Neuro-Ophthalmology & Neuro-Otology
Toxic and nutritional deficiency optic neuropathies
Nov. 24, 2024
Worddefinition
At vero eos et accusamus et iusto odio dignissimos ducimus qui blanditiis praesentium voluptatum deleniti atque corrupti quos dolores et quas.
Labyrinthine infarction …
- Updated 05.14.2024
- Released 08.15.2000
- Expires For CME 05.14.2027
Labyrinthine infarction
Introduction
Overview
The author explains the clinical presentation, pathophysiology, prevention, diagnostic work-up, and management of labyrinthine infarction. Although a variety of conditions can cause labyrinthine ischemia, labyrinthine infarction most commonly occurs in the setting of thromboembolic disease of the anterior inferior cerebellar artery or the basilar artery, and almost all of these cases present with acute prolonged vertigo and vestibular dysfunction of peripheral, central, or combined origin. Unlike labyrinthine dysfunction from a viral cause, the most common pattern of dysfunction with labyrinthine infarction includes a combined loss of auditory and vestibular function. Canal paresis associated with posterior circulation ischemic stroke, usually within the anterior inferior cerebellar artery territory, typically normalizes within 5 years.
Key points
|
• The clinical manifestations of ischemia of the inner ear can include acute vertigo, nausea and vomiting, imbalance, unilateral deafness, tinnitus, and canal paresis. | |
|
• A variety of conditions can cause labyrinthine ischemia, including thromboemboli of the posterior circulation, fat emboli, thromboangiitis obliterans, migrainous infarction, decompression illness, hyperlipidemia, macroglobulinemia, sickle cell disease, leukemia, polycythemia vera, and other causes of hypercoagulation or hyperviscosity. | |
|
• Most commonly, labyrinthine infarction occurs in the setting of thromboembolic disease of the anterior inferior cerebellar artery or the basilar artery. | |
|
• Unlike labyrinthine dysfunction from a viral cause, the most common pattern of dysfunction with labyrinthine infarction includes a combined loss of auditory and vestibular function. | |
|
• If neurologic symptoms are absent, it is more likely that viral labyrinthitis is the cause of acute audiovestibular symptoms, especially in the absence of abnormal findings on imaging. | |
|
• Although acute unilateral hearing loss and hypofunction of the vestibulo-ocular reflex typically indicate labyrinthine dysfunction, cerebellar findings should not be overlooked, including direction-changing positional nystagmus, which suggests dysfunction of the posterior cerebellar vermis. | |
|
• Small infarcts of the brainstem or cerebellum can produce vertigo without other localizing neurologic symptoms: roughly 1 in 10 patients with an isolated cerebellar infarction present with vertigo, nystagmus, and postural unsteadiness mimicking acute peripheral vestibular disorders. | |
|
• The blood supply to the inner ear flows through only one main blood vessel, the internal auditory artery (or labyrinthine artery), which typically originates from the anterior inferior cerebellar artery. |
Historical note and terminology
A series of important experimental studies in animals in the 1950s established that (1) cochlear function is extremely sensitive to anoxia, and electrical activity deteriorates within 60 seconds of interruption of blood flow; (2) cochlear function may return to normal if blood flow is restored within 8 minutes of complete obstruction but not if blood flow is interrupted for more than 30 minutes; (3) permanent obstruction of either the inferior cochlear vein or the internal auditory artery produces rapid loss of function and histologic changes; (4) external hair cells and the ganglion cells of the cochlea are particularly vulnerable to arterial obstruction, whereas the vestibular end organs are relatively resistant; (5) venous obstruction produces early epithelial edema, followed by hemorrhage into the epithelium and perilymphatic and endolymphatic spaces, hair cell damage with secondary ganglion cell degeneration, and later fibrosis and ossification; (6) arterial obstruction produces more rapid and severe damage than venous obstruction; (7) arterial obstruction produces early histologic changes in hair cells within 30 minutes, followed in a few hours by extensive necrosis including the supporting cells without hemorrhage and ultimately severe fibrosis and ossification by 6 months; (8) several patterns of end-organ involvement were observed with arterial obstruction corresponding to different arterial distributions within the inner ear; and (9) patterns of end-organ damage with arterial obstruction included involvement of all cochlear and vestibular end organs, involvement of the cochlea, saccule, and the posterior canal ampulla, or involvement of the utricle, part of the saccule, and the anterior and horizontal canal ampullae, but without cochlear involvement (21; 52; 53; 54; 55; 98; 97).
Clinical manifestations
Presentation and course
|
• Labyrinthine ischemia typically occurs in conjunction with brainstem and cerebellar ischemia due to involvement of the anterior inferior cerebellar artery (AICA) or the vertebrobasilar system. | |
|
• The preponderance of AICA-associated labyrinthine infarcts occurs because the labyrinth is supplied by the internal auditory artery, which, in turn, derives from the AICA. | |
|
• Clinically, labyrinthine infarction has been diagnosed based on several different scenarios: (1) AICA-territory infarcts with associated audiovestibular defects consistent with labyrinthine involvement; (2) an isolated acute audiovestibular defect is later followed by an AICA-territory infarct; or (3) an isolated acute audiovestibular defect occurs in conjunction with embolic infarctions outside of the AICA territory. | |
|
• The clinical manifestations of ischemia of the inner ear can include acute vertigo, nausea and vomiting, imbalance, unilateral deafness, tinnitus, and canal paresis. | |
|
• Arterial occlusion affecting the inner ear may present in several ways, with predominant auditory dysfunction, predominant vestibular dysfunction, or both. |
Labyrinthine ischemia typically occurs in conjunction with brainstem and cerebellar ischemia because of involvement of the anterior inferior cerebellar artery (AICA) or the vertebrobasilar system. The preponderance of AICA-associated labyrinthine infarcts occurs because the labyrinth is supplied by the internal auditory artery, which, in turn, typically derives from the AICA. Less commonly, labyrinthine ischemia may occur in conjunction with cochlear ischemia in the absence of brainstem or cerebellar involvement, or it may occur in isolation, for example, because of cardioemboli (13; 45; 95; 78). The constellation of clinical manifestations depends on the extent and distribution of the ischemia.
Spectrum of labyrinthine infarction. Clinically, labyrinthine infarction has been diagnosed based on several different scenarios:
(1) AICA-territory infarcts with associated audiovestibular defects consistent with labyrinthine involvement. This is the most common scenario. Labyrinthine infarction is most often presumed when patients show a combined loss of hearing and peripheral vestibular function associated with brainstem or cerebellar infarctions in the AICA territory (67; 78). Labyrinthine artery occlusion (sudden deafness and vertigo in one ear) may also herald a basilar artery stroke because the labyrinthine artery bifurcates from the AICA (117).
(2) An isolated acute audiovestibular defect is later followed by an AICA-territory infarct. Isolated labyrinthine infarction or ischemia has been described as an initial presentation of AICA infarctions based on subsequent progression of an isolated acute audiovestibulopathy into a full AICA-territory infarction involving the brainstem and cerebellum (50; 67; 78).
(3) An isolated acute audiovestibular defect occurs in conjunction with embolic infarctions outside of the AICA territory. Rarely, sudden deafness with or without caloric canal paresis may occur in association with infarctions involving the cerebellum or brainstem but in a non-AICA vascular territory. The sudden deafness in these cases was attributed either to a dominant posterior inferior cerebellar artery supplying the labyrinth, to the relative ischemic vulnerability of the labyrinth (63), or to a separate embolism to the labyrinth, which was not visualized with available imaging techniques (78). When there is an evident cardiac or artery-to-artery source of emboli, and when the associated non-AICA-territory infarctions are consistent with embolic phenomena, then a separate embolism to the labyrinth is the most likely explanation for the acute audiovestibular defects (78).
The clinical manifestations of ischemia of the inner ear can include acute vertigo, nausea and vomiting, imbalance, unilateral deafness, tinnitus, and canal paresis (85; 82; 23; 29; 83; 84; 94; 51; 112; 44; 68; 67; 78).
