Enlarged vestibular aqueduct as a cause of postneonatal deafness.

INTRODUCTION: The enlarged vestibular aqueduct (EVA) is the most frequent malformation of the inner ear associated with sensorineural hearing loss (5-15%). It exists when the diameter in imaging tests is greater than 1.5 mm at its midpoint. The association between hearing loss and EVA has been described in a syndromic and non-syndromic manner. It can appear as a familial or isolated form and the audiological profile is highly variable. The gene responsible for sensorineural hearing loss associated with EVA is located in the same region described for Pendred syndrome, where the SCL26A4 gene is located. OBJECTIVE: To describe a series of children diagnosed with EVA in order to study their clinical and audiological characteristics, as well as the associated genetic and vestibular alterations. METHOD: Retrospective study of data collection of children diagnosed with EVA, from April 2014 to February 2023. RESULTS: Of the 17 cases, 12 were male and 5 were female. 5 of them were unilateral and 12 bilateral. In 5 cases, a cranial traumatism triggered the hearing loss. Genetic alterations were detected in 3 cases: 2 mutations in the SCL26A4 gene and 1 mutation in the MCT1 gene. 13 patients (76.5%) were rehabilitated with hearing aids and 9 of them required cochlear implantation. DISCUSSION: The clinical importance of AVD lies in the fact that it is a frequent finding in the context of postneonatal hearing loss. It is convenient to have a high suspicion to diagnose it with imaging tests, to monitor its evolution, and to rehabilitate early.

Audiological evaluation (128-20,000Hz) in women with autoimmune thyroiditis: The role of antibodies vs. l-thyroxine deficiency.

BACKGROUND AND AIM: Although sensorineural hearing loss may have different aetiologies, we focused on autoimmune hearing loss since it may be reversible with corticosteroid therapy; this entity is sometimes associated with systemic autoimmune diseases. Hashimoto's thyroiditis or chronic autoimmune thyroiditis shows antibodies and may be harmful to hearing thresholds regardless of hypothyroidism effect. To date this effect has not been sufficiently studied and never with extended high frequencies. The aim of this work is to study by age groups whether hearing thresholds in the human auditory range (128-20,000Hz) are affected in Hashimoto's disease. MATERIALS AND METHODS: Two groups of 128 patients affected by Hashimoto's thyroiditis were included. First group: patients with pathological antithyroid antibodies who do not need L-thyroxine treatment. Second group: patients controlled with L-thyroxine substitutive treatment. Audiometric threshold study comparing between the groups of patients and a group of 209 controls was performed. All patients underwent complete otorhinolaryngological examination, antithyroid antibodies, TSH, T3 and T4 blood levels, tympanometry, conventional pure-tone audiometry, and extended-high-frequency audiometry. RESULTS: All patients were women. Both groups showed worst audiometric thresholds than the control group; both study groups showed worse hearing than controls, this difference was statistically significant in all frequencies. In the 8-20kHz frequency range, this difference was more than 10dB, and in the 9-16kHz and 20kHz range this difference was more than 20dB. When separated by age groups, in younger subjects (20-29 years) these differences were found in all frequencies, except for conversational frequencies (500-4,000Hz); between 30 and 49 years the difference is statistically significant in all frequencies; and from 50 to 69 years differences are found, especially in the conversational frequencies. CONCLUSIONS: This first work studying the human auditory range in the chronic autoimmune thyroiditis or Hashimoto's thyroiditis confirms that hearing loss related to the autoimmune disorder predominates at extended-high-frequencies initially. But ends up involving all frequencies in pure-tone conventional audiometry, then it may be detected in routine clinical tests. These results support the role of extended-high-frequencies audiometry to diagnose subclinical hearing loss in patients affected by Hashimoto's thyroiditis.

Estudio de la audición (128-20.000 Hz) en mujeres con tiroiditis autoinmune: papel de los anticuerpos frente al déficit de hormona tiroidea / Audiological evaluation (128-20.000 Hz) in women with autoinmune thyroiditis: The role of antibodies vs. l-thyroxine deficiency

