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PubMedDate not supplied

Recent advances in cochlear blood flow measurements.

Changes in blood flow to the inner ear have been thought to influence or underlie a number of cochlear diseases, including some forms of noise-induced hearing loss, sudden hearing loss, and Meniere's disease. Recently, important advances have been made in two technologies for the study of cochlear blood flow. The first is in the area of vital microscopic studies of cochlear microcirculation, and the second is based on the introduction of laser technology in the form of laser Doppler flowmetry. In this report, measurements are given of changes in cochlear circulation caused by carbon dioxide breathing, intravenous phenylephrine injection, systemic hemodilution, positive end expiratory pressure, and direct electrical stimulation of the cochlea. From these changes, we observe that cochlear blood circulation responds to systemic blood pressure alterations and is subject to local flow control mechanisms. Linearity and speed of response of the laser Doppler instrumentation also are shown. These advances show promise for contributing to our knowledge of control mechanisms of inner ear blood flow and for revealing the influence of various pharmacologic agents of potential clinical value.

Animals↗
PubMed1999

Cochlear implantation in auditory neuropathy.

OBJECTIVE: Auditory neuropathy is a recently described clinical entity characterized by sensorineural hearing loss in which the auditory evoked potential (ABR) is absent but otoacoustic emissions are present. This suggests a central locus for the associated hearing loss. In this study the results observed in a child with auditory neuropathy who received a cochlear implant are presented and compared with those of a matched group of children who were recipients of implants. METHODS: A single-subject, repeated-measures design, evaluating closed-set and open-set word recognition abilities was used to assess the subject and a control group of matched children with implants who had also experienced a progressive sensorineural hearing loss. RESULTS: The subject demonstrated improvements in vowel recognition (82% correct) by 1 year after implantation, which were only slightly lower than the control group. Consonant recognition and open-set word recognition scores were significantly lower. CONCLUSION: Caution should be exercised when considering cochlear implantation in children with auditory neuropathy. As with conventional hearing aids, less than optimal results may be seen.

Audiometry, Pure-Tone↗
PubMed1994

Cochlear implant-related osteoneogenesis in an animal model: fluorescent labeling.

Cochlear osteoneogenesis may result from a variety of pathologic conditions, including cochlear implantation. The etiology of cochlear osteoneogenesis following implantation, however, is not known. Cochlear implant-related osteoneogenesis has been demonstrated in laboratory animals, but the specific cause, extent, or time-course of this process has not been determined. In this preliminary study, fluorescent bone labels were used to assess osteoneogenesis in six chinchillas in three experimental categories: surgical trauma to the cochlea, chronic nonstimulated cochlear implantation, and intrascalar neomycin infusion. Computer image analysis was used to measure the area of labeled bone on representative mid-modiolar histologic sections. The amount of bone deposition was greatest in ears treated with intracochlear neomycin (mean = 2.3835 mm2, SD = 3.7308). Surgical trauma alone (mean = 0.9549 mm2, SD = 1.384) and chronic implantation without stimulation did not produce substantial bone growth when compared to contralateral control ears (mean = 0.0574 mm2, SD = 0.0731). Fluorochrome labeling was also used to differentiate types of bone deposition. The morphology and timing of new bone growth appeared to be related to the type of cochlear injury. These results confirm that intracochlear neomycin may contribute to osteoneogenesis in animal studies of cochlear implantation. This study supports the use of fluorescent bone labeling in the evaluation of cochlear osteoneogenesis.

Animals↗
PubMed1997

Viral-mediated gene transfer in the cochlea.

Gene transfer is an exciting new tool in medical therapy and scientific investigation, but only very recently has it begun to be developed in the auditory system. This paper describes in vivo and ex vivo experiments using an adenoviral vector (Ad. RSVntlacZ), which is a replication-deficient virus based on a human adenoviral (serotype 5) genomic backbone. The in vivo experiments demonstrate successful gene transfer into multiple types of cochlear cells. We observed a relatively efficient transduction, several weeks of sustained transgene expression and an absence of major lethal cytotoxicity in spiral ganglion and epithelial cells of the cochlea in adult animals. The ex vivo experiments were performed using fibroblasts transduced in vitro with Ad. RSVntlacZ. Two weeks after inoculation of the fibroblasts into the perilymph, we observed transplanted fibroblasts, which were adherent to the lining of the perilymphatic spaces, and were expressing the lacZ transgene. We speculate that, as the genetic basis of degenerative cochlear diseases is characterized on a mutational level, transgene expression will allow us to test hypotheses regarding the effects of specific genes on cochlear cell biology. Gene transfer will not only increase our understanding of the pathophysiology of hearing loss, but also may provide gene therapy for disease.

