The cause of an acoustic neuroma is unknown, and the condition is rare and generally affects only a small proportion of the population. As with other brain tumours, there are several risk factors - such as age, family history and any head injuries - which can increase the possibility of a tumour developing.
Around 7% of cases are caused by a rare genetic disorder known as neurofibromatosis type 2 (NF2). This condition typically causes acoustic neuromas that affect both ears at the same time.