Antecedentes y objetivo: Hay muchas causas de hipoacusia neurosensorial, entre las que tiene especial interés la patología autoinmune del oído interno por su posible reversibilidad con tratamiento esteroideo, a veces asociada a enfermedades sistémicas autoinmunes. La tiroiditis crónica autoinmune o de Hashimoto (TA) presenta anticuerpos que podrían afectar al oído interno independientemente del hipotiroidismo, efecto que no ha sido suficientemente estudiado y nunca mediante audiometría con extensión en altas frecuencias.El objetivo de este trabajo es estudiar si existe afectación de los umbrales auditivos en pacientes de TA, sin hipotiroidismo, en la totalidad del espectro auditivo humano (128 Hz – 20 kHz) distribuido por grupos de edad.Materiales y métodosSe han seleccionado 128 pacientes divididos en dos grupos. El primer grupo de pacientes presenta anticuerpos antitiroideos elevados sin necesitar tratamiento sustitutivo con tiroxina. El segundo grupo con tratamiento sustitutivo con tiroxina, bien controlados. Se comparan con el grupo control (GC) de 209 pacientes. En todos se realizó historia clínica, exploración otológica, estudio de niveles de anticuerpos antitiroideos, TSH (thyroid-stimulating hormone), T3 y T4 libres, timpanograma, estudio audiométrico convencional y con extensión en altas frecuencias.ResultadosTodos los pacientes fueron mujeres. Ambos grupos mostraron peor audición que los controles, siendo la diferencia estadísticamente significativa en todas las frecuencias; en el rango de frecuencias de 8 – 20 kHz con una diferencia de más de 10 dB, y en los rangos de 9-16 kHz y de 20 kHz de más de 20 dB. (AU)

Background and aim: Although sensorineural hearing loss may have different aetiologies, we focused on autoimmune hearing loss since it may be reversible with corticosteroid therapy; this entity is sometimes associated with systemic autoimmune diseases. Hashimoto's thyroiditis or chronic autoimmune thyroiditis shows antibodies and may be harmful to hearing thresholds regardless of hypothyroidism effect. To date this effect has not been sufficiently studied and never with extended high frequencies. The aim of this work is to study by age groups whether hearing thresholds in the human auditory range (128 to 20.000 Hz) are affected in Hashimoto's disease.Materials and methodsTwo groups of 128 patients affected by Hashimoto's thyroiditis were included. First group: patients with pathological antithyroid antibodies who do not need L-thyroxine treatment. Second group: patients controlled with L-thyroxine substitutive treatment. Audiometric threshold study comparing between the groups of patients and a group of 209 controls was performed. All patients underwent complete otorhinolaryngological examination, antithyroid antibodies, TSH, T3 and T4 blood levels, tympanometry, conventional pure-tone audiometry, and extended-high-frequency audiometry.ResultsAll patients were women. Both groups showed worst audiometric thresholds than the control group; both study groups showed worse hearing than controls, this difference was statistically significant in all frequencies. In the 8-20 kHz frequency range this difference was more than 10 dB, and in the 9-16 kHz and 20 kHz range this difference was more than 20 dB. When separated by age groups, in younger subjects (20-29 years) these differences were found in all frequencies, except for conversational frequencies (500 - 4,000 Hz); between 30 and 49 years the difference is statistically significant in all frequencies; and from 50 to 69 years differences are found, especially in the conversational frequencies. (AU)

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

Sordera neurosensorial congénita con malformación de oído interno ligada al X en una familia mexicana / Congenital sensorineural deafness X-linked, with inner ear malformation in a Mexican family

Introducción. Las sorderas o hipoacusias prelinguales son de etiología genética entre el 60 y el 68% de los casos; de estos, del 20 al 40% son malformaciones del oído interno. De los casos de hipoacusia no sindrómica ligada al X se han descrito siete tipos. De las malformaciones de oído interno, la partición coclear incompleta tipo III es la menos frecuente.Objetivo. Presentar el reporte clínico-genético de una familia mexicana, con indi-viduos varones afectados por sordera neurosensorial congénita con malformación de oído interno. Material y Métodos. Se realizó estudio de una familia en la que nueve miembros presentaban sordera. Se estudiaron cuatro de ellos y una madre sin manifestaciones, a través del estudio clínico general por médico genetista, el estudio audiológico (otos-copía y audiometría) por médico audiólogo y el estudio de tomografía computada (TC) por médico radiólogo.Resultados. Los pacientes estudiados presentaron sordera neurosensorial congéni-ta, de severa a profunda bilateral. A través de la TC, se evidenció malformación de oído interno. Tres pacientes presentaron partición coclear incompleta tipo III y un paciente partición incompleta tipo I. Debido al estudio clínico y al árbol genealógico, se definió diagnóstico de hipoacusia neurosensorial no sindrómica ligada al X. La TC de la madre sin manifestaciones no presentó evidencia de malformaciones en oído interno (MOI).Conclusión. El estudio de imagen es fundamental para definir presencia o no de MOI en todos los pacientes con hipoacusia y así poder guiar la terapéutica y el aseso-ramiento genético, así como realizar los estudios moleculares más adecuados

