Detailed analysis of inner ear malformations in CHARGE syndrome patients - correlation with audiological results and proposal for computed tomography scans evaluation methodology.

OBJECTIVES: The aim was to describe the spectrum of inner ear malformations in CHARGE syndrome and propose a Computed Tomography (CT) detailed scan evaluation methodology. The secondary aim was to correlate the CT findings with hearing thresholds. METHODS: Twenty ears of ten patients diagnosed with CHARGE syndrome were subjected to CT analysis focusing on the inner ear and internal acoustic canal. The protocol used is presented in detail. ASSR results were analyzed and correlated with inner ear malformations. RESULTS: Cochlear hypoplasia type III was the most common malformation found in 12 ears (60%). Cochlear hypoplasia type II, aplasia with a dilated vestibule, and rudimentary otocyst were also identified. In 20%, no cochlear anomaly was found. The lateral Semicircular Canal (SCC) absence affected 100% of ears, the absence of the posterior SCC 95%, and the superior SCC 65%. Better development of cochlea structures and IAC correlated significantly with the lower hearing thresholds. CONCLUSION: This study demonstrated that rudimentary SCC or a complete absence of these SCCs was universally observed in all patients diagnosed with CHARGE syndrome. This finding supports the idea that inner ear anomalies are a hallmark feature of the CHARGE, contributing to its distinct clinical profile. The presence of inner ear malformations has substantial clinical implications. Audiological assessments are crucial for CHARGE syndrome, as hearing loss is common. Early detection of these malformations can guide appropriate interventions, such as hearing aids or cochlear implants, which may significantly improve developmental outcomes and communication for affected individuals. Recognizing inner ear malformations as a diagnostic criterion presents implications beyond clinical diagnosis. A better understanding of these malformations can advance the knowledge of CHARGE pathophysiology. It may also help guide future research into targeted therapies to mitigate the impact of inner ear anomalies on hearing and balance function.

Detailed analysis of inner ear malformations in CHARGE syndrome patients - correlation with audiological results and proposal for computed tomography scans evaluation methodology

Abstract Objectives The aim was to describe the spectrum of inner ear malformations in CHARGE syndrome and propose a Computed Tomography (CT) detailed scan evaluation methodology. The secondary aim was to correlate the CT findings with hearing thresholds. Methods Twenty ears of ten patients diagnosed with CHARGE syndrome were subjected to CT analysis focusing on the inner ear and internal acoustic canal. The protocol used is presented in detail. ASSR results were analyzed and correlated with inner ear malformations. Results Cochlear hypoplasia type III was the most common malformation found in 12 ears (60%). Cochlear hypoplasia type II, aplasia with a dilated vestibule, and rudimentary otocyst were also identified. In 20%, no cochlear anomaly was found. The lateral Semicircular Canal (SCC) absence affected 100% of ears, the absence of the posterior SCC 95%, and the superior SCC 65%. Better development of cochlea structures and IAC correlated significantly with the lower hearing thresholds. Conclusion This study demonstrated that rudimentary SCC or a complete absence of these SCCs was universally observed in all patients diagnosed with CHARGE syndrome. This finding supports the idea that inner ear anomalies are a hallmark feature of the CHARGE, contributing to its distinct clinical profile. The presence of inner ear malformations has substantial clinical implications. Audiological assessments are crucial for CHARGE syndrome, as hearing loss is common. Early detection of these malformations can guide appropriate interventions, such as hearing aids or cochlear implants, which may significantly improve developmental outcomes and communication for affected individuals. Recognizing inner ear malformations as a diagnostic criterion presents implications beyond clinical diagnosis. A better understanding of these malformations can advance the knowledge of CHARGE pathophysiology. It may also help guide future research into targeted therapies to mitigate the impact of inner ear anomalies on hearing and balance function. Level of evidence: 4.

Complex benign horizontal canal positional vertigo: new perceptual management

Abstract Objective: Horizontal semicircular canal site pathology of benign paroxysmal positional vertigo demonstrating three types of nystagmi on positional test were studied. We have attempted to design a protocol for its diagnosis and treatment. Methods: 320 patients of HSC-BPPV were subjected to two types of positional tests. Of these, patients with bilateral steady apogeotropic nysatgmus were treated with VAV modification of Semont's maneuver. Patients with unsteady or changing apo/geotropic signs were converted into steady geotropic ones by repetitive positional tests; followed by barbecue maneuver with forced prolong positioning. Results: Overall 88% of patients had a total recovery. 92% of patients with geotropic nystagmus showed no symptoms after second maneuveral sitting. 85% of patients with apogeotropic nystagmus recovered fully after third maneuveral sitting. Conclusion: Correct identification of subtypes of HSC-BPPV is based on provoked nystagmus by positional tests. After locating the site and side on the basis of nystagmic pattern, physician can apply the appropriate PRM. Level of evidence: II a.

