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Hereditary deafness



Hereditary deafness Causes


Syndromic hearing impairment is linked with malformations of the external ear or other organs or with medical problems involving other organ systems. Nonsyndromic hearing impairment has no associated visible abnormalities of the external ear, nor are there any related medical problems; however, it can be linked with abnormalities of the middle ear and/or inner ear


Hereditary deafness Definition


Hereditary deafness is hearing loss that is passed down from parents to their children. This type of hearing loss may be inherited from one or both parents who may or may not possess a loss of hearing themselves.


Hereditary deafness Diagnosis


Genetic forms of hearing loss should be distinguished from acquired (or non-genetic) causes of hearing loss. The genetic forms of hearing loss are diagnosed by audiologic, otologic, and physical examination, family history, ancillary testing (such as CT examination of the temporal bone), and molecular genetic testing. Molecular genetic tests are available for several types of syndromic and nonsyndromic deafness, often only on a research basis.


Hereditary deafness Symptoms and Signs


With hereditary deafness, prelingual hearing loss is present before speech develops. All congenital hearing loss is prelingual, but not all prelingual hearing loss is necessarily congenital. On the other hand, postlingual hearing loss occurs after the development of normal speech.


Hereditary deafness Treatment


Ideally, the team evaluating and treating the deaf individual should be made up of an otolaryngologist with expertise in the management of early childhood otologic disorders, an audiologist experienced in the assessment of hearing loss in children, a clinical geneticist, and a pediatrician. The expertise of an educator of the deaf, a neurologist, and a pediatric ophthalmologist may also be needed


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