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Effects of chronic cochlear damage on threshold and frequency tuning of neurons in AI auditory cortex.

We describe the effects of long-term cochlear lesions on the frequency response properties of AI cortical neurons in the cat. Young animals were treated with amikacin to produce bilateral, basal to mid-turn cochlear lesions. After 12-24 months the response properties of single neurons or small unit clusters in primary auditory cortex were recorded in anesthetized animals. Responses to stimulus frequency and intensity were mapped in detail and frequency threshold curves (FTCs) and Q10dB values were derived. Subsequent to recording experiments, scanning electron microscopy of the sensory epithelium was used to characterize the degree and extent of the cochlear damage. In normal control animals, Q10dB values were, on average, lower than those derived by others from cochlear nerve fibre recordings in the same species. In amikacin-treated animals, deterioration was evident in the threshold and tuning properties of cortical neurons, particularly in those cells whose input originated in damaged cochlear regions. Often, neurons associated with 'normal' cochlear areas (as assessed by scanning microscopy) also had poor frequency tuning compared with controls. As an animal model of sensorineural hearing loss, we consider the cat with long-term cochlear lesions to be more appropriate than animals with acute or short-term pathology. We also suggest that in making physiological-psychophysical correlations, neural responses from the central auditory system (e.g. cortex) should perhaps be given more consideration than data derived at the cochlear level.

Amikacin↗

An active cochlear model showing sharp tuning and high sensitivity.

Recent in vivo measurements of cochlear-partition motion indicate very high sensitivity and sharp mechanical tuning similar to the tuning of single cochlear nerve fibers. Our experience with mathematical models of the cochlea leads us to believe that this type of mechanical response requires the presence of active elements in the cochlea. We have developed an active cochlear model which incorporates negative damping components; this model produces partition displacement in good agreement with many of the mechanical and neural tuning characteristics which have been observed in vivo by other researchers. We suggest that the negative damping components of our model may represent an active mechanical behavior of the outer hair cells, functioning in the electromechanical environment of the normal cochlea.

Animals↗

Inner ear anomalies in two cases of trisomy 18.

Two cases of trisomy 18 in which temporal bone defects were limited to the inner ears are described. Several abnormalities were present that have not been described previously in this syndrome. The first case involved a 1-month-old female infant who died of congenital heart defects. Cochlear nerve fibers were absent on the left side, with near-normal innervation on the right. Although the organ of Corti was present bilaterally, some of the outer hair cells were deformed, having small rounded cell bodies unsupported by Deiters' cells. On the right, similar abnormal cells were found in the tunnel of Corti. Vestibular defects in this case included reduced nerve supply of the left saccular macula, cysts in the superior and posterior cristae, and absence of the utriculoendolymphatic valve. The second case involved a newborn male infant with multiple congenital anomalies. The major cochlear defect was a deformity of the stria vascularis. In the lower apical turn, the stria was adherent to Reissner's membrane and extended beneath it into scala media. Large capillaries, which ran freely suspended in scala vestibuli, entered the upper portion of the stria. Severe atresia of the lateral and posterior semicircular ducts was found in the vestibular apparatus.

Chromosomes, Human, 16-18↗

Auditory brainstem implants: surgical aspects.

Patients with neurofibromatosis type 2 often develop bilateral life-threatening vestibular schwannoma necessitating tumor removal, which results in deafness. We developed the auditory brainstem implant (ABI) in order to be able to electrically stimulate the cochlear nucleus complex in patients with bilateral cochlear nerve injury from bilateral schwannoma. After tumor removal, the electrode array of the ABI is inserted into the lateral recess of the fourth ventricle and placed over the surface of the ventral and dorsal cochlear nuclei. The ABI is designed to stimulate auditory neural structures within the cochlear nucleus in order to convey salient cues about the frequency, amplitude, and temporal characteristics of sounds. To date, more than 200 patients have received an ABI device at our institution. Recently, penetrating ABIs were introduced, and preliminary results of penetrating ABIs are discussed in this paper. The surgical anatomy of the nucleus and surgical placement of the ABI in patients with neurofibromatosis type 2 are described, and surgical considerations in this group of challenging patients are detailed.