Arterial occlusion affecting the inner ear may present in several ways, with predominant auditory dysfunction, predominant vestibular dysfunction, or both. Those patients with predominant auditory dysfunction may present with sudden deafness (23; 118; 73; 65; 63), sometimes accompanied by transient dizziness and intermittent tinnitus (23; 29; 73; 65). Those with predominant vestibular dysfunction present typically with vertigo, nausea and vomiting, imbalance, canal paresis without hearing loss (83; 47; 63), and occasionally with tinnitus (112) or subsequent positional vertigo (77). Finally, patients may present with combined deficits, including vertigo and deafness (23; 29; 83; 118; 51; 116; 73; 65; 12; 99). These patterns reflect different vascular territories or susceptibilities to ischemia.
With ischemia of a labyrinth, there is typically a depression of vestibular activity on that side with resulting ipsilateral deafness or tinnitus and nystagmus beating away from the affected side and with a tendency to past-point and fall toward the ischemic labyrinth with Romberg testing or tandem gait (60; 61). Rarely, transient ischemia may produce brief excitation of inner ear structures resulting in ipsilateral tinnitus but horizontal-rotatory nystagmus beating toward the affected side (112). This is thought to result from transiently increased excitability of hair cells and neurons with the onset of ischemia, resulting in an increased firing rate (112). With more prolonged ischemia, however, the membrane depolarizes, and the axonal membrane becomes inexcitable, thus, producing the typical manifestations of labyrinthine hypofunction.
Hyperviscosity syndromes. Ischemia may also occur with vascular obstruction in the venules and capillaries that drain the inner ear, as occurs most commonly with conditions that produce marked serum hyperviscosity. The “hyperviscosity syndrome” includes many diverse clinical manifestations, including headache, fatigue, vertigo, nystagmus, sudden or progressive hearing loss, visual disturbances, and mucosal hemorrhages (92; 05). Ophthalmoscopic findings include markedly distended and tortuous (“sausage-shaped”) retinal veins and retinal hemorrhages similar to the pattern seen in retinal vein occlusion.
Vertebrobasilar ischemia. Vertebrobasilar ischemia with occlusion of the vertebrobasilar junction can present with episodes of monosymptomatic vertigo, likely due to labyrinthine ischemia (26; 22; 25). Peripheral vestibular abnormalities are common among such patients on electronystagmographic testing and presumably also on videonystagmography (26). Similarly, vertigo is among the most common prodromal symptoms of basilar artery occlusion; a number of these cases have clinical or pathologic evidence of peripheral vestibular dysfunction (119; 57; 20; 35).
Posterior inferior cerebellar artery (PICA) infarcts with mixed central and peripheral vestibular deficits. The spectrum of clinical presentation for PICA territory infarcts (lateral medullary syndrome or Wallenberg syndrome) includes a range of labyrinthine, brainstem, and cerebellar symptoms and signs, but isolated vertigo is possible and, indeed, fairly common (64). This syndrome is usually characterized by crossed sensory deficits affecting the face on the side of the infarct and affecting the trunk and extremities on the opposite side: specifically, there is a loss of pain and temperature sensation on the ipsilateral (ipsilesional) side of the face and the contralateral (contralesional) side of the body. Clinical symptoms and signs may include nystagmus, diplopia, skew deviation, facial pain, vertigo, dysphagia, dysarthria, dysphonia, ipsilesional hemiataxia, ipsilesional Horner syndrome, and possibly palatal myoclonus. Nystagmus and vertigo may result from involvement of the region of Deiters nucleus and other vestibular nuclei. Cases have been reported with mixed central and peripheral deficits following vertebral artery dissection and development of lateral medullary infarction and embolic internal auditory artery infarction (12). Findings on clinical examination in patients presenting with an acute vestibular syndrome include a normal bedside head impulse test, absence of acute hearing loss, variable spontaneous or gaze-evoked nystagmus, variable skew deviation, and variable (though usually severe) postural instability (11; 64; 48).
Anterior inferior cerebellar artery (AICA) infarcts with mixed central and peripheral vestibular deficits. The spectrum of clinical presentation for AICA territory infarcts also includes a range of labyrinthine, brainstem, and cerebellar symptoms and signs: ipsilateral Horner syndrome (rare), skew deviation (rare), pathological ocular torsion, nystagmus, ipsilateral facial numbness, ipsilateral facial paresis (possibly restricted to emotional facial paresis), vertigo, sudden ipsilateral hearing loss with or without tinnitus, dysarthria, vomiting, variable unsteadiness, ipsilateral truncal deviation, ipsilateral hemiataxia, and contralateral loss of pain and temperature sensation on the limbs and body (03; 94; 04; 32; 72; 70; 73; 65; 63; 67; 42; 88; 66; 111; 01; 46; 79; 64). Vertigo with hypoacusis occurs most commonly in anterior inferior cerebellar artery-territory infarctions that involve both the middle cerebellar peduncle and the inferior lateral pons (100). Hearing loss occurs in about two thirds of patients with anterior inferior cerebellar artery-territory infarcts (114). In some cases, the only neurologic sequela is isolated permanent ipsilateral hearing loss (88). Occasionally, isolated vertigo may occur as transient ischemic attacks preceding anterior inferior cerebellar artery-territory infarction or with partial anterior inferior cerebellar artery infarcts (03; 94; 64). It is difficult to establish labyrinthine infarction clinically in the setting of anterior inferior cerebellar artery territory infarction because central and peripheral signs are often mixed (111). Also, central lesions may uncommonly produce similar clinical manifestations of vertigo, nystagmus, hearing loss, canal paresis, and ataxia (102; 34; 33; 24; 64). In particular, vascular lesions at the root entry zone or involving the vestibular nuclei and portions of the cerebellum can present clinically as acute unilateral vestibulopathy, including with concomitant caloric canal paresis and an abnormal bedside head impulse test (28; 33; 24; 93; 11; 64; 109); this subtype of anterior inferior cerebellar artery stroke has been labeled AICAp with peripheral characteristics, which is indicated by a positive clinical head impulse test, as distinguished from AICAc with central characteristics, which is indicated by a negative clinical head impulse test (11).
Acute cochleovestibulopathy. An acute, persisting vestibular syndrome lasting more than 24 hours with ipsilateral sensorineural hearing loss suggests a vascular etiology (ie, labyrinthine infarction) rather than inflammatory disease (eg, labyrinthitis), especially in patients with cardiovascular risk factors (44; 67; 02; 16).
An acute isolated labyrinthine infarction may progress into brainstem or cerebellar stroke (50; 45), and in this way, labyrinthine infarction is comparable to amaurosis fugax or branch retinal artery occlusion.
HINTS and HINTS plus. An isolated labyrinthine infarction (without brainstem or cerebellar involvement) does not elicit a central "HINTS" examination result because it is a peripheral vestibulopathy, even if it carries a significant risk of posterior circulation stroke (16). The so-called “H.I.N.T.S.” or “HINTS” tripartite examination (ie, Head Impulse test, Nystagmus type, and Test-of-Skew) combines an assessment of the head impulse test (HIT), assessment of direction-changing horizontal nystagmus evoked by lateral gaze to the right and left, and assessment of vertical skew deviation by alternate cover testing (41; 10; 91; 90; 105; 104; 09; 115). The acronym mnemonic INFARCT—Impulse Negative (negative head impulse test); Fast phase Alternating (central pattern nystagmus); and Refixation during Cover Test (ie, skew deviation)—suggests a stroke or other central diagnosis (41). Typically, with labyrinthine infarction, the HINTS exam would not suggest a stroke or other central diagnosis: (1) the head impulse test will be abnormal with a visible refixation saccade with ipsilesional head impulses; (2) there will be a peripheral vestibular nystagmus characterized by unidirectional horizontal-torsional jerk nystagmus with a contralesional fast phase that intensifies with fixation removed; and (3) there will be no skew deviation evident with alternate cover testing. In the absence of other information, this HINTS result would generally be interpreted as suggesting a relatively benign peripheral vestibular condition, like vestibular neuritis. However, acute cochleovestibular loss is not always due to a viral or inflammatory inner ear condition; it may result from ischemia or infarction of the cochlea and labyrinth (40).
