Agujero Timpánico bilateral. Análisis de caso desde la literatura / Bilateral tympanic hole. Case analysis from the literature

La pared del conducto auditivo externo (CAE) parte de la formación del hueso timpánico; integrándose posteriormente a la porción petrosa del hueso temporal. El agujero timpánico o foramen de Huschke corresponde a un defecto en la osificación en donde existe fusión incompleta de porciones anteriores y posteriores del anillo timpánico dejando una abertura que comunica el CAE hacia anterior. Su presencia es normal hasta los 5 años de edad, tiempo en que se debiese obliterar. Su incidencia es baja (3-24%), pero la persistencia en adulto, conlleva sintomatología inespecífica caracterizada por otalgia, dolor en articulación temporomandibular (ATM), tinnitus, hipoacusia o manifestaciones complejas como descarga salival en CAE durante la masticación. Clínicamente puede complicar procedimientos de infiltración y artroscopias de ATM. Rara vez ocasiona, en pacientes mayores de 50 años, herniación de la cabeza del cóndilo mandibular. Su diagnóstico puede ser clínico por medio de otoscopia, donde se observa protuberancia de tejido en pared anterior del CAE, que aumenta de tamaño con la boca cerrada. También puede ser imagenológico con una tomografía computarizada. El tratamiento incluye desde medidas conservadoras para manejo del dolor e inflamación, hasta quirúrgicas con la implantación de injertos, placas o prótesis para cerrar la estructura o para reemplazar el cóndilo mandibular. El presente estudio pretende aportar incidencia dentro del área de estudio. Se analiza por observación directa, cráneo seco, completo, masculino, edad entre 12 a 15 años (según morfología del cóndilo mandibular y erupción dental). Se observa agujero de Huschke, bilateral, ambos permeables de diámetro 4 mm en ambos casos, determinados con regla milimetrada. La relevancia del defecto se asocia a la práctica clínica de otorrinolaringólogos, cirujanos maxilofaciales y odontólogos, ya sea como diagnóstico diferencial asociado a los síntomas inespecíficos, como para procedimientos más invasivos en la zona tales como infiltraciones o artroscopias de ATM

The wall of the external auditory canal (EAC) starts from the formation of the tympanic bone; later it is integrated to the petrous portion of the temporal bone. The tympanic foramen or foramen of Huschke corresponds to a defect in ossification where there is incomplete fusion of the anterior and posterior portions of the tympanic ring leaving an opening that communicates the EAC to its anterior aspect. Its presence is normal until 5 years of age, when it should be absolutely obliterated. Its incidence is low (3-24%), but its persistence in adults leads to non specific symptoms characterized by otalgia, pain in the temporomandibular joint (TMJ), tinnitus, hearing loss, or complex manifestations such as salivary discharge in the CAE during mastication. Clinically, it may complicate TMJ infiltration and arthroscopy procedures. It rarely causes herniation of the mandibular condyle head in patients older than 50 years. Its diagnosis can be clinical by means of otoscopy, where tissue protrusion is observed in the anterior wall of the CAE, which increases in size when the mouth is closed. It can also be imaging with computed tomography. Treatment includes from conservative measures to treat pain and inflammation, to surgical measures with the implantation of grafts, plates or prosthesis to close the structure or to replace the mandibular condyle. The present study aims to provide incidence within the study area. It is analyzed by direct observation, dry skull, complete, male, age between 12 to 15 years (according to mandibular condyle morphology and dental eruption). Huschke's foramen was observed, bilateral, both permeable, diameter 4mm in both cases, determined with a millimeter ruler. The relevance of the defect is associated with the clinical practice of otolaryngologists, maxillofacial surgeons and dentists, either as a differential diagnosis associated with nonspecific symptoms, or for more invasive procedures in the area such as infiltrations or TMJ arthroscopies.

