Research in Vestibular Science

Case Report

A Case of Delayed Audiovestibulopathy after Posterior Circulation Ischemic Stroke: Hyun Su Lee, Eun Kyung Jeon, Dong Hwan Kwon, Tae Hoon Kong; Res Vestib Sci. 2023;22(3):77-82. Published online September 15, 2023; DOI: https://doi.org/10.21790/rvs.2023.22.3.77

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Abstract PDF: Distinguishing central and peripheral causes of dizziness is vital. A case is presented where a 42-year-old man with a history of posterior circulation ischemic stroke developed acute unilateral vestibulopathy with hearing loss. Clinical examination revealed signs of vestibular dysfunction on the left side. Audiometry confirmed deafness on the left, but imaging ruled out new central issues. The patient was diagnosed with audiovestibulopathy and treated with steroids, antiviral agents, intratympanic injections, and hyperbaric oxygen therapy. Hearing loss persisted, but dizziness improved with vestibular rehabilitation. Poststroke patients should be closely monitored for peripheral complications. Further research should explore the benefits of antiplatelet therapy in vascular-related conditions, even without clear central lesions.

Original Article

Re-fixation Saccade at Video-Head Impulse Test in Patients with Sudden Sensorineural Hearing Loss: Dong Hyuk Jang, Sun Seong Kang, Hyun Joon Shim, Yong-Hwi An; Res Vestib Sci. 2023;22(2):46-51. Published online June 15, 2023; DOI: https://doi.org/10.21790/rvs.2023.22.2.46

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Abstract PDF: Objectives
This study was performed to evaluate characteristics and their prognostic value of video-head impulse test (vHIT) in sudden sensorineural hearing loss (SSNHL) with vertigo.
Methods
Of the 612 patients with a diagnosis of SSNHL from 2010 to 2018, 110 patients (18.0%) with vertigo and 39 patients (6.4%) with vHIT results were recruited. The patients were evaluated for their pure-tone hearing average (at initial, 1-month, and 6-month visit), the presence of re-fixation saccade and gains at vHIT, the canal paresis (CP) at ccaloric test.
Results
Patients with saccade (+) showed higher pure-tone averages than those with saccade (‒) on initial and follow-up audiograms. The improvement in pure-tone averages was less in the saccade (+) group than in the saccade (‒) group. There was no significant difference of hearing recovery between SSNHL patients with normal gain and those with decreased gain. There was no difference of hearing improvement between CP (+) and CP (‒) groups according to the presence of re-fixation saccade.
Conclusions
Concurrent re-fixation saccade at vHIT is a negative prognostic factor of hearing function in SSNHL. Re-fixation saccade in SSNHL may suggest widespread damages to both the cochlea and the vestibule, leading to the poor prognosis.

Case Report

Bilateral Sudden Hearing Loss Caused by Basilar Artery Dissection: A Case Report: Ho Byung Lee, Jieun Roh, Hyun Min Lee, Jae-Hwan Choi; Res Vestib Sci. 2021;20(4):151-155. Published online December 15, 2021; DOI: https://doi.org/10.21790/rvs.2021.20.4.151

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Abstract PDF: Bilateral sudden sensorineural hearing loss (SNHL) is rare, but a possible symptom of vertebrobasilar ischemia. A 69-year-old female patient with hypertension and atrial fibrillation presented with bilateral sudden hearing loss and vertigo without other neurological symptoms. On examination, she had left-beating horizontal nystagmus with positive head impulse on the left side. Pure tone audiometry revealed severe SNHL on both sides. Brain computed tomography angiography showed a dissection in the proximal portion of the basilar artery (BA) with occlusion of the mid-BA and bilateral anterior inferior cerebellar arteries (AICA), which confirmed on transfemoral cerebral angiography (TFCA). Left common carotid angiography demonstrated retrograde blood flow into the BA and right AICA via the left posterior communicating artery. During TFCA, her right hearing loss dramatically improved. Nine days later, follow-up TFCA showed an improvement of antegrade flow of the BA and AICA. We suggest that vertebrobasilar ischemia can be suspected in patients with bilateral sudden SNHL who present with risk factors for stroke.

