The autonomic nervous system (ANS) integrates the function of the internal organs for the homeostasis against various external environmental changes. The efferent components of the ANS are regulated by sensory signals arising from the viscera as well as non-visceral organs. The central neural networks that integrate these sensory signals and modify visceral motor output are complex, and synaptic reflexes formed in the brainstem and spinal cord integrate behavioral responses and visceral responses through the central neural networks. A detailed understanding of the neural network presented above may explain the role of the vestibular system on the homeostasis more extensively.
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Objectives To measure 125 Hz pure-tone thresholds in patients with low frequency sensorineural hearing loss (LFHL) and vertigo and to evaluate the necessity of 125 Hz thresholds for assessment of LFHL with vertigo. Methods: Pure tone audiometry including 125 Hz was performed in 25 dizzy patients with LFHL ≤500 Hz and 25 age-matched subjects with normal hearing. Patients with sudden sensorineural hearing loss and vertigo were excluded. Comparison of 125 Hz between LFHL and control groups, and comparison of 125 Hz and other frequencies in LFHL group was made. Results: Mean pure-tone thresholds at 125 Hz in LFHL group (41.7±7.5 dB) was higher than that in normal controls (12.8±6.4 dB). Three (12%) patients had normal thresholds at 125 Hz in LFHL group, whereas all subjects showed normal at 125 Hz in control group. None with average hearing thresholds at 250 and 500 Hz ≥35 dB had normal threshold at 125 Hz. There was a significant correlation between 125 Hz and other low frequencies in LFHL group (250 Hz; r=0.79, 500 Hz; r=0.66). Conclusions: Not every patient of LFHL with vertigo has abnormal hearing threshold at 125 Hz, although all subjects with normal hearing is within normal limits at 125 Hz. Measurement of 125 Hz pure-tone threshold is highly recommended when a mild LFHL exists.
Objectives Vertigo is considered a poor prognostic factor in patients with sudden sensorineural hearing loss (SSNHL). Benign paroxysmal positional vertigo (BPPV) is a common cause of vertigo and may accompany SSNHL. However, whether BPPV is a poor prognostic factor remains controversial. We identified features of prognostic utility in patients with both SSNHL and BPPV. Methods: We retrospectively evaluated data on all patients diagnosed with SSNHL at Keimyung University Dongsan Medical Center between January 2011 and December 2015. We reviewed medical records, the results of vestibular function testing, and audiographic data. Results: Of the 524 idiopathic SSNHL patients, 20 (3.8%) were also diagnosed with unilateral BPPV. The average pure tone average (PTA) of those with both SSNHL and BPPV was higher than that of those without BPPV. Of the 471 patients with SSNHL only, 143 (30%) exhibited complete recovery. Of the 33 SSNHL patients with vertigo, 3 (9%) exhibited complete recovery, but only 3 (15%) of those with both SSNHL and BPPV exhibited complete recovery. Of the 20 patients with both SSNHL and BPPV, 15 had ipsilateral BPPV and 5 contralateral BPPV. Conclusions: Patients with BPPV had a higher average initial PTA score and poorer hearing recovery after treatment, than patients with SSNHL alone. Patients with contralateral BPPV had a somewhat better prognosis than those with ipsilateral BPPV, but statistical significance was not attained.
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Which Is More Important for the Prognosis of Sudden Sensorineural Hearing Loss with Vertigo, Canal Paresis or Benign Paroxysmal Positional Vertigo? Yong-Hwi An, Hyun Joon Shim Research in Vestibular Science.2021; 20(3): 101. CrossRef
Objectives We investigated clinical significance of head shaking nystagmus (HSN) and perverted HSN (pHSN) in patients with peripheral and central vestibular disorders. Methods: We reviewed medical records of 822 consecutive subjects who were referred to a dizziness clinic. We performed neurologic examination including video-oculography in darkness for 60 seconds before, during and for 100 seconds after head-shaking. HSN was considered to develop when post-head-shaking nystagmus last at least 5 beats with latency from end of head-shaking of no more than 5 seconds, and a velocity at least 3°/sec. Results: In control group (n=45), there were observed spontaneous nystagmus (SN) in 2.2%, HSN in 17.8%, pHSN in 6.7%. In patients with peripheral vestibular disorder group (n=397), there were observed SN in 14.1%, HSN in 40.6%, pHSN in 9.8%. In patients with central vestibular disorder group (n=217), there were observed SN in 17.5%, HSN in 24.0%, pHSN in 13.4%. In unspecified dizziness group (n=208), there were observed SN in 1.9%, HSN in 13.0%, pHSN in 1.9%. pHSN was frequently observed in central vestibular disorders such as stroke, vestibular migraine, cerebellar ataxia, and vertebro-basilar insufficiency. However, pHSN was also observed at higher rate than expected in peripheral vestibular disorders including benign paroxysmal positional vertigo especially involving vertical canals, Meniere disease and even in unilateral vestibulopathy. Conclusions: Our results show that perverted HSN in dizzy populations was frequently observed not only in cases of central vestibular disorders but also in peripheral disorders. Perverted HSN can develop by any conditions that cause difference in vestibular velocity storage in vertical component of vestibular-ocular reflex.
