Internal auditory canal hypoplasia associated with bilateral vestibulocochlear nerve aplasia and deviant facial nerve course: A case report and MRI findings.

The evaluation of internal auditory canals and cochlea has gained significant importance due to the increasing number of cochlear implantations worldwide. This region's anatomical study is essential for cochlear implant surgery using magnetic resonance imaging as the method of choice. We report a case of a 6-year-old male patient diagnosed with a rare bilateral malformation of the internal auditory canals associated with an aberrant course of the facial nerve and vestibulocochlear nerve aplasia. This report raises the importance of identifying this rare malformation for appropriate management and reinforces awareness of possible complications.

Influence of socioeconomic factors on the perception of cochlear-vestibular symptoms and adherence to the treatment of congenital hypothyroidism / Influência dos fatores socioeconômicos na percepção de sintomas cócleo-vestibulares e na adesão ao tratamento do hipotireoidismo congênito

Abstract Objectives: to verify if there is an association between socioeconomic factors and adherence to treatment in congenital hypothyroidism and to verify if there is an association between socioeconomic factors and vestibulocochlear symptoms noticed by parents/caregivers of children diagnosed with congenital hypothyroidism. Methods: a cross-sectional, exploratory and descriptive study, with a convenience sample. The sample consisted of 108 children with clinical and laboratory diagnosis of congenital hypothyroidism, of both sexes, aged ≥ 5 years. The researchers applied a structured questionnaire to parents/caregivers, consisting of closed and objective questions about the presence or absence of tinnitus, hearing loss and dizziness/vertigo in children with congenital hypothyroidism. Results: There was no association between socioeconomic factors and adherence to treatment or perception of cochlear-vestibular symptoms. Conclusions: socioeconomic factors did not influence treatment adherence or perceived cochlear-vestibular symptoms by caregivers of children with congenital hypothyroidism.

Resumo Objetivos: verificar se existe associação entre fatores socioeconômicos e adesão ao tratamento no hipotireoidismo congênito e verificar se existe associação entre fatores socioeconômicos e sintomas vestibulococleares percebidos pelos pais / cuidadores de crianças diagnosticadas com hipotireoidismo congênito. Métodos: estudo transversal, exploratório e descritivo, com amostra de conveniência. A casuística foi composta por 108 crianças com diagnóstico clínico e laboratorial de hipotireoidismo congênito, de ambos os sexos com idade ≥ 5 anos. Foi aplicado um questionário estruturado para os pais/cuidadores, formado por questões fechadas e objetivas sobre a presença ou ausência de zumbido, hipoacusia e tontura/vertigem nas crianças com hipotireoidismo congênito. Resultados: não houve associação entre fatores socioeconômicos e adesão ao tratamento ou percepção dos sintomas cócleo-vestibulares. Conclusões: os fatores socioeconômicos não influenciaram na adesão ao tratamento nem na percepção de sintomas cócleo-vestibulares pelos cuidadores de crianças com hipotireoidismo congênito.

Children with unilateral cochlear nerve canal stenosis have bilateral cochleovestibular anomalies.

OBJECTIVES/HYPOTHESIS: To investigate the cochleovestibular apparatus bilaterally in children with isolated unilateral bony cochlear nerve canal (bCNC) stenosis. STUDY DESIGN: Retrospective review. METHODS: Imaging studies of children with unilateral bCNC stenosis (<1.0 mm) on computed tomography imaging (N = 36) were compared with controls imaged due to trauma without temporal bone injury (N = 32). Twenty-six measurements were obtained in each ear, assessing the bony internal auditory canal (IAC), cochlea, and vestibular end-organs, and were analyzed using one-way analysis of variance for intersubject comparisons and paired t tests for intrasubject comparisons with a Bonferroni adjustment for multiple comparisons (P = .0006). RESULTS: Patients with bCNC stenosis had a smaller IAC (P < .000) and cochlea (P < .000) on the stenotic side as compared with controls. Although the vestibular end-organ was also smaller in bCNC ears, this difference was not significant. The contralateral ear also had a smaller bCNC (P < .000) and cochlea (P < .000) as compared with controls, although to a lesser degree than the stenotic side. CONCLUSIONS: Children with unilateral bCNC stenosis have abnormal biometry of both the cochlea and the vestibular end-organ in the affected and the normal contralateral ear as compared with controls. LEVEL OF EVIDENCE: 3b Laryngoscope, 129:2403-2408, 2019.

