Sudden deafness with vertigo as a sole manifestation of anterior inferior cerebellar artery infarction

Sudden deafness without associated neurological symptoms and signs is typically attributed to a viral inflammation of the labyrinth. Although sudden deafness occurs with anterior inferior cerebellar artery (AICA) infarction, the deafness is usually associated with other brainstem or cerebellum signs such as crossed sensory loss, lateral gaze palsy, facial palsy, Horner syndrome or cerebellar dysmetria. An 84-year-old woman suddenly developed right-sided tinnitus, hearing loss, vertigo and vomiting. Audiometry and electronystagmography documented absent auditory and vestibular function on the right side. T2-weighted and diffusion-weighted MRI showed a tiny infarct in the right lateral inferior pontine tegmentum. AICA occlusion can cause sudden deafness and vertigo without brainstem or cerebellar signs.     

Vertigo and the anterior inferior cerebellar artery syndrome.

We present two patients with clinical features of infarction in the distribution of the anterior inferior cerebellar artery (AICA) who had vertigo as an isolated symptom for several months prior to infarction. Both had risk factors for cerebrovascular disease and other episodes of transient neurologic symptoms not associated with vertigo. At the time of infarction they developed vertigo, unilateral hearing loss, tinnitus, facial numbness, and hemiataxia. MRI identified hyperintense lesions in the lateral pons and middle cerebellar peduncle on T2-weighted images. Audiometry and electronystagmography documented absent auditory and vestibular function on the affected side. Since the blood supply to the inner ear and the vestibulocochlear nerve arises from AICA, a combination of peripheral and central symptoms and signs is characteristic of the AICA infarction syndrome. The vertigo that preceded infarction may have resulted from transient ischemia to the inner ear or the vestibular nerve.     

Hearing symptoms in migrainous infarction

BACKGROUND: In case reports, migraine headaches have been associated with fluctuating low-frequency hearing loss and sudden, unilateral hearing loss. Auditory symptoms associated with migrainous infarction have not previously been emphasized. OBJECTIVE: To describe migrainous infarction presenting with acute auditory symptoms. DESIGN: Case reports. SETTING: Tertiary care hospitals. PATIENTS: A 40-year-old man with a history of migraine suddenly developed bilateral hearing loss associated with severe, throbbing, occipital headache, tinnitus, vertigo, speech disturbance, and right hemiparesis. An early audiogram showed profound, down-sloping, sensorineural-type hearing loss bilaterally. Sixteen days later, a follow-up pure tone audiogram documented marked improvement in both sides to a pure tone average of 30 dB. Right hemiparesis and dysarthria also improved steadily for 2 months. A 25-year-old woman with a history of migraine with aura suddenly developed hyperacusis, unilateral hearing loss, and migraine headache early in migrainous infarction. Magnetic resonance imaging documented infarcts in the pons and cerebellum. CONCLUSIONS: In these patients, acute auditory symptoms are a part of the prodrome of migrainous infarction. We speculate that these symptoms may have resulted from migraine-associated vasospasm. Migrainous infarction should be considered in the differential diagnosis of acute auditory symptoms, including sudden, bilateral hearing loss.     

Auditory disturbance as a prodrome of anterior inferior cerebellar artery infarction

OBJECTIVES: To investigate the clinical and radiological features of patients presenting with an acute auditory syndrome as a prodromal symptom of anterior inferior cerebellar artery (AICA) infarction. METHODS: 16 consecutive cases of AICA infarction diagnosed by brain magnetic resonance imaging completed a standardised audiovestibular questionnaire and underwent a neuro-otological evaluation by an experienced neuro-otologist. RESULTS: Five patients (31%) had an acute auditory syndrome as a prodrome of AICA infarction one to 10 days before onset of other brain stem or cerebellar symptoms. Two types of acute auditory syndrome were found: recurrent transient hearing loss with or without tinnitus (n = 3), and a single episode of prolonged hearing loss with or without tinnitus (n = 2). The episodic symptoms were brief, lasting only minutes. The tinnitus preceding the infarction was identical to the tinnitus experienced at the time of infarction. At the time of infarction, all patients developed hearing loss, tinnitus, vertigo, and ipsilateral hemiataxia. The most commonly affected site was the middle cerebellar peduncle (n = 5). Four of the five patients had incomplete hearing loss and all had absence of vestibular function to caloric stimulation on the affected side. CONCLUSIONS: Acute auditory syndrome may be a warning sign of impending pontocerebellar infarction in the distribution of the AICA. The acute auditory syndrome preceding an AICA infarct may result from ischaemia of the inner ear or the vestibulocochlear nerve.     

