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PubMed2003

Use of a multichannel auditory brainstem implant for neurofibromatosis type 2.

Neurofibromatosis type 2 (NF 2) typically results in deafness due to disruption of the cochlear nerves, making peripheral devices such as cochlear implants ineffective. Auditory brainstem implants (ABIs), for direct electrical stimulation of the cochlear nucleus, have been used to provide auditory stimulation in this group of patients. Currently, 141 patients have been implanted in our institution, most recently using an advanced multichannel device. We report results of a recent series of 86 patients who received ABIs. Of this group, 60 had successful implantation, recovered from surgery, responded successfully to stimulation and underwent a full course of device programming and audiologic testing. This group had significant improvement in scores on several audiologic tests compared to baseline. When used to augment lip reading, improvement was also seen. The degree of improvement varied considerably among patients. ABI is a useful device for deaf patients with NF 2. As measured by audiologic testing, many patients receive substantial benefit with regard to sound and speech comprehension.

Brain Stem↗

PubMed1998

Synaptophysin immunoreactivity in the cat cochlear nuclei.

The presence of synaptophysin, a presynaptic Ca2+ binding glycoprotein, has been analyzed in the cochlear nucleus complex of the adult cat using an antisynaptophysin monoclonal antibody. Synaptophysin immunoreactivity was differently distributed between regions of cochlear nuclei. Terminal boutons contacting directly with neurons (cell bodies and dendrites) or in the neuropil of ventral and dorsal cochlear nuclei appeared immunostained. In the ventral cochlear nucleus, synaptophysin-labelled boutons were clearly defined in contact with spherical, globular, multipolar, octopus and cochlear-nerve root neurons. However, the dorsal cochlear nucleus showed a dense immunostained network of synaptophysin-labelled puncta, inside which some neuron cell bodies were observed. The present findings show a differential distribution of synaptophysin-immunostained boutons between the ventral and the dorsal cochlear nuclei of the cat. The present description of the different synaptophysin-labelled terminal boutons in the cochlear nuclei complex and their pattern of distribution, will be useful for further studies on development, degeneration or regeneration of the peripheral auditory pathway.

Animals↗

PubMed1985

The physiology of the normal and pathological cochlear neurones--some recent advances.

This is the first of two auditory science tutorials designed for otolaryngologists, audiologists, and others dealing with deafness and its consequences. This tutorial is intended as an update on recent advances in the understanding of cochlear function both in the normal person and in patients with hearing loss of cochlear origin. It draws largely on studies involving electrophysiological recordings from cochlear nerve fibres in "animal models" of cochlear hearing loss. These data allow considerable insight into the underlying causes, for example, loudness recruitment, tinnitus, and poor speech intelligibility. Areas of scientific ignorance are also mentioned. This is not an exhaustive review of the literature but rather a general overview. The style of the tutorial is not starchily scientific, contains minimum jargon, and is intended to make easy reading.

Animals↗

PubMed1999

Technical developments in intra-operative monitoring for the preservation of cranial motor nerves and hearing in skull base surgery.

Although EMG recordings from mimic muscles have become the standard for intra-operative facial nerve monitoring, few data are available concerning other motor cranial nerves (MCN). Auditory brainstem responses (ABR) are a proven tool for intra-operative hearing preservation, but have their limitations, suggesting the application of supplementary methods. This paper describes new developments of MCN and cochlear nerve monitoring in skull base surgery. Up to 2 x 8 EMG channels were recorded after bipolar stimulation of MCN using concentric coaxial probes. A special software enabled event-dependent registrations of all signals exceeding a definable threshold level. Selective recordings from masticatory muscles (N.V) were obtained using rectangular Teflon-insulated needle electrodes. For oculomotor (Nn.III/ VI) nerve recordings bipolar needle electrodes were precisely placed by orbital ultrasound guidance. Lower cranial nerves were monitored inserting needle electrodes into the soft palate (N.IX), tongue (N.XII) and vocal muscles (N.X) during laryngoscopy using a special applicator. For ABR recordings, click stimuli (95 dB HL) were applied monaurally through insert earphones. Electrocochleography was simultaneously recorded as a near-field potential without averaging after promontory (transtympanic) electrode placement using otomicroscopy. Regarding the ABR biosignal, a characteristic response pattern was detected following bipolar electrical stimulation of the auditory nerve possibly useful for its intra-operative identification.

Cochlear Nerve↗

PubMed1992

Growth cones and structural variation of synaptic end-bulbs in the cochlear nucleus of the adult cat brain.

