International Journal of Otolaryngology / 2013 / Article / Tab 3 / 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