Hipoacusia Neurosensorial en el síndrome de Alport: Una revisión Sistemática / Sensorineural hearing loss in Alport syndrome: A systematic review

El Síndrome de Alport (SA) es un desorden genético originado por mutaciones en el colágeno tipo IV que es el constituyente principal de las membranas basales. Clínicamente, se caracteriza por nefropatía hereditaria progresiva. En el oído interno, el colágeno IV se encuentra ubicado en la membrana basilar y en el ligamento espiral, por lo que las mutaciones en los genes codificadores provocan hipoacusia de tipo neurosensorial. La presente investigación tiene por objetivo caracterizar el comportamiento de la pérdida de audición en personas diagnosticadas con SA, mediante la revisión de estudios de la literatura. Se realizó una búsqueda en bases de datos con los criterios de inclusión establecidos, incorporando un total de siete artículos para su análisis. Teniendo en cuenta los hallazgos de las diversas investigaciones recopiladas, se concluye que la pérdida auditiva secundaria al SA es heterogénea. No hay un patrón común de presentación pues depende del modo de herencia y del tipo de mutación de la enfermedad, además su grado de severidad y progresión va paralelo a la función renal. Sin embargo, independientemente del momento en que se manifieste el deterioro auditivo, es de vital importancia una intervención audiológica oportuna, con el fin de detectar la hipoacusia lo más temprano posible, hacer un seguimiento riguroso de la funcionalidad auditiva y, en caso de ser necesario, adaptar ayudas auditivas de acuerdo con las necesidades comunicativas del paciente

Alport Syndrome (AS) is a genetic disorder originated by mutations in the collagen type IV which is the main constituent of the basal membranes. Clinically, is characterized by progressive hereditary nephropathy. In the inner ear, the collagen IV is ubicated in the basilar membrane and in the spiral ligament, whereby the mutations in the codifier gens, cause neurosensorial hearing loss. The aim of this investigation is to characterize how hearing loss behaves in people diagnosed with AS, by presenting a reviewing of reported studies in the literature. A database search was performed with the established criteria of inclusion and a total of seven articles were incorporated for its analysis. Considering the findings by the several investigations collected the conclusion is that the secondary hearing loss to AS is heterogenous. There is no a common pattern of presentation as it depends on the mode of heredity and the type of mutation of the disease, and besides of the degree of severity and progression that goes along with the renal function. Nevertheless, regardless of the moment that the auditive impair appears, it is of vital importance an opportune audiological intervention, aiming at detecting an alteration as soon as possible, make a rigorous tracking of the auditive functionality and, if necessary, incorporate hearing aids in accordance with the communicative needs of the patients

Comparative study of quantitative methods based on intravenous enhancement of 3D-FLAIR sequence for evaluation of endolymphatic hydrops / 中华放射学杂志

Objective:To compare the diagnostic value of three quantitative evaluation methods based on three-dimensional rapid fluid attenuation inversion recovery sequence (3D-FLAIR) vein-enhanced labyrinth images in endolymphatic hydrops.Methods:From October 2017 to April 2019, a retrospective study was conducted on 86 patients with unilateral otogenic vertigo who were admitted to Beijing Tongren Hospital, Capital Medical University. MRI was performed 8 h after the single-dose Gd-DTPA intravenously injection in all patients. Three evaluation methods were used to calculate the ratio of the endolymphatic area to the total lymphatic area, the ratio of the saccule to utricle area, and the ratio of the endolymphatic volume to the total lymphatic volume, respectively. The paired t test was used to compare the three ratios between the affected and healthy ears. With clinical diagnosis as the gold standard, the receiver operating characteristic (ROC) curve analysis was used to analyze the efficacy of three methods in diagnosing endolymphatic hydrops. Results:Totally 65 cases were finally diagnosed endolymphatic hydrops clinically. There were statistically significant differences of all the 3 ratios between the affected and healthy ears ( t=9.93, 7.22, 8.20, all P<0.001). The ROC curve showed that the area under the curve (AUC) of endolymph/total lymph area ratio, saccule/utricle area ratio, endolymph/total lymph volume ratio for diagnosis of endolymphatic hydrops were 0.882, 0.768, 0.884 (all P<0.001). And there were no significant differences between each paired AUCs (all P>0.05). Conclusions:All three methods of endolymph/total lymph area ratio, saccule/utricle area ratio, endolymph/total lymph volume ratio can quantitatively evaluate endolymphatic hydrops. The endolymphatic/total lymphatic area ratio method is still the most convenient method at present.

