Autoinflammation of the Inner Ear

Sensorineural hearing loss can result from dysfunction of the inner ear, auditory nerve, or auditory pathways in the central nervous system. Sensorineural hearing loss can be associated with age, exposure to ototoxic drugs or noise, or mutations in nuclear or mitochondrial genes. However, it is idiopathic in some patients. Although these disorders are mainly caused by dysfunction of the inner ear, little of the pathophysiology in sensorineural hearing loss is known due to inaccessibility of the living human inner ear for biopsy and pathological analysis. The inner ear has previously been thought of as an immune-privileged organ since the blood-labyrinth barrier is tightly controlled to the cochlear microenvironment from the circulation.



Recent studies revealed that bone marrow-derived macrophages are always present in the cochlea. These macrophages are activated in response to various type of insults, including noise exposure, ischemia and mitochondrial damage. Another study showed that a gain of function mutation of the NLRP3 gene is associated with autosomal-dominant syndromic/non-syndromic hearing loss with cochlear

autoinflammation. NLRP3 encodes the NLRP3 protein, a key component of the NLRP3 inflammasome that is expressed in immune cells, including monocytes and macrophages. Thus, macrophages and autoinflammation in the cochlea are associated with hearing loss, but function of macrophages in the cochlea and the pathophysiology of the autoinflammation are not well addressed. Our goal is to show the latest findings of cochlear immune cells including macrophages and the pathophysiology of autoinflammation in the cochlea.



As such, Topic Editors will welcome research article, brief research article, review, and mini-review- about, but not limited to the following themes:



• Distribution of immune cells including macrophages in the cochlea;

• Origin and early development of immune cells including macrophage in the cochlea;

• Functions of Immune cells including macrophages in the cochlea;

• Pathophysiology of noise induced hearing loss and its association with macrophages;

• Pathophysiology of sudden sensorineural hearing loss and its association with

Macrophages;

• Pathophysiology of age-related hearing loss and its association with macrophages;

• Pathophysiology of syndromic genetic hearing loss caused by autoinflammation and its

association with macrophages;

• Pathophysiology of non-syndromic genetic hearing loss caused by autoinflammation and

its association with macrophages;

• Treatment for genetic hearing loss caused by autoinflammation with anti-inflammatory

drugs.