Table 1.
General demographic and neuropsychological data for patient subgroups with and without auditory hedonic symptoms.
| FTLD |
AD |
Healthy controls* | |||
|---|---|---|---|---|---|
| Auditory hedonic | No auditory hedonic | Auditory hedonic | No auditory hedonic | ||
| General | |||||
| No. (F:M) | 31 (9:22)† | 25 (15:10) | 7 (2:5)†† | 10 (5:5) | 50 (23:27) |
| Syndrome: bvFTD/SD/PNFA | 19/11/1 | 3/8/14 | NA | NA | NA |
| Genetic: C9orf72/MAPT | 3/6 | 4/0 | NA | NA | NA |
| Age (years) | 64.7 (52–79) | 64.9 (52–75) | 66.3 (60–73) | 66.4 (53–80) | 67.5 (54–80) |
| Education (years) | 14 (11–21) | 15 (11–20) | 13 (11–17) | 13.9 (12–17) | 15.2 (10–18) |
| Symptom duration (years) | 6.1 (3–18) | 5.7 (3–21) | 6 (4–9) | 4.4 (2–6) | NA |
| MMSE | 24 (12–30) | 19 (1–30) | 24 (20–25) | 20 (13–30) | 29.6 (28–30) |
| IQ | |||||
| Verbal | 80 (40–119) | 82 (55–115) | 94 (71–115) | 84 (55–108) | 120 (101–137) |
| Performance | 101 (74–135) | 96 (66–135) | 86 (61–125) | 84 (57–119) | 115 (84–141) |
| Episodic memory | |||||
| RMT faces (/50) | 31 (24–50)a | 37 (25–46) | 36 (27–43) | 32 (23–46) | 43 (30–50) |
| RMT words (/50) | 34 (23–49) | 37 (18–48) | 34 (27–47) | 31 (27–42) | 48 (39–50) |
| Semantic processing | |||||
| BPVS (/150) | 110 (2–149) | 109 (25–149) | 133 (106–146) | 124 (52–147) | 147 (137–150) |
| Executive function | |||||
| Stroop inhibition (180 s) | 88 (40–180) | 103 (50–180) | 135 (42–180) | 107 (73–138) | 58 (35–103) |
| Digit span reverse (/12) | 4 (0–7) | 4 (0–7) | 3.5 (2–6) | 3 (1–5) | 5 (3–7) |
| Visuospatial | |||||
| VOSP object decision (/20) | 16 (8–20) | 16 (3–20) | 16.5 (14–18) | 16 (11–19) | 18 (12–20) |
Mean (range) data are shown unless otherwise indicated and maximum scores on neuropsychology tests are also indicated in parentheses. Significant differences (p < .05) between patients and healthy controls are in bold; *historical control group (to reference neuropsychological characterisation of disease groups); †four patients with environmental sound aversion alone, 10 with musicophilia alone, eight with music aversion alone, five with both musicophilia and environmental sound aversion, four with both music and environmental sound aversion (see text, Fig. 1); ††five patients with environmental sound aversion alone, two with both music and environmental sound aversion; asignificantly (p < .05) different from non-symptomatic patients with FTLD; AD, syndrome of Alzheimer's disease led by decline in episodic memory; BPVS, British Picture Vocabulary Scale; bvFTD, behavioural variant of frontotemporal dementia; C9orf72, mutation in open reading frame 72 on chromosome 9; FTLD, frontotemporal lobar degeneration; MAPT, mutation in microtubule associated protein tau gene; MMSE, Mini-Mental State Examination score; NA, not applicable; PNFA, progressive nonfluent aphasia; RMT, Recognition Memory Test; SD, semantic dementia; VOSP; Visual Object and Space Perception battery.