An unenhanced 3D-FLAIR sequence using long repetition time and constant flip angle to image endolymphatic hydrops.

OBJECTIVES: To evaluate a three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) sequence using a long repetition time (TR) and constant flip angle (CFA) in differentiating between perilymph and endolymph in a phantom study, and unenhanced endolymphatic hydrops (EH) imaging in a patient study. METHODS: Three solutions in similar ion and protein concentrations with endolymph, perilymph, and cerebrospinal fluid were prepared for variable flip angle (VFA) 3D-FLAIR (TR 10,000 ms) and CFA (120°) 3D-FLAIR using different TR (10,000, 16,000, and 20,000 ms). Fifty-two patients with probable or definite Meniere's disease received unenhanced CFA (120°) 3D-FLAIR using a long TR (20,000 ms) and 4-h-delay enhanced CFA (120°) 3D-FLAIR (TR 16,000 ms). Image quality, signal-to-noise ratio (SNR), and contrast-to-noise ratio (CNR) of them were compared. Agreement in the evaluation of the EH degree between them was analyzed. RESULTS: In the phantom study, CNRs between perilymphatic and endolymphatic samples of VFA 3D-FLAIR (TR 10,000 ms) and CFA 3D-FLAIR (TR 10,000, 16,000, and 20,000 ms) were 6.66 ± 1.30, 17.90 ± 2.76, 23.87 ± 3.09, and 28.22 ± 3.15 (p < 0.001). In patient study, average score (3.65 ± 0.48 vs. 4.19 ± 0.40), SNR (34.56 ± 9.80 vs. 51.40 ± 11.27), and CNR (30.66 ± 10.55 vs. 45.08 ± 12.27) of unenhanced 3D-FLAIR were lower than enhanced 3D-FLAIR (p < 0.001). Evaluations of the two sequences showed excellent agreement in the cochlear and vestibule (Kappa value: 0.898 and 0.909). CONCLUSIONS: The CFA 3D-FLAIR sequence using a long TR could be used in unenhanced EH imaging with high accuracy. CLINICAL RELEVANCE STATEMENT: Unenhanced imaging of endolymphatic hydrops is valuable in the diagnosis and follow-up of patients, especially those who cannot receive contrast-enhanced MRI. KEY POINTS: Ion and protein concentration differences can be utilized in differentiating endolymph and perilymph on MRI. Endolymphatic and perilymphatic samples could be differentiated in vitro on this 3D-FLAIR sequence. This unenhanced 3D-FLAIR sequence is in excellent agreement with the enhanced constant flip angle 3D-FLAIR sequence.

Delayed post gadolinium MRI descriptors for Meniere's disease: a systematic review and meta-analysis.

OBJECTIVES: Delayed post-gadolinium magnetic resonance imaging (MRI) detects changes of endolymphatic hydrops (EH) within the inner ear in Meniere's disease (MD). A systematic review with meta-analysis was conducted to summarise the diagnostic performance of MRI descriptors across the range of MD clinical classifications. MATERIALS AND METHODS: Case-controlled studies documenting the diagnostic performance of MRI descriptors in distinguishing MD ears from asymptomatic ears or ears with other audio-vestibular conditions were identified (MEDLINE, EMBASE, Web of Science, Scopus databases: updated 17/2/2022). Methodological quality was evaluated with Quality Assessment of Diagnostic Accuracy Studies version 2. Results were pooled using a bivariate random-effects model for evaluation of sensitivity, specificity and diagnostic odds ratio (DOR). Meta-regression evaluated sources of heterogeneity, and subgroup analysis for individual clinical classifications was performed. RESULTS: The meta-analysis included 66 unique studies and 3073 ears with MD (mean age 40.2-67.2 years), evaluating 11 MRI descriptors. The combination of increased perilymphatic enhancement (PLE) and EH (3 studies, 122 MD ears) achieved the highest sensitivity (87% (95% CI: 79.92%)) whilst maintaining high specificity (91% (95% CI: 85.95%)). The diagnostic performance of "high grade cochlear EH" and "any EH" descriptors did not significantly differ between monosymptomatic cochlear MD and the latest reference standard for definite MD (p = 0.3; p = 0.09). Potential sources of bias were case-controlled design, unblinded observers and variable reference standard, whilst differing MRI techniques introduced heterogeneity. CONCLUSIONS: The combination of increased PLE and EH optimised sensitivity and specificity for MD, whilst some MRI descriptors also performed well in diagnosing monosymptomatic cochlear MD. KEY POINTS: • A meta-analysis of delayed post-gadolinium magnetic resonance imaging (MRI) for the diagnosis of Meniere's disease is reported for the first time and comprised 66 studies (3073 ears). • Increased enhancement of the perilymphatic space of the inner ear is shown to be a key MRI feature for the diagnosis of Meniere's disease. • MRI diagnosis of Meniere's disease can be usefully applied across a range of clinical classifications including patients with cochlear symptoms alone.