Análise das respostas do VEMP cervical e ocular em indivíduos hígidos / Analysis of cervical and ocular VEMP responses in healthy individuals / Análisis de las respuestas VEMP cervicales y oculares en individuos sanos

Introdução: os potenciais evocados miogênicos vestibulares (VEMP) são respostas eletrofisiológicas que conseguem fornecer informações dos órgãos otolíticos sáculo, utrículo e do nervo vestibular. O VEMP é um exame complementar à avaliação vestibular, consistindo num exame rápido, de fácil aplicação e objetivo. Objetivo: analisar os parâmetros de latência, amplitude, limiar e índice de assimetria das respostas do VEMP cervical (cVEMP) e ocular (oVEMP) de indivíduos sem queixas vestibulares. Métodos: estudo transversal realizado com 53 indivíduos de ambos os sexos, sem queixas auditivas e vestibulares. Resultados: encontrou-se simetria de respostas nas latências, amplitudes e limiares de respostas do exame cVEMP. Entretanto, verificou-se diferença entre orelhas da latência P15 do exame oVEMP, sendo maior à direita no sexo feminino. Conclusão: Encontrou-se simetria nas respostas de todos os parâmetros avaliados do cVEMP. Houve assimetria apenas na latência de P15 do oVEMP no sexo feminino. Os limiares de resposta encontrados nos exames cVEMP e oVEMP foram iguais ou maiores que 75 dBNA.

Introduction: Vestibular evoked myogenic potentials (VEMP) are electrophysiological responses that can provide information on the otolithic organs saccule, utricle and of the vestibular nerve. VEMP is a complementary exam to the vestibular assessment; it is a quick exam, easy to apply and objective. Purpose: to analyze the parameters of latency, amplitude, threshold and asymmetry index of the cervical (cVEMP) and ocular VEMP (oVEMP) responses of individuals without vestibular complaints. Methods: cross-sectional study carried out with 53 individuals of both genders without hearing and vestibular complaints. Results: response symmetry was found in the latencies, amplitudes and thresholds of cVEMP test responses. However, there was a difference between the ears of the P15 latency of the oVEMP exam, and this was greater on the right ear in females. Conclusion: symmetry was found in the responses of all cVEMP evaluated parameters. There was asymmetry in oVEMP P15 latency only in female patients. The response thresholds found in the cVEMP and oVEMP tests were equal or greater than 75 dBHL.

Introducción: los potenciales miogénicos evocados vestibulares (VEMP) son respuestas electrofisiológicas que pueden proporcionar información sobre los órganos otolíticos el sáculo, el utrículo y el nervio vestibular. El VEMP es un examen complementario a la evaluación vestibular; es un examen rápido, fácil de aplicar y objetivo. Objetivo: analizar los parámetros de latencia, amplitud, umbral e índice de asimetría de las respuestas VEMP cervical (cVEMP) y ocular (oVEMP) de individuos sin quejas vestibulares. Métodos: estudio transversal realizado con 53 individuos de ambos sexos, sin quejas auditivas y vestibulares. Resultados: Se encontró simetría de respuestas en las latencias, amplitudes y umbrales de respuestas en el examen cVEMP. Sin embargo, hubo una diferencia entre los oídos de la latencia P15 del examen oVEMP, siendo mayor a la derecha en el sexo femenino . Conclusión: se encontró simetría en las respuestas de todos los parámetros evaluados de cVEMP. Hubo asimetría solo en la latencia P15 de oVEMP en el sexo femenino. Los umbrales de respuesta encontrados en las pruebas cVEMP y oVEMP fueron iguales o superiores a 75 dBHL.

Prosper Menière: the man who located vertigo in the inner ear / Prosper Menière: o homem que localizou a vertigem na orelha interna

ABSTRACT Since the Greek Aellus Galenum (129 AD - c.200/c.216), vertigo was considered a problem attributed to a cerebral disorder, diagnosed as the manifestation of apoplectiform cerebral congestion. In the mid-19th century, the Frenchman Prosper Menière changed this concept by placing the origin of this symptom in the inner ear. The main objective of this historical note is to highlight who Menière was, his work, and some aspects of the disease that bears his name.

RESUMO Desde o grego Aellus Galenum (129 d.C. - c.200/c.216), a vertigem era considerada um problema atribuído a um distúrbio cerebral, diagnosticado como a manifestação de uma 'congestão cerebral apoplectiforme'. Em meados do século 19, o francês Prosper Menière mudou esse conceito, colocando a origem desse sintoma na orelha interna. O principal objetivo desta nota histórica é ressaltar quem foi Menière, seu trabalho e alguns aspectos da doença que leva seu nome.

