Proband 52, a 48-year-old female with prelingual onset, bilateral, profound, steeply sloping high-frequency HI with type A tympanogram and bilateral reduced vision, nyctalopia, and photophobia starting at 5 years of age, showed two missense variants in ADGRV1, c.2261T>C p.(Val754Ala) and c.10878A>C p.(Lys3626Asn) (Table 1). The gene discussed is ADGRV1; the disease is Photophobia.