A 28-year-old woman presents with progressive bilateral asymmetric sensorineural hearing loss and tinnitus over the past 2 years. Gadolinium-enhanced MRI brain shows bilateral cerebellopontine angle (CPA) enhancing masses. The structure marked **A** (right CPA enhancing mass) is a vestibular schwannoma. Which of the following genetic and pathophysiological features is PATHOGNOMONIC for the underlying diagnosis in this patient?
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