Snapshot A 44-year-old man presents to his primary care physician for worsening hearing loss. His hearing loss is most severe in his right ear. He has noticed decreased facial sensation on the right side of the face. Physical examination is notable for a sensorineural hearing loss affecting the right ear and decreased sensation to pin-prick in the right face. An MRI brain demonstrates an enlarged mass in the internal acoustic meatus, extending to the cerebellopontine angle. Introduction Overview a Schwann cell-derived tumor arising from the 8th cranial nerve typically the vestibular portion usually unilateral (~90% of cases) Epidemiology demographics median age is ~50 years Associated conditions neurofibromatosis type 2 characteristically presents as bilateral acoustina neuromas secondary to a mutation in merlin in chromosome 22 Presentation Symptoms/physical exam cochlear nerve involvement sensorineural hearing loss tinnitus vestibular nerve involvement unsteady gait can have other cranial nerve involvement facial and trigeminal nerve involvement facial nerve facial paresis taste disturbaces trigeminal nerve facial paresthesia and pain Imaging MRI brain with gadolinium indication imaging study of choice fundings enhancing lesion in the internal auditory canal that may extend into the cerebellopontine angle Studies Audiometry indication best initial screening laboratory test Differential Neurofibromatosis type 2 differentiating factors associated with bilateral acoustic neuromas, cataracts, meningiomas, and ependymomas Meningioma differenting factors tumor involving the meninges only Treatment Medical radiation therapy indication small tumors or patients who are not surgical candidates options sterotactic radiosurgery sterotactic radiotherapy Surgical microsurgical tumor removal indication large tumors or rapidly growing tumors Complications Hearing loss Brainstem compression
QUESTIONS 1 of 4 1 2 3 4 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M2.ON.16.4694) A 45 year old male presents to clinic complaining of a ringing in his ears. The patient's vitals are: BP 120/65, RR 16, 100% on room air, HR 78 bpm and he appears to be in no acute distress. Upon obtaining a history you learn that this patient also complains that at times the "room feels like it's spinning." You also notice throughout the interview that you have to speak loudly as the patient has difficulty hearing. The patient is then lost to follow up for his current condition. The patient returns several years later with worsening symptoms and as part of this patient's workup a MRI is obtained (Image A). Which of the following chromosomes is most likely mutated in association with this patient's condition? QID: 107884 FIGURES: A Type & Select Correct Answer 1 Chromosome 13 0% (0/1) 2 Chromosome 7 100% (1/1) 3 Chromosome 17 0% (0/1) 4 Chromosome 22 0% (0/1) 5 The X chromosome 0% (0/1) M 6 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept