Table 1.

Summary of audiological test results and participant demographic and disease information. The key at the bottom shows the color codes used for PTA values, DPOAE, and ABR results.

							Left Ear				Right Ear
ID	Age (years)	Morquio Type	Height (cm)	BMI	ERT	ADL score	Hearing Loss Type	PTA	DPOAE (% present)	ABR	Hearing Loss Type	PTA	DPOAE (% present)	ABR
S1	12	A	94	16	Y	36.0	Conductive	35	17	Abnormal	Conductive	25	0	Normal
S2	15	B	135	19	N	36.0	Normal	10	100	Normal	Normal	5	100	Normal
S3	17	A	115	25	Y	51.0	Sensorineural	50	0	Normal	Sensorineural	45	8	Normal
S4	17	A	122	23	Y	45.0	Sensorineural	10	75	Abnormal	Normal	0	83	Normal
S5	17	A	99	24	Y	52.0	Mixed	35	0	Normal	Mixed	30	17	Normal
S6	18	A	99	45	Y	50.0	Mixed	55	0	Abnormal	Mixed	50	0	Abnormal
S7	19	A	137	24	N	47.0	Sensorineural	5	100	Abnormal	Sensorineural	5	92	Abnormal
S8	21	A	114	27	Y	56.0	Sensorineural	15	DNT	Abnormal	Normal	20	DNT	Normal
S9	22	A	122	23	N	49.0	Normal	5	92	Normal	Normal	5	100	Normal
S10	24	A	97	27	N	37.0	Conductive	65	0	Normal	Conductive	70	0	Normal
S11	28	A	122	22	Y	46.5	Sensorineural	15	50	Abnormal	Normal	10	75	Abnormal
S12	32	A	99	25	Y	55.0	Mixed	75	0	Abnormal	Mixed	90	0	Abnormal
S13	36	A	109	27	N	55.0	Mixed	20	33	Abnormal	Mixed	15	42	Abnormal
S14	38	A	97	25	N	55.0	Mixed	25	8	Abnormal	Conductive	15	58	Normal

ERT: enzyme replacement therapy ADL: Activity of daily living DNT: The test was not administrated.

PTA	DPOAE	ABR
Normal	Present (76-100%)	Normal
Slight to Mild
Moderateto Moderately Severe	Partially present (26-75%)	–
Severe	Absent (0-25%)	Abnormal