The first Brazilian clinical report of Kleefstra syndrome, including semicircular canals agenesis as a possible phenotype expansion.

OBJECTIVE: to report the first case series of Brazilian children diagnosed with Kleefstra syndrome, present a possible phenotype expansion to the syndrome and to raise physicians' awareness for this rare disease. RESULTS: seven patients with confirmed KS were evaluated, including 5 males and 2 females. Abnormal prenatal findings were observed in 4 patients. Most patients were born at term, with normal birth measurements. All patients had neurodevelopmental delay and 6 evolved with intellectual disability. Hearing loss was present in 57.1% of patients and 28.7% had congenital heart disease. In males, cryptorchidism was present in 75%. Despite the facial dysmorphisms, only 2 out of 7 patients had a pre-test clinical suspicion of KS. One specific patient presented bilateral agenesis of the semicircular canals, a very rare ear manifestation in Kleefstra syndrome, representing a possible phenotype expansion of the syndrome. CONCLUSION: this report aims to promote awareness among physicians evaluating patients in a context of neurodevelopmental delay or congenital malformations, especially congenital heart defects. We also highlight a possible phenotype expansion of the syndrome, with a case of semicircular anomaly, not reported in this syndrome so far.

Evaluation of the vestibulo-ocular reflex in patients with chronic otitis media / Avaliação do reflexo vestíbulo-ocular em pacientes com otite média crônica

Abstract Introduction In recent years, scientific evidence has shown that chronic otitis media may cause balance and vestibular dysfunction. Objective To compare the results of the video head impulse test (gain and symmetry of the vestibulo-ocular reflex and presence of covert and overt saccades) in patients with chronic otitis media and controls. Methods Cross-sectional study of patients with chronic otitis media (study group), aged between 18 and 60 years. The patients in the study group were further divided according to the chronic otitis media type as (1) non-suppurative, (2) suppurative, and (3) cholesteatomatous. For the comparative analysis, we selected volunteers with no history of ear and vestibular diseases (control group), who met the same inclusion and exclusion criteria as the study group. Patients in both groups underwent a video head impulse test. Results The study group consisted of 96 volunteers, and the control group of 61 individuals. The prevalence of vestibular symptoms was 66% in the study group and 3.2% in the control group (p< 0.001). The results show a higher prevalence of changes in the vestibulo-ocular reflex gain (22.9%) and corrective saccades (12.6%) in the chronic otitis media group compared to the control group (p< 0.001). Despite the higher prevalence of changes in gain, the average vestibulo-ocular reflex gains in the chronic otitis media groups were within the pre-defined values ​​of normality; however, the mean vestibulo-ocular reflex gain in the anterior semicircular canal was statistically worse in the cholesteatomatous chronic otitis media group compared to controls (p< 0.001). Regarding the corrective saccades, the prevalence of saccades was statistically higher in the suppurative and cholesteatomatous chronic otitis media subgroups compared to the non-suppurative and control groups (p= 0.004). Conclusion The present study demonstrated that chronic otitis media is associated with a higher prevalence of vestibular symptoms and also a higher prevalence of changes in gain and corrective saccades when compared to controls.

