Table 4.

Results of questionnaires and assessment of medical records in affected subjects with IKZF2 variants

Family	Subject	Sex	Agea (years)	Age of HL onsetb	Progressionc	Use of hearing aids	Risk factors for acquired HL	DHI	Other
W16-0482	II:2	M	56	45 years	Yes	Yes	Antibiotic used; noise exposure	0	Tinnitus; positive family anamnesis for rheumatism
	III:2	F	N/a	15 years	MD	MD	MD	MD
W22-1907	II:3	F	62	12 years	Yes	Yes	Recurrent ear infections	8	Tinnitus; symptoms of vertigo
	II:4	M	53	18 years	Yes	Yes		0
	III:1	M	39	Childhood	Yes	No	Ear surgery (tubes); recurrent ear infections	2	Tinnitus; medical history of congenital choanal atresia on the left side
	III:2	F	38	Childhood	Yes	No	Antibiotic use; ear surgery (tubes)	14
	III:3	M	36	Adolescence	Yes	No	MD	MD
W22-2757	II:2	M	59	32 years	Yes	Yes		0
	III:1	F	43	3–4 years	Yes	MD	MD	MD
	III:2	F	38	20–25 years	Yes	Yes	MD	MD

DHI, dizziness handicap inventory; F, female; HL, hearing loss; M, male; MD, missing data (applicable to retrospective assessment of medical records in case of uncompleted questionnaires); N/a, not applicable; y, years

^aAge at completing questionnaire (not applicable in case of data retrieval from medical records)

^bSelf-reported age of onset

^cSelf-reported progression

^dType of which unknown