Vestibular Functions of Hereditary Hearing Loss Patients with GJB2 Mutations

2015 ◽  
Vol 20 (3) ◽  
pp. 147-152 ◽  
Author(s):  
Keita Tsukada ◽  
Hisakuni Fukuoka ◽  
Shin-ichi Usami
Keyword(s):  
Gjb2 Gene ◽  
Caloric Test ◽  
Congenital Deafness ◽  
Lateral Semicircular Canal ◽  
Vestibular Evoked Myogenic Potential ◽  
Causative Gene ◽  
Myogenic Potential ◽  
Vestibular Testing ◽  
Vestibular Symptoms ◽  
Gjb2 Mutations

Objectives: Mutations in the GJB2 gene have been of particular interest as it is the most common causative gene for congenital deafness in all populations. Detailed audiological features, including genotype-phenotype correlations, have been well documented. However, in spite of abundant gene as well as protein expression in the vestibular end organs, neither vestibular symptoms nor vestibular functions have yet been elucidated. In the present study, vestibular functions were evaluated in patients diagnosed with GJB2-related deafness. Subjects and Methods: Vestibular functions were evaluated by caloric test and cervical vestibular evoked myogenic potential (cVEMP) testing in 24 patients with biallelic GJB2 mutations. Results and Discussion: Twenty-one of 23 patients (91.3%) had normal caloric responses and significantly lower cVEMP amplitudes than the control subjects. In the patients who were able to undergo vestibular testing, the mostly normal reactions to caloric testing indicated that the lateral semicircular canal was intact. However, the majority of GJB2 patients showed low cVEMP reactions, indicating a saccular defect.

Evaluation of Vestibular Functions in Patients with Vogt-Koyanagi-Harada Disease

2017 ◽  
Vol 22 (3) ◽  
pp. 190-195 ◽  
Author(s):  
Keishi Fujiwara ◽  
Shinya Morita ◽  
Kimiko Hoshino ◽  
Atsushi Fukuda ◽  
Yuji Nakamaru ◽  
...  
Keyword(s):  
Autoimmune Disorder ◽  
University Hospital ◽  
Caloric Test ◽  
Subjective Symptoms ◽  
Vestibular Evoked Myogenic Potential ◽  
Caloric Testing ◽  
Ocular Vemp ◽  
Cervical Vemp ◽  
Vestibular Symptoms ◽  
Vkh Disease

Vogt-Koyanagi-Harada (VKH) disease is an idiopathic, multisystem autoimmune disorder characterized by bilateral, diffuse granulomatous uveitis associated with neurological, audiovestibular, and dermatological manifestations. The purpose of this study is to investigate vestibular functions in patients with VKH disease. A total of 43 patients with VKH disease in Hokkaido University Hospital were enrolled in this study. Subjective symptoms such as dizziness or vertigo and the results of various vestibular examinations including nystagmus testing, caloric testing, and vestibular-evoked myogenic potential (VEMP) testing were investigated. Eight of 42 patients (19.0%) complained of subjective vestibular symptoms. On the other hand, 12 of 28 patients (42.9%) showed nystagmus, and 7 of 15 patients (46.7%) showed unilateral or bilateral weakness in the caloric test. VEMP testing was performed for 16 patients. Seven (43.8%) and 8 (50.0%) patients were evaluated as abnormal in cervical VEMP and ocular VEMP testing, respectively. The rate of detection of nystagmus was significantly higher than that of subjective symptoms. As vestibular dysfunction in patients with VKH disease cannot be detected through history taking alone, nystagmus testing, caloric testing, and VEMP testing should be performed to evaluate vestibular functions associated with VKH disease. It is considered that abnormal VEMP findings are associated with otolith organ dysfunction.

Prognosis of patients with idiopathic sudden hearing loss: role of vestibular assessment

2010 ◽  
Vol 125 (3) ◽  
pp. 251-257 ◽  
Author(s):  
S Korres ◽  
G A Stamatiou ◽  
E Gkoritsa ◽  
M Riga ◽  
J Xenelis
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Sudden Hearing Loss ◽  
Caloric Test ◽  
Test Results ◽  
Vestibular Evoked Myogenic Potential ◽  
Early Recovery ◽  
Profound Hearing Loss ◽  
Myogenic Potential ◽  
Vestibular Assessment

AbstractObjective:To evaluate the correlation between caloric and vestibular evoked myogenic potential test results, initial audiogram data, and early hearing recovery, in patients with idiopathic sudden hearing loss.Materials and methods:One hundred and four patients with unilateral idiopathic sudden hearing loss underwent complete neurotological evaluation. Results for vestibular evoked myogenic potential and caloric testing were compared with patients' initial and final audiograms.Results:Overall, abnormal vestibular evoked myogenic potential responses occurred in 28.8 per cent of patients, whereas abnormal caloric test results occurred in 50 per cent. A statistically significant relationship was found between the type of inner ear lesion and the incidence of profound hearing loss. Moreover, a negative correlation was found between the extent of the inner ear lesion and the likelihood of early recovery.Conclusion:In patients with idiopathic sudden hearing loss, the extent of the inner ear lesion tends to correlate with the severity of cochlear damage. Vestibular assessment may be valuable in predicting the final outcome.

scholarly journals Changes in Vestibular Function in Patients With Head-and-Neck Cancer Undergoing Chemoradiation

