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PubMed1991

Ultrastructure of the guinea pig cochlear aqueduct. An electron microscopic study of decalcified temporal bones.

The ultrastructure of the guinea pig cochlear aqueduct was examined using semi-thin and thin sections. The lumen of the cochlear aqueduct was occupied by a sparse meshwork of fibroblasts and delicate connective tissue trabeculae. The periotic tissue lining the bony wall of the aqueduct was composed of multiple layers of both elongated cells and densely arranged laminae of collagen fibrils. These structures were identical to those of the dura mater and the arachnoid. The opening to the perilymphatic space of the scala tympani also contained connective tissue trabeculae, but the arrangement of fibroblasts was more compact here than in the main part of the duct. These structural features suggest that fluid can move freely through cochlear aqueduct, and that the effects of sudden pressure changes in the CSF may be protected against by the densely and perpendicularly arranged fibroblast at the opening to the perilymphatic space.

Animals↗
PubMed1988

Early effects of gentamicin on inner ear glycocalyx cytochemistry.

The early effects of gentamicin treatment on the inner ear glycocalyx were investigated using two cationic probes, colloidal thorium and cationized ferritin. Gentamicin treatment resulted in a diminished thorium reactivity of both the endolymphatic and perilymphatic glycocalyx of the hair cells after 1 day and complete abolishment of reactivity after 5 days. Cationized ferritin reactivity of the perilymphatic and endolymphatic glycocalyx was not significantly influenced. The cytochemistry of the inner ear glycocalyx and the possible biochemical changes induced by gentamicin are discussed.

Animals↗
PubMedDate not supplied

Transmission of cerebrospinal fluid pressure via the cochlear aqueduct and endolymphatic sac.

The concept of perilymphatic and endolymphatic pressure balance is generally linked to the theory that the endolymphatic sac transmits cerebrospinal fluid (CSF) pressure changes to the endolymph to equalize CSF pressure changes transmitted to the perilymph via the cochlear aqueduct. This theory, and the significance of other mechanisms of CSF pressure influence on the labyrinth, were evaluated experimentally. Continuous measurements of perilymphatic, CSF, venous, and arterial pressures were performed on cats with the cochlear aqueduct patent or obstructed and the inferior cochlear vein intact or occluded. Intracranial pressure changes were induced by subarachnoid infusion of artificial CSF in live and dead animals. With the cochlear aqueduct patent, CSF pressure changes were transmitted to the perilymph without any significant dampening or time lag. With the cochlear aqueduct obstructed, CSF pressure changes induced significantly lower and delayed changes in perilymphatic pressure. Similar results were obtained whether the animals were alive or dead and the cochlear vein intact or blocked. This indicated a passive mechanism not induced by changes in labyrinthine fluid production or blood flow. Long-standing, stable elevation of CSF pressure with the cochlear aqueduct blocked induced a slowly increasing perilymphatic pressure, always stabilizing at a pressure rise significantly less than that of CSF. The results do not suggest any major pressure transfer via perineural or perivascular routes. The endolymphatic sac is postulated to mediate a reduced and delayed transfer of increased intracranial pressure to the labyrinth.

Animals↗
PubMed1984

Radiographic classification of the vestibular and cochlear aqueducts: the paired correlation between normal and abnormal vestibular aqueduct and cochlear aqueduct anatomy.

Multidirectional tomography (MDT) can be useful in determining the caliber, shape, and course of the vestibular aqueduct (VA) and cochlear aqueduct (CA). Clinical decisions have been based on the findings from MDT. Unfortunately, the clinical utility of these observations has been confusing and controversial because similar MDT techniques were not used. This study will address some of the difficult questions and clinical controversies derived from MDT observations. This new perspective has evolved with the use of high resolution computed tomography (HRCT). An analysis of 750 petrous bones for the occurrence of the various types of VAs and CAs using Gado's classification, further vestibular aqueduct and a variation of Gado's classification for the cochlear classification is reported. The distribution of the possible paired types of VA and CA are evaluated. MDT results indicate that the paired analysis in patients with inner ear dysfunction is not useful, cost effective, diagnostic, or of prognostic value. MDT can provide clinically valid observations of periaqueductal and perilabyrinthine pneumatization which is helpful in anticipating the size and position of the endolymphatic sac at the time of surgery for those few patients who may benefit from endolymphatic system surgery. However, when a comparison is made between MDT and CT of 60 ears in those same patients, the clinical limitations of MDT for inner ear diagnosis and prognosis became apparent. The future for HRCT scanning with reformatting holds potential for clinically meaningful visualization of inner and middle ear structures previously expected from MDT imaging.

