RESUMO
OBJECTIVE: To perform an otopathologic analysis of temporal bones (TBs) with CHARGE syndrome. STUDY DESIGN: Otopathologic study of human TB specimens. SETTING: Otopathology laboratories. METHODS: From the otopathology laboratories at the University of Minnesota and Massachusetts Eye and Ear Infirmary, we selected TBs from donors with CHARGE syndrome. These TBs were serially sectioned at a thickness of 20 µm, and every 10th section was stained with hematoxylin and eosin. We performed otopathologic analyses of the external ear, middle ear (middle ear cleft, mucosal lining, ossicles, mastoid, and facial nerve), and inner ear (cochlea, vestibule, internal auditory canal, and cochlear and vestibular nerves). The gathered data were statistically analyzed. RESULTS: Our study included 12 TBs from 6 donors. We found a high prevalence of abnormalities affecting the ears. The most frequent findings were stapes malformation (100%), aberrant course of the facial nerve (100%) with narrow facial recess (50%), sclerotic and hypodeveloped mastoids (50%), cochlear (100%) and vestibular (83.3%) hypoplasia with aplasia of the semicircular canals, hypoplasia and aplasia of the cochlear (66.6%) and vestibular (91.6%) nerves, and narrowing of the bony canal of the cochlear nerve (66.6%). The number of spiral ganglion and Scarpa's ganglion neurons were decreased in all specimens (versus normative data). CONCLUSIONS: In our study, CHARGE syndrome was associated with multiple TB abnormalities that may severely affect audiovestibular function and rehabilitation.
Assuntos
Síndrome CHARGE/complicações , Orelha Interna/anormalidades , Orelha Média/anormalidades , Osso Temporal/anormalidades , Anormalidades Múltiplas , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Michigan , MinnesotaRESUMO
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.
Assuntos
Orelha Interna/anormalidades , Doenças do Nervo Facial/diagnóstico , Doenças do Nervo Vestibulococlear/diagnóstico , Criança , Orelha Interna/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , MasculinoRESUMO
RESUMEN La estenosis del conducto auditivo interno con aplasia/hipoplasia del nervio cocleovestibular es una patología muy infrecuente. Suele ser unilateral y puede acompañarse de aplasia/hipoplasia del nervio facial y otras malformaciones del oído interno. Se presentan aquí dos casos clínicos de pacientes pediátricos con estenosis del conducto auditivo interno unilateral con compromiso del séptimo y octavo par craneal ipsilateral. Se describen las historias y evaluaciones clínicas, hallazgos audiovestibulares, hallazgos imagenológicos, tratamientos indicados y sus resultados.
ABSTRACT Congenital internal auditory canal stenosis associated with aplasia/hypoplasia of the cochleovestibular nerve is a very infrequent pathology. It is usually unilateral and may be accompanied by aplasia/hypoplasia of the facial nerve and other malformations of the inner ear. We hereby present two clinical cases of pediatric patients with congenital internal auditory canal stenosis, with involvement of the seventh and eighth ipsilateral cranial nerve. The medical histories and clinical evaluations, audiovestibular findings, imaging findings, treatments and their results are described.
Assuntos
Humanos , Masculino , Feminino , Criança , Perda Auditiva Neurossensorial/etiologia , Orelha Interna/anormalidades , Audiometria , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Constrição Patológica/diagnóstico por imagem , Paralisia Facial/etiologia , Perda Auditiva Neurossensorial/diagnóstico por imagemRESUMO
HYPOTHESIS: The presence of bony inner ear malformations may associate with a number of anatomical abnormalities affecting the middle ear structures. Those malformations may create pitfalls and complications for cochlear implantation. BACKGROUND: Inner ear malformations associate with varying degrees of hearing loss, and frequently require cochlear implantation for hearing rehabilitation. Therefore, the abnormalities affecting the middle- and inner-ear structures may increase the risk of surgical complications. METHODS: We examined 38 human temporal bones from donors with bony inner ear malformations. Using light microscopy, we analyzed the presence of abnormalities in the structures of the middle- and inner-ear. RESULTS: Our collection comprises of 38 specimens with inner-ear malformations (cochlear aplasia, nâ=â3; cochlear hypoplasia, nâ=â30; incomplete partition, nâ=â3; isolated vestibular malformation, nâ=â2). The anatomy of the middle ear was abnormal in most temporal bones with cochlear aplasia, cochlear hypoplasia, and incomplete partition type I (40%-100%). Some of those abnormalities (hypoplastic or obliterated mastoid, 55.2%; aplastic or obliterated round window, 71.0%; aberrant course of the facial nerve, 36.8%) may hinder the access to the round window using the conventional facial recess approach for cochlear implantation. The cochlear nerve and associated bony structures (internal auditory canal and bony canal for cochlear nerve) were normal in 71.0% of all temporal bones with inner ear malformations. CONCLUSION: Each different type of malformation may create specific surgical challenges to surgeons. Comprehensive preoperative imaging is fundamental toward the surgical success of cochlear implants in patients with malformations. Alternatives to circumvent those middle- and inner-ear abnormalities and potential complications are further discussed.
