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1.
J Otolaryngol Head Neck Surg ; 53: 19160216241265685, 2024.
Article in English | MEDLINE | ID: mdl-39113464

ABSTRACT

BACKGROUND: Correctly diagnosing dizziness in children is essential for appropriate management; nevertheless, healthcare professionals face challenges due to children's limited ability to describe their symptoms and their cooperation during physical examination. The objective of this study is to describe the first 100 patients seen at a newly established pediatric vertigo center. METHODS: This is a retrospective review of a consecutive series of 100 patients seen at our pediatric vertigo clinic in a tertiary referral center from August 2019 until June 2022. Comprehensive clinical data were collected. The diagnoses were established by 2 pediatric otolaryngologists based on validated diagnostic criteria. Trends in diagnosis, investigation, and treatment of these patients were analyzed. RESULTS: A total of 100 children were included in the study. Vestibular migraine was the most common diagnosis (20%) followed by benign paroxysmal vertigo of childhood (14%). Eleven patients had combined pathologies. Fifteen out of 70 children (21%) had abnormal audiograms, 30 out of 48 children (62.5%) had abnormal vestibular testing, and 6 out of 31 (19%) patients had abnormal imaging. Fifty-one children received medical treatment, 23 received vestibular physiotherapy, and 9 patients had particle repositioning maneuvers; moreover, 17 of these patients received multimodal treatment. CONCLUSIONS: Our analysis suggests that imaging and audiology testing have relatively low yield in the assessment of pediatric vertigo. On the other hand, vestibular testing detected a high proportion of abnormalities, such as saccadic pursuit, vertical nystagmus, central positional nystagmus, and abnormal directional preponderance, particularly associated with vestibular migraine. Given the complexity of diagnosing vertigo in children, it is critical to establish multidisciplinary specialized centers capable of providing accurate diagnosis and treatment for these children.


Subject(s)
Vertigo , Humans , Retrospective Studies , Male , Child , Female , Child, Preschool , Adolescent , Vertigo/diagnosis , Vertigo/therapy , Migraine Disorders/diagnosis , Migraine Disorders/therapy , Vestibular Function Tests , Benign Paroxysmal Positional Vertigo/therapy , Benign Paroxysmal Positional Vertigo/diagnosis , Tertiary Care Centers
2.
Codas ; 36(5): e20230241, 2024.
Article in Portuguese, English | MEDLINE | ID: mdl-39109754

ABSTRACT

PURPOSE: Propose normalization values of the Horus® computerized posturography platform, in children aged 4 to 6 years, without auditory and/or vestibular complaints. METHODS: Cross-sectional study, 216 children aged 4 to 6 years participated. All the children underwent to visual screening, audiological evaluation and computerized posturography, which consists of research on stability limits and seven sensory conditions. The results were statistically analyzed using the tests non-parametric Kruskal-Walli, post hoc Dunn-Bonferroni for pairwise age comparisons and the Mann-Whitney U for sex analysis. Categorical data were presented in relative frequency and quantitative data in mean and standard deviation. RESULTS: Standardization values were described for the stability limit and for the seven sensory conditions. There was a difference for the stability limit between sex at 4 years old(p<0.007) and, in the comparison between ages 4 and 5 (p=0.005) and 4 and 6 years old(p<0.001). In the residual functional balance, comparison between ages, there was a difference between 4 and 5, 4 and 6, 5 and 6 years, however for different data. The presence of statistical difference for different evaluation data also occurred in the analysis by sex. In the sensory systems, the findings between ages showed differences for the vestibular system, right and left optokinetic visual dependence, tunnel visual dependence and for the composite balance index. CONCLUSION: It was possible to establish normative values for the Horus® posturography in healthy children aged 4 to 6 years.


OBJETIVO: Propor valores de normatização da plataforma de posturografia computadorizada Horus®, em crianças de 4 a 6 anos, sem queixas auditivas e/ou vestibulares. MÉTODO: Estudo transversal. Participaram 216 crianças na faixa etária de 4 a 6 anos. Todas realizaram triagem visual, avaliação auditiva e posturografia computadorizada composta por pesquisa do limite de estabilidade e sete condições sensoriais. Analisaram-se os resultados estatisticamente por testes não paramétrico Kruskal-Walli, post hoc Dunn-Bonferroni para comparações par-a-par nas idades e U de Mann-Whitney para análise entre sexo. Os dados categóricos foram apresentados em frequência relativa e os dados quantitativos pela média e desvio padrão. RESULTADOS: Foram descritos valores de normatização para o limite de estabilidade e para as sete condições sensoriais. Houve diferença para o limite de estabilidade entre sexos aos 4 anos (p<0,007) e, na comparação entre as idades 4 e 5 anos (p=0,005) e 4 e 6 anos (p<0,001). No equilíbrio funcional residual, comparação entre idades, houve diferença entre 4 e 5, 4 e 6 e, 5 e 6 anos, entretanto para diferentes dados. A presença de diferença estatística para diferentes dados da avaliação, ocorreu também na análise por sexo. Nos sistemas sensoriais os achados entre idades mostraram diferença para o sistema vestibular, dependência visual optocinética direita e esquerda, dependência visual túnel e para índice de equilíbrio composto. Sugere-se que para esta população, as respostas na posturografia sejam analisadas por faixa etária e sexo. CONCLUSÃO: Foi possível estabelecer valores normativos para a posturografia Horus® em crianças hígidas na faixa etária de 4 a 6 anos.


