitters was significantly increased when electrical field stimulation was applied at a 10 Hz rate. The nonselective sodium-channel inhibitor tetrodotoxin (TTX) at 1 microM completely blocked the effect of stimulation, indicating the neural origin of both dopamine and glutamate. The dopamine receptor antagonist sulpiride at 100 microM and the dopamine receptor agonist bromocriptine at 20 microM did not change the release of glutamate. In contrast, both bromocriptine and sulpiride significantly increased the stimulation-evoked release of dopamine. The effect of sulpiride is most likely due to the blockade of dopamine autoreceptor. Possible explanations why bromocriptine increased the release include: (1) its partional agonist activity; (2) desensitizations of dopamine autoreceptors; or (3) the higher D1 receptor activity of bromocriptine than sulpiride. This study could provide further insights about the role of dopamine and glutamate in cochlear neurotransmission.

Animals↗
PubMed2002

Impact of dental devices on cochlear implants.

A cochlear implant (CI) converts mechanical sound energy into electrical signals that can be delivered to the cochlear nerve of profoundly deaf patients. The purpose of this study was to investigate whether electromagnetic interference with the CI occurs during the operation of the electric pulp tester, apex locator, electrocautery unit, electrosurgery unit, or panoramic radiograph machine. A mastoidectomy and cochleostomy were performed on a cadaver, and a CI was implanted. The dental devices were used intraorally, and the implant's circuitry was tested after each trial. A second CI was implanted in a human skull, which was then exposed to 50 panoramic radiographs, testing the implant's circuitry after each exposure. The probability of damage to the CI by any of the devices was negligible, except for the electrosurgery unit operated at level 7, which destroyed the CI's circuitry. Therefore, although the other devices seem safe, it is recommended that the electrosurgery unit not be used on a CI patient.

Cadaver↗
PubMed1998

Vestibular evoked myogenic potentials in patients with acoustic neuromas.

BACKGROUND: To diagnose acoustic neuromas (ANs), the auditory brainstem response test and the caloric test have been used in addition to magnetic resonance imaging. The auditory brainstem response and the caloric tests mainly reflect functions of the auditory pathway, ie, the cochlear nerve and the superior vestibular nerve, respectively. Because the vestibular evoked myogenic potential (VEMP) has been thought to originate in the inferior vestibular nerve, we hypothesized that the VEMP could provide different information from the auditory brainstem response and the caloric test and that it could be helpful in diagnosing ANs. In other words, we hypothesized that the VEMP could provide information concerning inferior vestibular nerve involvement in patients with ANs. OBJECTIVE: To find out if the VEMP could be useful in classifying ANs according to the involved nerves. DESIGN: We reviewed preoperative clinical tests, including VEMPs, in 21 patients (8 men, 13 women) with ANs confirmed surgically and histopathologically, comparing them with VEMPs in 8 normal subjects (5 men, 3 women). RESULTS: Whereas the first positive-negative peak of the VEMP, P13-N23, was ipsilaterally present on stimulation of the unaffected side in all patients with ANs and both sides in all normal subjects, it was absent on the affected side in 15 patients (71%) and significantly decreased in amplitude in 2 patients (9%). Thus, 17 (80%) of the 21 patients showed abnormal VEMPs. Three patients had abnormal VEMPs although they had normal caloric responses. Three patients had abnormal caloric responses although they had normal VEMPs. CONCLUSION: These results suggest that the VEMP could be useful for the diagnosis of AN, especially for classifying ANs according to the involved nerves.

Adult↗