Table 1.

Clinical features of patients with cone-rod dystrophy.

Patient	Sex	Age at onset	Symptoms	Age at exam.	Visual acuity OD/OS	Fundus	Visual field	ERG OD/OS Scotopic dim blue Photopic single white flash Light adapted 30-Hz flickers
II:2	F		None	64	20/20 20/16	Mild attenuation of retinal vessels	NA	40 μV/23 μV 181 μV/175 μV 90 μV/94 μV
II:3	F	40	Nystagmus Night blindness Photophobia	70	20/40 20/32	Mild attenuation of retinal vessels. Macular atrophy	OD:relative 20° central scotoma OS:absolute 20-30° central scotoma Normal PVF on both eyes	124 μV/173 μV 56 μV/60 μV 46 μV/56 μV
III:1	F	32	Nystagmus No photophobia No night blindness	44	20/100 20/100	Severe macular atrophy Rare bone spicule-shaped pigment deposits	Absolute 30° central scotoma and normal PVF on both eyes	48 μV/35 μV 44 μV/42 μV 32 μV/41 μV
III:2	M		None	38	20/25 20/20	Normal	Normal	253 μV/275 μV 30 μV/41 μV 70 μV/84 μV
III:9	F	35	Nystagmus Night blindness Photophobia	45	20/25 20/25	Mild attenuation of retinal vessels	Normal	130 μV/121 μV 34 μV/46 μV 41 μV/40 μV
III:10	M	Early childhood	Night blindness Mild photophobia	38	20/32 20/32	Posterior pole atrophy Mild attenuation of retinal vessels	Absolute 10° central scotoma and normal PVF on both eyes	157 μV/160 μV 51 μV/45 μV 88 μV/77 μV
IV:1	M		Photophobia	1	NA	Mild attenuation of retinal vessels. Abnormal pigmentation of the macular area.	NA	NA
IV:4	M		No photophobia No night blindness	11	20/32 20/50	Posterior pole atrophy Attenuated retinal vessels	Normal	NA/139 μV NA/24 μV NA/66 μV
IV:10	M	Early childhood	Nystagmus Photophobia No night blindness	19	20/200 20/200	Moderate pallor of the optic discs, and macular atrophy	Relative 20° central scotoma and normal PVF on both eyes	91 μV/89 μV 20 μV/13 μV 39 μV/42 μV

PVF = Peripheral Visual Field

OD/OS = oculus dexter/oculus sinister

NA = Not available

Normal value ranges are:

Scotopic dim blue: 160 μV - 250 μV

Photopic single white: 70 μV - 150 μV

Light adapted 30-Hz flickers: > 110 μV