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A: The patient has heterozygous ACTG1 mutation, c.895C>G. Pedigree is compatible with autosomal dominant hearing loss. His father and brother carried the same mutation. B: Pre-operative audiogram. C: Audiogram of brother. D: Post-operative audiogram (6 months after EAS). E: Hearing threshold with EAS. F: Japanese monosyllable test (65dB SPL in quiet) showing dramatic improvement with EAS.
