deafness autosomal dominant type 36

deafness autosomal dominant type 36

A bilateral, autosomal dominant form (OMIM:606705) form of sensorineural deafness, which is due to defects in the neural receptors of the inner ear, the nerve pathways to the brain or auditory cortex, that begins at 5–10 years of age and progresses to profound deafness within 10–15 years.

Molecular pathology
Defects of TMC1, which encodes a protein that may be required for normal cochlear hair cell function, cause deafness autosomal dominant type 36.
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