High-resolution computed tomography and pure-tone audiometry in patients with otosclerosis in the spongiotic phase.

OBJECTIVE: There is no consensus in the literature regarding the relationship between high-resolution computed tomography findings and hearing thresholds in pure-tone audiometry in otosclerosis. This study evaluated the association between high-resolution computed tomography findings and pure-tone audiometry in otosclerosis in the spongiotic phase. METHODS: A cross-sectional study was conducted of 57 ears with surgically confirmed stapes fixation and tomographic findings. Air conduction and bone conduction thresholds on audiometry, and air-bone gap, were analysed. RESULTS: There were no correlations between sites affected by otospongiosis and air conduction threshold, bone conduction threshold or air-bone gap in the analysed tomographic images, but the diameter of the otospongiotic focus was greater in the presence of extension of the otospongiotic foci to the cochlear endosteum. CONCLUSION: There were no relevant associations between high-resolution computed tomography findings and pure-tone audiometric measurements. However, the diameter of the otospongiotic focus was greater in the presence of extension of the otospongiotic foci to the cochlear endosteum.

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.

Radiological measurement of cochlear dimensions in cochlear hypoplasia and its effect on cochlear implant selection.

OBJECTIVE: To determine the effect of cochlear dimensions on cochlear implant selection in cochlear hypoplasia patients. METHODS: Temporal bone computed tomography images of 36 patients diagnosed with cochlear hypoplasia between 2010 and 2016 were retrospectively reviewed and compared with those of 40 controls without sensorineural hearing loss. RESULTS: Basal turn length and mid-modiolar height were significantly lower in the cochlear hypoplasia patients with subtypes I, II and III than in the control group (p < 0.001). Mid-scalar length was significantly shorter in subtype I-III patients as compared with the control group (p < 0.001). In addition, cochlear canal length (measured along the lateral wall) was significantly shorter in subtype I-IV patients than in the control group (subtypes I-III, p < 0.001; subtype IV, p = 0.002). CONCLUSION: Cochlear hypoplasia should be considered if basal turn length is less than 7.5 mm and mid-modiolar height is less than 3.42 mm. The cochlear implant should be selected according to cochlear hypoplasia subgroup. It is critically important to differentiate subtype II from incomplete partition type I and subtype III from a normal cochlea, to ensure the most appropriate implant electrode selection so as to optimise cochlear implantation outcomes.

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.

Management of transmodiolar and transmacular cochleovestibular schwannomas with and without cochlear implantation.

INTRODUCTION: Hearing rehabilitation with cochlear implants has attracted increasing interest also for patients with cochleovestibular schwannoma. The authors report their experience with the surgical management of tumors with rare transmodiolar or transmacular extension and outcomes after cochlear implantation (CI). METHODS: This retrospective case series included nine patients with either primary intralabyrinthine tumors or secondary invasion of the inner ear from the internal auditory canal. The primary endpoint with CI, performed in six patients, was word recognition score at 65 dB SPL (sound pressure level). Secondary endpoints were intra- and postoperative electrophysiological parameters, impedance measures, the presence of a wave V in the electrically evoked (via the CI) auditory brainstem responses, the specifics of postoperative CI programming, and adverse events. RESULTS: Hearing rehabilitation with CI in cases of transmodiolar tumor growth could be achieved only with incomplete tumor removal, whereas tumors with transmacular growth could be completely removed. All six patients with CI had good word recognition scores for numbers in quiet conditions (80-100% at 65 dB SPL, not later than 6 to 12 months post CI activation). Four of these six patients achieved good to very good results for monosyllabic words within 1-36 months (65-85% at 65 dB SPL). The two other patients, however, had low scores for monosyllables at 6 months (25 and 15% at 65 dB SPL, respectively) with worsening of results thereafter. CONCLUSIONS: Cochleovestibular schwannomas with transmodiolar and transmacular extension represent a rare entity with specific management requirements. Hearing rehabilitation with CI is a principal option in these patients.

