Genetic Hearing Loss: A Comprehensive Review Hearing loss can be attributed to a variety of inherited and environmental noises (medications, infections, etc.). In recent decades, our improved understanding of genetics, molecular biology, and audiologic pathways has led to the discovery of a variety of Hereditary Hearing Impairments (HHI). HHI can be categorized as syndromic HL and ... Article
Article  |   November 01, 2015
Genetic Hearing Loss: A Comprehensive Review
Author Affiliations & Notes
  • Yarah Haidar
    Department of Otolaryngology – Head and Neck Surgery, University of California, Irvine, Irvine, CA
  • Jay Bhatt
    Department of Otolaryngology – Head and Neck Surgery, University of California, Irvine, Irvine, CA
  • Zachariah Chandy
    School of Medicine, University of California, Irvine, Irvine, CA
  • Kasra Ziai
    Division of Neurotology and Skull Base Surgery, Department of Otolaryngology – Head and Neck Surgery, University of California, Irvine, Irvine, CA
  • Hossein Mahboubi
    Department of Otolaryngology – Head and Neck Surgery, University of California, Irvine, Irvine, CA
  • Hamid R. Djalilian
    Director - Division of Neurotology and Skull Base Surgery Department of Otolaryngology – Head and Neck Surgery University of California, Irvine, Irvine, CA
  • Financial Disclosure: The authors have no financial interests related to the content of this article.
    Financial Disclosure: The authors have no financial interests related to the content of this article.×
  • Nonfinancial Disclosure: Yarah Haidar, Jay Bhatt, Zachariah Chandy, Kasra Ziai, Hossein Mahboubi, and Hamid R. Djalilian have previously published in the subject area.
    Nonfinancial Disclosure: Yarah Haidar, Jay Bhatt, Zachariah Chandy, Kasra Ziai, Hossein Mahboubi, and Hamid R. Djalilian have previously published in the subject area.×
Article Information
Hearing Disorders / Special Populations / Genetic & Congenital Disorders / Articles
Article   |   November 01, 2015
Genetic Hearing Loss: A Comprehensive Review
SIG 6 Perspectives on Hearing and Hearing Disorders: Research and Diagnostics, November 2015, Vol. 19, 64-76. doi:10.1044/hhd19.2.64
History: Received August 5, 2015 , Revised September 14, 2015 , Accepted September 22, 2015
SIG 6 Perspectives on Hearing and Hearing Disorders: Research and Diagnostics, November 2015, Vol. 19, 64-76. doi:10.1044/hhd19.2.64
History: Received August 5, 2015; Revised September 14, 2015; Accepted September 22, 2015

Hearing loss can be attributed to a variety of inherited and environmental noises (medications, infections, etc.). In recent decades, our improved understanding of genetics, molecular biology, and audiologic pathways has led to the discovery of a variety of Hereditary Hearing Impairments (HHI). HHI can be categorized as syndromic HL and non-syndromic HL, in which HL is associated with other pathophysiological manifestations. Currently, greater than 300 syndromic HLs are recognized, while more than 100 chromosomal loci and 40 genes are attributed for non-syndromic HLs. Improved understanding of the genes impaired in HL, including their structure and function, will soon lead to improvements in screening and possibly treatments. The following is a review of genetic HL where we discuss the definition, classifications, etiology, epidemiology, recognized syndromes, genetics, diagnostics, and screening methods of HL.

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