Recent Advances in Cerebellar Ischemic Stroke Syndromes Causing Vertigo and Hearing Loss

Cerebellar ischemic stroke is one of the common causes of vascular vertigo. It usually accompanies other neurological symptoms or signs, but a small infarct in the cerebellum can present with vertigo without other localizing symptoms. Approximately 11 % of the patients with isolated cerebellar infarction simulated acute peripheral vestibulopathy, and most patients had an infarct in the territory of the medial branch of the posterior inferior cerebellar artery (PICA). A head impulse test can differentiate acute isolated vertigo associated with PICA territory cerebellar infarction from more benign disorders involving the inner ear. Acute hearing loss (AHL) of a vascular cause is mostly associated with cerebellar infarction in the territory of the anterior inferior cerebellar artery (AICA), but PICA territory cerebellar infarction rarely causes AHL. To date, at least eight subgroups of AICA territory infarction have been identified according to the pattern of neurotological presentations, among which the most common pattern of audiovestibular dysfunction is the combined loss of auditory and vestibular functions. Sometimes acute isolated audiovestibular loss can be the initial symptom of impending posterior circulation ischemic stroke (particularly within the territory of the AICA). Audiovestibular loss from cerebellar infarction has a good long-term outcome than previously thought. Approximately half of patients with superior cerebellar artery territory (SCA) cerebellar infarction experienced true vertigo, suggesting that the vertigo and nystagmus in the SCA territory cerebellar infarctions are more common than previously thought. In this article, recent findings on clinical features of vertigo and hearing loss from cerebellar ischemic stroke syndrome are summarized.     

Bilateral sudden deafness as a prodrome of anterior inferior cerebellar artery infarction

BACKGROUND: Acute ischemic stroke in the distribution of the anterior inferior cerebellar artery is known to be associated with hearing loss, facial weakness, ataxia, nystagmus, and hypalgesia. There have been few reports on bilateral deafness and vertebrobasilar occlusive disease. Furthermore, previous reports have not emphasized the inner ear as a localization of bilateral deafness. OBJECTIVE: To describe the presentation of acute ischemic stroke in the distribution of the anterior inferior cerebellar artery as sudden bilateral hearing loss with minimal associated signs. DESIGN AND SETTING: Case report and tertiary care hospital. PATIENT: A 66-year-old man with diabetes mellitus developed sudden bilateral deafness, unilateral tinnitus, and vertigo 7 days before the onset of dysarthria, facial weakness, and ataxia. T2-weighted magnetic resonance imaging scans showed hyperintensities in the right lateral pons and right middle cerebral peduncle and a possible abnormality of the left middle cerebellar peduncle. A magnetic resonance angiogram showed moderately severe stenosis of the distal vertebral artery and middle third of the basilar artery. The patient's right limb coordination and gait improved steadily over several weeks, but there was no improvement in hearing in his right ear. CONCLUSIONS: The relatively isolated onset of deafness as well as the severity and persistence of the hearing loss led us to conclude that the hearing loss in this case was likely due to prominent hypoperfusion of the internal auditory artery, with labyrinthine infarction as the earliest event. Vertebrobasilar occlusive disease should be considered in the differential diagnosis of sudden bilateral deafness.     

The spectrum of audiologic and vestibular findings in patients with Meniere's disease.

A retrospective study of 55 patients with Meniere's disease suggested a subgrouping based on presentation of auditory symptoms. Patients were defined as "definite" (documented fluctuating hearing loss and episodic vertigo), "probable" (history of fluctuating hearing loss and episodic vertigo) and "possible" (history of fluctuating tinnitus and episodic vertigo). An in-depth discussion of the natural history, audiometric and vestibular findings is presented.     

Isolated hypalgesia in ipsilateral face without hearing disorders: a variant of AICA infarction

We reported a 78-year-old woman who had a sudden-onset episode of vertigo, nausea and vomiting. Half an hour later, she felt right hemiataxia, and then noticed numbness on the entire right side of the face. She did not complain of hearing disorders or sensory disorders of left half body involving face. Pure tone audiogram and auditory brainstem responses were normal. Magnetic resonance imaging showed right hypersignals in the dorsolateral pons and middle cerebellar peduncle, as well as in cerebellum. Magnetic resonance angiography showed occlusion of the right anterior inferior cerebellar artery (AICA). Neuroradiological findings suggested ischemia in the whole territory of the right AICA. This is a case of AICA infarction and clinicians should be aware that sensory defect was only in the ipsilateral face without hearing disorders can be the manifestation of AICA infarction.     

Final diagnosis of patients with clinically suspected vestibular neuritis showing normal caloric response

Vestibular neuritis is one of the most common peripheral causes of acute vestibular syndrome, of which the diagnosis is generally based on a comprehensive interpretation of clinical and laboratory findings following reasonable exclusion of other disorders. This study aimed to investigate the final diagnosis of patients admitted to hospital under the clinical impression of vestibular neuritis who showed no unilateral caloric paresis.Forty-five patients who visited the emergency department with isolated acute spontaneous vertigo were included. Among them, six patients (13%) developed definitive spontaneous vertigo lasting longer than 20 min again after discharge from hospital, accompanied by hearing loss, which was audiometrically documented, leading to a final diagnosis of definite Ménière’s disease. Nine patients (20%) revisited our clinic with recurrent episodic vertigo without any documented hearing loss or auditory symptoms such as hearing loss, tinnitus or ear fullness, which led to a final diagnosis of possible Ménière’s disease. In four patients (9%), initial spontaneous vertigo and nystagmus changed to positional vertigo and nystagmus on the second hospital day. In 26 patients (58%), neither another episode of vertigo nor auditory symptoms developed during follow-up period (7–92 months), a condition to which the authors gave an arbitrary diagnosis of “mild unilateral vestibular deficit”. In conclusion, patients admitted to hospital under clinical impression of vestibular neuritis may have various final diagnoses, and “mild unilateral vestibular deficit” was the most common final diagnosis among patients who did not meet the diagnostic criteria of vestibular neuritis.     

Recurrent audiovestibular disturbance initially mimicking Ménière’s disease in a patient with anterior inferior cerebellar infarction

An anterior inferior cerebellar artery (AICA) stroke is characterized by vertigo, tinnitus, and deafness in addition to facial weakness, hemiataxia, and hypalgesia. Sometimes, it can present as sudden deafness with vertigo, without brainstem or cerebellar signs. We report a 55-year-old woman with hypertension and diabetes, showing recurrent audiovestibular disturbance before a typical pattern of AICA infarction, which was initially diagnosed as Ménière’s disease. In elderly patients with recurrent hearing loss and vertigo lasting several minutes, lack classic brainstem or cerebellar signs, if they have vascular risk factors, physicians may also consider the potential symptom of AICA infarction.     

Unilateral sudden deafness as a primary symptom of brainstem and cerebellar infarction]

We report a case with a unilateral sudden sensorineural hearing loss caused by an infarction of brainstem and cerebellum. The patient was a 74-year-old male presented with a sudden onset of hearing loss and tinnitus in the right ear and dizziness. Steroid was administered on suspicion of idiopathic sudden deafness. However, the initial symptoms were deteriorated approximately 2 weeks later. He newly complained of the numbness of the right face and double vision, and he was transferred to our hospital for further evaluation. Neurological examination demonstrated horizontal nystagmus, diminution in the right facial sensation, right peripheral facial palsy, right hearing loss and cerebellar ataxia. Urgent MRI disclosed fresh infarctions of the right middle cerebellar peduncle and cerebellum localized in the territory of anterior inferior cerebellar artery. In general, idiopathic sudden deafness and Meniere's disease are frequent diagnosis in cases of sudden hearing loss with vertigo, but these symptoms may rarely be caused by cerebrovascular disorder. In patients with risk factors for arteriosclerosis, cerebrovascular disorder should be taken into consideration even if idiopathic sudden deafness may be suspected clinically. We emphasize the diagnostic importance of careful observation on neurological findings and early detection of radiological abnormalities on MRI.     

