Audiological profile and cochlear functionality in Williams syndrome

Silva, Liliane Aparecida Fagundes; Kawahira, Rachel Sayuri Honjo; Kim, Chong Ae; Matas, Carla Gentile

Liliane Aparecida Fagundes Silva

Departamento de Fisioterapia, Fonoaudiologia e Terapia Ocupacional, Faculdade de Medicina – FM, Universidade de São Paulo – USP – São Paulo (SP), Brasil.

https://orcid.org/0000-0001-8985-0447

Rachel Sayuri Honjo Kawahira

Unidade de Genética, Faculdade de Medicina – FM, Instituto da Criança, Hospital das Clinicas – HC, Universidade de São Paulo – USP – São Paulo (SP), Brasil.

https://orcid.org/0000-0002-9223-7341

Chong Ae Kim

Unidade de Genética, Faculdade de Medicina – FM, Instituto da Criança, Hospital das Clinicas – HC, Universidade de São Paulo – USP – São Paulo (SP), Brasil.

https://orcid.org/0000-0002-1754-1300

Carla Gentile Matas

Departamento de Fisioterapia, Fonoaudiologia e Terapia Ocupacional, Faculdade de Medicina – FM, Universidade de São Paulo – USP – São Paulo (SP), Brasil.

https://orcid.org/0000-0002-9408-7172

Conflict of interests: nothing to declare.

Correspondence address: Liliane Aparecida Fagundes Silva Departamento de Fisioterapia, Fonoaudiologia e Terapia Ocupacional, Faculdade de Medicina, Universidade de Sao Paulo – USP Rua Cipotânea, 51, Cidade Universitária, São Paulo (SP), Brasil, CEP: 05360-160. E-mail: liliane.a.fagundes@gmail.com

Author contributions

LAFS: responsible for the collection, tabulation, and analysis of data, as well as the manuscript elaboration; RSHK: responsible for subject selection, data analysis, and manuscript elaboration; CAK and CGM: responsible for study design and general guidance of the stages of manuscript execution and elaboration.

Patient	Right Ear				Left Ear
	Type of Tympanometric curve	Hearing loss			Tympanometric curve	Hearing loss
	Type of Tympanometric curve	Type	Degree	Frequencies (kHz)	Tympanometric curve	Type	Degree	Frequencies (kHz)
1	A	SNHL	Mild	6 and 8	A	SNHL	Mild	6 and 8
2	A	SNHL	Mild to moderate	6 and 8	A	SNHL	Mild	6 and 8
3	A	Normal thresholds			A	SNHL	Mild	3 and 6
4	C	SNHL	Mild	6 and 8	C	SNHL	Mild	6 and 8
5	A	SNHL	Mild	0.25 and 0.5	A	Normal thresholds
6	A	SNHL	Mild	4	A	Normal thresholds
7	Ad	SNHL	Mild	0.25 and 6	Ad	SNHL	Mild	0.25
8	A	SNHL	Moderate	3 to 8	A	MHL	Mild to severe*	All
9	B	MHL	Moderate to severe*	All	A	SNHL	Mild to moderate	4 to 8
10	Ad	SNHL	Mild	6 and 8	A	SNHL	Mild	1, 2, 3, 6, and 8
11	B	MHL	Mild to moderate*	All	B	MHL	Mild to moderate^* * degree of hearing loss considering the air-conduction thresholds	All