Paroxismia vestibular: reporte de un caso / Vestibular paroxysmia: case report

Presentamos el caso de un paciente joven quien presenta 4 a 5 crisis diarias de vértigo espontáneo de segundos de duración, todos o casi todos los días desde hace 9 meses. Estas crisis no tienen gatillo posicional, y hay completa ausencia de sintomatologia entre crisis. Como discutimos en el artículo, este cuadro coíncide con los recientemente publicados criterios para una paroxismia vestibular, entidad supuestamente secundaria a la compresión neurovascular del nervio vestibular. El paciente respondió de forma inmediata y completa a carbamazepina a dosis bajas, el tratamiento de elección en la paroxismia vestibular.

We present the case of a young patient, with a 9-month long history of 4 to 5 daily spells of spontaneous vertigo, each lasting only seconds. There is no positional trigger, and there is a complete lack of symptoms between attacks. As is discussed in the article, this matches the recently published criteria for Vestibular Paroxysmia, an entity allegedly secondary to neurovascular compression of the vestibular nerve. The patient responded immediately and completely to carbamazepine at low dosage, the preferred treatment for vestibular paroxysmia.

Deficiencia del nervio coclear: logros obtenidos y expectativas con el implante coclear / Coclear nerve deficiency: achievements and expectations with the coclear implant / Deficiência de nervos coculares: realizações e expectativas como implante cocular

Introducción: la deficiencia del nervio coclear se define como un nervio coclear hipoplásico o aplásico, presente en más del 18% de los niños con hipoacusias neurosensoriales profundas y cuya indicación de implante coclear sigue generando controversias. Objetivo: Analizar el protocolo de estudio en pacientes con sospecha de deficiencia del nervio coclear y exponer los resultados clínico-audiológicos pos-implante coclear en nuestro servicio. Material y método: Estudio retrospectivo de historias clínicas en el período 2011-2017, analizando los estudios solicitados dentro de la evaluación preimplante coclear y el estudio extendido en pacientes con sospecha de deficiencia del nervio coclear. Resultados: Dentro de la población, un caso correspondió a un paciente con hipoacusia neurosensorial profunda bilateral con sospecha de deficiencia del nervio coclear (Birman: GRADO I y Casselman: TIPO I/IIa bilateral). El potencial evocado auditivo de tronco encefálico eléctrico permitió determinar el oído a implantar, mostrando mejor configuración de ondas para el oído derecho. Actualmente, presenta una óptima adaptación al implante (categoría de performance auditivo: 2-categoría de Moog-Geers: 3), con una puntuación de 8/40 en el cuestionario IT-MAIS. Conclusión: El protocolo de estudio prequirúrgico extendido es aplicable a pacientes con sospecha de deficiencia del nervio coclear. Estudios funcionales aportan datos de utilidad para determinar cuál de estos pacientes podrían beneficiarse tras la colocación del mismo. El asesoramiento familiar sobre las limitaciones y los objetivos reales es fundamental.

Introduction: cochlear nerve deficiency is defined as a hypoplastic or aplastic cochlear nerve present in more than 18% of children with profound sensorineural hearing loss. Cochlear implant indication continues to generate controversy. Objective: Analyze the study protocol in patients with suspected cochlear nerve deficiency and expose the clinical-audiological results after cochlear implant in our service. Material and method: Retrospective study of medical records in the period 2011-2017. Analyzing the studies requested within the cochlear pre-implant evaluation and the extended study in patients with suspected cochlear nerve deficiency. Results: Within the population, one case corresponded to a patient with bilateral profound sensorineural hearing loss with suspicion of Cochlear nerve deficiency (Birman GRADE I and Casselman TYPE I/IIa bilateral). The auditory evoked potentials by electrical stimulation allowed to determine the ear to be implanted, showing better configuration of waves for the right ear. Currently, it presents an optimal adaptation to the implant (Auditory per formance category: 2- Category of Moog-Geers: 3), with a score of 8/40 in the IT-MAIS questionnaire. Conclusion: The extended preoperative study protocol is appropriate for patients with suspected cochlear nerve deficiency. Functional studies provide useful data to determine which patients could benefit. Family counseling about real limitations and goals is critical.

Introdução: a deficiência do nervo coclear é definida como um nervo coclear hipoplásico ou aplástico presente em mais de 18% das crianças com perda auditiva neurossensorial profunda onde a indicação do implante coclear continua a gerar polêmica. Objetivo: Analisar o protocolo do estudo em pacientes com suspeita de deficiência do nervo coclear e apresentar os resultados clínico-audiológicos após o implante coclear em nosso serviço. Material e método: Estudo retrospectivo dos registros clínicos no período de 2011-2017, analisando os estudos solicitados na avaliação pré-implante coclear e o estudo prolongado em pacientes com suspeita de deficiência de nervo coclear. Resultados: Dentro da população, um caso correspondeu a um paciente com perda auditiva neurossensorial profunda bilateral com suspeita de deficiência do nervo coclear (Birman: GRADO I e Casselman: TIPO bilateral I/IIa). O potenciais evocados auditivos elétrico permitiu determinar a orelha a ser implantada, mostrando melhor configuração de ondas para a orelha direita. Atualmente, apresenta uma ótima adaptação ao implante (categoria de desempenho auditivo: 2- categoria de Moog-Geers: 3), com uma pontuação de 8/40 no questionário IT-MAIS. Conclusão: O protocolo de estudo pré-operatório estendido é aplicável a pacientes com suspeita de deficiência do nervo coclear. Estudos funcionais fornecem dados úteis para determinar quais pacientes poderiam se beneficiar do implante coclear. O aconselhamento familiar sobre limitações e metas reais é crítico.

Evaluation of central vestibular syndrome in dogs using brainstem auditory evoked responses recorded with surface electrodes / Avaliação da síndrome vestibular central em cães usando respostas evocadas auditivas do tronco cerebral gravadas com eletrodos de superfície

The present study aimed to analyse the wave morphology, amplitude, latency, and intervals of the brainstem auditory evoked responses (BAERs) in dogs with central vestibular syndrome (CVS) recorded with surface electrodes. Ten dogs with CVS were examined by mono- and binaural stimulation, using the Neuropack electrodiagnostic system, with stimulus intensities of 90 dBSPL. BAERs examinations revealed morphological changes of waves I, II, III, and V and decreased amplitudes of all waves in 7/10 dogs. P values obtained were = 0.014 for wave I amplitude, 0.031 for II, and III and 0.032 for V. Comparing the latencies of waves I, II, III, and V generated by right and left monoaural stimulation in dogs with CVS, we did not observe significant differences (P > 0.05). No statistical differences were observed for BAERs latencies of the waves recorded after binaural and monaural stimulation (left or right). As far as we know, this is the first study of BAERs using surface electrodes, obtained from dogs with CVS.(AU)

Este estudo destina-se à análise da morfologia, da amplitude, da latência e do intervalo das ondas das respostas evocadas auditivas no tronco cerebral (BAERs) em cães com síndrome vestibular central (CVS) registrados com eletrodos de superfície. Dez cães com CVS foram examinados por estimulação mono e binaural usando um sistema de eletrodiagnóstico Neuropack, com intensidade do estímulo de 90 dBSPL. Os exames BAERs relevaram alterações morfológicas das ondas I, II, III e V, bem como baixas amplitudes para todas as ondas no caso dos 7/10 cães. Os valores de P obtidos foram = 0.014 para ampitude da onda I, 0.031 para a II e 0.032 para a V. Compararam-se as latências das ondas I, II, III e V geradas pelo estímulo monoaural direito e esquerdo em cães com CVS e não foram constatadas diferenças significativas (P > 0.05). Igualmente não foram observadas diferenças estatísticas no caso das latências BAERs no que diz respeito às ondas gravadas depois de estímulos binaural e monoaural (esquerdo ou direito). Pelo que é de conhecimento dos autores da presente pesquisa, este é o primeiro estudo sobre BAERs usando eletrodos de superfície obtidos em cães com CVS.(AU)

