Table 1 Summary of clinical data for the hearing-impaired members of Family 304.

Subject	Gender ^a	Age of test (year)	Age of onset (year)	PTA (dB HL) ^b	Hearing impairment ^c	Audiogram	Tinnitus	Vertigo	Mutation ^d detected
III:2	F	57	Not clear	L:71.25	Severe	Downslope	+	−	c. 524C > A
				R:67.5	Moderately severe	Downslope
III:3	M	60	N/A	L:32.50	Mild	Downslope	−	−	−
				R:30.00	Mild	Downslope
III:8	F	50	23	L:76.25	Severe	Downslope	+	−	c. 524C > A
				R:77.50	Severe	Downslope
III:10	F	49	21	L:75.00	Severe	Downslope	+	−	c. 524C > A
				R:98.75	Profound	Downslope
III:5	M	55	N/A	L:32.50	Mild	U-shape	−	−	−
				R:42.50	Moderate	U-shape
III:12	F	47	0	L:>100	Profound	Flat	+	−	c. 524C > A
				R:66.25	Moderately severe	Downslope
III:14	F	44	42	L:21.25	Normal	U-shape	−	−	−
				R:21.25	Normal	U-shape
III:16	F	43	0	L:77.50	Severe	Downslope	−	−	c. 524C > A
				R:81.25	Severe	Downslope
III:18	F	39	0	L:68.33	Moderately severe	Downslope	+	−	c. 524C > A
				R:>100	Profound	Flat
IV:2	M	35	Not clear	L:78.75	Severe	Downslope	+	−	c. 524C > A
				R:90.00	Severe	Downslope
IV:3	F	30	Not clear	L:53.75	Moderate	Downslope	+	−	c. 524C > A
				R:50.00	Moderate	Downslope
IV:6	M	30	N/A	L:38.75	Mild	U-shape	−	−	-
				R:40.00	Mild	U-shape
IV:12	M	24	0	L:72.50	Severe	Downslope	+	−	c. 524C > A
				R:73.75	Severe	Downslope
IV:13	F	24	Not clear	L:43.75	Moderate	Downslope	+	−	c. 524C > A
				R:53.75	Moderately severe	Downslope
IV:20	F	2	0	L:110dBnHL	Profound	/	−	−	c. 524C > A
				R:90dBnHL	Profound	/
IV:21	F	14	0	L:70.00	Moderately severe	Downslope	+	−	c. 524C > A/c.235delC
				R:71.25	Severe	Downslope

N/A, not available; +, positive finding; −, negative finding.
^aM, male; F, female.
^bPTA, pure-tone air-conduction averages (0.5, 1, 2 and 4 kHz). L, left ear, R, right ear. For the IV:20, who was 2 years old, her thresholds were the results of 40Hz AERP (auditory event-related potential) by tone burst.
^cDiagnosed at the time of test.
^dRefSeq: NM_004004, GRCh37/hg19 chr13.

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