The updated form of the HINTS examination approach, the “H.I.N.T.S. plus” or “HINTS plus” quadripartite examination, addresses some of the limitations of HINTS. The HINTS plus examination is simply the HINTS examination plus an assessment of bedside hearing by finger rub (91; 90; 105). Most cases of labyrinthine infarction with both labyrinthine and cochlear involvement will be identified by the HINTS plus approach because, fortunately, the most common pattern of dysfunction with labyrinthine infarction is a combined loss of auditory and vestibular function (45).
Despite the numerous studies promoting HINTS or HINTS plus, the individual elements of the quartet of findings may be misleading at times. Patients with peripheral vestibulopathy may have a skew deviation, suggesting (wrongly) that the lesion is central. In fact, up to a quarter of individuals with an acute unilateral vestibulopathy have a skew deviation (27; 59), although such skew deviations with peripheral vestibular disorders are usually of small magnitude (39; 18) and may be evident only with a Maddox rod testing rather than with alternate cover testing (27). Consequently, some patients with labyrinthine infarction, including those with isolated labyrinthine infarction and those with a skew deviation, might not be separately identified with the HINTS plus approach (95). Furthermore, when used in isolation by emergency physicians, the HINTS examination has not been shown to be sufficiently accurate to rule out a stroke (including labyrinthine infarction) in those presenting with an acute vestibular syndrome (96).
Prognosis and complications
The overall prognosis depends on the underlying etiology. In isolated labyrinthine infarction, vertigo, nystagmus, and autonomic manifestations resolve over days to weeks, but deafness and canal paresis typically remain (83; 43; 118; 36; 51). If no brainstem symptoms develop and brain imaging is normal, the risk of recurrence or subsequent stroke is rare (83; 51). Late effects can include benign paroxysmal positioning vertigo, especially with infarction in the territory of the anterior vestibular artery (77). In this situation, ischemia of the utricle results in displacement of otoconia from the macula, which may then lodge in the still functioning posterior semicircular duct, producing the symptoms of positioning nystagmus (62). Patients with labyrinthine ischemia due to vertebrobasilar insufficiency can have an overall good prognosis with anticoagulation or antiplatelet therapy (22). Rarely, surgical correction of a rotational vertebral artery syndrome is necessary (112). However, patients with labyrinthine infarction combined with brainstem or cerebellar infarcts have a worse prognosis (25), particularly if associated with occlusive disease of the basilar artery (20; 35).
Clinical vignette
Case 1. Labyrinthine infarction with combined audiovestibulopathy and pathologic confirmation at autopsy (51).
An 84-year-old woman developed acute vertigo with nausea and vomiting, followed by a brief syncopal spell as well as subsequent right-sided deafness, left-beating spontaneous nystagmus, and profound imbalance with a tendency to fall to the right (51). Electronystagmography showed absent caloric responses on the right. MRI of the brain and an echocardiogram were normal. The vertigo, nystagmus, and autonomic symptoms resolved over several days, although she continued to have profound right-sided hearing loss, poor balance, and nonspecific dizziness. She was treated with aspirin. Six years later, an audiogram showed profound sensorineural hearing loss on the right and age-appropriate mild sensorineural hearing loss on the left. The patient died of myocardial infarction 7 years after the onset of her symptoms. At autopsy, there was no evidence of brainstem or cerebellar infarction. Also, despite some atherosclerotic changes in the posterior circulation, the vertebrobasilar system, right anterior inferior cerebellar artery, and right internal auditory artery were patent. There were marked degenerative changes in the right cochlea, utricular macule, and cristae of the anterior and horizontal semicircular ducts and less severe changes in the crista of the posterior duct and the saccular macule. In the cochlea, there was complete destruction of the organ of Corti in the basal portion and loss of hair cells in the apex without evident fibrosis or ossification. In the vestibular labyrinth, the hair cells and underlying nerve fibers were destroyed in the anterior and horizontal duct cristae but were preserved in some areas of the posterior duct crista. There was mild fibrosis, but no ossification, of the vestibular labyrinth. Patchy degeneration was evident in the right vestibulocochlear nerve. Additionally, a marked loss of Scarpa ganglion cells with degenerative changes was found in remaining cells. These findings demonstrated the residua of labyrinthine infarction, likely resulting from a transient period of decreased perfusion in the internal auditory artery.
Case 2. Labyrinthine infarction with combined audiovestibulopathy from cardiac septic emboli due to bacterial endocarditis (78).
A previously healthy 42-year-old woman with fever, headache, and pharyngitis for 3 days developed sudden bilateral visual loss and dysarthria and then became confused and agitated, requiring sedation. Examination showed tachycardia (142 beats/minute), fever (39.6°C), nuchal rigidity, and tender maculopapular rashes in both hands and feet consistent with Janeway lesions. Fundus examination showed multiple scattered retinal hemorrhages with a white center in both eyes consistent with Roth spots. Hematologic assays showed leukocytosis (12,700/μL with 94% neutrophils), mild anemia (hemoglobin 9.6 g/dL; hematocrit 29.9%), and mild thrombocythemia (135,000/μL). CSF studies showed a marked neutrophilic pleocytosis (1,020/mm3 with 80% neutrophils), elevated RBC count (200/mm3), elevated CSF protein (166.0 mg/dL), and a normal CSF glucose but with a low CSF/serum glucose ratio (55 mg/dL with corresponding serum glucose 122 mg/dL, with a CSF/serum glucose ratio of 0.45, whereas the normal ratio is 0.6). Because of suspected bacterial meningitis, the patient was started on vancomycin (2.6 g per day) and ceftriaxone (4 g per day). Blood cultures later grew methicillin-resistant Staphylococcus aureus. Transthoracic echocardiography showed a hypermobile vegetation attached to the mitral valve, which precipitated mitral valve replacement 20 days after the onset of symptoms.
When the patient regained consciousness 1 day after her heart surgery, she complained of dizziness, hearing loss, and tinnitus in the right ear. Examination showed no spontaneous nystagmus with or without visual fixation, but horizontal head-shaking induced a left-beating nystagmus. Horizontal saccades and smooth pursuit were normal. She fell to the right when she attempted to stand or walk. The remainder of her neurologic examination was unrevealing. She had no weakness, sensory changes, or cerebellar dysfunction.
Video head-impulse tests were abnormal for the right horizontal and posterior canals. Bithermal caloric tests showed a right canal paresis of 81%. Pure-tone audiometry showed some residual hearing in the lower frequencies in the right ear 250 to 500 Hz but complete deafness at or above 1000 Hz in the right ear. Measurements of ocular torsion and subjective visual vertical were normal. Cervical and ocular vestibular-evoked myogenic potentials (VEMPs) showed no responses during right ear stimulation. Brain MRI with gadolinium showed contrast enhancement of the vestibule, cochlea, and semicircular canals on the right side as well as multiple scattered infarctions in the frontal and occipital lobes. MR angiography was normal.
Her clinical history, neuro-otologic and general neurologic examinations, and the results of diagnostic testing were consistent with multiple infarctions due to endocarditis with septic emboli from a cardiac vegetation.
Embolic labyrinthine infarction was diagnosed based on the occurrence of acute audiovestibular symptoms consistent with labyrinthine infarction in the presence of a cardiac source of embolism, no concurrent AICA infarction involving the brainstem or cerebellum, and concurrent embolic infarctions observed in the non-AICA territories.
She was discharged after 6 weeks of intravenous vancomycin and anticoagulation. She had minimal residual dizziness, but her hearing loss and tinnitus remained unchanged.
Case 3. Labyrinthine infarction with isolated vestibulopathy in a patient with a patent foramen ovale (78).