To explore the potential for structural variation and new growth at the synapse, we studied the morphological patterns of the end-bulbs of cochlear nerve axons in adult cats by using rapid Golgi, reduced silver, and electron microscopic methods. Horseradish peroxidase labeling of these endings in the anterior division of the antroventral cochlear nucleus was produced by anterograde transport following injection into the cochlea. Three types of end-bulbs were distinguished, regardless of method: reticular, coalescent, and ringed forms, all synapsing on spherical bushy cells. The reticular variety corresponds to the classically described end-bulb and constitutes the majority in all regions of the tonotopic map. The ringed end-bulb, described here for the first time, forms an excitatory synaptic cuff around the base of a bushy cell's main dendrite; these endings were localized to the region receiving cochlear input in the 1-6 kHz range, which is used in vocalization. The coalescent ending forms a small fraction of the end-bulb population throughout the region studied. The findings raise the possibility of functional differences between these synaptic types. Growth cones and retraction clubs were present on most, if not all, of the end-bulbs in every adult cat studied. A systematic survey of the end-bulb patterns revealed a continuous gradient of variation, in which each synaptic type forms a distinct mode. These findings lead us to hypothesize that the end-bulbs are in a continual state of structural and functional flux. These endings should prove useful for studies on the modifiable properties of central synapses.

Animals↗

PubMed2000

Microanatomical variations in the cerebellopontine angle associated with vestibular schwannomas (acoustic neuromas): a retrospective study of 1006 consecutive cases.

OBJECT: Great advances in neuroimaging, intraoperative cranial nerve monitoring, and microsurgical technique have shifted the focus of acoustic neuroma surgery from prolonging life to preserving cranial nerve function in patients. An appreciation of the vascular and cranial nerve microanatomy and the intimate relationship between neurovascular structures and the tumor is essential to achieve optimum results. In this paper the authors analyze the microanatomical variations in location of the facial and cochlear nerves in the cerebellopontine angle (CPA) associated with acoustic neuromas and, additionally, describe the frequency of involvement of surrounding neural and vascular structures with acoustic tumors of varying size. The authors base these findings on their experience with 1006 consecutive patients who underwent surgery via a retrosigmoid or translabyrinthine approach. METHODS: Between July 1969 and January 1998, the senior author (D.M.L.) performed surgery in 1022 patients for acoustic neuroma: 705 (69%) via the retrosigmoid (suboccipital); 301 (29%) via the translabyrinthine; and 16 (2%) via the middle fossa approach. Patients undergoing the middle fossa approach were excluded from the study. The remaining 1006 patients were subdivided into three groups based on tumor size: Group I tumors (609 patients [61%]) were smaller than 2.5 cm; Group II tumors (244 patients [24%]) were between 2.5 and 4 cm; and Group III tumors (153 patients [15%]) were larger than 4 cm. The senior author's operative notes were analyzed for each patient. Relevant cranial nerve and vascular "involvement" as well as anatomical location with respect to the tumor in the CPA were noted. "Involvement" was defined as adherence between neurovascular structure and tumor (or capsule), for which surgical dissection was required to free the structure. Seventh and eighth cranial nerve involvement was divided into anterior, posterior, and polar (around the upper or lower pole) locations. Anterior and posterior locations were further subdivided into upper, middle, or lower thirds of the tumor. The most common location of the seventh cranial nerve (facial) was the anterior middle third of the tumor for all groups, although a significant number were found on the anterior superior portion. The posterior location was exceedingly rare (< 1%). Interestingly, patients with smaller tumors (Group I) had an incidence (3.4%) of the seventh cranial nerve passing through the tumor itself, equal to that of patients with larger tumors. The most common location of the eighth cranial nerve complex was the anterior inferior portion of the tumor. Not surprisingly, larger tumors (Group III) had a higher incidence of involvement of fourth cranial nerve (41%), fifth cranial nerve (100%), ninth-11th cranial nerve complex (99%), and 12th cranial nerve (31%), as well as superior cerebellar artery (79%), anterior inferior cerebellar artery (AICA) trunk (91.5%), AICA branches (100%), posterior inferior cerebellar artery (PICA) trunk (59.5%), PICA branches (79%), and the vertebral artery (VA) (93.5%). A small number of patients in Group III also had AICA (3.3%), PICA (3.3%), or VA (1.3%) vessels within the tumor itself. CONCLUSIONS: In this study, the authors show the great variation in anatomical location and involvement of neurovascular structures in the CPA. With this knowledge, they present certain technical lessons that may be useful in preserving nerve function during surgery and, in doing so, hope to provide neurosurgeons and neurootologists with valuable information that may help to achieve optimum outcomes in patients.

