Surgical Anatomy of the Labyrinthine and Subarcuate Arteries and Clinical Implications.

OBJECTIVE: To study the surgical anatomy of the labyrinthine artery (LA) and the subarcuate artery (SA), their anatomic relationships, and clinical implications, as injury of the LA can result in hearing loss. METHODS: Ten formalin-fixed, latex-colored specimens were studied (20 sides). After retrosigmoid craniotomy and neurovascular dissection under microscopic magnification, 4-mm 0° and 30° endoscopic lenses were used to improve visualization. Results were statistically analyzed. RESULTS: The LA was a constant artery that followed the vestibulocochlear nerve into the internal auditory canal. The SA was an inconstant artery that ended in the dura mater around the subarcuate fossa in 35% of cases. The LA originated from the anterior inferior cerebellar artery in 89.3% of specimens and from the basilar artery in 10.7% of specimens. The SA branched off from the anterior inferior cerebellar artery when present. The origin of the LA was inferomedial to the vestibulocochlear nerve in most cases (71.4%), whereas the SA was usually lateral (70%). The distal portion of the LA was inferomedial to the vestibulocochlear nerve in 71.4% of cases. The distal portion of the SA was superolateral to the nerve in all cases (P < 0.00001). CONCLUSIONS: Knowledge of the different trajectory and anatomic relationship of the LA and the SA with the vestibulocochlear nerve is of paramount importance to differentiate them during surgery. The LA is usually inferomedial to the vestibulocochlear nerve at its distal and proximal aspects, whereas the SA usually originates lateral and ends superolateral to the nerve.

Acute vertigo and sensorineural hearing loss from infarction of the vestibulocochlear nerve: A case report.

RATIONALE: Acute unilateral audiovestibulopathy is a common neurotological syndrome. Differential diagnoses of acute unilateral audiovestibulopathy include viral infection, vascular insults, and tumors. Regarding vascular causes, ischemic stroke in the anterior inferior cerebellar artery (AICA) territory is known to be the leading cause of acute audiovestibular loss. Previous reports of AICA infarction with audiovestibulopathy failed to demonstrate magnetic resonance imaging (MRI)-positive vestibulocochlear infarctions. Only 1 report demonstrated acute infarction involving the vestibulocochlear nerve on diffusion weighted imaging (DWI)-MRI. PATIENT CONCERNS: A 67 year old man complained of sudden left hearing loss and vertigo. The patient showed left horizontal gaze-evoked nystagmus (GEN) and the head impulse test (HIT) was positive on the left side. Videonystagmography revealed spontaneous rebound nystagmus toward the right side; head-shaking nystagmus toward the right side. The patient presented with left caloric paresis (20.1%). Pure tone audiometry (PTA) revealed severe sensorineural hearing loss on the left side. DIAGNOSIS: MRI of temporal bone showed multifocal acute infarctions in the left inferior cerebellum. Moreover, images revealed tiny infarctions along the left vestibulocochlear nerve and the cochlea, implying acute vestibulocochlear nerve and labyrinthine infarction. There was no evidence of steno-occlusion of major cerebral vessels on MR angiography. INTERVENTIONS: Immediate stroke management was done. OUTCOMES: Neurological symptoms gradually improved after 3 to 5 days. LESSONS: We present a case illustrating a rare but significant finding of vestibulocochlear nerve infarction revealed by DWI-MRI. Prompt imaging protocol enabled the detection of significant findings in this patient with acute unilateral audiovestibulopathy. Clinicians should be aware of the vestibulocochlear nerve and labyrinth on MRI in patients with cerebellar stroke.

Ruptured intrameatal AICA aneurysms--a report of two cases and review of the literature.