Frequency	Group	N	Right Ear		Left Ear
Frequency	Group	N	Average ± Standard Deviation	p-value	Average ± Standard Deviation	p-value ⁺ + p-value obtained through T-test for independent samples
0.25 kHz	SG	44	14.77 ± 12.49	0.006*	12.27 ± 9.22	0.003*
0.25 kHz	CG	34	6.18 ± 4.52	0.006*	5.59 ± 2.43	0.003*
0.5 kHz	SG	44	15.23 ± 12.29	0.001*	12.27 ± 9.22	0.004*
0.5 kHz	CG	34	5.00 ± 3.06	0.001*	5.59 ± 3.49	0.004*
1 kHz	SG	44	12.50 ± 11.21	0.003*	12.05 ± 12.88	0.004*
1 kHz	CG	34	4.12 ± 3.18	0.003*	2.94 ± 3.57	0.004*
2 kHz	SG	44	11.59 ± 15.30	0.007*	10.23 ± 13.49	0.024*
2 kHz	CG	34	1.77 ± 3.03	0.007*	2.95 ± 3.98	0.024*
3 kHz	SG	44	15.00 ± 18.71	0.009*	12.50 ± 15.41	0.034*
3 kHz	CG	34	3.23 ± 3.93	0.009*	4.71 ± 4.83	0.034*
4 kHz	SG	44	17.50 ± 19.13	0.004*	18.86 ± 21.49	0.012*
4 kHz	CG	34	4.12 ± 3.63	0.004*	5.88 ± 5.37	0.012*
6 kHz	SG	44	21.36 ± 18.78	0.002*	24.55 ± 23.95	0.005^* * p-value statistically significant;
6 kHz	CG	34	7.06 ± 5.32	0.002*	8.53 ± 3.43	0.005^* * p-value statistically significant;
8 kHz	SG	44	25.23 ± 22.33	0.002*	24.32 ± 19.04	0.000*
8 kHz	CG	34	7.94 ± 4.70	0.002*	5.29 ± 4.13	0.000*

TEOAE	Presence of Response	SG (n=25)	CG (n=34)	χ²	GL	p-value ⁺ + p-value obtained through Pearson's chi-square test (χ2)
1 kHz	Yes	56.00%	88.24%	7.896	1	0.005^* * statistically significant p-value;
1 kHz	No	44.00%	11.76%	7.896	1	0.005^* * statistically significant p-value;
1,5 kHz	Yes	64.00%	100.00%	14.443	1	0.000*
1,5 kHz	No	36.00%	0.00%	14.443	1	0.000*
2 kHz	Yes	52.00%	91.18%	11.662	1	0.001*
2 kHz	No	48.00%	8.82%	11.662	1	0.001*
3 kHz	Yes	60.00%	100.00%	16.376	1	0.001*
3 kHz	No	40.00%	0.00%	16.376	1	0.001*
4 kHz	Yes	28.00%	64.71%	7.776	1	0.005*
4 kHz	No	72.00%	35.29%	7.776	1	0.005*
*Response*	Yes	64.00%	100.00%	14.443	1	0.000*
*Response*	No	36.00%	0.00%	14.443	1	0.000*

		N	Average (dB SPL)	Standard Deviation	Minimum	Median	Maximum	T-Value	DF	p-value ⁺ + p-value obtained through unpaired T-test
1 kHz	SG	25	3.74	2.51	0.04	3.71	9.44	5.56	48	0.000*
1 kHz	CG	34	9.69	5.52	1.17	7.83	21.53	5.56	48	0.000*
1,5 kHz	SG	25	5.84	4.68	0.10	4.09	16.00	3.96	56	0.000*
1,5 kHz	CG	34	11.18	5.66	3.62	10.34	24.97	3.96	56	0.000*
2 kHz	SG	25	6.44	5.33	0.13	6.41	15.19	4.11	52	0.000*
2 kHz	CG	34	12.22	5.35	1.27	13.38	21.83	4.11	52	0.000*
3 kHz	SG	25	8.35	5.61	0.18	9.53	16.42	4.74	55	0.000*
3 kHz	CG	34	15.80	6.42	6.07	15.64	26.70	4.74	55	0.000*
4 kHz	SG	25	5.05	4.76	0.39	4.15	20.58	3.42	56	0.001*
4 kHz	CG	34	10.00	6.38	1.92	8.13	25.12	3.42	56	0.001*
*Response*	SG	25	18.52	4.68	11.35	16.65	27.12	3.14	51	0.003^* * statistically significant p-value;
*Response*	CG	34	22.40	4.69	13.28	21.70	30.06	3.14	51	0.003^* * statistically significant p-value;

[1] Conflict of interests: nothing to declare.

Brasil

Brasil

Audiological profile and cochlear functionality in Williams syndrome

ABSTRACT

Purpose

Methods

Results

Conclusion