Original Articles

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; Res Vestib Sci. 2021;20(3):101-107. Published online September 15, 2021; DOI: https://doi.org/10.21790/rvs.2021.20.3.101

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Abstract PDF

Objectives
This study was performed to determine characteristics and the prognostic values in idiopathic sudden sensorineural hearing loss (SSNHL) with comorbid ipsilateral canal paresis (CP) and/or benign paroxysmal positional vertigo (BPPV).
Methods
Of the 338 patients with a diagnosis of idiopathic SSNHL, 29 patients (8.6%) with CP and 24 patients (7.1%) with BPPV were recruited and compared to 23 patients with SSNHL and vertigo but without CP or BPPV. The patients were evaluated for their initial hearing threshold, type of canal involved, response to repositioning maneuvers, and hearing outcome for 6 months.
Results
Patients with CP (+) BPPV (‒) showed lower pure-tone averages than those with CP (‒) BPPV (+) on initial and follow-up audiograms. The improvement in pure-tone averages was less in the CP (+) BPPV (‒) group than in the CP (‒) BPPV (+) group. The improvement in speech discrimination scores was less in the CP (+) BPPV (‒) group than in the CP (‒) BPPV (‒) group. BPPV most commonly involved the posterior canal (15 of 24, 62.5%), followed by the horizontal canal (13 of 24, 54.2%). Three of 24 patients (12.5%) had recurrences of BPPV.
Conclusions
CP is a more serious sign for hearing recovery than BPPV, although both CP and BPPV are negative prognostic indicators of auditory function in SSNHL. Concurrent CP and/or BPPV in SSNHL suggest combined damage to the vestibule and may indicate severe and widespread labyrinthine damage, leading to a poor prognosis.

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Re-fixation Saccade at Video-Head Impulse Test in Patients with Sudden Sensorineural Hearing Loss
Dong Hyuk Jang, Sun Seong Kang, Hyun Joon Shim, Yong-Hwi An
Research in Vestibular Science.2023; 22(2): 46. CrossRef

Positional Audiometry in Patients with Light Cupula: A Preliminary Study: Jiyeon Lee, Dong-Han Lee, Jung Eun Shin, Chang-Hee Kim; Res Vestib Sci. 2020;19(3):89-94. Published online September 15, 2020; DOI: https://doi.org/10.21790/rvs.2020.19.3.89

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Abstract PDF

Objectives
Light cupula is characterized by persistent geotropic direction-changing positional nystagmus in a supine head-roll test. The purpose of this study is to investigate if hearing level is influenced by the change of head position in light cupula under the assumption that relative density difference similarly occurs between the tectorial membrane and endolymph.
Methods
Twelve patients with unilateral light cupula who underwent positional audiometry were included in this study. Pure tone thresholds were compared among three head positions.
Results
Hearing threshold in pure tone audiometry (PTA) of the affected ear was not different from that of the healthy ear. PTA thresholds of the affected side were not significantly different in three head positions; upright seating, cochlear apex-up, and cochlear apex-down positions.
Conclusions
Although positional change of nystagmus direction is the most significant clinical feature of light cupula, positional change of hearing level was not observed in those patients. The lack of positional influence on hearing may be explained as follows: (1) the heavier endolymph phenomenon occurs only in the vestibular end organ without involving the cochlea; (2) the light cupula phenomenon is more likely to occur due to light debris mechanism rather than heavier endolymph or lighter cupula mechanism; and (3) the effects of light cupula could be modified by outer hair cells, which work for tuning in the cochlea, even though light tectorial membrane or heavy endolymph occurs.

Citations

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Light cupula phenomenon: a systematic review
Nilüfer Bal, Melike Altun, Elif Kuru, Meliha Basoz Behmen, Ozge Gedik Toker
The Egyptian Journal of Otolaryngology.2022;[Epub] CrossRef

Clinical Characteristics of Sudden Sensorineural Hearing Loss Accompanying Benign Paroxysmal Positional Vertigo: Sung Min Park, Bin Kwon, Sung Won Li, Seok Min Hong, Sung Kyun Kim; Res Vestib Sci. 2020;19(2):71-78. Published online June 15, 2020; DOI: https://doi.org/10.21790/rvs.2020.19.2.71