Sudden bilateral hearing loss with vertigo due to vertebral artery occlusion]

We report a case of sudden bilateral hearing loss associated with an occlusion of the right vertebral artery. A 66-year-old man was admitted to hospital suffering from sudden onset vertigo, deafness, and vomiting. He could not walk due to truncal ataxia. There was positional nystagmus to the right; spontaneous and gaze-evoked nystagmus were absent. He had no facial nerve palsy, dysarthria, pyramidal tract signs, limb ataxia, and sensory impairment. Pure tone audiometry demonstrated a profound sensorineural deafness of both ears. A tentative diagnosis of sudden deafness was made. He was treated with intravenous infusion of corticosteroid; hearing loss of the left ear slightly improved. Cranial MR imaging demonstrated fresh small infarcts in the right cerebellar tonsil, the left cerebellar medulla, and the left middle cerebellar peduncle. MR angiography demonstrated an occlusion of the right vertebral artery. It is possible that reduced perfusion of the anterior inferior cerebellar arteries and internal auditory arteries on both sides resulted in multiple "border zone" infarcts and damage to the inner ear. Clinicians should be aware of the possibility of vertebrobasilar occlusive disease in case of sudden bilateral hearing impairment, even when brainstem or cerebellar signs are absent.     

A case of medial medullary infarction with persistent primitive hypoglossal artery]

A 66-year-old woman was admitted to our hospital because of vomiting, dizziness and vertigo. Neurological examination on admission revealed only upbeat nystagmus without cranial nerve symptoms, paresis, cerebellar signs or sensory disturbances. Magnetic resonance(MR) images demonstrated a new T 2 high intensity and T 1 iso-intensity signal lesion in the right upper medial medulla. This medial medullary infarction caused central vestibular dysfunction. MR angiography and digital subtraction angiography demonstrated a persistent primitive hypoglossal artery (PPHA) originating from the right internal carotid artery to the vertebrobasilar artery associated with the stenosis of the right internal carotid artery at the level of the cervical bifurcation. This is the first report of medullary infarction with persistent carotid-basilar anastomosis. We suspected this medullary infarction was caused by artery to artery embolism in the branch of the right vertebral artery through the PPHA distal originated from the stenosis of the right internal carotid artery.     

Acute bilateral hearing loss as a “worsening sign” in a patient with critical basilar artery stenosis

We report a patient who presented with an acute-onset transient vertigo and unsteady gait with bilateral hearing loss. Brain MRI revealed a critical basilar artery (BA) stenosis at the lower pons and infarction in various areas on both sides in the territories of the posterior inferior cerebellar arteries (PICA). Further, we could not visualize the right anterior inferior cerebellar artery (AICA). The bilateral hearing loss may be ascribed to stroke due to the critical BA stenosis, causing hypoperfusion injury extending from the PICA to the AICA on both sides. Local intra-arterial thrombolytic therapy with the administration of 1 × 10⁶ IU of urokinase aided partial recanalization of the BA, after which the right AICA reappeared. The neurological function of the patient recovered to normal, and no hemorrhagic complications were observed. Therefore, practitioners should be alert when treating patients with acute bilateral hearing loss, which may be related to an underlying catastrophic stroke.     

Horizontal canal benign paroxysmal positioning vertigo with ipsilateral hearing loss

The etiology of benign paroxysmal positioning vertigo (h-BPPV) of the horizontal semicircular is unknown. Insight was obtained from two patients with h-BPPV and associated hearing impairment. Based on the different inner ear lesion patterns in neurolabyrinthitis contrary to ischemic labyrinthine infarction we assessed multiple vestibulo-cochlear functions for the first time in two patients who suffered from h-BPPV with sudden unilateral hearing loss. While in patient no. 1 the lesion pattern (posterior canal paresis in addition to the sudden hearing loss) could possibly be caused by ischemia of the common cochlear artery, the lesion pattern of patient no. 2 (hearing loss and ipsilateral paresis of the posterior and horizontal semicircular canal) exceeds the typical vascular labyrinthine territories and may indicate viral neurolabyrinthitis.     