Cochlea↗
PubMed1993

Surgical anatomy for infralabyrinthine approach to the internal auditory canal.

Surgical anatomy for infralabyrinthine approach to the internal auditory canal was evaluated using human temporal bone in order to understand advantages and disadvantages of this approach. The procedure is extracranial without any damage of labyrinth. Lateral exposure of the internal auditory canal allows for easy separation of the cochlear and vestibular nerves. There are some potential problems of little importance stemming from anatomical variations, such as high jugular bulb, forward positioned sigmoid sinus and poor pneumatization of the infralabyrinthine space. This approach offers access to the eighth cranial nerve with minimal risk and morbidity.

Cochlear Nerve↗
PubMed1984

Distribution of primary vestibular fibers in the brainstem and cerebellum of the monkey.

Attempts were made to determine the central projections of ganglion cells innervating individual semicircular ducts in the monkey by implanting or injecting tritiated amino acids (leucine and/or proline), or horseradish peroxidase (HRP), selectively into a single ampulla. Central transport via the vestibular ganglion in animals receiving isotope implants or injections fell into three categories: (1) transport from ganglion cells innervating all receptive elements of the labyrinth, (2) transport from ganglion cells innervating the three semicircular ducts, and (3) transport from cells of the inferior vestibular ganglion innervating the posterior semicircular duct. Transneuronal transport of isotope was observed in secondary vestibular fibers in animals where proline was used and survival exceeded 12 days. Transneuronal labeling of secondary auditory fibers was independent on the [3H]amino acid used, and occurred with survivals of 10 or more days. HRP implanted into the ampulla of the lateral semicircular duct in several animals produced retrograde transport to efferent vestibular and cochlear neurons, but did not result in transganglionic labeling of primary vestibular or auditory fibers. Primary vestibular fibers terminate throughout the superior (SVN) and medial vestibular nuclei (MVN). Within SVN, terminals are most pronounced in its central large-celled portion, but extend into peripheral parts of the nucleus, except for a small medial area near its junction with the oral pole of MVN. Primary projections to MVN are homogenously distributed throughout the nucleus excepting a small circular area of sparse terminals along its ventral margin. Primary vestibular afferents terminate mainly in rostral and caudal portions of the inferior vestibular nucleus (IVN), but do not reach cell group 'f'. Projections to the lateral vestibular nucleus (LVN) are restricted to its ventral part. Primary projections to the accessory vestibular nuclei reach the interstitial nucleus of the vestibular nerve (NIVN) and cell group 'y'. Fibers project beyond the vestibular nuclei (VN) to terminate ipsilaterally in the accessory cuneate nucleus (ACN), the subtrigeminal lateral reticular nucleus (SLRN), and well-defined portions of the reticular formation (RF). Projections to SVN and MVN are derived primarily from ganglion cells innervating the semicircular ducts, while projections to caudal IVN, cell group 'y' and ACN are related mainly to macular portions of the vestibular ganglion. NIVN receives both macular and duct afferents.(ABSTRACT TRUNCATED AT 400 WORDS)

Afferent Pathways↗
PubMed1999

Dlx5 regulates regional development of the branchial arches and sensory capsules.