Evaluation of central vestibular syndrome in dogs using brainstem auditory evoked responses recorded with surface electrodes / Avaliação da síndrome vestibular central em cães usando respostas evocadas auditivas do tronco cerebral gravadas com eletrodos de superfície

The present study aimed to analyse the wave morphology, amplitude, latency, and intervals of the brainstem auditory evoked responses (BAERs) in dogs with central vestibular syndrome (CVS) recorded with surface electrodes. Ten dogs with CVS were examined by mono- and binaural stimulation, using the Neuropack electrodiagnostic system, with stimulus intensities of 90 dBSPL. BAERs examinations revealed morphological changes of waves I, II, III, and V and decreased amplitudes of all waves in 7/10 dogs. P values obtained were = 0.014 for wave I amplitude, 0.031 for II, and III and 0.032 for V. Comparing the latencies of waves I, II, III, and V generated by right and left monoaural stimulation in dogs with CVS, we did not observe significant differences (P > 0.05). No statistical differences were observed for BAERs latencies of the waves recorded after binaural and monaural stimulation (left or right). As far as we know, this is the first study of BAERs using surface electrodes, obtained from dogs with CVS.(AU)

Este estudo destina-se à análise da morfologia, da amplitude, da latência e do intervalo das ondas das respostas evocadas auditivas no tronco cerebral (BAERs) em cães com síndrome vestibular central (CVS) registrados com eletrodos de superfície. Dez cães com CVS foram examinados por estimulação mono e binaural usando um sistema de eletrodiagnóstico Neuropack, com intensidade do estímulo de 90 dBSPL. Os exames BAERs relevaram alterações morfológicas das ondas I, II, III e V, bem como baixas amplitudes para todas as ondas no caso dos 7/10 cães. Os valores de P obtidos foram = 0.014 para ampitude da onda I, 0.031 para a II e 0.032 para a V. Compararam-se as latências das ondas I, II, III e V geradas pelo estímulo monoaural direito e esquerdo em cães com CVS e não foram constatadas diferenças significativas (P > 0.05). Igualmente não foram observadas diferenças estatísticas no caso das latências BAERs no que diz respeito às ondas gravadas depois de estímulos binaural e monoaural (esquerdo ou direito). Pelo que é de conhecimento dos autores da presente pesquisa, este é o primeiro estudo sobre BAERs usando eletrodos de superfície obtidos em cães com CVS.(AU)

El vértigo como primer síntoma de un infarto cerebeloso / The dizziness as the first symptom of a cellebelar stroke

Introducción: El vértigo es un motivo frecuente de consulta en los servicio de urgencias, por lo tanto es primordial para iniciar un tratamiento adecuado diferenciar un origen periférico de uno central. La historia clínica y la exploración neurológica completa son fundamentales para llegar a un diagnóstico, ya que las pruebas de imagen tienen una baja sensibilidad en la fase aguda y no siempre están disponibles en los servicios de urgencias. Objetivo: Presentar el caso clínico de una paciente de 74 años con vértigo agudo que sugiere inicialmente un origen periférico, pero que se debe a un infarto cerebeloso. Diseño: Reporte de caso. Materiales y métodos: Se presenta el caso de una paciente de 74 años que acude a urgencias por presentar de manera súbita vértigo objetivo con los movimientos cefálicos, intenso cortejo vegetativo, sin síntomas otológicos, con factores de riesgo cardiovascular importantes; al examen físico nistagmo horizontal derecho, no agotable, dismetría, disdiadococinesia izquierda e inestabilidad marcada que le impide la bipedestación. En tomografía craneal, se observa hipodensidad en región superior del hemisferio cerebeloso izquierdo sugestiva de lesión isquémica aguda en territorio vascular de la arteria cerebelosa superior. Resultados: Se diagnosticó mediante resonancia magnética nuclear, infarto isquémico en fase subaguda en el territorio de arteria cerebelosa superior izquierda. Conclusiones: Es importante diferenciar el vértigo aislado por causa vascular de trastornos más benignos que se relacionan con el oído interno, ya que la estrategia terapéutica y el pronóstico difieren considerablemente

