The importance of central auditory evaluation in Friedreich's ataxia

A cross-sectional, retrospective study was carried out. Thirty patients underwent the anamnesis, otorhinolaryngology examination, pure tone audiometry, acoustic immittance measures and brainstem auditory evoked potential (BAEP) assessments.

Results

The observed alterations were: 43.3% in the pure tone audiometry, bilateral in 36.7%; 56.6% in the BAEP test, bilateral in 50%; and 46.6% in the acoustic immittance test. There was a significant difference (p < 0.05) in the comparison between the tests performed.

Conclusion

In the audiological screening, there was a prevalence of the descending audiometric configuration at the frequency of 4kHz, and absence of the acoustic reflex at the same frequency. In the BAEP test, there was a prevalence of an increase of the latencies in waves I, III and V, and in the intervals of interpeaks I-III, I-V and III-V. In 13.3% of the patients, wave V was absent, and all waves were absent in 3.3% of patients.

Keywords
spinocerebellar degenerations; hearing disorders; Friedreich ataxia; evoked potentials, auditory; ataxic gait

P	Age(y) and Sex	Disease duration (y)	SARA
1	43/M	25	20
2	41/M	7	3.5
3	30/F	18	8
4	24/M	8	4
5	29/M	13	14
6	17/M	3	13
7	63/F	38	7
8	6/F	5	19
9	37/F	19	16
10	41/F	20	29.5
11	27/F	12	14
12	25/F	12	12
13	55/F	30	7
14	44/M	10	3.5
15	55/M	12	14
16	37/M	17	19
17	51/M	30	29
18	27/M	10	16
19	46/M	18	10
20	72/M	42	28
21	52/F	18	3
22	30/M	4	4.5
23	37/M	19	18
24	44/M	18	9.5
25	22/M	14	5
26	42/F	31	25
27	63/M	18	19
28	42/M	21	8
29	28/M	21	8
30	30/M	17	13

Correlation	Pearson's coefficient correlation (r)	p
Age and disease duration	0.7171	^* * Significant comparison; p-values for Pearson's coefficient correlation shown. 0.0000
Age and SARA	0.1911	0.0312
Disease duration and SARA	0.4169	^* * Significant comparison; p-values for Pearson's coefficient correlation shown. 0.0022

Symptoms	N. patients	Frequency (%)
Incoordination of movement	20	66.7
Gaitimbalance	17	56.7
Dizziness	15	50.0
Dysarthria	14	46.7
Headache	10	33.4
Dysphagia	9	30.0
Diplopia	9	30.0
Falling	8	26.7
Tremor	8	26.7
Depression	8	26.7
Fatigue	7	23.4
Anxiety	7	23.4
Pain, difficulty in neck movement	6	20.0
Tingling in extremities	4	13.4
Insomnia	3	10.0
Hearingloss	3	10.0
Olfactory alteration	2	6.7
Gustatory alteration	2	6.7
Dysphonia	1	3.4

