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Hearing Loss: Molecular Biological Insights, 2nd Edition

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: 20 January 2026 | Viewed by 412

Special Issue Editor


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Guest Editor
Unit of Excellence, Institute of Biology and Molecular Genetics (IBGM), University of Valladolid-CSIC, 47003 Valladolid, Spain
Interests: cochlea; inner ear; molecular mechanisms
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Disabling hearing loss is the most common sensory disorder, affecting over 5% of the world’s population and presenting alarmingly rising incidence rates. Obstacles to the development of therapeutics to treat these patients arise from the location of the cochlear tissue and the complexity that underlies the auditory function, which is regulated by a large number of genes and molecular pathways and is under the influence of environmental factors. These all act in concert to modify the severity and onset of disease; hearing problems may become apparent long after the harmful events have taken place, which further hinders the identification of the causing agents and prevents any efficacious treatment. Recent advancements in high-throughput screening approaches and single-cell transcriptomics, the development of novel tools to achieve the delivery of molecules into the inner ear, and the generation of new animal and human models of disease will all contribute to the identification of the molecular mechanisms leading to auditory disability and the elucidation of molecular targets for therapeutic strategies.

I am delighted to invite you to contribute to this Special Issue launched by the International Journal of Molecular Sciences on “Hearing Loss: Molecular Biological Insights”. The aim of this Special Issue is to provide a meeting point where scientists in the field of hearing can bring together recent discoveries on the molecular mechanisms leading to hearing disability, as well as report on the latest advancements in molecular biology-based diagnostics and therapeutics for hearing loss. This Special Issue should help us gain an overview of the work that is currently being conducted to decipher the molecular basis of hearing and the impressive achievements that are already being reached, as well as spark a debate on the most promising leads for the development of novel therapies against this devastating disorder.

In this Special Issue, original research articles and reviews are welcome. Research areas may include (but are not limited to) the following:

  • Genetic and molecular bases of hearing loss;
  • Epigenetics of hearing loss;
  • Molecular biological techniques to study the structure and function of the auditory organ;
  • Multi-omic approaches for the elucidation of the molecular mechanisms underlying hearing loss;
  • Molecular biology of inner ear development;
  • Animal models and human organoid-based models to study the molecular biology of inner ear development and function;
  • Molecular mechanisms involved in age-related or drug-induced hearing loss;
  • Application of CRISPR/Cas and other molecular biology techniques to analyze the effect of altering molecular processes on inner ear development and function;
  • Diagnostic markers of hearing dysfunction;
  • Molecular pathways associated with otoprotection or inner ear cell regeneration;
  • Intracochlear delivery of molecular agents as a possible therapy against hearing loss.

I am looking forward to receiving your valuable contributions.

Dr. María Beatriz Durán-Alonso
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

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Keywords

  • genetics
  • microRNA
  • epigenetics
  • CRISPR
  • multi-omics
  • presbycusis
  • ototoxicity
  • development

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Published Papers (1 paper)

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Review

14 pages, 774 KB  
Review
Audiovestibular Dysfunction in Hyper-IgE Syndrome: A Systematic Review of Characteristics, Pathophysiology, Diagnosis, and Management
by Jiann-Jy Chen, Chih-Wei Hsu, Brendon Stubbs, Tien-Yu Chen, Chih-Sung Liang, Yen-Wen Chen, Bing-Yan Zeng and Ping-Tao Tseng
Int. J. Mol. Sci. 2025, 26(20), 9932; https://doi.org/10.3390/ijms26209932 - 12 Oct 2025
Viewed by 264
Abstract
Hyper-IgE syndrome (HIES) is a rare genetic immunodeficiency characterized by elevated serum IgE levels and associated immune dysregulation, manifesting in recurrent infections, eczema, and skeletal abnormalities. Emerging evidence suggests a link between HIES and audiovestibular dysfunction, potentially mediated by IgE-driven inflammation in the [...] Read more.
Hyper-IgE syndrome (HIES) is a rare genetic immunodeficiency characterized by elevated serum IgE levels and associated immune dysregulation, manifesting in recurrent infections, eczema, and skeletal abnormalities. Emerging evidence suggests a link between HIES and audiovestibular dysfunction, potentially mediated by IgE-driven inflammation in the inner ear, which is not immunologically privileged. However, the nature of this association remains poorly understood. This systematic review synthesizes current evidence on the characteristics, pathophysiology, diagnostic approaches, and management of audiovestibular dysfunction in HIES patients. Literature searches across PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect (up to 6 August 2025) were conducted in accordance with PRISMA guidelines. Key findings indicate that HIES-related audiovestibular issues, including sensorineural hearing loss and vestibular impairment, may arise from IgE-mediated endolymphatic sac inflammation, leading to hydrops and hair cell damage. Diagnostic tools such as audiometry, electrocochleography, and allergen challenge tests show promise, with elevated IgE correlating with abnormal otoacoustic emissions and prolonged auditory brainstem response latencies. Treatment focuses on immunomodulation (e.g., corticosteroids, dupilumab) to mitigate IgE effects, though evidence is limited to case reports. A proposed schematic diagram illustrates pathophysiology, emphasizing IgE’s role in inner ear toxicity. Timely recognition and intervention may prevent progression to permanent hearing loss or vestibular disability, improving quality of life. Future research should explore genetic–immunologic mechanisms and prospective trials for targeted therapies. Trial registration: PROSPERO CRD420251120600. Full article
(This article belongs to the Special Issue Hearing Loss: Molecular Biological Insights, 2nd Edition)
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