Subject(s)
Postural Balance , Humans , Cross-Sectional Studies , Child, Preschool , Male , Female , Reference Values , Child , Postural Balance/physiology , Vestibular Function Tests/methods , Vestibular Function Tests/instrumentation , Vestibular Function Tests/standards , Diagnosis, Computer-Assisted/standards , Diagnosis, Computer-Assisted/methods
3.
J Int Adv Otol ; 20(3): 247-254, 2024 May.
Article in English | MEDLINE | ID: mdl-39128093

ABSTRACT

BACKGROUND:  Fibromyalgia syndrome (FMS) is a chronic pain condition that may be associated with dysfunction in the central nervous system. OBJECTIVE: The aim of this study was to assess the vestibulo-spinal reflex (VSR) and vestibulo-ocular reflex (VOR) in FMS using the cervical vestibular evoked myogenic potential (cVEMP) and ocular vestibular evoked myogenic potential (oVEMP) tests, respectively, and to evaluate their relation to disease severity. METHODS:  This study included 30 female FMS patients and 30 well-matched healthy controls. They underwent full history taking and assessment of the severity of dizziness/vertigo using the Dizziness Handicap Inventory; assessment of the severity of FMS symptoms using the Revised Fibromyalgia Impact Questionnaire; bedside examination of the dizzy patient; videonystagmography, cVEMP, and oVEMP tests; basic audiologic evaluation; and uncomfortable loudness level (UCL) testing. RESULTS:  Dizziness was reported in 46.6% and vertigo in 11.1% of patients. Abnormalities in cVEMP (50%) and oVEMP (63.3%) were mostly unilateral, irrespective of FMS severity. Disease duration affected only the oVEMP amplitude. Fibromyalgia syndrome patients had a statistically significant lower UCL and narrower dynamic range compared to controls. CONCLUSION:  The VSR and VOR are commonly affected in FMS patients, and findings suggest central sensitization involving the brain stem. We recommend routine cVEMP and oVEMP testing to assess brainstem function in FMS patients.


Subject(s)
Fibromyalgia , Reflex, Vestibulo-Ocular , Vestibular Evoked Myogenic Potentials , Humans , Fibromyalgia/physiopathology , Fibromyalgia/complications , Fibromyalgia/diagnosis , Female , Vestibular Evoked Myogenic Potentials/physiology , Adult , Middle Aged , Reflex, Vestibulo-Ocular/physiology , Case-Control Studies , Dizziness/physiopathology , Dizziness/etiology , Dizziness/diagnosis , Vertigo/physiopathology , Vertigo/diagnosis , Vertigo/etiology , Severity of Illness Index , Vestibular Function Tests/methods
4.
J Int Adv Otol ; 20(3): 241-246, 2024 May.
Article in English | MEDLINE | ID: mdl-39128107

ABSTRACT

BACKGROUND:  Assessment of nystagmus fixation suppression can be used as an additional diagnostic tool for patients with an acute vestibular syndrome to distinguish between a central or peripheral cause. We investigated the ability of physicians to detect fixation suppression using a nystagmus simulation model. METHODS:  We used a nystagmus simulator to measure the accuracy of the nystagmus fixation suppression test. Fixation suppression was assessed randomly in 6170 trials by 20 otorhinolaryngologists and neurologists, segregated into 2 groups based on their neurootological experience, a beginner and an experienced group. The simulator presented random nystagmus slow velocity (SPV) reductions and presented 3 conditions with either changed nystagmus frequency, amplitude, or both. RESULTS:  The cutoff for the discernment of fixation suppression ranged from 1.2 to 14°/s nystagmus velocity difference. The more intense the baseline nystagmus was, the more difficult was the detection of fixation suppression. There was not significant difference (P >.05) in the cutoff values in the experts group compared to the novices for all 3 different conditions. Both, novices and experts, detected frequency changes easier than differences of the nystagmus amplitude. Test sensitivity was very low (19%-65%) for discernment of small nystagmus velocity differences of <2°/s by experts. CONCLUSION:  In our study, there was no difference between experts and novices in detection of nystagmus suppression by visual fixation. The examiners could only detect large suppression effects at low-intensity baseline nystagmus. Overall, the sensitivity and accuracy of a clinical fixation suppression test is low and the assistance with a video-oculography device is highly recommended.


