Achados audiológicos na Síndrome de Vogt-Koyanagi-Harada / Audiological findings in the Vogt-Koyanagi-Harada Syndrome

RESUMO A síndrome de Vogt-Koyanagi-Harada (SVKH) é rara, multissistêmica e autoimune. Atinge principalmente os olhos, provocando uma panuveíte crônica bilateral, porém traz afecções em outras áreas e tecidos que são ricos em melanócitos, como olhos, orelha interna, meninges e a pele. Sua origem ainda não é totalmente conhecida. Geralmente, a SVKH atinge indivíduos de origem hispânica, do Oriente Médio, indianos, nativos americanos e asiáticos. Descrição dos aspectos audiológicos acometidos pela síndrome e as possíveis intervenções fonoaudiológicas para um caso específico. Paciente de 53 anos, sexo feminino, compareceu à Clínica de Fonoaudiologia, Faculdade de Odontologia de Bauru (FOB) com queixas audiológicas e diagnóstico médico da SVKH. A paciente apresentou perda auditiva sensório-neural bilateralmente, emissões otoacústicas evocadas ausentes e queixas vestibulares de vertigem postural e desequilíbrio ao andar, bem como queixa de zumbido agudo contínuo. O caso apresentado mostrou perda auditiva sensório-neural, vertigem, zumbido e acometimento ocular bilateral. Apesar do tratamento com corticoesteroide, a perda auditiva se manteve. Desta forma, precedente à indicação do AASI, o fonoaudiólogo deve atentar-se para o acompanhamento audiológico do caso, realização ou não de tratamento medicamentoso e ocorrência de sintomas sugestivos da síndrome, favorecendo o encaminhamento para o médico e participando ativamente do processo terapêutico envolvendo a audição.

ABSTRACT The Vogt-Koyanagi-Harada syndrome (VKHS) is a rare, multisystemic and autoimmune disease. The syndrome mainly affects the eyes, followed by bilateral chronic panuveitis, however, the syndrome may also affect the melanocytes tissues, for example, the eyes, inner ear, meninges and skin. The syndrome origin mechanism is not yet completely known. Commonly, the specific ethnic groups that are affected by the VKHS are as follows: Hispanics, Asians, Indians, Native Americans and ethnic groups from the Middle East. The audiological characteristics of the syndrome and the possible audiologist interventions for a specific case will be reported. The patient was attended at the Clinic of Speech Therapy, Faculdade de Odontologia de Bauru (FOB). She is 53 years old and presented audiological complaints. She was diagnosed with VKHS by a specialist doctor. Throughout the audiologist assessment, she presented bilateral sensorineural hearing loss, absent otoacoustic evoked emissions, complaints about postural vertigo and acute tinnitus. The specific case reported presented sudden sensorineural hearing loss, vertigo, tinnitus and bilateral ocular disease. Even though drug treatment was performed, the hearing loss remained. Therefore, before the hearing aid (HA) fitting, the audiologist should perform the hearing management, investigate if the patient takes the drug treatment and the occurrence of suggestive symptoms of the syndrome. These are some points that help in the reference to the specialist doctor and the audiologist strongly participates in what concerns the hearing.

Hipoacusia en enfermedad de Vogt-Koyanagi-Harada: caso clínico y revisión de literatura / Hearing loss in Vogt-Koyanagi-Harada Disease: case report and literature review

RESUMEN La patología autoinmune de oído interno se caracteriza por presentar hipoacusia sensorioneural bilateral, asimétrica y progresiva, que responde a terapia médica, presentándose aislada o como parte de una enfermedad sistémica como la enfermedad de Vogt-Koyanagi-Harada. Se presenta el caso de una paciente que consulta por cefalea, visión borrosa y sintomatología audiológica, con diagnóstico de enfermedad de Vogt-Koyanagi-Harada. Revisamos la literatura disponible respecto a la enfermedad y sus manifestaciones otológicas.

ABSTRACT Autoimmune inner ear disease presents a bilateral, asymmetric and progressive sensorineural hearing loss, that responds to medical therapy, presenting alone or associated to a systemic disease such as Vogt-Koyanagi-Harada disease. We present the case of a patient with headache, blur vision and audiologic symptoms, diagnosed with Vogt-Koyanagi-Harada disease. We review the literature about the disease and its otologic manifestations.