Assuntos
Implante Coclear/métodos , Orelha Interna/anormalidades , Orelha Média/anormalidades , Implantes Cocleares/efeitos adversos , Orelha Interna/cirurgia , Orelha Média/cirurgia , Feminino , Humanos , Masculino , Tomografia Computadorizada por Raios XRESUMO
Objetivo Caracterizar o sistema auditivo periférico de indivíduos com síndrome de Down, por meio da audiometria convencional e de altas frequências. Métodos Estudo do tipo transversal e observacional. Participaram 15 indivíduos com síndrome de Down, de ambos os gêneros, entre 7 e 15 anos de idade. Foram realizados os seguintes procedimentos: Meatoscopia, Timpanometria com pesquisa do reflexo acústico ipsilateral e contralateral, Audiometria Tonal, Audiometria Vocal e Audiometria de Altas Frequências. Resultados Houve predomínio de perda auditiva condutiva de grau leve, em uma ou ambas as orelhas. As médias dos limiares auditivos para a audiometria convencional ficaram abaixo de 20 dBNA e, para a audiometria de altas frequências, ficaram entre 20 e 40 dBNA. O coeficiente de correlação de Pearson revelou correlação moderada positiva, entre os limiares de 9 a 14 kHz e a idade. Conclusão De forma geral, não foram observadas diferenças significativas, quando comparadas as orelhas direita e esquerda de indivíduos com síndrome de Down, na audiometria tonal, imitanciometria e logoaudiometria. A maioria das crianças apresentou alteração de orelha média e perda auditiva condutiva. A audiometria de altas frequências sugere o início de prejuízo da função coclear, que pode estar associado às otites médias frequentes e/ou à degeneração coclear precoce. .
Purpose This study sought to characterize the peripheral auditory system of individuals with Down syndrome (DS) using conventional and high-frequency audiometry. Methods We performed a cross-sectional and observational study. Fifteen individuals with DS, who were of both genders and between 7 and 15 years of age, participated in this study. The following procedures were performed: otoscopy, tympanometry with ipsilateral and contralateral acoustic reflex, pure-tone audiometry, vocal audiometry and high-frequency audiometry. Results There was a predominance of mild conductive hearing loss in one or both ears. The mean hearing thresholds for conventional audiometry were below 20 dB HL and between 20 and 40 dB HL for high-frequency audiometry. The Pearson correlation coefficient indicated a moderate positive correlation between the 9-14 kHz thresholds and age. Conclusion Overall, no significant differences were observed when comparing the right and left ears of individuals with DS, in regards to pure-tone audiometry, immittance testing and speech audiometry. Most children showed middle ear abnormalities and conductive hearing loss. Moreover, high-frequency audiometry suggested the onset of impaired cochlear function, which may be associated with frequent otitis media episodes and/or early cochlear degeneration. .
Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Síndrome de Down/complicações , Orelha Interna/lesões , Perda Auditiva/diagnóstico , Limiar Auditivo , Cóclea/anormalidades , Orelha Interna/anormalidades , Tuba Auditiva/anormalidades , Perda Auditiva Condutiva , Perda Auditiva de Alta Frequência , Perda Auditiva Neurossensorial , Hipotonia Muscular , Estudo Observacional , Otite Média com Derrame , Presbiacusia , Estudos ProspectivosRESUMO
Se describe la displasia de Mondini asociada a pérdida auditiva y meningitis bacteriana recurrente. La malformación de Mondini representa el 30 por ciento de las anomalías congénitas del oído interno, puede ser unilateral o bilateral, y su principal característica consiste en el desarrollo coclear incompleto, causante de grados variables de hipoacusia neurosensorial. Se considera que la ocurrencia de esta malformación se produce por disrupción del desarrollo embrionario durante la séptima semana de gestación, en la cual se detiene el desarrollo coclear. Se presenta el caso de una niña de 12 años, atendida en los Servicios de Pediatría y Otorrinolaringología del Hospital Pediátrico Universitario William Soler, por presentar 3 infecciones meningoencefálicas, en las cuales se aisló Streptococcus pneumoniae serotipo 19F, y se constató hipoacusia neurosensorial severa en el oído izquierdo. La tomografía axial computarizada de alta resolución del oído (cortes axiales y coronales), evidenció la malformación coclear y vestibular, con presencia de tejido en el oído medio que se comunicaba directamente con el oído interno del lado izquierdo a nivel de la ventana oval. Se destaca la importancia de la sospecha clínica de displasia de Mondini, y el impacto científico de la tomografía computarizada del hueso temporal, para el diagnóstico precoz de fístula congénita en el oído interno asociada a meningitis bacteriana recurrente
Mondini dysplasia associated to hearing loss and recurrent bacterial meningitis was described in this paper. Mondini malformation accounts for 30 percent of congenital anomalies in the inner ear, either unilateral or bilateral, and its main characteristic is the incomplete cochlear development causing various grades of neurosensory hypoacusis. It is considered that the occurrence of this malformation results from the disruption of the embryonal development on the 7th week of gestation when the cochlear development ceases. This is the case of 12 years-old girl who was attended to at the pediatric and otorhinolaryngology service of William Soler" university pediatric hospital because she presented with three meningoencephalic infections from which Streptococcus pneumonia serotype 19F was isolated. It was also confirmed that she suffered severe neurosensory hypoacusis in her left ear. High-resolution computerized axial tomography of the ear (axial and coronal planes) evinced the cochlear and vestibular malformation, with tissue of the middle ear communicating directly with the inner ear of the left size at the oval window. This paper underlined the importance of the clinical suspicion of Mondini disease, and the scientific impact of the computerized axial tomography of the temporal bone in order to early diagnose the congenital fistula in the inner ear associated to recurrent bacterial meningitis
Assuntos
Humanos , Feminino , Criança , Meningite Pneumocócica/complicações , Orelha Interna/anormalidades , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva NeurossensorialRESUMO
En candidatos a implante coclear con malformaciones del oído interno donde se encuentra un nervio coclear anormal, los estudios tradicionales y las imágenes muchas veces no pueden dar respuesta definitiva acerca de la funcionalidad y presencia del nervio coclear. Para esto ayudarían los estudios de electrofisiología. Se presentan tres casos clínicos de pacientes con malformaciones del oído interno que fueron evaluados con ePEAT para ayudar a determinar su candidatura a implante coclear. Los estudios electrofisiológicos no reemplazan a los estudios tradicionales de evaluación auditiva ni a los estudios por imágenes, sino que los complementan. Los casos presentados, demuestran que en casos de malformaciones de oído interno o CAI muy estrecho, en que se cuestiona seriamente la existencia de un nervio coclear funcional, y en casos de neuropatía auditiva, se hace necesario evaluar la función de la cóclea separadamente de la del nervio auditivo y la función del tronco. Para esto se utilizamos los ePEAT. Los ePEAT entregan información valiosísima ya que nos permite conocer las reales capacidades de los pacientes para transmitir un estímulo auditivo hacia el sistema nervioso central, definiendo mejor las expectativas con el uso implante, asistiéndonos en nuestra toma de decisiones.