Adenoviridae↗
PubMed1992

[Preoperative vestibular diagnosis in therapy of Menière's disease].

Surgical treatment of Meniere's disease is indicated in patients with severe attacks who have failed drug therapy. A decision between a conservative or destructive procedure demands firstly exclusion of patients with non-otogenic diseases, bilateral disease or complete unilateral loss of function, and secondly assessment of the laterality of the disease, of residual labyrinthic function and of vestibular compensation. A total of 185 patients with Meniere's disease were investigated. All of them had a history and clinical signs that indicated surgery. Canal function, otolith responses and vestibular compensation were examined, in addition to routine preoperative tests as recommended by the Committee on Hearing and Equilibrium of the American Academy of Ophthalmology and Otolaryngology. Of the 185 patients 22% had findings that contraindicated surgery, 4% suffered from Meniere's disease in the opposite ear, 6% showed no signs of vestibular compensation and 12% had dead labyrinths on the side of the cochlear disease. No caloric response was elicited from the labyrinth by thermal bilateral testing in 25% of the 185 patients. In patients who were thought to have dead labyrinths based on routine caloric tests, residual canal function was detected by means of 20 degrees C stimulation in the prone and supine position. Without extensive vestibular testing the indications for surgical treatment of Meniere's disease will be incorrect in 47% of patients.

Functional Laterality↗
PubMed1988

Retrograde cochlear neuronal degeneration in human subjects.

The purpose of this study was to identify the structural changes in the organ of Corti that correlate with retrograde cochlear neuronal degeneration. Thirty-eight temporal bones with excellent histological preparation from 23 subjects having hearing losses caused by cochlear disease were selected for study. Cytohistograms were prepared for inner and outer hair cells, inner and outer pillar cells, inner phalangeal cells, and cochlear neurons. The extent of neuronal degeneration was found to be directly related to the extent of injury to inner pillar cells and inner phalangeal cells, but not to loss of inner or outer hair cells. In most cochleas the loss of dendritic nerve fibers exceeded the loss of cell bodies. The findings support the concept that retrograde neuronal degeneration is initiated by injury to the dendritic nerve fibers, secondary to collapse and/or degeneration of the inner pillar cells and inner phalangeal cells.

Adult↗
PubMed1991

Hearing loss and pneumococcal meningitis: an animal model.

Clinical studies of predisposing factors in the development of hearing loss secondary to bacterial meningitis have produced conflicting results. An animal model of meningogenic labyrinthitis was developed for more precise study of these parameters. Rabbits were inoculated intrathecally with 10(5) pneumococci to induce meningitis. Hearing thresholds were measured using auditory-evoked responses to 1 kHz, 10 kHz, and click stimuli before infection and every 12 hours thereafter. Profound deafness occurred in all subjects at an average of 48 hours following infection. The incidence and severity of hearing loss was strongly correlated with the duration of meningitis. Temporal bone histology revealed acute inflammation of all perilymphatic spaces including the cochlear aqueduct. This model demonstrated that the risk and severity of hearing loss increase with the duration of meningitis and suggested that the cochlear aqueduct is an anatomic pathway for the extension of infection from the cerebrospinal fluid to the cochlea. The implications for therapy in humans is discussed.

Acoustic Stimulation↗
PubMed1995

Modulation of cochlear responses in the guinea pig by low-frequency, phase-shifted maskers following noise trauma.