Microtia e atresia congênita do canal auditivo em cão: relato de caso / Microtia and congenital external auditory canal atresia in dog: case report

Descreve-se o caso de um cão, Pinscher, fêmea de 11 meses de idade, com alteração anatômica da orelha do lado direito. Após avaliação, foi realizado o diagnóstico clínico e o radiográfico de microtia com atresia do canal auditivo do lado direito. Como o animal não apresentava sinais de alterações no sistema vestibular ou otite, optou-se pelo acompanhamento clínico do caso. A microtia, caracterizada pela hipoplasia parcial ou completa da pina, assim como outras anomalias do conduto auditivo, é raramente descrita em medicina veterinária. De acordo com a revisão de literatura realizada, este é o primeiro relato ocorrido no Brasil de um cão com microtia e atresia congênita do canal auditivo sem a associação de síndrome vestibular.(AU)

This paper describes an 11-month-old female Pinscher dog, with anatomical changes of the ear on the right side. After physical exam, clinical and radiographic diagnosis of microtia with ear canal atresia on the right side was made. As the animal did not show any signs of vestibular system changes or ear infections, the veterinarians opted for monitoring the patient. Microtia, characterized by the partial or complete hypoplasia of pina, as well as other abnormalities of ear canal are rarely described in veterinary medicine. As far as the author´s knowledge by literature review carried out, this is the first report of a dog with microtia and congenital ear canal atresia not associated with vestibular syndrome in Brazil.(AU)

Vibrant Soundbridge and Bone Conduction Hearing Aid in Patients with Bilateral Malformation of External Ear

Introduction Hearing loss is the most common clinical finding in patients with malformation of the external ear canal. Among the possibilities of treatment, there is the adaptation of hearing aids by bone conduction and the adaptation of implantable hearing aids. Objective To assess speech perception with the use of Vibrant Soundbridge (VBS - MED-EL, Innsbruck, Austria) associated with additional amplification in patients with bilateral craniofacial malformation. Method We evaluated 11 patients with bilateral malformation over 12 years with mixed hearing loss or bilateral conductive. They were using the Softband (Oticon Medical, Sweden) and bone conduction hearing aid in the ear opposite the one with the VSB.We performed the evaluation of speech perception using the Hearing in Noise Test. Results Participants were eight men and three women with a mean of 19.5 years. The signal / noise ratio presented significant results in patients fitted with VSB and bone conduction hearing aid. Conclusion The results of speech perception were significantly better with use of VBS combined with bone conduction hearing aids.

Colesteatoma congénito del conducto auditivo externo. Caso clínico / Congenital cholesteatoma of the external auditory meatus. Case report / Colesteatoma congênito do canal auditivo externo. Caso

Se expone el caso poco frecuente de colesteatoma congénito del conducto auditivo externo en un lactante de 6 meses de edad que se presentó inicialmente como absceso.

We review the unusual presentation of a congenital cholesteatoma of the external auditory meatus in a 6 months old infant, initially presented as an abscess.

O caso raro de colesteatoma congênito do canal auditivo externo em crianças de 6 meses de idade, que apresentou inicialmente como abscesso está exposto.

Revisión sobre el manejo de atresia de conducto auditivo externo mediante el uso de dispositivos auditivos implantables osteointegrados: a propósito de un caso exitoso con un implante Bonebridge® / Review of the management of external auditory canal atresia using osseo integrated implantable hearing devices: report of a successful case with Bonebridge® implant

La atresia de conducto auditivo externo tiene una incidencia de 1 por 1.000 ó 2.000 nacidos vivos, predominantemente unilateral. Su manejo actual es controversial, en especial la cirugía de reconstrucción. En el último tiempo se han desarrollado dispositivos osteointegrados, que han revolucionado el tratamiento y cuestionado la indicación de cirugía. El liderazgo por un tiempo importante lo ha tenido el dispositivo tipo BAHA, pero que ha tenido problemas en el largo plazo, principalmente en relación con el tornillo del implante. En respuesta a estos problemas, se ha diseñado un nuevo implante osteointegrado de nombre Bonebridge®, con beneficios frente a su antecesor. Como en nuestro medio se tiene poca experiencia en este dispositivo se presenta una revisión a propósito de un caso clínico exitoso. El caso corresponde a un paciente de 18 años con hipoacusia de conducción máxima bilateral congénita, con diagnóstico de atresia de conducto auditivo externo bilateral, con inadecuada ganancia auditiva del cintillo óseo tradicional. Tanto la revisión bibliográfica como el caso clínico nos permiten aseverar que Bonebridge® corresponde a tratamiento adecuado en el manejo de la atresia del conducto auditivo externo, con ciertos beneficios sobre otros dispositivos y la cirugía.