Multi-parameter MRI signal analysis of labyrinth in patients with sudden deafness / 中华放射学杂志

Objective:To explore the clinical application value of each sequence by analyzing the characteristics of labyrinthine signal on MRI in patients with unilateral sudden deafness.Methods:Totally 52 patients of unilateral sudden deafness with inner ear MRI were analyzed retrospectively at Beijing Tongren Hospital, Capital Medical University from January 2016 to July 2019, all of which could find abnormalities in the labyrinth, including 17 cases of plain scan and 35 cases of enhanced scan, with sequences including plain T 1WI, enhanced T 1WI, plain and enhanced delayed 3D fluid attenuation inversion recovery (3D-FLAIR). The affected labyrinthine signal characteristics of each sequence were analyzed and the involvement sites were judged. The ability of each sequence to show labyrinthine abnormal signal was evaluated and scored. The Friedman test and Wilcoxon signed rank sum test were used to compare the subjective scores of the ability to show labyrinthine high signal in different sequences in plain and enhanced patients, respectively. Fisher′s exact probability method was used to analyze the relationship between the affected sites and the recovery of hearing, tinnitus and vertigo symptoms. Results:Fifty-two patients (100%, 52/52) showed labyrinthine high signal on T 1WI, 8 (15.4%, 8/52) showed higher signal and 3 (5.8%, 3/52) showed low signal on T 2WI. Thirty-five (100%, 35/35) showed high signal on enhanced T 1WI, among which 27 had enhancement (77.1%, 27/35). Fifty-two (100%, 52/52) showed significant high signal of the affected labyrinth on 3D-FLAIR (17 plain scan, 35 enhanced scan). The scores were 2 (2, 2), 3 (2, 3), 3 (3, 4) and 4 (4, 4) of T 1WI, enhanced T 1WI, plain and enhanced 3D-FLAIR respectively. The overall difference in subjective scores of plain T 1WI, enhanced T 1WI and enhanced 3D-FLAIR in enhanced patients was statistically significant (χ2=64.528, P<0.001), and the comparison between the two was statistically different (all corrected P<0.05). The plain 3D-FLAIR score was higher than the plain T 1WI in patients with a statistically significant difference ( Z=-3.729, P<0.001). Twenty-seven cases (51.9%, 27/52) exhibited high signal at the ampulla of semicircular canals, with a statistically significant difference in the distribution of hearing recovery or not ( P=0.001). Conclusions:Both T 1WI and 3D-FLAIR sequences can effectively identify the labyrinthine high signal, but the latter was better than the former of its ability to display, especially delayed enhanced 3D-FLAIR. The high signal at the ampulla of semicircular canals was a characteristic predictor of non-recovery of hearing.

Variaciones anatómicas del origen de la arteria laberíntica en una muestra de encéfalos en Colombia / Anatomical variations of the origin of the labyrinthine artery in a brains sample in Colombia

Resumen Introducción. La arteria laberíntica transcurre a través del meato acústico interno hasta llegar al oído interno, aportando allí su irrigación. Se han descrito dos posibles variantes anatómicas de su origen: como rama directa de la arteria basilar o como rama de la arteria cerebelosa anteroinferior. Objetivo. Identificar la prevalencia del origen de la arteria laberíntica. Materiales y métodos. Se realizó un estudio analítico descriptivo con métodos cuantitativos sobre el origen de la arteria laberíntica en una muestra de 29 encéfalos obtenidos por muestreo a conveniencia. Resultados. Se determinó que la arteria laberíntica se originó de la arteria basilar en 22 (75.9%) encéfalos; en los 7 (24.1%) restantes esta surgía como rama de la arteria cerebelosa anteroinferior. Conclusión. El origen de la arteria laberíntica en la muestra seleccionada tiene una mayor prevalencia en la arteria basilar respecto a la arteria cerebelosa anteroinferior, hallazgo que se asemeja a algunas investigaciones contrastadas con este estudio.

Abstract Introduction: The labyrinthine artery runs through the internal auditory meatus until the inner ear, providing irrigation. Two possible anatomical variants of its origin have been described: as a direct branch of the basilar artery or as a branch of the anterior inferior cerebellar artery. Objective: To identify the prevalence of labyrinthine artery origin. Materials and methods: A descriptive analytical study on the origin of the labyrinthine artery was carried out using quantitative methods in a sample of 29 brains obtained by convenience sampling. Results: It was established that the labyrinthine artery originated from the basilar artery in 22 (75.9%) brains; in the remaining 7 (24.1%), it emerged as a branch of the anterior inferior cerebellar artery. Conclusion: The origin of the labyrinthine artery in the selected sample has a higher prevalence in the basilar artery than in the anterior inferior cerebellar artery, finding that resembles some investigations contrasted with this study.