BACKGROUND: Aneurysms of the distal part of the anterior-inferior cerebellar artery (AICA) are rare. Most are located in the cerebellopontine angle close to the internal auditory meatus. To our knowledge, only 13 patients with the aneurysm located inside the internal auditory meatus have been reported in the literature. METHOD AND FINDINGS: We present two cases of ruptured intrameatal AICA aneurysms that were treated in our center in the past year. The locations and clinical manifestations are discussed and all 13 previously published cases are reviewed. CONCLUSION: Exposure and decompression of all structures in the internal auditory meatus, careful manipulation of the nerves and vessels, temporary trapping of the aneurysm, and careful dissection of the neck to avoid partial clipping are the essential steps when treating intrameatal AICA aneurysms to reduce the risk of postoperative neurological deficits.

Is microvascular compression of the vestibulocochlear nerve a cause of unilateral hearing loss?

OBJECTIVES: We sought to confirm earlier findings in the literature that microvascular compression of the vestibulocochlear nerve might cause unilateral sensorineural hearing loss. We measured the length and width of the internal auditory canal (IAC) to investigate a possible association between a narrow porus, the presence of an anterior inferior cerebellar artery (AICA) loop, and the development of a microvascular compression syndrome. METHODS: We performed a prospective blinded analysis of 167 magnetic resonance imaging scans of the cerebellopontine angle. The presence of an AICA loop was scored. We analyzed these 167 patients for unilateral sensorineural hearing loss, which was defined as an interaural difference of 20 dB at 1 frequency or 10 dB at 2 or more frequencies. Furthermore, the width and length of the IAC on magnetic resonance imaging were measured. RESULTS: An AICA loop was identified in 94% of the 167 patients. There were 196 type I loops, 106 type II loops, and 14 type III loops. Sixty-six patients had unexplained unilateral hearing loss. There was no association between type II and III vascular loops, the width of the IAC, and unilateral hearing loss (p > .05). CONCLUSIONS: In this study we found no association between the depth of extension of the AICA loop into the IAC and the presence of unilateral hearing loss.

Cavernous malformation of the internal auditory canal.

Cavernous malformations of the internal auditory canal are a rare clinical entity that, however, should be considered in the differential diagnosis of intracanalicular masses. Even though this type of malformation is usually associated with an evident gadolinium enhancement at MR examination, in some patients, like in this case, the signal characteristics may be not sufficiently specific to allow the correct preoperative diagnosis. Nevertheless, the clinical history, in particular, a rapid onset of cranial nerve deficits, lead to the suspicion of a vascular malformation.

Cavernous malformation within a schwannoma: review of the literature and hypothesis of a common genetic etiology.

The finding of cavernous malformations within tumors of the central or peripheral nervous system is a rare occurrence. We report a case of a histologically proven cavernous malformation found within an eighth cranial nerve schwannoma in a 76-year-old man. The patient presented with progressive loss of hearing on the left, facial pain and dysesthesia. Symptoms improved significantly after the tumor was subtotally resected through a left retrosigmoid craniotomy. Including the present report, 34 cases of cavernous malformations associated with tumors of nervous system origin, 24 cases (71%) involving tumors of Schwann cell origin, and 9 cases (26%) involving gliomas have been published. The cases were classified into two forms based on the type of association. Conjoined association, in which the cavernous malformation is located within the tissue of the nervous system tumor, and discrete association, in which the cavernous malformation and nervous system tumor are in separate locations. We explore the etiology of this association and hypothesize that a common genetic pathway may be involved in a majority of these cases.

The vestibulocochlear nerve.

The eighth nerve provides sensory connection for balance and sound. Our ability to evaluate its morphology and pathology with magnetic resonance imaging (MRI) has been substantially improved over the past 20 years. This article reviews the relevant anatomy of the eighth nerve. A thorough discussion of the potential pathologic processes affecting its function also is included. Foremost, is a review of acoustic schwannomas, emphasizing the role of imaging in the work-up and ongoing evaluation of these patients. Discussion and examples of the plethora of neoplastic, inflammatory, infectious, congenital, ischemic, and compressive pathologies involving the nerve are included as well.

Microvascular decompression of the vestibulocochlear nerve for disabling positional vertigo: the House Ear Clinic experience.