We report the generation and analysis of mice homozygous for a targeted deletion of the Dlx5 homeobox gene. Dlx5 mutant mice have multiple defects in craniofacial structures, including their ears, noses, mandibles and calvaria, and die shortly after birth. A subset (28%) exhibit exencephaly. Ectodermal expression of Dlx5 is required for the development of olfactory and otic placode-derived epithelia and surrounding capsules. The nasal capsules are hypoplastic (e.g. lacking turbinates) and, in most cases, the right side is more severely affected than the left. Dorsal otic vesicle derivatives (e. g. semicircular canals and endolymphatic duct) and the surrounding capsule, are more severely affected than ventral (cochlear) structures. Dlx5 is also required in mandibular arch ectomesenchyme, as the proximal mandibular arch skeleton is dysmorphic. Dlx5 may control craniofacial development in part through the regulation of the goosecoid homeobox gene. goosecoid expression is greatly reduced in Dlx5 mutants, and both goosecoid and Dlx5 mutants share a number of similar craniofacial malformations. Dlx5 may perform a general role in skeletal differentiation, as exemplified by hypomineralization within the calvaria. The distinct focal defects within the branchial arches of the Dlx1, Dlx2 and Dlx5 mutants, along with the nested expression of their RNAs, support a model in which these genes have both redundant and unique functions in the regulation of regional patterning of the craniofacial ectomesenchyme.

Animals↗
PubMed1991

Effect on cochlear potentials of lateral semicircular canal destruction.

Recording of the cochlear potentials was successfully performed during experimental labyrinthectomy in the guinea pig and in three patients with acoustic neuromas during translabyrinthine removal of the tumors. In the guinea pig, complete interruption of the duct of the lateral semicircular canal including the endolymphatic canal caused little change in the endocochlear DC potential of the first cochlear turn and input-output function curve of the N1 component of the compound action potential elicited by 8-kHz tone bursts. Further drilling of the vestibular labyrinth in the guinea pig caused decline of these potentials when the vestibular was opened. In patients with acoustic neuromas, the interruption of the duct of the lateral semicircular canal hardly altered the N1 input-output function curve and N1 input-latency function curve during the 1-hour observation period. Consistent preservation of cochlear function even after interruption of lateral semicircular canals suggests the possibility of partial surgical labryrinthectomy with preservation of hearing for lesions involving semicircular canals.

Acoustic Stimulation↗
PubMed1990

Prenatal development of the cochlear nerve in the albino rat.

Twenty-eight prenatal (14 to 20 days) Wistar albino rats were studied with Cajal-de Castro's silver reduced stain. The histological sections were oriented in a parasagittal oblique plane, parallel to the cochlear root, in order to observe the origin, course and destination of the cochlear fibres in as few consecutive sections as possible. In all the prenatal days studied, the fibres arising in the first half coil of the cochlea (hook's bundle), show a different orientation than those of the other cochlear nerve fibres, which constitute the spiralized bundle, originating in the basal, medial and apical portions of the cochlea. The hook-bundle does not participate in the curling of the cochlear nerve and it describes a particular course. At first, its fibres are situated in a lateral position in the nerve when it is inside the internal auditive duct. Then, the hook-fibres take a postero-inferior position just at the entry of the bulbar cochlear nuclei region. Furthermore, we have seen no hook's afferents branching in the ventral cochlear nucleus, and it could be suggested that these fibres project directly to the superficial dorso-medial area of the postero-ventral cochlear nucleus.

Animals↗
PubMed2006

The large vestibular aqueduct--case report and review of the literature.

Patients with a large vestibular aqueduct (LVA) suffer from a loss of hearing in childhood at an early onset. An acute loss of hearing can be precipitated by minor head trauma. Until now there seems to be no sufficient therapy for stopping the progression of a loss of hearing. It has been shown that a cochlear implantation is a worthwhile procedure if the patient is almost deaf. We report the case of a patient with a bilateral LVA. A loss of hearing was confirmed at the age of 16 months. Exposure to loud noise triggered an acute progression of the hearing loss. At the age of 18 years, LVA was confirmed radiologically, revealing an enlarged endolymphatic duct and sac in MRI scans and an enlarged vestibular aqueduct in the CT scan. We successfully performed a cochlear implant (MED-EL, Combi 40+ flex). Proceeding from this case report, the paper reviews the literature on LVA.