2020 ◽  
pp. 014556132094948
Author(s):  
Roland Hülse ◽  
Boris A. Stuck ◽  
Karl Hörmann ◽  
Nicole Rotter ◽  
Julia Nguyen ◽  
...  
Keyword(s):  
Head And Neck Cancer ◽  
Head And Neck ◽  
Neck Cancer ◽  
Pure Tone ◽  
Pure Tone Audiometry ◽  
Head Impulse Test ◽  
Video Head Impulse Test ◽  
Vestibular Evoked Myogenic Potential ◽  
Myogenic Potential ◽  
Vestibular Testing

Introduction: While the cochleotoxicity of cisplatin has been well investigated, less is known about the effects of platinum-based chemotherapy on the vestibular system. In particular, there is a lack of prospective studies using modern laboratory vestibular testing that examine the effects of cisplatin on the semicircular canals and on the otolith organs. The aim of the present study was, therefore, to investigate the vestibulotoxic effect of cisplatin in patients with head and neck tumors who are undergoing chemoradiation. Methods: Forty-five patients undergoing cisplatin-based chemoradiation for head and neck cancer received a vestibular assessment consisting of anamnesis, a horizontal video head impulse test (vHIT), ocular and cervical vestibular evoked myogenic potential testing, as well as pure tone audiometry. This assessment was performed before therapy, 6 weeks after therapy, and 3 months after therapy. Results: Video head impulse test showed a significantly reduced median gain 6 weeks after chemoradiation. In addition, significantly more refixational saccades could be detected after therapy. Vestibular evoked myogenic potential testing results also revealed significant changes, whereas pure tone audiometry did not. None of the patients mentioned “dizziness” during the follow-up examinations. Conclusion: We demonstrated a vestibulotoxic effect of cisplatin-based chemoradiation in patients with head and neck cancer. Future studies are needed to better understand cisplatin-induced vestibulotoxicity and to identify possible vestibuloprotective substances. Still, before and after chemoradiation, patients should undergo not only auditory testing but also vestibular testing in order to detect potential vestibular loss as soon as possible and to quickly initiate vestibular physiotherapy.

scholarly journals Case Report: Isolated Idiopathic Saccular Dysfunction

2021 ◽  
Vol 12 ◽  
Author(s):  
Sofia Waissbluth ◽  
Javier Oyarzún
Keyword(s):  
Case Report ◽  
Semicircular Canal ◽  
Clinical Presentation ◽  
Potential Response ◽  
Vestibular Evoked Myogenic Potential ◽  
True Incidence ◽  
Myogenic Potential ◽  
Vestibular Testing ◽  
History Of ◽  
The Right

Advances in vestibular testing have now allowed us to test each semicircular canal as well as the utricle and saccule, independently. This has led to the discovery of new patterns of vestibular dysfunction that were once impossible to evaluate. This report describes the case of a 20-year-old woman with a 2-month history of recurrent dizziness. She had a complete audiovestibular assessment. The only abnormality observed was the absence of a cervical vestibular-evoked myogenic potential response for the right side, hence an isolated saccular dysfunction. In conclusion, isolated otolithic dysfunction is probably an overlooked and neglected clinical presentation. Its true incidence is unknown, and further research is needed to understand this clinical entity.

Clinical Experience with the Vestibular Evoked Myogenic Potential

2004 ◽  
Vol 15 (03) ◽  
pp. 198-215 ◽  
Author(s):  
David A. Zapala ◽  
Robert H. Brey
Keyword(s):  
Test Performance ◽  
False Positive ◽  
Patient Population ◽  
Clinical Performance ◽  
Tone Burst ◽  
Caloric Test ◽  
Older Patient ◽  
Vestibular Evoked Myogenic Potential ◽  
Myogenic Potential ◽  
Age Related

The vestibular evoked myogenic potential (VEMP) is a promising test of the descending vestibulocollic system. Our aim was to determine whether the VEMP can be applied to an older patient population and can detect lesions in descending vestibulospinal pathways. We also compared VEMP clinical performance with that of the standard caloric test. VEMP test performance was retrospectively analyzed in relation to clinical diagnosis and other vestibular test performance in 62 patients (age, 30–85 years) referred for vestibular testing to Mayo Clinic, Jacksonville, Florida. The VEMP was evoked using a 250 Hz tone burst. Results suggest age-related changes in VEMP amplitude and latency in this patient population. VEMP tests were sensitive to lesions not detected by electronystagmography. VEMP and caloric sensitivity and specificity were essentially equal (d′ = 1). Combining both tests improved sensitivity. However, VEMP false-positive rates hampered specificity. VEMP testing may be refined to improve false-positive rates and clinical utility.

Indication of the Side of Delayed Endolymphatic Hydrops by Vestibular Evoked Myogenic Potential and Caloric Test

ORL ◽  
2010 ◽  
Vol 72 (5) ◽  
pp. 242-246 ◽  
Author(s):  
Naoya Egami ◽  
Munetaka Ushio ◽  
Tatsuya Yamasoba ◽  
Toshihisa Murofushi ◽  
Shinichi Iwasaki
Keyword(s):  
Endolymphatic Hydrops ◽  
Caloric Test ◽  
Vestibular Evoked Myogenic Potential ◽  
Myogenic Potential ◽  
Delayed Endolymphatic Hydrops
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