Resumo Introdução Nos últimos anos, evidências científicas demonstraram que a otite média crônica se associa a alterações de equilíbrio e disfunção vestibular. Objetivo Comparar os resultados do teste do impulso cefálico por vídeo (ganho e simetria do reflexo vestíbulo-ocular e presença de sacadas cobertas e descobertas) em pacientes com otite média crônica e controles. Método Estudo transversal que envolveu pacientes com otite média crônica (grupo de estudo), entre 18 a 60 anos. Os pacientes no grupo estudo ainda foram divididos de acordo com o tipo de otite média crônica em (1) não supurativa, (2) supurativa e (3) colesteatomatosa. Para análise comparativa, selecionamos voluntários sem história de doenças otológicas e vestibulares (grupo controle), que obedeceram aos mesmos critérios de inclusão e exclusão do grupo de estudo. Os pacientes de ambos grupos foram submetidos ao teste de impulso cefálico por vídeo. Resultados O grupo estudo foi composto por 96 voluntários e o grupo controle por 61 indivíduos. A prevalência de sintomas vestibulares foi de 66% no grupo de estudo e 3,2% no grupo controle (p < 0,001). Os resultados mostram maior prevalência de alterações do ganho do reflexo vestíbulo-ocular (22,9%) e de sacadas corretivas (12,6%) no grupo otite média crônica em comparação ao grupo controle (p < 0,001). Apesar da maior prevalência de alterações de ganho, a média dos ganhos do reflexo vestíbulo-ocular dos grupos de otite média crônica estava dentro dos valores pré-definidos de normalidade; porém, a média do ganho do reflexo vestíbulo-ocular no canal semicircular anterior foi estatisticamente pior no grupo otite média crônica colesteatomatosa em comparação aos controles (p< 0,001). Em relação às sacadas corretivas, a prevalência de sacadas foi estatisticamente maior nos subgrupos otite média crônica supurativa e colesteatomatosa em comparação aos grupos não supurativa e controle (p = 0,004). Conclusão A otite média crônica se associa à maior prevalência de sintomas vestibulares e também maior prevalência de alterações no ganho e de sacadas corretivas em comparação a controles.

Evaluation of the vestibulo-ocular reflex in patients with chronic otitis media.

INTRODUCTION: In recent years, scientific evidence has shown that chronic otitis media may cause balance and vestibular dysfunction. OBJECTIVE: To compare the results of the video head impulse test (gain and symmetry of the vestibulo-ocular reflex and presence of covert and overt saccades) in patients with chronic otitis media and controls. METHODS: Cross-sectional study of patients with chronic otitis media (study group), aged between 18 and 60 years. The patients in the study group were further divided according to the chronic otitis media type as (1) non-suppurative, (2) suppurative, and (3) cholesteatomatous. For the comparative analysis, we selected volunteers with no history of ear and vestibular diseases (control group), who met the same inclusion and exclusion criteria as the study group. Patients in both groups underwent a video head impulse test. RESULTS: The study group consisted of 96 volunteers, and the control group of 61 individuals. The prevalence of vestibular symptoms was 66% in the study group and 3.2% in the control group (p <  0.001). The results show a higher prevalence of changes in the vestibulo-ocular reflex gain (22.9%) and corrective saccades (12.6%) in the chronic otitis media group compared to the control group (p <  0.001). Despite the higher prevalence of changes in gain, the average vestibulo-ocular reflex gains in the chronic otitis media groups were within the pre-defined values ​​of normality; however, the mean vestibulo-ocular reflex gain in the anterior semicircular canal was statistically worse in the cholesteatomatous chronic otitis media group compared to controls (p <  0.001). Regarding the corrective saccades, the prevalence of saccades was statistically higher in the suppurative and cholesteatomatous chronic otitis media subgroups compared to the non-suppurative and control groups (p =  0.004). CONCLUSION: The present study demonstrated that chronic otitis media is associated with a higher prevalence of vestibular symptoms and also a higher prevalence of changes in gain and corrective saccades when compared to controls.

Características clínico-demográficas en sujetos con vértigo posicional paroxístico benigno al ingreso a rehabilitación vestibular en un hospital de la Ciudad Autónoma de Buenos Aires / Clinical-demographic characteristics of subjects with benign paroxysmal positional vertigo at vestibular rehabilitation unit admission in a hospital in the Autonomous City of Buenos Aires

RESUMEN Objetivo: Describir las características clínico-demográficas de sujetos con VPPB al ingreso a rehabilitación vestibular en un hospital de rehabilitación de la Ciudad Autónoma de Buenos Aires en el periodo comprendido entre los años 2010 y 2021. Materiales y método: Estudio descriptivo, observacional y transversal. Se llevó a cabo mediante la técnica de muestreo no probabilístico. Se categorizó a las variables en características clínico-demográficas, aquellas relacionadas al diagnóstico y a la evaluación. Resultados: Se obtuvo una muestra de 102 sujetos con VPPB, de los cuales 79 fueron mujeres con una mediana de edad de 71 años, encontrándose afectado el canal semicircular posterior en el 90,28% de los casos, con una predominancia del oído interno derecho en el 72,6%. Conclusión: Se conocieron las características clínico-demográficas de los sujetos con VPPB entre los años 2010 y 2021 al ingreso a rehabilitación vestibular. La muestra estaba compuesta principalmente por mujeres, de edad avanzada, con 3 o más medicamentos y cuya afección más frecuente fue la canalitiasis de canal posterior derecho.