Subject(s)
Fixation, Ocular , Nystagmus, Pathologic , Vestibular Diseases , Vestibular Function Tests , Humans , Nystagmus, Pathologic/diagnosis , Nystagmus, Pathologic/physiopathology , Fixation, Ocular/physiology , Vestibular Function Tests/methods , Vestibular Diseases/diagnosis , Vestibular Diseases/physiopathology , Sensitivity and Specificity , Clinical Competence , Male , Female , Adult , Nystagmus, Physiologic/physiology
5.
Otol Neurotol ; 45(8): 925-931, 2024 Sep 01.
Article in English | MEDLINE | ID: mdl-39142314

ABSTRACT

OBJECTIVES: To evaluate the differences between audio-vestibular function testing and inner ear gadolinium magnetic resonance imaging (MRI) in distinguishing definite Ménière disease (DMD) and probable Ménière disease (PMD), and to provide a reference for early clinical diagnosis and intervention. METHODS: A total of 116 patients diagnosed with DMD (n = 80) and PMD (n = 36) were enrolled. The differences in the results of pure tone audiometry, caloric test, and tympanic injection of gadolinium for contrast-enhanced MRI between the two groups were compared and analyzed. Parameters that could differentiate between the two conditions were identified, and the sensitivity and specificity and the area under the curve (AUC) of individual and combined indices in the differential diagnosis of DMD and PMD were evaluated. RESULTS: The hearing threshold and hearing asymmetry rate of the DMD group were significantly higher than those of the PMD group (p < 0.001), 98.8% and 30.6%, respectively. The abnormal rates of canal paresis (CP) and severity of endolymphatic hydrops in the DMD group were higher than those in the PMD group (p < 0.05). When combined with high-frequency hearing thresholds, hearing asymmetry, hearing curve type, endolymphatic hydrops, and abnormal CP, the diagnostic accuracy of DMD was improved compared to using high-frequency alone (p < 0.05). CONCLUSION: This study showed that PMD and DMD may represent two different stages in the development of MD disease. The comprehensive assessment of audio-vestibular function testing and inner ear MRI proves beneficial for early diagnosis, potentially contributing to the preservation of inner ear function.


Subject(s)
Ear, Inner , Magnetic Resonance Imaging , Meniere Disease , Vestibular Function Tests , Humans , Meniere Disease/diagnostic imaging , Meniere Disease/diagnosis , Male , Female , Magnetic Resonance Imaging/methods , Middle Aged , Adult , Ear, Inner/diagnostic imaging , Ear, Inner/physiopathology , Vestibular Function Tests/methods , Aged , Diagnosis, Differential , Audiometry, Pure-Tone/methods , Caloric Tests , Gadolinium , Sensitivity and Specificity
6.
Article in Chinese | MEDLINE | ID: mdl-39118506

ABSTRACT

Objective:To establish the normal values of subjective visual vertical (SVV) in different head deflection angles and analyze its test and retest reliability, in order to provide a reference for the clinical application of SVV in the evaluation of vestibular disorders. Methods:Thirty-one healthy young people were selected to wear VR glasses, and the SVV data were tested in five different head-tilt, namely, 0° in the upright head position, 45°in the left head position, 45° in the right head position, 90° in the left head position, and 90° in the right head position, and were re-tested 2 weeks later. Results:①The mean values of SVV at 5 different head-tilt angles of 0°, left 45°, right 45°, left 90°, and right 90° were -0.07±1.71, 4.30±5.39, -6.51±5.58, -3.76±7.42, and 0.40±8.02, respectively, The 95% confidence limits of SVV at 0°, left 45°, right 45°, left 90°, right 90°, and right 90° were (-3.42, 3.28), (-6.26, 14.86), (-17.45, 4.43), (-18.30, 10.78), and(-15.32, 16.12), respectively; ②The absolute values of SVV at 4 different head-tilt angles of left 45°, right 45°, left 90°, and right 90° were 5.62±3.96, 6.90±5.07, 6.82±4.70 and 6.48±4.68, respectively. The 95% confidence limits of SVV at left 45°, right 45°, left 90°, right 90°, and right 90° were(0,12.11),(0,15.21),(0,14.53)and(0,14.16), respectively. The asymmetry ratio is 10% for the absolute value of the 45 ° deviation and 3% for the absolute value of the 90° deviation; ③Intra-class correlation coefficients(ICC) for 0°, left 45°, right 45°, left 90°, right 90°were 0.757, 0.673, 0.674, 0.815, and 0.856, respectively. Conclusion:SVV has good retest reliability and high stability, and the SVV normal value data of different head deviation angles established in the present study can be used as a reference for the diagnosis and evaluation of vestibular disorders.


