Congenital deafness

Congenital deafness
Congenital deafness may present at birth and it is the aim of newborn hearing screening to identify these babies at an early age. Some congenital conditions may not become evident until the child is older.

Congenital deafness
Caused by one of two changes in a gene called Connexin 26. About one in 21 individuals of Ashkenazi Jewish descent has one of the two mutations.
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Congenital deafness similarly may or may not be genetic. For example, it may be associated with a white forelock, and be caused by a genetic disease called Waardenburg syndrome. In fact, more than half of congenital hearing loss is inherited.

Down Syndrome ... congenital deafness, conductive hearing loss
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radiation therapy Confrontation Congenital Congenital achromatopsia, Congenital anemia Congenital anomaly Congenital arthrogryposis Congenital bilateral absence of vas deferens Congenital central hypoventilation syndrome Congenital deafness ...

Aminoglycosides cross the placenta and there have been several reports of total irreversible, bilateral congenital deafness in children whose mothers received streptomycin during pregnancy.

Other factors that are thought to cause congenital deafness include exposure to methyl mercury and drugs such as quinine.

However, primary features often include distinctive facial abnormalities; unusually diminished coloration (pigmentation) of the hair, the skin, and/or the iris of both eyes (irides); and/or congenital deafness.

Depending upon whether someone is born without hearing (congenital deafness) or loses hearing later in life (after learning to hear and speak, which is known as post-lingual deafness), ...

Hearing--normal structure and function of the auditory system; diseases and disorders of the ear including hereditary and other forms of congenital deafness such as Waardenburg syndrome and Usher syndrome, sudden deafness, ...

When associated with autosomal recessive inheritance and congenital deafness, it is called the jervell-lange nielsen syndrome.
When associated with autosomal dominant inheritance and no deafness is present, it is called the romano-ward syndrome.

Significant quality of life improvements have been demonstrated for children with congenital deafness who receive cochlear implants (Schorr, Roth, & Fox, 2009), ...

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In rare patients with 2 abnormal copies of the genetic abnormality (particularly LQT1), the disorder is associated with congenital deafness and, in the past, was referred to as the Jervell and Lange-Nielsen syndrome.

It is estimated that WS affects one out of 42,000 people. Researchers believe that WS is responsible for two to three percent of congenital deafness (deafness at birth) cases. The disorder appears to affect males and females equally.

Hearing is impaired somewhat from birth and there is some worsening with age but the profound congenital deafness found in type I does not occur. Hence, many individuals develop speech. Balance and coordination are usually normal.

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See also: Deafness, Symptom, Hearing Loss, Fever, Injury