ABSTRACT Objective: To describe the clinical-demographic characteristics of subjects with benign paroxysmal positional vertigo (BPPV) at vestibular rehabilitation unit admission in a rehabilitation hospital in the Autonomous City of Buenos Aires between 2010 and 2021. Materials and method: A descriptive, observational, and cross-sectional study was conducted, using the non-probabilistic sampling technique. Variables were categorized into clinical-demographic, diagnostic, and assessment variables. Results: Of the total of 102 subjects with BPPV, 79 were female, with a median age of 71 years. The posterior semicircular canal was affected in 90.28% of the cases, and the right inner ear had a prevalence of 72.6%. Conclusion: We described the clinical-demographic characteristics of subjects with BPPV at vestibular rehabilitation unit admission between 2010 and 2021. Our sample consisted mainly of elderly women who required 3 or more medications, and the most prevalent disorder was right posterior canalithiasis.

Factores asociados a falla terapéutica con maniobras de reposición canalicular en pacientes con diagnóstico de vértigo posicional paroxístico benigno en la Clínica de Otorrinolaringología de Antioquia, Medellín, Colombia / Factors associated with therapeutic failure with canalicular repositioning maneuvers in patients diagnosed with benign paroxysmal positional vertigo at Antioquia Otorhinolaryngology Clinic, Medellin, Colombia"

Introducción: múltiples factores se han relacionado con el desarrollo de la recurrencia del vértigo posicional paroxístico benigno (VPPB). Objetivo: determinar la asociación entre la falla terapéutica de las maniobras de reposición canalicular (MRC) y las variables sociodemográficas y clínicas en los pacientes con diagnóstico de VPPB. Diseño: estudio observacional de cohorte retrospectiva. Materiales y métodos: revisión de historias clínicas de la consulta de vértigo de la Clínica Orlant, Medellín, Colombia. Resultados: se incluyeron 41 pacientes con diagnóstico de VPPB a quienes se les realizó MRC y seguimiento clínico entre 1 y 8 semanas. El 90,2 % eran de sexo femenino, con una mediana de edad de 58 (±183) años; se encontró uso de vestibulosupresores en un 68,3 %, y es la betahistina el más consumido (43,9 %). El 51,2 % de pacientes presentaron falla terapéutica y se identificó una asociación con el número total de MRC realizadas y el uso de vibrador mastoideo (p < 0,001), teniendo en cuenta que los pacientes mejoraron clínicamente al final del seguimiento con una media de 77 % (p < 0,001). No se encontraron asociaciones estadísticamente significativas con el resto de variables. Conclusión: no hubo asociación entre la falla terapéutica y las variables estudiadas, excepto número de MRC, el uso del vibrador mastoideo y la mejoría clínica final, posiblemente porque el vibrador mastoideo se aplica a los pacientes en quienes hay persistencia de síntomas y signos con las maniobras desencadenantes, y por factores fisiopatológicos no esclarecidos; con esto finalmente se logra una mejoría clínica con más de dos MRC

Introduction: Multiple factors have been related to the development of recurrence of benign paroxysmal positional vertigo (BPPV). Objective: To determine the association between therapeutic failure of canalicular repositioning maneuvers (CRM) with sociodemographic and clinical variables in patients with a diagnosis of BPPV. Design: Observational retrospective cohort study. Materials and methods: Review of medical records of the vertigo clinic of the Orlant Clinic, Medellín - Colombia. Results: 41 patients with a diagnosis of BPPV who underwent CRM and clinical follow-up between 1 and 8 weeks were included. 90.2% were female, with a median age of 58 (± 18.3) years, use of vestibulosuppressants was found in 68.3%, betahistine being the most consumed (43.9%). 51.2% of patients presented therapeutic failure, identifying an association with the total number of CRMs performed and the use of a mastoid vibrator with (p < 0.001), taking into account that the patients improved clinically at the end of follow-up with a mean of 77% (p < 0.001). No statistically significant associations were found with the rest of the variables. Conclusion: There was no association between therapeutic failure and the variables studied except number of CRM, use of the mastoid vibrator and final clinical improvement, possibly because the mastoid vibrator is applied to patients in whom there are persistence of symptoms and signs with the triggering maneuvers for unclear pathophysiological factors, finally achieving clinical improvement with more than two CRMs.

Importance of Vestibulo-ocular Reflex Gain and Refixation Saccade Analysis in Individuals with Auditory Neuropathy Spectrum Disorder

Abstract Introduction Auditory neuropathy spectrum disorder (ANSD) features the presence of otoacoustic emissions, poor speech identification score and absent auditory brainstem response. Objective The present study was designed to evaluate the functioning of all six semicircular canals in individuals with ANSD and to compare it with those of normalhearing individuals. Methods A total of 50 individuals participated in the present study, in which Group I comprised25normal-hearingindividuals, and GroupII comprised25individualswithANSD. All of the participants underwent case history, pure tone audiometry, immittance, otoacoustic emissions, auditory evoked response and video head impulse test (vHIT). Results The independent sample t-test revealed significantly lower vestibulo-ocular reflex gain values in individuals with ANSD. A presence of 100% corrective refixation saccades was observed in the same group. The Pearson correlation test revealed no significant correlation between vestibulo-ocular reflex (VOR) gain with duration of hearing loss and pure tone thresholds for any of the three orthogonal planes. The chisquared test revealed no association between the VOR gain values and the presence or absence of saccades in any of the semicircular canals (p>0.05). Conclusion Huge percentages of individuals with ANSD have been found to have associated vestibular dysfunction as well. Therefore, the vHIT can be used as one of the important tests of the vestibular test battery to evaluate all six semicircular canals in individuals with ANSD.

Importance of Vestibulo-ocular Reflex Gain and Refixation Saccade Analysis in Individuals with Auditory Neuropathy Spectrum Disorder.

Introduction Auditory neuropathy spectrum disorder (ANSD) features the presence of otoacoustic emissions, poor speech identification score and absent auditory brainstem response. Objective The present study was designed to evaluate the functioning of all six semicircular canals in individuals with ANSD and to compare it with those of normal-hearing individuals. Methods A total of 50 individuals participated in the present study, in which Group I comprised 25 normal-hearing individuals, and Group II comprised 25 individuals with ANSD. All of the participants underwent case history, pure tone audiometry, immittance, otoacoustic emissions, auditory evoked response and video head impulse test (vHIT). Results The independent sample t-test revealed significantly lower vestibulo-ocular reflex gain values in individuals with ANSD. A presence of 100% corrective refixation saccades was observed in the same group. The Pearson correlation test revealed no significant correlation between vestibulo-ocular reflex (VOR) gain with duration of hearing loss and pure tone thresholds for any of the three orthogonal planes. The chi-squared test revealed no association between the VOR gain values and the presence or absence of saccades in any of the semicircular canals ( p > 0.05). Conclusion Huge percentages of individuals with ANSD have been found to have associated vestibular dysfunction as well. Therefore, the vHIT can be used as one of the important tests of the vestibular test battery to evaluate all six semicircular canals in individuals with ANSD.

Bilateral posterior semicircular canal dysfunction: a new finding with video head impulse test.

Development of the video head impulse test (vHIT) assessing all three semicircular canals in both labyrinths has uncovered the existence of new vestibular failure patterns and made bilateral posterior canal dysfunction detection possible. We conducted a retrospective analysis of 41 patients with bilateral posterior semicircular canal failure and compared results to 37 controls, with normal posterior semicircular canal function. Mean calculated gain showed significant difference between patients and controls in right [0.54 (SD 0.016)] and left [0.57 (SD 0.014)] posterior semicircular canals. There was a peak in prevalence between 71 and 80 years. Presentation was chronic in 78% of patients, and gait instability was the most common complaint. Sixty eight percent of cases were classified as idiopathic. Significant difference between groups was seen regarding the presence of Meniere's disease, presbycusis, and positional down-beat nystagmus (posDBN). This new vHIT pattern is most often seen in elderly patients, mainly of idiopathic etiology and presents together with sensorineural hearing loss and posDBN. Our findings suggest idiopathic cases may well contribute to the so-called "presbyastasis".

Video head impulse test (v-hit) em indivíduos com diabetes mellitus tipo 1 / Video head impulse test (v-hit) in individuals with type 1 diabetes mellitus

RESUMO Objetivo Verificar a função dos canais semicirculares do labirinto de indivíduos com diabetes tipo 1, submetidos ao Video Head Impulse Test (v-HIT), e compará-los com indivíduos sem diabetes. Métodos Estudo transversal, observacional, analítico, realizado com uma amostra de conveniência, formada por 35 indivíduos diabéticos e 100 não diabéticos. Todos os participantes foram submetidos à avaliação vestibular por meio do v-HIT. Resultados A casuística foi composta por 135 participantes, divididos em dois grupos. O grupo de estudo foi composto por indivíduos com diabetes tipo 1, totalizando 21 mulheres e 14 homens. A idade variou entre 18 e 71 anos, com média de 35,37 anos e desvio padrão de 10,98. O grupo sem diabetes foi composto por 77 mulheres e 23 homens. A idade variou entre 20 e 83 anos, com média de 46,44 e desvio padrão de 19,82. Os grupos foram pareados entre si, com relação à idade (p=0,098) e sexo (p=0,052). Os pacientes diabéticos apresentaram ganho diminuído nos canais semicirculares posteriores e anterior esquerdo. A velocidade apresentou diferença significativa nos canais lateral esquerdo, anterior direito e posterior esquerdo no grupo com diabetes mellitus tipo 1, porém não apresentou correlação com o ganho dos canais semicirculares. Conclusão Os participantes com diabetes mellitus tipo 1 apresentaram um ganho diminuído nos canais semicirculares posteriores e no canal anterior esquerdo quando comparados com indivíduos não diabéticos.

ABSTRACT Purpose To verify the function of the labyrinth semicircular channels of type 1 diabetes individuals submitted to the Video Head Impulse Test (v-HIT) and to compare them with individuals without diabetes. Methods Cross-sectional, observational, analytical study conducted with a convenience sample of 35 diabetic and 100 non-diabetic individuals. All participants were submitted to vestibular evaluation using v-HIT. Results The sample consisted of 135 participants divided into two groups. The study group was composed of individuals with type 1 diabetes, totaling 21 women and 14 men. The age range was between 18 and 71 years, with a mean of 35.37 years and standard deviation of 10.98. The group without diabetes was composed of 77 women and 23 men. The age range was between 20 to 83 years, with a mean of 46.44 and standard deviation of 19.82. The groups were matched for age (p=0.098) and gender (p=0.052). Diabetic patients showed decreased gain in the posterior and left anterior semicircular canals. Velocity showed a significant difference in the left lateral, anterior right and posterior left canals in the group with DM1, however velocity did not show correlation with the gain of the semicircular canals. Conclusion participants with type 1 diabetes mellitus showed a decreased gain in the posterior semicircular canals and in the left anterior canal when compared to non-diabetic individuals.

Study of Sigmoid Sinus Variations in the Temporal Bone by Micro Dissection and its Classification - A Cadaveric Study

Abstract Introduction Sigmoid sinus (SS) variations have been classified variously in the literature. These classifications suffer from some form of shortcoming from a clinical point of view for their application. Objective We propose a clinically relevant classification of the SS in relation to the posterior semicircular canal (PSCC) and to the exposure of the presigmoid dural plate. The positioning of the SS was analyzed with reference to the volume of themastoid and to the level of mastoid pneumatization. Methods A total of 94 formalin-preserved human cadaveric temporal bones were microdissected to carry out a complete mastoidectomy. The SS, the presigmoid dural plate, and the PSCCwere exposed, and the position of the former was analyzed in relation to the latter two in order to classify the position of the SS into three grades. Results GradeI hadthebest exposureof the presigmoid dura andof the PSCC,while grade III had the poorest exposure of the presigmoid dura and of the PSCC. Grade I SS was associated with good pneumatization and highermastoid volumescompared with grades II and III. Conclusions The SS exhibits considerable anatomic variability. A favorable positioning of the SS is associated with a large mastoid volume and pneumatization. A careful preoperative study of the imaging may help in understanding the positioning of the SS and the safety of various transmastoid approaches.

Study of Sigmoid Sinus Variations in the Temporal Bone by Micro Dissection and its Classification - A Cadaveric Study.

Introduction Sigmoid sinus (SS) variations have been classified variously in the literature. These classifications suffer from some form of shortcoming from a clinical point of view for their application. Objective We propose a clinically relevant classification of the SS in relation to the posterior semicircular canal (PSCC) and to the exposure of the presigmoid dural plate. The positioning of the SS was analyzed with reference to the volume of the mastoid and to the level of mastoid pneumatization. Methods A total of 94 formalin-preserved human cadaveric temporal bones were microdissected to carry out a complete mastoidectomy. The SS, the presigmoid dural plate, and the PSCC were exposed, and the position of the former was analyzed in relation to the latter two in order to classify the position of the SS into three grades. Results Grade I had the best exposure of the presigmoid dura and of the PSCC, while grade III had the poorest exposure of the presigmoid dura and of the PSCC. Grade I SS was associated with good pneumatization and higher mastoid volumes compared with grades II and III. Conclusions The SS exhibits considerable anatomic variability. A favorable positioning of the SS is associated with a large mastoid volume and pneumatization. A careful preoperative study of the imaging may help in understanding the positioning of the SS and the safety of various transmastoid approaches.

Surgical Treatment for Recurrent Benign Paroxysmal Positional Vertigo

Abstract Introduction Benign paroxysmal positional vertigo is a generally benign condition that responds to repositioning maneuvers and frequently resolves spontaneously. However, for some patients it can become a disabling condition in which surgery must be considered. Two different surgical techniques exist, singular neurectomy and posterior semicircular canal occlusion. Objective The objective of this study is to review the current status of singular nerve section and posterior semicircular canal occlusion as treatments for intractable benign paroxysmal positional vertigo, and to determine if there are published data available that favors one over the other. Data Sources MEDLINE and OLDMEDLINE databases of the National Library of Medicine. Data Synthesis Four studies regarding singular neurectomy and 14 reports on semicircular canal occlusion were analyzed. Both techniques are reported to provide similar symptomatic benefit, with low risk of hearing loss and balance impairment. However, anatomical and clinical studies of singular neurectomy show it to be a more challenging technique, and considering that it is indicated in a very small number of cases, it may be difficult to master. Conclusions Both singular neurectomy and semicircular canal occlusion can be safe and effective in those few patients that require surgery for intractable positional vertigo. Although semicircular canal occlusion requires a postauricular transmastoid approach, it is ultimately easier to learn and perform adequately, and thus may be considered the best alternative.

Surgical Treatment for Recurrent Benign Paroxysmal Positional Vertigo.

Introduction Benign paroxysmal positional vertigo is a generally benign condition that responds to repositioning maneuvers and frequently resolves spontaneously. However, for some patients it can become a disabling condition in which surgery must be considered. Two different surgical techniques exist, singular neurectomy and posterior semicircular canal occlusion. Objective The objective of this study is to review the current status of singular nerve section and posterior semicircular canal occlusion as treatments for intractable benign paroxysmal positional vertigo, and to determine if there are published data available that favors one over the other. Data Sources MEDLINE and OLDMEDLINE databases of the National Library of Medicine. Data Synthesis Four studies regarding singular neurectomy and 14 reports on semicircular canal occlusion were analyzed. Both techniques are reported to provide similar symptomatic benefit, with low risk of hearing loss and balance impairment. However, anatomical and clinical studies of singular neurectomy show it to be a more challenging technique, and considering that it is indicated in a very small number of cases, it may be difficult to master. Conclusions Both singular neurectomy and semicircular canal occlusion can be safe and effective in those few patients that require surgery for intractable positional vertigo. Although semicircular canal occlusion requires a postauricular transmastoid approach, it is ultimately easier to learn and perform adequately, and thus may be considered the best alternative.

