Amusia and its electrophysiological correlates in neurofibromatosis type 1

Eighteen people with NF1 and 22 healthy volunteers, matched for age, sex and educational level, were evaluated with the Montreal Battery Evaluation of Amusia – short version. The integrity of cortical primary auditory processing areas was evaluated by evoked potential mismatch negativity.

Results:

Amusia was correlated with NF1 (p = 0.001, odds ratio = 42.0, confidence interval 4.5–39.6). Patients with NF1 exhibited a greater prevalence of amusia than healthy controls (67% vs. 4.5%) and difficulties in both melodic and temporal music perception. Worse performance on the Montreal Battery Evaluation of Amusia was correlated with a greater mismatch negativity latency in NF1 group.

Conclusions:

Amusia is a common feature in NF1 and may result from impairment of activity in primary auditory processing areas.

Keywords:
neurofibromatosis 1; music; evoked potentials; auditory perceptual disorders; pitch discrimination

Test	Melodic organization			Temporal organization		Musical memory
Test	Scale	Contour	Interval	Rhythm	Meter	Musical memory
Items	15 pairs of melodies per test:			Similar to melodic tests	14 musical phrases	14 melodies
	7 pairs of identical melodies				7 in a double meter;	7 previously presented
	7 pairs of different melodies¹ 1 In melodic organization tests, the difference occurred in the pitch of a single note in the second melody; the altered note was selected according to the component of evaluation (scale, contour, or interval). In the rhythm test, the alteration occurred in the duration of two adjacent notes.				7 in a triple meter	7 novel
	1 strategic item² 2 This item contained pairs of melodies that were clearly different.
Goal	Identify whether the melodies heard in each pair were equal or different			Similar to melodic tests	Categorize the musical phrase as a march (double meter) or waltz (triple meter)	Identify whether the melody was previously presented or is novel

Groups	Total MBEA		Melodic		Temporal		Memory
	84 items		42 items		28 items		14 items
	Mean	SD	Mean	SD	Mean	SD	Mean	SD
NF1 patients (n = 18)	57.39	7.92	26.94	4.94	18.44	2.50	12.11	1.97
NF1 patients (n = 18)	68.30%		62.80%		65.80%		86.50%
Controls (n = 22)	71.59	4.87	33.64	3.75	24.77	2.59	13.23	0.87
Controls (n = 22)	85.20%		80.00%		88.50%		94.50%

Groups	Gender			Age			Schooling
Groups	Male	Female	p-value^a	Mean	SD	p-value^b	Middle	Higher	p-value^a
NF1 patients (n = 18)	6	12	0.842	24.17	5.94	0.902	6	12	0.498
NF1 patients (n = 18)	33.3%	66.7%					33.3%	66.7%
Controls (n = 22)	8	14		24.36	4.10		5	17
Controls (n = 22)	36.4%	63.6%					13.6%	86.4%
Total (n = 40)	14	26		24.27	4.95		9	31
Total (n = 40)	35.0%	65.0%		24.27	4.95		22.5%	77.5%

Variables		Total MBEA score
		Controls				NF1 patients
		n	Mean	SD	p-value	n	Mean	SD	p-value
Gender
	Female	14	71.71	4.71	0.88^b b Student t-test;	12	56.92	9.51	0.732^b b Student t-test;
	Male	8	71.38	5.45	0.88^b b Student t-test;	6	58.33	3.56	0.732^b b Student t-test;
Schooling
	Middle	5	71.60	3.36	1.0^b b Student t-test;	6	59.00	6.42	0.558^b b Student t-test;
	Higher	17	71.59	5.32	1.0^b b Student t-test;	12	56.58	8.72	0.558^b b Student t-test;
Music study or practice
	Yes	9	72.56	6.82	0.45^b b Student t-test;	10	53.60	7.21	0.018^b b Student t-test;
	No	13	70.92	3.04	0.45^b b Student t-test;	8	62.13	6.27	0.018^b b Student t-test;
Age^a a Age in years: It was not possible to obtain average and standard deviation values for this parameter since it was not organized in categories;		-	-	-	0.09^c c Pearson's r.	-	-	-	0.582^c c Pearson's r.

Variable	With amusia				Without amusia
	n	Occurrence	Latency		n	Occurrence	Latency
	n	Occurrence	Mean	SD	n	Occurrence	Mean	SD
NF1 patients	10	9 (90%)	171.76	8.81	5	4 (80%)	139.16	6.11
Controls	1	1 (100%)	160^* * It was not possible to obtain mean and standard deviation values for MMN latency in the control group with amusia, as the data corresponded to a single individual.	^* * It was not possible to obtain mean and standard deviation values for MMN latency in the control group with amusia, as the data corresponded to a single individual.	18	16 (89%)	166.22	5.67

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[1] Correspondence: Bruno Cézar Lage Cota; Centro de Referência em Neurofibromatoses do Hospital das Clínicas da UFMG; Alameda Álvaro Celso, 55; 30150-260 Belo Horizonte MG, Brasil; Email: brucezar@hotmail.com.br