<unk>  is a life limiting condition. It is a rare genetic disorder that involves noncancerous tumors of the nerves that transmit balance and sound impulses from the inner ear to the brain. The prognosis is affected by early age onset, a higher number of meningiomas and schwannomas and having a decrease in mutation. An early diagnosis is the best way to ensure improvement in management. Although, even with an early diagnosis, some patients still die very young. Meningiomas and schwannomas occur in around half of patients with<unk> . Meningiomas are tumors that are both intracranial and intraspinal. Schwannomas are tumors that are often centered on the internal auditory canal. Patients with<unk>  who have meningiomas have a higher risk of mortality, and the treatment can be very challenging. Individuals who develop schwannomas frequently develop hearing loss and deafness. These individuals may also develop tinnitus after being presented with unilateral hearing loss. The first symptom that individuals may encounter is dizziness or imbalance. Truncating mutations lead to smaller and non-functional protein products. Studies have shown that missense mutations and large deletions can both cause predominantly mild phenotypes. Phenotype is more variable in patients with splice-site mutations, and a milder disease in patients with mutations in exons 9–15. Patients with a missense mutation have a greater survival rate than nonsense and frameshift mutations.
Neurofibromatosis type II
Neurofibromatosis type 4
Tuberous sclerosis
Von Hippel–Lindau disease
Fox–Fordyce disease
Progeria
Fabry disease
Irlen syndrome