Síndrome da deiscência do canal semicircular superior: relato de dois casos / Semicircular superior canal dehiscence: cases reports

RESUMO A Síndrome da Deiscência do Canal Semicircular Superior (SDCSS) é caracterizada pelo desgaste da camada óssea que recobre o canal semicircular superior. São sintomas comuns da SDCSS a presença de vertigem associada à nistagmos induzidos por estímulos sonoros intensos ou por modificações das pressões intracraniana ou da orelha média. O objetivo deste trabalho é descrever os achados audiólogicos e vestibulares de dois pacientes com diagnóstico de Síndrome da Deiscência do Canal Semicircular Superior, com diagnóstico confirmado por meio de tomografia computadorizada. Foram realizadas meatoscopia, anamnese, audiometria tonal e vocal seguida das medidas de imitância acústica, Weber audiométrico, pesquisa do fenômeno de Túlio e manobra de Valsalva, realizados pela mesma pesquisadora em uma única sessão. Foram observados gap aéreo-ósseo, curva timpanométrica tipo A e reflexos acústicos presentes. O gap aéreo-ósseo apresenta-se com maior amplitude nas frequências baixas. As queixas auditivas não foram relatadas pelas pacientes como os primeiros sintomas. O Weber mostrou lateralização, nos dois casos, confirmando a presença de gap. O fenômeno de Túlio apresentou-se positivo para vertigem em ambos os casos. A manobra de Valsalva apresentou alteração em apenas um caso.

ABSTRACT The Superior Semicircular Canal Dehiscence Syndrome (SSCDS) is characterized by bone wear layer overlying the superior semicircular canal. Common symptoms of SSCDS the presence of vertigo associated with nystagmus induced by intense sound stimuli or changes in intracranial pressure or middle ear. The aim of this study is to describe the audiological and vestibular findings of two patients diagnosed with Superior Semicircular Canal Deiscence Syndrome, with confirmed diagnosis by computed tomography. Meatoscopy, anamnesis, pure tone audiometry and vocal followed by the acoustic impedance measurements, audiometric Weber, research Tulio phenomenon and Valsalva maneuver, performed by the same researcher in one session were held. Air-bone gap were observed, type A tympanometric curve and acoustic reflex. The air-bone gap is presented with greater amplitude at low frequencies. Hearing complaints were not reported by patients as the first symptoms. Weber showed lateralization in both cases, confirming the presence of gap. The Thulium phenomenon is positive for vertigo in both cases. The Valsalva maneuver showed a change in only one case.

Is it important to repeat the positioning maneuver after the treatment for benign paroxysmal positional vertigo? / É importante realizar o reteste da manobra de posicionamento após o tratamento da vertigem postural paroxística benigna?

INTRODUCTION: Benign paroxysmal positional vertigo (BPPV) is the most common cause of peripheral vestibular dysfunction. OBJECTIVE: To assess whether the performance of the Dix-Hallpike maneuver after the Epley positioning maneuver has prognostic value in the evolution of unilateral ductolithiasis of posterior semicircular canal. METHODS: A prospective cohort study in monitored patients at otoneurology ambulatory with a diagnosis of BPPV; they were submitted to the therapeutic maneuver and then to a retest in order to evaluate the treatment effectiveness; all cases were reassessed one week later and the retest prognostic value was evaluated. RESULTS: A sample of 64 patients which 47 belonging to negative retest group and 17 belonging to positive retest. Performed the maneuver in all patients, the retest presented 51.85% sensitivity, 91.89% specificity, 82.35% positive predictive value and 72.34% negative predictive value. CONCLUSION: The study shows that doing the retest after repositioning maneuver of particles in BPPV is effectual, since it has high specificity. .