Introduction. The pre-lingual deafness or hearing loss are of genetic cause in be-tween 60% and 68% of cases, among these, between 20% and 40% are malforma-tion of the inner ear. From the non-syndromic hearing loss cases that are linked to the X chromosome, seven types have been described. Among these inner ear malforma-tions, incomplete cochlear partition type III is the less frequent.Objective. Present the clinical genetical report of a Mexican family, with male in-dividuals affected by congenital neurosensory deafness with inner ear malformation.Materials and methodology. A study on a family in which nine members were affected by deafness was done. Four of them, plus a mother without manifestation, were studied through a general clinical study by a geneticist, an audiological study (otoscopy and audiometry) by an audiologist, and a computed tomography (CT) scan by a radiologist.Results. The studied patients presented congenital neurosensory deafness, from se-vere to deep bilateral. Via the CT, the inner ear malformation was made clear. Three of the patients presented incomplete cochlear partition type III and one patient in-complete cochlear partition type I. Due to the clinical study and the family tree, it was diagnosed non-syndromic neurosensory deafness linked to X. The CT of the mother without manifestation did not show evidence of inner ear malformations.Conclusion. The study by image is fundamental to define whether there is or not a presence of inner ear malformations in any patient with heading loss to be able to guide the therapeutics and the genetic counseling, as well as to make more accurate molecular studies

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.

Disección anatómica del oído medio y el oído interno / Middle and inner ear anatomical dissection / Dissecção anatómica do ouvido médio e do ouvido interno

RESUMEN Introducción: El oído medio y el oído interno presentan una compleja constitución anatómica; La disección como método de enseñanza proporciona una comprensión óptima de la configuración tridimensional de sus estructuras debido a su carácter práctico y realista. Se propone un protocolo de disección aislada del oído medio e interno que ofrece una visión amplia de sus estructuras e incorpora esquemas didácticos. Metodología: En tres cadáveres humanos en estado de conservación se delimitaron dos cuadrángulos en la cara _ superior del hueso temporal mediante referentes anatómicos para extraer una estructura paralelepípeda correspondiente a la porción petrosa del hueso temporal, sobre la cual se realizó disección. Resultados: Se creó un protocolo de disección siguiendo esta secuencia: Extracción parcial de la porción petrosa del hueso temporal, visualización del oído medio, exposición de la membrana timpánica y visualización del oído interno. Conclusión: Esta herramienta pedagógica es aplicable al proceso de enseñanza y constituye un recurso cuyo carácter didáctico resulta potencialmente útil para los estudiantes facilitando el aprendizaje activo de los aspectos teóricos y prácticos del oído.

ABSTRACT Introduction: The middle and the inner ear have a complex anatomical constitution. As a traditional teaching method, the dissection provides an optimal knowledge of the three-dimensional configuration of its structures due to its practical and realistic qualities. It proposes a protocol of isolated dissection of the middle and the inner ear offering a broad vision of its structures and incorporates didactic schemes. Materials and Method: We delimited two quadrants in the upper face of the temporal bone of three conserved human corpses by employing anatomical referents to extract a parallelepiped structure that corresponds to the petrous portion of the temporal bone. On this portion, the dissection was performed. Results: we created a dissection protocol following this sequence: The petrous portion of the temporal bone partial removal, middle ear visualization, tympanic membrane exposure and inner ear visualization. Conclusion: This pedagogical instrument is applicable to the process of education and constitutes a resource whose didactic character results potentially useful for the students facilitating the active learning of the theoretical and practical issues of the ear anatomy

RESUMO Introdução: O ouvido médio e interior apresentam uma constituição anatómica complexa; a dissecação como método de ensino proporciona uma compreensão óptima da configuração tridimensional das suas estruturas devido ao seu carácter prático e realista. É proposto um protocolo de dissecção isolada do ouvido médio e interno, que oferece uma visão ampla das suas estruturas e incorpora diagramas didácticos. Metodologia: Em três cadáveres humanos conservados, dois quadrantes foram delimitados na face superior do osso temporal por meio de referências anatómicas, a fim de extrair uma estrutura paralelepipédica correspondente à porção petrosa do osso temporal, sobre a qual foi realizada a dissecação. Resultados: Foi criado um protocolo de dissecação seguindo esta sequência: extracção parcial da porção petrosa do osso temporal, visualização do ouvido médio, exposição da membrana timpánica e visualização do ouvido interno. Conclusão: Esta ferramenta pedagógica é aplicável ao processo de ensino e constitui um recurso cujo carácter didáctico é potencialmente útil para os estudantes, facilitando a aprendizagem activa dos aspectos teóricos e práticos do ouvido.