Avaliação da vertical visual subjetiva em adultos jovens / Evaluation of subjective visual vertical in young adults

RESUMO Objetivo Avaliar a vertical visual subjetiva em indivíduos adultos jovens sem queixas vestibulares e/ou alterações do equilíbrio corporal. Método Estudo do tipo observacional, descritivo, analítico, de delineamento transversal, no qual foram avaliados 50 adultos jovens, com idade entre 18 e 30 anos. Foram excluídos do estudo indivíduos com alteração neurológica, alteração cognitiva evidente, deficiência física que influenciasse no equilíbrio corporal, alteração visual sem uso de lentes corretivas, uso de medicamentos com ação sobre o sistema nervoso central e/ou vestibular, relato de ingestão alcoólica 24 horas antes da avaliação e indivíduos com alterações e/ou queixas vestibulares. Os participantes foram submetidos à anamnese e à avaliação da vertical visual subjetiva, por meio do teste do balde. O teste foi realizado em três condições sensoriais diferentes: 1- Indivíduo sentado, com os dois pés sobre superfície estável (piso de paviflex); 2- Indivíduo sentado, com os pés em cima de uma espuma; 3- Indivíduo em pé sobre uma espuma. Resultados A vertical visual subjetiva não apresentou diferença significativa (p= 0,93) entre as condições sensoriais estudadas. Conclusão Em adultos jovens hígidos, o sistema proprioceptivo não influenciou significativamente a avaliação da vertical visual subjetiva, realizada por meio do teste do balde.

ABSTRACT Objective To evaluate subjective visual vertical in young adults without vestibular complaints and/or body balance problems. Methods This was a descriptive cross-sectional, observational and analytical study that assessed 50 young adults aged 18 to 30 years. Adult were excluded from the study if they had neurological and cognitive disorders, physical disability that affected their balance, visual impairment with no use of corrective lenses, use of drugs with effects on the central nervous system and/or the vestibular system and self-report of alcoholic use 24 hours before the assessment, and adults with vestibular problems and/or complaints The participants answered questions in a medical history interview and underwent subjective visual vertical assessment with the bucket method. The test was performed under three different sensory conditions: 1 - Subjects sitting with both feet on a stable surface (Paviflex® flooring); 2- Subjects sitting with their feet on top of foam; 3- Subjects on top of foam. Results The subjective visual vertical did not show a significant difference (p = 0.93) among the study sensory conditions. Conclusion The proprioceptive system did not significantly influence the measurement of the subjective visual vertical in young healthy adults.

The role of magnetic resonance imaging in Ménière disease: the current state of endolymphatic hydrops evaluation / O papel da ressonância magnética na doença de Ménière: a avaliação da hidropsia endolinfática nos dias atuais

ABSTRACT Technical advances in magnetic resonance imaging have allowed to accurately detect and grade endolymphatic space distension in Ménière disease; this was only possible in post-mortem histological studies until a few years ago. Magnetic resonance imaging rules out other causes of vertigo and hearing loss, and is able to evaluate the cochlear and vestibular compartments of the endolymphatic space using a dedicated protocol.

RESUMO Os avanços técnicos na ressonância magnética têm permitido detectar e classificar com acurácia a distensão do espaço endolinfático na doença de Ménière; isso só era possível nos estudos histológicos post-mortem até poucos anos atrás. Além de afastar outras causas de vertigem e de perda auditiva, a ressonância magnética é capaz de avaliar os compartimentos coclear e vestibular do espaço endolinfático por meio de um protocolo dedicado.

The progress of topical drug delivery to the inner ear for the inhibition of inflammation and fibrosis following cochlear implantation / 中华耳鼻咽喉头颈外科杂志

Nowadays, more than 300 000 deaf people around the world benefit from cochlea implantation. With the extension of cochlear implantation indications, it has become a research focus for how to apply the anti-inflammatory and anti-fibrotic drugs safely and effectively to better retain residual hearing, as well as to protect hair cells and the spiral ganglion cells from apoptosis. Due to the blood-labyrinth barrier, perioperative systemic steroids are often unable to achieve satisfactory drug concentration in the inner ear. Also, given that higher doses of steroids will cause more serious side effects and sometimes steroids are strictly limited to apply on some patients with certain diseases, the attention has been attracted on topical drug delivery to the inner ear. In this review, the methods of topical drug delivery to the inner ear are divided into four categories: transtympanic injection of liquid solutions, tympanic implantation of drug delivery systems, intracochlear injection and modified implant electrode. The results in each category and up to date progress are reviewed.