Subject(s)
Head , Humans , Reproducibility of Results , Young Adult , Male , Female , Adult , Posture , Vestibular Diseases/diagnosis , Reference Values , Vestibular Function Tests/methods
7.
J Int Adv Otol ; 20(2): 164-170, 2024 Mar 27.
Article in English | MEDLINE | ID: mdl-39145690

ABSTRACT

Hyperventilation-induced nystagmus test (HINT) is capable of generating a response in 77.2% of cases of acute unilateral vestibulopathy (AUVP); both nystagmus toward the affected side (excitatory pattern) and toward the healthy side (inhibitory pattern) have been described. The aim of the study is to investigate the clinical and prognostic role of the test by evaluating its correlation with vestibulo-ocular reflex (VOR) gain. We evaluated 33 AUVP patients by performing the HINT and video head impulse test (V-HIT) during the acute phase and then at 15 and 90 days after the onset of the symptoms. The correlation between the VOR gain of the affected side and test responses was evaluated first, phase by phase, and then considering the pattern shown during the first assessments. Patients with a negative HINT had a higher mean VOR gain than patients with a positive test at both 15 and 90 days. Patients who showed an inhibitory pattern at the first assessment had a continuous improvement in V-HIT performance, while patients with an initial excitatory response had a transient decrease in gain at the subsequent evaluation (P=.001). No difference between these 2 groups emerged at 90 days (P=.09). The finding of a negative HINT during the follow-up correlates with good V-HIT performance and could be an indicator of good recovery. The inhibitory pattern is associated with a subsequent improvement; and it would be indicative of compensation. but, despite this, the prognostic value of the test is limited.


Subject(s)
Head Impulse Test , Hyperventilation , Nystagmus, Pathologic , Reflex, Vestibulo-Ocular , Humans , Reflex, Vestibulo-Ocular/physiology , Male , Hyperventilation/physiopathology , Hyperventilation/complications , Female , Middle Aged , Nystagmus, Pathologic/physiopathology , Nystagmus, Pathologic/diagnosis , Head Impulse Test/methods , Adult , Aged , Acute Disease , Vestibular Diseases/physiopathology , Vestibular Diseases/diagnosis , Vestibular Function Tests/methods , Prognosis
8.
Pediatr Neurol ; 158: 86-93, 2024 Sep.
Article in English | MEDLINE | ID: mdl-39002355

ABSTRACT

BACKGROUND: To improve diagnostic precision in pediatric vertigo, particularly in Vestibular Migraine of Childhood (VMC), probable VMC (pVMC), Recurrent Vertigo of Childhood (RVC), and unspecified categories, by delineating clinical characteristics and prevalence to refine diagnostics and treatments. METHODS: Retrospective analysis of 102 pediatric patients (five to 18 years; 46 females, 56 males) at the Dizziness Center of the Otolaryngology Department in a tertiary-level hospital from January 2019 to December 2023. Patients were classified into VMC, pVMC, RVC, and indeterminate groups. Evaluations included audiometry and vestibular tests (video head impulse test [vHIT] or caloric testing), conducted in the audiology unit and vestibular testing laboratory. Data were analyzed using IBM SPSS 20.0. RESULTS: Diagnoses were 8.8% VMC, 31.4% pVMC, 51.0% RVC, and 8.8% indeterminate. Nausea and vomiting were common in VMC and pVMC; cochlear symptoms like tinnitus and hearing loss predominated in VMC. Although vestibular testing showed no significant group differences, VMC had more vHIT abnormalities and RVC had more caloric test anomalies. CONCLUSIONS: This study highlights the need for comprehensive diagnostics in pediatric vestibular disorders, revealing unique and overlapping traits across VMC, pVMC, and RVC. Insights call for further research to refine diagnostic criteria and improve treatment methods.


Subject(s)
Migraine Disorders , Tertiary Care Centers , Vertigo , Humans , Female , Male , Child , Adolescent , Vertigo/diagnosis , Vertigo/etiology , Migraine Disorders/diagnosis , Migraine Disorders/complications , Retrospective Studies , Child, Preschool , Vestibular Diseases/diagnosis , Recurrence , Vestibular Function Tests , Head Impulse Test , Caloric Tests
9.
FP Essent ; 542: 14-22, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39018126

ABSTRACT

Vertigo, an unexpected feeling of self-motion, is no longer characterized simply by symptom quality but by using triggers and timing. Evaluating vertigo by triggers and timing not only distinguishes serious central causes from benign peripheral causes, but also narrows the differential diagnosis by further classifying vertigo as spontaneous episodic vestibular syndrome, triggered episodic vestibular syndrome, or acute vestibular syndrome. A targeted physical examination can then be used to further delineate the cause within each of these three vestibular categories. Neuroimaging and vestibular testing are not routinely recommended. In the management of vertigo, vestibular hypofunction can be treated with vestibular rehabilitation, which can be self-administered or directed by a physical therapist. Pharmacotherapy sometimes is indicated for vertigo based on triggers, timing, and the specific condition, but it is not always beneficial and is used more often for symptom reduction than as a cure. Transtympanic corticosteroid or gentamicin injections are recommended for patients who do not benefit from nonablative therapy. Surgical ablative therapy is reserved for patients who have not benefited from less definitive therapy and have nonusable hearing.