Introduction: Vertigo is a frequent reason for consultation in the emergency department, therefore it is essential to differentiate between peripheral and central origin to initiate a suitable treatment. The clinical history and neurological examination are essential to reach a diagnosis, because imaging tests have low sensitivity in the acute phase and are not always available in the Emergency Department. Objective: To present the clinical case of a 74 year old patient with acute vertigo which initially suggested a peripheral origin, but it was due to an acute cerebellar infarction. Design: Case report. Materials and methods: We present the case of a patient of 74 years who came to the emergency department because of a sudden objective vertigo with was presented with head movements, severe vegetative symptoms without otologic symptoms. Important cardiovascular risk factors was identified; physical examination revealed an inexhaustible, horizontal, right nistagmus; dysmetria, left dysdiadochokinesia and marked instability. In tomography, a suggestive image of acute ischemic injury in vascular territory of the superior cerebellar artery hemisphere was seen. Results: A subacute ischemic stroke in the territory of the left superior cerebellar artery was diagnosed by nuclear magnetic resonance. Conclusions: It is important to differentiate isolated vertigo caused by more benign inner ear disease from those caused by vascular disorders, since therapeutic strategy and prognosis differ considerably.

Cochleovestibular nerve involvement in multifocal fibrosclerosis.

OBJECTIVES: To report a case of multifocal fibrosclerosis with a nine-year follow up, and to discuss this disease's radiological appearance and management. The disease is a rare systemic disorder of unknown cause characterised by fibrous proliferation involving multiple anatomical sites. CASE REPORT: A 50-year-old woman presented with histological findings characterised by similar inflammatory processes involving the meninges, pituitary gland, peritoneum, retroperitoneum and orbits, prompting a search for a common pathophysiology. A diagnosis of multifocal fibrosclerosis was postulated. Symptom improvement was noted after treatment with prednisone and azathioprine. CONCLUSION: This is the first documented case of involvement of the cochleovestibular nerve in a patient with multifocal fibrosclerosis. The rare association between fibrotic diseases and masses showing various clinical patterns should be kept in mind by otolaryngologists, and imaging performed to investigate for multifocal fibrosclerosis. However, diagnosis can only be confirmed with tissue biopsy and histopathological examination.

Auditory neuropathy/Auditory dyssynchrony in children with Cochlear Implants.

UNLABELLED: The electrical stimulation generated by the Cochlear Implant (CI) may improve the neural synchrony and hence contribute to the development of auditory skills in patients with Auditory Neuropathy/Auditory Dyssynchrony (AN/AD). AIM: Prospective cohort cross-sectional study to evaluate the auditory performance and the characteristics of the electrically evoked compound action potential (ECAP) in 18 children with AN/AD and cochlear implants. MATERIAL AND METHODS: The auditory perception was evaluated by sound field thresholds and speech perception tests. To evaluate ECAP's characteristics, the threshold and amplitude of neural response were evaluated at 80 Hz and 35 Hz. RESULTS: No significant statistical difference was found concerning the development of auditory skills. The ECAP's characteristics differences at 80 and 35 Hz stimulation rate were also not statistically significant. CONCLUSIONS: The CI was seen as an efficient resource to develop auditory skills in 94% of the AN/AD patients studied. The auditory perception benefits and the possibility to measure ECAP showed that the electrical stimulation could compensate for the neural dyssynchrony caused by the AN/AD. However, a unique clinical procedure cannot be proposed at this point. Therefore, a careful and complete evaluation of each AN/AD patient before recommending a Cochlear Implant is advised. Clinical Trials: NCT01023932.

Auditory neuropathy/auditory dyssynchrony in children with cochlear implants / Neuropatia auditiva/dessincronia auditiva em crianças usuárias de implante coclear

