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Hearing Loss, Central

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Definition: Hearing loss due to disease of the AUDITORY PATHWAYS (in the CENTRAL NERVOUS SYSTEM) which originate in the COCHLEAR NUCLEI of the PONS and then ascend bilaterally to the MIDBRAIN, the THALAMUS, and then the AUDITORY CORTEX in the TEMPORAL LOBE. Bilateral lesions of the auditory pathways are usually required to cause central hearing loss. Cortical deafness refers to loss of hearing due to bilateral auditory cortex lesions. Unilateral BRAIN STEM lesions involving the cochlear nuclei may result in unilateral hearing loss.  coord IM with VESTIBULOCOCHLEAR NERVE DISEASES (IM) for hearing loss due to cochlear nerve dis   
See Also Vestibulocochlear Nerve Diseases 		Other names Deafness, Cortical; Cortical Deafness; Central Hearing Loss
 
SubstanceCAS Registry & nameCategoriesSource
Auditory neuropathy  0   *Hearing Loss, Central.
Deafness, neurosensory, autosomal recessive 47  0   *Hearing Loss, Central.
Deafness, autosomal recessive 55  0   *Hearing Loss, Central.
Deafness, autosomal recessive 51  0   *Hearing Loss, Central.
Deafness, autosomal dominant nonsyndromic sensorineural 53  0   *Hearing Loss, Central.
Deafness, autosomal dominant nonsyndromic sensorineural 3  0   *Hearing Loss, Central.
Jensen syndrome  0   *Dementia *Hearing Loss, Central *Optic Atrophy.
Mitochondrial myopathy with lactic acidosis  0   *Acidosis, Lactic *Growth Disorders *Hearing Loss, Central *Mitochondrial Myopathies.
Groll Hirschowitz syndrome  0   *Cachexia *Demyelinating Diseases *Hearing Loss, Central.
Santos Mateus Leal syndrome  0   *Hearing Loss, Central *Hirschsprung Disease *Hypertelorism *Polydactyly.
Ruzicka Goerz Anton syndrome  0   *Cervical Rib Syndrome *Hearing Loss, Central *Ichthyosis *Intellectual Disability *Thyroid Neoplasms *Limb Deformities, Congenital.
Optic atrophy polyneuropathy deafness  0   *Hearing Loss, Central *Polyneuropathies *Optic Atrophies, Hereditary *Muscular Disorders, Atrophic.
Deafness nephritis ano rectal malformation  0   *Abnormalities, Multiple *Anus, Imperforate *Hearing Loss, Central *Nephritis, Hereditary Anal Canal/abnormalities Rectum/abnormalities.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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