Malformaciones congénitas del oído interno. Revisión de tema / Congenital Malformations of the Inner Ear

Existe una gran variedad de malformaciones congénitas que pueden afectar al oído interno, con distintas fisiopatologías, diferentes estructuras alteradas y edad de aparición de los síntomas. Por lo anterior, es necesario conocer e identificar dichas alteraciones, con el fin de actuar oportunamente y reducir el riesgo de las complicaciones, entre otras ­de gran importancia­ las alteraciones en el área del lenguaje y en el ámbito social.

There are a great variety of congenital malformations that can affect the inner ear, with a diversity of physiopathologies, involved altered structures and age of symptom onset. Therefore, it is important to know and identify these alterations opportunely to lower the risks of all the complications, being of great importance, among others, the alterations in language development and social interactions.

Screening of microRNAs targeting Notch signaling pathway implicated in inner ear development and the role of microRNA-384-5p / 中华耳鼻咽喉头颈外科杂志

Objective@#To screen the key microRNAs targeting Notch signaling pathway in inner ear and investigate its potential regulating function.@*Methods@#The interaction network and the Core-Notch network, involved with key genes in Notch signal pathway and differential-expressed microRNAs in inner ear, were constructed by bioinformatics methods. The important microRNAs in regulating Notch signaling pathway were screened via topological and GO analysis, followed by in vivo and in vitro investigation.@*Results@#MiRNA-384-5p was identified as a key regulator specifically expressed in mouse brain and inner ear, which could down-regulate Notch1. The Notch1 expression was found significantly down-regulated in miRNA-384-5p-mimic-transfected HeLa cells. The dual-luciferase reporter gene assay further confirmed the effect of miRNA-384-5p on the down-regulation of Notch1 and Dll4 in Notch signaling pathway.@*Conclusions@#The Core-Notch network is constructed to screen microRNAs implicated in inner ear development, and miRNA-384-5p is screened and verified to be target-regulating the Notch signaling pathway, which could be the potential target in the regeneration of impaired hair cells.

Effect of melatonin on expression of Prestin protein in the inner ear of mice following radiotherapy / 中华耳鼻咽喉头颈外科杂志

Objective@#To investigate the effect of melatonin on the expression of prestin protein in the inner ear of mice following a single dose radiation therapy, so as to provide the basis for the mechanism study of radiation induced inner ear injury and its prevention.@*Methods@#Sixty 4-week-old male mice were randomly divided into six groups, including the control group (A group), 50 mg/kg MLT group (B group), 5 mg/kg MLT group (C group), 50 mg/kg MLT + radiotherapy group (D group), 5 mg/kg MLT+ radiotherapy group (E group), and 16 Gy radiotherapy group (F group). Each experimental group was randomly subdivided into two subgroups, which were killed to harvest the cochlea on the 3rd and 7th days following 16 Gy radiation. The specimens were used for immunostaining and Western blot to detect the expression of prestin protein. SPSS 19.0 software was used for statistical analysis.@*Results@#Prestin protein mainly distributed in the lateral membrane above the outer hair cell nucleus. When compared with A, B and C group, the expression of prestin protein in the inner ear was significantly up-regulated in F group (P<0.05). However, D and E group reduced the abnormal expression of prestin following radiotherapy when compared with F group, the difference was statistically significant (P<0.05), and the effect of D group was more significant than E group (P<0.05).@*Conclusions@#The prestin protein of cochlea is mainly distributed in the lateral membrane above the outer hair cell nucleus. Following the high-dose radiotherapy, the prestin expression is upregulated, and melatonin can control the abnormal expression of prestin protein induced by radiotherapy with dose dependent.

