Table 5.
Analysis of studies available for aggregate patient data.
| Author | N˚ ARAS | ESRD | SNHL | OT | |||
|---|---|---|---|---|---|---|---|
| N˚ | N˚ (%) | Median Age | N˚ (%) | Median Age | N˚ (%) | Median Age | |
| Chen, 2018 ‡ [8] | 4 | n/a | 23 | 3/4 (75%) | n/a | 4/4 (100%) | n/a |
| Savige, 2017 ‡ [9] | 13 | 12/13 (92%) | n/a | 12/13 (92%) | n/a | 13/13 (100%) | n/a |
| Nabais, 2015 [10] | 15 | 15/15 (100%) | 23 | 9/10 (90%) | 32 | 3/9 (30%) | 30 |
| Wang, 2014 [11] | 14 | n/a | n/a | 6/9 (67%) | n/a | 0/8 (0%) | n/a |
| Wang, 2014 ‡ [12] | 15 | 14/15 (93%) | 27.2 | 15/15 (100%) | n/a | 13/15 (87%) | n/a |
| Yao, 2012 [13] | 24 | n/a | n/a | 8/24 (30%) | n/a | 7/12 (58%) | n/a |
| Temme, 2012 [14] | 29 | 3/29 | n/a | n/a | n/a | n/a | n/a |
| Artuso, 2012 [15] | 2 | 1/2 (50) | n/a | n/a | n/a | n/a | n/a |
| Pierides, 2009 [16] | 42 | 18/42 (43%) | n/a | n/a | n/a | n/a | n/a |
| Shaw, 2007 [17] | 7 | 6/6 (100%) | 25 | 7/7 (100%) | 32 | 7/7 (100%) | 32 |
| Wei, 2006 [18] | 13 | 3/13 (23%) | 17 | 6/12 (50%) | 22 | 3/12 (25%) | 26 |
| Dagher, 2002 ‡ [19] | 11 | 8/11 (72%) | 24 | 10/11 (91%) | n/a | 10/11 (91%) | n/a |
| Heidet, 2000 [20] | 60 | 44/60 (73%) | 22 | 27/35 (77%) | n/a | 16/26 (62%) | n/a |
| Torra, 1999 [21] | 5 | 2/5 (40%) | 33 | 4/5 (80%) | 13.5 | 0/5 (0%) | n/a |
| Boye, 1998 [22] | 10 | 8/31 (26%) | n/a | n/a | n/a | n/a | n/a |
| Pooled data | 264 | 134/242 (55%) | 23.5 | 107/145 (74%) | 27 | 76/115 (66%) | 30 |
n/a: not available for information; ESRD, end-stage renal disease; SNHL, sensorineural hearing loss; OT, ocular abnormalities. ‡ These studies were performed on patients (and their family members) who had ocular abnormalities and visited department of ophthalmology.