International Journal of Otolaryngology

Clinical Study

Long-Term Effect of Enzyme Replacement Therapy with Fabry Disease

Table 3

Results along a time series. Thirteen of the 17 patients had tinnitus, while 9 had vertigo. SSNHL was found in 10 ears of 6 patients. Repetitive SSNHL was found in 3 of the 6 patients with SSNHL. The incidence of SSNHL tended to increase over the follow-up period, and all patients with renal dysfunction had SSNHL.


ID	Sex	Age	Fabry type	Tinnitus	Vertigo	SSNHL	Time to onset	SSNHL outcome	Hearing outcome	Hearing change	Renal function	Heart function	Period	Before ERT (dB)	After ERT (dB)

1	F	40	hetero	−	−	—	—	—	—	—	—	—	8 M	8.0	6.0
						—	—	—	—	—				6.0	5.0
2	F	52	hetero	+	−	—	—	—	—	—	—	—	12 M	8.0	10.0
						—	—	—	—	—				10.0	12.0
3	F	31	hetero	+	−	—	—	—	—	—	—	—	12 M	14.0	17.0
						—	—	—	—	—				14.0	15.0
4	M	55	heart	+	+	—	—	—	—	—	—	—	17 M	8.0	11.0
						—	—	—	Worth	Slow				6.0	13.0
5	M	14	classical	−	−	—	—	—	—	—	—	—	26 M	6.0	9.0
						—	—	—	—	—				6.0	9.0
6	M	38	classical	+	−	—	—	—	Worth	Slow	—	—	27 M	6.0	24.0
						—	—	—	Worth	Slow				9.0	15.0
7	F	23	hetero	+	+	—	—	—	—	—	—	—	28 M	8.0	11.0
						—	—	—	—	—				11.0	10.0
8	F	30	hetero	+	+	—	—	—	—	—	—	—	36 M	5.0	10.0
						—	—	—	—	—				5.0	9.0
9	M	25	classical	−	+	—	—	—	—	—	—	Worth	43 M	9.0	8.0
						—	—	—	—	—				11.0	8.0
10	F	47	hetero	+	+	Grade 1	54 M	Healing	—	Sudden	—	Worth	58 M	10.0	11.0
						Grade 1	41 M	Healing	—	Sudden				10.0	9.0
11	F	41	hetero	+	+	—	—	—	Worth	Sudden	—	—	67 M	12.0	18.0
						—	—	—	—	—				12.0	15.0
12	F	34	hetero	+	−	—	—	—	—	—	—	—	67 M	4.0	9.0
						Grade 1	64 M	Healing	—	Sudden				4.0	8.0
13	F	49	hetero	+	+	Grade 1/Grade 3	20/67 M	Healing/Healing	Worth	Sudden	Worth	Worth	68 M	10.0	21.0
						—	—	—	—	—				14.0	14.0
14	M	35	classical	+	−	Grade 1	48 M	Healing	—	Sudden	—	—	68 M	6.0	9.0
						Grade 1	60 M	Healing	—	Sudden				6.0	11.0
15	M	30	classical	+	+	Grade 1	66 M	Healing	—	Sudden	Worth	Worth	77 M	8.0	9.0
						—	—	—	—	—				9.0	11.0
16	M	39	classical	+	+	—	—	—	Worth	Slow	Worth	—	88 M	8.0	15.0
						Grade 1	84 M	No change	Worth	Sudden				7.0	21.0
17	M	12	classical	−	−	—	—	—	—	—	—	—	90 M	2.0	3.0
						Grade 1	90 M	Healing	Stable	Sudden				5.0	5.0