What Is Usher Syndrome?
7. Hearing loss
Most Usher syndrome cases begin with hearing difficulty noted when the child is a baby. Hearing screening for newborns is standard practice. In rare cases, this symptom can develop when the child has reached adolescence. This initial symptom is caused by the abnormal development of sound receptor cells in the inner ear.
A loss of balance is another common symptom of the condition, which is caused by further abnormal development of vestibular hair cells. These cells are responsible for head movement and sensing gravity. Balance issues are most obvious in type 1 cases and are tested through an electronystagmogram (ENG). A hearing valuation is often conducted to determine the severity of hearing loss. The test uses loud sounds at different frequencies to determine what point the person begins to hear.
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