Introduction
Neurofibromatosis Type 2 (NF2) is an autosomal-dominant tumor-prone
disorder characterized by the development of distinctive nervous system
tumors, including meningiomas, ependymomas and peripheral, spinal and
cranial nerve schwannomas, in addition to skin anomalies and visual
symptoms. Bilateral vestibular schwannomas are pathognomonic. So, the
most common entry in the disease is through hearing impairment, which
usually occurs in the second decade of life. But, unlike adults,
children most frequently present with ocular, dermatological, and
neurological symptoms. Brainstem ischemic syndrome, which is an
under-recognized entity of unknown origin, occurs in teenagers without
any previously known NF2 diagnosis, presenting as an acute or subacute
event, which involves the midbrain or pons.