Table 1.
Demographics of Patients Subdivided by the Age at Which They First Displayed NF2-Related Symptoms
| Age of First NF2-Related Symptoms (Y) | P Value | P Value for Trend (Excluding Asymptomatic) | |||||
|---|---|---|---|---|---|---|---|
| Asymptomatic | <16 | 16-25 | 26-39 | ≥40 | |||
| Number | 67 | 91 | 66 | 64 | 65 | n/a | n/a |
| Truncating mutation, n (%) | 7 (10.5) | 38 (41.8) | 17 (25.8) | 2 (3.1) | 1 (1.5) | <.001 | <.001 |
| Died, n (%) | 2 (3.0) | 23 (25.3) | 13 (19.7) | 10 (15.6) | 12 (18.5) | .006 | .207 |
| Died within 15 y of diagnosis, n (%) | 1a (1.5) | 11 (12.1) | 6 (9.1) | 5 (7.8) | 7 (10.8) | .182 | .678 |
| Mean age at death, y | 33.1 | 31.9 | 37.5 | 55.3 | 72.7 | <.001 | <.001 |
| Most common presenting symptoms, n (%) | |||||||
| 1 | n/a | Vision loss, 21 (23.1) | Hearing loss, 36 (54.5) | Hearing loss, 40 (62.5) | Hearing loss, 35 (53.9) | ||
| 2 | n/a | Hearing loss, 19 (20.9) | Weakness/wasting, seizures, headache, tinnitus, 4 (6.1) | Pain, 5 (7.8) | Pain, 5 (7.7) | ||
| 3 | n/a | Weakness/ wasting, 14 (15.4) | Weakness/ wasting, 3 (4.7) | Weakness/wasting, headaches, poor balance, 4 (6.2) |
Abbreviations: n, number; %, percentage.
aSuicide death.