Adolescent↗
PubMed1988

Computer ranking of the sequence of appearance of 40 features of the brain and related structures in staged human embryos during the seventh week of development.

The sequence of events in the development of the brain in human embryos, already published for stages 8-17, is here continued for stages 18 and 19. With the aid of a computerized bubble-sort algorithm, 58 individual embryos were ranked in ascending order of the features present. The increasing structural complexity provided 40 new features in these two stages. The chief characteristics of stage 18 (approximately 44 postovulatory days) are rapidly growing basal nuclei; appearance of the extraventricular bulge of the cerebellum (flocculus), of the superior cerebellar peduncle, and of follicles in the epiphysis cerebri; and the presence of vomeronasal organ and ganglion, of the bucconasal membrane, and of isolated semicircular ducts. The main features of stage 19 (approximately 48 days) are the cochlear nuclei, the ganglion of the nervus terminalis, nuclei of the prosencephalic septum, the appearance of the subcommissural organ, the presence of villi in the choroid plexuses of the fourth and lateral ventricles, and the stria medullaris thalami.

Brain↗
PubMed2006

Temporal bone imaging in GJB2 deafness.

OBJECTIVE: To describe temporal bone findings on computed tomography (CT) imaging in GJB2-related hearing loss (HL). We asked whether evaluation of the temporal bone is required in individuals with biallelic GJB2 mutations. STUDY DESIGN: Randomized, blinded, controlled, prospective measurement. METHODS: Blood from 264 pediatric cochlear implant users was analyzed for mutations in the GJB2 gene. Thirty-six aspects of the temporal bone on CT imaging were evaluated in 53 individuals (106 ears) with biallelic disease causing GJB2 mutations. A subset of patients was age matched and compared with normally hearing individuals. Subjects with biallelic GJB2 mutations were tested for mutations in the SLC26A4 gene to rule out Pendred syndrome as a confounding cause of large vestibular aqueduct syndrome. RESULTS: Approximately 53% of ears of subjects (72% of subjects) with biallelic GJB2 mutations had at least one temporal bone anomaly. The most common findings were 1) dilated endolymphatic fossa (28%); 2) hypoplastic modiolus (25%); 3) large vestibular aqueduct (8%); 4) hypoplastic horizontal semicircular canal (8%); 5) hypoplastic cochlea (4%). Compared with normally hearing individuals, the GJB2 group had hypoplasia of the cochlear nerve canal, lateral semicircular canal vestibule, internal auditory canal (t tests, P < .001), and were 11 times more likely to have a hypoplastic modiolus. Dilated endolymphatic fossae were 1.4 times more common in the GJB2 group, and large vestibular aqueducts were 3 times more common in the GJB2 group, as compared with normally hearing controls. CONCLUSIONS: Temporal bone anomalies are common in GJB2-related HL, and imaging of the temporal bone should be included in routine evaluation of these individuals.

Child↗
PubMed2005

Suboccipital retrosigmoid approach for removal of vestibular schwannomas: facial nerve function and hearing preservation.

In this report, we discuss the pertinent bony, arachnoid, and neurovascular anatomy of vestibular schwannomas that has an impact on the surgical technique for removal of these tumors, with the goal of facial nerve and hearing preservation. The surgical technique is described in detail starting with anesthesia, positioning, and neurophysiological monitoring and continuing with the exposure, technical nuances of tumor removal, hemostasis, and closure. Positive prognostic factors for hearing preservation are also highlighted.

Anesthesia, General↗
PubMed2004

3-T imaging of the cochlear nerve and labyrinth in cochlear-implant candidates: 3D fast recovery fast spin-echo versus 3D constructive interference in the steady state techniques.