Auditory Brain Stem Implants↗

Prediction of the nerves of origin of vestibular schwannomas with vestibular evoked myogenic potentials.

OBJECTIVE: To determine whether the nerves of origin of vestibular schwannomas can be predicted using vestibular evoked myogenic potentials (VEMPs). STUDY DESIGN: The study was a retrospective analysis. SETTING: The ear, nose, and throat department of Tokyo Medical and Dental University. PATIENTS: Twenty-eight patients undergoing removal of vestibular schwannomas were included in the study. INTERVENTIONS: Patients underwent pure tone audiometry, VEMP testing, caloric testing, and magnetic resonance imaging preoperatively. Hearing level, caloric weakness, maximum tumor size, and the nerves of origin of tumors were compared with VEMP testing. MAIN OUTCOME MEASURE: Results of VEMP testing. RESULTS: Comparisons between VEMPs and results of the other three examinations revealed no correlations. Complete disappearance of VEMPs was observed only in patients with tumors arising from inferior vestibular nerves. Patients in whom hearing was preserved tended to have preserved VEMPs. Some patients showed damaged hearing and normal VEMP results, although with inferior vestibular schwannomas. A patient with a tumor arising from a cochlear nerve exhibited preservation of VEMP, preserved caloric response, and moderate hearing loss. CONCLUSIONS: Inferior vestibular nerve function and hearing level were reflected in VEMP results. Prediction of the nerve of origin of a tumor was possible only in certain restricted cases.

Adult↗

Nonlinear mechanics at the apex of the guinea-pig cochlea.

A heterodyne laser interferometer was used to observe the sound-evoked displacement patterns of Reissner's membrane and various other structures in the apical turn of the guinea-pig cochlea. Most structures (including the basilar membrane) were similarly tuned, and had best frequencies in the 200-350Hz range. A distinct notch was usually observed approximately 0.7 octaves above the best frequency, and amplitude- and phase-plateaus were observed at higher frequencies. In most other respects, however, the mechanical tuning resembled the frequency-threshold curves of low frequency cochlear nerve fibers. In five reasonably intact, in vivo preparations, the frequency of the mechanical sensitivity notch was intensity-dependent: Compressive nonlinearities were observed above approximately 80 dB SPL on the low-frequency side of the notch, with antagonistically expansive nonlinearities on the high-frequency side. Two-tone suppression was observed in one of these preparations. Stimulus-related baseline position shifts were observed in another in vivo preparation. No such nonlinearities were observed in structurally damaged and/or > 1 hour post-mortem preparations. However, more robust nonlinearities were observed in all preparations at higher levels of stimulation (e.g. > 100-110 dB SPL). These high-level nonlinearities diminished only slowly after death, and gave rise to various effects, including time-dependent (i.e. adapting) and severely distorted (e.g. peak-split and/or dc-shifted) responses.

Acoustic Stimulation↗

Cochlear implantation in a patient with profound hearing loss with the A1555G mitochondrial mutation.

OBJECTIVE: This study aimed to describe the performance of a cochlear implant in a patient with profound hearing loss with the A1555G mitochondrial mutation. SETTING: The study was conducted at two university hospitals. PATIENT: A 50-year-old Japanese man in whom bilateral profound hearing loss developed after administration of streptomycin at the age of 23 participated. The pedigree of the family showed exclusively maternal transmission of hearing impairment. INTERVENTION: Genetic study and auditory rehabilitation with a cochlear implant were performed. RESULTS: The A1555G point mutation was identified from the patient's mitochondrial DNA. Since activation of the implant, the patient has been using it successfully with a monosyllable recognition score of 78% using Japanese word lists for speech audiometry. CONCLUSIONS: The current case indicated that cochlear implantation may be a valuable choice of therapy for the patient with profound hearing loss with the A1555G mutation. The excellent auditory performance with a cochlear implant suggests that hearing loss associated with this mutation is primarily caused by insult to the cochlear tissue containing rich mitochondria (i.e., hair cells or stria vascularis or both), not to the cochlear nerve and its central connections.