Static and dynamic balance of children and adolescents with sensorineural hearing loss / Equilíbrio estático e dinâmico de crianças e adolescentes com perda auditiva sensório-neural

ABSTRACT Objective To assess the static and dynamic balance performance of students with normal hearing and with sensorineural hearing loss. Methods A cross-sectional study assessing 96 students, 48 with normal hearing and 48 with sensorineural hearing loss of both sexes, aged 7 and 18 years. To evaluate static balance, Romberg, Romberg-Barré and Fournier tests were used; and for the dynamic balance, we applied the Unterberger test. Results Hearing loss students showed more changes in static and dynamic balance as compared to normal hearing, in all tests used (p<0.001). The same difference was found when subjects were grouped by sex. For females, Romberg, Romberg-Barré, Fournier and Unterberger test p values were, respectively, p=0.004, p<0.001, p<0.001 and p=0.023; for males, the p values were p=0.009, p<0.001, p<0.001 and p=0.002, respectively. The same difference was observed when students were classified by age. For 7 to 10 years old students, the p values for Romberg, Romberg-Barré and Fournier tests were, respectively, p=0.007, p<0.001 and p=0.001; for those aged 11 and 14 years, the p values for Romberg, Romberg-Barré, Fournier and Unterberger tests were p=0.002, p<0.001, p<0.001 and p=0.015, respectively; and for those aged 15 and 18 years, the p values for Romberg-Barré, Fournier and Unterberger tests were, respectively, p=0.037, p<0.001 and p=0.037. Conclusion Hearing-loss students showed more changes in static and dynamic balance comparing to normal hearing of same sex and age groups.

RESUMO Objetivo Avaliar o desempenho do equilíbrio estático e dinâmico de escolares ouvintes e com perda auditiva sensório-neural. Métodos Estudo de corte transversal que avaliou 96 escolares, sendo 48 ouvintes e 48 com perda auditiva sensório-neural, de ambos os sexos, na faixa etária entre 7 e 18 anos. Para a avaliação do equilíbrio estático, foram utilizados os testes de Romberg, Romberg-Barré e Fournier, e, para avaliar o equilíbrio dinâmico, foi usado o teste de Unterberger. Resultados Os escolares com perda auditiva apresentaram maior ocorrência de alterações no equilíbrio estático e dinâmico, comparados aos ouvintes, em todos os testes empregados (p<0,001). O mesmo foi observado quando os escolares foram agrupados pelos sexos. Para o sexo feminino, os valores de p para os testes de Romberg, Romberg-Barré, Fournier e Unterberger foram, respectivamente, p=0,004, p<0,001, p<0,001 e p=0,023; para o sexo masculino, foram p=0,009, p<0,001, p<0,001 e p=0,002. A mesma diferença foi observada quando os escolares foram estratificados pelas faixas etárias. Para aqueles de 7 a 10 anos, os valores de p foram, respectivamente, para os testes de Romberg, Romberg-Barré e Fournier p=0,007, p<0,001 e p=0,001; entre 11 e 14 anos, para os testes de Romberg, Romberg-Barré, Fournier e Unterberger, foram, respectivamente p=0,002, p<0,001, p<0,001, p=0,015; entre 15 e 18 anos, para os testes Romberg-Barré, Fournier e Unterberger foram, respectivamente, p=0,037, p<0,001 e p=0,037. Conclusão Os escolares com perda auditiva apresentaram maior ocorrência de alterações no desempenho do equilíbrio estático e dinâmico que os ouvintes, do mesmo sexo e faixa etária.

HRCT for diagnosis of internal ear injuries caused by temporal bone trauma / 中国医学影像技术

Objective To explore the value of HRCT for the diagnosis of internal ear injuries caused by temporal bone trauma.Methods Totally 106 patients with temporal bone trauma were scanned by HRCT,and 12 patients with internal ear injuries were collected.MPR of temporal bone (cochlea,vestibule,horizontal semicircular canal,anterior semicircular canal and posterior semicircular canal) was performed on Philips workstation.The locations,types,and the involving structures were observed.Results Among the 106 cases of temporal bone trauma,12 cases were internal ear injuries,including 8 cases of fractures of inner ear,3 cases of pneumolabyrinth,and 1 case of foreign body in the cochlea,which 3 cases complicated with traumatic labyrinthine ossification.Conclusion HRCT and MPR can clearly reveal internal ear injuries,which are effective methods for diagnosis of internal ear injuries.

HRCT for diagnosis of internal ear injuries caused by temporal bone trauma / 中国医学影像技术

Objective To explore the value of HRCT for the diagnosis of internal ear injuries caused by temporal bone trauma.Methods Totally 106 patients with temporal bone trauma were scanned by HRCT,and 12 patients with internal ear injuries were collected.MPR of temporal bone (cochlea,vestibule,horizontal semicircular canal,anterior semicircular canal and posterior semicircular canal) was performed on Philips workstation.The locations,types,and the involving structures were observed.Results Among the 106 cases of temporal bone trauma,12 cases were internal ear injuries,including 8 cases of fractures of inner ear,3 cases of pneumolabyrinth,and 1 case of foreign body in the cochlea,which 3 cases complicated with traumatic labyrinthine ossification.Conclusion HRCT and MPR can clearly reveal internal ear injuries,which are effective methods for diagnosis of internal ear injuries.