The electrical stimulation generated by the Cochlear Implant (CI) may improve the neural synchrony and hence contribute to the development of auditory skills in patients with Auditory Neuropathy/Auditory Dyssynchrony (AN/AD). AIM: Prospective cohort cross-sectional study to evaluate the auditory performance and the characteristics of the electrically evoked compound action potential (ECAP) in 18 children with AN/AD and cochlear implants. MATERIAL AND METHODS: The auditory perception was evaluated by sound field thresholds and speech perception tests. To evaluate ECAP's characteristics, the threshold and amplitude of neural response were evaluated at 80Hz and 35Hz. RESULTS: No significant statistical difference was found concerning the development of auditory skills. The ECAP's characteristics differences at 80 and 35Hz stimulation rate were also not statistically significant. CONCLUSIONS: The CI was seen as an efficient resource to develop auditory skills in 94 percent of the AN/AD patients studied. The auditory perception benefits and the possibility to measure ECAP showed that the electrical stimulation could compensate for the neural dyssynchrony caused by the AN/AD. However, a unique clinical procedure cannot be proposed at this point. Therefore, a careful and complete evaluation of each AN/AD patient before recommending a Cochlear Implant is advised. Clinical Trials: NCT01023932.

A estimulação elétrica gerada pelo Implante Coclear (IC) pode ser capaz de melhorar a sincronia neural e contribuir para o desenvolvimento das habilidades auditivas de sujeitos portadores de Neuropatia Auditiva/Dessincronia Auditiva (NA/DA). OBJETIVO: Estudo de coorte prospectivo transversal para avaliar o desempenho auditivo e as características do Potencial de Ação Composto Eletricamente Evocado no Nervo Auditivo (ECAP) em 18 crianças portadoras de NA/DA e usuárias de IC. MATERIAL E MÉTODOS: Percepção auditiva e características do ECAP foram avaliadas respectivamente, por meio da determinação dos limiares tonais e testes de percepção de fala e pelas medidas de limiar e amplitude da resposta neural para as frequências de estimulação de 35 e 80Hz. Não foram encontradas diferenças estatisticamente significativas no desenvolvimento das habilidades auditivas entre os sujeitos avaliados. RESULTADOS: Não foram observadas modificações estatisticamente significativas nas características de limiar e amplitude do ECAP para as duas frequências de estimulação testadas. CONCLUSÕES: O IC caracterizou-se como um efetivo recurso para o desenvolvimento das habilidades auditivas em 94 por cento dos sujeitos, sendo a estimulação elétrica capaz de compensar a alteração da sincronia neural decorrente da NA/DA. A avaliação criteriosa de cada caso de maneira específica deve ser realizada no momento anterior à indicação cirúrgica. Clinical Trials: NCT01023932.

Alterações no sistema vestibulococlear decorrentes da exposição ao agrotóxico: revisão de literatura / Vestibulocochlear system alterations caused by exposure to pesticides: a literature review

Os agrotóxicos são amplamente utilizados no meio rural e sua larga utilização, desprovida de medidas de biossegurança apropriadas, vem provocando impactos potenciais tanto para a degradação ambiental quanto para o organismo humano, aumentando a incidência de intoxicações ocupacionais. O objetivo do presente estudo foi revisar e discutir a respeito da exposição aos agrotóxicos e sua influência na função e estrutura do sistema auditivo. Foram revisados periódicos nacionais e internacionais relevantes indexados no PubMed, Medline, Bireme e LILACS, assim como capítulos de livros e dissertações publicados no período entre 1987 e 2009. As pesquisas sugerem que a exposição, principalmente crônica, aos agrotóxicos induz a alterações funcionais no sistema vestibulococlear, manifestando ainda uma série de sinais e sintomas. Apesar da escassez de dados sobre a influência do organofosforado nas características morfológicas do aparelho auditivo, os estudos indicam que a exposição de caráter agudo aos agrotóxicos pode também ser responsável por alterações no sistema auditivo, caracterizadas por lesões estruturais na cóclea, principalmente nas células ciliadas externas.

Pesticides are widely used in agriculture, and the widespread application of these agents, without the appropriate use of bio-security measures, has been causing potential impacts for environmental degradation, as well as for the human organism, increasing the incidence of occupational poisoning. The aim of the present study was to review the available literature and to carry out a discussion regarding pesticides exposure and its harmful effects on the auditory system's function and structure. Relevant national and international journals indexed in PubMed, Medline, Bireme and LILACS were reviewed, as well as book chapters and dissertations published between 1987 and 2009. The available data suggest that chronic exposure to pesticides lead to functional alterations in the vestibulocochlear system, as well as a series of signs and symptoms. In spite of the scarce data describing organophosphorus influence on morphological characteristics of the auditory system, the literature suggest that acute exposure to pesticides can also be responsible for auditory system alterations, characterized by structural lesions in the cochlea, mainly in outer hair cells.

