Hearing and Myelinopathies


Molecular bases of auditory function and pathophysiology of hearing loss. Hearing loss is one of the most prevalent medical problems. It is estimated that up to 6% of the population of the developed world suffers it, their rates are expected to increase due to noise and other external factors. In Spain 200 profound deaf people are born every year and more than one and a half million people suffer chronic hearing problems of greater or lesser degree. Mutations in the IGF1 gene cause an extremely rare syndromic disease, which occurs with sensorineural deafness. The neurofunctional evaluation of hearing by evoked potentials has allowed us to describe that the deficit of IGF-1 causes sensorineural deafness in the mouse. The inner ear of this animal presents serious morphological alterations, to highlight the early neuronal death and its accelerated neurodegeneration. The final molecular causes are found in the altered spatial / temporal expression of transcription factors, MEF2 and FOXM1, among others, which cause cell failure in the terminal differentiation phase. As well as in the chronic inflammation that the cochlea presents. Other affected systems include the osseous, and between the sensory ones we find the vestibule and the retina. In this context, we study the intracellular signaling networks that participate in the response to this factor in different species. Focusing on the pathways that control autophagy, the inflammatory response and, in short, the activity of stress kinases. Overall, our work contributes to define the molecular and cellular bases of auditory degeneration and to investigate the potential clinical utility of IGF-1 and its targets as neuroprotectants. Publications: http://www.ncbi.nlm.nih.gov/pubmed?term=varela-nieto i. Group belonging to IdiPAZ and CIBER-ER. On the Internet: www.targear.eu

Varela Nieto, Isabel
Profesor Investigación