Low-frequency acoustic biasing using an intensive phase-shifted, low-frequency masker was studied according to its ability to determine disorders of cochlear micromechanics following noise trauma in the guinea pig as animal model. Statistical analyses proved that this technique allowed electrophysiological differentiation of controls versus groups with different degrees of experimentally induced threshold shifts. To substantiate group differences an intensity of at least 70 dB SPL was required for the 52 Hz masker and the difference in relation to the test-tone intensity had to be +/- 10 or +/- 20 dB SPL. The noise-traumatized cochlea could be identified by means of a threshold shift for the 5 microV pseudothreshold, a low modulation span of the compound action potential amplitude (< 25-50 microV frequency dependent), and reduced positive summating potential amplitude with negative non-modulating values within the different measurement phases for 1 and 2 kHz stimulation.

Acoustic Stimulation↗
PubMed2001

Inner ear lesion alters acoustically induced c-Fos expression in the rat auditory rhomboencephalic brainstem.

The pattern of c-Fos expression was mapped in the adult rat's brain following unilateral cochlear lesions. In normal and cochlear lesioned rats, c-Fos expression was induced with sound stimuli. Acoustic stimulation consisted of pulses of four tones. An additional control group consisted of non-stimulated rats. In the cochlear nuclei (CN), c-Fos activation was scarce in isolated rats and increased strongly following sound stimulation. Following unilateral cochlear lesion, acoustically driven expression was decreased in all CN in both the lesioned and the untreated sides. The ventromedial periolivary nucleus and the rostral periolivary nucleus showed c-Fos activation in isolated conditions and were strongly activated following sound stimulation. The rest of the superior olivary complex showed no c-Fos activation in isolated rats and a weak activation following sound stimulation. Following unilateral cochlear lesions, acoustically driven expression was decreased in some, but not all superior olivary nuclei in both the lesioned and the untreated sides. In the lateral lemniscus complex, c-Fos activation was scarce in isolated rats and increased strongly after stimulation. Following unilateral cochlear lesion, acoustically driven expression decreased bilaterally in all nuclei. We have found that unilateral inner ear lesions lead to bilateral impairment of the capability of acoustic pathway neurons, to being c-Fos-activated following sound stimulation.

Acoustic Stimulation↗
PubMedDate not supplied

[Experimental perilymphatic fistula: the electrocochleographic findings].

The aim of the present study was to evaluate the effect of experimental perilymphatic fistula on cochlear electrophysiological responses. Six albino guinea pigs, in which recording electrodes were implanted in order to record the electrocochleogram, were used. Experimental protocol investigated cochlear responses before the induction of the fistula in the round window membrane perforation and then two month later. Compound action potential thresholds increased immediately after perforation of the round window membrane. The thresholds rapidly recovered. This phenomenon was accompanied by clear-Out reduction in amplitude upon suprathreshold stimulation. This latter observation associated with a decrease in the amplitude of the threshold seems to be a specific audiologic sign of the fistula. The clinical implication are discussed.

Acoustic Stimulation↗
PubMed1987

Effect of galactose and isosorbide on cochlear blood flow.

Cochlear blood flow in the rabbit was determined before and after injection of hyperosmotic solutions of galactose and isosorbide. After doses similar to those which can produce hearing improvements in patients with Ménière's disease, cochlear blood flow was increased by 30-60%.

Animals↗
PubMed1979

Histopathology of 30 non-operated acoustic schwannomas.

Thirty of 1720 temporal bones from the Wittmaack's Collection contain nonoperated acoustic neuromas, 22 of them large tumors. Histologically, each tumor has to be considered as an individual. Most of the tumors show a mixture of Antoni type A and B. Tumor vascularization is pronounced in half the cases. Vessels of the internal acoustic meatus are found in all cases. Protein contents of the perilymphatic spaces is medium to pronounced in 23 of 30 cases in comparison to the small protein contents of the healthy side. Ganglion geniculi is invaded in 11 cases (9 von Recklinghausen's disease), Ganglion Scarpae in 26 cases (9 von Recklinghausen's disease), and Ganglion spirale in 9 cases (7 von Recklinghausen's disease). Cochlear and vestibular nerve fibers within the internal auditory meatus were affected by the tumor in 28 of 30 cases, the facial nerve only in 11 cases, among them 9 cases of von Recklinghausen's disease. Twenty-six of 30 schwannomas have a portion within the cribriform area of the cochlea fundus, which explains the limitation to radical tumor surgery without damage of the cochlear nerve. These histological findings explain the site of damage of hearing to expand between the cochlea to the auditory brain stem nuclei, and support the audiological experience that a correlation of "acoustic neuroma" and "retrocochlear lesion" is often not correct.

