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Table 1 Clinical features of family members carrying the m.7511T > C mutation

From: Mitochondrial mutations in maternally inherited hearing loss

Individual Sex Age of onset (years) Age at time of test (years) Severity (R/L) Audiometric configuration (R/L) Progression Speech discrimination (R/L) DPOAE
III-3 F 13 42 Moderate/Moderate Gently sloping/Flat Yes 95%/85% Bilateral NR
III-5 F 35 41 Moderate/Moderate Gently sloping/Gently sloping Yes 90%/95% Bilateral NR
III-7 F 39 Normal/Normal NA No Not tested R: NR except 1 kHz,
L: NR except 1 kHz and 3 kHz
IV-2 F 7 7 Mild/Mild Flat/Flat No 100%/100% NR at 4 kHz
IV-3 F 5 9 Moderate/Moderate Steeply sloping/Gently sloping Yes 95%/90% Bilateral NR
IV-4 M 22 Normal/Normal NA No Not tested Bilateral NR
IV-6 F 0 8 Mild/Moderate Low frequency/Low frequency No Not tested Not tested
  1. R right ear, L left ear, NR no response, NA not applicable, DPOAE distortion-product otoacoustic emissions