In cochlear implant candidates with inner ear malformations, where there is an abnormal cochlear nerve, traditional studies and images cannot often provide definitive answers about the functionality and presence of the cochlear nerve. In these cases, electrophysiology studies can be used. We present 3 cases of patients with inner ear malformations who were evaluated with ePEAT to determine their candidacy for a cochlear implant. Electrophysiological studies do not replace traditional hearing screening studies or imaging studies, but complement them. The cases presented in this study demonstrate that in patients with inner ear malformations or very narrow internal auditory canal, where we question the existence of a functional cochlear nerve, and in cases of auditory neuropathy, it is necessary to evaluate the cochlear function separately from the auditory nerve and from the brainstem. In these cases we use ePEAT. ePEAT give us valuable information about the real abilities of patients to transmit an auditory stimulus to the central nervous system, which help us to define expectations with cochlear implant use, assisting us in our decision-making.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Seleção de Pacientes , Implante Coclear/métodos , Estimulação Elétrica/métodos , Orelha Interna/anormalidades , Orelha Interna/fisiopatologia , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Cóclea/fisiopatologia , Eletrofisiologia , Perda Auditiva Neurossensorial/etiologia , Orelha Interna/diagnóstico por imagemRESUMO
UNLABELLED: Oculo-auriculo-vertebral spectrum (OAVS) is a rare condition characterized by the involvement of the first branchial arches. PURPOSE: To investigate the ear abnormalities of a sample of patients with OAVS. MATERIALS AND METHODS: The sample consisted of 12 patients with OAVS seen at the Clinical Genetics Unit, UFCSPA/CHSCPA. The study included only patients who underwent mastoid computed tomography and with normal karyotype. We performed a review of its clinical features, giving emphasis to the ear findings. RESULTS: Nine patients were male, the ages ranged from 1 day to 17 years. Ear abnormalities were observed in all patients and involved the external (n = 12), middle (n = 10) and inner ear (n = 3). Microtia was the most frequent finding (n = 12). The most common abnormalities of the middle ear were: opacification (n = 2), displacement (n = 2) and malformation of the ossicular chain. Agenesis of the internal auditory canal (n = 2) was the most frequent alteration of the inner ear. CONCLUSIONS: Ear abnormalities are variable in patients with OAVS and often there is no correlation between findings in the external, middle and inner ear. The evaluation of these structures is important in the management of individuals with OAVS.
Assuntos
Pavilhão Auricular/anormalidades , Orelha Interna/anormalidades , Orelha Média/anormalidades , Síndrome de Goldenhar/complicações , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios XRESUMO
Oculo-auriculo-vertebral spectrum (OAVS) is a rare condition characterized by the involvement of the first branchial arches. PURPOSE: To investigate the ear abnormalities of a sample of patients with OAVS. MATERIALS AND METHODS: The sample consisted of 12 patients with OAVS seen at the Clinical Genetics Unit, UFCSPA/CHSCPA. The study included only patients who underwent mastoid computed tomography and with normal karyotype. We performed a review of its clinical features, giving emphasis to the ear findings. RESULTS: Nine patients were male, the ages ranged from 1 day to 17 years. Ear abnormalities were observed in all patients and involved the external (n=12), middle (n=10) and inner ear (n=3). Microtia was the most frequent finding (n=12). The most common abnormalities of the middle ear were: opacification (n=2), displacement (n=2) and malformation of the ossicular chain. Agenesis of the internal auditory canal (n=2) was the most frequent alteration of the inner ear. CONCLUSIONS: Ear abnormalities are variable in patients with OAVS and often there is no correlation between findings in the external, middle and inner ear. The evaluation of these structures is important in the management of individuals with OAVS.
O espectro óculo-aurículo-vertebral (EOAV) é uma condição rara caracterizada pelo envolvimento dos primeiros arcos branquiais. OBJETIVOS: Verificar as alterações auriculares de uma amostra de pacientes com EOAV. MATERIAL E MÉTODOS: A amostra foi constituída de 12 pacientes com EOAV atendidos no Serviço de Genética Clínica da UFCSPA/CHSCPA. Foram incluídos no estudo apenas pacientes submetidos à tomografia computadorizada de mastoide e com cariótipo normal. Realizou-se uma revisão dos seus achados clínicos, dando-se ênfase aos achados auriculares. RESULTADOS: Nove pacientes eram do sexo masculino, idades variaram de 1 dia a 17 anos. Anormalidades auriculares foram observadas em todos os pacientes e envolveram a orelha externa (n=12), média (n=8) e interna (n=3). A microtia foi o achado mais frequente (n=12). As alterações mais comuns da orelha média foram: opacificação da mesma (n=2), e o deslocamento (n=2) e a malformação da cadeia ossicular (n=2). A agenesia de conduto auditivo interno (n=2) foi a anormalidade mais frequente da orelha interna. CONCLUSÕES: Alterações auriculares são variáveis em pacientes com EOAV, não existindo muitas vezes uma correlação entre os achados da orelha externa, média e interna. A avaliação destas estruturas é importante dentro do manejo de indivíduos com EOAV.