Subject(s)
Vertigo , Humans , Vertigo/therapy , Vertigo/diagnosis , Vertigo/etiology , Diagnosis, Differential , Physical Examination/methods , Family Practice/methods , Gentamicins/therapeutic use , Anti-Bacterial Agents/therapeutic use , Vestibular Function Tests/methods
10.
Otol Neurotol ; 45(8): e607-e613, 2024 Sep 01.
Article in English | MEDLINE | ID: mdl-39052909

ABSTRACT

OBJECTIVES: Lateral semicircular canal BPPV (LSC-BPPV) is diagnosed with the Head Yaw Test (HYT) by observing nystagmus direction and comparing the nystagmus intensity on both sides according to Ewald's laws. Head Pitching Test (HPT) is a diagnostic maneuver performed in the upright position by bending the patient's head forward (bowing) and backward (leaning) and observing the evoked nystagmus. We aimed to assess the sensitivity of HPT in correctly diagnosing LSC-BPPV through the quantitative measurement of Bowing and Leaning nystagmus slow-phase velocity (SPV). METHODS: One hundred cases of LSC-BPPV were prospectively enrolled. HPT was performed, looking for pseudospontaneous, bowing, and leaning nystagmus. HYT was considered for the "final diagnosis." HPT was defined as "diagnostic" if the nystagmus was present in at least one position, "undiagnostic" if no nystagmus was detectable. The direction and the SPV of nystagmus in all positions were analyzed and compared to determine the degree of agreement between HPT and HYT. OUTCOMES: Sixty-four geotropic and 36 apogeotropic forms were diagnosed. HPT was diagnostic in 80 cases, with no difference between the two forms. According to Ewald's laws, the direction of stronger nystagmus evoked by HPT agreed with the HYT results in 39/52 (75%) cases in geotropic forms and 21/28 (75%) cases in apogeotropic forms. The agreement between HPT and HYT was "substantial" considering all the cases and "almost complete" considering only the patients with diagnostic HPT. CONCLUSION: Quantitative HPT is a valid test in diagnosing the affected side and form of LSC-BPPV, even if less reliable than HYT.


Subject(s)
Benign Paroxysmal Positional Vertigo , Nystagmus, Pathologic , Semicircular Canals , Humans , Male , Female , Benign Paroxysmal Positional Vertigo/diagnosis , Benign Paroxysmal Positional Vertigo/physiopathology , Middle Aged , Aged , Nystagmus, Pathologic/diagnosis , Nystagmus, Pathologic/physiopathology , Adult , Semicircular Canals/physiopathology , Semicircular Canals/physiology , Prospective Studies , Vestibular Function Tests/methods , Head Movements/physiology , Sensitivity and Specificity , Aged, 80 and over
11.
Otol Neurotol ; 45(8): e588-e594, 2024 Sep 01.
Article in English | MEDLINE | ID: mdl-39052916

ABSTRACT

INTRODUCTION: Recently, end-point nystagmus, traditionally observed in an upright position, has been identified in the Dix-Hallpike position among healthy subjects, suggesting a physiological origin.However, its characteristics in individuals with vestibular hypofunction remain unexplored. OBJECTIVE: To elucidate the impact of vestibular hypofunction on the characteristics of positional end-point nystagmus. METHODS: Thirty-one patients diagnosed with acute unilateral vestibulopathy according to Bárány Society criteria were selected. A video head impulse test was conducted in all participants, followed by McClure and Dix-Hallpike maneuvers with and without gaze fixation, and with the initial position of the eye in the straight-ahead position or in the horizontal end-point position. Nystagmus direction, sense, latency, slow-phase velocity, and duration were recorded. The relationship between these characteristics and video head impulse test values was analyzed. RESULTS: Positional end-point nystagmus was observed in 92.6% of subjects with vestibular hypofunction, significantly more than in healthy individuals. Nystagmus direction varied depending on the performed positional test and on the vestibulo-ocular reflex gains. Gaze occlusion and the initial horizontal end-point position increased its frequency. CONCLUSION: Vestibular hypofunction influences the manifestation of positional end-point nystagmus. Recognizing this nystagmus can aid in resolving diagnostic uncertainties and preventing the misdiagnosis of benign paroxysmal positional vertigo in subjects with acute unilateral vestibulopathy.


Subject(s)
Head Impulse Test , Nystagmus, Pathologic , Humans , Male , Female , Middle Aged , Aged , Nystagmus, Pathologic/diagnosis , Nystagmus, Pathologic/physiopathology , Adult , Head Impulse Test/methods , Nystagmus, Physiologic/physiology , Vestibular Diseases/diagnosis , Vestibular Diseases/physiopathology , Aged, 80 and over , Vestibular Function Tests/methods , Vestibular Neuronitis/diagnosis , Vestibular Neuronitis/physiopathology , Vestibular Neuronitis/complications , Reflex, Vestibulo-Ocular/physiology
12.
Medicina (B Aires) ; 84(3): 555-559, 2024.
Article in Spanish | MEDLINE | ID: mdl-38907973