Síndrome de Vogt-Koyanagi-Harada en niños / Vogt-Koyanagi-Harada syndrome in children

Vogt-Koyanagi-Harada (VKH) syndrome is a systemic inflammatory disease that causes chronic and bilateral granulomatous panuveitis, usually described in adults. Objectives: To describe manifestations and complications of VKH in pediatric patients. Methods: Retrospectivedescriptive study upon patients <14 years-old with VKH, attended from January 1985 to July 2010 in three different centers. Results: A total of 17 patients (34 eyes) were studied; 9 (53%) female. The mean age was 10.8 years-old. Among extraocular manifestations; neurological (71%), dermatological (29%) and auditive (24%) signs were observed. Ocular findings included optic-disc involvement (94%), anterior uveitis (79%), choroiditis (77%), serous retinal detachment (71%) and vitritis (71%). Initial visual acuity (VA) was ≤0.05 in 47% of cases and ≥0.6 in 12% of patients. 71% presented complications: glaucoma (20 eyes), sinechiae (10 eyes), maculopathy (6 eyes) cataract (5 eyes) and ptisis bulbi (1 eyes). 35% received only corticosteroids and 65% inmunosupressive drugs. After treatment, 6% had VA ≤0.05 and 59% ≥0.6. Ten patients (59%) recurred: 30% compromising posterior pole, and 50% recurred >3 times. Conclusions: VKH in children is infrequent. It presents with optic-disc involvement and complications of posterior pole. It requires a high degree of suspicion, quick evaluation and early treatment, which include inmunosupressive and extended corticosteroid therapy. Nevertheless, a high rate of recurrence is seen among this group of patients. (AU)

Recurrent Unilateral Vogt-Koyanagi-Harada Disease with Posterior Scleritis

No abstract available.

Echographic findings in the late stages of Vogt-Koyanagi-Harada disease in mexican population / Resultados ecográficos nas fases atrasadas da doença de Vogt-Koyanagi-Harada na população mexicana

Purpose: To correlate clinical findings of Vogt-Koyanagi-Harada disease with standardized echography findings in a cross-sectional, descriptive and observational study. Methods: Patients with Vogt-Koyanagi-Harada disease in the convalescent and recurrence phases were evaluated with standardized ocular echography. Eyes with opaque media were excluded. Clinical findings were correlated with echographic data. Results: Thirty-seven eyes of 25 patients were included. Best corrected visual acuity was in average 20/100 (0.70 logMAR). Clinical findings included: sunset glow fundus (92%), pigment migration (92%), nummular chorioretinal depigmented scars (68%) and subretinal fibrosis (64.8%). Standardized echography was able to recognize all the cases with subretinal fibrosis (n=24) described clinically. Standardized echography showed a 100% sensitivity and specificity of finding subretinal fibrosis. Subretinal fibrosis in patients with Vogt-Koyanagi-Harada represents a risk factor for low vision. In our patients’ eyes, presence of subretinal fibrosis had a 2.5 time relative risk of having a visual acuity equal or worst to 20/70. Conclusions: Standardized echography represents a useful tool in patients with VKH in the chronic (convalescence and recurrence) phase of the disease. Subretinal fibrosis, a sight threatening complication in the convalescence and recurrent phases of Vogt-Koyanagi-Harada, can be diagnosed with ocular echography, with characteristic images. Knowledge of these images can be useful in cases with opaque media and bilateral anterior segment granulomatous inflammatory disease. .

Objetivo: Correlacionar achados clínicos da síndrome de Vogt-Koyanagi-Harada com resultados ecográficos padronizado da doença em um estudo transversal, descritivo e observacional. Métodos: Pacientes com a doença de Vogt-Koyanagi-Harada, o convalescente e recorrência em fases padronizadas foram avaliados com ecografia ocular. Olhos com material opaco foram excluídos. Achados clínicos foram correlacionados com dados ecográficos. Resultados: Um total de 25 pacientes e trinta e sete olhos foram incluídos no estudo. A acuidade visual (AV) 20/100 foi em média 0.70 logMAR. Os achados clínicos incluídos: sunset glow fundus (92%), pigmento migração (92%), numular despigmentado cicatrizes coriorretinianas (68%) e fibrose sub-retiniana (64,8%). A ecografia padronizada foi capaz de reconhecer todos os casos de fibrose sub-retiniana (n= 24) descrito clinicamente. A ecografia revelou um padrão 100% de sensibilidade e especificidade do diagnóstico fibrose sub-retiniana. Sub-retiniana em pacientes com fibrose Vogt-Koyanagi-Harada representa um fator de risco para a baixa visão. Em nossos pacientes olhos, presença de fibrose subretiniana tinham um risco relativo 2,5 hora de ter uma acuidade visual igual ou pior para 20/70. Conclusão: Ecografia padronizadarepresenta uma ferramenta útil em pacientes portadores da doença na fase crônica (convalescença, e recidiva). Fibrose sub-retiniana, uma visão ameaçadora e complicação na convalescença e recorrentes nas fases da Síndrome de Vogt-Koyanagi- Harada, podem ser diagnosticados com ecografia ocular, com imagens características. O conhecimento dessas imagens pode ser útil em casos com material opaco e segmento anterior bilateral da doença inflamatória granulomatosa. .

