Usher Syndrome Type 1F is characterized by profound hearing loss which is present at birth, and adolescent-onset retinitis pigmentosa, a disorder that significantly impairs vision. Affected individuals usually require cochlear implants to help with speech development. Balance is often impaired, and visual acuity typically begins to decline around age 10.

A mutation in the PCDH15 gene, located on chromosome 10, has a carrier frequency of 1 in 141 in the Ashkenazi Jewish population. Currently, there is no treatment for Usher Syndrome Type 1F