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Updated: May 31, 2026
06:12
Long-term Sensory Conflict in Freely Behaving Mice
Published on: February 20, 2019
Mouse models and translational research progress of hereditary vestibular dysfunction.
Summary
Mouse models are crucial for understanding hereditary vestibular dysfunctions (HVDs), aiding in the development of diagnostic biomarkers and therapeutic strategies for these complex genetic conditions.
Area of Science:
- Genetics and Molecular Biology
- Neuroscience and Sensory Systems
- Translational Medicine
Background:
- Hereditary vestibular dysfunctions (HVDs) are genetic disorders causing vestibular dysfunction, often with hearing loss.
- Current diagnostic methods are limited, and effective treatments for HVDs are lacking.
- Mouse models are essential for studying HVDs, understanding disease mechanisms, and developing therapies.
Purpose of the Study:
- To review the current state and applications of mouse models in hereditary vestibular dysfunction research.
- To analyze the role of various mouse models in elucidating disease mechanisms and advancing treatment strategies.
- To highlight the potential of emerging technologies like CRISPR-Cas9 and multi-omics in HVD research.
Main Methods:
- Systematic literature review of English and Chinese articles (2000-2026) from major databases (PubMed, Web of Science, Embase, CNKI).
- Keywords included 'hereditary vestibular dysfunction,' 'mouse models,' 'gene therapy,' 'CRISPR-Cas9,' and 'Usher syndrome.'
- Analysis focused on spontaneous mutation models, genetic engineering models, CRISPR-Cas9 models, and IMPC knockout models.
Main Results:
- Spontaneous and genetically engineered mouse models have revealed key molecular mechanisms in HVDs, including cytoskeletal roles and ion channel function.
- CRISPR-Cas9 technology has significantly improved the efficiency and precision of HVD mouse model creation.
- Therapeutic interventions like AAV-mediated gene therapy, CRISPR gene repair, and novel drugs show promise in preclinical studies.
- Multi-omics approaches have identified potential diagnostic biomarkers for HVDs.
Conclusions:
- Mouse models are indispensable for advancing the understanding and treatment of hereditary vestibular dysfunctions.
- Despite challenges in clinical translation (species differences, delivery efficiency), technological advancements offer hope for personalized HVD diagnosis and treatment.
- Continued development in gene editing, nanodelivery, and multi-omics will drive progress in managing HVDs.
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