Diagnosis and treatment of the short-arm type posterior semicircular canal BPPV / Diagnóstico e tratamento da VPPB de braço curto do canal semicircular posterior

Abstract Introduction The Epley maneuver is useful for the otoconia to return from the long arm of the posterior semicircular canal into the utricle. To move otoconia out of the posterior semicircular canal short arm and into the utricle, we need different maneuvers. Objective To diagnose the short-arm type BPPV of the posterior semicircular canal and treat them with bow-and-yaw maneuver. Methods 171 cases were diagnosed as BPPV of the posterior semicircular canal based on a positive Dix-Hallpike maneuver. We first attempted to treat patients with the bow-and-yaw maneuver and then performed the Dix-Hallpike maneuver again. If the repeated Dix-Hallpike maneuver gave negative results, we diagnosed the patient with the short-arm type of BPPV of the posterior semicircular canal and considered the patient to have been cured by the bow-and-yaw maneuver; otherwise, probably the long-arm type BPPV of the posterior semicircular canal existed and we treated the patient with the Epley maneuver. Results Approximately 40% of the cases were cured by the bow-and-yaw maneuver, giving negative results on repeated Dix-Hallpike maneuvers, and were diagnosed with short-arm lithiasis. Conclusion The short-arm type posterior semicircular canal BPPV can be diagnosed and treated in a convenient and comfortable manner.

Resumo Introdução A manobra de Epley é útil para o retorno da otocônia do braço longo do canal semicircular posterior para o utrículo. Diferentes manobras são necessárias para mover a otocônia para fora do braço curto do canal semicircular posterior e para dentro do utrículo. Objetivo Diagnosticar a VPPB do tipo braço curto do canal semicircular posterior e tratá-la com a manobra de incline and balance. Método Foram diagnosticados 171 casos como VPPB de canal semicircular posterior com base na manobra de Dix-Hallpike positiva. Primeiro tentamos tratar os pacientes com a manobra de incline and balance e, em seguida, executamos a manobra de Dix-Hallpike novamente. Se a repetição da manobra de Dix-Hallpike desse resultados negativos, diagnosticávamos o paciente como VPPB do canal semicircular posterior do tipo braço curto e considerávamos que ele ou ela havia sido curado pela manobra de incline and balance; caso contrário, provavelmente o paciente apresentava VPPB do canal semicircular posterior do tipo braço longo e tratávamos o paciente com a manobra de Epley. Resultados Aproximadamente 40% dos casos foram curados pela manobra de incline and balance, com resultados negativos nas manobras de Dix-Hallpike repetidas, e foram diagnosticados com litíase de braço curto. Conclusão A VPPB de canal semicircular posterior do tipo braço curto pode ser diagnosticada e tratada de maneira conveniente e confortável.

A novel maneuver for diagnosis and treatment of torsional-vertical down beating positioning nystagmus: anterior canal and apogeotropic posterior canal BPPV / Uma nova manobra para diagnóstico e tratamento de nistagmo vertical para baixo com componente de torção: forma apogeotrópica da VPPB do canal anterior e canal posterior

Abstract Introduction In patients with benign paroxysmal positional vertigo, BPPV; a torsional-vertical down beating positioning nystagmus can be elicited in the supine straight head-hanging position test or in the Dix-Hallpike test to either side. This type of nystagmus can be explained by either an anterior canal BPPV or by an apogeotropic variant of the contralateral posterior canal BPPV Until now all the therapeutic maneuvers that have been proposed address only one possibility, and without first performing a clear differential diagnosis between them. Objective To propose a new maneuver for torsional-vertical down beating positioning nystagmus with a clear lateralization that takes into account both possible diagnoses (anterior canal-BPPV and posterior canal-BPPV). Methods A prospective cohort study was conducted on 157 consecutive patients with BPPV. The new maneuver was performed only in those with torsional-vertical down beating positioning nystagmus with clear lateralization. Results Twenty patients (12.7%) were diagnosed with a torsional-vertical down beating positioning nystagmus. The maneuver was performed in 10 (6.35%) patients, in whom the affected side was clearly determined. Seven (4.45%) patients were diagnosed with an anterior canal-BPPV and successfully treated. Two (1.25%) patients were diagnosed with a posterior canal-BPPV and successfully treated with an Epley maneuver after its conversion into a geotropic posterior BPPV. Conclusion This new maneuver was found to be effective in resolving all the cases of torsional-vertical down beating positioning nystagmus-BPPV caused by an anterior canal-BPPV, and in shifting in a controlled way the posterior canal-BPPV cases of the contralateral side into a geotropic-posterior-BPPV successfully treated during the followup visit. Moreover, this new maneuver helped in the differential diagnosis between anterior canal-BPPV and a contralateral posterior canal-BPPV.

