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. 2010 Jan 8;86(1):65–71. doi: 10.1016/j.ajhg.2009.11.015

Table 1.

Summary of Clinical Data for Some of the Affected Individuals of Chinese Family GZ-Z052

Patient Sex Age (Yrs)
Use of Amino-Glycosides Hearing Test PTA (dB)
Tinnitus Uric Acid Level (μMOL/L)
Visual Impairment Test Peripheral Neuropathy Test
At Testing At Onset Left Ear Right Ear Plasmaa Urineb
IV-1 Male 50 10 No 106 106 No 231 3846 Normal Normal
IV-9 Male 36 14 No 88 96 No NT NT NT NT
IV-13 Male 30 8 No 69 71 Yes 289 4148 Normal Normal
IV-21 Male 31 6 Yes 89 84 Yes 399 2785 Normal Normal
IV-34 Male 28 5 No 82 87 No 322 3138 Normal Normal
IV-36 Male 24 8 No 85 78 Yes 380 4624 Normal Normal
III-2 Female 71 50 No 94 113 No 288 3187 Normal Normal
III-5 Female 56 53 No 57 33 Yes 156 3541 Normal Normal
III-11 Female 64 55 No 21 27 No 310 3166 Normal Normal
III-15 Female 57 50 No 41 60 Yes NT NT NT NT
III-17 Female 55 51 No 63 22 No 304 3535 Normal Normal
III-25 Female 53 40 No 31 61 No NT NT NT NT
III-29 Female 47 45 No 33 70 No 298 5078 Normal Normal

NT, not tested; PTA, pure tone average.

a

The normal range is 90-420 μmol/L.

b

The normal range is 2400-5900 μmol/L.