OBJECTIVE: To review characteristics of and outcome in patients undergoing microvascular decompression of the vestibulocochlear nerve. Patients studied had a diagnosis of disabling positional vertigo caused by a vascular loop compressing the VIIIth cranial nerve. STUDY DESIGN: Retrospective chart review and telephone interview. SETTING: Private practice tertiary neurotologic referral center. PATIENTS: Twenty patients with disabling positional vertigo underwent 25 retrosigmoid craniotomies for microvascular decompression between November 1990 and June 1999. The 4 men and 16 women ranged in age from 30 to 71 years (mean age, 46 yr). MAIN OUTCOME MEASURES: Charts were reviewed and patients were contacted by telephone and asked to rate severity of symptoms (tinnitus and dizziness) on a 4-point scale (none = 1, mild = 2, moderate = 3, and severe = 4) before and after surgery. They were also asked to rate their overall disability from their symptoms on the six-point scale established by the American Academy of Otolaryngology-Head and Neck Surgery. Preoperative and postoperative four-frequency (500 Hz, 1 kHz, 2 kHz, and 4 kHz) pure-tone average and speech discrimination scores were calculated and compared. Complications of surgery are also reported. RESULTS: Postoperative tinnitus score and dizziness score showed significant improvement from preoperative scores (p < or = 0.047 and p < or = 0.001, respectively), with 80% of patients improved in dizziness rating; 85% improved in their overall disability rating, and the difference from preoperative to postoperative was significant (p < or = 0.001). The mean postoperative pure-tone averages (15.4 dB) and speech discrimination scores (99%) did not differ from preoperative scores (11.9 dB and 98%). One patient lost all vestibular function in the operated ear (hearing remained intact) as the only complication of surgery. When asked, 83% of patients responded that they would have the surgery again. CONCLUSIONS: Diagnosing disabling positional vertigo secondary to vascular compression of the VIIIth cranial nerve remains the clinical challenge; a clear history plus air-contrast computed tomographic or magnetic resonance imaging make the diagnosis. Microvascular decompression of the vestibulocochlear nerve is a safe and effective operation for these carefully selected patients.

Internal auditory artery infarction: clinicopathologic correlation.

OBJECTIVE: To study the pathophysiology of labyrinthine infarction. BACKGROUND: The syndrome of sudden onset vertigo or hearing loss is commonly attributed to inner ear vascular disease, yet histologic studies of isolated labyrinthine infarction in humans have been rare and have not included a complete examination of the vertebrobasilar vascular system. METHODS: Temporal bones, brainstem, cerebellum, and the supplying blood vessels were subjected to gross and microscopic postmortem examinations in a 92-year-old woman who had a sudden onset of vertigo and hearing loss in the right ear 7 years before death. RESULTS: There were prominent atherosclerotic changes at the vertebrobasilar junction, but the internal auditory artery and its branches were patent on both sides. Histologic studies showed degenerative changes in the cochlea and vestibular labyrinth on the right. The posterior canal ampulla and saccular macule were relatively preserved showing partial areas of intact sensory epithelium with underlying nerve fibers. The right vestibulocochlear nerve showed a fibrotic scar and multiple patchy areas of degeneration. These findings are most consistent with a transient period of reduced perfusion of the internal auditory artery. CONCLUSION: The partial sparing of the inferior vestibular labyrinth may indicate a decreased vulnerability to ischemia because of its better collateral blood supply.

Is there a correlation between vascular loops and unilateral auditory symptoms?

OBJECTIVES: To assess whether contact of a vascular loop formed by the anterior inferior cerebellar artery (AICA) with the eighth cranial nerve correlated with unilateral auditory symptoms so as to produce a "vascular compression syndrome." STUDY DESIGN: Prospective evaluation of patients with unilateral auditory symptoms using magnetic resonance imaging (MRI) scans to identify contact of a vascular loop with the eighth cranial nerve. METHODS: One hundred twelve patients with idiopathic unilateral auditory symptoms (42 women and 70 men, mean age of 51 years) were evaluated with MRI. Location of the vascular loop and contact with the eighth cranial nerve were assessed in each case. The asymptomatic contralateral ears of the patients were used as controls. A power analysis had determined the size of the sample to be studied. RESULTS: The arterial loop was found to be in contact with the eighth cranial nerve in 28 (25%) of the 112 symptomatic ears and in 24 (21.4%) of the asymptomatic (control) ears. The statistical analysis revealed that the difference was not statistically significant. CONCLUSION: The results suggest that radiologic demonstration of contact between a vascular loop formed by the AICA and the eighth cranial nerve on MRI scans should be considered a normal anatomic finding and should not, on its own, be used to support the diagnosis of a "vascular compression syndrome."

