Alu repeats in the deleted region lead to limited mechanisms of loss by recombination on that allele, leaving only the option of biallelic NF2 single-nucleotide pathogenic variants, which occur as the cause in <30% of sporadic vestibular schwannomas with both hits identified.11 It is possible that this could have occurred in the single case we observed with a large 22q11.2 deletion and a schwannoma, or the tumor could have occurred as a chance association through biallelic single-nucleotide pathogenic variants within the NF2 gene. The gene discussed is NF2; the disease is Vestibular schwannoma.