Abstract
Although congenital sensorineural hearing loss (SHL) in the bilateral cochleae mainly results from genetic abnormalities, chronic SHL progressing in later life is often influenced by systemic immune disturbances, including autoimmunity, chronic inflammation, and immunosenescence. We have investigated the relationship between the inner ear and systemic immunity and reviewed the possibilities to prevent SHL, including autoimmune SHL and age-related SHL. We also demonstrated two lymphocyte populations, interleukin 1 receptor type II (IL-1R2)-positive T cells (T1R2) and naturally occurring regulatory T cells (nTregs) in CD4+ T cells, which increase with aging, suppress host immune function and promote organ degeneration. Alterations in systemic immunity by fewer microbial antigen challenges in the living environment, elimination of immune suppressive lymphocytes, or immune rejuvenation with a reconstituted thymus may contribute not only to renew the cochlear function in SHL, but also to extend the healthy life of functional organs in a vigorous and youthful body, one of humanity’s greatest dreams.
Highlights
Sjögren’s syndrome and Behcet’s disease typically show oral lesions, it is well known that they are systemic symptoms beyond otolaryngology, and the head and neck area
Congenital sensorineural hearing loss (SHL) in the bilateral cochleae mainly results from genetic abnormalities, chronic SHL progressing in later life is often influenced by systemic immune disturbances, including autoimmunity, chronic inflammation, and immunosenescence
The profound atrophy of thyme tissue is central to immunosenescence [60] and leads to perturbed output of new T cells extended from hematopoietic stem cells (HSCs), as well as lymphoid progenitors and increased memory lymphocytes with accumulation of dysfunctional senescent cells [61] [62] [63] [64]
Summary
Age-related SHL, known as presbycusis, connected with systemic aging develops due to mitochondrial DNA damage in the cochlea following oxidative stress [6] and shows a delay in progression with exercise or caloric restriction [7] [8] [9]. Cochlear function and pathology are affected by, or coordinated with, the systemic environment including the immune system. We have studied preventive treatments for progressive bilateral SHL caused by disturbances in systemic immunity with autoimmune diseases or aging, but not caused by genetic abnormalities or acoustic trauma [11]-[18].
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