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["Additional file 2. Clinical data from all atypical individuals and USH2 probands with USH2A deletions, duplications and c.7595-2144A>G."]
[{"$nulls":10},"Visual acuity (LogMAR)"]
["Diagnosis","Family (proband)a","Diagnosis","Age of onset of hearing loss","Hearing loss progression","Configuration of hearing lossb","Vestibular testing","Communication","Age of RP onset; presenting symptomc","Visual fields (degrees diameter)d","Right eye","Left eye","Ethnicity","Family history"]
["Atypical Usher",95,"Atypical_Usher","Congenital","No","Bilat ↓ slope 75-130","Bilat hypofunction","lipreading, speech, hearing aids","17; night blindness","10 (right eye only)",0.2,2.7,"Caucasian","None"]
[null,128,"Atypical_Usher","Adult","Yes","Bilat ↓ slope 20-100","L Canal paresis","speech","13; night blindness",15,0.3,0.3,"Caucasian","Two affected siblings. Maternal aunt with RP."]
[null,153,"Atypical_Usher","Congenital","No","Bilat ↓slope 14/40-100","Bilat hypofunction","speech,hearing aids","13; night blindness","35 + peripheral islands",0.3,0.32,"Caucasian","None"]
[null,223,"Atypical_Usher","Congenital","No","Bilat ↓ slope 80-130 (atypical configuration)","Not tested","speech, hearing aids","18; night blindness",20,0.4,0.26,"Caucasian","None"]
[null,236,"Atypical_Usher","Congenital","Reported","Bilat ↓ slope 45-130 (steep)","Normal","speech,hearing aids","25; night blindness","30 + peripheral islands",0.28000000000000003,0.98,"Caucasian","None"]
[null,423,"Atypical_Usher","Congenital","No","Bilat ↓ slope 90-130","Bilat vest failure","speech, hearing aids","10; visual field constriction. Unusual depigmented patches throughout midperipheral and peripheral retina not typically seen in RP","35  + peripheral islands",0.2,0.2,"Pakistani","None. Consanguineous."]
[null,426,"Atypical_Usher","Congenital","Reported rapid deterioration in 20s","Bilat ↓ slope 100-130 (profound across frequencies)","Bilat vest failure","British sign language","16; visual field constriction","50 + peripheral islands",0.1,0.1,"Caucasian","Son and husband have hearing loss."]
[null,501,"Atypical_Usher","Adult","Reported","Bilat ↓ slope 70-90","Bilat hypofunction","speech","42; night blindness. Significant retinal and RPE atrophy with no pigmentation","unable to perform",0.32,2.6,"Caucasian","None"]
[null,520,"Atypical_Usher","9 years","Yes now profound","Bilat ↓ slope 90-130","Bilat hypofunction","speech","28; night blindness. Anterior retinal pigmentation with telangiectatic vessels and localised retinal exudates",50,0.5,0.22,"Indian","None"]
[null,631,"Atypical_Usher","ND","No","Flat 70-80 (ski slope)","Normal","speech; mild LDs;","15; night blindness","20 (left eye only)",2.6,0.3,"Caucasian","None"]
[null,633,"Atypical_Usher","ND","No","Bilat ↓ slope","Not tested","speech, hearing aids","11; decreased central vision. Irregular atrophy and pigmentation affecting predominantly the posterior pole","unable to perform",1.6,2.6,"Caucasian","None"]
["USH2",24,"USH2","Congenital","Reported","Bilat ↓ slope 40-100","Normal","speech,hearing aids","14; night blindness",20,0.5,0.5,"Caucasian","(see figure 1 for family trees)"]
[null,"46","USH2","Congenital","Reported","Bilat ↓ slope 30-80","Normal","speech, hearing aids","28; night blindness",60,0.2,0.1,"Caucasian"]
[null,"148","USH2","Congenital","No","Bilat ↓ slope 35-70","Normal","speech","9; night blindness","not tested",0.2,0.16,"Caucasian"]
[null,"151","USH2","Congenital","Reported","Bilat ↓ slope 45-70","Lcanal paresis","speech, hearing aids","21; night blindness","40 + peripheral islands",0.06,0.14000000000000001,"Caucasian"]
[null,283,"USH2","Congenital","No","Bilat ↓ slope 40-80","Normal","speech, hearing aids","15; night blindness",120,-0.12,-0.12,"Caucasian"]
[null,"309","USH2","Congenital","ND","ND","ND","speech","7; night blindness",130,0.18,0.18,"Caucasian"]
[null,657,"USH2","Congenital","No ","Bilat ↓ slope 55-80/90","L canal paresis","speech, hearing aids","11; night blindness","unable to perform",0.96,2.7,"Caucasian"]
[null,707,"USH2","Congenital","Reported","Bilat ↓ slope 40-100","Normal","speech","12; night blindness",20,0.26,0.26,"Caucasian"]
[]
["a Clinical data given for the affected proband from each family only"]
["b All of the cases (except the proband from family 631 which was plateau) showed virtually symmetrical down sloping hearing loss;  dBHL levels from 250Hz to 8kHz are listed ie. 'Bilat ↓ slope 20-100' means 20dBHL at 250Hz decreasing to 100dBHL at 8kHz"]
["c Fundus appearance was typical of Usher syndrome in all cases except the probands from families 501, 520 and 633."]
["d Approximate visual field size averaged between the two eyes"]
[]
["ND Means no data."]

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