A 73-year-old man with a known patent foramen ovale developed acute vertigo with nausea and vomiting on his way to the bathroom on awakening. He denied associated aural fullness, tinnitus, hearing loss, or sensorimotor symptoms. The vertigo lasted into the afternoon with associated severe imbalance, but it subsided after symptomatic medication was administered at a local hospital for a presumed diagnosis of “vestibular neuritis.”
When evaluated at an otolaryngology unit at a tertiary medical center 6 days after symptom onset, he showed spontaneous nystagmus beating leftward and upward without fixation. Bedside head impulse tests were abnormal for the right horizontal canal. Rightward ocular torsion was observed on fundus photos. Measurements showed that the subjective visual vertical was significantly tilted to the right on both monocular and binocular viewing. Bithermal caloric tests showed complete right canal paralysis. Ocular VEMPs showed no responses during right ear stimulation, but findings of cervical VEMPs were symmetric. Audiometry was normal. Brain MRI disclosed a tiny acute infarction in the left parahippocampal gyrus (posterior cerebral artery territory). The findings of MR angiography were normal.
-
Symmetric sloping high-frequency sensorineural hearing loss (pure-tone audiometry)
Pure-tone audiometry shows symmetric responses between the ears . There is evidence of bilateral symmetric sloping high-frequency sensorineural hearing loss. Lt = left; Rt = right; O= air conduction, unmasked, right ear; < =...
Embolic labyrinthine infarction was diagnosed based on the occurrence of acute audiovestibular symptoms consistent with labyrinthine infarction in the presence of a cardiac source of embolism, no concurrent AICA infarction involving the brainstem or cerebellum, and concurrent embolic infarctions observed in the non-AICA territories.
Biological basis
|
• Labyrinthine infarction results from ischemia localized to the peripheral labyrinth, although there may be concomitant infarction of the brainstem and cerebellum. | |
|
• The blood supply to the inner ear flows through only one main blood vessel, the internal auditory artery (or labyrinthine artery), which typically originates from the anterior inferior cerebellar artery. | |
|
• Venous drainage of the labyrinth is via the vein of the cochlear aqueduct for the cochlea and saccule. | |
|
• The anterior inferior cerebellar artery, a branch of the basilar artery, supplies the lateral pons, the middle cerebellar peduncle, the flocculus, the anterior part of the cerebellar lobules, and the inner ear. |
Etiology and pathogenesis
Labyrinthine infarction results from ischemia localized to the peripheral labyrinth, although there may be concomitant infarction of the brainstem and cerebellum. In addition, labyrinthine infarction may be incomplete, with partial sparing of the inferior vestibular labyrinth because of better collateral blood flow to this portion of the labyrinth (51). Sudden bilateral hearing impairment suggests vertebrobasilar occlusive disease (110; 35; 15; 72; 08).
The blood supply to the inner ear flows through only one main blood vessel, the internal auditory artery (or labyrinthine artery), which typically originates from the anterior inferior cerebellar artery (AICA), but sometimes originates directly from the vertebral or basilar artery and uncommonly from the posterior inferior cerebellar artery (PICA) (06; 80; 81; 30; 16). The internal auditory artery divides into two main branches within the internal auditory canal: (1) the anterior vestibular artery and (2) the common cochlear artery (06; 81; 30). The anterior vestibular artery supplies the anterior superior vestibule, including the utricle with its macula, the superior part of the saccule, and the ampullae and cristae of the anterior and horizontal semicircular ducts (06; 81; 30). The common cochlear artery divides into the main cochlear artery and the vestibulocochlear artery, which together supply the cochlea (30). In addition, a posterior vestibular branch of the vestibulocochlear artery supplies the posterior vestibule, including the inferior saccule along with its macula, and the ampulla and crista of the posterior semicircular duct (06; 81; 30). The internal auditory artery and its branches are terminal or end arteries, so even transient ischemia can cause permanent inner ear damage (16). The organ of Corti is particularly sensitive to ischemia (106), especially at its apical region, so low-frequency hearing loss is common with inner ear ischemia (71). Inner ear ischemia should be suspected when unilateral posterior canal hypofunction occurs in combination with unilateral sensorineural hearing loss on the same side because both receptor organs are supplied by the common cochlear artery (vestibulocochlear artery) (99; 16).
Venous drainage of the labyrinth is via the vein of the cochlear aqueduct for the cochlea and saccule. The vein of the vestibular aqueduct is utilized by the utricle and semicircular canals (06; 81). The vein of the cochlear aqueduct empties into the bulb of the jugular vein, whereas the vein of the vestibular aqueduct drains into the superior petrosal sinus (06; 81). Labyrinthine ischemia attributed to impaired venous drainage most commonly results from hyperviscosity syndromes (87; 101; 92; 05; 103). Increased blood viscosity produces obstruction in the labyrinthine venules and capillaries with decreased blood flow and inner ear ischemia as well as subsequent hemorrhage and, later, fibrosis and ossification (52; 53). Similar changes in the eye produce visual disturbances, markedly distended and tortuous (“sausage-shaped”) retinal veins, and retinal hemorrhages.
The anterior inferior cerebellar artery, a branch of the basilar artery, supplies the lateral pons, the middle cerebellar peduncle, the flocculus, the anterior part of the cerebellar lobules, and the inner ear (03). The course of the anterior inferior cerebellar artery is somewhat variable (108). In patients with anterior inferior cerebellar artery territory infarction, the most consistently involved areas were the lateral pons and the middle cerebellar peduncle (03; 100). Because of the sharp angulation of the anterior inferior cerebellar artery at its origin, it is rarely occluded by emboli (118); rather, most occlusions are due to either basilar artery plaques extending into the anterior inferior cerebellar artery or microatheroma of the anterior inferior cerebellar artery origin (04). Whether isolated or in combination with other symptoms and signs, vertigo can occur in anterior inferior cerebellar artery-distribution infarctions due to involvement of several central and peripheral sites, including the labyrinth, the eighth nerve, the vestibular nuclei, the vestibulocerebellum, or some combination of each. However, isolated vertigo from anterior inferior cerebellar artery-territory infarctions is rare (04). Labyrinthine infarction, in conjunction with anterior inferior cerebellar artery territory infarction, has been anticipated based on animal studies (55), supported by clinical studies (94), and reported on in histologic studies (32). The limited histopathologic data are controversial (31).
Damage to vestibular end organs during ischemia is mediated in part by glutamate (107) and reactive oxygen species (38). Morphological changes induced by glutamate cytotoxicity are evident in the bouton-type endings and the nerve chalices (formed by vestibular nerve branches that surround type I hair cells) by 30 minutes of ischemia in animal models (107).
Epidemiology
The condition is uncommon but probably under-recognized Most reports are individual case reports or small case series.
Prevention
No specific information is available. Presumably, the risk factors are the same as general stroke.
Differential diagnosis
Confusing conditions
Most commonly, labyrinthine infarction occurs in the setting of thromboembolic disease of the anterior inferior cerebellar or the basilar artery (29; 32; 94; 04; 35; 51; 116; 112; 72; 70; 73; 65; 66; 109). As a result of such lesions, there is often mixed central and peripheral vestibular dysfunction, making recognition of the components difficult (69; 64). Without associated central nervous system signs, it may be difficult to recognize the ischemic basis of the clinical presentation. Indeed, if neurologic symptoms are absent, it is more likely that viral labyrinthitis is the cause of acute audiovestibular symptoms, especially in the absence of abnormal findings on imaging (70; 49). Nevertheless, labyrinthine infarction may cause acute unilateral peripheral vestibulopathy that mimics vestibular neuritis, and concomitant labyrinthine infarction should be suspected in patients with acute vestibular syndrome and acute infarction in the territory of the anterior inferior cerebellar artery (89). Other considerations may include inflammatory, viral, and other infectious and traumatic causes of labyrinthine dysfunction. Unlike labyrinthine dysfunction from a viral cause, the most common pattern of dysfunction with labyrinthine infarction includes a combined loss of auditory and vestibular function (68). Although acute unilateral hearing loss and hypofunction of the vestibulo-ocular reflex typically indicate labyrinthine dysfunction, cerebellar findings should not be overlooked, including direction-changing positional nystagmus, which suggests dysfunction of the posterior cerebellar vermis (109). Small infarcts of the brainstem or cerebellum can produce vertigo without other localizing neurologic symptoms: approximately 11% of the patients with an isolated cerebellar infarction present with vertigo, nystagmus, and postural unsteadiness mimicking acute peripheral vestibular disorders (69; 64; 76).
