Table 3. Otological and audiological examination results.

Patient	Age (Y)	Sex	Mutation reported in WFS1	AoO (Y)	Otoscopy	FI RE/LE (dB HL)	MSRS RE/LE (%)	ENG	ASSR	CT temporal bone
II:2	85	F	+	9	Normal	98/100	3/0	Na	Na	Na
II:3	75	M	-		Normal	10/12	Na	Na	Na	Na
III:1	57	F	-		Normal	22/18	100/100	Na	Na	Na
III:2	57	M	+	8	Normal	90/97	15/0	Normal	Conform pure tone audiogram	No anatomic abnormalities
III:3	51	M	+	14	Normal	58/60	93/90	Na	Na	Na
IV:1	26	F	-		Normal	5/7	Na	Na	Na	Na
IV:2	23	M	-		Normal	18/20	100/100	Na	Na	Na

For the patients the age in years (Y) is shown as well as whether they carry the WFS1 mutation and their age of onset of audiological complaints (AoO). The results of the audiological examination tests are depicted for each ear separately. The ears of the mutation carrying individuals have an increased Fletcher Index (FI; mean threshold for 0.5, 1, and 2 kHz) and a decreased maximum speech recognition score (MSRS). Abbreviations: female (F); male (M); right ear (RE); left ear (LE); decibel hearing loss (dB HL); electronystagmography (ENG); auditory steady-state response (ASSR); computed tomography (CT); not available (Na).