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Abstract PDF

Objectives
Idiopathic unilateral sudden sensorineural hearing loss (SSNHL) with simultaneous benign paroxysmal vertigo (BPPV) is known to be associated with poor hearing recovery. We aimed to investigate clinical findings in patients with SSNHL with BPPV and analyze prognostic factors including presence of BPPV related to hearing outcome.
Methods
We retrospectively reviewed the electronic medical data of 14 patients with concurrent SSNHL and BPPV (combined group). We selected 52 patients without BPPV as a control group who have matched initial threshold of pure tone audiometry and age of each patient in the combined group. We evaluated clinical characteristics of all participants and compared hearing outcomes between the 2 groups. A multivariate logistic regression analysis was performed to investigate the factors related to hearing recovery.
Results
Initial mean pure tone audiometry (PTA) threshold of combined group was 90.36±26.2 dB. Posterior canal was most commonly involved (n=7, 50%), and 8 (57%) patients showed abnormal video head impulse test results. There was no significant difference between hearing recovery rates of combined and control group (p=0.237) and mean pure tone audiometry threshold changes were not significantly different between the 2 groups (p=0.942). Old age (≥60 years), high initial PTA threshold (＞90 dB), and obesity (body mass index ≥ 25 kg/m²) were poor prognostic predictors.
Conclusions
There was no difference between hearing outcomes of combined group and profound SSNHL only group. BPPV was not a significant prognostic factor of SSNHL patients.

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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

Comparison of Predictive Parameters between the Video Head Impulse Test and Caloric Test: Chun Han, Seung Won Paik, Hui Joon Yang, Sang Yoo Park, Ji Hyeon Lee, Young Joon Seo; Res Vestib Sci. 2020;19(2):55-61. Published online June 15, 2020; DOI: https://doi.org/10.21790/rvs.2020.19.2.55

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Abstract PDF

Objectives
The aim of this paper was to determine if a correlation exists between video head impulse test (vHIT) and electronystagmography with caloric test (ENG). More specifically, comparison of covert and overt value from vHIT test with cold and warm stimulation value from ENG test.
Methods
Retrospective study of our single institue from the period of January 2015 to January 2017 enrolled 91 patients. Patients were divided into 3 groups by their diagnosis of either vestibular neuritis (VN), Meniere disease, or sudden sensorineural loss with vertigo accordingly. Each of the patients’ both ENG and vHIT data were recorded and parameters were evaluated.
Results
VN group was the only group to show a significant correlation between canal paresis (CP) with covert and overt saccades. Further analysis was done in the VN group and result showed covert saccade showing a larger area under the receiver operation characteristic curve value (0.77) compared to overt saccades (0.70), implying that covert saccade is a more accurate parameter for the prediction of the CP value. Furthermore, a positive correlation was seen between the gain value and the cold caloric stimulation value as well as between warm caloric stimulation value.
Conclusions
The value of our study lies in the fact that we have attempted to find a correlation between different parameters of 2 different vestibular tests. We concluded that the evaluation of overt nystagmus by the bedside head thrust test is inappropriate for predicting CP, and a vHIT is required to accurately evaluate vestibular function.

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A Comparative Analysis of the Vestibulocochlear Function in Patients with Isolated Semicircular Canal Hypofunction Using a Video Head Impulse Test
Yu Jung Park, Min Young Lee, Ji Eun Choi, Jae Yun Jung, Jung Hwa Bahng
Research in Vestibular Science.2023; 22(2): 34. CrossRef

Does 125-Hz Pure-Tone Thresholds Have Prognostic Value in Patients with Sudden Sensorineural Hearing Loss and Vertigo?: Yong-Hwi An, Hyun Joon Shim; Res Vestib Sci. 2020;19(1):6-11. Published online March 15, 2020; DOI: https://doi.org/10.21790/rvs.2020.19.1.6

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Abstract PDF: Objectives
To analyze 125-Hz pure-tone thresholds in dizzy patients with sudden sensorineural hearing loss (SSNHL) and to investigate the relationship between 125-Hz thresholds and the prognosis of SSNHL with vertigo.
Methods
Hearing and vestibular function tests including 125-Hz pure-tone were performed in 132 patients with SSNHL and 65 subjects with normal hearing. Audiometric follow-up was performed at 6 months after initial visit. Intergroup and intragroup comparison of 125 Hz was made between SSNHL and control groups.
Results
Twenty-four patients (18.2%) had normal thresholds at 125 Hz in SSNHL group, whereas all subjects showed normal at 125 Hz in control group. None with average hearing threshold at 250 and 500 Hz≥30 dB had normal threshold at 125 Hz. There was no significant relationship between 125-Hz threshold and results of vestibular function test. There was no correlation between 125-Hz threshold and hearing recovery in SSNHL group.
Conclusions
There might be no need to assess 125-Hz pure-tone threshold in patients with SSNHL, because it is enough to evaluate thresholds of 250 and 500 Hz for low frequency.

