Table 5

Patients with Usher syndrome 2—clinical information

No	Age/sex	Age/diag deafness	Audiogram	Vestibular function	Age diag RP (years)	Age visual symptom (years)	VA	ERG (mV)	Cat	Field (°)	Pigment degen
1^*	34/M	18 months	Sloping mod/sev	+	35	32	6/6	<75	no	35	Moderate
5^*	71/F	2 years	Sloping profound	++	17	15	6/9	ext	no	10	Moderate
7^*	39/F	6 years	Sloping mod/sev	neg	21	10	6/9	ext	no	15	Moderate
9	56/M	Infant	Sloping mod/sev	NP	38	20	6/12	ext	early PSLO	10	Moderate
13^*	36/M	Infant	Sloping mod/sev	++	25	15	6/9	ext	no	10	Extensive
15	16/F	5 years	Sloping mod/sev	NP	13	none	6/6	<75	no	full	Normal fundi
17^*	18/M	Infant	Sloping mod/sev	NP	14	12	6/12	<75	no	moderate constriction	Moderate
19^*	24/M	Infant	Sloping mod/sev	+	17	14	6/9	ext	no	moderate constriction	Moderate
20^*	59/F	Infant	Sloping mod/sev	++	58	54	6/18	ext	mod PSLO	10	Moderate
21^*	33/F	6 years	Sloping mod/sev	++	17	17	6/12	ext	no	20	Moderate
26^*	30/M	15 months	Sloping mod/sev	++	30	22	6/6	<75	no	moderate constriction	Retinitis punctata albescens
29^*	38/F	Infant	Sloping mod/sev	++	30	30	6/5	<75	no	15	Moderate
33^*	45/F	2 years	Sloping mod/sev	++	43	43	6/18	<75	pseud opha kos	45	Mild
35	30/M	5 years	Sloping mod/sev	NP	10	10	6/12	<75	mild PSLO	10	Moderate

RP = retinitis pigmentosa; NP = not performed; PSLO = posterior subcapsular lens opacities; ext = extinguished; Cat = cataract; diag = diagnosis; pigment degen = pigmentary degeneration; ^*clinically reassessed.