ABSTRACT

Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) is a late onset neurodegenerative disorder. Its genetic basis has recently been identified in the gene encoding a subunit of the Replication Factor C (RFC1). We present the case of a 62-year-old woman who experienced a history of a biphasic presentation of imbalance and gait disorders, with rapid onset of symptoms followed by slow and progressive neurological deterioration. The diagnostic process was challenging, and numerous tests were conducted to rule out acquired and genetic causes of ataxia, leading to a diagnosis of late-onset idiopathic cerebellar ataxia. Subsequently, vestibular function tests identified severe bilateral vestibulopathy. This led to considering CANVAS among the diagnoses, which was ultimately confirmed through genetic testing (biallelic expansion of the pentanucleotide AAGGG in the RFC1 gene). This case highlights the importance of this new described genetic disease and its subacute presentation variant, emphasizing the relevance of objective vestibular function tests in idiopathic ataxias to achieve proper diagnosis and eventual genetic counseling for offspring.


El síndrome de ataxia cerebelosa, neuropatía y arreflexia vestibular (CANVAS) es un trastorno neurodegenerativo progresivo que se manifiesta en etapas tardías de la vida. Su base genética ha sido recientemente identificada en el gen que codifica la subunidad 1 del factor C de replicación (RFC1). Presentamos el caso de una mujer de 62 años con una historial de desequilibrio y deterioro de la marcha de presentación bifásica, con un inicio rápido de los síntomas seguido de un deterioro neurológico lento y progresivo. El proceso diagnóstico fue complejo y se realizaron numerosas pruebas para descartar causas adquiridas y genéticas de la ataxia, arribando al diagnóstico de ataxia cerebelosa de inicio tardío idiopática. Ulteriormente, las pruebas de función vestibular identificaron una grave vestibulopatía bilateral. Esto llevó a considerar el CANVAS entre los diagnósticos, que finalmente fue confirmado mediante pruebas genéticas (expansión bialélica del penta-nucleótido AAGGG en el gen RFC1). Este caso subraya la importancia de esta nueva enfermedad genética y su variante de presentación subaguda y enfatiza la relevancia de las pruebas objetivas de función vestibular en las ataxias consideradas idiopáticas para lograr un diagnóstico adecuado y un eventual asesoramiento genético a la descendencia.


Subject(s)
Cerebellar Ataxia , Humans , Female , Middle Aged , Cerebellar Ataxia/genetics , Cerebellar Ataxia/diagnosis , Bilateral Vestibulopathy/diagnosis , Bilateral Vestibulopathy/genetics , Bilateral Vestibulopathy/complications , Syndrome , Replication Protein C/genetics , Vestibular Function Tests
13.
J Otolaryngol Head Neck Surg ; 53: 19160216241250354, 2024.
Article in English | MEDLINE | ID: mdl-38888938

ABSTRACT

OBJECTIVES: This study aims to provide an overview of dizziness post head injury in those with prominent features for central vestibular dysfunction (CVD) in comparison to those with a post-traumatic peripheral vestibular etiology. STUDY DESIGN: Retrospective. SETTING: University Health Network (UHN) Workplace Safety and Insurance Board (WSIB) database from 1988 to 2018 were evaluated for post-traumatic dizziness. METHODS: The UHN WSIB neurotology database (n = 4291) between 1998 and 2018 was retrospectively studied for head-injured workers presenting with features for CVD associated with trauma. All patients had a detailed neurotological history and examination, audiovestibular testing that included video nystagmography (VNG) and cervical vestibular-evoked myogenic potentials (cVEMPs). Imaging studies including routine brain and high-resolution temporal bone computed tomography (CT) scans and/or intracranial magnetic resonance imaging (MRI) were available for the majority of injured workers. RESULTS: Among 4291 head-injured workers with dizziness, 23 were diagnosed with features/findings denoting CVD. Complaints of imbalance were significantly more common in those with CVD compared to vertigo and headache in those with peripheral vestibular dysfunction. Atypical positional nystagmus, oculomotor abnormalities and facial paralysis were more common in those with CVD. CONCLUSION: Symptomatic post-traumatic central vestibular injury is uncommon. It occurred primarily following high-impact trauma and was reflective for a more severe head injury where shearing effects on the brain often resulted in diffuse axonal injury. Complaints of persistent imbalance and ataxia were more common than complaints of vertigo. Eye movement abnormalities were highly indicative for central nervous system injury even in those with minimal change on CT/MRI.


