TABLE 1.

Clinical features of the 112 EVA families

Demographics	Number of patients (%), n = 117
Gender
Male	70 (59.83%)
Female	47 (40.17%)
Test age (years)
<10	91 (77.78%)
≥10	26 (22.22%)
Onset age (years)
0–2	84 (71.79%)
>2	33 (28.21%)
Degree of HL
Profound	51 (43.59%)
Severe	53 (45.30%)
Moderate	11 (9.40%)
Mild	2 (1.71%)
Evolution of HL
Stable	38 (32.48%)
Progressive	36 (30.77%)
Fluctuating	43 (36.75%)