Table 1.

Clinical and Demographic Data Including Longitudinal Observations in Six Probands With CHED Variably Associated With Hearing Impairment

Family/ ID	Age, y*	BCVA		CCT, μm		Other Information	Hearing Impairment
		RE	LE	RE	LE
C2/II:1	5	0.5	0.4	1098	1078	LE convergent strabismus	Y - onset at 5 y, mild
C3/II:1	70	0.01	0	UA	PK	LE PK at 10 y, vision lost after injury at 36 y	Y
C4/II:2	7	0.02†	0.03†	UA	UA	Horizontal nystagmus RE PK at 7 y LE PK at 8.5 y, rePK	Y - onset at 14 y, mild
	35	HM	0.05	PK	PK
C5/II:2	10	0.4	0.4	1032	1032	Visual impairment noticed at 5 y	Y - onset at 6 y, perceptive, mild nonprogressive, hearing aid since 8.5 y
						Good near vision
B1/II:1	3	0.54	0.64	1046	1036	Nil	N
B2/II:1	6	0.25†	0.25†	UA	UA	RE exotropia RE PK at 10 y, rePK, rePK+cataract LE PK at 7 y, DMEK+cataract	N
	10	0.05†	0.66	UA	PK
	47	0.66	0.66	PK	PK+ DMEK

CCT, central corneal thickness; DMEK, Descemet membrane endothelial keratoplasty; HM, hand movement; LE, left eye; N, no; PK, penetrating keratoplasty; RE, right eye; rePK, repeated penetrating keratoplasty; UA, unavailable data; Y, yes.

* At examination.

^†Prior to PK.