Table 1.

Summary of the clinical findings of the seven patients

Family	Patient	Age at first visit (years)	Sex	NHS		First PTA		Latest PTA		DPOAE		ABR		ASSR								Follow-up period (months)	Vertigo	CT findings	HA
				Rt.	Lt.	Rt. (dB)	Lt. (dB)	Rt. (dB)	Lt. (dB)	Rt.	Lt.	Rt. (dB)	Lt. (dB)	Rt. (dB)				Lt. (dB)
														500	1 k	2 k	4 k	500	1 k	2 k	4 k
48	077RYU	7	M	NA		46	45	47	45	Negative	Negative	NA		NA				NA				155	No	No abnormality	Yes
	078RYU	3	F	NA		47	45	43	43	Negative	Negative	50	60	NA				NA				232	No	No abnormality	Yes
57	095RYU	5	F	NA		42	42	38	45	Negative	Negative	50	50	40	40	40	50	40	40	50	50	76	No	No abnormality	No
	097RYU	2	M	NA		47	51	51	48	NA	NA	60	60	60	50	50	60	60	50	50	60	70	No	No abnormality	Yes
83	146RYU	4	M	Pass	Pass	40	40	40	45	Negative	Negative	40	50	50	50	50	40	50	50	50	50	56	No	No abnormality	No
	147RYU	0	M	Pass	Refer	41	45	41	41	Negative	Negative	40	50	40	30	40	40	40	40	50	50	61	No	No abnormality	Yes
145	350RYU	0	M	Refer	Refer	41	41	41	41	Negative	Negative	50	60	40	50	50	50	50	60	50	60	47	No	No abnormality	Yes

NHS newborn hearing screening, PTA pure tone audiometry, DPOAE distortion product otoacoustic emissions, ABR auditory brainstem response, ASSR auditory steady-state response, HA hearing aid, Rt. right, Lt. left, NA not assessed