Associated or underlying disorders
A variety of conditions can cause labyrinthine ischemia, including thromboemboli of the posterior circulation (17; 66; 73; 71; 47; 99), dissection of the vertebral artery (19), fat emboli (37), thromboangiitis obliterans (56), migrainous infarction (71); Susac syndrome (113; 14); decompression illness, particularly in the presence of a right-to-left cardiac shunt (58), hyperlipidemia (103), macroglobulinemia (101; 92), sickle cell disease (87; 05), intravascular lymphomatosis (07), leukemia (05), polycythemia vera (05), and other causes of hypercoagulation or hyperviscosity (37; 05).
Diagnostic workup
|
• Diagnostic studies can include imaging of the brain and cerebral vessels, audiograms and brainstem auditory evoked potentials, electronystagmography or videonystagmography with bithermal caloric irrigation, and cervical vestibular-evoked myogenic potential studies. | |
|
• Audiovestibular tests do not readily distinguish ischemic and nonischemic causes of combined vertigo and sudden sensorineural hearing loss. | |
|
• Audiograms demonstrate sensorineural hearing loss that may be profound. | |
|
• Brain imaging is important to exclude brainstem or cerebellar lesions, but, unfortunately, current imaging techniques are inadequate to allow confident identification of isolated labyrinthine infarctions as a cause of acute audiovestibulopathy. | |
|
• In cases of clinically suspected labyrinthine ischemia resulting from hyperviscosity, the following blood studies can be considered: serum viscosity determination, complete blood count, syphilis serologies, sedimentation rate, serum protein, serum protein electrophoresis, and lipid studies. |
Diagnostic studies can include imaging of the brain and cerebral vessels, audiograms and brainstem auditory evoked potentials, electronystagmography or videonystagmography with bithermal caloric irrigation, and cervical vestibular-evoked myogenic potential studies. Audiovestibular tests do not readily distinguish ischemic and non-ischemic causes of combined vertigo and sudden sensorineural hearing loss (99).
Audiograms demonstrate sensorineural hearing loss that may be profound. Brainstem auditory evoked potentials may show an absence of wave 1 or all waveforms, but they may also show an absence of wave 1 with delay of waves 3 and 5 if there is also dysfunction in the retrocochlear eighth nerve and brainstem auditory nuclei and pathways (116).
Electronystagmography or videonystagmography with bithermal caloric testing demonstrates ipsilateral horizontal canal paresis. Canal paresis associated with posterior circulation ischemic stroke, usually within the territory of the anterior inferior cerebellar artery, typically normalizes within 5 years (74).
Brain imaging is important to exclude brainstem or cerebellar lesions and occasionally may be supportive of labyrinthine infarction, especially with delayed contrast (86); but unfortunately, current imaging techniques are inadequate to allow confident identification of isolated labyrinthine infarctions as a cause of acute audiovestibulopathy (13; 78; 16). In particular, MRI does not generally visualize the inner ear well enough to identify infarction. Conventional or MR angiography in cases of sudden bilateral deafness may demonstrate stenosis of the distal vertebral arteries and proximal basilar artery (72).
In cases of clinically suspected labyrinthine ischemia resulting from hyperviscosity, the following blood studies can be considered: serum viscosity determination, complete blood count, syphilis serologies, sedimentation rate, serum protein, serum protein electrophoresis, and lipid studies.
Management
|
• Acute vertigo and concomitant nausea and vomiting can be suppressed with a variety of medications. | |
|
• Vestibular rehabilitation should be begun early. |
Management depends on the underlying etiology. Acute vertigo and concomitant nausea and vomiting can be suppressed with a variety of medications (60). Vestibular rehabilitation should be begun early (60). Anticoagulation can effectively stop attacks of episodic vertigo due to vertebrobasilar insufficiency; this treatment is particularly effective in patients with recurrent symptoms, despite antiplatelet therapy (22).
Media
References
- 01
- Ahn BH, Kim HA, Yi HA, Oh SY, Lee H. Abnormal cervical vestibular-evoked myogenic potential in anterior inferior cerebellar artery territory infarction: frequency, pattern, and a determinant. J Neurol Sci 2011;307(1-2):114-9. PMID 21571323
- 02
- Akdal G, Halmagyi GM. Sudden vertigo in a 49-year-old man. J Clin Neurosci 2012;19(12):1699. PMID 23281553
- 03
- Amarenco P, Hauw JJ. Cerebellar infarction in the territory of the anterior and inferior cerebellar artery: a clinicopathologic study of 20 cases. Brain 1990;113:139-55. PMID 2302529
- 04
- Amarenco P, Rosengart A, DeWitt LD, Pessin MS, Caplan LR. Anterior inferior cerebellar artery territory infarcts: mechanisms and clinical features. Arch Neurol 1993;50:154-61. PMID 8431134
- 05
- Andrews JC, Hoover LA, Lee RS, Honrubia V. Vertigo in the hyperviscosity syndrome. Otolaryngol Head Neck Surg 1988;98:144-9. PMID 3128757
- 06
- Axelsson A. The vascular anatomy of the cochlea in the guinea pig and in man. Acta Otolaryngologica 1968;(Suppl)243:1-134. PMID 5754509
- 07
- Berger JR, Jones R, Wilson D. Intravascular lymphomatosis presenting with sudden hearing loss. J Neurol Sci 2005;232:105-9. PMID 15850590
- 08
- Bovo R, Ortore R, Ciorba A, Berto A, Martini A. Bilateral sudden profound hearing loss and vertigo as a unique manifestation of bilateral symmetric inferior pontine infarctions. Ann Otol Rhinol Laryngol 2007;116(6):407-10. PMID 17672241
- 09
- Carmona S, Martínez C, Zalazar G, et al. The diagnostic accuracy of truncal ataxia and HINTS as cardinal signs for acute vestibular syndrome. Front Neurol 2016;7:125. PMID 27551274
- 10
- Chen L, Lee W, Chambers BR, Dewey HM. Diagnostic accuracy of acute vestibular syndrome at the bedside in a stroke unit. J Neurol 2011;258(5):855-61. PMID 21153732
- 11
- Chen L, Todd M, Halmagyi GM, Aw S. Head impulse gain and saccade analysis in pontine-cerebellar stroke and vestibular neuritis. Neurology 2014;83(17):1513-22. PMID 25253747
- 12
- Choi KD, Chun JU, Han MG, Park SH, Kim JS. Embolic internal auditory artery infarction from vertebral artery dissection. J Neurol Sci 2006;246:169-72. PMID 16580695
- 13
- Choi SY, Park JH, Kim HJ, Kim JS. Vestibulocochlear nerve infarction documented with diffusion-weighted MRI. J Neurol 2015;262:1363-5. PMID 25808505
- 14
- Cohen DA, Tajfirouz D, Vodopivec I, et al. Fluorescein angiography findings in Susac syndrome: a multicenter retrospective case series. J Neuroophthalmol 2023;43(4):481-90. PMID 37075250
- 15
- Deschauer M, Georgiadis D, Lindner A. Hearing loss as the leading symptom in anterior inferior cerebellar artery infarction. Fortschr Neurol Psychiatr 1998;66:109-12. PMID 9565760
- 16
- Dlugaiczyk J. Rare disorders of the vestibular labyrinth: of zebras, chameleons and wolves in sheep's clothing. Laryngorhinootologie 2021;100(S01):S1-40. PMID 34352900
- 17
- Dziewas R, Schilling M, Fallenberg EM, Allroggen A, Ludemann P, Henningsen H. [The megadolichobasilar artery – a clinical chameleon.] Nervenarzt 2003;74:172-4. PMID 12596019
- 18
- Eggers SDZ, Kattah JC. Approaching acute vertigo with diplopia: a rare skew deviation in vestibular neuritis. Mayo Clin Proc Innov Qual Outcomes 2020;4(2):216-22.** PMID 32280933
- 19
- Eliezer M, Verillaud B, Guichard JP, et al. Labyrinthine infarction caused by vertebral artery dissection: consideration based on MRI. J Neurol 2019;266(10):2575-7. PMID 31267205
- 20
- Ferbert A, Bruckman H, Drummen R. Clinical features of proven basilar artery occlusion. Stroke 1990;21:1135-42. PMID 2389292
- 21
- Fernandez C. The effect of oxygen lack on cochlear potentials. Ann Otol Rhinol Laryngol 1955;64:1193-203. PMID 13283493
- 22
- Fife TD, Baloh RW, Duckwiler GR. Isolated dizziness in vertebrobasilar insufficiency: clinical features, angiography, and follow-up. J Stroke Cerebrovasc Dis 1994;4(1):4-12. PMID 26487528
- 23
- Fisher CM. Vertigo in cerebrovascular disease. Arch Otolaryngol 1967;85:529-34. PMID 6023709
- 24
- Francis DA, Bronstein AM, Rudge P, du Boulay EP. The site of brainstem lesions causing semicircular canal paresis: an MRI study. J Neurol Neurosurg Psychiatry 1992;55:446-9. PMID 1619409
- 25
- Gomez CR, Cruz-Flores S, Malkoff MD, Sauer CM, Burch CM. Isolated vertigo as a manifestation of vertebrobasilar ischemia. Neurology 1996;47:94-7. PMID 8710132
- 26
- Grad A, Baloh RW. Vertigo of vascular origin: clinical and electronystagmographic features in 84 cases. Arch Neurol 1989;46:281-4. PMID 2919982
- 27
- Green KE, Gold DR. HINTS examination in acute vestibular neuritis: do not look too hard for the skew. J Neuroophthalmol 2021;41(4):e672-8. PMID 32701756
- 28
- Guiang RL, Ellington OB. Acute pure vertiginous disequilibrium in cerebellar infarction. Eur Neurol 1977;16:11-5. PMID 615701
- 29
- Gussen R. Sudden deafness of vascular origin: a human temporal bone study. Ann Otol 1976;85:94-100. PMID 1259320
- 30
- Haidara A, Peltier J, Zunon-Kipre Y, N'da HA, Drogba L, Gars DL. Microsurgical anatomy of the labyrinthine artery and clinical relevance. Turk Neurosurg 2015;25(4):539-43. PMID 26242329
- 31
- Harker LA. Associate editor's comments. Ann Otol Rhinol Laryngol 1990;99:271-2.
- 32
- Hinojosa R, Kohut RI. Clinical diagnosis of anterior inferior cerebellar artery thrombosis. Autopsy and temporal bone histopathologic study. Ann Otol Rhinol Laryngol 1990;99:261-72. PMID 2327695
- 33
- Hopf HC. Vertigo and masseter paresis: a new local brainstem syndrome probably of vascular origin. J Neurol 1987;235:42-5. PMID 3430182
- 34
- Huang CY, Yu YL. Small cerebellar strokes may mimic labyrinthine lesions. J Neurol Neurosurg Psychiatry 1985;48:263-5. PMID 3981196
- 35
- Huang MH, Huang CC, Ryu SJ, Chu NS. Sudden bilateral hearing impairment in vertebrobasilar occlusive disease. Stroke 1993;24:132-7. PMID 8418537
- 36
- Izumi M, Terao S, Sobue G, et al. Clinical features of anterior inferior cerebellar artery territory infarcts: a study of ten patients. No To Shinkei 1996;49:152-6. PMID 9046527
- 37
- Jaffe BF. Sudden deafness: a local manifestation of systemic disorders: fat emboli, hypercoagulation and infections. Laryngoscope 1970;80:788-801. PMID 5452517
- 38
- Kang HS, Park JJ, Ahn SK, Hur DG, Kim HY. Effect of high dose intravenous vitamin C on idiopathic sudden sensorineural hearing loss: a prospective single-blind randomized controlled trial. Eur Arch Otorhinolaryngol 2013;270(10):2631-6. PMID 23208525
- 39
- Kattah JC. Use of HINTS in the acute vestibular syndrome. An overview. Stroke Vasc Neurol 2018;3(4):190-6.** PMID 30637123
- 40
- Kattah JC. Update on HINTS Plus, with discussion of pitfalls and pearls. J Neurol Phys Ther 2019;43 Suppl 2:S42-5.** PMID 30883493
- 41
- Kattah JC, Talkad AV, Wang DZ, Hsieh YH, Newman-Toker DE. HINTS to diagnose stroke in the acute vestibular syndrome: three-step bedside oculomotor examination more sensitive than early MRI diffusion-weighted imaging. Stroke 2009;40(11):3504-10.** PMID 19762709
- 42
- Khurana D, Sreekanth VR, Prabhakar S. A case of emotional facial palsy with ipsilateral anterior inferior cerebellar artery territory infarction. Neurol India 2002;50:102-4. PMID 11960165
- 43
- Kido T, Sekitani T, Okinaka Y, Tahara T, Hara H. A case of cerebellar infarction occurred with the 8th cranial nerve symptoms. Auris Nasus Larynx 1994;21:111-7. PMID 7993224
- 44
- Kim JS, Lee H. Inner ear dysfunction due to vertebrobasilar ischemic stroke. Semin Neurol 2009;29(5):534-40. PMID 19834865
- 45
- Kim HA, Lee H. Recent advances in understanding audiovestibular loss of a vascular cause. J Stroke 2017;19(1):61-6. PMID 28030893
- 46
- Kim DR, Lee HJ, Kim HJ, Hong SK. Dynamic changes in the inner ear function and vestibular neural pathway related to the progression of labyrinthine infarction in patient with an anterior inferior cerebellar artery infarction. Otol Neurotol 2011;32(9):1596-9. PMID 22064670
- 47
- Kim HA, Lee SR, Lee H. Acute peripheral vestibular syndrome of a vascular cause. J Neurol Sci 2007;254(1-2):99-101. PMID 17257625
- 48
- Kim HA, Yi HA, Lee H. Recent advances in cerebellar ischemic stroke syndromes causing vertigo and hearing loss. Cerebellum 2016;15(6):781-8. PMID 26573627
- 49
- Kim JH, Choi H, Kang JW. Audiovestibular impairment presenting as a portent of vertebrobasilar infarction. Br J Hosp Med (Lond) 2014;75(1):48-9. PMID 24401972
- 50
- Kim JS, Cho KH, Lee H. Isolated labyrinthine infarction as a harbinger of anterior inferior cerebellar artery territory infarction with normal diffusion-weighted brain MRI. J Neurol Sci 2009;278:82-4. PMID 19135217
- 51
- Kim JS, Lopez I, DiPatre PL, et al. Internal auditory artery infarction: clinicopathologic correlation. Neurology 1999;52:40-4. PMID 9921846
- 52
- Kimura R, Perlman HB. Extensive venous obstruction of the labyrinth. A. Cochlear changes. Ann Otol Rhinol Laryngol 1956a;61:332-51. PMID 13355160
- 53
- Kimura R, Perlman HB. Extensive venous obstruction of the labyrinth. B. Vestibular changes. Ann Otol Rhinol Laryngol 1956b;61:332-51. PMID 13363222
- 54
- Kimura R, Perlman HB. Arterial obstruction of the labyrinth: part I. Cochlear changes. Ann Otol Rhinol Laryngol 1958a;67:5-24. PMID 13521620
- 55
- Kimura R, Perlman HB. Arterial obstruction of the labyrinth: part II. Vestibular changes. Ann Otol Rhinol Laryngol 1958b;67:25-40. PMID 13521621
- 56
- Kirikae I, Nomura Y, Shitara T, Kobayashi T. Sudden deafness due to Buerger’s disease. Arch Otolaryngol 1962;75:502-5. PMID 14456277
- 57
- Kitamura K, Berreby M. Temporal bone histopathology associated with occlusion of vertebrobasilar arteries. Ann Otol Rhinol Laryngol 1983;92:33-8. PMID 6824277
- 58
- Klingmann C, Benton PJ, Ringleb PA, Knauth M. Embolic inner ear decompression illness: correlation with a right-to-left shunt. Laryngoscope 2003;113(8):1356-61. PMID 12897559
- 59
- Korda A, Zamaro E, Wagner F, et al. Acute vestibular syndrome: is skew deviation a central sign? J Neurol 2022;269(3):1396-403. PMID 34244842
- 60
- Lanska DJ. Vertigo and other forms of dizziness. In: Corey-Bloom J, editor. Adult neurology. St. Louis: Mosby-Year Book Inc., 1998:89-105, 409.