Adult↗

PubMed1993

Protein gene product 9.5 in the developing cochlea of the rat: cellular distribution and relation to the cochlear cytoskeleton.

Protein gene product 9.5 was immunolocalized in the adult and early postnatal (P2-P15) rat cochlea, and its distribution compared with a 200 kDa highly phosphorylated neurofilament subunit (neurofilament 200) and alpha-tubulin. In the adult, Protein gene product 9.5 was expressed exclusively in cochlear nerve fibres and ganglion cells, a small percentage of these (Type II ganglion cells and olivocochlear bundle fibres) being intensely positive for both protein gene product and neurofilament 200. In postnatal development, pillar and Deiters' cells were at first (P2-P15) strongly positive for protein gene product 9.5, and hair cells moderately so. At P2, all nerve fibres and ganglion cells showed co-expression of protein gene product 9.5 and neurofilament 200, but at later stages, the subset of intensely co-labelled neurons appeared, nerve fibres at P7 onwards and ganglion cells from P12. There was no overt correlation between the onset of protein gene product 9.5 and alpha-tubulin expression in any cochlear component. Protein gene product 9.5 expression in ganglion cells was at first (P2 and P7) mainly nuclear, and later also cytoplasmic. It is concluded that there is a clear correlation of high levels of protein gene product 9.5 and neurofilament protein expression, and that protein gene product 9.5 is expressed in some non-neuronal cells of the cochlea during its early development, persisting until after hearing has commenced.

Animals↗

PubMed1982

Preservation of central auditory function in the deafness mouse.

Deafness mice are profoundly deaf from birth as a result of genetically determined cochlear dysfunction. Evoked potentials in response to direct electrical stimulation of the cochlear nerve can readily be recorded in the inferior colliculus of deafness mice, and such responses are larger in amplitude than those in control mice. These observations indicate that at least some central connections become functional in the deafness central auditory pathway in the absence of peripheral stimulation, and are relevant to the general problem of restoring function by direct nerve stimulation in the profoundly deaf.

Animals↗

PubMed2006

Improved preservation of hearing and facial nerve function in vestibular schwannoma surgery via the retrosigmoid approach in a series of 200 patients.

OBJECT: The aim of this study was to evaluate and present the results of current surgical treatment of vestibular schwannomas (VSs) and to report the refinements in the operative technique. METHODS: The authors performed a retrospective study of 200 consecutive patients who had undergone VS surgery over a 3-year period. Patient records, operative reports, follow-up data, and neuroradiological findings were analyzed. The main outcome measures were magnetic resonance imaging, neurological status, patient complaints, and surgical complications. Complete tumor removal was achieved in 98% of patients. Anatomical preservation of the facial nerve was possible in 98.5% of patients. In patients treated for tumors with extension Classes T1, T2, and T3, the rate of facial nerve preservation was 100%. By the last follow-up examination, excellent or good facial nerve function had been achieved in 81% of the cases. By at least 1 year postsurgery, no patients had total facial palsy. In the patients with preserved hearing, the rate of anatomical preservation of the cochlear nerve was 84%. The overall rate of functional hearing preservation was 51%. There was no surgery-related permanent morbidity in this series of patients. Cerebrospinal fluid leakage was diagnosed in 2% of the patients. The mortality rate was 0%. CONCLUSIONS: The goal of VS treatment should be total removal in one stage and preservation of neurological function, as they determine a patient's quality of life. This goal can be safely and successfully achieved using the retrosigmoid approach.

Adolescent↗

PubMed1998

Facial nerve stimulation from cochlear implants.

OBJECTIVE: To evaluate the incidence of facial nerve stimulation from cochlear implants and to better define the segment of nerve being stimulated and the causes of stimulation. STUDY DESIGN: Retrospective patient case review and a temporal bone dissection study. SETTING: A tertiary care setting. PATIENTS: All patients given a cochlear implant at the Hospital of the University of Pennsylvania. This encompassed only adult patients. INTERVENTION: All patients had surgical insertion of either a 3M single channel, Nucleus 22-channel, or CLARION multichannel cochlear implant. MAIN OUTCOME MEASURES: Demonstration of facial nerve stimulation with a cochlear implant and determination of affected electrodes; measurement of electrode location and distances between the labyrinthine segment of the facial nerve and the cochlea in temporal bone dissections: and determination of the relationship between the labyrinthine facial nerve and the cochlea using computed tomography evaluation. RESULTS: The overall incidence of facial nerve stimulation using all three devices was 14% (8 of 58). Otosclerosis and otosyphilis appear to be predisposing conditions to stimulation. The mid-cochlear electrodes, located near the labyrinthine facial nerve, appear to cause stimulation of the VIIth nerve most commonly. Computed tomographic evaluation of the bone between the labyrinthine fallopian canal and the cochlea may provide some indication of potential facial nerve problems. CONCLUSION: Facial nerve stimulation from the use of cochlear implants is more prevalent in patients with otosclerosis and otosyphilis. The labyrinthine segment of the facial nerve is the most likely area being stimulated in most patients. Preoperative computed tomographic evaluation may be beneficial in determining the possibility of this problem.

