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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↗

Temporal bone imaging for cochlear implantation.

Computerized tomography (CT) imaging of the inner ear structures is helpful when evaluating a patient for cochlear implantation. The accuracy of CT scan imaging in determining the presence of cochlear ossification was assessed. A retrospective study of 104 implant patient charts, operative reports, and CT scans was performed. The CT scan was in agreement with the operative findings in 78% of patients included in the study. Twenty-two percent were found to have ossification at surgery which was not detected radiographically. One patient with extensive otospongiosis and an obstructed cochlea on CT scan had a patent cochlea demonstrated by magnetic resonance imaging (MRI) which was corroborated at surgery. In 32 children with postmeningitic hearing loss, the CT scan had only a 53% accuracy in assessing cochlear ossification. Since 69% of these patients were found to have some degree of cochlear ossification, otologists should expect to encounter some degree of bony obstruction within the basal turn of the cochlea even when the CT scan is normal. Nevertheless, cochlear implantation can successfully be performed in these cases.

Adolescent↗

Orientation for cochlear implant surgery in cases with round window obstruction: a computer reconstruction study.

In order to improve cochlear implant surgery in patients with obstructed round windows, surgical orientations of the round window and scala tympani relative to the stapes footplate were examined in ten normal temporal bones using a computer-aided, three-dimensional reconstruction and measurement technique. The round window was found to be exactly inferior to the midpoint of the inferior margin of the stapes footplate in most cases. An optimal point on the promontory wall for drilling to reach the bottom of the scala tympani of the basal turn was found to lie approximately 1.5 mm anterolateral or anterolateral inferior to a point 3 mm inferior to the midpoint of the inferior margin of the stapes footplate. A combination of the transmeatal and facial recess approaches made it possible to consistently reach the scala tympani, and demonstrated that this approach was also applicable to patients with obstructed round windows.

Adolescent↗

Holmium: YAG laser surgery in obliterated cochleas: an experimental study in human cadaver temporal bones.

A pulsed holmium:YAG laser (lambda = 2120 nm) was used to reopen the basal turn of artificially obliterated human cochleas in freshly dissected cadavers. This allowed intracochlear insertion of the stimulation electrode of a cochlear implant under simulated surgical conditions. Laser energy was transmitted through a 400-microns nylon fiber via the opened facial recess directly to the round window niche. At an energy level of 500 mJ per 2.5 microseconds pulse, a repetition rate of 2 Hz, and an exposure time of 20-30 s, the photo-ablative mechanism of laser-bone interaction led to a 8-10 mm recanalization of the basal turn of the cochlea without damaging surrounding structures. The artificial bony occlusion and the recanalized basal turn of the cochlea were visualized by means of computed tomography and studied under light microscopy, using a histologic thin-section technique.

Aluminum Silicates↗

Spiral ligament pathology: a major aspect of age-related cochlear degeneration in C57BL/6 mice.

Data from systematic, light microscopic examination of cochlear histopathology in an age-graded series of C57BL/6 mice (1.5-15 months) were compared with threshold elevations (measured by auditory brain stem response) to elucidate the functionally important structural changes underlying age-related hearing loss in this inbred strain. In addition to quantifying the degree and extent of hair cell and neuronal loss, all structures of the cochlear duct were qualitatively evaluated and any degenerative changes were quantified. Hair cell and neuronal loss patterns suggested two degenerative processes. In the basal half of the cochlea, inner and outer hair cell loss proceeded from base to apex with increasing age, and loss of cochlear neurons was consistent with degeneration occurring secondary to inner hair cell loss. In the apical half of the cochlea with advancing age, there was selective loss of outer hair cells which increased from the middle to the extreme apex. A similar gradient of ganglion cell loss was noted, characterized by widespread somatic aggregation and demyelination. In addition to these changes in hair cells and their innervation, there was widespread degeneration of fibrocytes in the spiral ligament, especially among the type IV cell class. The cell loss in the ligament preceded the loss of hair cells and/or neurons in both space and time suggesting that fibrocyte pathology may be a primary cause of the hearing loss and ultimate sensory cell degeneration in this mouse strain.

Aging↗

The effect of nimodipine on cochlear potentials and Na+/K(+)-ATPase activity in normal and hydropic cochleas of the albino guinea pig.