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.

[Management of transmodiolar and transmacular cochleovestibular schwannomas with and without cochlear implantation. German version]. / Management transmodiolärer und transmakulärer Vestibularis- und Cochlearisschwannome mit und ohne Cochleaimplantation.

INTRODUCTION: Hearing rehabilitation with cochlear implants has attracted increasing interest also for patients with cochleovestibular schwannoma. The authors report their experience with the surgical management of tumors with rare transmodiolar or transmacular extension and outcomes after cochlear implantation (CI). METHODS: This retrospective case series included nine patients with either primary intralabyrinthine tumors or secondary invasion of the inner ear from the internal auditory canal. The primary endpoint with CI, performed in six patients, was word recognition score at 65 dB SPL (sound pressure level). Secondary endpoints were intra- and postoperative electrophysiological parameters, impedance measures, the presence of a wave V in the electrically evoked (via the CI) auditory brainstem responses, the specifics of postoperative CI programming, and adverse events. RESULTS: Hearing rehabilitation with CI in cases of transmodiolar tumor growth could be achieved only with incomplete tumor removal, whereas tumors with transmacular growth could be completely removed. All six patients with CI had good word recognition scores for numbers in quiet conditions (80-100% at 65 dB SPL, not later than 6 to 12 months post CI activation). Four of these six patients achieved good to very good results for monosyllabic words within 1-36 months (65-85% at 65 dB SPL). The two other patients, however, had low scores for monosyllables at 6 months (25 and 15% at 65 dB SPL, respectively) with worsening of results thereafter. CONCLUSIONS: Cochleovestibular schwannomas with transmodiolar and transmacular extension represent a rare entity with specific management requirements. Hearing rehabilitation with CI is a principal option in these patients.

[Cochleo-vestibular lesions and prognosis in patients with profound sudden sensorineural hearing loss: a comparative analysis].

Objective: To investigate the characteristics of cochleo-vestibular dysfunction in patients with profound sudden deafness, and the prognosis of inner ear hemorrhage. Methods: From January 2017 to December 2018, 92 inpatients with profound sudden sensorineural hearing loss were enrolled in the Department of Otorhinolaryngology, First Affiliated Hospital of Sun Yat-sen University. Our studied patients included 47 males and 45 females, aged 20-78 (39.3±6.1) years. According to the results of inner ear magnetic resonance imaging (MRI), the patients were divided into two groups: inner ear hemorrhage group and non-inner ear hemorrhage group. The clinical features, vestibular tests and audiological examination results during follow up were compared between the two groups. SPSS 22.0 software was used for statistical analysis. Results: The inner ear hemorrhage group consisted of 32 cases (34.8%, 32/92), all of whom complained of vertigo (100%, 32/32). Simultaneous vertigo attack and hearing loss occurred in 78.1% of this group (24/32). Neither semicircular canals function, nor cervical vestibular evoked myogenic potential (c-VEMP), nor ocular vestibular evoked myogenic potential (o-VEMP) in the affected side was normal (100%, 32/32). The rates of benign paroxysmal positional vertigo (BPPV) and disequilibrium were 37.5% (12/32) and 25.0% (8/32) respectively. Hearing improved in 28.1% (9/32) two weeks after treatment, and became stable at one month's follow up. In 60 cases without inner ear hemorrhage, 58.3% of them (35/60) experienced vertigo, which occurred simultaneously with hearing loss in 21 patients (60%, 21/35). The abnormal rates of semicircular canals function, c-VEMP and o-VEMP were 71.6% (43/60), 78.3% (47/60) and 66.7% (40/60), respectively. The incidence of BPPV was 16.7% (10/60) and 8.3% (5/60) in cases with disequilibrium. Hearing improved in 58.3% (35/60) two week after treatment, and became stable at three months' follow up. Significant difference was found in either vertigo rate, or simultaneous vertigo/hearing loss rate, or abnormal c-VEMP/o-VEMP rates, or accompanying BPPV, or disequilibrium rates between the two groups (P<0.05 each). Moreover, we observed better hearing recovery in non-inner ear hemorrhage group in the two weeks, one month, three months and six months' follow up, when compared with those in inner ear hemorrhage groups (P<0.05 each). Conclusions: Inner ear hemorrhage is associated with more severe cochlea-vestibular lesion and poorer prognosis, in comparison to the non-inner ear hemorrhage,in patients with profound sudden sensorineural hearing loss.