- 61
- Lanska DJ, Goetz CG. Romberg's sign: description, adoption, and adaptation in the 19th century. Neurology 2000;55(8):1201-6. PMID 11071500
- 62
- Lanska DJ, Remler B. Benign paroxysmal positioning vertigo: classic descriptions, origins of the provocative positioning technique, and conceptual developments. Neurology 1997;48:1167-77. PMID 9153438
- 63
- Lee H. Sudden deafness related to posterior circulation infarction in the territory of the nonanterior inferior cerebellar artery: frequency, origin, and vascular topographical pattern. Eur Neurol 2008;59(6):302-6. PMID 18408371
- 64
- Lee H. Isolated vascular vertigo. J Stroke 2014;16(3):124-30. PMID 25328871
- 65
- Lee H, Ahn BH, Baloh RW. Sudden deafness with vertigo as a sole manifestation of anterior inferior cerebellar artery infarction. J Neurol Sci 2004;222(1-2):105-7. PMID 15240204
- 66
- Lee H, Cho YW. Auditory disturbance as a prodrome of anterior inferior cerebellar artery infarction. J Neurol Neurosurg Psychiatry 2003;74(12):1644-8. PMID 14638883
- 67
- Lee H, Kim HJ, Koo JW, Kim JS. Progression of acute cochleovestibulopathy into anterior inferior cerebellar artery infarction. J Neurol Sci 2009b;278:119-22. PMID 19108852
- 68
- Lee H, Kim JS, Chung EJ, et al. Infarction in the territory of anterior inferior cerebellar artery: spectrum of audiovestibular loss. Stroke 2009a;40(12):3745-51. PMID 19797177
- 69
- Lee H, Sohn SI, Cho YW, Lee SR, et al. Cerebellar infarction presenting isolated vertigo: frequency and vascular topographical patterns. Neurology 2006;67(7):1178-83. PMID 17030749
- 70
- Lee H, Sohn SI, Jung DK, et al. Sudden deafness and anterior inferior cerebellar artery infarction. Stroke 2002;33(12):2807-12. PMID 12468774
- 71
- Lee H, Whitman GT, Lim JG, et al. Hearing symptoms in migrainous infarction. Arch Neurol 2003b;60(1):113-6. PMID 12533097
- 72
- Lee H, Whitman GT, Lim JG, Lee SD, Park YC. Bilateral sudden deafness as a prodrome of anterior inferior cerebellar artery infarction. Arch Neurol 2001;58:1287-9. PMID 11493170
- 73
- Lee H, Yi HA, Baloh RW. Sudden bilateral simultaneous deafness with vertigo as a sole manifestation of vertebrobasilar insufficiency. J Neurol Neurosurg Psychiatry 2003a;74(4):539-41. PMID 12640087
- 74
- Lee H, Yi HA, Chung IS, Lee SR. Long-term outcome of canal paresis of a vascular cause. J Neurol Neurosurg Psychiatry 2011;82(1):105-9. PMID 20587486
- 75
- Lee H, Yi HA, Lee SR, Lee SY, Park BR. Ocular torsion associated with infarction in the territory of the anterior inferior cerebellar artery: frequency, pattern, and a major determinant. J Neurol Sci 2008;269:18-23. PMID 18191152
- 76
- Lee SH, Kim JS. Acute diagnosis and management of stroke presenting dizziness or vertigo. Neurol Clin 2015;33(3):687-98. PMID 26231280
- 77
- Lindsay JR, Hemenway WG. Postural vertigo due to unilateral sudden partial loss of vestibular function. Ann Otol Rhinol Laryngol 1956;65:696-706. PMID 13363229
- 78
- Liqun Z, Park KH, Kim HJ, Lee SU, Choi JY, Kim JS. Acute unilateral audiovestibulopathy due to embolic labyrinthine infarction. Front Neurol 2018;9:311. PMID 29770122
- 79
- Martines F, Dispenza F, Gagliardo C, Martines E, Bentivegna D. Sudden sensorineural hearing loss as prodromal symptom of anterior inferior cerebellar artery infarction. ORL J Otorhinolaryngol Relat Spec 2011;73(3):137-40. PMID 21494055
- 80
- Mazzoni A. Internal auditory canal arterial relations at the porus acusticus. Ann Otol Rhinol Laryngol 1969;78:797-814. PMID 5799404
- 81
- Mazzoni A. The vascular anatomy of the vestibular labyrinth in man. Acta Otolaryngologica 1990;(Suppl)472:1-83. PMID 2239254
- 82
- Millikan CH. Neurology: its role in ophthalmology and otolaryngology. Ophthalmology 1964;68:208-17. PMID 14130928
- 83
- Millikan CH, Futrell N. Occlusion of the internal auditory artery. Ann Neurol 1990a;28:258.
- 84
- Millikan CH, Futrell N. Vertigo of vascular origin. Arch Neurol 1990b;47:12-3. PMID 2294887
- 85
- Millikan CH, Siekert RG, Whisnant JP. The syndrome of occlusion of the labyrinthine division of the internal auditory artery. Trans Am Neurol Assoc 1959;84:11-3. PMID 14422630
- 86
- Monak AA, Kaileva NA, Kulesh AA, Guseva AL, Usmanov VB, Parfenov VA. Labyrinthine infarction as a cause of acute cochleovestibular syndrome [in Russian]. Neurology, Neuropsychiatry, Psychosomatics 2023;15(1):71-6.