Histology and immunocytochemical localization of glial fibrillary acidic protein in the inner ear of Scincella tsinlingensis / Histología y localización inmunocitoquímica de la proteína ácida fibrilar glial en el oído interno de Scincella tsinlingensis

SUMMARY: The microstructure of inner ear in Scincella tsinlingensis was observed by light microscopy and the expression of glial fibrillary acidic protein (GFAP) in membranous labyrinth among the juvenile age group, subadult age group and adult age group were also detected by methods of immunohistochemistry. The inner ear in S. tsinlingensis resembled those in other Scincid lizards in their anatomy and histology. Large and elongate cochlear duct was slightly bowed or arched laterally. There was no hint of limbic modifications and the limbic lip was absent in cochlear recess. The basilar papilla elongated anteroventrally possessed specialized tectorial sallets. GFAP staining was significantly distributed in supporting cells of the sensory epithelia of cochlear duct, while the utricular macula and canal ampullae showed immunopositive for the GFAP antibody, with weaker staining in the saccular macula. The membranous inner ear of three different age groups revealed the similar pattern of GFAP expression, which suggested that the distribution of supporting cells were independent of age in S. tsinlingensis.

RESUMEN: La microestructura del oído interno en Scincella tsinlingensis fue analizada mediante microscopía óptica y por otra parte, fue cuantificada la expresión de la proteína ácida fibrilar glial (GFAP) en el laberinto membranoso, entre los grupos de edad juvenil, subadulto y adulto, utilizándose métodos inmunohistoquímicos. El oído interno de S. tsinlingensis se asemejaba al de otros lagartos Scincid tanto en su anatomía como en su histología. El conducto coclear mayor estaba ligeramente arqueado o arqueado lateralmente. No había indicios de modificaciones límbicas y no se evidenció el labio en el receso coclear. La papila basilar alargada anteroventralmente poseía sallets tectoriales especializados. La tinción de GFAP se distribuyó significativamente en las células del epitelio sensorial del conducto coclear, mientras que la mácula utricular y la ampolla del canal mostraron inmunopositividad para el anticuerpo GFAP, con una tinción más débil en la mácula sacular. El oído interno membranoso de los tres grupos de edad diferentes reveló un patrón similar de expresión de GFAP, lo que sugiere que la distribución de las células de soporte son independiente de la edad en S. tsinlingensis.

Comparação das respostas do VEMP cervical e ocular em indivíduos com e sem doenças otoneurológicas / Comparison of cervical and ocular VEMP responses in individuals with and without otoneurological diseases / Comparación de respuestas VEMP cervicales y oculares en individuos con y sin enfermedades otoneurológicas

Introdução: a avaliação vestibular é realizada tradicionalmente por meio da prova calórica para avaliação do labirinto em indivíduos com tonturas, porém este exame não avalia toda a via vestibular. Os potenciais evocados miogênicos vestibulares (VEMP) consistem em um exame de rápida execução, considerado método de escolha para avaliação dos órgãos otolíticos e do nervo vestibular. Objetivo: analisar as respostas do exame VEMP cervical (cVEMP) e ocular (oVEMP) em indivíduos com doenças vestibulares e compará-las àquelas obtidas em indivíduos de mesma faixa etária e sexo sem queixas de tontura pregressas e atuais. Métodos: Estudo observacional, transversal e analítico. Os participantes foram divididos em dois grupos: grupo com tontura (GT), composto por indivíduos com diversas doenças vestibulares e o grupo sem queixa de tontura (GC). Ambos os grupos foram submetidos à meatoscopia, ao VEMP cervical e ocular. Resultados: A amostra foi composta por 45 indivíduos com a idade entre 23 e 68 anos, sendo 27 indivíduos do grupo GC e 18 do grupo GT. No exame cVEMP as latências, amplitudes, índice de assimetria e o índice de assimetria corrigido foram iguais no GT quando comparado com o GC. No exame oVEMP encontrou-se a amplitude esquerda maior e a latência N10 menor estatisticamente no grupo GT quando comparado ao grupo GC. Dentre as doenças do grupo GT os indivíduos com deiscência de canal superior tiveram valores maiores na amplitude esquerda, o que pode ter interferido nos resultados. Conclusão: Não foram evidenciadas diferenças nas respostas do cVEMP entre os grupos neste estudo. Encontrou-se aumento da amplitude esquerda e o valor menor da latência N10 direita no grupo com tontura na análise do exame oVEMP. Acredita-se que a heterogeneidade de doenças vestibulares no grupo com tontura e o reduzido número de participantes em ambos os grupos tenham contribuído para esse desfecho.