Resumo Introdução Em pacientes com vertigem posicional paroxística benigna, VPPB, um nistagmo vertical para baixo com componente de torção pode ser provocado no teste head hanging supino executado na posição reta ou no teste de Dix-Hallpike para qualquer um dos lados. Esse tipo de nistagmo pode ser explicado por uma VPPB do canal anterior ou por uma variante apogeotrópica da VPPB do canal posterior contralateral. Até agora, todas as manobras terapêuticas propostas abordam apenas uma possibilidade, sem antes fazer um diagnóstico diferencial claro entre elas. Objetivo Propor uma nova manobra para nistagmo vertical para baixo com componente de torção com uma lateralização clara que leve em consideração os dois diagnósticos possíveis, VPPB do canal anterior e VPPB do canal posterior. Método Um estudo de coorte prospectivo foi conduzido em 157 pacientes consecutivos com VPPB. A nova manobra foi feita apenas nos pacientes com nistagmo vertical para baixo com componente de torção, com lateralização nítida. Resultados Vinte pacientes (12,7%) foram diagnosticados com nistagmo vertical para baixo com componente de torção. A manobra foi feita em 10 (6,35%) pacientes, nos quais o lado afetado foi claramente determinado. Sete (4,45%) pacientes foram diagnosticados com VPPB do canal anterior e tratados com sucesso. Dois (1,25%) pacientes foram diagnosticados com VPPB do canal posterior e tratados com sucesso com a manobra de Epley após sua conversão para VPPB geotrópica de canal posterior. Conclusão Essa nova manobra mostrou-se eficaz na resolução de todos os casos de VPPB com nistagmo vertical para baixo com componente de torção causada por VPPB do canal anterior. E na mudança de forma controlada dos casos de VPPB do canal posterior do lado contralateral para uma VPPB geotrópica de canal posterior tratada com sucesso durante a consulta de seguimento. Além disso, essa nova manobra auxiliou no diagnóstico diferencial entre a VPPB do canal anterior e a VPPB do canal posterior contralateral.

Association of Serum Calcium and Vitamin D with Benign Paroxysmal Positional Vertigo

Abstract Introduction Benign paroxysmal positional vertigo (BPPV) is one of the common disorders of the peripheral vestibular system. The prevalence of BPPV is found to be higher among middle-aged women. Objectives To estimate the serum levels of calcium and vitamin D in patients with BPPV, and to study their association. Methods The present is a hospital-based prospective case-control study. Venous blood samples of the 49 patients with BPPV and an equal number of age- and gender-matched individuals were recruited and submitted to an analysis of the serum levels of calcium and vitamin D. Results Among the cases, 67.3% were found to be females, and 32.7% were males. Most of the 30 cases (61.3%) were aged >40 years. The mean age of the cases was 44.39 years. The mean serum level of vitamin D in the cases was of 21.26 ng/ml compared with 17.59 ng/ml in the controls. The mean serum level of calcium was of 9.33 mg/dl in the cases, compared with 8.95 mg/dl in the controls. There was no significant difference in the serum levels of vitamin D and calcium between cases and controls. Conclusion We could not establish any correlation between the serum levels of calcium and vitamin D with BPPV. However, a negative relationship was found between the serum levels of vitamin D and the number of episodes of BPPV (p = 0.012).

"Sitting-up vertigo as an expression of posterior semicircular canal heavy cupula and posterior semicircular canal short arm canalolithiasis".

Background: Vestibular symptoms on sitting-up are frequent on patients seen by vestibular specialists. Recently, a benign paroxysmal positional vertigo (BPPV) variant which elicits vestibular symptoms with oculomotor evidence of posterior semicircular canal (P-SCC) cupula stimulation on sitting-up was described and named sitting-up vertigo BPPV. A periampullar restricted P-SCC canalolithiasis was proposed as a causal mechanism. Objective: To describe new mechanisms of action for the sitting-up vertigo BPPV variant. Methods: Eighteen patients with sitting-up vertigo BPPV were examined with a pre-established set of positional maneuvers and follow-up until they resolved their symptoms and clinical findings. Results: All patients showed up-beating torsional nystagmus (UBTN) and vestibular symptoms on coming up from either Dix-Hallpike (DHM) or straight head-hanging maneuver. Sixteen out of 18 patients presented a sustained UBTN with an ipsitorsional component to the tested side on half-Hallpike maneuver (HH). A slower persistent contratorsional down-beating nystagmus was found in eleven out 18 patients tested on nose down position (ND). Conclusions: Persistent direction changing positional nystagmus on HH and ND positions indicative of P-SCC heavy cupula was found in 11 patients. A sustained UBTN on HH with the absence of findings on ND, which is suggestive of the presence of P-SCC short arm canalolithiasis, was found on 5 patients. All patients were treated with canalith repositioning maneuvers without success, but they resolved their findings by means of Brandt-Daroff exercises. We propose P-SCC heavy cupula and P-SCC short arm canalolithiasis as two new putative mechanisms for the sitting-up vertigo BPPV variant.