In experimental endolymphatic hydrops (EEH) a decrease in the endocochlear potential (EP) has been reported and is thought to be due to decreased activity of the enzyme Na+/K(+)-ATPase in the stria vascularis. By stimulating Na+/K(+)-ATPase, the EP, and thereby cochlear function as a whole, might be restored. On the other hand, stimulation of stria vascularis Na+/K(+)-ATPase might result in excessive production of endolymph and thus produce or augment hydrops. In this study we have investigated the effect of intraperitoneally applied nimodipine on cochlear potentials and on Na+/K(+)-ATPase activity in the stria vascularis, both in normal cochleas (control) and in cochleas with EEH. Nimodipine is an L-type Ca(2+)-channel blocking agent with Na+/K(+)-ATPase stimulating properties at concentrations as low as 1.5 nM. The compound action potential (CAP), evoked by 2,4 and 8 kHz tone bursts was found to be depressed in the EEH ears with and without nimodipine treatment, and in the nimodipine treated control ears. Statistical analysis (ANOVA) showed that the effects of EEH and nimodipine on the CAP were additive. The negative summating potential (SP), measured extracochlearly at the apex, in response to 4 and 8 kHz tone bursts was significantly enhanced in the EEH ears. Nimodipine treatment did not affect the SP, neither in the control, nor in the EEH ears. Cytochemically, Na+/K(+)-ATPase activity appeared to be decreased in the oedematous stria vascularis of hydropic cochleas. No effect of nimodipine on Na+/K(+)-ATPase activity could be established ultracytochemically, neither in the controls nor in the EEH ears. In the lower turns of some of the nimodipine treated control cochleas a mild hydrops was seen during light-microscopic evaluation. Although it was not possible to prove a stimulatory effect of nimodipine on the enzyme Na+/K(+)-ATPase cytochemically, the finding of mild endolymphatic hydrops in nimodipine treated control ears suggests (a history of) increased endolymph production. This hydrops might be responsible for the depression of the CAP in the nimodipine treated ears.

Animals↗

Contributions of age, cochlear integrity, and auditory environment to avian cochlear nucleus metabolism.

Most commercially raised broiler chickens display progressive cochlear degeneration with age [Hear. Res. 166 (2002) 82]. Recent work examining the effects of age and cochlear degeneration on avian cochlear nucleus (nucleus magnocellularis, NM) metabolism showed that changes in metabolic activity occur with age and cochlear damage [Hear. Res. 175 (2003) 101]. The auditory environment also differed between facilities housing young and adult birds. The relative contributions of age, cochlear degeneration, and auditory environment to these changes in NM metabolism are unknown. Using cytochrome oxidase (CO) histochemistry, NM neuron metabolism is examined in several age groups of birds under varying conditions. When normal cochlear integrity and auditory environment are held constant, CO staining is significantly decreased in adult vs. young birds. When age and auditory environment are held constant, CO staining is significantly decreased in birds with damaged vs. normal cochleae. When age and normal cochlear integrity are held constant, CO staining is significantly decreased in birds living in a quiet vs. noisy environment. All factors examined cause changes in CO staining, which is indicative of NM metabolic activity. Results are discussed in the context of mitochondrial aging, afferent regulation, and auditory deprivation and enrichment.

Aging↗

Simultaneous measurement of electrocochleography and cochlear blood flow during cochlear hypoxia in rabbits.

In this study, a new monitoring system is developed to measure cochlear blood flow (CBF) and electrocochleography (ECochG) during transient ischemic episodes of the cochlea. A newly designed otic probe was used for the simultaneous recordings of laser-Doppler CBF and ECochG directly from the round window (RW). The probe enabled the recording of high amplitude compound action potentials (CAP) and cochlear microphonics (CM) with few averages. Experiments were conducted on rabbits to generate episodes of cochlear ischemia by using timed compressions of the internal auditory artery (IAA). The computer monitoring system extracted and measured CAP and CM components from ECochG in real-time. Results indicate that CM and CAP generally followed CBF during compressions and releases of IAA. Both CBF values and CAP amplitudes showed an overshoot following the reperfusion. CAP amplitude measures were found to be very sensitive to ischemia showing very rapid amplitude, latency and morphological changes. CM amplitude decreased more slowly than the CAP and CBF. Simultaneous recordings of CBF and ECochG using the otic probe provide a valuable neuromonitoring tool to investigate the dynamic behavior of the cochlea during ischemia.

Acoustic Stimulation↗

Mouse homolog of KIAA0143 protein: hearing deficit induces specific changes of expression in auditory brainstem neurons.

Hearing deficit induced by mechanical cochlear damage, intense noise or ototoxic drugs produces a variety of structural and functional changes in the inner ear and the auditory brainstem. In the present study, we identified a novel gene that has activity dependent plasticity in the superior olivary complex by using suppression subtractive hybridization. We cloned a gene that encodes mouse homolog of KIAA0143 protein, one derived from a series of unidentified human genes. This gene termed mKIAA0143 shows differential expression of mRNA in the lateral superior olive between mice with hearing deficit and those with normal hearing ability. The mRNA thus obtained encodes a unique membrane-bound protein that consists of 819 amino acids. The gene locus was mapped using genomic DNA databases to the mouse chromosome 15D1. Green fluorescent protein-tagged mKIAA0143 was expressed in COS-1 cells. It was amply seen in the cellular plasma membrane.