The external auditory canal atresia has an incidence of 1 per 1000 or 2000 live births predominantly unilateral. Its management is controversial, especially with the decision of reconstructive surgery. Lately, have developed osseo integrated bone conduction devices, which have come to revolutionize the treatment and questioning the indication for surgery. The leadership for a long time its has had it the BAHA device type, but has several problems in long-term, mainly in relation to the screw used in the implant. In response to this problems, was designed a new osseo integrated implant named Bonebridge®, showing benefits over its predecessor. As in our setting there is little experience with this device, we present a review about a successful clinical case. The case is a patient of 18 years with a maximal bilateral hearing loss caused by a congenital bilateral atresia of the external auditory canal, and inadequate audition gain with traditional bone headband. As much the bibliographical overhaul as the clinical case allows to assert that Bone-bridge® corresponds to an alternative of treatment in the atresia of the external auditory canal and with certain benefits by on the other devices, as the surgery.

Validación del protocolo para la medición del efecto de oclusión durante el proceso de selección de audífonos / Validation of protocol for measuring the effect of occlusion during the selection process hearing

Se presenta en este proyecto, los resultados de la validación del protocolo para la medición del efecto de oclusión durante el proceso de adaptación de audífonos diseñado en el año 2008. Se realizaron ajustes a los nueve apartados planteados en la versión original del protocolo y del formato de registro. Por medio de la técnica de validación por jueces expertos se revisaron el formato de registro y los procedimientos incluidos en el protocolo ya ajustado, obteniendo un CVR entre 0.80 y 1. Adicionalmente se correlacionaron los datos obtenidos de la aplicación del protocolo y del reporte audiológico previo con el fin de establecer el nivel de convergencia de estas dos mediciones, siendo el reporte audiológico el criterio definido para determinar la validez del instrumento. A partir de una muestra de 30 sujetos seleccionados por medio de muestreo por conveniencia se aplicó el coeficiente phi (dada la escala de medida de ambas mediciones) obteniendo un valor de 0.40. Se relacionaron igualmente los antecedentes personales, los antecedentes otológicos y los hallazgos otoscópicos con los resultados de la aplicación del protocolo, encontrando que existe una fuerte relación entre la presencia de oclusión y la presencia de rinitis. Se recomienda para futuras investigaciones aplicar el protocolo en una población más grande con el fin de seguir hallando evidencia al respecto de la validez de las inferencias hechas por el instrumento.

We present in this project, the results of the protocol validation for occlusion effect measurement during the process of adaptation of hearing aids designed in the year 2008. Initially, adjustments were made to the nine sections established in the original version of the protocol and record format. By means of the validation technique, expert judges revised the record format and the procedures included in the protocol, obtaining a CVR between 0.80 and 1 The collected data in the protocol and the previous audiological report were correlated with the purpose to establish the convergence level of these two measurements, the audiological report is considered the define criterion. From a sample of 30 subjects selected by means of convenience sampling, it was applied the coefficient phi (given a measurement scale for both measurements) of 0.40. The personal background, otologic background and otologic findings were correlated with the protocol application results, finding a strong relation between the presence of occlusion and the presence of rhinitis. It is recommended for future investigations to apply the protocol in a bigger population in order to keep on finding evidence regarding the validity of the inference done by the instrument.

Uso do BAHA na reabilitação auditiva de pacientes com atresia de meato acústico externo / BAHA usage in auditory rehabilitation of pacients with external auditory canal stenosis

A prótese implantável de condução óssea (BAHA) tem sido utilizado e estudado na Europa e nos EUA já tendo sido implantado em mais de 15000 pacientes em todo o mundo, e recentemente começou a ser utilizado no Brasil...

