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OBJECTIVE: We report on vascular compression syndrome of the 12th cranial nerve (hypoglossal), an occurrence not previously reported, and demonstrate, through corresponding objective electrophysiological evidence, that microvascular decompression of the hypoglossal nerve root can cure hemilingual spasm. CLINICAL PRESENTATION: A 52-year-old man had lower face muscle twitching and tongue spasms, which worsened with talking, chewing, or emotional stress. Carbamazepine offered only temporary relief, and relief from injections of botulinum toxin was insignificant. He was referred for surgical treatment. High-resolution magnetic resonance imaging of his posterior fossa contents revealed no obvious evidence of any compressive vessel along the facial nerve, but a compressive vessel along the hypoglossal nerve was apparent. INTERVENTION: The presence of preoperative tongue spasms encouraged interoperative monitoring of tongue motor responses. The facial nerve exit zone was explored, but microsurgical inspection of the seventh/eighth cranial nerve complex did not reveal any compressive vessel. However, at the anterolateral aspect of the medulla oblongata, the hypoglossal nerve was clearly compressed and distorted laterally by a large tortuous vertebral artery. When the artery was mobilized away from the nerve, the abnormal late electromyographic response to transcranial electrical stimulation disappeared; immediately after shredded Teflon was interpositioned between the artery and the nerve, the abnormal spontaneous tongue fasciculation also disappeared. The patient has remained spasm free 6 months after surgery. CONCLUSION: Hemilingual spasm may be caused by vascular contact/compression along cranial nerve XII at the lower brainstem and belong to the same family of cranial nerve hyperactivity disorders as hemifacial spasm.

OBJECTIVE The functional anatomy (i.e., tonotopy) of the human cochlear nerve is unknown. A better understanding of the tonotopy of the central nervous system segment of the cochlear nerve and of the pathophysiology of tinnitus might help to ameliorate the disappointing results obtained with microvascular decompressions in patients with tinnitus. METHODS We assume that vascular compression of the cochlear nerve can induce a frequency-specific form of hearing loss and that when the nerve is successfully decompressed, this hearing loss can recuperate. Thirty-one patients underwent a microvascular decompression of the vestibulocochlear nerve for vertigo or tinnitus. Preoperative audiograms were subtracted from postoperative audiograms, regardless of the surgical result with regard to the tinnitus and vertigo, because the hearing improvement could be the only sign of the vascular compression. The frequency of maximal improvement was then correlated to the site of vascular compression. A tonotopy of the cochlear nerve was thus obtained. RESULTS A total of 18 correlations can be made between the site of compression and postoperative maximal hearing improvement frequency when 5-dB hearing improvement is used as threshold, 13 when 10-dB improvement is used as threshold. A clear distribution can be seen, with clustering of low frequencies at the posterior and inferior side of the cochlear nerve, close to the brainstem, and close to the root exit zone of the facial nerve. High frequencies are distributed closer to the internal acoustic meatus and more superiorly along the posterior aspect of the cochlear nerve. CONCLUSION The tonotopic organization of the cisternal segment of the cochlear nerve has an oblique rotatory structure as a result of the rotatory course of the cochlear nerve in the posterior fossa. Knowledge of this tonotopic organization of the auditory nerve in its cisternal course might benefit surgeons who perform microvascular decompression operations for the vestibulocochlear compression syndrome, especially in the treatment of unilateral severe tinnitus.

The monitoring of auditory function by recording brain stem auditory evoked potentials in patients undergoing removal of acoustic tumors is hampered by the small amplitude of the brain stem auditory evoked potentials. Because several thousands of responses must be added, it takes several minutes to obtain an interpretable record. Recordings done directly from the exposed eighth nerve have much higher amplitudes, and, therefore, interpretable responses can be obtained after only a few responses have been added. However, it is difficult to place the recording electrode in an optimal position and the electrode may interfere with the removal of the tumor. In this report, we show that evoked potentials from the cochlear nucleus, which can be recorded by placing an electrode in the lateral recess of the fourth ventricle, have a large amplitude, and that the electrode placed in this way does not interfere with the removal of the tumor. This way of monitoring, therefore, yields interpretable responses within 15 to 20 seconds, or less, and makes it possible to detect injuries to the entire intracranial portion of the eighth nerve, just as brain stem auditory evoked potentials do, but 20 to 50 times faster.