Possible reasons for cerebrospinal fluid gusher in cochlear implantation with inner ear abnormality / 中华耳鼻咽喉头颈外科杂志

Objective@#To discuss the possible reasons for cerebrospinal fluid (CSF) gusher in cochlear implantation (CI) with inner ear abnormality.@*Method@#A retrospective analysis was performed on 340 cases who underwent CI from January 2013 to December 2016 in Division of Otology, Otorhinolaryngology Hospital, the First Affiliated Hospital of Zhengzhou University. Among them, 96 cases had inner ear abnormalities. Imaging examinations were performed on these patients, and classification of inner ear malformation was done according to the results.@*Results@#Among the cases with inner ear abnormality, 9.4% (9/96) suffered from CSF gusher during CI. The inner ear abnormalities were found to be as follows: 3 cases had incomplete partition type Ⅰ; 1 case had incomplete partition type Ⅰ with semicircular canal dysplasia; 1 case had common cavity deformity; 1 case had enlarged vestibular aqueducts and common cavity deformity; 2 cases had Mondini deformity. All of these cases had bony defect in the fundus of the internal acoustic meatus observed on CT scans. Another case was type 1 cochlear aqueduct with round window aplasia.@*Conclusions@#Defects in the modiolus or fundus of the internal acoustic meatus is the main reason for CSF gusher during CI. A patent cochlear aqueduct is another possible reason.

The correlation of cochlea endolymphatic hydrops degree and hearing loss and symptoms in patients with Meniere's disease / 中华放射学杂志

Objective To evaluate the correlation between the degree of cochlea endolymphatic hydrops(EH) and hearing loss and symptoms in patients with unilateral Meniere's disease. Methods Fifty seven patients with unilateral Meniere's patients were retrospectively quantitatively analyzed, which evaluated the correlation between the cochlea EH and hearing loss and symptoms. The affected ears in the experimental group(57 ears) and the asymptomatic ears in the control group(57 ears), were confirmed by bilateral intratympanic Gd-DTPA injection and 3D real IR MRI scan after 24 h. The maximum length of endolymph space and labyrinth chamber along the modiolus cochleae and their ratio which represented the endolymph space proportion of each turn(R1, the basal turn;R2, the middle turn;R3, the apical turn) were calculated. And the paired t test was used to compare the differences in EH degree between the experimental and control group;Pearson correlation analysis was used to analyze the correlation between the cochlea EH degree and hearing loss and duration of symptoms. Results R1, R1 and R3 of ipsilateral cochlea were higher than normal cochlea(ipsilateral R1:0.354±0.097 vs. normal R1:0.185±0.031, P0.05). In addition, there was a correlation between the degree of EH in the apical turn and low, medium, high-frequency hearing loss(r=0.271, 0.269, 0.329, respectively; all P<0.05). Conclusions The degree of cochlea EH in the basal and second turn showed great relevance with the high-frequency hearing loss, and the apical turn EH degree was relevant with the low, medium, high-frequency hearing loss, but there was no correlation between the EH degree of each turn, ages and symptoms.

Malformación congénita del oído interno bilateral y del conducto auditivo interno derecho en un paciente con hipoacusia profunda neurosensorial bilateral: Presentación de caso / Congenital Malformation of the Bilateral Inner Ear and the Right Internal Auditory Canal in a Patient with Deep Bilateral Sensorineural Hearing Loss: Case Report

La tomografía computarizada y la resonancia magnética se consideran estudios complementarios que constituyen una herramienta de gran valor para el diagnóstico de malformaciones que afectan el oído interno. Para el estudio de los elementos óseos la tomografía brinda mayor detalle; por su parte, la resonancia magnética se emplea para la evaluación del laberinto membranoso y de los nervios vestibulococlear y facial (VIII y VII pares craneales). Este artículo presenta el caso de un niño de 1 año que cursa con grave hipoacusia neurosensorial bilateral; por medio de TC y RM se pudo determinar malformación congénita del oído interno bilateral tipo cavidad común, la cual está asociada a atresia del conducto auditivo interno derecho. Esto permitió descartar otras patologías dentro de su diagnóstico diferencial, como aplasia coclear, anomalía quística cocleovestibular y aplasia de canales semicirculares.

