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.

IDSexAgeFabry
type
TinnitusVertigoSSNHLTime to
onset
SSNHL
outcome
Hearing
outcome
Hearing
change
Renal
function
Heart
function
PeriodBefore
ERT (dB)
After
ERT (dB)

1F40 hetero8 M8.0 6.0
6.0 5.0
2F52 hetero+12 M8.0 10.0
10.0 12.0
3F31 hetero+12 M14.0 17.0
14.0 15.0
4M55 heart++17 M8.0 11.0
WorthSlow6.0 13.0
5M14 classical26 M6.0 9.0
6.0 9.0
6M38 classical+WorthSlow27 M6.0 24.0
WorthSlow9.0 15.0
7F23 hetero++28 M8.0 11.0
11.0 10.0
8F30 hetero++36 M5.0 10.0
5.0 9.0
9M25 classical+ Worth43 M9.0 8.0
11.0 8.0
10F47 hetero++Grade 154 MHealingSudden Worth58 M10.0 11.0
Grade 141 MHealingSudden10.0 9.0
11F41 hetero++WorthSudden67 M12.0 18.0
12.0 15.0
12F34 hetero+67 M4.0 9.0
Grade 164 MHealingSudden4.0 8.0
13F49 hetero++Grade 1/Grade 320/67 MHealing/HealingWorthSudden Worth Worth68 M10.0 21.0
14.0 14.0
14M35 classical+Grade 148 MHealingSudden68 M6.0 9.0
Grade 160 MHealingSudden6.0 11.0
15M30 classical++Grade 166 MHealingSudden Worth Worth77 M8.0 9.0
9.0 11.0
16M39 classical++WorthSlow Worth88 M8.0 15.0
Grade 184 MNo changeWorthSudden7.0 21.0
17M12 classical90 M2.0 3.0
Grade 190 MHealingStableSudden5.0 5.0

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