David Lobo

Sudden presentation of immune-mediated inner ear disease: characterization and acceptance of a cochleovestibular dysfunction

Since the McCabe report, growing indirect evidence has accumulated to indicate the implication of immune mechanisms in the pathogenesis of immune-mediated inner-ear disease (IMIED). A clinical study of a group of patients affected by this condition was performed in order to characterize the immune group, based on a recently reported profile, and compared with the vascular, viral and idiopathic aetiologies of sudden deafness. Patients affected by immune-mediated inner-ear disease had the best and the earliest recovery rate of hearing (p = 0.0028 and p = 0.017, respectively). However, this group of patients also had the higher rate of recurrence (p = 0.034), supporting the typical clinical course of the autoimmune disorders. On the basis of the results the criteria used in the diagnosis of the sudden presentation of the immune-mediated inner ear disease could be accepted leading to the characterization of this condition. Likewise, the role of the supporting cells in the pathogenesis of the IMIED is discussed.

Immunologic work-up study for inner ear disorders: Looking for a rational strategy

Conclusions. For the evaluation of a patient with suspected immune-mediated inner ear disease (IMIED), an exhaustive immunologic work-up study is not recommended if financial resources are limited. Analysis of antinuclear antibodies (ANA) and the immunophenotype of peripheral blood lymphocytes (PBL) proved to be the most useful tests in our population to support the clinical diagnosis of IMIED. Objective. Owing to the lack of specific serological markers for the diagnosis of IMIED, an exhaustive immunologic work-up study for patients with suspected IMIED is usually performed. The aim of this study was to estimate the use of resources and the costs involved in the routine laboratory tests used for the diagnosis of IMIED. Material and methods. This was a comparative study of two groups of patients with a high suspicion of diverse clinical forms of IMIED who were subjected to different serologic test designs. The cost of the classical immunologic work-up study used for the diagnosis of IMIED (n=125) was estimated in comparison with that of a more restricted examination, based on a recently reported high-risk profile, involving the analysis of ANA and PBL (n=57). Results. The diagnostic efficiency of the two protocols was similar. The cost of a complete immunologic work-up study was €241.77 and that of the limited analysis was €53.12.

Adverse Effects of Glucocorticoid Therapy for Inner Ear Disorders

Background: Because of their anti-inflammatory effects and suppression of the immune system, glucocorticoids have been widely used in otolaryngologic disorders and perioperative conditions. Objective: The objective of the present study was to determine the incidence of adverse effects after the administration of glucocorticosteroids in patients affected by diverse inner ear disorders. Methods: One hundred and sixty-three patients affected by sudden sensorineural hearing loss, 39 with progressive sensorineural hearing loss and 16 with fluctuating sensorineural hearing loss were subjected to glucocorticosteroid therapy with 6-methylprednisolone at a starting dose of 1 mg/kg body weight per day; this therapy was tapered during the next 21–28 days. In 20 patients with profound hearing loss (>70 dB), 3 boluses of prednisolone-21-hydrogen-succinate (500 mg per day) were administered. After receiving the boluses, these patients continued with the oral 6-methylprednisolone scheme. Results: Mild adverse effects were observed in 16 patients (7.01%). Only 2 patients (0.9%) with sudden sensorineural hearing loss showed severe complications: peptic ulcer and avascular necrosis of the femoral heads. Conclusions: The low percentage of severe adverse effects observed in the present study validates the use of corticosteroids for the treatment of inner ear disorders although we should not underestimate these rare complications.

Controversies and Criticisms on Designs for Experimental Autoimmune Labyrinthitis

Although immune-mediated inner ear disease was reported around 25 years ago, numerous attempts to identify the inner ear antigens have been performed. Experimental animal models have been used to study the immune mechanisms involved in hearing loss and to develop new therapies. Because animal models of autoimmune labyrinthitis have been developed by means of different antigens, we cannot yet show a valid immunopathologic explanation. A critical analysis of the more relevant experimental models employed has been performed in order to validate the methodology. Comparison between these models and animals with spontaneous systemic autoimmune disease has raised more questions concerning the pathophysiology of autoimmune hearing loss. A new pathogenetic theory is suggested, involving the supporting cells of the organ of Corti.

Diagnostic tests for immunomediated hearing loss: a systematic review

OBJECTIVE To quantitatively evaluate the diagnostic accuracy of diagnostic tests for immunomediated hearing loss. DATA SOURCES We searched Medline and the Cochrane Database of Systematic Reviews for potentially relevant studies. STUDY SELECTION Twenty-five studies met the inclusion criteria of this systematic review. The diagnosis of immunomediated hearing loss was based on the clinical presentation and the response to corticosteroid administration. DATA EXTRACTION The following data were extracted from the selected studies and entered into a standardised database: population demographics; exclusion and inclusion criteria; diagnostic tests; sensitivity; specificity; the number of true positive, true negative, false positive and false negative values; therapy used, including dose and duration; and delay between symptom onset and therapy commencement. DATA SYNTHESIS This systematic review combined data from 679 patients with immunomediated hearing loss, reported by 22 research teams. Substantial heterogeneity was found among the included studies; for this reason, summary sensitivity and specificity values were not computed. CONCLUSIONS The results of diagnostic tests for immunomediated hearing loss depend on many factors, and there is a risk of potential bias. This is the first time that such a systematic review has been presented; such a review is a more rigorous method of demonstrating the utility of the available diagnostic tests.