Assuntos
Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pavilhão Auricular/anormalidades , Orelha Interna/anormalidades , Orelha Média/anormalidades , Síndrome de Goldenhar/complicações , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios XRESUMO
Objetivo: Descrever as características audiométricas e malformações das orelhas média e interna em dois pacientes portadores de síndrome velocardiofacial. Método: Avaliação audiométrica, tomografia computadorizada dos ossos temporais e análise de DNA, para marcadores múltiplos da região 22q11, foram realizadas em dois pacientes com sinais clínicos da síndrome velocardiofacial. Resultados: Perdas auditivas condutivas relacionadas com otite média crônica e malformações das orelhas média e interna foram encontradas, estas últimas com a utilização de reformatações baseadas em aquisições multislice da tomografia computadorizada dos ossos temporais. Conclusão: Consideramos de grande importância realizar uma completa avaliação e monitoramento da evolução da função auditiva, bem como do surgimento de sintomas relacionados à função vestibular em pacientes com a síndrome velocardiofacial. Do ponto de vista radiológico chamamos a atenção para o uso de técnicas de alta qualidade para o estudo tomográfico dos ossos temporais. Palavras chave: orelha, perda auditiva, velocardiofacial.
Objective: To describe audiometric characteristics and middle and inner ear malformations in two patients with velocardiofacial syndrome. Method: Audiometric evaluation, computerized tomography of the temporal bones and analysis of DNA for multiple markers of 22q11 region were performed in two patients with clinical signs of velocardiofacial syndrome. Results: Conductive hearing loss related to chronic otites media and middle and inner ear malformations were found, the latter with the use of reformations based on multislice acquisitions on of the computerized tomography of the temporal bones. Conclusion: We consider it to be highly important to carry out a thorough evaluation and monitoring of the hearing evolution, as well as the occurrence of symptoms related to the vestibular function in patients with velocardiofacial syndrome. From the radiological point of view, attention should be given to the use of high quality techniques for the tomographic study of temporal bones.
Assuntos
Humanos , Masculino , Criança , Audiometria , Síndrome de DiGeorge , Orelha Interna/anormalidades , Orelha Média/anormalidadesRESUMO
INTRODUCTION: Episodes of recurrent bacterial meningitis can occur in patients due to either congenital or acquired disorders. Congenital deformity of the bony labyrinth can be linked to a fistulous tract communicating it with the intracranial subarachnoid space. Mondini deformity is a frequent malformation in congenitally deaf patients. CASE REPORT: We report the case of an adolescent with a history of being unable to hear in one ear who, from the age of 10 years, began to suffer repeated bacterial meningoencephalitis with microbiological recovery of Streptococcus pneumoniae on three occasions. The type of germ recovered in the cerebrospinal fluid (CSF) and the history of congenital deafness that was detected when the patient was 3 years old were the diagnostic clues to the possible anomaly of the inner ear with a CSF fistula. The clinically proven CSF rhinorrhea contributed to the diagnosis of an ear anomaly with a fistula. Computerised axial tomography and magnetic resonance studies of the petrous portion of the temporal bone revealed the malformation that was later found and closed during the surgical intervention on the affected ear. The clinical absence of rhinorrhea, a year's progression without new infections after operating on the patient and post-surgery imaging studies were all proof that the fistula had closed. CONCLUSIONS: Mondini dysplasia with CSF fistula must be included as a possible diagnosis when faced with a patient with recurrent bacterial meningoencephalitis. Imaging studies, especially magnetic resonance, enable the clinician to check the diagnosis and the CSF fistula can be closed with ear surgery.
