To describe the clinical course of a pediatric patient presenting with acute auditory hallucinations with workup revealing a new diagnosis of NF2. 

NF2 is an autosomal dominant neurocutaneous disorder characterized by vestibular schwannomas, meningiomas, neurofibromas, gliomas, cerebral calcifications, cataracts, and cognitive impairment. Acute onset psychosis is an uncommon sequela of NF2, and if present, is usually attributed to the known prior history of NF2. However, here we present a case of acute psychosis that precipitated the initial diagnosis of NF2. 

A 13-year-old Hispanic male with no known intracranial tumors or other neurocutaneous stigmata presented with acute-onset psychosis, agitation, delusions, and painless monocular vision loss. Upon further review, he was reported to have a remote history of morning headaches, right upper extremity weakness and paresthesias thought to be due to a trampoline accident, and multiple dermal lesions that were diagnosed as pilonidal cysts despite biopsy and sectioning. He presented with complaints of hearing a constant “airplane sound,” poor sleep, agitation, persecutory delusions and paranoia, pressured speech, and discontinuity of thought. On exam, he was agitated and not oriented to situation, responding rapidly to questions with the incorrect answer, hyperkinetic, and generally distressed and labile. MRI brain with and without contrast demonstrated bilateral enhancing vestibular schwannomas, a meningioma, and nerve sheath tumor, all radiographically diagnostic for NF2. Though he was initially treated empirically for encephalitis with steroids, he improved rapidly upon initiation of antipsychotics; all other workup for the psychosis was unremarkable and a pathogenic variant of NF2 was confirmed. 

It is important to look for stigmata of NF2 in patients with psychosis as bilateral vestibular schwannomas can cause auditory hallucinations secondary to continuous tinnitus. Early identification of NF2 will lead to targeted therapeutic/surgical intervention that may relieve cranial nerve compression and thus reduce the odds of developing psychosis.