Patient	Audiology assessment		BAEP
Patient	RE	LE	RE	LE
1	Normal	Normal	Abnormal	Abnormal
2	Normal	Normal	Normal	Normal
3	Normal	Normal	Abnormal	Abnormal
4	Normal	Normal	Normal	Normal
5	Sloping from 4 kHz	Sloping from 4 kHz	Abnormal	Abnormal
6	Normal	Normal	Normal	Normal
7	SNHL severe	SNHL severe	Abnormal	Abnormal
8	Normal	Normal	Normal	Normal
9	Normal	Normal	Abnormal	Abnormal
10	SNHL mild	Normal	Abnormal	Normal
11	Normal	Normal	Normal	Normal
12	Normal	Normal	Normal	Normal
13	Sloping from 3 kHz	Sloping from 3 kHz	Abnormal	Abnormal
14	Normal	Normal	Normal	Normal
15	Sloping from 2 kHz	Sloping from 2 kHz	Abnormal	Abnormal
16	Normal	Normal	Abnormal	Abnormal
17	Sloping from 2 kHz	Sloping from 2 kHz	Abnormal	Abnormal
18	Normal	SNHL severe	Normal	Abnormal
19	Normal	Normal	Abnormal	Abnormal
20	Sloping from 2 kHz	Sloping from 2 kHz	Abnormal	Abnormal
21	Sloping from 4 kHz	Sloping from 4 kHz	Abnormal	Abnormal
22	Normal	Normal	Normal	Normal
23	Sloping from 4 kHz	Sloping from 4 kHz	Normal	Normal
24	Notchat 6 kHz	Notchat 6 kHz	Abnormal	Abnormal
25	Normal	Normal	Normal	Normal
26	Sloping from 4 kHz	Sloping from 4 kHz	Abnormal	Abnormal
27	SNHL severe	SNHL moderate	Abnormal	Abnormal
28	Normal	Normal	Normal	Normal
29	Normal	Normal	Normal	Normal
30	Normal	Normal	Normal	Normal

Patient	Acoustic immittance
	Right ear		Left ear
	Tympanometry curve	Acousticreflex	Tympanometry curve	Acousticreflex
1	Type A	Absent at 3–4 kHz	Type A	Absent at 3–4 kHz
2	Type A	Present	Type A	Present
3	Type A	Absent at 4 kHz	Type A	Absent at 4 kHz
4	Type A	Present	Type A	Present
5	Type A	Absent at 4 kHz	Type A	Absent at 4 kHz
6	Type A	Present	Type A	Present
7	Type A	Absent	Type A	Absent
8	Type A	Present	Type A	Present
9	Type A	Absent at 4 kHz	Type A	Absent at 4 kHz
10	Type A	Present	Type A	Present
11	Type A	Present	Type A	Present
12	Type A	Present	Type A	Present
13	Type A	Absent at 3–4 kHz	Type A	Absent at 3–4 kHz
14	Type A	Present	Type A	Present
15	Type A	Absent at 3–4 kHz	Type A	Absent at 3–4 kHz
16	Type A	Present	Type A	Present
17	Type A	Absent at 2–4 kHz	Type A	Absent at 2–4 kHz
18	Type A	Absent at 3–4 kHz	Type As	Absent at 3–4 kHz
19	Type A	Present	Type A	Present
20	Type A	Absent at 3–4 kHz	Type A	Absent at 3–4 kHz
21	Type A	Absent at 4 kHz	Type A	Absent at 4 kHz
22	Type A	Present	Type A	Present
23	Type A	Absent at 4 kHz	Type A	Absent at 4 kHz
24	Type A	Present	Type A	Present
25	Type A	Present	Type A	Present
26	Type A	Absent at 4 kHz	Type A	Absent at 4 kHz
27	Type A	Absent	Type A	Absent
28	Type A	Present	Type A	Present
29	Type A	Present	Type A	Present
30	Type A	Present	Type A	Present

Variables			Normal (n)	Abnormal (n)	Total (n)	P
Audiology findings
	BAEP
		Normal	12	5	17
		Abnormal	1	12	13	^* * Significant comparison; p values for Fisher's exacttests shown. 0.0007
		Total	13	17	30
	Acoustic immittance
		Normal	14	3	17
		Abnormal	-	13	13	^* * Significant comparison; p values for Fisher's exacttests shown. 0.0000
		Total	14	16	30
BAEP findings, N
	Acoustic immittance
		Normal	12	1	13
		Abnormal	4	13	17	^* * Significant comparison; p values for Fisher's exacttests shown. 0.0002
		Total	16	14	30

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[1] Correspondence: Bianca Simone Zeigelboim; Departamento de Otoneurologia da Universidade Tuiuti do Paraná, Rua Sydnei Antonio Rangel Santos, 238; 82010-330 Curitiba PR, Brasil; E-mail: biancacwb@yahoo.com.br