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Gene therapy of inherited and acquired hearing loss

Total Cost €


EC-Contrib. €






 iHEAR project word cloud

Explore the words cloud of the iHEAR project. It provides you a very rough idea of what is the project "iHEAR" about.

treatment    safety    aav    wp1    vitro    optimized    hair    ear    world    efficiency    employed    proof    promoters    substantial    protect    inducible    monogenetic    marker    transgene    protective    mouse    strategies    lines    mutations    proteins    fluorescent    primary    once    causative    inner    neurons    opportunity    tools    correct    genome    demonstrated    transduction    mirnas    600    therapeutic    receptor    precision    spiral    burden    caused    otic    models    expression    proper    samples    human    murine    financial    incl    transfer    children    stem    experiments    ipsc    appropriate    efficient    treat    corrective    translatability    explored    pluripotent    vector    lentiviral    hc    toolbox    impaired    translatable    viral    sgn    performing    people    editing    patients    perform    patient    vivo    hearing    genes    million    wp2    constructs    configurations    generate    acquired    clinically    model    cells    congenital    inherited    medicine    designs    collection    gene    ganglion    360    cell    therapy    social    vectors    alterations    whom    wp3    adeno   

Project "iHEAR" data sheet

The following table provides information about the project.


Organization address
address: Carl-Neuberg-Strasse 1
postcode: 30625

contact info
title: n.a.
name: n.a.
surname: n.a.
function: n.a.
email: n.a.
telephone: n.a.
fax: n.a.

 Coordinator Country Germany [DE]
 Total cost 1˙999˙500 €
 EC max contribution 1˙999˙500 € (100%)
 Programme 1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
 Code Call ERC-2018-COG
 Funding Scheme ERC-COG
 Starting year 2019
 Duration (year-month-day) from 2019-05-01   to  2024-04-30


Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 


 Project objective

To address the substantial financial and social burden caused by hearing loss in 360 million people world-wide, I aim to improve hearing via gene therapy to correct inherited and protect from acquired hearing loss. In vitro experiments will establish the best vector configurations for transfer of therapeutic genes and miRNAs into inner ear hair cells (HC) and spiral ganglion neurons (SGN). The efficiency of the best-performing vector designs will then be explored in vivo using fluorescent marker proteins. Cell-type specific and inducible promoters as well as receptor-targeted vectors will be employed as a safety measure and to ensure transgene expression in HC and SGN target cells. Once efficient transduction of appropriate target cells and proper expression of therapeutic proteins are demonstrated, I will perform proof-of-concept studies in hearing loss models, incl. established mouse models, to correct (WP1) or protect (WP2) from impaired hearing. To ensure translatability of these findings, I will generate human induced pluripotent stem cells (iPSC) from patients with hearing loss (WP3), so that I can test optimized constructs in human otic cells. Moreover, I have access to a collection of well-characterized samples from over 600 hearing loss patients, including children with congenital hearing loss in whom many novel monogenetic alterations were identified. These resources provide the unique opportunity to generate a novel toolbox for the treatment of hearing loss. In addition to lentiviral and adeno-associated viral (AAV) vector delivery of corrective or protective genes to treat hearing loss, I will apply state-of-the-art genome editing tools to model and correct mutations causative for hearing loss in cell lines, primary cells from murine models, human patients and patient-derived iPSC. This work will contribute to development of clinically translatable approaches for precision medicine strategies to improve hearing loss treatment.

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The information about "IHEAR" are provided by the European Opendata Portal: CORDIS opendata.

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