- 87
- Morgenstein KM, Manace ED. Temporal bone histopathology in sickle cell disease. Laryngoscope 1969;79(12):2172-80. PMID 5362685
- 88
- Nagaratnam N, Mak J, Phan TA, Kalouche H. Sudden permanent hearing loss following anterior inferior cerebellar artery infarction. Int J Clin Pract 2002;56:153-4. PMID 11926705
- 89
- Nam HW, Yoo D, Lee SU, Choi JY, Yu S, Kim JS. Pearls & oy-sters: labyrinthine infarction mimicking vestibular neuritis. Neurology 2021;97(16):787-90. PMID 34045269
- 90
- Newman-Toker DE, Curthoys IS, Halmagyi GM. Diagnosing stroke in acute vertigo: the HINTS family of eye movement tests and the future of the "eye ECG." Semin Neurol 2015;35(5):506-21. PMID 26444396
- 91
- Newman-Toker DE, Kerber KA, Hsieh YH, et al. HINTS outperforms ABCD2 to screen for stroke in acute continuous vertigo and dizziness. Acad Emerg Med 2013;20(10):986-96. PMID 24127701
- 92
- Nomura Y, Mori S, Tsuchida M, Sakurai T. Deafness in cryoglobulinemia. Ann Otol Rhinol Laryngol 1982;91:250-5. PMID 7092044
- 93
- Norrving B, Magnusson M, Holtas S. Isolated acute vertigo in the elderly: vestibular or vascular disease. Acta Neurol Scand 1995;91:43-8. PMID 7732773
- 94
- Oas JG, Baloh RW. Vertigo and the anterior inferior cerebellar artery syndrome. Neurology 1992;42:2274-9. PMID 1461378
- 95
- Ogawa K, Suzuki Y, Takahashi K, Akimoto T, Kamei S, Soma M. Clinical study of seven patients with infarction in territories of the anterior inferior cerebellar artery. J Stroke Cerebrovasc Dis 2017;26(3):574-81. PMID 27989483
- 96
- Ohle R, Montpellier RA, Marchadier V, et al. Can emergency physicians accurately rule out a central cause of vertigo using the HINTS examination? A systematic review and meta-analysis. Acad Emerg Med 2020;27(9):887-96. PMID 32167642
- 97
- Perlman HB, Fernandez C. Experiments on temporary obstruction of the internal auditory artery. Laryngoscope 1959;69:591-613. PMID 13673604
- 98
- Perlman HB, Kimura R. Experimental obstruction of the venous drainage and arterial supply of the inner ear. Ann Otol Rhinol Laryngol 1957;66:537-47. PMID 13459249
- 99
- Pogson JM, Taylor RL, Young AS, et al. Vertigo with sudden hearing loss: audio-vestibular characteristics. J Neurol 2016;263(10):2086-96. PMID 27435969
- 100
- Roquer J, Lorenzo JL, Pou A. The anterior inferior cerebellar artery infarcts: a clinical-magnetic resonance imaging study. Acta Neurol Scand 1998;97:225-30. PMID 9576636
- 101
- Ruben RJ, Distenfeld A, Berg P, Carr R. Sudden sequential deafness as the presenting symptom of macroglobulinemia. JAMA 1969;209:1364-5. PMID 4979452
- 102
- Rubenstein RL, Norman DM, Schindler RA, Kaseff L. Cerebellar infarction- a presentation of vertigo. Laryngoscope 1980;90:505-14. PMID 7359972
- 103
- Saadah HA. Vestibular vertigo associated with hyperlipidemia: response to antilipemic therapy. Arch Intern Med 1993;153:1846, 9. PMID 8333820
- 104
- Saber Tehrani AS, Kattah JC, Kerber KA, et al. Diagnosing stroke in acute dizziness and vertigo: pitfalls and pearls. Stroke 2018;49(3):788-95. PMID 29459396
- 105
- Saber Tehrani AS, Kattah JC, Mantokoudis G, et al. Small strokes causing severe vertigo: frequency of false-negative MRIs and nonlacunar mechanisms. Neurology 2014;83(2):169-73. PMID 24920847
- 106
- Sando I, Ogawa A, Jafek BW. Inner ear pathology following injury to the eighth cranial nerve and the labyrinthine artery. Ann Otol 1982;91:136-41. PMID 6979284
- 107
- Sasaki A, Matsubara A, Tabuchi K, Hara A, Namba A, Yamamoto Y, Shinkawa H. Immunoelectron microscopic analysis of neurotoxic effect of glutamate in the vestibular end organs during ischemia. Acta Otolaryngol 2012;132(7):686-92. PMID 22497451
- 108
- Scoleri P, Widner SA, Cass SP. An anatomic variant of the anterior inferior cerebellar artery in a patient with Meniere’s disease. Otol Neurotol 2001;22:519-25. PMID 11449111
- 109
- Shaikh AG, Miller BR, Sundararajan S, Katirji B. Gravity-dependent nystagmus and inner-ear dysfunction suggest anterior and posterior inferior cerebellar artery infarct. J Stroke Cerebrovasc Dis 2014;23(4):788-90. PMID 23800506
- 110
- Shimomura T, Suzuki T, Takahashi S. A case of brain stem infarction with bilateral hearing impairment and tinnitus at the onset. Rinsho Shinkeigaku 1990;30:1104-8. PMID 2279361
- 111
- Son EJ, Bang JH, Kang JG. Anterior inferior cerebellar artery infarction presenting with sudden hearing loss and vertigo. Laryngoscope 2007;117(3):556-8. PMID 17334322
- 112
- Strupp M, Planck JH, Arbusow V, et al. Rotational vertebral artery occlusion syndrome with vertigo due to “labyrinthine excitation.” Neurology 2000;54:1376-9. PMID 10746615
- 113
- Sveinsson O, Kolloch J, Träisk F, Brundin L. Susac syndrome - an unusual syndrome which can be mistaken for multiple sclerosis [Article in Swedish]. Lakartidningen 2020;117:FSSS. PMID 32045006
- 114
- Takamatsu K, Ohta T. A study of infarction in the region of the anterior inferior cerebellar artery. Rinsho Shinkeigaku 1995;35:621-5. PMID 8521637
- 115
- Venhovens J, Meulstee J, Verhagen WIM. Acute vestibular syndrome: a critical review and diagnostic algorithm concerning the clinical differentiation of peripheral versus central aetiologies in the emergency department. J Neurol 2016;263(11):2151-7. PMID 26984607
- 116
- Verghese J, Morocz IA. Acute unilateral deafness. J Otolaryngol 1999;28:362-4. PMID 10604169
- 117
- von Campe G, Regli F, Bogousslavsky J. Heralding manifestations of basilar artery occlusion with lethal or severe stroke. J Neurol Neurosurg Psychiatry 2003;74(12):1621-6. PMID 14638878
- 118
- Watanabe Y, Ohi H, Shojaku H, Mizukoshi K. Sudden deafness from vertebrobasilar artery disorder. Am J Otol 1994;15:423-6. PMID 8579154
- 119
- Williams D, Wilson TG. The diagnosis of the major and minor syndromes of basilar insufficiency. Brain 1962;85:741-71. PMID 14000840
Contributors
All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.
Author
-
Douglas J Lanska MD MS MSPH
Dr. Lanska of the University of Wisconsin School of Medicine and Public Health and the Medical College of Wisconsin has no relevant financial relationships to disclose.
See Profile
Patient Profile
- Age range of presentation
-
- 2 to 65+ years
- Sex preponderance
-
- male=female
- Heredity
-
- none
- Population groups selectively affected
-
- none selectively affected
- Occupation groups selectively affected
-
- none selectively affected
ICD & OMIM codes
- ICD-10
-
- Degenerative and vascular disorders of ear: H93.0
- Other and unspecified peripheral vertigo: H81.3
- Other specified diseases of inner ear: H83.8
- Sensorineural hearing loss, unspecified: H90.2
- ICD-11
-
- Other specified diseases of inner ear: AB3Y
- Other peripheral vertigo: AB34.1
- Acquired sensorineural hearing loss: AB51.1
- Degenerative or vascular disorders of ear: AB71
This is an article preview.
Start a Free Account
to access the full version.
-
Nearly 3,000 illustrations, including video clips of neurologic disorders.
-
Every article is reviewed by our esteemed Editorial Board for accuracy and currency.
-
Full spectrum of neurology in 1,200 comprehensive articles.
-
Listen to MedLink on the go with Audio versions of each article.
Questions or Comment?
MedLink®, LLC
3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122
Toll Free (U.S. + Canada): 800-452-2400
US Number: +1-619-640-4660
Support: service@medlink.com
Editor: editor@medlink.com
ISSN: 2831-9125
Also in This Category
-
-
Neuro-Ophthalmology & Neuro-Otology
Nystagmus
Nov. 22, 2024
-
Neuro-Ophthalmology & Neuro-Otology
Third nerve palsy
Nov. 22, 2024
-
Developmental Malformations
Von Hippel-Lindau disease
Sep. 28, 2024
-
Neuro-Ophthalmology & Neuro-Otology
Giant cell arteritis
Sep. 25, 2024
-
Neuro-Ophthalmology & Neuro-Otology
Ischemic optic neuropathy
Sep. 25, 2024
-
Neuro-Ophthalmology & Neuro-Otology
Transient visual loss
Sep. 25, 2024
-
Neuro-Oncology
Vestibular schwannoma
Sep. 19, 2024