Objectives The purpose of this study was to examine the clinical manifestations and significance of pseudo-spontaneous nystagmus (PSN) and head-shaking nystagmus (HSN) in horizontal canal benign paroxysmal positional vertigo (HC-BPPV). Methods: Two hundred fifty-two patients diagnosed as HC-BPPV were reviewed retrospectively. After excluding 55 patients with ipsilateral vestibular diseases, multiple canal BPPV, or those who were lost to follow-up, we analyzed the direction of PSN and HSN in patients with HC-BPPV. We also compared the clinical characteristics and treatment outcome between PSN-positive and PSN-negative groups. Results: Our study included 197 patients composed of 80 patients with geotropic HC-BPPV and 117 patients with apogeotropic HC-BPPV. PSN was observed in 13.7% patients and HSN was observed in 45.2%. The incidence of HSN was higher in apogeotropic HC-BPPV, while the proportion of PSN was not statistically significant between the two subtypes. There was no directional preponderance in geotropic HC-BPPV, while ipsilesional PSN and contralesional HSN showed higher incidence in apogeotropic HC-BPPV. The dizziness handicap inventory score in the PSN-positive group was higher than that in the PSN-negative group (p<0.001), and the duration of symptom onset in the PSN-positive group was shorter than that in the PSN-negative group (p=0.047). However, there was no significant difference in the treatment outcome between the two groups. Conclusions: The incidence of HSN was higher than that of PSN in patients with apogeotropic HC-BPPV. Patients with HC-BPPV showing PSN demonstrated more severe initial symptoms and visited the hospital in a shorter period of time after the onset of symptoms.
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Benign Paroxysmal Positional Vertigo: Diagnostic Criteria and Updated Practice Guideline in Diagnosis Dae Bo Shim Research in Vestibular Science.2020; 19(4): 111. CrossRef
Objectives In vestibular neuritis (VN), the lesion preferentially affects the superior vestibular nerve because of the anatomic arrangement. It is well known that VN patients have a higher score of metabolic syndrome or a higher incidence of vertebral artery hypoplasia than controls. However, it is unclear whether the frequency of cardiovascular risk factors can affect the selective involvement of the branch of the vestibular nerve. Thus, we investigated the influence of cardiovascular risk factors on the development of total- or divisional VN. Methods: 61 patients with VN were enrolled. Video head impulse tests and caloric tests were performed to determine which vestibular divisionswere affected. The patients were divided into divisional-VN (superior or inferior) and total-VN groups. Statistical analysis of the frequency of cardiovascular risk factors was performed only in superior and total VN groups because the number of inferior VN patients was too small to be statistically analyzed. Results: Nineteen (31.1%) patients were classified as the total-VN group. In the divisional-VN group (42 patients, 65.6%), 40 were superior VN. The frequency of cardiovascular risk factors are not significantly different in superior VN and total-VN groups (All patients 50/61 [82.0%], superior-VN 36/40 [90.0%], total-VN 13/19 [68.4%]). The frequency of having more than one cardiovascular risk factor was slightly higher in the superior VN group, (13 [68.4%] vs. 36 [90.0%], p=0.062) but did not show any significant difference. Conclusions: The number of cardiovascular risk factors did not differ in superior VN patients compared to total VN patients.
