Review
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World J Methodol. Jun 26, 2014; 4(2): 91-98
Published online Jun 26, 2014. doi: 10.5662/wjm.v4.i2.91
New prospects in the diagnosis and treatment of immune-mediated inner ear disease
David R Lobo, Jose Ramon García-Berrocal, Rafael Ramírez-Camacho
David R Lobo, Department of Otolaryngology, Hospital El Escorial, San Lorenzo de El Escorial, 28200 Madrid, Spain
David R Lobo, Instituto Antolí-Candela, 28043 Madrid, Spain
Jose Ramon García-Berrocal, Rafael Ramírez-Camacho, Department of Otorhinolaryngology, Hospital Puerta de Hierro, Majadahonda, 28222 Madrid, Spain
Author contributions: Lobo DR, García-Berrocal JR and Ramirez-Camacho R solely contributed to this paper.
Correspondence to: David R Lobo, MD, PhD, Department of Otolaryngology, Hospital E Escorial, Carretera Guadarrama s/n, San Lorenzo de El Escorial, 28200 Madrid, Spain. dlobo28@gmail.com
Telephone: +34-918-973021 Fax: +34-918-973031
Received: November 26, 2013
Revised: February 10, 2014
Accepted: April 11, 2014
Published online: June 26, 2014
Processing time: 267 Days and 15 Hours
Abstract

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.

Keywords: Autoimmune inner ear disease; Diagnostic tests; Biologic therapy agents; Gene therapy; Stem cells

Core tip: Readers interested in inner ear pathology will find in this review a brief summary of autoimmune inner ear disease, with special focus on the major advances achieved in the knowledge of its etiology and pathophysiology, and the diagnostic and therapeutic challenges that remain and may guide research in the next few years and beyond.