Clinical significance of a hearing and imaging workup in a child with cochlear nerve aplasia and a normal internal auditory canal.

Modern magnetic resonance imaging has significantly improved the diagnosis of cochlear nerve deficiencies. A careful assessment of all clinical, imaging, and auditory data is of utmost importance in such cases in order to properly establish the site of the abnormality. We report the case of a 3-year-old girl with unilateral cochlear nerve aplasia, normal middle and inner ear anatomy, and an absence of otoacoustic emissions, all of which erroneously suggested cochlear damage. We also briefly review the embryogenesis of the inner ear and auditory pathway. A patient with similar findings in the setting of a bilateral hearing loss and insufficient imaging would be at risk of inappropriate cochlear implantation.

Facial paralysis and vestibular syndrome in feedlot cattle in Argentina / Paralisia facial e síndrome vestibular de bovinos em confinamento

This paper reports 6 outbreaks of neurological disease associated with paralysis of the facial and vestibulocochlear nerves caused by intracranial space occupying lesions in feedlot cattle. The clinical signs observed were characterized by head tilt, uni or bilateral drooping and paralysis of the ears, eyelid ptosis, keratoconjunctivitis, and different degrees of ataxia. Morbidity and mortality rates ranged from 1.1 to 50 percent and 0 to 1 percent, respectively. Gross lesions observed included yellow, thickened leptomeninges, and marked enlargement of the roots of cranial nerves VII (facial) and VIII (vestibulocochlear). Histopathologically, there was severe, chronic, granulomatous meningitis and, in one case, chronic, granulomatous neuritis of the VII and VIII cranial nerves. Attempts to identify bacterial, viral, or parasitic agents were unsuccessful. Based on the morphologic lesions, the clinical condition was diagnosed as facial paralysis and vestibular syndrome associated with space occupying lesions in the meninges and the cranial nerves VII and VIII. Feedlot is a practice of growing diffusion in our country and this is a first report of outbreaks of facial paralysis and vestibular disease associated with space occupying lesions in Argentina.

Descrevem-se 6 surtos de uma doença neurológica com paralisia dos nervos facial e vestibulo-coclear causada por lesões intracraniais que ocupam espaço em bovinos em confinamento. Os sinais clínicos foram desvio da cabeça, queda e paralisia das orelhas, ptose palpebral, ceratoconjuntivite e diferentes graus de ataxia. As taxas de morbidade e mortalidade foram de 1.1 por cento-50 por cento e de 0-1 por cento, respectivamente. As lesões macroscópicas incluíram engrossamento das meninges, que se apresentavam amareladas, e marcado engrossamento das raízes dos nervos cranianos VII (facial) e VIII (vestíbulo-coclear). Histologicamente observaram-se meningite crônica granulomatosa e, em um caso, neurite granulomatosa crônica do VII e VIII pares cranianos. Cultivos para bactérias ou vírus resultaram negativos. De acordo com as lesões observadas o quadro clínico foi diagnosticado como paralisia facial e síndrome vestibular associadas a lesões que ocupam espaço nas meninges e nervos cranianos VII e VIII. O confinamento é uma prática em expansão na Argentina e este é o primeiro relato, neste país, de surtos de paralisia facial e síndrome vestibular associados com lesões que ocupam espaço.

Facial paralysis and vestibular syndrome in feedlot cattle in Argentina / Paralisia facial e síndrome vestibular de bovinos em confinamento