Acoustic Stimulation↗

Changes in programming over time in postmeningitis cochlear implant users.

OBJECTIVE: Although successful cochlear implantation of patients with deafness following meningitis is expected, long-term stability of electrical current requirements has not been systematically evaluated. This study evaluated changes in programming for patients deafened by bacterial meningitis and stability of auditory performance over time. STUDY DESIGN AND SETTING: In this retrospective descriptive study, cochlear implant (CI) stimulation mode and performance of 14 patients deafened by meningitis were compared with those of an age-matched control group of patients deafened by other causes. RESULTS: There were no significant differences in mean performance between the meningitis group and control group (P > 0.05). However, the postmeningitis group required progressively higher stimulation levels and higher programming modes over time as compared to the control group. CONCLUSIONS: Even with deafness accompanied by labyrinthine ossification attributed to meningitis, neural elements were present and could be stimulated. Because increasing levels of stimulation were required over time, postmeningitic children with CIs, and those with cochlear ossification in particular, may need frequent programming adjustments to maintain performance. SIGNIFICANCE: These patients need close follow-up of stimulation levels and programming modes postoperatively in order to perform optimally with CIs. EBM RATING: B-3.

Adolescent↗

Otoneurologic evaluation of child vertigo.

In this study 282 children with vertigo are subdivided (according to previous experiences) into three large groups: (1) vertigo and cochlear diseases; (2) vertigo as an isolated symptom; and (3) vertigo and C.S.N. diseases. Due to the difficult etiopathogenetic investigation of the patients from the second group, the authors focused on that group as they are less studied, are without associated symptoms (deafness--first group; CNS diseases--second group) and where vertigo appears as an idiopathic and an isolated symptom. A careful anamnestic, clinical and instrumental analysis leads to the following observations: (1) in decreasing order of frequency we find the third group, followed by the first and finally by the second; (2) in spite of the overall lower incidence of the second group, this latter includes the paroxismal benign vertigo (PBV) which is overall the second most frequent vertiginous form (after vertigo due to cranial trauma). In this group the authors underline the reasonably high incidence of the iatrogenic syndromes, insisting on the need of their accurate prevention of these risks; (3) the authors confirm that, nowadays, a reliable etiopathogenetic cause of the apparently isolated vertigo (except for the ascertained iatrogenic forms) cannot be identified. Moreover, in spite of its frequency, PBV is the less known form of vertigo, of which we cannot give a certain diagnosis and which can be identified only the the exclusion of all the other known forms through instrumental and clinical observations.

Acoustic Impedance Tests↗

Cochlear implants in children: what constitutes a complication?

The surgeon must ultimately accept the responsibility for any complications that occur as the result of a cochlear implant. Listening to the cochlear implant team members and responding to their needs may enhance the child's progress. Surgical complications, (i.e., skinflap problems, infection, and facial paralysis) are indeed infrequent, but nonsurgical problems are not. Surgical and nonsurgical experiences were reviewed in 55 children. Ages ranged from 23 months to 18 years at the time of cochlear implantation, which occurred from 1984 to 1995. There were no surgical complications. However, the most common surgical obstacle was ossification, which was present in 40% and undetected by computed tomographic scanning in 16.3% of children. Ossification occurred at the round window and scala tympani in 32.7% and involved the cochlea more extensively in 7.3% of children. In only one child was the cochlea entirely ossified. There were, however, many nonsurgical problems that were viewed as complications in patient management. The single most important complication was device failure. This occurred in 10.9% (5/46) of children with the Cochlear Corporation multichannel implant. Head banging and other temper tantrums, parental interference with rehabilitation, socioeconomic factors, poor compliance by the family unit, equipment problems, educational deficiencies, and impatience with habilitative training were some of the other problems.

Adolescent↗

Surgical techniques for cochlear implantation in the very young child.

Early cochlear implantation to treat prelingually deafened children has been shown to improve speech perception and overall performance. The current age limit for implantation is 24 months in accordance with US Food and Drug Administration guidelines, but it is believed that earlier implantation is possible and may result in better performance. Implantation in children younger than 36 months, however, is complicated by the altered anatomy of the temporal bone in this young age group. We have developed specific modifications in the cochlear implantation technique for this young age group. This technique was used in implantation for 17 children younger than 36 months. The ages ranged from 16 to 36 months and averaged 30 months. All patients except one had complete electrode insertion without complication. The technique of cochlear implantation must be modified not only for differences in anatomy in these young children but also for the expected continued growth of the temporal bone and related structures. Cochlear implantation can be safely performed on children as young as 16 months.

Age Factors↗

Systematic approach to electrode insertion in the ossified cochlea.