Association of Serum Calcium and Vitamin D with Benign Paroxysmal Positional Vertigo.

Introduction Benign paroxysmal positional vertigo (BPPV) is one of the common disorders of the peripheral vestibular system. The prevalence of BPPV is found to be higher among middle-aged women. Objectives To estimate the serum levels of calcium and vitamin D in patients with BPPV, and to study their association. Methods The present is a hospital-based prospective case-control study. Venous blood samples of the 49 patients with BPPV and an equal number of age- and gender-matched individuals were recruited and submitted to an analysis of the serum levels of calcium and vitamin D. Results Among the cases, 67.3% were found to be females, and 32.7% were males. Most of the 30 cases (61.3%) were aged > 40 years. The mean age of the cases was 44.39 years. The mean serum level of vitamin D in the cases was of 21.26 ng/ml compared with 17.59 ng/ml in the controls. The mean serum level of calcium was of 9.33 mg/dl in the cases, compared with 8.95 mg/dl in the controls. There was no significant difference in the serum levels of vitamin D and calcium between cases and controls. Conclusion We could not establish any correlation between the serum levels of calcium and vitamin D with BPPV. However, a negative relationship was found between the serum levels of vitamin D and the number of episodes of BPPV ( p = 0.012).

Complex benign horizontal canal positional vertigo: new perceptual management.

OBJECTIVE: Horizontal semicircular canal site pathology of benign paroxysmal positional vertigo demonstrating three types of nystagmi on positional test were studied. We have attempted to design a protocol for its diagnosis and treatment. METHODS: 320 patients of HSC-BPPV were subjected to two types of positional tests. Of these, patients with bilateral steady apogeotropic nysatgmus were treated with VAV modification of Semont's maneuver. Patients with unsteady or changing apo/geotropic signs were converted into steady geotropic ones by repetitive positional tests; followed by barbecue maneuver with forced prolong positioning. RESULTS: Overall 88% of patients had a total recovery. 92% of patients with geotropic nystagmus showed no symptoms after second maneuveral sitting. 85% of patients with apogeotropic nystagmus recovered fully after third maneuveral sitting. CONCLUSIONS: Correct identification of subtypes of HSC-BPPV is based on provoked nystagmus by positional tests. After locating the site and side on the basis of nystagmic pattern, physician can apply the appropriate PRM. LEVEL OF EVIDENCE: II a.

A novel maneuver for diagnosis and treatment of torsional-vertical down beating positioning nystagmus: anterior canal and apogeotropic posterior canal BPPV.

INTRODUCTION: In patients with benign paroxysmal positional vertigo, BPPV; a torsional-vertical down beating positioning nystagmus can be elicited in the supine straight head-hanging position test or in the Dix-Hallpike test to either side. This type of nystagmus can be explained by either an anterior canal BPPV or by an apogeotropic variant of the contralateral posterior canal BPPV Until now all the therapeutic maneuvers that have been proposed address only one possibility, and without first performing a clear differential diagnosis between them. OBJECTIVE: To propose a new maneuver for torsional-vertical down beating positioning nystagmus with a clear lateralization that takes into account both possible diagnoses (anterior canal-BPPV and posterior canal-BPPV). METHODS: A prospective cohort study was conducted on 157 consecutive patients with BPPV. The new maneuver was performed only in those with torsional-vertical down beating positioning nystagmus with clear lateralization. RESULTS: Twenty patients (12.7%) were diagnosed with a torsional-vertical down beating positioning nystagmus. The maneuver was performed in 10 (6.35%) patients, in whom the affected side was clearly determined. Seven (4.45%) patients were diagnosed with an anterior canal-BPPV and successfully treated. Two (1.25%) patients were diagnosed with a posterior canal-BPPV and successfully treated with an Epley maneuver after its conversion into a geotropic posterior BPPV. CONCLUSION: This new maneuver was found to be effective in resolving all the cases of torsional-vertical down beating positioning nystagmus-BPPV caused by an anterior canal-BPPV, and in shifting in a controlled way the posterior canal-BPPV cases of the contralateral side into a geotropic-posterior-BPPV successfully treated during the followup visit. Moreover, this new maneuver helped in the differential diagnosis between anterior canal-BPPV and a contralateral posterior canal-BPPV.