Assuntos
Surdez/etiologia , Orelha Interna/anormalidades , Doenças do Labirinto , Meningites Bacterianas , Adolescente , Rinorreia de Líquido Cefalorraquidiano , Criança , Pré-Escolar , Surdez/patologia , Orelha Interna/cirurgia , Feminino , Fístula/diagnóstico , Fístula/patologia , Fístula/cirurgia , Humanos , Doenças do Labirinto/líquido cefalorraquidiano , Doenças do Labirinto/diagnóstico , Doenças do Labirinto/patologia , Meningites Bacterianas/líquido cefalorraquidiano , Meningites Bacterianas/complicações , Meningites Bacterianas/diagnóstico , Meningites Bacterianas/patologia , RecidivaRESUMO
Las anomalías congénitas del hueso temporal son bastante variables, tanto desde el punto de vista anatómico como en lo que se refiere al tipo y severidad de la sordera resultante. En este caso se presentan dos pacientes pediátricos de 2 y 3 años, que presentaban meningitis a repetición de causa no determinada. La TC permitió descubrir la patología causante de las afecciones en estos pacientes y fue decisiva en el diagnóstico del origen de las meningitis a repetición que presentaban. En los pacientes pediátricos que presentaban meningitis a repetición es importante considerar en primera instancia a los procesos fistulosos como los causantes de esta patología. Dentro de los huesos que pueden presentar estas fístulas se encuentran el peñasco, el cual no debe ser dejado de lado en el examen clínico de estos pacientes. Las fístulas de peñasco vienen asociadas con malformaciones de oído interno y el método de imagen por excelencia para su evaluación sigue siendo la tomografía computada
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Cóclea/anormalidades , Orelha Interna/anormalidades , Meningites Bacterianas/etiologia , Meningite/etiologia , Osso Petroso , Síndrome de Klippel-Feil/complicações , Meningites Bacterianas/diagnóstico , Meningite/diagnóstico , Osso Petroso/anormalidades , Recidiva , Osso Temporal/anormalidades , Tomografia Computadorizada por Raios X/métodosRESUMO
Las anomalías congénitas del hueso temporal son bastante variables, tanto desde el punto de vista anatómico como en lo que se refiere al tipo y severidad de la sordera resultante. En este caso se presentan dos pacientes pediátricos de 2 y 3 años, que presentaban meningitis a repetición de causa no determinada. La TC permitió descubrir la patología causante de las afecciones en estos pacientes y fue decisiva en el diagnóstico del origen de las meningitis a repetición que presentaban. En los pacientes pediátricos que presentaban meningitis a repetición es importante considerar en primera instancia a los procesos fistulosos como los causantes de esta patología. Dentro de los huesos que pueden presentar estas fístulas se encuentran el peñasco, el cual no debe ser dejado de lado en el examen clínico de estos pacientes. Las fístulas de peñasco vienen asociadas con malformaciones de oído interno y el método de imagen por excelencia para su evaluación sigue siendo la tomografía computada (AU)
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Meningite/etiologia , Meningites Bacterianas/etiologia , Orelha Interna/anormalidades , Cóclea/anormalidades , Síndrome de Klippel-Feil/complicações , Osso Petroso/diagnóstico por imagem , Meningite/diagnóstico , Meningites Bacterianas/diagnóstico , Osso Temporal/anormalidades , Recidiva , Tomografia Computadorizada por Raios X/métodos , Osso Petroso/anormalidadesRESUMO
Os pacientes com hipertensäo têm queixa de surdez progressiva, muitas vezes acompanhada de zumbidos. A doença pode, também, manifestar-se por episódios repetidos de meningite, surdez súbita devido à ocorrência de fístula perilinfática ou pela saída profusa de líquido cefalorraquidiano, gusher, durante cirurgia, estapedectomia ou estapedotomia4,11,12. A paciente, R.V.C., 36 anos, foi submetida a estapedectomia para tratamento de otosclerose e, durante o ato cirúrgico, ocorreu otoliquorréia. A hipertensäo perilinfática foi controlada e foi possível a colocaçäo da prótese. O resultado cirúrgico foi satisfatório, houve ganho auditivo no ouvido direito operado (cerca de 30 dB). Foi feito estudo tomográfico que näo esclareceu a causa da hipertensäo perilinfática. Defeitos no modíolo, no aqueduto coclear ou alargamento do conduto auditivo interno devem ser investigados 2,14