Antibiotics, Antitubercular↗

Postsynaptic targets of type II auditory nerve fibers in the cochlear nucleus.

Type II auditory nerve fibers, which provide the primary afferent innervation of outer hair cells of the cochlea, project thin fibers centrally and form synapses in the cochlear nucleus. We investigated the postsynaptic targets of these synapses, which are unknown. Using serial-section electron microscopy of fibers labeled with horseradish peroxidase, we examined the border of the granule-cell lamina in mice, an area of type II termination that receives branches having swellings with complex shapes. About 70% of the swellings examined with the electron microscope formed morphological synapses, which is a much higher value than found in previous studies of type II swellings in other parts of the cochlear nucleus. The high percentage of synapses enabled a number of postsynaptic targets to be identified. Most of the targets were small dendrites. Two of these dendrites were traced to their somata of origin, which were cochlear-nucleus "small cells" situated at the border of the granule-cell lamina. These cells did not appear to receive any terminals containing synaptic vesicles that were large and round, indicating a lack of input from type I auditory nerve fibers. Nor did type II swellings or targets participate in the synaptic glomeruli formed by mossy terminals and the dendrites of granule cells. Other type II synapses were axosomatic and their targets were large cells, which were presumed multipolar cells and one cell with characteristics of a globular bushy cell. These large cells almost certainly receive additional input from type I auditory nerve fibers, which provide the afferent innervation of the cochlear inner hair cells. A few type II postsynaptic targets-the two small cells as well as a large dendrite-received synapses that had accompanying postsynaptic bodies, a likely marker for synapses of medial olivocochlear branches. These targets thus probably receive convergent input from type II fibers and medial olivocochlear branches. The diverse nature of the type II targets and the examples of segregated convergence of other inputs illustrates the synaptic complexity of type II input to the cochlear nucleus.

Animals↗

The mechanism of speech perception in patients with a multichannel cochlear implant.

The mechanism of speech perception in patients with a multichannel cochlear implant was investigated by administering vowel, consonant, and speech recognition tests, and comparing sound spectrograms with the activities recorded from the cochlear implant electrodes. Vowel recognition was found to be adequate, but consonant recognition was poor. Although the cochlear hair cells of such patients are known to be functionally damaged, the cochlear nerve was found to have the capacity to characterize frequency. The signals from the implanted device were very simple compared with those from the normal cochlea; however, the patients could nevertheless understand speech well after cochlear implantation.

Adult↗

[Cerebellopontine angle surgery. Part 2: Specific remarks].

OBJECTIVES: Cerebellopontine angle tumors are uncommon lesions that can potentially be cured by microsurgical removal. The primary objective of the surgical treatment differs between vestibular schwannoma and meningioma. This feature may be influenced by the site of tumor origin and displacement of neurovascular structures as well as by their different tumor biology. METHODS: A review of the current literature was conducted. RESULTS AND CONCLUSIONS: Relevant cranial nerves and vascular involvement as well as anatomical location with respect to the cerebellopontine angle are discussed for vestibular schwannoma and meningioma. The main factors influencing the surgical outcome are outlined with special reference to facial and cochlear nerve function and cerebrospinal fluid leakage. The retrosigmoid approach offers a comparable success rate for hearing conservation and probably a superior outcome in terms of facial nerve function when compared with the middle fossa approach. The intrameatal limitations of the retrosigmoid approach can be excluded by the intraoperative assistance of an endoscope. The advantages of endoscope-assisted surgery may include improved visualization of relevant structures, more complete tumor removal, and a lowered risk of cerebrospinal fluid leakage.