Ossification of the fluid spaces of the cochlea occurs often in candidates for cochlear implantation, especially children. When noted before surgery on computerized tomography, ossification previously was thought to contraindicate cochlear implantation because of possible mechanical obstruction and uncertainty about the level of function that could be achieved by stimulating an ossified cochlea. However, during the preceding 6 years, techniques have been developed that permit implantation in ossified cochleas. We present a systematic approach that has been developed to treat the three clinically important categories of cochlear ossification: round window niche obliteration, inferior segment obstruction, and upper segment obstruction. Case reports are presented for each of these three drill-out procedures, demonstrating results often similar to those expected for implantation of the nonossified cochlea.

Adult↗

Accuracy of high-resolution computed tomography in cochlear implantation.

Multichannel cochlear implants are a proven method for the auditory rehabilitation of individuals who have severe-to-profound sensorineural hearing loss. These devices typically require insertion into the scala tympani of the cochlea to provide auditory stimulations. A patent scala provides the best chance for an adequate insertion of the electrode array. Preoperative high-resolution computed tomography imaging has traditionally been used to determine the patency of the scala tympani. Its ability to accurately predict the patency of the cochlea has been questioned in several retrospective studies. A prospective study was undertaken in 28 consecutive individuals undergoing cochlear implant surgery to compare the findings on high-resolution computed tomography with the surgical findings in an attempt to determine high-resolution computed tomography's accuracy. Cochlear obstruction caused by ossification was accurately predicted in six of six individuals but overestimated in the round window region in three individuals. High-resolution computed tomography accurately predicted patent cochleas in 19 individuals. No false-negative results were encountered. In this study sensitivity of high-resolution computed tomography was 100%, and specificity was 86%. High-resolution computed tomography appears to be more helpful than previously reported for determining cochlear patency.

Acoustic Stimulation↗

Methotrexate management of immune-mediated cochleovestibular disorders.

Immune-mediated cochleovestibular disorders continue to present a management challenge to the otolaryngologist. The traditional treatment of these disorders, corticosteroids and/or cyclophosphamide (Cytoxan), has been associated with serious and occasionally life-threatening complications. In this study we report our experience in treating 25 patients with immune-mediated cochleovestibular disorders with methotrexate, a less toxic immunosuppressive agent that has been used extensively in patients with rheumatoid arthritis. Mean duration of treatment was 12.9 months, and adverse reactions were acceptable and reversible. Hearing improved in 69.6% of patients, and vestibular symptoms subsided or improved in 80% of patients. The results of this study suggest that methotrexate treatment is effective in a substantial number of patients with immune-mediated cochleovestibular disorders and has acceptable adverse reactions. A prospective, randomized study is needed to compare the efficacy of methotrexate with that of other immunosuppressive agents.

Adult↗

Hydrops in the cochlea can be induced by sound as well as by static pressure.

The Reissner's membrane (RM) was visualised by confocal microscopy in the isolated temporal bone of the guinea pig. The function of the organ was followed by measuring its physiological response. Static pressure applied in the basal coil caused a distention of the RM in the apical coil into the scala vestibuli. The sensitivity to a test tone was reduced. When the pressure was relieved, the RM returned to its original position and the response recovered. If the increased pressure was maintained, the RM would bulge further. The RM could then be reversibly stretched and return gradually, with a delay, to its original position. Alternatively, it could be over-stretched and return with an over-shoot past its original position toward the organ of Corti. In response to repetitive tone pulses of above 80 dB, hydrops of the RM also developed. This was accompanied by a reduced sensitivity. A slow recovery to the original position, or over-shoot, and return of responsiveness could be seen. Above 106 dB sustained loss was generally seen. It is concluded that the RM can accommodate increased scala media pressure by distention. This will relieve the organ of Corti from part of the pressure and may protect the organ from trauma.

Animals↗

Carboplatin-induced early cochlear lesion in chinchillas.

Carboplatin preferentially damages inner hair cells (IHC) and type I spiral ganglion neurons (SGNs) in the chinchilla; however, the temporal sequence of events leading to the destruction of these structures is poorly understood. To better understand the mechanisms leading up to the destruction of IHCs and type I SGNs, we measured the activity in single auditory nerve fibers for the first 8 h following carboplatin treatment and also monitored the development of histopathologies in SGNs and IHCs using a dose of carboplatin that killed approximately 50% of the IHCs. The spontaneous discharge rate (SDR) showed a slight increase around 3 h post carboplatin followed by a significant decline at 4-5 h. The saturation driven discharge rate (DDR) showed a significant increase 1-5 h post carboplatin. These physiological changes were associated with the formation of small vacuoles in type I afferent terminals and proximal nerve fibers 1-6 h post carboplatin; signs of IHC damage were first observed around 24-48 h. Thus, the neurotoxic effects of carboplatin occur approximately a day before the IHCs are damaged. The large fluctuations in SDR and DDR that occur several hours after carboplatin treatment are most likely due to the neurotoxic effects of carboplatin.

Animals↗