Introduction: The vestibular assessment is traditionally carried out with the caloric test to evaluate the labyrinth in individuals with dizziness. However, this examination does not evaluate the entire vestibular pathway. The vestibular evoked myogenic potentials (VEMP) are a quick test, considered a choice method to assess the otolith organs and the vestibular nerve. Purpose: To analyze the responses of the cervical (cVEMP) and ocular VEMP (oVEMP) examinations in individuals with vestibular diseases and compare them with the results obtained in individuals of the same age group and gender without previous and current complaints of dizziness. Methods: In this observational, cross-sectional, analytical study, the participants were divided into two groups: the dizziness group (DG), composed of individuals with several vestibular diseases, and the group without complaints of dizziness (CG). Both groups underwent meatoscopy, and cervical and ocular VEMP. Results: The sample comprised 45 individuals aged 23 to 68 years ­ 27 individuals in the CG and 18 in the DG. In the cVEMP exam, the latencies, amplitudes, asymmetry index, and the corrected asymmetry index were the same in the GT when compared to the GC. In the cVEMP examination, the latencies, amplitudes, asymmetry index, and corrected asymmetry index were equal in the DG when compared with the CG. In the oVEMP examination, the left amplitude was statistically greater and the N10 latency, smaller in the DG when compared with the CG. Of the diseases in the DG, individuals with superior canal dehiscence had higher left amplitude values, which may have interfered with the results.Conclusion: There were no differences in cVEMP responses between the groups in this study. An increase in the left amplitude and the lower value in the right N10 latency were found in the DG in the analysis of the oVEMP examination. It is believed that the heterogeneity of vestibular diseases in the DG and the small number of participants in both groups have contributed to this outcome.

Introducción: La evaluación vestibular se lleva a cabo tradicionalmente a través de la prueba calórica para evaluar el laberinto en individuos con mareos, sin embargo, este examen no evalúa toda la vía vestibular. Los potenciales miogénicos evocados vestibulares (VEMP) consisten en una prueba de funcionamiento rápido, considerada el método de elección para evaluar los órganos otolíticos y el nervio vestibular. Objetivo: Analizar las respuestas del examen VEMP cervical y ocular en individuos con enfermedades vestibulares y compararlas con las obtenidas en individuos del mismo grupo de edad y sexo sin quejas de mareos previas y actuales. Métodos:Estudio observacional, transversal y analítico. Los participantes se dividieron en dos grupos: grupo con mareos (GT), compuesto por individuos con varias enfermedades vestibulares y el grupo sin quejas de mareos (CG). Ambos grupos se sometieron a meatoscopia, VEMP cervical y ocular. Resultados: La muestra consistió en 45 individuos con edades comprendidas entre 23 y 68 años, 27 individuos del grupo GC y 18 del grupo GT. En el examen cVEMP, las latencias, amplitudes, índice de asimetría y el índice de asimetría corregido fueron los mismos en el GT en comparación con el CG. En el examen oVEMP, la amplitud izquierda fue mayor y la latencia N10 fue estadísticamente menor en el grupo GT en comparación con el grupo CG. Entre las enfermedades en el grupo GT, los individuos con dehiscencia del canal superior tenían valores más altos en la amplitud izquierda, lo que puede haber interferido con los resultados. Conclusión: No hubo diferencias en las respuestas de cVEMP entre los grupos en este estudio. Se encontró un aumento en la amplitud izquierda y un valor más bajo de la latencia N10 derecha en el grupo con mareos en el análisis del examen oVEMP. Se cree que la heterogeneidad de las enfermedades vestibulares en el grupo de mareos y el pequeño número de participantes en ambos grupos contribuyeron a este resultado.

Clinical Profile and Results Obtained in Patients Treated by Auditory Brainstem Implants. / Perfil clínico y resultados obtenidos en los pacientes tratados mediante implante auditivo del tronco del encéfalo.