Cerebellar Neoplasms↗

[Effect of white noise on activity and gene expression of nitric oxide synthase in cochlear nuclei of guinea pigs].

NADPH-diaphorase (NADPH-d) histochemistry method and semiquantitative polymerase chain reaction (PCR) technology were adopted to study nitric oxide synthase (NOS) neurons and to investigate the content change of NOS mRNA and the relationship between it and auditory threshold and to explore the possible role of NOS neurons in cochlear nuclei of guinea pigs after exposed to white noise. The results showed that the quantity and staining intensity of positive NOS neurons in cochlear nuclei increased obviously after exposed to noise. It reached the peak 2 week later and remained 5 week than normal. The regulation of NOS mRNA was consistent with that of morphological observation. NOS mRNA content in 2 w group was the highest and was 4.02 times higher than normal. After exposed to noise, ABR threshold shift was in positive correlation with NOS mRNA content in cochlear nuclei (r = 0.9655, P < 0.01). It is suggested that positive NOS neurons in cochlear nuclei might be involved in controlling injury and repair of cochlear nerves. The high expression of NOS gene might be an important factor in pathogenic mechanism of noise deafness.

Animals↗

Possible functional roles of Na+,K+-ATPase in the inner ear and their relevance to Ménière's disease.

This article reviews the functions of the enzyme Na+,K+-ATPase in epithelial tissues and discusses early and recent biochemical, physiologic and morphologic studies of the enzyme in the inner ear. The purpose of the investigation was to learn whether a relationship between perturbations in activity of the enzyme and Ménière's disease is possible. It is concluded that the preponderance of the evidence indicates that Na+,K+-ATPase plays a role in regulating ion transport into the scala media, but that the significance of the distribution of the enzyme along only one cell type (the marginal) in the functional chains of cells of the outer cochlear wall needs further study. The possible vasoconstrictive effects of ouabain perfusions employed by some investigators must also be taken into account. Recent cytochemical and autoradiographic studies have demonstrated high levels of Na+, K+-ATPase on cochlear nerve fibers, especially near the foramina nervosa and within the organ of Corti. Thus, perturbations in Na+,K+-ATPase activity in the inner ear not only could affect certain aspects of fluid balance, but also could account for the sensory disturbances experienced by patients who have Ménière's disease.

Animals↗

Angiogenesis from the eighth cranial nerve to vestibular schwannomas.

Vascularization between vestibular schwannomas and the adjacent eighth cranial nerve was examined in order to elucidate the angiogenic effect of these tumours. Specimens from 12 patients were examined by light microscopy and immunohistochemistry (series 1), while specimens from another 17 patients were examined by light and transmission electron microscopy (series 2). Slight to marked angiogenesis was found in 5 patients from series 1 and 7 patients from series 2. Ultrastructural examination indicated that these blood vessels were more fragile than those in the normal eighth cranial nerve. These findings suggested that sudden hearing loss in vestibular schwannoma patients may be related to the disruption of such blood vessels. In addition, surgical injury to these blood vessels may cause postoperative hearing loss despite preservation of the cochlear nerve.

Adult↗

The Chorimac-12. A multichannel cochlear implant for total deafness. Description and clinical results.

More than 90% of cases of patients suffering from acquired or congenital deafness may be provided with some sound sensation owing to the electrical stimulation of the few cochlear nerve fibers that are still present in the deaf cochlea. In order to obtain some speech discrimination without lip reading it is necessary to elicite different frequency sound sensations. Thus electrically proofs compartments must be performed all along the cochlear keyboard. The placement of 12 intra-cochlear electrodes is greatly facilitated by using an electrodes-bearer which is introduced through a very simple and classical surgical procedure. The indications for implanting depend on undiscussible criteria (total deafness, auditory response to electrical stimulation of the round window, patient's motivation). Clinical results consist of psychoaffective improvement and auditory possibilities. All patients are able to discriminate homosyllabic words in closed lists without lips reading, but the percentages of these performances greatly vary from one patient to the other. Some patients, generally suffering from acquired total deafness, are able to discriminate words or sentences without reference list and without help of lips reading. These results depend on the patient's socialization level, on the pre or post-lingual status of the deafness, on an eventual cochlear ossification, and on the value of several pre operative electro physiological data. Associated handicaps (autism, blindness, tetraplegia) are not contra indications and often represent supplementary reasons for implanting. The value of these post-operative results may be predicted owing to a series of electrical pre-operative tests and clinical data. Thus it is possible to determine the respective indications of multi and single channel cochlear implants.

