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We describe a family with both hearing loss (HL) and thrombocytopenia, caused by pathogenic variants in three genes. The proband was a child with neonatal thrombocytopenia, childhood-onset HL, hyper-laxity and severe myopia. The child’s mother (and some of her relatives) presented with moderate thrombocytopenia and adulthood-onset HL. The child’s father (and some of his relatives) presented with adult-onset HL. An HL panel analysis, completed by whole exome sequencing, was performed in this complex family. We identified three pathogenic variants in three different genes: <i>MYH9</i>, <i>MYO7A</i> and <i>ACTG1</i>. The thrombocytopenia in the child and her mother is explained by the <i>MYH9</i> variant. The post-lingual HL in the paternal branch is explained by the <i>MYO7A</i> variant, absent in the proband, while the congenital HL of the child is explained by a de novo <i>ACTG1</i> variant. This family, in which HL segregates, illustrates that multiple genetic conditions coexist in individuals and make patient care more complex than expected.