Musashi-1 is the candidate of the regulator of hair cell progenitors during inner ear regeneration.

Background: Hair cell loss in the cochlea is caused by ototoxic drugs, aging, and environmental stresses and could potentially lead to devastating pathophysiological effects. In adult mammals, hair cell loss is irreversible and may result in hearing and balance deficits. In contrast, nonmammalian vertebrates, including birds, can regenerate hair cells through differentiation of supporting cells and restore inner ear function, suggesting that hair cell progenitors are present in the population of supporting cells.

Cholesterol influences potassium currents in inner hair cells isolated from guinea pig cochlea.

Objective: There is a correlation between serum hyperlipidemia and hearing loss. Cholesterol is an integral component of the cell membrane and regulates the activity of ion channels in the lipid bilayer. The aim of this study was to investigate the effects of cholesterol on the potassium currents in IHCs by using the cholesterol-depleting drug, MβCD, and water-soluble cholesterol.

Nitric oxide influences potassium currents in inner hair cells isolated from guinea-pig cochlea.

Objective: Nitric oxide (NO) is a diffusible second messenger, which regulates neurotransmission, serving as the principal endothelium-derived relaxing factor. NO also acts as an ion channel modulator. Nitric oxide synthase (NOS) has been identified in the inner ear, although its physiological role remains unclear.

Asymptomatic fibrous dysplasia of the temporal bone.

Introduction: Fibrous dysplasia is a bone disorder of unknown origin in which normal bone is replaced with fibrotic tissue and disorganised bone trabeculae. The temporal bone is rarely affected. Because of the slowly progressive course of the disease, many mild cases may never be recognised and are found incidentally.

Massively parallel DNA sequencing facilitates diagnosis of patients with Usher syndrome type 1.

Usher syndrome is an autosomal recessive disorder manifesting hearing loss, retinitis pigmentosa and vestibular dysfunction, and having three clinical subtypes. Usher syndrome type 1 is the most severe subtype due to its profound hearing loss, lack of vestibular responses, and retinitis pigmentosa that appears in prepuberty. Six of the corresponding genes have been identified, making early diagnosis through DNA testing possible, with many immediate and several long-term advantages for patients and their families.