Diagnosis and treatment of the short-arm type posterior semicircular canal BPPV.

INTRODUCTION: The Epley maneuver is useful for the otoconia to return from the long arm of the posterior semicircular canal into the utricle. To move otoconia out of the posterior semicircular canal short arm and into the utricle, we need different maneuvers. OBJECTIVE: To diagnose the short-arm type BPPV of the posterior semicircular canal and treat them with bow-and-yaw maneuver. METHODS: 171 cases were diagnosed as BPPV of the posterior semicircular canal based on a positive Dix-Hallpike maneuver. We first attempted to treat patients with the bow-and-yaw maneuver and then performed the Dix-Hallpike maneuver again. If the repeated Dix-Hallpike maneuver gave negative results, we diagnosed the patient with the short-arm type of BPPV of the posterior semicircular canal and considered the patient to have been cured by the bow-and-yaw maneuver; otherwise, probably the long-arm type BPPV of the posterior semicircular canal existed and we treated the patient with the Epley maneuver. RESULTS: Approximately 40% of the cases were cured by the bow-and-yaw maneuver, giving negative results on repeated Dix-Hallpike maneuvers, and were diagnosed with short-arm lithiasis. CONCLUSION: The short-arm type posterior semicircular canal BPPV can be diagnosed and treated in a convenient and comfortable manner.

Management of Lateral Semicircular Canal Benign Paroxysmal Positional Vertigo.

Benign paroxysmal positional vertigo (BPPV) is the most common cause of peripheral vestibular vertigo. It is caused by free-floating otoconia moving freely in one of the semicircular canals (canalolithiasis) or by otoliths adhered to the cupula (cupulolithiasis). The posterior canal is the most common canal affected, followed by the lateral canal. Diagnosis of the side affected is critical for successful treatment; therefore, suppressing visual fixation is essential to examination of these patients' eye movement. On the basis of our experience, we have adopted the Zuma maneuver and the modified Zuma maneuver for both apogeotropic and geotropic variants of lateral canal BPPV. Knowledge of the anatomy and pathophysiologic mechanisms of the semicircular canals is essential for correct management of these patients. Hence, using a single maneuver and its modification may facilitate daily neurotological practice.

Dehiscencia del canal semicircular superior: análisis de la producción científica / Superior semicircular canal dehiscence: analysis of scientific production

RESUMEN Introducción: La dehiscencia del canal semicircular es una vestibulopatía periférica rara y poco prevalente, que se caracteriza por una falta de cobertura ósea del canal semicircular superior en la zona más próxima a la duramadre de la fosa cerebral media. Objetivo: Conocer el estado actual y la calidad científica de las publicaciones sobre la dehiscencia del canal semicircular superior (DCSS). Material y método: Se ha realizado una búsqueda bibliométrica con posterior revisión, selección y análisis a partir de ítems relacionados con estudios DCSS en la base de datos Pubmed desde el año 1998 hasta 2017. Resultados: Al estudiar el tipo de publicaciones, el 77% eran artículos originales, 12% revisiones, 9% casos clínicos, 2% cartas al director y respuestas al editor. Los artículos han sido publicados en un total de 108 revistas, siendo Otology and Neurotology la que mayor número de manuscritos presenta con un total de 87, seguida de Head and Neck Surgery con 28 y Laryngoscope con 22. Estados Unidos se posiciona como el principal contribuyente a la literatura mundial sobre este tema (42%), seguido de Europa (33%). El idioma de referencia es el inglés con 91% de publicaciones (382). Según el índice de Lotka, la actividad productiva de los autores es de tipo medio/bajo, ya que de los 217 autores que firman en primer lugar, solo 19 tienen más de 10 artículos. Según el índice de impacto Journal Citation Reports, 60% de las publicaciones se localiza en los cuartiles Q1 (116 publicaciones) y Q2 (141 publicaciones), lo que indica que la calidad de los trabajos es alta. La temática ha ido variando con el paso del tiempo, siendo actualmente el diagnóstico con 34,3%, seguido del tratamiento con 25,7%, los temas que más interesan. Aunque se trata principalmente de un tema del campo de la otorrinolaringología, en los últimos años ha despertado interés en otras áreas como la neurología y la radiología. Conclusión: Este estudio revela como los trabajos sobre DCSS presentan un escaso número de autores, las publicaciones se concentran en pocas revistas, pero de una alta calidad, y el estado actual del tema está en fase de crecimiento exponencial.

