Nonsyndromic hearing loss

Nonsyndromic hearing loss is a partial or total loss of hearing that is not associated with other signs and symptoms. In contrast, syndromic hearing loss occurs with signs and symptoms affecting other parts of the body.Nonsyndromic hearing loss can be classified in several different ways. One common way is by the condition's pattern of inheritance: autosomal dominant (DFNA), autosomal recessive (DFNB), X-linked (DFNX), or mitochondrial (which does not have a special designation). Each of these types of hearing loss includes multiple subtypes. DFNA, DFNB, and DFNX subtypes are numbered in the order in which they were first described. For example, DFNA1 was the first type of autosomal dominant nonsyndromic hearing loss to be identified.The characteristics of nonsyndromic hearing loss vary among the different types. Hearing loss can affect one ear (unilateral) or both ears (bilateral). Degrees of hearing loss range from mild (difficulty understanding soft speech) to profound (inability to hear even very loud noises). The term "deafness" is often used to describe severe-to-profound hearing loss. Hearing loss can be stable, or it may be progressive, becoming more severe as a person gets older. Particular types of nonsyndromic hearing loss show distinctive patterns of hearing loss. For example, the loss may be more pronounced at high, middle, or low tones.Most forms of nonsyndromic hearing loss are described as sensorineural, which means they are associated with a permanent loss of hearing caused by damage to structures in the inner ear. The inner ear processes sound and sends the information to the brain in the form of electrical nerve impulses. Less commonly, nonsyndromic hearing loss is described as conductive, meaning it results from changes in the middle ear. The middle ear contains three tiny bones that help transfer sound from the eardrum to the inner ear. Some forms of nonsyndromic hearing loss, particularly a type called DFNX2, involve changes in both the inner ear and the middle ear. This combination is called mixed hearing loss.Depending on the type, nonsyndromic hearing loss can become apparent at any time from infancy to old age. Hearing loss that is present before a child learns to speak is classified as prelingual or congenital. Hearing loss that occurs after the development of speech is classified as postlingual.

Different Conditions

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Related ICD 10 COde
ICD 10 Code H90.1

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Acquired hearing impairment

Conductive hearing loss occurs when there is a problem conducting sound waves anywhere along the route through the outer ear, tympanic membrane (eardrum), or middle ear (ossicles), bilateral.

Organ Affected

Entire ear (body structure)

Abbreviated Terms

  • conduction deafness
  • Related ICD 10 COde
    ICD 10 Code H90.0

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    Disorders with hearing impairment

    Loss of hearing that occurs sometime the course of life and is not present at birth. The hearing impairment is sustained after the acquisition of language, which can occur due to disease, trauma, or as a side-effect of a medicine. Conductive hearing loss may occur as a result of a problem in the outer or middle ear such as an obstruction (cerumen, foreign body), damage to the osicles, middle ear infections, and/or perforation of the tympanic membrane. Sensorineural hearing loss is a type of hearing loss in which the root cause lies in the vestibulocochlear nerve (Cranial nerve VIII), the inner ear, or central processing centres of the brain. Mixed conductive and sensorineural hearing loss refers to a mix of both conductive and sensorineural hearing loss.

    Organ Affected

    Entire ear (body structure)

    Abbreviated Terms

  • hearing loss NOS
  • Also Known As

  • post-lingual hearing loss
  • Related ICD 10 COde
    ICD 10 Code H90

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