Neurovascular decompression of the eighth cranial nerve in patients with hemifacial spasm and incidental tinnitus: an alternative way to study tinnitus.

OBJECT: The authors sought to clarify the clinical characteristics of tinnitus resulting from neurovascular compression (NVC) of the eighth cranial nerve. METHODS: The authors explored the eighth cranial nerve in the cerebellopontine cistern during neurovascular decompression (NVD) of the facial nerve in 10 patients with hemifacial spasm who suffered from incidental tinnitus on the same side. The diagnosis of NVC of the eighth cranial nerve was confirmed in all patients. This condition was found in only seven of 114 patients with hemifacial spasm alone, indicating that NVC of the eighth cranial nerve is one of the causes of tinnitus (p < 0.001, chi-square test). The tinnitus resolved or was markedly improved after NVD of the eighth cranial nerve in eight patients (80%). Both pulsatile and continuous tinnitus responded well to NVD. All patients experienced various degrees of sensorineural hearing disturbance, but other neurotological examinations provided poor diagnostic value. CONCLUSIONS: It is the authors' opinion that sensorineural hearing loss and positive findings on magnetic resonance imaging are the most reliable evidence for the presence of tinnitus caused by NVC of the eighth cranial nerve.

Neurovascular compression syndrome of the eighth cranial nerve. What are the most reliable diagnostic signs?

Forty-three surgical cases were retrospectively analyzed to establish diagnostic criteria and operative indications for vertigo and tinnitus due to neurovascular compression (NVC) of the eighth cranial nerve (8th N). Many NVC syndromes were mistakenly diagnosed as Ménière's disease or benign paroxysmal positional vertigo. NVC was confirmed in 31 of the 43 patients. Neurovascular decompression (NVD) resulted in complete recovery or marked improvement of subjective symptoms in all 19 cases with vertigo (100%), and in 19 of 29 patients with tinnitus (65.5%). Multiple factor analysis revealed that abnormal caloric responses have high diagnostic value for vertigo due to NVC. Vertigo due to NVC is of short duration (a few sec to a few min.) in the early phase of the disease, which becomes longer and hearing becomes impaired as the history of NVC lengthens. Low pitch pulsatile and high pitch continuous tinnitus are probably due to NVC and are cured by NVD if hearing is still preserved. Tinnitus associated with hemifacial spasm is strongly indicative of NVD. Decompression of the 8th N should be performed in the early phase of disease, since cochlear and vestibular functions are irreversibly impaired if NVC continues for a long period of time.

P-glycoprotein expression in capillary endothelial cells of the 7th and 8th nerves of guinea pig in relation to blood-nerve barrier sites.

Expression of P-glycoprotein (P-gp) was detected by immunohistochemical staining and Western blot analysis in the peripheral nerves (7th and 8th nerves) of the guinea pig using anti-P-gp monoclonal antibody C219. P-gp was detected in the capillary endothelial cells of these nerves. Immunoreactivity in these nerves was similar to that in the brain. Besides these nerves, positive staining was observed in the sciatic nerve, although immunoreactivity was somewhat lower than that of the 7th and 8th nerves. The present investigation suggested that P-gp in the endothelial cells of the peripheral nerves might play a very important role as a part of the blood-nerve barrier function, since P-ag acts as an extension pump in these cells.

The vasculature of the peripheral portion of the human eighth cranial nerve.