INTRODUÇÃO: A vertigem posicional paroxística benigna (VPPB) é a causa mais comum de disfunção vestibular periférica. OBJETIVO: Avaliar se a realização do reteste de Dix-Hallpike após a manobra de posicionamento de Epley tem valor prognóstico na evolução da vertigem posicional da ductolitíase paroxística benigna de canal semicircular posterior unilateral. MÉTODO: Estudo prospectivo do tipo coorte de pacientes em acompanhamento no ambulatório de otoneurologia com diagnóstico de VPPB; foram submetidos a manobra terapêutica e posteriormente ao reteste para avaliar a eficácia do tratamento; todos os casos foram reavaliados em uma semana e analisado o valor prognostico do reteste. RESULTADOS: Amostra de 64 pacientes, 47 do grupo reteste negativo e 17 do reteste positivo; realizada manobra de Epley em todos os pacientes. O reteste apresentou sensibilidade de 51,85%; especificidade de 91,89%; valor preditivo positivo de 82,35% e valor preditivo negativo de 72,34%. CONCLUSÃO: O estudo mostra que é válido realizar o reteste após a manobra de reposicionamento de partículas na VPPB, visto que possui alta especificidade. .

Is it important to repeat the positioning maneuver after the treatment for benign paroxysmal positional vertigo?

INTRODUCTION: Benign paroxysmal positional vertigo (BPPV) is the most common cause of peripheral vestibular dysfunction. OBJECTIVE: To assess whether the performance of the Dix-Hallpike maneuver after the Epley positioning maneuver has prognostic value in the evolution of unilateral ductolithiasis of posterior semicircular canal. METHODS: A prospective cohort study in monitored patients at otoneurology ambulatory with a diagnosis of BPPV; they were submitted to the therapeutic maneuver and then to a retest in order to evaluate the treatment effectiveness; all cases were reassessed one week later and the retest prognostic value was evaluated. RESULTS: A sample of 64 patients which 47 belonging to negative retest group and 17 belonging to positive retest. Performed the maneuver in all patients, the retest presented 51.85% sensitivity, 91.89% specificity, 82.35% positive predictive value and 72.34% negative predictive value. CONCLUSION: The study shows that doing the retest after repositioning maneuver of particles in BPPV is effectual, since it has high specificity.

Superior Semicircular Canal Dehiscence Syndrome without Vestibular Symptoms

Introduction: Superior semicircular canal dehiscence syndrome is mainly characterized by vestibular symptoms induced by intense sound stimuli or pressure changes, which occur because of dehiscence of the bony layer covering the superior semicircular canal. Case Report Here, we report a case of the syndrome with pulsatile tinnitus and ear fullness, in the absence of vestibular symptoms. Discussion: Signs and symptoms of the syndrome are rarely obvious, leading to the requirement for a minimum workup to rule out or make diagnosis more probable and thus avoid misconduct.

A multicenter observational study on the role of comorbidities in the recurrent episodes of benign paroxysmal positional vertigo.

OBJECTIVE: Primary objective of this study was to find a statistical link between the most worldwide comorbidities affecting the elderly population (hypertension, diabetes, osteoarthrosis, osteoporosis and depression) and recurrent episodes of BPPV. Secondary objective was defining possible "groups of risk" for people suffering recurrent positional vertigo related to the presence of a well documented comorbidity. METHODS: This was an observational, cross-sectional, multicenter, spontaneous, non-pharmacological study. The data of 1092 patients suffering BPPV evaluated in 11 different Departments of Otolaryngology, Otoneurology and Neurology, referring Centers for positional vertigo evaluation, were retrospectively collected. RESULTS: Regarding evaluated comorbidities (hypertension, diabetes, osteoarthrosis, osteoporosis and depression), data analysis showed the presence of at least one comorbid disorder in 216 subjects (19.8%) and 2 or more in 408 subjects (37.4%). Moreover there was a statistical significant difference between the number of comorbidities and the number of recurrences, otherwise said as comorbidity disorders increased the number of relapses increased too. CONCLUSION: The presence of a systemic disease may worsen the status of the posterior labyrinth causing a more frequent otolith detachment. This condition increases the risk for patients suffering BPPV to have recurrent episodes, even if correctly managed by repositioning maneuvers. The combination of two or more of aforementioned comorbidities further increases the risk of relapsing BPPV, worsened by the presence of osteoporosis. On the basis of this results it was possible to define "groups of risk" useful for predicting BPPV recurrence in patients with one or more comorbidity.