Adolescent↗
PubMed1997

Cochlear microphonics in Ménière's disease.

The pathophysiology of hearing deterioration in Ménière's disease (MD) is unclear. Hair cell loss has been proposed to be the cause of severe hearing loss in Ménière's disease. The cochlear microphonic (CM) is known to be the receptor potential of the outer hair cells in the cochlea. This study measured the CM in Ménière's disease and investigated its relationship with the degree of hearing impairment and endolymphatic hydrops. Transtympanic electrocochleography (ECoG) using rarefaction (RAR) and condensation (CON) tonebursts at 1 kHz was performed on 130 ears of 119 patients. Ninety six ears were diagnosed to have MD and 34 were diagnosed non-Ménière's disease (NMD). The mean amplitude of the CM was 33.10 +/- 46.04 microV in the MD group and 13.15 +/- 12.77 microV in the NMD group (p < 0.001). Enlarged negative summating potential and action potential ratios (SP:AP > 40%) were found in 81.3% of the MD group and 17.6% of the NMD group. In the MD group, the CM in the group with an enlarged negative SP was 36.98 +/- 49.78 microV, and 16.31 +/- 15.88 microV in the group without (p < 0.01). The CM was 34.33 +/- 49.28 microV in the pure-tone average (PTA) < or = 25 dB group, 46.97 +/- 58.31 microV in the 26-40 dB group, 29.12 +/- 42.62 microV in the 41-70 dB group, and 26.20 +/- 22.41 microV in the > 70 dB group (p > 0.05). The CMs in 11 pairs of MD ears and sensorineural hearing loss (SNHL) ears with matching hearing (MD 44 dB, SNHL 45 dB) were measured. They were 71.42 +/- 75.94 microV and 7.90 +/- 5.89 microV, respectively (p < 0.01). Our study shows that the CM is higher in ears with endolymphatic hydrops, evidenced by an enlarged SP:AP ratio, than ears without and the CM shows no statistical difference in groups with different levels of hearing loss. These findings suggest that hearing loss with a large CM in Ménière's disease patients may be the result of an alteration of cochlear mechanics and only severe hearing loss with a small CM is caused by hair cell loss. The CM measurement, to evaluate the hair cell status, may be helpful in identifying patients whose hearing may be recoverable if the underlying hydrops can be corrected. Our data do not permit the conclusion that an enlarged CM can be used in the diagnosis of MD.

Adult↗
PubMed1990

Labyrinthine ossification after meningitis: its implications for cochlear implantation.

Labyrinthine ossification can be found in a high percentage of patients with profound deafness resulting from bacterial meningitis. Radiographic evidence of ossification can be found as early as 2 months after the acute infection, indicating that the intracochlear process probably begins much earlier. If long, intracochlear cochlear implants are to be most successfully used in these patients, an aggressive approach to clinical management following the meningitis should be taken. Illustrative case reports and suggested guidelines for evaluation and treatment are given.

Calcinosis↗
PubMed1990

Imaging for cochlear implants.

Insertion of a sound amplification device into the round window niche (extracochlear implant) or into the coils of the cochlea (intracochlear implant) can give significant benefits to some carefully selected, severely deaf patients. Imaging has an essential role in selective and pre-operative assessment. Severe otosclerosis and post-meningitic labyrinthitis ossificans are common causes of deafness in these patients and can be demonstrated by computed tomography (CT). The most suitable side for operation can be assessed. We describe our experiences with 165 patients, 69 of whom were found suitable for implants. Thin (1 mm) section CT in axial and coronal planes is the best imaging investigation of the petrous temporal bones but the place of magnetic resonance scanning to confirm that the inner ear is fluid-filled and polytomography to show a multichannel implant in the cochlea is discussed. No implants were used for congenital deformities, but some observations are made of this type of structural deformity of the inner ear.

Cochlea↗