<b>Results</b> We identified a cosegregating heterozygous missense mutation, c.296G>A (p.Arg99His) in the gene encoding interferon lambda receptor 1 (<i>IFNLR1</i>) - a protein that functions in the Jak/ STAT pathway- are associated with <i>ADNSHL</i> Morpholino knockdown of ifnlr1 leads to a significant decrease in hair cells and non-inflation of the swim bladder in late-stage zebrafish, which can be reversed by injection with normal Zebrafish <i>ifnlr1</i> mRNA.