Table 4.

Prevalence of extraskeletal features.

		All patients (n = 568)
		n	%	95% CI
Any skin abnormality	Yes	85	15.0%	12.3%–18.1%
Morphological skin abnormality	Yes	24	4.2%	2.9%–6.2%
Cutis laxa	Yes	66	11.6%	9.2%–14.5%
Skin lesion	Yes	6	1.1%	0.5%–2.3%
Any deafness	Yes	97	17.1%	14.2%–20.4%
Mixed deafness	Yes	25	4.4%	3.0%–6.4%
Conductive deafness	Yes	32	5.6%	4.0%–7.8%
Sensorineural deafness	Yes	36	6.3%	4.6%–8.6%
Other deafness	Yes	4	0.7%	0.3%–1.8%
Any valvulopathy	Yes	67	11.8%	9.4%–14.7%
Aortic valvulopathy	Yes	8	1.4%	0.7%–2.8%
Mitral valvulopathy	Yes	43	7.6%	5.7%–10.0%
Pulmonary valvulopathy	Yes	4	0.7%	0.3%–1.8%
Tricuspid valvulopathy	Yes	15	2.6%	1.6%–4.3%
Sclera	White	120	21.1%	18.0%–24.7%
	Blue	369	65.0%	61.0%–68.8%
	Gray/purple	79	13.9%	11.3%–17.0%
Joint Hyperlaxity	Yes	217	38.2%	34.3%–42.3%
Any dental defect	Yes	109	19.2%	16.2%–22.6%
Dentinogenesis imperfecta	Yes	89	15.7%	12.9%–18.9%
Other dental defects	Yes	52	9.2%	7.0%–11.8%

CI, confidence interval.