Adolescent↗

Why not the eighth nerve? Neurovascular compression--probable cause for pulsatile tinnitus.

Carotid arteriograms on three patients with unilateral pulsatile tinnitus demonstrated an ipsilateral atypical trigeminal artery extending from the cavernous portion of the internal carotid artery to form the posterior inferior cerebellar artery. Illustrations and a dissection of a human fetus with a similar finding show this artery crossing the cochlear nerve near its insertion in the pons. Evidence is presented suggesting that neurovascular compression of the eighth nerve is the source of pulsatile tinnitus in these patients.

Adult↗

Postnatal changes in cochlear polyamine metabolism in the rat.

Ornithine decarboxylase (ODC), the initial enzyme in the polyamine biosynthetic pathway, is increased in the developing rat cochlea, suggesting that polyamine biosynthesis is important in cochlear development. Although cochlear polyamines have been detected in adult rats, they have not been identified in developing rats. We quantified polyamines in the developing and mature rat cochlea and further characterized ODC in the early postnatal period. Putrescine and spermidine in combined tissues of the organ of Corti and lateral wall of the cochlea were highest during the first 10 postnatal days, then declined to adult levels shortly thereafter. Spermine demonstrated a similar developmental trend. A high spermidine to spermine ratio was noted during this period as was rapidly increasing ODC activity. A high spermidine/spermine ratio was also noted in the cochlear nerve of developing and mature rats, suggesting that spermidine may be necessary for function and maintenance of the nerve. This is the first report of polyamines in the developing rat cochlea. The period of increased polyamine synthesis coincides with the critical period for ototoxicity induced by alpha-difluoromethylornithine, a specific ODC inhibitor, and the period of rapid cochlear development.

Analysis of Variance↗

[Electrophysiological identification of the cochlear and vestibular nerves in the cerebellopontine angle: experimental study and clinical implication].

Electrical stimulation was applied on the surface of the eighth cranial nerve in the cerebellopontine angle of dogs and the evoked potentials were recorded with surface electrodes on the scalp. The shape of the evoked potentials recorded was different according to the portion of the nerve stimulated electrically. It was, therefore, possible to precisely identify the nerve stimulated from the surface recorded evoked potentials. This electrophysiological method may be helpful for the surgeon to precisely locate the cochlear and vestibular nerves in various operations in the cerebellopontine angle in human. Our preliminary experience of this monitoring method in acoustic neuroma excision was presented.

Animals↗

[Correlation of changes in compound action potential (CAP) tuning curves and cochlear lesion in guinea pigs after explosion].

The purpose of the present study was to investigate the sensitivity of compound action potential (CAP) tuning curves to changes of the cochlear status in guinea pigs after explosion and their ability to reflect specific histological variations. The results were as follows: 1. The CAP tuning curves were abnormally broad and the Q 10 dB values were reduced by a factor of 1 after explosion, indicating wider tuning. 2. The degree of broadening of the CAP tuning curves seemed to increase as the hair cell loss increased. 3. After explosion, the tip of the tuning curve shifted to frequencies significantly higher or lower than that of the signal, it might be related to the location of hair cell loss in the cochlea. 4. In animals for which damage was restricted to only three rows of outer hair cells, changes of the CAP tuning curves were observed. It provides further evidence that the tuning properties of cochlear nerve fibers are dependent upon the integrity of the outer hair cells even though the great majority of fibers innervate inner hair cells only.

Animals↗