INTRODUCTION: Cochlear implants have been able to treat some types of hearing loss, but those related to cochlear nerve impairment made it necessary to find new ways to manage these deficits; leading to auditory brainstem implants (ABI). AIM: Our objective is to present the clinical profile of patients treated through an ABI and the results obtained from 1997 to 2017. MATERIAL AND METHODS: On the one hand, patients with statoacoustic nerve tumours (VIIIcranial nerve) were selected, and on the other hand, patients withoutVIII tumours with congenital malformations of the inner ear. Before and after the placement of the ABI, hearing was assessed through tonal audiometry, from which the PTA (Pure Tone Average) and the CAP (Categories of Auditory Performance) scale were obtained. RESULTS: A total of 20 patients undergoing ABI surgery were included. Eight were of tumour cause (40%) and 12 non-tumour (60%). In 15 subjects (75%) a suboccipital approach was performed and in 5 (25%) translabyrinthine. The mean of active electrodes before the implantation of Cochlear® (Nucleus ABI24) was 13/21 (61.90%) versus 8.5/12 (70.83%) of the Med-el® (ABI Med-el). An improvement in the mean PTA of 118.49dB was found against 46.55dB at 2years. On the CAP scale, values of1 were obtained in the preimplantation and of 2.57 (1-5) in the 2-year revision. CONCLUSION: The ABI is a safe option, and with good hearing results when the indication is made correctly.

Hipoacusia Neurosensorial súbita y COVID 19 / Sudden Sensirineural Hearing Loss (SSHL) and COVID 19

La enfermedad por COVID 19 es una infección viral causada por el agente etiológico SARS Cov 2; presenta una gama de características clínicas incluyendo síntomas neurológicos, afectando neuronas, células gliales y nervios; formando parte del grupo de etiología de la hipoacusia neurosensorial. Se realiza este reporte de caso de hipoacusia súbita neurosensorial unilateral acompañada de tinnitus en paciente con antecedente de SARSCov2 Positivo, describiendo los principales hallazgos otológicos y audiológicos en el proceso de evaluación diagnóstico. La afectación por el virus nCoV-19 tiene incidencia en la pérdida auditiva, como se describe en estudios de casos reportados internacionales.

COVID 19 disease is a viral infection caused by the etiologic agent SARS Cov 2; presents a range of clinical features including neurological symptoms, affecting neurons, glial cells, and nerves; forming part of the etiology group of sensorineural hearing loss. This case report of sudden unilateral sensorineural hearing loss accompanied by tinnitus is made in a patient with a history of SARSCov2 Positive, describing the main otological and audiological findings in the diagnostic evaluation process. The involvement by the nCoV-19 virus has an incidence on hearing loss, as described in international reported case studies.

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.

Traumatic Injury of the Petrous Part of the Temporal Bone: Keys for Reporting a Complex Diagnosis. / Patología traumática de peñascos, un diagnóstico complejo. Claves para el informe.

Fractures of the petrous part of the temporal bone are a common lesion of the base of the skull; most of these fractures result from high-energy trauma. In patients with multiple trauma, these injuries can be detected on CT scans of the head and neck, where the direct and indirect signs are usually sufficient to establish the diagnosis. It is important to these fractures because the temporal bone has critical structures and the complexity of this region increases the risk of error unless special care is taken. This article reviews the key anatomical points, the systematization of the imaging findings, and the classifications used for temporal bone fracture. We emphasize the usefulness of identifying and describing the findings in relation to important structures in this region, of looking for unseen fractures suspected through indirect signs, and of identifying anatomical structures that can simulate fractures. We point out that the classical classifications of these fractures are less useful, although they continue to be used for treatment decisions.

Can positron emission tomography support the characterization of immune-mediated inner ear disease? / ¿Puede la tomografía por emisión de positrones ayudar en la caracterización de la enfermedad inmunomediada del oído interno?

INTRODUCTION: To evaluate the utility of 18F-FDG PET/TC as an imaging tool for the characterization of immune-mediated inner ear disease (IMIED), providing measurements of the inner ear region activity as well as detecting possible involvement of other organs. MATERIAL AND METHODS: The study included 28 patients with IMIED and 4 sex-matched and age-matched control subjects with no history of ear disease. Eighteen patients were considered to be suffering from primary IMIED and 10 patients from secondary. PET/CT scans with 18F-FDG were performed to assess systemic involvement as well as inner ear region activity. Interpretation of PET/CT scans was performed independently by 2nuclear medicine physicians blinded to clinical history. In order to assess inter-rater agreement before performing the analysis of the inner ear, different Bland & Altman plots and the intraclass correlation coefficients were estimated. RESULTS: Different metabolically active foci findings were reported in 13 patients. Four patients diagnosed as primary IMIED showed thyroid and aorta activity. Regarding the inner-ear semiquantitative analysis, the inter-rater agreement was not sufficiently high. Comparisons between groups, performed using Mann-Whitney test or Kruskal-Wallis tests, showed no differences. CONCLUSIONS: The study showed 18F-FDG PET/TC could be an important tool in the evaluation of IMIED as it can support the characterization of this entity providing the diagnosis of unknown or underestimated secondary IMIED. Nevertheless, we consider PET is not an adequate tool to approach the inner ear because of the small size and volume of the cochlea which makes the assessment very difficult.