Subject(s)
Craniocerebral Trauma , Dizziness , Vestibular Diseases , Humans , Retrospective Studies , Male , Craniocerebral Trauma/complications , Craniocerebral Trauma/physiopathology , Female , Adult , Dizziness/etiology , Dizziness/physiopathology , Middle Aged , Vestibular Diseases/etiology , Vestibular Diseases/physiopathology , Vestibular Diseases/diagnosis , Vestibular Evoked Myogenic Potentials , Vestibular Function Tests , Magnetic Resonance Imaging , Tomography, X-Ray Computed
14.
J Neurol ; 271(8): 4909-4924, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38748235

ABSTRACT

Patients with persistent postural-perceptual dizziness (PPPD) perceive postural instability larger than the observed sway. It is unknown whether the concept of postural misperception prevails during vestibular stimulation and whether it may account for the unsteadiness patients complain during body movements. We tested the hypothesis of an abnormal sensory-perceptual scaling mechanism in PPPD by recording objective, perceived, and the reproduced postural sway under various standing conditions, modulating visual and proprioceptive input, by binaural galvanic vestibular stimulation (GVS). We related postural sway speed to individual vestibular motion perceptional thresholds and disease-related PPPD questionnaires in 32 patients and 28 age-matched healthy control subjects (HC). All participants showed normal vestibular function tests on quantitative testing at the time of enrollment. The perception threshold of GVS was lower in patients. Compared to HC, patients showed and perceived larger sway on the firm platform. With GVS, posturo-perceptual ratios did not show group differences. The ratio of reproduced to real postural sway showed no group differences indicating normal postural sway perception during vestibular stimulation. Noticeably, only in patients, reproduced postural instability became larger with lower individual thresholds of vestibular motion detection. We conclude that posturo-perceptual (metacognitive) scaling of postural control seems to be largely preserved in PPPD during GVS. Vestibular stimulation does not destabilize patients more than HC, even in challenging postural conditions. Low individual thresholds of vestibular motion perception seem to facilitate instability and postural misperception on solid grounds. This conclusion is important for an effective physical therapy with vestibular exercises in PPPD.


Subject(s)
Dizziness , Motion Perception , Postural Balance , Humans , Male , Female , Motion Perception/physiology , Middle Aged , Postural Balance/physiology , Dizziness/physiopathology , Aged , Adult , Electric Stimulation , Vestibule, Labyrinth/physiology , Vestibule, Labyrinth/physiopathology , Sensory Thresholds/physiology , Posture/physiology , Vestibular Function Tests/methods , Proprioception/physiology
15.
Neurologist ; 29(4): 238-242, 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38797929

ABSTRACT

OBJECTIVE: Acute unilateral peripheral vestibulopathy (AUPVP) is a frequent form of peripheral vestibular vertigo characterized by unilateral vestibular organ dysfunction. Diagnostic challenges in anamnesis and bedside examination can lead to potential misdiagnoses. This study investigated the sensitivity of bedside examinations in diagnosing AUPVP. METHODS: This retrospective analysis examined 136 AUPVP inpatients at a level 3 university hospital between 2017 and 2019. Demographic data and bedside test results were collected. Instrumental otoneurological tests included caloric testing and video head impulse test (HIT). The sensitivity of each bedside parameter was computed based on the instrumental diagnostics, and statistical analyses were performed. RESULTS: The study included 76 men and 60 women, with a mean age of 59.2 years. Spontaneous nystagmus exhibited a sensitivity of 92%, whereas the absence of skew deviation was identified with a sensitivity of 98%. Abnormal bedside HIT showed a sensitivity of 87%. The combined HINTS (HIT, nystagmus, and test of skew) had a sensitivity of 83%. The Romberg test and Fukuda test demonstrated sensitivities of 26% and 48%, respectively. CONCLUSION: The sensitivity of bedside tests varied from 26% to 98%. This aligns with previous literature, highlighting the challenge of differentiating AUPVP from vestibular pseudoneuritis solely through bedside examination. Although the tests excel in excluding central causes, they are insufficient for diagnosing AUPVP with certainty. In addition, the bedside examination sensitivities vary widely, and early radiological imaging can be misleading. Therefore, this study underlines the necessity of prompt otoneurological testing for accurate exclusion of vestibular pseudoneuritis and thus improve patient outcomes.


Subject(s)
Head Impulse Test , Point-of-Care Testing , Vestibular Neuronitis , Humans , Male , Middle Aged , Female , Retrospective Studies , Aged , Point-of-Care Testing/standards , Vestibular Neuronitis/diagnosis , Adult , Sensitivity and Specificity , Caloric Tests , Aged, 80 and over , Nystagmus, Pathologic/diagnosis , Vestibular Function Tests/methods
16.
J Laryngol Otol ; 138(S2): S14-S17, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38779899

ABSTRACT

BACKGROUND: Differential diagnosis of acute vertigo syndrome is challenging given the similarities between clinical presentations of posterior circulation stroke and peripheral vestibular dysfunction. The Head Impulse, Nystagmus, Test of Skew ('HINTS') assessment is a clinical bedside test used to aid diagnosis. METHODS: Comprehensive training on use of the Head Impulse, Nystagmus, Test of Skew assessment was provided to one stroke consultant, and the effectiveness of the test in that setting was assessed. Further education was completed with more members of the stroke and emergency department multi-disciplinary team. Quality improvement measures including magnetic resonance imaging use and bed utilisation were explored. RESULTS: Following training of one stroke consultant, the Head Impulse, Nystagmus, Test of Skew assessment was found to be a feasible, accurate bedside test within this acute stroke service. Further training for the multi-disciplinary team was completed, but outcome measures were not explored because of the coronavirus disease 2019 pandemic and maternity leave. CONCLUSION: There is a role for trained members of the multi-disciplinary team to successfully use the Head Impulse, Nystagmus, Test of Skew assessment in hyperacute stroke settings, to aid diagnosis in acute vertigo syndrome.