Úvea y enfermedades sistémicas / Uvea and systemic diseases

El ojo, además de entregarnos el sentido de la visión, también nos permite conocer la salud general del organismo. Muchas enfermedades sistémicas se manifiestan en el órgano visual antes, durante o después del debut de ellas a nivel sistémico. Este compromiso visual es variado y depende de la enfermedad en cuestión, destacando la escleritis, uveítis y vasculitis retinales. Conocer el estado ocular permitirá al clínico realizar diagnósticos más asertivos y oportunos, realizar el tratamiento más adecuado y definir pronóstico en varias enfermedades, que tienen al globo ocular como un órgano blanco. El objetivo de esta revisión es atraer la atención del lector sobre el compromiso oftalmológico en varias enfermedades

The eye besides giving us sight, it let us know the general health of the whole body as well. Many systemic diseases become evident in the visual organ before, during or after its systemic onset. The visual involvement in these diseases is wide and depends on the underlying disease. It is worth to stand out the escleritis, uveitis and retinal vasculitis. The knowledge of the ocular health will let the physician to diagnose more accurately and in time, to start the most appropriate treatment and to define the prognosis of several diseases that have the eye as a target organ. The aim of this review is to draw the reader's attention to the ophthalmic involvement in several systemic diseases

Visual rehabilitation in pre phthisis bulbi's patient: case report / Reabilitação visual em paciente com pré phthisis bulbi: relato de caso

Uveitis is a group of intraocular inflammatory diseases that affects mainly patients of working age. Vogt-Koyanagi-Harada disease is a chronic, granulomatous, systemic and autoimmune disease with manifestations in the eye, skin, and hearing and nervous systems. Ocular complications include cataract, glaucoma, choroidal neovascular membrane formation, and subretinal fibrosis, which can lead to phthisis bulbi with profound low vision or even blindness. The purpose of this article is to present the visual rehabilitation of a young patient with pre phthisis bulbi as a complication of the uveitis. During the active stage of the disease the patient quit her studies in a crucial phase of her life. After clinical treatment and surgery, training program of optical device adaptation and residual visual optimization were performed. Due to the great patient's motivation, it was possible to reintegrate her to social life.

Uveíte é o termo utilizado para descrever um grupo de doenças inflamatórias oculares que ocorrem predominantemente na idade produtiva. Vogt-Koyanagi-Harada é uma doença sistêmica, autoimune, granulomatosa, crônica com manifestações oculares, neurológicas, auditivas e cutâneas. As principais complicações incluem catarata, glaucoma, membrana neovascular e fibrose sub-retiniana, que podem evoluir para atrofia ocular, baixa visão profunda e cegueira. O propósito deste artigo é apresentar um caso de reabilitação visual em paciente jovem com pré phthisis bulbi consequente às complicações da uveíte. Após tratamento clínico e cirurgia, um programa de treinamento e adaptação de recursos ópticos foi realizado para otimizar o uso do resíduo visual, motivando a paciente para reintegrar-se socialmente e voltar aos estudos.

Edema of the Photoreceptor Layer in Vogt-Koyanagi-Harada Disease Observed Using High-Resolution Optical Coherence Tomography

PURPOSE: To evaluate the characteristics of fluid accumulation in the uveitic stage of Vogt-Koyanagi-Harada (VKH) disease using high resolution optical coherence tomography (OCT3). METHODS: Twenty-eight eyes in 14 patients with VKH disease were reviewed retrospectively. These 28 eyes were divided into 19 eyes with intraretinal fluid (C group) and 9 eyes without intraretinal fluid (N group). Changes in visual acuity and fluid accumulation observed using OCT were compared between the two groups. RESULTS: Visual acuity at the time of presentation was significantly worse in the C group than in the N group (p=0.005). The photoreceptor layer appeared to be double-layered due to a cystoid space in the C group. Layered structures and strands found in the cystoid space. Expanding sponge-form edema led to the development of a cystoid space in the photoreceptor layer. Intraretinal fluid resolved earlier than subretinal fluid. There were no observed differences in visual acuity between the two groups after four days of treatment. CONCLUSIONS: Accumulation of intraretinal fluid was related to poor initial visual acuity, but not to final visual acuity. High resolution OCT findings indicate that edema of the photoreceptor layer participates in the development of a cystoid space.