Cirugía endoscópica nasosinusal: revisión de 1.093 casos

Resumen Se presenta una serie de 1.093 pacientes intervenidos a lo largo de 10 anos de cirugia endoscopica por patologia nasosinusal en un hospital de tercer nivel. Se describe la tecnica empleada y se analizan los resultados a largo plazo, asi como las complicaciones menores y mayores, en especial las fistulas de L.C.R. y sus causas

Anti-adhesive properties of polytetrafluoroethylene (Gore-Tex) in middle ear surgery. An experimental study.

Conclusions. Polytetrafluoroethylene (PTFE) may represent a material that prevents adhesions when implanted in the middle ear. This characteristic can be used to improve the design of prostheses. Objective. Prostheses in the middle ear have a relatively high failure rate due to extrusion, deformation and adhesion. Adhesion of prostheses to the walls of the tympanic cavity is the commonest cause of long-term hearing loss. The anti-adhesive properties of PTFE have been previously studied in abdominal and vascular surgery. To the best of our knowledge, this is the first work in which the properties of PTFE have been studied in the context of middle ear pathology. Material and methods. In order to induce otitis media with effusion, 27 Wistar rats underwent a technique based on permanent cauterization of the pharyngeal Eustachian tube. PTFE was subsequently implanted in the middle ear. Rats that underwent cauterization without implantation and cauterization with implantation of hydroxyapatite and Plastipore were included as controls. Results. Histopathological study of the tympanic bullae at 15 and 90 days after implantation demonstrated good biocompatibility of the PTFE implant under the analyzed conditions, with formation of a capsule around the material and no adhesions between the ear tissues and the prosthesis.

New prospects in the diagnosis and treatment of immune-mediated inner ear disease

Autoimmune inner ear disease (AIED) represents a very fertile research field and the advancements in the understanding of this disease have a direct application not only in patients affected with this condition but also in other inner ear disorders that share the same injury mechanism, damage to the inner ear hair cells. AIED also presents many challenges that have still to be overcome. Firstly, access to the inner ear is limited, as many interventions such as biopsies can result in great irreversible damage. Secondly, there are no completely specific markers for AIED. Lack of a definitive diagnosis can result in the treatment of patients not affected with the disease and, therefore, no response. Finally, some patients become refractory to glucocorticoids and new therapies are needed. This review offers an overview of the animal models that have contributed to the understanding of AIED pathophysiology, the value of currently available diagnostic tests, and therapeutic options, with a special focus on new therapies for non responders or patients refractory to glucocorticoids. Among these new options for therapy, biological agents have been tested recently, whereas gene and stem cell therapy may have a role in the future. The intratympanic route of administration avoids the systemic side effects associated with currently used drugs, and may become a more frequent approach in the future.

Enfermedad inmunomediada del oído interno: validación diagnóstica mediante un análisis sistemático de la literatura científica

Introduccion Se han desarrollado numerosas pruebas para ayudar en el diagnostico de la enfermedad inmunomediada del oido interno (EIOI), si bien su utilidad es motivo de controversia. Material y metodo Se ha revisado los estudios diagnosticos de cohortes de pacientes con sospecha de EIOI en los que se hubieran realizado diferentes tests serologicos y se ha recogido la respuesta al tratamiento inmunosupresor. Resultados Tras un analisis de 790 articulos, se ha observado una gran heterogeneidad tanto en las caracteristicas clinicas de los pacientes incluidos en los estudios (sordera subita, sordera fluctuante, sordera rapidamente progresiva, enfermedad de Meniere), los criterios de inclusion y exclusion, la dilacion entre el diagnostico y la instauracion del tratamiento medico o los criterios de respuesta a este. La sensibilidad y la especificidad de las pruebas se situan en torno a 0,5, con un intervalo muy amplio. Conclusiones El diagnostico de la EIOI se basa en la presentacion clinica y la respuesta a los corticoides. En este momento no hay ninguna prueba estandar para el diagnostico de la EIOI. Los tests serologicos pueden apoyar el diagnostico, pero se necesita de mas estudios con un alto rigor metodologico para conocer su verdadera importancia en la practica clinica diaria.

[Immunomediated inner ear disease: diagnostic validation by means of a systematic analysis of the scientific literature].

INTRODUCTION Numerous tests have been developed to help in the diagnosis of the immunomediated inner ear disease (IMIED), although their usefulness is still the subject of some dispute. MATERIAL AND METHOD Studies of cohorts of patients with suspected IMIED have been reviewed. A number of different serological tests were carried out and their response to immunosuppressive therapy was noted. RESULTS After reviewing 790 articles, the studies analyzed present great heterogeneity in the clinical characteristics of the patients recruited (sudden deafness, fluctuating and rapidly progressive hearing loss, Menières disease), the inclusion and exclusion criteria, the delay between the diagnosis and the start of medical treatment or the response criteria. CONCLUSIONS The diagnosis of IMIED is based on clinical presentation and response to corticosteroids. At present no test represents the gold standard in the diagnosis of IMIED. Serologic tests can support the diagnosis, but a methodologically more rigorous approach is needed to identify the true clinical importance of these tests for daily practice.