CT and MRI are complementary studies and are a valuable tool for the diagnosis of malformations affecting the inner ear. Tomography provides greater detail in the study of bony elements. On the other hand, MRI is used to evaluate the membranous labyrinth and the vestibule-cochlear and facial nerve (cranial nerves VIII and VII). This review presents the case of a one-year old child coursing with severe bilateral sensorineural hearing loss. Bilateral congenital malformation common cavity type was diagnosed through CT and MRI, associated with atresia of the right internal auditory canal. This enabled to rule out other pathologies within its differential diagnosis, such as cochlear aplasia, cochleovestibular cystic anomaly and aplasia of semicircular canals.

Outcomes of Severe to Profound Idiopathic Sudden Sensorineural Hearing Loss

OBJECTIVES: While a severe to profound sudden sensorineural hearing loss (SSNHL) may cause serious disability in verbal communication, there have been little studies focusing on this high degree SSNHL. The present study was aimed to investigate the characteristics of hearing recovery in a high degree SSNHL (>70 dB). METHODS: Three hundred and two SSNHL patients were enrolled. For a long-term follow-up, 46 patients were evaluated. Hearing level was examined by pure tone audiometry on day 1, week 3, month 3, month 6, and year 1 or after. According to the degree of the initial hearing loss, the patients were divided into 4 groups from 70 to > or =100 dB. RESULTS: After 3 weeks, the recovery rate and mean hearing gain was 61%, 23.85 dB in the 70 dB group, whereas 10%, 6.61 dB in the > or =100 dB group. There was a significant correlation between 3-week recovery and final hearing outcome. However, there was almost no recovery after 3 months. CONCLUSION: An early recovery can be a prognostic factor for the final recovery in severe to profound SSNHL. Since recovery after 3 months is rare, an early hearing intervention like hearing aid or cochlear implantation should be considered in the high degree SSNHL to restore the patient's verbal communication.

Avaliação e tratamento da Vertigem Postural Paroxística Benigna: o que tem sido realizado nos últimos anos / Assessment and treatment of Benign Positional Paroxysmal Vertigo: what has been done in recent years / Evaluación y tratamiento del Vértigo Posicional Paroxístico Benigno: qué se hahecho en los últimos años

A Vertigem Postural Paroxística benigna é uma afecção do sistema vestibular periférico, caracterizada por crise vertiginosa súbita, de curta duração, desencadeada por movimentações cefálicas em diferentes posições. Levando em consideração o grande número de pacientes com essa fisiopatologia, o presente estudo objetivou apresentar uma revisão da literatura sobre esse assunto. O levantamento bibliográfico foi conduzido nas bases de dados LILACS, Medline e SciELO, focando-se nos artigos científicos publicados entre os anos de 2004 e 2014. Verificou-se nesta revisão que estudos sobre a Vertigem Postural Paroxística Benigna foram realizados nos últimos anos, abordando os métodos de avaliação e tratamento. Foi possível observar que o acometimento dos canais laterais ou de mais de um canal, assim como a vertigem postural positiva subjetiva e as orientações ao paciente, precisam ser mais explorados.

Benign Paroxysmal Positional Vertigo is a disorder of the peripheral vestibular system, characterized by sudden vertigo of short duration, triggered by head movements in different positions. Taking into consideration the large number of patients with this pathophysiology, the present study aimed to present a review of the literature on this subject. The literature survey was conducted in LILACS, Medline and SciELO databases, focusing on the scientific papers published between 2004 and 2014. It was found in this review that studies on Benign Paroxysmal Positional Vertigo have been made in recent years, addressing the methods of evaluation and treatment. It was observed that the onset of side canals or more than one canal, as well as the positive subjective postural vertigo, and instructing the patient, need to be further explored.

El vértigo posicional paroxístico benigno es un trastorno del sistema vestibular periférico, que se caracteriza por crisis vertiginosa súbita, de corta duración, provocada por movimientos de la cabeza en diferentes posiciones. Teniendo en cuenta el gran número de pacientes con esta fisiopatología, el presente estudio tuvo como objetivo presentar una revisión de la literatura sobre este tema. El estudio de la literatura se realizó en las bases de datos LILACS, Medline y SciELO, centrándose en los trabajos científicos publicados entre 2004 y 2014. Se encontró en esta revisión que estudios sobre el Vértigo Posicional Paroxístico Benigno se han hecho en los últimos años, con foco en los métodos de evaluación y tratamiento. Se observó que la acometida de los canales secundarios o de más de un canal, así como el vértigo postural positivo subjetivo, e las instrucciones al paciente, necesitan ser mas explorados.