Subject(s)
Head Impulse Test , Stroke , Vertigo , Humans , Diagnosis, Differential , Stroke/complications , Stroke/diagnosis , Head Impulse Test/methods , Vertigo/diagnosis , Nystagmus, Pathologic/diagnosis , COVID-19/complications , COVID-19/diagnosis , Vestibular Function Tests/methods , Syndrome , Vestibular Diseases/diagnosis
17.
JAMA Netw Open ; 7(5): e2412846, 2024 May 01.
Article in English | MEDLINE | ID: mdl-38780940

ABSTRACT

Importance: Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing. Objectives: To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL. Design, Setting, and Participants: This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023. Main Outcomes and Measures: The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay. Results: A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment. Conclusions and Relevance: This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.


Subject(s)
Vestibular Diseases , Humans , Female , Male , Child, Preschool , Vestibular Diseases/epidemiology , Vestibular Diseases/complications , Child , Hearing Loss, Bilateral/epidemiology , Cochlear Implantation , Cohort Studies , Postural Balance/physiology , Vestibular Function Tests , Prevalence , Paris/epidemiology , Infant
18.
Medicina (Kaunas) ; 60(5)2024 May 13.
Article in English | MEDLINE | ID: mdl-38792986

ABSTRACT

Background and Objectives: Besides classical stapedotomy, reverse stapedotomy has been used for many years in the management of otosclerosis. Our study aims to investigate whether reversing the surgical steps in stapedotomy impacts vestibular function and hearing improvement. Materials and Methods: A cohort of 123 patients underwent either classic or reverse stapedotomy procedures utilizing a fiber-optic argon laser. Audiological assessments, following the guidelines of the Committee on Hearing and Equilibrium, were conducted, including pure tone average, air-bone (AB) gap, overclosure, and AB gap closure. Vestibular evaluation involved pre- and postoperative comparison of rotatory test parameters, including frequency, amplitude, and slow phase velocity of nystagmus. Results: The study demonstrated an overall median overclosure of 3.3 (3.3, 5.0) dB and a mean AB gap closure of 20.3 ± 8.8 dB. Postoperative median AB gap was 7.5 (7.5, 11.3) dB in the reverse stapedotomy group and 10.0 (10.0, 12.5) dB in the classic stapedotomy group. While overclosure and AB gap closure were marginally superior in the reverse stapedotomy group, these differences did not reach statistical significance. No significant disparities were observed in the frequency, slow phase velocity, or amplitude of nystagmus in the rotational test. Conclusions: Although not always possible, reverse stapedotomy proved to be a safe surgical technique regarding postoperative outcomes. Its adoption may mitigate risks associated with floating footplate, sensorineural hearing loss, and incus luxation/subluxation, while facilitating the learning curve for less experienced ear surgeons.


Subject(s)
Otosclerosis , Stapes Surgery , Humans , Stapes Surgery/methods , Stapes Surgery/adverse effects , Male , Female , Middle Aged , Adult , Otosclerosis/surgery , Otosclerosis/physiopathology , Audiometry/methods , Cohort Studies , Treatment Outcome , Audiometry, Pure-Tone/methods , Aged , Vestibular Function Tests/methods
20.
J Laryngol Otol ; 138(S2): S8-S13, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38779893

ABSTRACT

BACKGROUND: Patients presenting to the emergency department with acute vertigo pose a diagnostic challenge. While 'benign' peripheral vestibulopathy is the most common cause, the possibility of a posterior circulation stroke is paradoxically the most feared and missed diagnosis in the emergency department. OBJECTIVES: This review will attempt to cover the significant advances in the ability to diagnose acute vertigo that have occurred in the last two decades. The review discusses the role of neurological examinations, imaging and specific oculomotor examinations. The review then discusses the relative attributes of the Head Impulse-Nystagmus-Test of Skew plus hearing ('HINTS+') examination, the timing, triggers and targeted bedside eye examinations ('TiTrATE'), the associated symptoms, timing and triggers, examination signs and testing ('ATTEST') algorithm, and the spontaneous nystagmus, direction, head impulse testing and standing ('STANDING') algorithm. The most recent technological advancements in video-oculography guided care are discussed, as well as other potential advances for clinicians to look out for.


Subject(s)
Vertigo , Humans , Vertigo/diagnosis , Vertigo/therapy , Acute Disease , Head Impulse Test/methods , Algorithms , Neurologic Examination/methods , Vestibular Function Tests/methods , Diagnosis, Differential , Emergency Service, Hospital , Nystagmus, Pathologic/diagnosis
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