Comparison of Treatment Outcomes between Intratympanic Steroid Injection and Oral Diuretics in Patients with Acute Low Frequency Sensorineural Hearing Loss with Vertigo: Yong-Hwi An, Hyun Joon Shim; Res Vestib Sci. 2019;18(4):111-117. Published online December 15, 2019; DOI: https://doi.org/10.21790/rvs.2019.18.4.111

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Abstract PDF: Objectives
Sudden sensorineural hearing loss and Meniere disease can be managed with intratympanic steroid injection (ITSI) as a primary treatment. The aim of this study was to compare the effectiveness of ITSI versus oral diuretics for acute low frequency sensorineural hearing loss (LFHL) with vertigo.
Methods
A total 76 patients with LFHL that had developed within previous 2 weeks were enrolled and categorized into 2 groups: treated with ITSI four times on 4 consecutive days (ITSI group; 42 patients) and treated with diuretics orally for 2 weeks (diuretics group; 34 patients). After 6 months, we analyzed treatment outcomes using subjective improvement and audiometric change.
Results
Hearing thresholds at low frequencies ≤500 Hz were significantly improved in both ITSI and diuretics group (p＜0.05). The cure rate of ITSI group was not significantly higher than that of diuretics group (54.8% vs. 52.9%, p＞0.05). For subjective symptoms, there were no significant differences of improvement rate in both groups (ITSI 64.3% vs. diuretics 61.8%, p＞0.05). In pure tone audiometry, the improvement rate of ITSI group was not significantly different from that of diuretics group (78.6% vs. 70.6%, p＞0.05). There was a significant correlation between the cure rate and duration of symptoms.
Conclusions
Both ITSI and diuretics are effective treatment modalities for acute LFHL with vertigo within 2 weeks of development. There is no difference of treatment outcomes between ITSI and diuretics in patients with acute LFHL and vertigo.

The Value of Posterior Semicircular Canal Function in Predicting Hearing Recovery of Sudden Sensorineural Hearing Loss: Jung-Woo Shin, Sang Woo Kim, Youn Woo Kim, Wook Jang, Bo He Kim, Yun-Sung Lim, Seok-Won Park, Chang Gun Cho, Joo Hyun Park; Res Vestib Sci. 2019;18(4):103-110. Published online December 15, 2019; DOI: https://doi.org/10.21790/rvs.2019.18.4.103

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Abstract PDF

Objectives
Postulated etiologies for idiopathic sudden sensorineural hearing loss (SSNHL) include viral cochleitis, microvascular events. If SSNHL is caused by vascular compromise of common cochlear artery that supplies cochlea and posterior semicircular canal (PC), PC also can be damaged with cochlea. We aim to evaluate the prognostic value of PC function in relation to hearing recovery of SSNHL.
Methods
Seventy-six patients who were diagnosed and treated for SSNHL and who underwent video head impulse test (vHIT) and follow-ups for more than 3 months were reviewed retrospectively. We defined impairment of PC function as lower PC gain (＜0.7) or definite overt/covert saccade in vHIT. Patients were divided into 3 groups: group 1, SSNHL without dizziness; group 2, SSNHL with dizziness and impaired PC function; group 3, SSNHL with dizziness, but intact PC function. Hearing thresholds were repeatedly measured on the initial visit, 1 week, 1 month, and 3 months after treatment. Treatment outcome was analyzed by comparing hearing recovery rate using Siegel’s criteria and posttreatment audiometric changes among 3 groups.
Results
Thirty-two (29.6%), 33 (30.6%), and 43 patients (39.8%) were included into the groups 1, 2, and 3, respectively. The hearing recovery rate of the group 2 (39.4%) was significantly lower than that of groups 1 (65.6%) and 3 (65.1%) (p=0.043). Pre- and posttreatment changes of the PTA threshold was significantly lower in group 2 than group 1 (p=0.009). The change of speech discrimination in each group were not different.
Conclusions
Our findings suggest that the presence of PC impairment may be a poor prognostic sign for hearing recovery in patients with SSNHL.

