Bilateral Deafness and Skin Lesions in a 23-Year-Old Man

Sumaira Nabi, MBBS; Shahzad Ahmed, MBBS; Mazhar Badshah, MBBS

Disclosures

October 12, 2016

The management of NF2 is challenging and must involve a multidisciplinary approach. The treatment of choice for vestibular schwannomas is surgical resection.[20] Tumors that lead to brainstem compression, hydrocephalus, hearing loss, or facial nerve paresis are best managed surgically. Tumors found incidentally on neuroimaging are better managed conservatively and monitored.

The timing and procedure of surgery are important. In addition to open surgery, stereotactic surgery, radiosurgery, and radiation therapy are alternative options.[23] Radiation therapy has shown variable outcomes, and its use is controversial.[24] Cochlear implants may be offered to patients with severe deafness.[25] Meningiomas are also surgically managed.

Targeted drug therapy is the probable future treatment for NF2.[26] Bevacizumab, a monoclonal antibody against vascular endothelial growth factor, has been reported in the literature to cause regression in tumor size, with resultant hearing improvement in patients with NF2. Other experimental therapies include everolimus, lapatinib, sorafenib, and erlotinib, which have all demonstrated variable results.[27]

The prognosis is dismal, with numerous patients dying in their youth. Despite the advent of advanced imaging techniques and treatment modalities, morbidity and mortality are still significant. Young age at onset and a greater number of meningiomas are poor prognostic markers. Drug therapies working at the molecular and genetic levels are the breakthrough expected in years to come and are anticipated to remarkably improve outcomes in patients with NF2.[26]

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