Assuntos
Humanos , Feminino , Adulto , Pressão do Líquido Cefalorraquidiano/fisiologia , Surdez/genética , Orelha Interna/anormalidades , Ligação Genética , Complicações Intraoperatórias/diagnóstico , Otosclerose/cirurgia , Perilinfa , Cirurgia do Estribo , Audiometria , Cefalexina/uso terapêutico , Surdez/fisiopatologia , Surdez/cirurgia , Dexametasona/uso terapêutico , Doenças Cocleares/fisiopatologia , Perda Auditiva Neurossensorial/reabilitação , Complicações Intraoperatórias/fisiopatologia , Manitol/uso terapêutico , Tomografia Computadorizada por Raios XRESUMO
Se reporta el caso de una paciente femenina de 71 años con malformación congénita de oído interno de tipo Michel asociada a colesteatoma del oído medio. Presentaba anacusia derecha y otorrea purulenta fétida desde la infancia acompañada de dolor en mastoides, por lo que fue intervenida quirúrgicamente encontrando cavidad auditiva única con aplasia de oído interno, trayecto anómalo del nervio facial, dehiscencia de golfo de la yugular y de la carótida interna. Se limpió la cavidad y se protegieron las estructuras con aponeurosis temporal. El postoperatorio cursó satisfactoriamente con epitelización completa, sin otorrea ni recidiva colesteatomatosa
Assuntos
Humanos , Feminino , Idoso , Colesteatoma/congênito , Colesteatoma/diagnóstico , Orelha Interna/anormalidades , Orelha Interna/cirurgiaRESUMO
Perilymphatic hypertension is usually related to gushers which occur in the operating room when the stapes footplate is punctured or removed. These patients present defects in the modiolus, or enlarged cochlear aqueducts, which allow easy access of spinal fluid to the perilymphatic space. This syndrome was detected in children and appears to present consistent audiological patterns. They present a mixed hearing loss and in spite of the presence of an air-bone gap the stapedial reflexes are present. These children benefit from the use of hearing aids but show very little insertion gain in the low frequencies when tested with real-ear measurements.
Assuntos
Pressão do Líquido Cefalorraquidiano/fisiologia , Orelha Interna/anormalidades , Perda Auditiva Neurossensorial/fisiopatologia , Perilinfa/fisiologia , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Auxiliares de Audição , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/reabilitação , Humanos , MasculinoRESUMO
Six temporal bones obtained from four individuals with Alagille's syndrome, aged 4 months and 3, 6, and 7 years, were studied histopathologically. The external auditory canals and tympanic membranes were normal. Although the stapes, the interossicular joints, and the subarcuate fossae were slightly underdeveloped in the majority of cases, the other structures in the middle ear were almost normal. However, severe anomalies were observed in structures in the inner ear. In all cases, both the bony and membranous structures of the posterior semicircular canal were partially or totally absent, and, in three ears, those of the anterior semicircular canal were also partially absent; the lateral semicircular canal, however, was normal in all cases. The cochlea was observed to be shortened in only one case.
Assuntos
Anormalidades Múltiplas/patologia , Orelha Interna/anormalidades , Orelha Média/anormalidades , Osso Temporal/patologia , Criança , Pré-Escolar , Orelha Interna/patologia , Orelha Média/patologia , Humanos , Lactente , SíndromeRESUMO
For this study, 100 human temporal bones from 73 individuals, aged 31 gestational weeks to 39 years, each with anomalies of the middle and/or inner ear, were studied to identify the features, locations, and frequencies with which congenital anomalies occurred in these structures. The temporal bones had been removed at autopsy, fixed, dehydrated, embedded in celloidin, sectioned horizontally or vertically at 20 microns, stained with hematoxylin and eosin, and studied under a light microscope. The anomaly in the middle ear most often found was wide dehiscence of the facial canal; hypoplastic cochlea was most frequently observed anomaly in the inner ear. The implications of these findings for development of the ear during fetal life are discussed.