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.

Resultados audiológicos, complicaciones y experiencia de la cirugía de implante coclear en pacientes con malformación congénita del oído interno tipo partición incompleta tipo III / Audiological results, complications and experience of cochlear implant surgery in patients with congenital malformation of the internal ear type incomplete partition type III

Introducción: La hipoacusia neurosensorial congénita afecta a 1 de cada 1000 recién nacidos vivos, el 50% de estos casos tienen causas genéticas y de estas, entre 1-5% está asociado a alteraciones en el cromosoma X, como lo es la partición incompleta tipo III. Objetivo: Describir la técnica quirúrgica empleada, los resultados funcionales y las complicaciones presentadas durante el periodo pre y post operatorio en un grupo de pacientes pediátricos con diagnóstico radiológico de malformación del oído interno tipo partición incompleta tipo III con seguimiento de 06 meses. Diseño: reporte de casos. Materiales y métodos: Análisis descriptivo de pacientes pediátricos con diagnóstico radiológico de malformación del oído interno tipo partición incompleta tipo III, llevados a cirugía de implante coclear entre Enero de 2015 a Enero de 2017. Resultados: Encontramos 03 pacientes de sexo masculino con edad promedio de 3 años al momento de la cirugía (04 oídos). 01 paciente fue llevado a cirugía de implante coclear bilateral secuencial y dos recibieron implante coclear unilateral. El diagnóstico preoperatorio se realizó apoyado en los hallazgos de Tomografía Computarizada de Alta Resolución y evaluación audiológica preimplante coclear. En 02 pacientes hubo estabilización de la pérdida auditiva en el oído contralateral y recibieron rehabilitación auditiva con audífono convencional. 01 paciente presentó empeoramiento del oído contralateral y requirió cirugía de implante coclear secuencial. Conclusiones: En nuestra experiencia los resultados audiológicos de la cirugía de implante coclear en pacientes con partición incompleta tipo III han sido muy satisfactorios.

Introduction: Congenital sensorineural hearing loss affects one per 1000 live births, 50% of these cases have genetic etiology; and of between them, 1-5% has X chromosome-related causes, an example of this type o disease is the cochlear incomplete partition type III. Objective: To describe the surgical technique used, the functional results and the complications presented during the pre and postoperative period in a group of pediatric patients with radiological diagnosis of inner ear malformation type cochlear incomplete partition type III with follow-up of 06 months. Design: Case report. Materials and methods: A descriptive analysis of pediatric patients with radiological diagnosis of inner ear malformation type incomplete partition type III who were undergone to cochlear implant surgery from 2015/01 to 2017/01 was held. Results: We found 03 male patients with an average age of 3 years at the time of surgery. One patient was undergone to sequential bilateral cochlear implant surgery and two to a unilateral cochlear implant. The preoperative diagnosis was based on the findings of High Resolution Computed Tomography and cochlear preimplantation audiological assessment. In 02 patients there was stabilization of the hearing loss in the contralateral ear and they received hearing rehabilitation with a conventional hearing aid. 01 patient presented worsening of the contralateral ear and required sequential cochlear implant surgery. Conclusions: We recommend hearing rehabilitation with hearing aids or cochlear implant surgery according to the level of hearing loss, avoiding stapes surgery, especially in patients with moderate sensorineural hearing loss, because of a greater risk of higher hearing loss. In our experience, we do not recommend Stapes Surgery as an option for auditory rehabilitation.

