NF2-related schwannomatosis: A view from within the inner ear.

NF2-related schwannomatosis (NF2-SWN, formerly known as neurofibromatosis type 2) is an autosomal dominant disorder associated with the growth of bilateral schwannomas on the cochleo-vestibular nerves and meningiomas. NF2-SWN is caused by pathogenic variations in the NF2, moesin-ezrin-radixin-like (MERLIN) tumour suppressor gene. The mostly benign tumours can cause progressive sensorineural hearing loss, tinnitus and balance dysfunction.