OBJECTIVE: Microvascular compressions of the cochlear nerve can lead to tinnitus. The tinnitus initially is related to nonsynchronous signal transmission in the auditory nerve, neurophysiologically characterized by a peak II amplitude decrease. Chronic compression can lead to a focal demyelination, resulting in an increase in Iinterpeak latency I-III with tinnitus and frequency-specific hearing loss as a consequence. Decompressing the cochlear nerve may result in improvement in tinnitus if the auditory nerve is not too damaged for recovery. The aim of the study is to find a cut-off point for this recovery based on clinical data. MATERIALS AND METHODS: Twenty patients undergo a microvascular decompression of the vestibulocochlear nerve for unilateral intractable tinnitus. Pre- and postoperative visual analogue scale for tinnitus intensity and tinnitus questionnaires for tinnitus distress are analyzed before and after microvascular decompression. RESULTS: Of the 20 patients studied, 10 had improvements on their tinnitus visual analogue score intensity postoperatively, 8 were unchanged, and 2 worsened. On the Tinnitus Questionnaire scores, 7 of 13 patients improved and 6 of the 13 patients worsened. If decompression is performed before the end of the 4th year of tinnitus duration, a significant tinnitus intensity improvement can be obtained (P < .05); after 4 years, improvement cannot be obtained (P = .55). However, the tinnitus distress does not seem to decrease significantly. CONCLUSION: Microvascular decompression of the cochlear nerve can improve tinnitus intensity in selected patients if decompression is performed early, before the end of the 4th year. Tinnitus distress does not seem to change.

Responses from the surface of the dorsal column nuclei and the dorsal surface of the spinal cord were recorded using monopolar electrodes after stimulation of the lower limbs (common peroneal nerve at the knee and posterior tibial nerve at the ankle) in patients undergoing neurosurgical operations for spasmodic torticollis. Those responses were smaller in amplitude than responses to stimulation of the upper limbs (median nerve at the wrist), and the waveforms differed. The negative deflection that is prominent in the response to stimulation of the upper limbs is more variable, broader, and relatively smaller in amplitude than the response to upper limb stimulation. Another difference between responses to upper and lower limb stimulation was that multiple peaks were superimposed on the initial response to stimulation of the lower limbs, but were not as consistently seen in the responses to upper limb stimulation. The negative peak in the response from the dorsal column nuclei to lower limb stimulation was of about the same latency as the P27peak in the far-field response (somatosensory evoked potential) to stimulation of the peroneal nerve.

Microvascular compression syndromes such as trigeminal neuralgia, hemifacial spasm, and disabling positional vertigo involve an artery or vein compressing a cranial nerve. A cranial nerve is composed of a central nervous system (CNS) segment and a peripheral nervous system (PNS) segment separated by the root entry/exit zone (REZ). Although vascular compression can occur at any point along the cranial nerve, it has been generally assumed that only vascular contact at the REZ of the affected cranial nerve can cause symptoms. On the basis of personal surgical experience, we propose that vascular compression of the CNS segment alone causes symptoms. This has important repercussions for the future diagnosis and treatment of microvascular compression syndromes, especially the cochleovestibular compression syndrome.For the anatomic study, four autopsy specimens and one surgical biopsy specimen of the vestibulocochlear nerve were microscopically and ultramicroscopically analyzed for structural differences between the CNS and PNS segments. For the clinical study, five patients with the clinical picture of cochleovestibular compression syndrome were treated by microsurgical decompression at the level of the CNS segment and not the REZ. One patient underwent reoperation for recurrent symptoms 4 years later, and a 4-mm vestibular neurectomy was performed at that stage. We performed an epidemiological analysis to demonstrate that the known incidences of trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia are related to the length of their respective CNS segments.Histological differences between the PNS and CNS segments suggest that the PNS segment is more resistant to compression. This was confirmed by neurophysiological data from intraoperative monitoring in posterior fossa surgery and experimental studies. We found a clear epidemiological correlation between the length of the CNS segment, which differed among cranial nerves, and the incidence of the microvascular compression syndrome. Successful decompression of the CNS segment in patients without compression at the REZ of the vestibulocochlear nerve for disabling positional vertigo provides clinical support for this hypothesis.The evidence we present supports the hypothesis that vascular compression syndromes arise from vascular contact along the CNS segment of the cranial nerves.