Citations

Citations to this article as recorded by

Artificial Neural Network-Assisted Classification of Hearing Prognosis of Sudden Sensorineural Hearing Loss With Vertigo
Sheng-Chiao Lin, Ming-Yee Lin, Bor-Hwang Kang, Yaoh-Shiang Lin, Yu-Hsi Liu, Chi-Yuan Yin, Po-Shing Lin, Che-Wei Lin
IEEE Journal of Translational Engineering in Healt.2023; 11: 170. CrossRef
Vestibular mapping in Ramsay-Hunt syndrome and idiopathic sudden sensorineural hearing loss
Joon-Pyo Hong, Jung-Yup Lee, Min-Beom Kim
European Archives of Oto-Rhino-Laryngology.2023; 280(12): 5251. CrossRef
A Comparative Analysis of the Vestibulocochlear Function in Patients with Isolated Semicircular Canal Hypofunction Using a Video Head Impulse Test
Yu Jung Park, Min Young Lee, Ji Eun Choi, Jae Yun Jung, Jung Hwa Bahng
Research in Vestibular Science.2023; 22(2): 34. CrossRef
Video head impulse test for the assessment of vestibular function in patients with idiopathic sudden sensorineural hearing loss without vertigo
N Battat, O J Ungar, O Handzel, R Abu Eta, Y Oron
The Journal of Laryngology & Otology.2023; 137(12): 1374. CrossRef
Vestibular mapping assessment in idiopathic sudden sensorineural hearing loss
Hee Won Seo, Jae Ho Chung, Hayoung Byun, Seung Hwan Lee
Ear & Hearing.2022; 43(1): 242. CrossRef
Association of Metabolic Syndrome with Sensorineural Hearing Loss
Hwa-Sung Rim, Myung-Gu Kim, Dong-Choon Park, Sung-Soo Kim, Dae-Woong Kang, Sang-Hoon Kim, Seung-Geun Yeo
Journal of Clinical Medicine.2021; 10(21): 4866. CrossRef

Case Report

A Case of Sensorineural Hearing Loss and Vertigo during Epidural Nerve Block: Byeong Min Lee, Jin hong Noh, Seong Ki Ahn, Hyun Woo Park; Res Vestib Sci. 2018;17(4):170-174. Published online December 21, 2018; DOI: https://doi.org/10.21790/rvs.2018.17.4.170

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Abstract PDF: Epidural anesthesia has significantly advanced in neuraxial anesthesia and analgesia. It is used for surgical anaesthesia and treatment of chronic pain. Hearing loss during or after epidural anesthesia is rare, and it is known to occur by the change of the intracranial pressure. Cerebrospinal fluid is connected with perilymph in the cochlear and vestibule that is important to hearing and balance. If the intracranial pressure is abruptly transferred to the inner ear, perilymph can be leak, that called perilymphatic fistula, dizziness, and hearing loss can occur suddenly. We report a 65-year-old woman who presented with acute onset dizziness and hearing loss during the epidural nerve block for back pain, wherein we speculated a possibility of perilymphatic fistula as the mechanism of hearing loss and dizziness. The mechanism of dizziness and hearing loss was suspected with perilymphatic fistula.

Original Articles

Incidence of Progression into Ménière Disease from Idiopathic Sudden Sensorineural Hearing Loss: Midterm Follow-up Study: Byeong Min Lee, Jin Hyun Seo, Hyun Woo Park, Hyun Jin Lee, Dong Gu Hur, Seong Ki Ahn; Res Vestib Sci. 2018;17(3):95-101. Published online September 18, 2018; DOI: https://doi.org/10.21790/rvs.2018.17.3.95

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Abstract PDF: Objectives
Ménière disease is a clinical syndrome characterized by the four major symptoms of episodic vertigo, sensorineural hearing loss, tinnitus, and aural fullness. Sensorineural hearing loss, especially low frequency, is the characteristic type of audiogram in Ménière's disease. However, it is difficult to distinguish idiopathic sudden sensorineural hearing loss (ISSNHL) with vertigo from the first attack of Ménière disease. The purpose of this study was to investigate the incidence of progression into Ménière Disease from low frequency ISSNHL.
Methods
Two hundred eighty-three patients were included in this study. We classified the patients with ISSNHL according to the hearing loss in audiogram and analyzed how many of them actually progressed to Ménière disease based on diagnosis criteria.
Results
Among the 240 patients, 37.1% (89 patients) were confirmed low frequency ISSNHL and 14.6% (13 patients) of them were diagnosed with Meniere disease.
Conclusions
This study showed that the progression from low frequency ISSNHL to Ménière disease was higher than other frequency ISSNHL, as in other studies.