Inner-ear malformations as a cause of single-sided deafness.

OBJECTIVE: To determine the prevalence and distribution of inner-ear malformations in congenital single-sided deafness cases, as details of malformation type are crucial for disease prognosis and management. METHODS: A retrospective study was conducted of 90 patients aged under 16 years with congenital single-sided deafness. Radiological findings were evaluated using computed tomography and magnetic resonance imaging. Inner-ear malformations were identified and cochlear nerve status was determined in affected ears. RESULTS: Out of 90 ears, 42 (46.7 per cent) were found to have inner-ear malformation. Isolated cochlear aperture stenosis was the most common anomaly (n = 18, 20 per cent), followed by isolated cochlear aperture atresia (n = 11, 12.2 per cent) and cochlear hypoplasia (n = 7, 7.8 per cent). Cochlear nerve deficiency was encountered in 41 ears (45.6 per cent). The internal auditory canal was also stenotic in 49 ears (54.4 per cent). CONCLUSION: Inner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type.

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.

[The progress of topical drug delivery to the inner ear for the inhibition of inflammation and fibrosis following cochlear implantation].

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.

[Magnetic resonance imaging study of endolymphatic hydrops based on 3D-FLAIR sequence after single-dose intravenous gadolinium injection].

Objective: To investigate the visualization of endolymph in patients with otogenic vertigo by intravenous administration of single dose of gadolinium contrast agents and magnetic resonance three-dimensional fluid-attenuated inversion recovery sequence (3D-FLAIR MRI), and further assess the extent of endolymphatic hydrops. Methods: From Beijing Tongren Hospital of Capital Medical University between October 2017 and June 2018, 30 patients (16 males, 14 females) with unilateral otogenic vertigo were involved in this study, with the age of 30 to 68 years, mean age of (53±10) years. Eight hours after intravenous administration of single dose (0.1 mmol/kg, body weight) of gadopentetate (Gd-DTPA), 3D-FLAIR sequence was performed in 30 patients. The location of endolymphatic hydrops was observed and then the degree of hydrops was quantitatively elevated by two radiologists. The consistency test was used to analyze the location and degree of endolymphatic hydrops in the two radiologists and the paired t-test was used to compare the difference between the affected and healthy side of endolymphatic spaces of the patients with otogenic vertigo. Results: In 30 patients, the gadolinium distributed in all parts of the perilymph inside the inner ear, and can accurately outline the boundaries of the peri-and endolymph. Twenty-six patients (26/30, 86.7%) were found to have unilateral endolymphatic hydrops, including 18 mild hydrops, 8 significant hydrops. The two radiologists had a very good agreement on the assessment of endolymphatic hydrops(kappa=0.864, ICC=0.959). In the 3D-FLAIR MR images of 26 patients with endolymphatic hydrops, the saccule (26/26, 100%) had a higher rate of hydrops than the cochlea and utricle(16/26, 61.5%; 14/26, 53.8%), and two radiologists had a very good agreement on the location of endolymphatic hydrops(kappa=0.820). Moreover, there was a significantly statistical difference between the affected and healthy area of the endolymphatic space in this study (P<0.01). Conclusion: The technique of 3D-FLAIR MR imaging through single dose intravenous gadolinium injection is feasible, which can estimate endolymphatic hydrops in patients with otogenic vertigo, and accurately classify the degree of hydrops.

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.