The vasculature of the peripheral portion of the human eighth cranial nerve (VIIIN) was investigated by light and transmission electron microscopy. Arterioles and venules running longitudinally around the VIIIN formed the extrinsic vascular system. The anatomical relationship between these extrinsic vessels and the VIIIN sheath was similar to that between blood vessels on the surface of the brain and the pia mater. In the endoneurium, postcapillary venules and large capillaries were sparsely distributed and longitudinally arranged, and these microvessels formed the intrinsic microvascular system, which was supported by the extrinsic vascular system via anastomosing vessels. The ultrastructural features of the internal auditory artery and its main branches were the same as those of other intracranial arteries. Ultrastructural study also revealed myo-endothelial junctions in anastomosing arterioles, and endothelio-pericytic junctions in extrinsic and anastomosing venules. Microvascular endothelial cells were connected by tight junctions in both the vestibular ganglion and the rest of the VIIIN. These features of the vasculature were considered to be effective for maintenance of the endoneurial fluid and regulation of the circulation in the peripheral portion of the human VIIIN.

Gliosis of the eighth nerve transitional region in patients with cerebellopontine angle schwannoma.

The central-peripheral transitional region (TR) of the eighth nerve root was obtained from 8 patients with cerebellopontine angle schwannoma and investigated by light microscopy, electron microscopy, and immunohistochemistry. As a control, the TRs of 6 autopsy patients without any otologic disease were studied. Astrocytic proliferation at the TR was more prominent in the schwannoma patients than in the normal controls. Mild gliosis of the mantle zone of the TR was found in 5 schwannoma patients and moderate gliosis was noted in 3. The distribution of capillaries at the TR differed between the schwannoma patients and the normal controls. In the schwannoma patients, capillaries were rarely found in the mantle zone of the TR but were densely clustered at the glial fringe zone, especially near its border with the peripheral nervous tissue. Stasis of the capillaries in the glial fringe zone was frequently noted. These histological findings may indicate the occurrence of gliosis in response to eighth nerve degeneration. We suggest that there is increased susceptibility of the eighth nerve to injury at the TR in patients with schwannoma.

Ultrastructure of astrocytes in the transitional region of the human eighth cranial nerve.

The nerve root segment where the transition from central to peripheral nervous tissue occurs is referred to as the transitional region (TR). Part of the TR is a continuation of the subpial glial limiting membrane (SGLM) which covers the surface of the brain. To assess the physiological and pathophysiological roles of astrocytes in the TR of the human eighth cranial nerve, electron microscopy was performed on surgically resected specimens of the eight cranial nerve root obtained from patients with cerebellopontine angle tumors. The astrocytic glial dome was sharply bounded by a basement membrane and was mainly composed of the bodies and processes of fibrous astrocytes. Desmosomes and gap junctions were found between these astrocytic processes. Half-desmosomes were found on the edge of the astrocytic glial dome and at the luminal surface of the perivascular glial limiting membrane. These ultrastructural features indicate that the astrocytes of the TR might have some motility capacity that allows adjustment to changes in the pressure exerted by the vessels, the peripheral nervous tissue, and the cerebrospinal fluid. In addition, it is also suggested that lack of structures linking the central and peripheral portions of the nerve and the abrupt change of nerve composition at the TR might predispose this area to injury by tractional forces or pathologic conditions such as tumor or arterial compression.

A case of cerebellar infarction occurred with the 8th cranial nerve symptoms.

A rare case, 32-year-old man, of cerebellar infarction with the occurrence of the 8th cranial nerve symptoms was reported. On the neuro-otological examination, hearing test and caloric test showed a severe hearing loss and no response on the right side, respectively, and the spontaneous horizontal nystagmus fixed to the left direction was observed. Magnetic resonance (MR) imaging showed the infarctions in the areas of anterior inferior cerebellar artery (AICA) and posterior inferior cerebellar artery (PICA) on the right side. On the 14th illness day, the 8th cranial nerve symptoms disappeared, and on the 12th illness day, right hearing level and caloric response were significantly improved. We suggested that such an early recovery of the subjective symptoms and neuro-otological findings may be attributable to the recanalized circulation disturbance or the development of collateral circulation.