ABSTRACT Introduction: The dehiscence of the semicircular canal is a rare and not very prevalent peripheral vestibulopathy, characterized by a lack of bony coverage of the superior semicircular canal in the area closest to the dura of the middle cerebral fossa. Aim: To know current status and scientific quality of publications of the superior semicircular canal dehiscence (SSCD). Material and method: Bibliometric research with review, selection and analysis from ítems related with SSCD studies in the Pubmed database from 1998 to 2017. Results: 77% of publications were original articles, 12% reviews, 9% clinical cases, 2% letters and answers to the editor. The articles have been published in 108 journals. The top publishing journal is Otology and Neurotology with 87 publications, followed by Head and Neck Surgery with 28 and Laryngoscope with 22. USA is the main global contributor to the world literature on this subject (42%) followed by Europe (33%). The publication reference language is English, with the 91% of publications (382). According to Lotka's index, the general production activity of the authors is at the middle/low level. According to JCR impact factor, there are 60% of the publications in Q1 (116 articles) and Q2 (141 articles) quartiles, which indicates that the quality is high. The subject of the publications has varied over time, being currently diagnostic with 34.3%, followed by treatment with 25.7%, the subjects that most interest. In recent years it has aroused interest in other areas such as neurology or radiology. Conclusion: The work on DCSS has a small number of authors, the publications are limited to a few journals, but of a high quality, and the current state of the subject is in phase of exponential growth.

Tumarkin-like phenomenon as a sign of therapeutic success in benign paroxysmal positional vertigo / Fenômeno Tumarkin-like como sinal de sucesso terapêutico na vertigem posicional paroxística benigna

ABSTRACT Objective: To describe an unusual patient reaction to maneuvers used in the treatment of posterior canal benign paroxysmal positional vertigo (PC-BPPV) that we termed the "Tumarkin-like phenomenon". Methods: At a private practice, 221 outpatients were diagnosed and treated for PC-BPPV. The treatment consisted of performing the Epley or Semont maneuvers. At the end of these maneuvers, when assuming the sitting position, the patients' reactions were recorded. Results: Thirty-three patients showed a Tumarkin-like phenomenon described by a self-reported sensation of suddenly being thrown to the ground. In the follow-up, this group of patients remained without PC-BPPV symptoms up to at least 72 hours after the maneuvers. Conclusion: The occurrence of a Tumarkin-like phenomenon at the end of Epley and Semont maneuvers for PC-BPPV may be linked with treatment success.

RESUMO Objetivo: Descrever uma reação incomum dos pacientes às manobras utilizadas no tratamento da vertigem posicional paroxística benigna do canal posterior (VPPB-CP), a qual denominamos de fenômeno Tumarkin-like. Métodos: Em uma clínica privada, 221 pacientes ambulatoriais foram diagnosticados e tratados para VPPB-CP. O tratamento consistiu em realizar as manobras de Epley ou de Semont. Ao término da manobra, ao serem colocados na posição sentado, as reações dos pacientes foram filmadas. Resultados: Trinta e três pacientes apresentaram o fenômeno de Tumarkin-like, descrito como uma sensação súbita de ser jogado no chão. O acompanhamento mostrou que todos eles permaneceram sem sintomas de VPPB até pelo menos 72 horas após as manobras. Conclusão: A ocorrência do fenômeno Tumarkin-like no final das manobras de Epley e Semont para VPPB-CP pode estar associado ao sucesso terapêutico.

Dehiscencia del canal semicircular superior como causa de tinnitus pulsátil / Superior semicircular canal dehiscence as cause of pulsatile tinnitus

RESUMEN El tinnitus se presenta en forma crónica en alrededor del 10% de los adultos, siendo el 4% de estos casos tinnitus pulsátil (TP). El TP se caracteriza por ser rítmico y sincrónico al latido cardiaco. Existen múltiples causas descritas, pero en un grupo importante de casos, no se logra objetivar su origen. Nuestro objetivo es presentar casos de dehiscencia del canal semicircular superior (DCSS) como causa de tinnitus pulsátil y su estudio. Se presentan dos pacientes evaluadas por tinnitus pulsátil. En ambos casos se descartan causas sistémicas, ECO doppler carotídeo sin alteración, angio TAC y RNM sin hallazgos. En reconstrucción de Pöschl se sospecha DCSS, por lo que se estudia con potenciales miogénicos evocados cVEMP y oVEMP con disminución de umbral y respuesta aumentada en oído afectado. En los casos expuestos el tinnitus aparece como síntoma único asociado a la presencia de DCSS, que fue confirmada con estudio imagenológico y VEMPs. El estudio con angio TAC permite pesquisar diversas causas asociadas. Los VEMPs confirman el diagnóstico, teniendo el oVEMP mayor sensibilidad. Como conclusión la DCSS es una entidad a tener presente como diagnóstico diferencial del tinnitus pulsátil y ante su sospecha se debe explorar con VEMPs.