Polycythemia vera (PV) is well known chronic myeloproliferative neoplasm, caused by clonal expansion of an abnormal hematopoietic stem cell. Patients with PV may present diverse neurologic symptoms including headache, dizziness or vertigo, tinnitus. However, the attention has not been directed to the neurootological findings in patients with PV. Here, we present a 71-year-old male patient with PV suffered from vertigo and headache. He demonstrated gaze-evoked nystagmus and perverted head shaking nystagmus. Transcranial Doppler showed decrement of blood flow velocity in posterior circulation. The patient’s neuro-otologic findings were normalized as polychethemia and blood flow improved with repetitive phlebotomy and medications such as hydroxyurea and aspirin. Considering the neurological and hemodynamic findings in our patient, the mechanism of vertigo in PV could be explained by central vestibulopathy because of vascular insufficiency rather than peripheral vestibulopathy because of inner ear blood hyperviscosity.
Vestibular schwannoma (VS) can have various symptoms and almost half of the patients have balance problems. Some patients with VS can suffer from recurrent vertigo mimicking Meniere’s disease. We present the case of a 61-year-old male patient who was suffering from recurrent vertigo due to VS while symptoms improved by chemical labyrinthectomy with gentamicin. We consider that chemical labyrinthectomy can be an option in the treatment of patients with recurrent vertigo for VS.
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Diagnosis and Management of Vestibular Schwannoma: Focus on Dizziness Sung Il Nam Research in Vestibular Science.2021; 20(4): 119. CrossRef
Vertical gaze palsy is usually associated with lesions of the rostral midbrain and thalamo-mesencephalic junction. The rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), the interstitial nucleus of Cajal, and the posterior commissure located in the midbrain are the critical area in supranuclear control of vertical eye movements. We describe a case of vertical one-and-a-half syndrome accompanying contralateral abduction and incomplete depression palsy due to thalamo-mesencephalic infarction. These vertical eye movement abnormalities are presumed to be caused by damage to the ipsilateral riMLF, interstitial nucleus of Cajal, and oculomotor fascicles.
Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowing progressive ataxic disorder characterized by bilateral vestibulopathy, cerebellar ataxia and somatosensory impairment. Autonomic dysfunction is recently considered as a core feature in CANVAS in addition to these symptoms. In most cases, patients with CANVAS show cerebellar atrophy in brain imaging, but some cases show minimal or no atrophy of cerebellum. Brain (18F)-fluoro-2-deoxy-D-glucose positron emission tomography (18F-FDG PET) study can be a complimentary tool to diagnosis CANVAS in cases of no structural abnormality such as cerebellar atrophy. Hereby, we present a case of CANVAS with minimal atrophy of cerebellum but showing a prominent hypometabolism in cerebellum, thalamus and posterior cingulate cortex in 18F-FDG PET.
It is known that about 30% of patients with sudden hearing loss present with vertigo or dizziness. In clinical practice, this is called sudden hearing loss with vertigo (SHLV) although definite diagnostic criteria have not been established. Dizziness in SHLV is known to be caused by the dysfunction of the vestibular end-organs as well as the superior vestibular nerve or both vestibular nerve divisions. Lesions of the inferior vestibular nerve or a single semicircular canal have also been reported in these patients. Herein we report a 71-year-old male patient with SHLV who demonstrated vestibular dysfunction involving only the posterior semicircular canal. The patient showed normal results in the bithermal caloric test and the cervical vestibular evoked myogenic potentials test as well as positional test. Video head impulse test showed decreased gain only in the posterior semicircular canal. This case is significant in showing that dizziness in SHLV patients can occur by an abnormality involving only a single semicircular canal.
A 84-year-old woman presented with a two weeks history of dizziness, slurred speech and ataxia. The neurological exam showed spontaneous left beating nystagmus, horizontal gaze evoked nystagmus and limb ataxia. A few weeks earlier, she had an upper airway infection. Brain MRI revealed diffuse leptomeningeal enhancement in the both cerebellar hemisphere and brain single photon emission computed tomography (SPECT) showed hyperperfusion in both cerebellar hemisphere. Extensive laboratory studies with cerebrospinal fluid analysis did not reveal any etiologic factors. She was started on methylprednisolone (1 g/day for 7 days), gradually improved over the weeks. Post infectious cerebellar ataxia is a neurologic complication that occasionally follows systemic viral and bacterial infections. This case demonstrates that cerebellar abnormalities can be detected by brain magnetic resonance imaging and SPECT.
Vestibular Paroxysmia and facial spasm may be caused by vascular compression of the vestibular and facial root entry zone. We report a case of paroxysmal nystagmus accompanied by facial spasm and which is well visualized by three-dimensional reconstruction images. The 3-dimensional reconstruction image supports the view that vestibular paroxysmia may occur with hemifacial spasm simultaneously due to vascular compression.