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Disorders and syndromes which cause hearing loss. Info on Genetics • Alport Syndrome • Auditory Neuropathy •
Auditory Processing Disorders • CHARGE Syndrome • Connexion 26 • Congenital Ear Problems •
Large Vestibular Aqueduct General Information
Alport Syndrome--Hearing loss associated with a kidney disorder. Auditory Neuropathy--Term used to describe a problem with the auditory nerves. These nerves carry sound to the brain.
Auditory Processing Disorder--The mechanisms of hearing are intact, but the brain is unable to make sense of the sounds it is hearing.
CHARGE Syndrome--CHARGE Syndrome refers to children with a specific set of birth defects. "CHARGE" originally came from the first letter of some of the most common features seen in these children: C = coloboma, H = heart defects, A = atresia of the choanae, R = retardation of growth and development, G = genital and urinary abnormalities, E = ear abnormalities and/or hearing loss. The diagnosis of CHARGE is based on finding several of these and possibly other features in a child. The diagnosis should be made by a medical geneticist who has ruled out other disorders with overlapping findings
Connexion 26--genetic mutation that results in hearing loss.
Congenital Ear Problems (including cytomegolovirus) Large Vestibular Aqueduct Syndrome (LVAS, EVA)--the vestibulae of some individuals can be enlarged, causing progressive and sometimes, fluctuating hearing losses.
Long QT Syndrome--Deafness that occurs with an irregular heart rhythm. Pendred Syndrome--A progressive sensorineural hearing loss associated with thyroid dysfunction. (Genetic, recessive.) Majority of persons with Pendred have enlarged vestibular aqueducts which can cause fluctuations in hearing, and/or Mondini Malformation of the cochlea, (the cochlea does not make the standard 2 and 1/2 turns)
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