Two families segregated reported mutations (p.R104X and p.R95W) and one family segregated a novel mutation (p.R132GfsX26) of FGF3. All individuals homozygous for p.R104X or p.R132GfsX26 had fully penetrant features of LAMM syndrome. The gene discussed is FGF3; the disease is deafness with labyrinthine aplasia, microtia, and microdontia.