Sudden Sensorioneural Hearing Loss and Autoimmune Systemic Diseases

Abstract Introduction Several authors have demonstrated the relationship between sudden sensorineural hearing loss (SNHL) and systemic autoimmune diseases (SAD). Immunemediated SNHL can rarely present as unilateral sudden SNHL and manifests itself in the contralateral ear only after years. It presents clinical relevance for being one of the few SNHL that may be reversible given that early and appropriate treatment is applied. Objective The objective of this study is to describe the clinical presentations and audiological findings from patients with idiopathic sudden SNHL and SAD associated with a probable diagnosis of immune-mediated SNHL. Furthermore, we strive to estimate the prevalence of SAD in patients with sudden SNHL. Methods This is an observational retrospective cohort. We have selected and studied patients with SAD. Revision of available literature on scientific repositories. Results We evaluated 339 patients with sudden SNHL. Among them, 13 (3.83%) patients suffered from SAD. Three patients had bilateral involvement, a total of 16 ears. We evaluate and describe various clinical, epidemiological, and audiological aspects of this sample. Conclusion In our sample of patients with sudden SNHL, the prevalence of SAD was found relevant. The majority had tinnitus and dizziness concomitant hearing loss, unilateral involvement and had experienced profound hearing loss at the time of the installation. In spite of instituted treatment, most cases showed no improvement in audiometric thresholds. Apparently, patients with sudden SNHL and SAD have a more severe initial impairment, higher percentage of bilateral, lower response to treatment, and worse prognosis than patients with sudden SNHL of unknown etiology.

Variables with prognostic value in the onset of idiopathic sudden sensorineural hearing loss / Variáveis com valor prognóstico no momento da instalação da perda auditiva neurossensorial súbita idiopática

ABSTRACT INTRODUCTION: The establishment of an individualized prognostic evaluation in patients with a diagnosis of idiopathic sudden sensorineural hearing loss (ISSHL) remains a difficult and imprecise task, due mostly to the variety of etiologies. Determining which variables have prognostic value in the initial assessment of the patient would be extremely useful in clinical practice. OBJECTIVE: To establish which variables identifiable at the onset of idiopathic sudden sensorineural hearing loss have prognostic value in the final hearing recovery. METHODS: Prospective, longitudinal cohort study. Patients with ISSHL followed by the Department of Otology-Neurotology of a quaternary hospital were included. The following variables were evaluated and correlated with final hearing recovery: age, gender, vertigo, tinnitus, initial degree of hearing loss, contralateral ear hearing, and elapsed time to treatment. RESULTS: 127 patients with ISSHL were evaluated. Rates of absolute and relative recovery were 23.6 dB and 37.2% respectively. Complete hearing improvement was observed in 15.7% patients; 27.6% demonstrated significant improvement and improvement was noted in 57.5%. CONCLUSION: During the onset of ISSHL, the following variables were correlated with a worse prognosis: dizziness, profound hearing loss, impaired hearing in the contralateral ear, and delay to start treatment. Tinnitus at the onset of ISSHL correlated with a better prognosis.

RESUMO Introdução: Elaborar avaliação prognóstica individualizada em pacientes com diagnóstico deperda auditiva neurossensorial súbita idiopática (PANSI) permanece tarefa árdua e imprecisa devido, em grande parte, à variedade de etiologias. A determinação de quais variáveis teriam valor prognóstico na avaliação inicial do paciente seria de extrema utilidade na prática clínica. Objetivo: Estabelecer quais variáveis, identificáveis no momento de instalação da perda auditiva neurossensorial súbita idiopática, têm valor prognóstico na recuperação auditiva final. Método: Estudo de coorte prospectivo, longitudinal. Incluídos pacientes com PANSI acompanhados pela Disciplina de Otologia-Neurotologia de um hospital quaternário. As seguintes variáveis foram avaliadas e correlacionadas com a recuperação auditiva final: idade, gênero, vertigem, zumbido, grau de perda auditiva inicial, audição na orelha contralateral, tempo para início de tratamento. Resultado: Foram avaliados 127 pacientes com PANSI. As taxas de recuperação absoluta e relativa foram 23,6 dB e 37,2% respectivamente. Apresentaram melhora completa da audição 15,7% dos pacientes; 27,6% apresentaram melhora significativa e 57,5% melhora. Conclusão: No momento da instalação da PANSI, as seguintes variáveis correlacionaram-se com pior prognóstico: vertigem, perda auditiva profunda, audição alterada na orelha contralateral e demora para início do tratamento. Presença de zumbido na instalação da PANSI correlacionou-se com melhor prognóstico.