ABSTRACT Tinnitus occurs chronically in about 10% of adults, being pulsatile tinnitus a 4% of these cases (TP). TP is characterized by being rythmic and sychronous to the heart beat. There are many described causes, but in a significant group of cases it is not possible to determine its origin. Our aim is present clinical cases of superior semicircular canal dehiscence (SSCD) as the cause of pulsatile tinnitus and its study. Clinical cases: Two patients present pulsatile tinnitus in her right ear. System causes were discarded, normal Carotid Doppler ultrasonography, Anglo CT scan and MRI without findings. In Pöschl reconstruction SSCD can be observed. Evoked myogenic potentials (VEMPs) by suspicion of SSCD Syndrome, cVEMP and oVEMP with a elevated amplitudes and lower thresholds ipsilateral response. In the cases exposed, tinnitus appears as a single symptom associated with the presence of SSCD which was confirmed with imaging studies and VEMPs. The AngioTAC allows to investigate several associated causes. The VEMPs confirm the diagnosis, with oVEMP having a greater sensitivity. The SSCD is an entity to have in my mind as a differential diagnosis of pulsatile tinnitus and, if suspected, should be explored with VEMPs.

The influence of the subarcuate artery in the superior semicircular canal dehiscence and its frequency on stillbirths: illustrative cases and systematic review.

Literature describes that on the 25th gestational week the labyrinth is fully formed and with adult size. However, recent studies have shown that the cranial and labyrinth development continues until 3 years of age. OBJECTIVES: To demonstrate through tomographic study the frequency of semicircular canal dehiscence on nine specimens of stillbirths between 32 and 40 weeks and, through literature review, present another possible etiology for its cause. METHODS: Tomographic study of the temporal bone of 9 specimens of stillbirths between 32 and 40 weeks. RESULTS: A frequency of 88.89% of alterations were found in our study, with 44% presenting bilateral alterations and 44% unilateral alteration; 11.11% had no dehiscence. CONCLUSION: The tomographic study showed superior semicircular canal dehiscence (SSCD) in 88% of the specimens studied, protrusion of the superior semicircular canal (SSC) in all fetuses, and an enlarged SSC that may be caused by the expansion process provoked by the subarcuate artery entering the subarcuate canaliculus, leading to SSCD.

Revisión sobre los conocimientos actuales de dehiscencia del canal semicircular posterior / Review of current knowledge of the dehiscence in the semicircular posterior canal

La dehiscencia del canal semicircular posterior es una patología rara y con baja incidencia, por ello hemos realizado una revisión de los conocimientos actuales de esta entidad. Se ha realizado una búsqueda bibliográfica desde 1998 hasta diciembre de 2016 de toda la literatura publicada sobre la misma en las bases de datos Allied and Complementary Medicine Database and the Embase, Health Management Information Consortium, Scopus, Consortium, Medline, PsycINFO y Scielo. Se han encontrado y revisado 53 trabajos relacionados con el tema. La dehiscencia del canal semicircular posterior presenta una prevalencia variable; 0,3%-4,5% en adultos y 1,2%-20% en niños. Su localización puede ser hacia el golfo de la yugular o fosa cerebral posterior. Los pacientes pueden ser asintomáticos o presentar clínica auditiva y/o vestibular. La tomografía computarizada y la prueba de potenciales vestibulares miogénicos evocados permiten establecer el diagnóstico de certeza. En el tratamiento quirúrgico la vía de abordaje de elección es la transmastoidea y las técnicas del cierre del canal son el "plugging" y el "resurfacing".

The posterior semicircular canal dehiscence is a rare pathology and it has a low incidence. We have realized a review about the current knowledge of this entity. We have performed a bibliographic research from 1998 to 2016 December about the literature published in this subject, in the data basis Allied and Complementary Medicine Database and the Embase, Health Management Information Consortium, Scopus, Consortium, Medline, PsycINFO y Scielo. I thas been found and reviewed 53 papers about the topic. The posterior semicircular canal dehiscence has a variable prevalence: 0,3%-4-5% in adults and 1,2%-20% in children. The location can be in the jugular bulb or in the posterior brain fossa. Some patients can be asymptomatic, whereas others can have auditory and/or vestibular signs and symptoms. Computed tomography and test of vestibular evoked myogenic potentials allow the diagnosis of certainty. In the surgical treatment the approach of choice is transmastoid and techniques to close the canal are plugging and resurfacing.