BACKGROUND AND PURPOSE: High-resolution imaging of the internal auditory canal and labyrinth at 1.5 T is often performed by using three-dimensional (3D) fast spin-echo or T2* techniques. We evaluated both techniques at 3 T in the preoperative assessment of patients being considered for cochlear implants. METHODS: Sagittal 3D fast recovery fast spin-echo (FRFSE) and 3D constructive interference in the steady state (CISS) images were acquired in eight patients at 3.0 T by using dual surface coils. Contrast-to-noise ratios (CNRs) for the intracanalicular nerve and CSF were measured in the internal auditory canal. Two neuroradiologists reviewed the images to determine whether the techniques provided images of diagnostic quality. RESULTS: CNRs for 3D CISS were twice those obtained with 3D FRFSE. Both techniques provided images of diagnostic quality, though spurious signal intensity loss at the apex of the superior semicircular canals was encountered on 3D FRFSE images in four of eight patients. CONCLUSION: Both 3D FRFSE and 3D CISS provide high-resolution images of the internal auditory canal and labyrinth at 3.0 T. We predict that the superior CNRs obtained with 3D CISS will prove advantageous as we move to smaller fields of view at higher field strength.

Artifacts↗
PubMed1990

Quantification of the relation between electrophysiologic and morphologic changes in experimental endolymphatic hydrops.

The degree of hydrops in guinea pig ears that had undergone unilateral endolymphatic sac and duct obliteration was quantified. For 15 guinea pigs from a prior study, the correlation between the degree of hydrops and several electrophysiologic measures of the functional state of the cochlea was determined. A significant correlation between the degree of hydrops and a reduction in the low-frequency cochlear microphonic evoked by a 29-Hz tone was found. No correlation was found between the degree of hydrops and the magnitude of the summating potential evoked by 8-kHz probes. A weak correlation was found between the degree of hydrops and a reduction in the compound action potential evoked by 2-kHz probes. Possible differences in the way the endolymphatic duct obliteration exerts its effect on the different cochlear potentials are discussed.

Animals↗
PubMedDate not supplied

Medical devices of the head, neck, and spine.

There are many medical devices used for head, neck, and spinal diseases and injuries, and new devices are constantly being introduced. Many of the newest devices are variations on a previous theme. Knowing the specific name of a device is not important. It is important to recognize the presence of a device and to have an understanding of its function as well as to be able to recognize the complications associated with its use. The article discusses the most common and important devices of the head, neck, and spine, including cerebrospinal fluid shunts and the Codman Hakim programmable valve; subdural drainage catheters, subdural electrodes, intracranial electrodes, deep brain stimulators, and cerebellar electrodes; coils, balloons, adhesives, particles, and aneurysm clips; radiation therapy catheters, intracranial balloons for drug installation, and carmustine wafers; hearing aids, cochlear implants, and ossicular reconstruction prostheses; orbital prostheses, intraocular silicone oil, and lacrimal duct stents; anterior and posterior cervical plates, posterior cervical spine wiring, odontoid fracture fixation devices, cervical collars and halo vests; thoracic and lumbar spine implants, anterior and posterior instrumentation for the thoracic and lumbar spine, vertebroplasty, and artificial disks; spinal column stimulators, bone stimulators, intrathecal drug delivery pumps, and sacral stimulators; dental and facial implant devices; gastric and tracheal tubes; vagus nerve stimulators; lumboperitoneal shunts; and temperature- and oxygen-sensing probes.

Adult↗
PubMed1990

Experimental endolymphatic hydrops: are cochlear and vestibular symptoms caused by increased endolymphatic pressure?

The correlation between inner ear pressure and cochlear function was investigated in guinea pigs with unilaterally obliterated endolymphatic sacs and ducts. In 11 animals that developed endolymphatic hydrops, auditory thresholds as monitored by auditory evoked action potentials had increased with recruitment. Most of these animals also experienced episodes of spontaneous nystagmus. In control ears endolymphatic pressure did not differ more than 0.5 cm H2O from perilymphatic pressure. In six of 11 hydropic ears, endolymphatic pressure was more than 0.5 cm H2O higher than perilympathic pressure; auditory thresholds in all these ears had deteriorated within 2 weeks before pressure recording. No further hearing deterioration within this period was noted in five hydropic ears with endolymphatic pressure equal to or lower than perilymphatic pressure. Endolympathic-perilymphatic pressure gradients may contribute to auditory threshold increase in endolymphatic hydrops, but are not its only cause.

Animals↗