Bone Anchored Hearing Aid is an implantable bone conduction hearing aid of the temporal bone. This device has been used and studied in Europe and in the USA having already been implanted more than 15000 patient around the word and has just beginning in Brazil...

Diffuse cystic bronchiectasis associated with left microtia and external auditory canal atresia: a very rare coincidence

To present a case in which diffuse cystic bronchiectasis was associated with left microtia/external auditory canal atresia. A 10-year-old girl suffering from cough, fever, dyspnea and sputum for 6 months was transferred to our clinic due to the diagnosis of bronchopneumonia. She had recurrent episodes of bronchopneumonia. On examination, left microtia and left external auditory canal atresia were detected. Thorax CT revealed diffuse cystic bronchiectasis on the left lung. Radiological examination showed atresia of the left external auditory canal. We could not find any etiopathological reason causing bronchiectasis. The patient underwent left pneumonectomy and postoperative histopathology was reported as bronchiectasis. This report shows a unique case in which an association of diffuse cystic bronchiectasis and left microtia/external auditory canal atresia was observed. Hence in newborns with microtia and/or external auditory canal atresia, the probability of development of bronchiectasis should be borne in mind and such patients should be followed up more carefully regarding this rare association

Townes-Brocks syndrome. A neonate with extended spectrum and thrombocytopenia

Townes-Brocks syndrome is a rare malformation syndrome characterized by thumb, auricular, renal, and anal anomalies. Both familial and isolated cases with clinical heterogeneity were reported in the medical literature. We present a sporadic case with typical clinical features and extended spectrum, that has previously not been described. Left sided facial palsy, external auditory canal atresia, club hand with radius aplasia, bilateral clubfoot, and sacral skin tag were the additional findings. Isolated patent ductus arteriosus was the associated congenital heart defect. Thrombocytopenia was another documented hematological abnormality

Preservation of facial nerve in surgery of first branchial cleft anomaly: five cases of duplicated external ear canal

We report five cases of duplicated external ear canal with especial attention to its histology and surgical care of facial nerve for their complete resection. These five patients were selected from 50 cases of first bronchial cleft anomalies including atretic external ear canals, preauricular fistula, cyst and sinuses. The characteristic histopathology and clinical features such as external opening around the auricle and upper neck above the level of the hyoid bone were not observed in other patients. There were no other abnormalities in the real external ear canal [EEC] and auricle. Three of them were connected to the natural EEC with an opening, hence producing ear discharges. Histologic appearances of well formed canal included a circumferential epithelial lined up and canal consisting of stratified squamous epithelium with skin adnexes. A remarkable cartilaginous skeleton [type 2 of first branchial cleft anomaly according to Work's classifications] was also observed. All of them were treated surgically, with preservation of the facial nerve and excising the superficial parotid gland along with the ductal opening and the tract

Tomografía computada del oído con malformaciones congénitas: evaluación prequirúrgica / Computed tomography of the congenital anomalies of the ear: preoperatory evaluation

Se exploraron con tomografía computada de alta resolución 60 oídos de 44 pacientes con malformaciones congénitas de los oídos externo y medio, valorando las alteraciones anatómicas según un criterio pre-establecido. Se establecen algunos parámetros para dar mayor objetividad a este análisis. Se encontrtó alteración bilateral en 26 pacientes y unilateral en 28. De los 60 oídos, 39 (65 por ciento) tenían atresia del conducto auditivo externo y 21 (35 por ciento) estenosis. El oído medio era de tamaño adecuado en 43 (72 por ciento) oídos inadecuado en 17 (28 por ciento). El trayecto del nervio facial se encontró normal en 39 (65 por ciento) y alterado en 21 (35 por ciento) oídos. La proporción de anomalías en el curso del nervio facial fue mucho mayor en los oídos con atresia, 19 de 39 (49 por ciento) que presentaban estenosis del conducto. Se propone una escala de puntuación para seleccionar a los candidatos a la cirugía, según el pronóstico funcional y riesgo