Table 1. Phenotypic Characteristics of HSAN 1 Subjects with Dementia and Sensorineural Hearing Loss.

Patient	Symptom Onset (yrs)	Age (yr) First Examined	Gender	First Symptom(s)	Extremity Sensory Loss	Distal Limb Weakness	Cerebellar ataxia	Dementia Onset(yrs)	Death (yrs)
K1 V7	30	59	F	Hearing loss	Yes*	No	No	40s	61
K1 V-12	35	48	M	Loss of sensation Hearing loss	Yes	No	No	42	Alive
K1 V-14	30	44	F	Hearing loss	Yes	No	No	41	-
K1 V-16	32	38	F	Loss of sensation Hearing loss	Yes	Yes	No	30s	Alive
K1 VI-11	20s	30	F	Hearing loss	Yes	No	No	30s	Alive
K1 VI-23	35	45	M	Hearing loss	Yes*	No	No	40	51
K1 VI-30	20s	30	M	Loss of sensation	Yes	No	No	30s	Alive
K1 V1-33	35	43	M	Hearing loss	Yes*	Yes	No	30s	46
K1 VI-36	35	42	M	Hearing loss	Yes*	No	No	40s	48
K1 VII 13	20s	18	F	Hearing loss	Yes	No	No	30s	Alive
K1 VII 15	20s	20	F	Loss of sensation	Yes	No	No	40s	Alive
K2 II-2	28	32	F	Hearing loss	Yes*	No	No	30s	46
K2 III-1	27	38	M	Hearing loss	Yes*	No	Yes	30s	Alive
K2 III-3	16	39	F	Hearing loss	Yes*	No	Yes	30s	Alive
K3 IV-16	35	47	F	Hearing loss	Yes*	No	No	40s	55
K3 IV-17	30s	40	M	Loss of sensation Hearing loss	Yes	No	Yes	40s	47
K3 V-1	30s	40	F	Loss of sensation Hearing loss	Yes*	No	Yes	40s	44
K4 III-3	30s	40s	M	Loss of sensation Hearing loss	Yes	No	No	30s	Alive

^*Foot ulcers; -- Unavailable; K kindred