Long-Term Effect of Enzyme Replacement Therapy with Fabry Disease

Table 2

Criteria for improvement. In Japan, outcome of therapy has been classified as follows.

(1) Healing	(1) When hearing level at 0.25, 0.5, 1, 2, 4 kHz returned to less than 20 dB.; (2) When hearing level returned to the same as that in the other side.
(2) Marked; recovery	When the value of the arithmetic means of the hearing levels at the five above-mentioned frequencies was greater than 30 dB.
(3) Recovery	When the value of the arithmetic means of the hearing levels at the five above-mentioned frequencies was less than 30 dB and greater than 10 dB.
(4) Unchangeable	When the value of the arithmetic means of the hearing levels at the five above-mentioned frequencies was less than 10 dB. (Including exacerbation)