This paper reports 6 outbreaks of neurological disease associated with paralysis of the facial and vestibulocochlear nerves caused by intracranial space occupying lesions in feedlot cattle. The clinical signs observed were characterized by head tilt, uni or bilateral drooping and paralysis of the ears, eyelid ptosis, keratoconjunctivitis, and different degrees of ataxia. Morbidity and mortality rates ranged from 1.1 to 50 percent and 0 to 1 percent, respectively. Gross lesions observed included yellow, thickened leptomeninges, and marked enlargement of the roots of cranial nerves VII (facial) and VIII (vestibulocochlear). Histopathologically, there was severe, chronic, granulomatous meningitis and, in one case, chronic, granulomatous neuritis of the VII and VIII cranial nerves. Attempts to identify bacterial, viral, or parasitic agents were unsuccessful. Based on the morphologic lesions, the clinical condition was diagnosed as facial paralysis and vestibular syndrome associated with space occupying lesions in the meninges and the cranial nerves VII and VIII. Feedlot is a practice of growing diffusion in our country and this is a first report of outbreaks of facial paralysis and vestibular disease associated with space occupying lesions in Argentina.(AU)

Descrevem-se 6 surtos de uma doença neurológica com paralisia dos nervos facial e vestibulo-coclear causada por lesões intracraniais que ocupam espaço em bovinos em confinamento. Os sinais clínicos foram desvio da cabeça, queda e paralisia das orelhas, ptose palpebral, ceratoconjuntivite e diferentes graus de ataxia. As taxas de morbidade e mortalidade foram de 1.1 por cento-50 por cento e de 0-1 por cento, respectivamente. As lesões macroscópicas incluíram engrossamento das meninges, que se apresentavam amareladas, e marcado engrossamento das raízes dos nervos cranianos VII (facial) e VIII (vestíbulo-coclear). Histologicamente observaram-se meningite crônica granulomatosa e, em um caso, neurite granulomatosa crônica do VII e VIII pares cranianos. Cultivos para bactérias ou vírus resultaram negativos. De acordo com as lesões observadas o quadro clínico foi diagnosticado como paralisia facial e síndrome vestibular associadas a lesões que ocupam espaço nas meninges e nervos cranianos VII e VIII. O confinamento é uma prática em expansão na Argentina e este é o primeiro relato, neste país, de surtos de paralisia facial e síndrome vestibular associados com lesões que ocupam espaço.(AU)

[Intralabyrinthine schwannoma of the vestibulocochlear nerve]. / Wewnatrzblednikowe nerwiaki nerwu przedsionkowo-slimakowego.

AIM: Presenting and discussing the patient with intravestibular schwannoma of the vestibulocochlear nerve, the course of disease, audiologic and radiologic examination and treatment possibilities. METHODS: Analysis of clinical presentation and treatment of the patient with intravestibular schwannoma and review of available literature on intralabyrinthine schwannomas. RESULTS: A 34-year-old male was diagnosed with a tumor localized in the left vestibule and semicircular canals without accompanying vertigo at any time of the disease but with tinnitus and total deafness of the left ear. Preoperative diagnosis was established on the basis of MRI with contrast. Localization of the tumor laterally to the fundus of internal auditory canal was essential for the diagnosis. Surgical treatment was chosen via translabyrinthine approach. Postoperative course was uneventful. DISCUSSION: Intralabyrinthine schwannoma are rare pathology. Their occurrence implies that schwannomas can originate at any segment of vestibulocochlear nerve. Most commonly intralabyrinthine schwannomas are observed at intracochlear or intravestibular localization. Present trials of these tumors classification were presented. The symptoms emerging in the course of disease are hearing loss, tinnitus, and vertigo, thus there were described patients with intralabyrinthine schwannomas diagnosed and treated as Meniere disease. Treatment depends on the intensity of symptoms and tumor expansion, while approach is dependant on its localization. CONCLUSION: Elaborate radiologic examination including MRI with contrast is essential in case of atypical course of disease with hearing loss, tinnitus and vertigo, or with early diagnosis of Meniere disease.

An uncommon presentation of an VIII nerve tumor.

Vestibular Shwannomas are responsible for 80-90% of the cerebelar-pontine angle tumors and their incidence is of 0.8 to 20.5% of all tumors. Unilateral and progressive hearing loss is the most frequent and premature symptom, and tinnitus is the second most common complaint. Only 5% of the patients have normal audiograms. In this case the patient complained of ipsilateral facial numbness and weak blink, posterior pinna hypoesthesia (Hitzelberger +), tear reduction and positive Romberg test. He also had mouth twisting but no other involvement of other cranial nerves. Hearing acuity was normal.