Vestibular schwannoma (VS) are benign cranial nerve sheath tumors of the vestibulocochlear nerve. Their incidence is mostly sporadic, but they can also be associated with -related schwannomatosis (NF2), a hereditary tumor syndrome. Metastasis associated in colon cancer 1 (MACC1) is known to contribute to angiogenesis, cell growth, invasiveness, cell motility and metastasis of solid malignant cancers. In addition, MACC1 may be associated with nonsyndromic hearing impairment. Therefore, we evaluated whether MACC1 may be involved in the pathogenesis of VS. Sporadic VS, recurrent sporadic VS, NF2-associated VS, recurrent NF2-associated VS and healthy vestibular nerves were analyzed for mRNA and protein expression by quantitative polymerase chain reaction and immunohistochemistry. expression levels were correlated with the patients' clinical course and symptoms. mRNA expression was significantly higher in sporadic VS compared to NF2-associated VS ( < 0.001). The latter expressed similar MACC1 concentrations as healthy vestibular nerves. Recurrent tumors resembled the expression of the primary tumors. mRNA expression was significantly correlated with deafness in sporadic VS patients ( = 0.034). Therefore, MACC1 might be a new molecular marker involved in VS pathogenesis.