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Sensorineural Hearing Loss-Congenital-Genetics

Synonyms

Congenital hearing loss; Hereditary hearing loss

Definitions

  1. Sensorineural hearing loss: hearing loss due to disease or pathology of the inner ear (cochlea) or to the nerve pathways from the inner ear to the brain
  2. Congenital hearing loss: hearing loss present at birth

Hereditary hearing loss (HHL) is caused by a genetic defect or by the interaction of multiple disordered genes. Almost half of profound congenital hearing loss is due to genetic factors. Its presence may be suggested by a family history of hearing loss or if the hearing loss is accompanied by other syndromic features or abnormalities. HHL is suspected when there is no apparent acquired cause of hearing loss identified. This entry provides a review of hereditary sensorineural hearing loss. Genetic conditions responsible for conductive hearing loss are not covered here. The entry reviews the basics of genetic inheritance. The “classic” and well-characterized