Vestibular Function and Prognosis of Sudden Sensorineural Hearing Loss with Vertigo: Se A Lee, Hyo Jun Kim, Bo Gyung Kim, Jong Dae Lee; Res Vestib Sci. 2018;17(3):90-94. Published online September 18, 2018; DOI: https://doi.org/10.21790/rvs.2018.17.3.90

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Abstract PDF: Objectives
Vertigo combined with sudden sensorineural hearing loss (SSNHL) is known as a poor prognostic factor. We investigated clinical findings and vestibular function tests in patients of SSNHL with vertigo to find the prognostic factors.
Methods
We retrospectively evaluated data on the patients diagnosed with SSNHL with vertigo at Bucheon Soonchunhyang University Hospital from March 2009 to February 2018. We reviewed medical records and the results of vestibular function tests and audiometry.
Results
Of the 68 patients, 30 (44.1%) showed profound hearing loss and 53 (77.9%) showed poor recovery. Age and the degree of initial hearing loss showed negative prognostic factor in hearing recovery. Abnormal results of cervical vestibular evoked myogenic potentials (cVEMP) also showed significantly differences between good and poor recovery groups.
Conclusions
In this study, most of the patients of SSNHL with vertigo showed poor recovery. Age, degree of initial hearing loss, and the abnormal result of the cVEMP have a negative effect on the prognosis of hearing recovery.

Prognosis of Sudden Low Frequency Hearing Loss during Long-term Follow-up: Ji Hyung Kim, Sang Hyun Kwak, Seong Hoon Bae, Sung Hunh Kim, Gi-Sung Nam; Res Vestib Sci. 2018;17(3):102-108. Published online September 18, 2018; DOI: https://doi.org/10.21790/rvs.2018.17.3.102

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Abstract PDF

Objectives
The prognosis of sudden low frequency hearing loss (SLFHL) is relatively good, but recurrences of hearing loss and possible progression to Meniere’s disease is still a clinically important concern. This study was conducted to confirm the rate at which SLFHL proceeds to Meniere’s disease.
Methods
We retrospectively analyzed the medical records of 160 SLFHL patients who were followed up for more than 6 months from September 2005 to August 2013. Progression, initial hearing level, recovery and recurrence of hearing loss were reviewed.
Results
106 patients (66.25%) had complete hearing recovery, 32 (20%) had recurrent hearing loss. Of the 32 recurrent group, 15 (9.38%) had progressed to Meniere’s disease after average of 1.7±1.4 years. The mean age of nonrecurrent group was higher than recurrent group (55.3±14.6 and 48.0±13.4, respectively, p=0.011). The threshold of 250Hz was significantly higher in the nonrecurrent group compared with recurrent group (p=0.047).
Conclusions
In patients with SLFHL, recurrence at relatively young age should be considered with the possibility of progression to Meniere’s disease.

Citations

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Findings of Intravenous Gadolinium Inner Ear Magnetic Resonance Imaging in Patients With Acute Low-Tone Sensorineural Hearing Loss
Hee Won Seo, Yikyung Kim, Hyung-Jin Kim, Won-Ho Chung, Young Sang Cho
Clinical and Experimental Otorhinolaryngology.2023; 16(4): 334. CrossRef

Case Report

Superficial Siderosis with Peripheral Dizziness: Report of 2 Cases: Tae-Hoon Kim, Jin-Hyuk Huh, Moon-Suh Park, Jae-Yong Byun; Res Vestib Sci. 2018;17(2):60-66. Published online June 15, 2018; DOI: https://doi.org/10.21790/rvs.2018.17.2.60

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Abstract PDF

Superficial siderosis (SS) of the central nervous system is a rare disease, which is caused by the accumulation of iron from the hemoglobin in the superficial layer of the brain, spinal cord, and central parts of cranial nerves. The etiology of SS is the accumulation of hemosiderin in the subarachnoid space due to chronic or repeated hemorrhage resulting in progressive and irreversible neurological dysfunction. The cause of the disease is aneurysm, trauma, tumor, and vascular malformation. In most cases, the cause of bleeding is unknown. Clinical features include sensorineural hearing loss, cerebellar ataxia, and myelopathy. Until now, magnetic resonance imaging (MRI) has only been diagnosed and there is no standardized treatment. We will investigate clinical features and MRI findings of SS disease in the central nervous system using 2 patient cases.

Citations

Citations to this article as recorded by

A Case of Patient with Bilateral Cochleovestibular Function Loss due to Infratentorial Superficial Siderosis
Gyuman Lee, Youngmin Mun, Dae Bo Shim
Research in Vestibular Science.2023; 22(3): 83. CrossRef

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