A Case of Vogt-Koyanagi-Harada Disease in a Patient With Graves Disease

A case of Vogt-Koyanagi-Harada disease (VKH) that developed in a 36-year-old woman with Graves' disease was described. The patient was treated with Lugol's solution and presented with bilateral serous retinal detachment. She had also suffered from methimazole-induced hypersensitivity and steroid-induced myopathy. Fluorescein angiography showed multiple leakage points and a lumbar puncture revealed pleocytosis, which was compatible with VKH. High dose steroid pulse therapy was successful. Altered immune regulation associated with drug-induced hypersensitivity may contribute to the development of VKH in patients with Graves' disease.

Síndrome de Vogt Koyanagi Harada / Vogt-Koyanagi-Hada syndrome

El síndrome de Vogt Koyanagi Harada es una uveitis difusa, bilateral, aguda, granulomatosa, asociada a manifestaciones dermatológicas, auditivas y del sistema nervioso central. Es la panuveitis bilateral más frecuentemente encontrada en la población mestiza e indígena mexicana. Una respuesta autoinmune, regulada por factores genéticos y aparentemente dirigada contra los melanocitos parece ser la causa del proceso inflamatorio. El diagnóstico básicamente es clínico, aunque la florangiografía de retina y el estudio ecográfico pueden ayudar a sustentar el diagnóstico básicamente es clínico, aunque la florangiografía de retina y el estudio ecográfico pueden ayudar a sustentar el diagnóstico en algunos casos. En el presente artículo revisamos los conocimientos que en relación a fisiopatogenia, diagnóstico, pronóstico, tratamiento médico y quirúrgico se tienen actualmente

Possível associaçäo entre melanoma maligno de coróide e Síndrome de Vogt Koyanagi Harada / Possible association between choroidal malignant melanoma and Vogt Koyanagi Harada Syndrome

O artigo apresenta um caso com manifestaçöes oculares e extra-oculares clássicas de síndrome de Vogt Koyanagi Harada. Com o passar do tempo, um melanoma maligno de coróide, já com extensäo extra bulbar, foi diagnosticado, sugerindo a possibilidade de uma oftalmia simpática com manifestaçöes sistêmicas simulando a síndrome de Vogt Koyanagi Harada. O emprego de droga citotóxica, assim como a gravidez, contribuiram para um desfecho dramático

Distribución de morbilidad en el departamento de uveítis: servicio de oftalmología del Hospital del Salvador, 1987-1991 / Distribution of morbidity in the uveitis department: oftalmology unit of the Salvador Hospital, 1987-1991

En 277 pacientes con uveítis, 176 mujeres (36,5 por ciento ), siguiendo la clasificación recomendada por el Grupo Internacional de Estudio de la Uveítis (I.U.S.G.) encontramos la siguiente distribución: 1. Anteriores en 126 (45,5 por ciento ); 2. Difusas en 68 (24,5 por ciento ); 3. Posteriores en 65 (23,5 por ciento ) y 4. Intermedias en 18 (6,5 por ciento ). El promedio general de edad fue de 39,2 años (2-78): para las anteriores de 43,2 años, para las difusas de 37 años, para las posteriores de 36,1 años y para las intermedias de 22,3 años. Se encontró la causa en 137 pacientes (49,4 por ciento ) siendo el diagnóstico más frecuente la toxoplasmosis con un 8 por ciento del total de uveítis. En las uveítis anteriores la etiología fue precisada en 41 casos (34,1 por ciento ) siendo las causas más frecuentes virus herpes, enfermedades reumáticas e iridociclitis HLA B27 positiva. En las uveítis difusas determinamos la etiología en 35 casos (51,5 por ciento ) siendo las causas más frecuentes el síndrome de Vogt-Koyanagi-Harada, la enfermedad de Behcet y la tuberculosis. En las uveítis posteriores determinamos la etiología en 43 casos (66,1) siendo las principales causas toxoplasmosis, toxocariasis y tuberculosis. Las uveítis intermedias fueron consideradas como de causa conocida

Sindrome de Vogt-Koyanagi-Harada / Vogt-Koyanagi-Harada syndrome

Se informan tres pacientes con el sindrome de Vogt-Koyanagi-Harada, conocido tambien como uveomeningoencefalitis, entidad poco conocida, de causa no claramente determinada, caracterizada por un proceso inflamatorio que compremete uvea, retina, meninges y piel.