表现为孤立性眩晕的大脑中动脉穿支梗死

目的孤立性眩晕主要见于前庭周围性病变,罕见于大脑中动脉(MCA)穿支脑梗死,本文探讨MCA穿支梗死出现孤立性眩晕与头晕的临床特征。方法报道3例表现为孤立性眩晕和头晕的MCA穿支急性腔隙性脑梗死患者的临床表现、影像学资料,同时通过PubMed检索之前报道的病例,回顾总结其临床特征。结果包括文献共5例表现为眩晕和头晕的MCA穿支梗死病例。3例表现为孤立性眩晕和头晕,1例表现为持续性头晕伴随短暂性言语不清,1例有轻偏瘫,2例有眼球震颤。眩晕与头晕严重程度均为中度,经治疗后眩晕和头晕多在病程7 d内完全缓解。4例梗死灶位于左侧内囊或放射冠区,1例位于右侧放射冠。颈部及头部增强血管成像有2例显示右侧椎动脉纤细狭窄,4例完成了头部增强CTA检查均提示前循环血管正常。本文3例患者在核磁共振成像(MRI)检查证实为急性腔隙性脑梗死之前,均未有明确诊断。结论 MCA穿支脑梗死可以表现为孤立性眩晕和头晕,严重程度较轻,持续时间通常数日,自主神经症状不明显。为了避免漏诊或误诊,推荐神经影像学检查,特别是MRI弥散成像(DWI)可作为老年首次眩晕和头晕的常规检查项目。     

Isolated limb sensory disturbance accompanied with sudden deafness from vertebral artery dissection: a case report

A 52-year-old male presented with right sudden deafness and left isolated limb sensory disturbance following posterior neck pain. An examination revealed a mild degree of right sensory neural hearing loss and decreased pain and thermal sensation in the left side below the L3 level. Brain MRI showed a small lesion in the midlateral portion of the right middle-rostral medulla. The combination of the image findings suggests a right lateral medullary infarction caused by a vertebral artery (VA) dissection. In our case, the fibers carrying the modalities of the lateral spinothalamic tract restricted to below the L3 level, sparing other sensations, suggest a selective involvement. This finding suggests that sensory dermatomal representation in the modality may be preserved at least up to the level of the medulla oblongata. Moreover, in our case, the small emboli originating from the dissected VA possibly caused a small selective cochlear infarction. This type of embolism may give rise to various combinations of symptoms depending on the branches involved. For patients with an acute onset of headache or neck pain who present with isolated limb sensory disturbance accompanied by sudden deafness, even without the classical brainstem signs, a VA dissection and medullary infarction should therefore be considered.     

Schwannoma of the vestibulocochlear nerve presenting as isolated paroxysmal vertigo

We report a patient with a schwannoma of the eighth cranial (vestibulocochlear) nerve who presented with isolated episodes of paroxysmal vertigo and positive Dix-Hallpike maneuver, and without the common features of hearing loss, disequilibrium and tinnitus. There are no previous reports of paroxysmal episodes of vertigo as the sole manifestation of schwannoma of the vestibulocochlear nerve. Hence, recurrent paroxysmal vertigo should therefore prompt the physician to rule out schwannoma of the vestibulocochlear nerve as a potential cause even in the presence of normal hearing tests.     

Bulbovascular compression by megadolichobasilar artery manifested as neurogenic and refractory hypertension]

A 37-year-old man with juvenile and refractory hypertension was admitted to our hospital for progressive left hearing loss, vertigo, and dizziness. Neurological examination revealed left hearing loss and exaggerated deep tendon reflexes and vestibular dysfunction. MRI and cerebral angiography disclosed megadolichobasilar artery (MDBA). Moreover, modified MR cisternography at the medulla disclosed marked compression and deformity of the left rostral ventrolateral medulla (RVLM) by the dolichoectatic right vertebral artery. In the literatures, bulbovascular compression has been reported in 4 among 9 patients with MDBA (including the present patient), for whom MRI of the medulla was presented. All 4 patients suffered from hypertension, and at least 3 of them showed juvenile and refractory hypertension. Ipsilateral pyramidal tract disturbance (Opalski syndrome) was observed in 3 patients. Considering the recent concept that the cardiovascular center can be localized at the RVLM, juvenile and refractory hypertension, and possibly Opalski syndrome in the present patient can be attributed to bulbovascular compression by MDBA. In the patients with MDBA and hypertension or Opalski syndrome, MR cisternography of the medulla is warranted to evaluate compression by MDBA.