A method for intraoperative topographic mapping of the trigeminal nerve root using electrophysiological methods is described. A series of 15 patients under general anesthesia during microvascular decompression and selective posterior fossa trigeminal rhizotomy was studied. This method was used to study the localization of fibers of individual subdivisions of the intradural portion of the trigeminal nerve and as a guide for performing physiologically controlled, selective, microsurgical trigeminal rhizotomy.

During a 14-month period, 129 individuals underwent 140 operations for microvascular decompression to relieve hemifacial spasm, disabling positional vertigo, tinnitus, or trigeminal neuralgia at our institution. Seven patients were operated upon twice on the same side and 4 were operated upon on both sides at different times. In each case, the brainstem auditory evoked potentials were monitored intraoperatively by the same neurophysiologist. In 75 of these operations, compound action potentials were also recorded from the exposed 8th nerve. Comparison of speech discrimination scores before the operation and at the time fo discharge showed that at discharge, discrimination had decreased in 7 patients by 15% or more and increased in 4 patients by 15% or more, in 2 patients by as much as 52%. Essentially similar results were obtained when preoperative speech discrimination scores were compared with results obtained from the 87 patients who returned for a follow-up visit between 3 and 6 months after discharge. Only one patient lost hearing (during a second operation to relieve hemifacial spasm). Another patient (also operated upon to relieve hemifacial spasm) suffered noticeable hearing loss postoperatively, but had recovered nearly normal hearing by 4 months after the operation. Nine patients had an average elevation of the hearing threshold for pure tones in the speech frequency range (500 to 2000 Hz) of 11 dB or more at 4 to 5 days after the operation; 8 of these had fluid in their middle ears that most likely contributed to the hearing loss. Threshold elevations occurred at 4000 Hz and 8000 Hz in 19 and 29 ears, respectively.

The effect of manipulations in the cerebellopontine (CP) angle on the cochlear nerve was studied in 12 rhesus monkeys to elucidate how surgical procedures in the CP angle may affect the peripheral auditory system. Brain stem auditory evoked potentials and compound action potentials from the cochlear nerve were recorded throughout the experiments and were studied to determine the level of injury to the cochlear nerve. After the manipulations, the animals were perfused with fixatives and their temporal bones were removed and examined histologically. The monkeys showed electrophysiological changes similar to those observed in humans as a result of surgical manipulations in the CP angle. Avulsions of the internal auditory artery and cochlear nerve fibers at the area cribrosa were the most common histological findings. The auditory evoked potentials were suddenly and irreversibly lost during the manipulations in 3 monkeys, and hemorrhagic foci were identified histologically in these animals at the fundus of the internal auditory canal (the area cribrosa). No morphological changes at locations other than the area cribrosa were identified in these 3 monkeys. The results of this study indicate that injury to the internal auditory artery at the area cribrosa may play an important role in the abrupt loss of hearing experienced by some patients while they are undergoing operations in the CP angle. This study also led to speculation regarding the possible existence of collateral circulation to the cochlea as a factor in injury to the internal auditory artery during acoustic neurinoma operations.

Facial muscle responses in patients with hemifacial spasm undergoing microvascular decompression operations were recorded. Two peripheral branches of the facial nerve were stimulated and the electrical responses of muscles innervated by these branches were studied to see how the lateral spread of activity that is known to be present in these patients was affected by decompressing the facial nerve. In some of the patients the hemifacial spasm ceased when the dura mater was opened, in some it ceased when the arachnoid was opened, and in others the spasm persisted until the offending vessel was dissected away from the nerve. The lateral spread of activity elicited by antidromic stimulation of a branch of the facial nerve was less affected by opening of the dura mater or arachnoid: it usually persisted until the blood vessel that had been compressing the facial nerve was removed and reappeared when the vessel that had been compressing the facial nerve was allowed to slip back onto the nerve. This seems to indicate that microvascular decompression of the facial nerve is effective in alleviating hemifacial spasm because it removes the actual cause of the disorder rather than simply causing local injury to the nerve as a result of the procedure.