Similar to proband ENG_CMK who had CFEOM, developmental delay, sensorineural hearing impairment, and an MRI revealing small CN3 (and inability to resolve CN4), absent CN8, and brain malformations, Sema3f−/− mice have CN3 defasciculation and CN4 absence consistent with CFEOM, as well as hearing impairment and brain malformations.36 This evidence concerns the gene SEMA3F and Hearing impairment.