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Genetics and Age-Related Hearing Loss

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Free Radicals in ENT Pathology

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Abstract

Age-related hearing loss—presbycusis—comprises the top communication disorder and neurodegenerative condition of our aged population, and its management is a tremendous burden on our healthcare system. There are currently no biomedical treatments to slow its progression, or reverse its symptoms, which can reduce professional productivity, family communication and relationships, and sometimes lead to depression or other psychosocial deficits. Advances in our knowledge of the biological underpinnings, including the genetics of presbycusis, can pave the way for novel biotherapeutic treatments in the future. We have learned much from utilizing genetically engineered animal models, including inbred and knockout mouse strains, to advance our knowledge of the biological mechanisms, at systems, cellular, genetic and molecular levels, as summarized in the current chapter. Unfortunately, much less is known about human genes underlying presbycusis, with only one gene strongly linked to age-related hearing loss so far.

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Abbreviations

ABR:

Auditory brainstem response

ARHL:

Age-related hearing loss, presbycusis

AVCN:

Anteroventral cochlear nucleus

DCN:

Dorsal cochlear nucleus

PCR:

Polymerase chain reaction quantitative measure of gene expression

PTS:

Permanent threshold shift, permanent hearing loss

PVCN:

Posteroventral cochlear nucleus

ROS:

Reactive oxygen species, free radicals

SGN:

Spiral ganglion neuron, auditory nerve fiber

SNP:

Single nucleotide polymorphism

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Acknowledgments

This work was supported by NIH Grant P01 AG009524 from the National Institute on Aging. The authors thank Drs. Joseph Walton, Xiaoxia Zhu, Bo Ding, Mary D’Souza, Sherif Tadros, and Susan Frisina R.N. for collaborative contributions leading up to the present report, and Shannon Salvog for project support.

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Authors and Affiliations

  1. Departments of Chemical & Biomedical Engineering and Communication Sciences & Disorders, Global Center for Hearing & Speech Research, University of South Florida—Research Park, 3802 Spectrum Blvd., Suite 210, Tampa, FL, 33612, USA

    Robert D. Frisina & D. Robert Frisina

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  1. Robert D. Frisina
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  2. D. Robert Frisina
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Correspondence to Robert D. Frisina .

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Editors and Affiliations

  1. Cochlear Signaling & Tissue Engin., University of Michigan Kresge Hearing Research Institute, Ann Arbor, Michigan, USA

    Josef Miller

  2. Dept of Speech , Lang & Hearing Sci, Univ of Florida Health Sciences Center, Gainesville, Florida, USA

    Colleen G. Le Prell

  3. Div . of Otolaryngology Head & Neck Surgery, SIU School of Medicine, Springfield, Illinois, USA

    Leonard Rybak

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Frisina, R.D., Frisina, D.R. (2015). Genetics and Age-Related Hearing Loss. In: Miller, J., Le Prell, C., Rybak, L. (eds) Free Radicals in ENT Pathology. Oxidative Stress in Applied Basic Research and Clinical Practice. Humana Press, Cham. https://doi.org/10.1007/978-3-319-13473-4_14

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