Adult↗

PubMed2001

Tonotopic projections of the auditory nerve to the cochlear nucleus angularis in the barn owl.

The nucleus angularis (NA), one of the two cochlear nuclei of birds, plays an important role in the processing of sound intensity. To begin investigating the NA in detail in the barn owl, which is a popular animal model for neural mechanisms of sound localization, a frequency map for this nucleus is presented here. Focal injections of horseradish peroxidase or neurobiotin were placed either in the NA or in the cochlear nucleus magnocellularis, labeling small groups of auditory nerve fibers of known characteristic frequency (CF) from 0.25 to 9.6 kHz. The courses of their axonal branches were used to construct a composite average map of the tonotopic frequency representation in the nucleus angularis. Nucleus angularis in the barn owl, as seen in frontal sections, resembles a sheet of cells bent approximately into an S shape. The lowest frequencies were found represented at the ventromedial extreme. The representation of increasingly higher frequencies then followed the S shape, with the highest frequencies located at the ventrolateral tip. Auditory nerve fibers of a given CF always entered the nucleus angularis within a well-restricted area and then traveled along their isofrequency band within the NA while branching off terminals. The isofrequency bands were typically slanted from caudo-ventro-medial to rostro-dorso-lateral. The basic tonotopic organization is comparable to that found in other birds, the major differences being the large size and unusual shape of the barn owl's nucleus angularis.

Animals↗

PubMed1984

Results of the round window electrical stimulation in 460 cases of total deafness.

In order to avoid false and quantitatively unprecise responses the stimulating electrode must be directly placed on the round window membrane. That necessitates a transmeatal approach removing the eardrum, which is commonly achieved under local anesthesia. The positive response is subjectively easy to identify; it may be objectively registered by means of brain stem evoked responses; this registration is indispensable in case of children who need general anesthesia. This positive response signifies that some cochlear nerve fibers are still present, and therefore that the rehabilitation of the cochlear implant is possible. Among the 460 totally deaf, stimulated patients, more than 93% of them presented a positive response. The threshold level voltage value represents a statistically significant representation of the functional value of the electrode nerve interface. The features of the tone decay tests, which may be also subjectively or objectively performed, present important data which may be used to predict the clinical results that can be obtained by the cochlear implant.

Adolescent↗

PubMed1993

[First cochlear implant in Poland].

The authors present a 30 year old patient whose hearing was profoundly impaired in the course of meningitis 12 years ago. They present details on the preparation of the patient for the surgery and indications for the implant, consecutive stages of the surgery and the first stage of the rehabilitation process are described. The results of the capability of electrical conductivity of the cochlear nerve as the direct effect of the cochlear implant and the first audiogram illustrating the hearing effect after the first 2 months of rehabilitation are presented as well.

Adult↗

PubMed1983

Cis-platinum ototoxicity: clinical experience and temporal bone histopathology.

A clinical and pathologic study of cis-platinum ototoxicity was performed. Twenty-four patients treated with cis-platinum for head and neck cancer were studied prospectively. Post cis-platinum hearing losses were subclinical, detected in 25% of patients, isolated to 4 and 8 kHz, and did not exceed 25 dB at any frequency. Temporal bones from a 9-year-old with frontal lobe astrocytoma and with cis-platinum ototoxicity were studied with routine and special techniques to elucidate oto and neurotoxicity. Degenerative changes in the outer hair cells in the lower turns of the cochlea, in the spiral ganglion, and cochlear nerve were the most striking findings.

Adult↗

PubMed1989

The narrow internal auditory canal in children: a contraindication to cochlear implants.

We suggest a new explanation for the lack of auditory response to electric stimulation in children with cochlear implants: the very narrow internal auditory canal, 1 to 2 mm in diameter, and the probable absence of the cochlear nerve. This defect can be seen on high-resolution computed tomographic x-ray studies and may represent aplasia of the auditory-vestibular nerve. We report on eight children with this anomaly, three of whom have received implants and failed to respond with a sensation of sound. Identification of this problem on screening x-ray films is a contraindication to cochlear implantation for auditory stimulation.