In vitro study of morphological changes of the cultured otocyst isolated from the chick embryo / Estudio in vitro de los cambios morfológicos del otocisto cultivado aislado del embrión de pollo

The aim of this study was to observe morphological changes of the cultured otocysts isolated from various stages of the chick embryo. Isolated otocysts were dissected from embryonic day, E2.5-4.5 of incubation (HH stage 16-26) according to stages of developing inner ear. Morphology of the chick otocyst exhibited an ovoid shape. The width and height of the otocyst were 0.2 mm and 0.3 mm, respectively. Elongation of a tube-like structure, the endolymphatic duct, was found at the dorsal aspect of the otocyst. The cultured otocyst is lined by the otic epithelium and surrounding periotic mesenchymal cells started to migrate outwards the lateral aspect of such epithelium. Notably, the acoustic-vestibular ganglion (AVG) was observed at the ventrolateral aspect of the otocyst. Appearance of AVG in vitro can be applied for studying chemical-induced ototoxicity and sensorineural hearing loss. It was concluded that the organ-cultured otocyst of the chick embryo could be used as a model to study sensory organ development of avian inner ear.

El objetivo de este estudio fue observar los cambios morfológicos de otocistos cultivados aislados en las diversas etapas del desarrollo del embrión de pollo. Otocistos aislados fueron obtenidos de embriones día, E2.5-4.5 de incubación (HH etapa 16-26) de acuerdo a las etapas de desarrollo del oído interno. El otocisto de pollo presentó una morfología ovoide. El ancho y la altura del otocisto fue de 0,2 mm y 0,3 mm, respectivamente. En la cara dorsal del otocisto se visualizó el alargamiento de una estructura similar a un tubo, el conducto endolinfático. El otocisto cultivado está revestido por epitelio ótico y células mesenquimatosas perióticas que comienzan a migrar hacia el exterior de la cara lateral en búsqueda del epitelio. En particular, el ganglio acústico-vestibular (GAV) fue observado en la parte ventrolateral del otocisto. La aparición de GAV in vitro puede ser aplicado para el estudio de la ototoxicidad inducida por productos químicos y la pérdida de audición neurosensorial. Se concluyó que el otocisto cultivado de embrión de pollo podría ser utilizado como un modelo para estudiar el desarrollo de órganos sensoriales del oído interno aviar.

Inner ear malformations: a practical diagnostic approach. / Malformaciones del oído interno: una aproximación diagnóstica práctica.

Pediatric sensorineural hearing loss is a major cause of disability; although inner ear malformations account for only 20-40% of all cases, recognition and characterization will be vital for the proper management of these patients. In this article relevant anatomy and development of inner ear are surveyed. The role of neuroimaging in pediatric sensorineural hearing loss and cochlear preimplantation study are assessed. The need for a universal system of classification of inner ear malformations with therapeutic and prognostic implications is highlighted. And finally, the radiological findings of each type of malformation are concisely described and depicted. Computed tomography and magnetic resonance imaging play a crucial role in the characterization of inner ear malformations and allow the assessment of the anatomical structures that enable the selection of appropriate treatment and surgical approach.

Malformation of the eighth cranial nerve in children. / Malformaciones del octavo par en niños.

INTRODUCTION AND OBJECTIVES: Prevalence of congenital sensorineural hearing loss (SNHL) is approximately 1.5-6 in every 1,000 newborns. Dysfunction of the auditory nerve (auditory neuropathy) may be involved in up to 1%-10% of cases; hearing losses because of vestibulocochlear nerve (VCN) aplasia are less frequent. The objectives of this study were to describe clinical manifestations, hearing thresholds and aetiology of children with SNHL and VCN aplasia. METHODOLOGY: We present 34 children (mean age 20 months) with auditory nerve malformation and profound HL taken from a sample of 385 children implanted in a 10-year period. We studied demographic characteristics, hearing, genetics, risk factors and associated malformations (Casselman's and Sennaroglu's classifications). Data were processed using a bivariate descriptive statistical analysis (P<.05). RESULTS: Of all the cases, 58.8% were bilateral (IIa/IIa and I/I were the most common). Of the unilateral cases, IIb was the most frequent. Auditory screening showed a sensitivity of 77.4%. A relationship among bilateral cases and systemic pathology was observed. We found a statistically significant difference when comparing hearing loss impairment and patients with different types of aplasia as defined by Casselman's classification. Computed tomography (CT) scan yielded a sensitivity of 46.3% and a specificity of 85.7%. However, magnetic resonance imaging (MRI) was the most sensitive imaging test. CONCLUSIONS: Ten percent of the children in a cochlear implant study had aplasia or hypoplasia of the auditory nerve. The degree of auditory loss was directly related to the different types of aplasia (Casselman's classification) Although CT scan and MRI are complementary, the MRI is the test of choice for detecting auditory nerve malformation.