Glucocorticoid influence on prognosis of idiopathic sudden sensorineural hearing loss / Influência dos corticosteroides no prognóstico auditivo da perda auditiva neurossensorial súbita idiopática

INTRODUCTION: Idiopathic Sudden Sensorineural Hearing Loss (ISSHL) is defined when a loss of at least 30 dB occurs in over 3 continuous frequencies, in up to 72 hours, of which etiology is not established, despite adequate investigation. Different types of treatment regimens have been proposed, but only glucocorticoids have shown some evidence of benefit in the literature. OBJECTIVE: To analyze whether the type of treatment or time of treatment with glucocorticoids have any influence on hearing recovery in ISSHL. METHODS: Observational retrospective cohort study. One hundred twenty-seven patients with ISSHL, treated at outpatient clinics between the years 2000 and 2010, were studied. We evaluated the prognostic correlation of the type of treatment and time to treatment with glucocorticoids and ISSHL. RESULTS: The absolute hearing gain and the relative hearing gain was as follows: 23.6 dB and 37.2%. Complete recovery was observed in 15.7% of patients, significant recovery in 27.6% and recovery in 57.5%. CONCLUSION: In this study, there was no difference between the use and nonuse of glucocorticoids in hearing improvement. However, when started within seven days after onset, the use of glucocorticoids was a factor of better prognosis. .

INTRODUÇÃO: A perda auditiva neurossensorial súbita idiopática (PANSSI) é definida pela queda dos limiares auditivos tonais de, pelo menos, 30 dB em três frequências contíguas em até 72 horas e apesar de uma investigação apropriada, a etiologia da lesão não é encontrada. Diversos tipos de tratamentos já foram idealizados para a PANSSI, no entanto, os corticosteroides são os que encontram as melhores evidências de efetividade na literatura. OBJETIVO: Avaliar se o tipo de tratamento e o tempo de demora em iniciar o tratamento com corticosteroides têm correlação com a melhora dos limiares auditivos na PANSSI. MÉTODOS: Estudo de coorte retrospectivo observacional. Foram avaliados 127 pacientes com PANSSI provenientes do ambulatório entre os anos de 2000 e 2010. Foi avaliada a correlação prognóstica do tipo de tratamento e tempo de demora para o início de tratamento e a PANSSI. RESULTADOS: As taxas de recuperação absoluta e relativa foram 23,6 dB e 37,2% respectivamente. Apresentaram melhora completa 15,7% dos pacientes, 27,6% apresentaram melhora significativa e 57,5% melhora. CONCLUSÃO: Neste estudo, não houve diferença entre o uso ou não de corticosteroide na melhora auditiva. Contudo, quando iniciado até sete dias, o uso de corticosteroide foi fator de melhor prognóstico. .

Comparação entre critérios de recuperação auditiva na perda neurossensorial súbita / Comparison of hearing recovery criteria in sudden sensorineural hearing loss

Inúmeros métodos de análise da recuperação auditiva na perda auditiva neurossensorial súbita idiopática (PANSI) dificultam a comparação adequada dos diversos tratamentos encontrados na Literatura. OBJETIVO: Comparar diversos critérios de recuperação auditiva na PANSI, baseados na Literatura. MATERIAL E MÉTODO: Foi realizado um estudo clínico observacional de coorte, a partir de um protocolo prospectivo, em pacientes com PANSI, atendidos entre 2000 e 2010. Foram comparados cinco critérios de recuperação auditiva significativa e quatro critérios para recuperação completa, pela audiometria tonal, por meio de teste não paramétrico e de comparações múltiplas, ambos com um nível de significância de 5%. Após determinação do critério de recuperação auditiva mais rígido, foram adicionados parâmetros da audiometria vocal. RESULTADOS: Houve diferença significativa, entre esses critérios (p < 0,001), quando analisados em conjunto. A recuperação auditiva para, pelo menos, grau leve, ocorreu em apenas 35 (27,6%) pacientes. Ao adicionarmos parâmetros da audiometria vocal, apenas 34 pacientes (26,8%) tiveram melhora significativa. CONCLUSÕES: Existe falta de uniformidade entre os critérios de recuperação auditiva utilizados pela literatura. O critério de mudança de categoria funcional para um grau, pelo menos leve, foi o mais rígido. O uso da audiometria vocal não foi fundamental para definir recuperação auditiva significativa.

The countless methods available to analyze hearing recovery in idiopathic sudden sensorineural hearing loss (ISSHL) cases hinder the comparison of the various treatments found in the literature. OBJECTIVE: This paper aims to compare the different criteria for hearing recovery in ISSHL found in the literature. MATERIALS AND METHODS: This is an observational clinical cohort study from a prospective protocol in patients with ISSHL, treated between 2000 and 2010. Five criteria were considered for significant hearing recovery and four for complete recovery by pure tone audiometry, using non-parametric tests and multiple comparisons at a significance level of 5%. After determining the stricter criteria for hearing recovery, vocal audiometry parameters were added. RESULTS: There was a significant difference between the criteria (p < 0.001) as they were analyzed together. Mild auditory recovery occurred in only 35 (27.6%) patients. When speech audiometry was added, only 34 patients (26.8%) showed significant improvement. CONCLUSIONS: There is a lack of consistency among the criteria used for hearing recovery. The criterion of change of functional category by one degree into at least mild hearing recovery was the stricter. Speech audiometry did not prove essential to define significant hearing recovery.