Acoustic Stimulation↗

PubMed1997

"En-bloc" removal of small- to medium-sized acoustic neuromas with retrosigmoid-transmeatal approach.

Surgery of acoustic neuroma (AN) has significantly refined over the past years due to a series of advances in diagnostics and surgical technique. Electrophysiologic investigation performed during surgery has greatly contributed to this progress, increasing the surgeon's understanding of the mechanism of damage and suggesting various changes in his or her surgical strategy.In this context, the advantages of the retrosigmoid "en-bloc" removal of small to medium size ANs have been examined in the present study. At the ENT Department of the University of Verona, 103 subjects with AN were operated on, from January 1990 to December 1995, with a retrosigmoid-transmeatal approach. Eighteen subjects (17.4%) presented pure a intracanalar (IC) tumor and 85 (82.6%) had both IC and extracanalar (EC) involvement. All the IC tumors (n = 18) and 70 of the IC-EC neuromas with an EC size less than 25 mm are reported in this paper for a total of 88 patients. The first 48 patients were operated on via the classic procedures described in the literature, characterized by removal of the tumor after "debulking" and limited exposure of the internal auditory canal (IAC). The following 40 subjects were operated on according to the technique of "en-bloc" removal of the tumor and wide exposure of the IAC.In the "en-bloc" group the tumor was first detached from the cerebellar flocculus and the pons, when necessary. The tumor was not debulked to preserve the anatomic relationship with the nerves and to facilitate identification, cleavage and dissection of the tumor from the neural structures. Thereafter, the posterior wall of the IAC was drilled out and opened in a circumferential range from 180 to 270 degrees . The IAC dura was subsequently opened, and the distal end of the AN along with the vestibular nerves were identified. The vestibular nerves were sectioned in the distal portion of the IAC and dissected with the tumor from the underlying facial and cochlear nerves. Dissection continued medially to the IAC porus. The AN was progressively dissected from the cochlear and facial nerves in the cerebellopontine angle (CPA) with multiple direction maneuvers, as required by the characteristics and degree of adherence to the neural structures.The anatomic and functional results obtained with this new procedure ("en-bloc" removal) were compared with the classic "debulking" technique. The statistical analysis shows an improvement in postoperative outcome for both auditory and facial nerve function. The "en-bloc" removal procedure along with the wide exposure of the content of the IAC and electrophysiologic monitoring of the seventh and eighth cranial nerves are, in our experience, the recommended strategies for improving outcomes in small to medium size ANs.

Journal Article↗

PubMed1975

The sharpening of cochlear frequency selectivity in the normal and abnormal cochlea.

In the normal (anaesthetized) animal cochlea, the frequency threshold curves for single primary fibres are up to an order of magnitude sharper than the analogous function derived from various reported measurements of the basilar membrane amplitude of vibration. This enhanced neural frequency selectivity is found in the same species and under conditions similar to those in which the mechanical measurements are taken. The sharpening process (at least near threshold) appears to be linear and is not dependent upon lateral inhibitory mechanisms. The variability of the neural frequency selectivity and its vulnerability to metabolic, chemical and pathological influences suggests the hypothesis that the sharpening is due to some form of "second filter" subsequent to the relatively broadly tuned basilar membrane. All fibres recorded from in the cochlear nerve in the normal cochlea show this enhanced frequency selectivity; in contrast, in pathological cochleas, all fibres, or a substantial proportion, have high-threshold, broadly tuned characteristics, approximating to those of the basilar membrane. The frequency selectivity of normal cochlear fibres is adequate to account for the analogous psychophysical measures of hearing. It is proposed that loss of this normal frequency selectivity occurs in deafness of cochlear origin, accounting for widening of the critical band. A new hypothesis for recruitment is proposed on this basis.

Acoustic Stimulation↗

PubMedDate not supplied

Auditory nerve and brain stem responses to sound stimuli at various frequencies.

The responses of the auditory nerve and brain stem auditory nuclei have been recorded with ear lobe and scalp electrodes in response to unfiltered click stimuli which excite mainly high-frequency nerve fibers. In order to overcome this limitation, normal subjects and patients with hearing loss were stimulated with third-octave filtered clicks with center frequencies of 250, 500 and 1 000 Hz. Analysis of the responses suggests that these lower-frequency stimuli excite lower-frequency cochlear nerve fibers. It is concluded that low-frequency filtered clicks can be used to complement unfiltered clicks in order to obtain a more complete objective audiogram.

Acoustic Stimulation↗