TY - JOUR
T1 - Autoimmune sensorineural hearing loss
T2 - An immunologic perspective
AU - Solares, C. Arturo
AU - Hughes, Gordon B.
AU - Tuohy, Vincent K.
N1 - Funding Information:
This work was supported primarily by grants from the Deafness Research Foundation, New York, NY, the Samuel Rosenthal Foundation, Cleveland, OH, and the Triple-T Foundation, Chardon, OH. Additional support came from National Institutes of Health grants NS-37476, NS-36054, and AI-51837.
PY - 2003/5
Y1 - 2003/5
N2 - Autoimmune sensorineural hearing loss (ASNHL) typically produces a bilateral rapidly progressive loss of hearing that may occur suddenly. The diagnosis is made by excluding ototoxicity, systemic disease, and other factors that mimic ASNHL and by showing a therapeutic response to corticosteroid treatment. Although autoantibodies and autoreactive T cells have been implicated in the etiopathogenesis of ASNHL, several central issues remain unresolved, including the relative prominence of B cell or T cell autoimmunity in the initiation and progression of ASNHL, the identity of the putative inner ear self-antigen(s) that target ASNHL, and the development and application of immunosuppressive therapies for preventing the progressive hearing loss which may be profound and require cochlear implantation. In this review, we will examine the seminal human and animal studies that have led to our current views regarding the autoimmune etiopathogenesis of ASNHL. In addition, we will address the need for developing an inner ear-specific mouse model for ASNHL that may define the stages leading to the development of ASNHL and may also provide new diagnostic markers and help develop novel and effective treatments for preventing progressive hearing loss in ASNHL.
AB - Autoimmune sensorineural hearing loss (ASNHL) typically produces a bilateral rapidly progressive loss of hearing that may occur suddenly. The diagnosis is made by excluding ototoxicity, systemic disease, and other factors that mimic ASNHL and by showing a therapeutic response to corticosteroid treatment. Although autoantibodies and autoreactive T cells have been implicated in the etiopathogenesis of ASNHL, several central issues remain unresolved, including the relative prominence of B cell or T cell autoimmunity in the initiation and progression of ASNHL, the identity of the putative inner ear self-antigen(s) that target ASNHL, and the development and application of immunosuppressive therapies for preventing the progressive hearing loss which may be profound and require cochlear implantation. In this review, we will examine the seminal human and animal studies that have led to our current views regarding the autoimmune etiopathogenesis of ASNHL. In addition, we will address the need for developing an inner ear-specific mouse model for ASNHL that may define the stages leading to the development of ASNHL and may also provide new diagnostic markers and help develop novel and effective treatments for preventing progressive hearing loss in ASNHL.
KW - Antibodies
KW - Autoimmunity
KW - Deafness
KW - Sensorineural hearing loss
KW - T cells
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U2 - 10.1016/S0165-5728(03)00077-8
DO - 10.1016/S0165-5728(03)00077-8
M3 - Review article
C2 - 12742646
AN - SCOPUS:0038297685
SN - 0165-5728
VL - 138
SP - 1
EP - 7
JO - Journal of Neuroimmunology
JF - Journal of Neuroimmunology
IS - 1-2
ER -