Vestibulo-ocular reflex dynamics with head-impulses discriminates Usher patients type 1 and 2.

Usher Syndrome classification takes into account the absence of vestibular function but its correlation with genotype is not well characterized. We intend to investigate whether video Head Impulse Test (vHIT) is useful in screening and to differentiate Usher Syndrome types. 29 Usher patients (USH) with a genetically confirmed diagnosis and 30 healthy controls were studied with vHIT and dizziness handicap inventory questionnaire (DHI). Statistical significant differences between USH1, USH2 and controls were found in the vestibulo-ocular-reflex (VOR) gain of all SCCs, with USH1 patients consistently presenting smaller gains. VOR gain of the right lateral SCC could discriminate controls from USH1, and USH2 from USH1 with an overall diagnostic accuracy of 90%. USH1 DHI correlated with VOR (ρ = - 0,971, p = 0.001). Occurrence rate of covert and overt lateral semicircular canals refixation saccades (RS) was significantly different between groups, being higher in USH1 patients (p < 0.001). USH1 peak velocity of covert and overt saccades was higher for lateral semicircular canals (p < 0.05 and p = 0.001) compared with USH2 and controls. Covert saccades occurrence rate for horizontal SCCs could discriminate USH1 from USH2 patients and controls with a diagnostic accuracy of 85%. vHIT is a fast and non-invasive instrument which allowed us to screen and distinguish Usher patients from controls with a high precision. Importantly, its use allowed further discrimination between USH1 from USH2 groups. Moreover, VOR gain seems to correlate with vertigo-related quality of life in more severe phenotypes.

The Portuguese version of the visual vertigo analog scale

Abstract Objectives: Visual vertigo occurs after a vestibular disorder compromising daily living. It can be assessed by "the Visual Vertigo Analogue Scale" (VVAS), a self-administered questionnaire without Portuguese version. To perform the translation, cross cultural adaptation, and validation of VVAS from English to Portuguese. Methods: Prospective study involving the translation and cross-cultural adaptation of the VVAS into the Portuguese language, according to recognized guidelines. It was completed by 63 healthy controls and 198 participants with vestibulopathy who also completed the Dizziness Handicap Inventory (DHI) to further explore the link between DHI and VVAS. Groups were compared for severity of visual vertigo and VVAS reliability and internal consistency were tested. Results: The VVAS score was significantly higher in vestibular group (p < 0.001). A Cronbach's α of 0.9 confirmed the valid internally consistent of the applied version. The severity score of VVAS showed a positive strong correlation with DHI (p < 0.0001). Conclusion: The present Portuguese translation of the scale showed satisfactory properties for the assessment of self-perceived and severity of visual vertigo in a significant group of vestibular Portuguese patients. Level of evidence: 2.

The Portuguese version of the visual vertigo analog scale.

OBJECTIVES: Visual vertigo occurs after a vestibular disorder compromising daily living. It can be assessed by "the Visual Vertigo Analogue Scale" (VVAS), a self-administered questionnaire without Portuguese version. To perform the translation, cross cultural adaptation, and validation of VVAS from English to Portuguese. METHODS: Prospective study involving the translation and cross-cultural adaptation of the VVAS into the Portuguese language, according to recognized guidelines. It was completed by 63 healthy controls and 198 participants with vestibulopathy who also completed the Dizziness Handicap Inventory (DHI) to further explore the link between DHI and VVAS. Groups were compared for severity of visual vertigo and VVAS reliability and internal consistency were tested. RESULTS: The VVAS score was significantly higher in vestibular group (p < 0.001). A Cronbach's α of 0.9 confirmed the valid internally consistent of the applied version. The severity score of VVAS showed a positive strong correlation with DHI (p < 0.0001). CONCLUSION: The present Portuguese translation of the scale showed satisfactory properties for the assessment of self-perceived and severity of visual vertigo in a significant group of vestibular Portuguese patients.

Labyrinthine haemorrhage secondary to cerebral venous thrombosis.

Cerebral venous thrombosis (CVT) can rarely present with acute ipsilateral cochlear and/or vestibular loss, to date always in the absence of a clear local otogenic process evidenced by magnetic resonance imaging (MRI). This association has been putatively attributed to inner ear anoxia due to blockage of inner ear venous drainage. We present a nonreported case of thrombosis of the left transverse and sigmoid sinuses presenting with acute unilateral vestibulopathy in which MRI disclosed concurrent ipsilateral labyrinthine haemorrhage. A 69-year-old female presented with acute vertigo without hearing loss or other accompanying neurological symptoms. Bedside examination revealed spontaneous right-beating nystagmus and an impaired left head impulse response, with an otherwise normal neurological examination. Audiometry and head and ear computed tomography were unremarkable, whereas MRI showed a nonenhancing hyperintensity of the left inner ear consistent with labyrinthine haemorrhage, and additional venography disclosed thrombosis of the left transverse and sigmoid sinuses. Oral anticoagulation was started, and the patient experienced gradual improvement of symptoms. The current case provides support for the existence of inner ear anoxia in CVT cases presenting with acute ipsilateral vestibular loss, which can ultimately be complicated by secondary bleeding, as seen in our patient. Importantly, presentation in our case closely mimicked that of peripheral vestibular neuritis, and only MRI venography enabled us to make a prompt diagnosis. This raises an important question as to when a diagnosis of vestibular neuritis can be made securely in the absence of MRI assessment with or without venography to completely discard labyrinthine haemorrhage with or without CVT.

How to manage an unusual presentation of a thyroglossal duct cyst?

Lingual thyroglossal duct cysts are rare congenital anomalies of the neck. They can be accidentally detected or manifest with disabling symptoms. These cysts can be potentially difficult to manage, but their complete resection is curative.

Benign paroxysmal positional vertigo--a review of 101 cases.

INTRODUCTION AND OBJECTIVE: Benign paroxysmal positional vertigo is one of the most common vestibular disorders, with a lifetime prevalence of 2.4%. This study aimed to assess age, gender, lesion type and site, association with other vestibular diseases, progression and recurrence in a Portuguese population. METHODS: This was a retrospective observational study of 101 patients diagnosed with benign paroxysmal positional vertigo by the same senior doctor, in a tertiary academic hospital, between January 2009 and May 2011. RESULTS: A total of 101 cases were pooled, with a mean age of 56.57±15.33 years (15-90 years). From these, 72.3% were women. The posterior canal was affected in 72.3%, the lateral in 24.7%, the anterior in 2% and multiple canals in 1%. Unilateral canal and left labyrinth involvement were more frequent. The therapeutic maneuver used most was Epley's. Recurrence was observed in 10.9% of the cases. It was idiopathic in 83.2% of cases. No association was found between the number of maneuvers necessary to treat benign paroxysmal positional vertigo and etiology. CONCLUSION: Benign paroxysmal positional vertigo is more frequent in female subjects, in the 6th decade and involves preferably the posterior semicircular canal of the right labyrinth. In most cases it is idiopathic and treatment with repositioning maneuvers has a mean success of 90%. Our results were in accordance with the literature; nevertheless, in this study the left labyrinth was most affected and the follow-up period was variable.