Neuropathology of Hearing and Myelinopathies

In the Neuropathology of Hearing and Myelinopathies Group, we study the molecular basis of hearing, the pathology of hypoacusis and myelinopathies.

Hearing loss is one of the most prevalent medical problems. It is estimated that up to 8% of the population of the developed world suffers from it and its index is expected to increase due to aging, noise and other environmental factors. In Spain, 200 deep deaf people are born every year and more than one and a half million people suffer chronic hearing problems of greater or lesser degree. Our work contributes to define the genetic, molecular and cellular basis of inner ear development, to understand the physiopathology of hearing, and to investigate the potential clinical utility of IGF-1 and its intracellular targets as otoprotectors. We have developed technology and transferred it through the Neurofunctional Non-Invasive Evaluation facility. We are interested in training and dissemination as ways of generating social awareness about the importance of hearing.


Our group has achieved a national and international leadership position in the field of Hearing Neurobiology. We have achieved excellent ratings in both CSIC and CIBERER evaluations of the last five years. A pillar of our success is the multidisciplinary and multi-institutional composition, with UCM professors, clinicians and two indefinite CIBERER hires. Having the latter has been the key to setting and achieving more ambitious objectives, leading European projects and positioning ourselves as benchmarks in our field. The support of IIBAS, CIBERER and the Network of laboratories of the Community of Madrid has allowed us to transfer our experience to the Non-Invasive Neurofunctional Evaluation (ENNI) facility of the CSIC.

The scientific objectives that our group is pursuing are:


1) To study the genetic and molecular bases of hereditary hearing loss associated with the deficit in insulin-like growth factor type I, its high affinity receptor and its intracellular targets, specifically p38/DUSP1 and NLRP3. To identify transcriptional networks that regulate inner ear senescence.

2) To study the environment-genome interaction in animal models of hearing loss to identify genetic predisposition factors. Among the environmental factors inducing damage, we study noise, ototoxics and nutritional status. In these damage processes, the oxidative-inflammatory state is key to the development of hearing disorders.


3) To study the pathobiology of the human vestibular schwannoma, a rare cancer, in collaboration with H. La Paz and with IdiPAZ.


4) To develop tools for the study of these objectives: primary cultures 2 and 3D, animal models, ex vivo cultures, equipment, microsurgical procedures, biomarker panels and new routes of administration and visualization (MRI) of drugs to the inner ear. Keep the ENNI competitive, a unique service of the network of laboratories of the Community of Madrid.


5) To develop in these models preclinical studies of: i) ototoxicity of new molecules; (ii) drug repositioning; (iii) otoprotective potential of new molecules; and (iv) novel biocompatible nanomaterials for drug delivery. 


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Metabolic and Immune Diseases

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Neurological Diseases and Aging

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Rare Diseases

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