Superior Semicircular Canal Dehiscence Syndrome - Diagnosis and Surgical Management

Abstract Introduction Superior semicircular canal dehiscence syndrome was described by Minor et al in 1998. It is a troublesome syndrome that results in vertigo and oscillopsia induced by loud sounds or changes in the pressure of the external auditory canal or middle ear. Patients may present with autophony, hyperacusis, pulsatile tinnitus and hearing loss. When symptoms are mild, they are usually managed conservatively, but surgical intervention may be needed for patients with debilitating symptoms. Objective The aim of this manuscript is to review the different surgical techniques used to repair the superior semicircular canal dehiscence. Data Sources PubMed and Ovid-SP databases. Data Synthesis The different approaches are described and discussed, as well as their limitations.We also review the advantages and disadvantages of the plugging, capping and resurfacing techniques to repair the dehiscence. Conclusions Each of the surgical approaches has advantages and disadvantages. The middle fossa approach gives a better view of the dehiscence, but comes with a higher morbidity than the transmastoid approach. Endoscopic assistance may be advantageous during the middle cranial fossa approach for better visualization. The plugging and capping techniques are associated with higher success rates than resurfacing, with no added risk of hearing loss.

Superior Semicircular Canal Dehiscence Syndrome - Diagnosis and Surgical Management.

Introduction Superior semicircular canal dehiscence syndrome was described by Minor et al in 1998. It is a troublesome syndrome that results in vertigo and oscillopsia induced by loud sounds or changes in the pressure of the external auditory canal or middle ear. Patients may present with autophony, hyperacusis, pulsatile tinnitus and hearing loss. When symptoms are mild, they are usually managed conservatively, but surgical intervention may be needed for patients with debilitating symptoms. Objective The aim of this manuscript is to review the different surgical techniques used to repair the superior semicircular canal dehiscence. Data Sources PubMed and Ovid-SP databases. Data Synthesis The different approaches are described and discussed, as well as their limitations. We also review the advantages and disadvantages of the plugging, capping and resurfacing techniques to repair the dehiscence. Conclusions Each of the surgical approaches has advantages and disadvantages. The middle fossa approach gives a better view of the dehiscence, but comes with a higher morbidity than the transmastoid approach. Endoscopic assistance may be advantageous during the middle cranial fossa approach for better visualization. The plugging and capping techniques are associated with higher success rates than resurfacing, with no added risk of hearing loss.

Diseño de dispositivo para detección de alteraciones del sistema vestibular / Design device for tamper detection of the vestibular system / Design de dispositivo para detecção de distúrbios do sistema vestibular

Los desórdenes vestibulares como el vértigo con mayor prevalencia en la población afectada pueden diagnosticarse con diferentes herramientas incluido el test de agudeza visual dinámica. Test que a nivel clínico únicamente incluye la monitorización de velocidad de movimiento de rotación de la cabeza sin mayores herramientas que permitan el diagnóstico puntual del canal semicircular horizontal acotado en la dinámica completa del movimiento. En el presente artículo se presenta una herramienta tecnológica completa para el desarrollo del test que permite el sensado de los movimientos de la cabeza en los ejes x, y, z y la interacción con una interfaz gráfica para la evaluación de la agudeza visual con errores encontrados al final de la implementación inferiores al 2% en ángulos de inclinación y flexión y del 4% al 15% para el ángulo máximo de rotación.

Vestibular disorders as the vertigo, with higher prevalence in the affected population, can be diagnosed using different tools including the dynamic vestibular acuity test. Test including at clinical level only the speed motorization of head without more tools to allow the specific diagnose of the horizontal semicircular canal bounded in the complete dynamic of movement. This article present a complete technologic tool to the development of the test, allowing the sensing of head movements in the x, y, z axis and the interaction with a graphical interface to evaluate the visual acuity, with found differences at the end of the implementation lower of 2% in flexion and inclination angles and of 4% to 15% to the maximum rotation angle.

Distúrbios vestibulares como a vertigem, com maior prevalência na população afetada, pode ser diagnosticada através de diferentes ferramentas, incluindo o teste de acuidade vestibular dinâmico. Incluindo o teste a nível clínico somente a motorização velocidade da cabeça sem mais ferramentas para permitir o diagnóstico específico do canal semicircular horizontal delimitada a dinâmica de movimentos. Este artigo apresenta uma ferramenta tecnológica completa para o desenvolvimento do teste, Permitindo que o sensor de movimentos da cabeça nos x, y, eixo z ea interação com uma interface gráfica para avaliar a acuidade visual, com diferenças encontradas no final da Implementação inferior de 2% em ângulos de flexão e de inclinação e de 4% a 15% para o ângulo de rotação máxima.