Ahsan, Syed F

Kandinov, Aron

Smith, Matthew

English

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Henry Ford Health System Henry Ford Health System Scholarly Commons Otolaryngology Articles Otolaryngology - Head and Neck Surgery 5-1-2017 Middle Ear Mass Causing Vertigo and Facial Nerve Weakness. Aron Kandinov Matthew Smith Henry Ford Health System Syed F. Ahsan Henry Ford Health System Follow this and additional works at: https://scholarlycommons.henryford.com/otolaryngology_articles Recommended Citation Kandinov A, Smith M, and Ahsan SF. Middle ear mass causing vertigo and facial nerve weakness JAMA Otolaryngol Head Neck Surg 2017 May 1;143(5):515-516. This Article is brought to you for free and open access by the Otolaryngology - Head and Neck Surgery at Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Otolaryngology Articles by an authorized administrator of Henry Ford Health System Scholarly Commons. Middle EarMass Causing Vertigo and Facial NerveWeaknessAron Kandinov, BS; Matthew Smith, MD; Syed F. Ahsan, MDAn elderly woman presented with 2 episodes of vertigo lasting a few minutes while sta-tionary over 1 day and a several-year history of right-sided hearing loss. She had not expe-riencedotalgia, otorrhea, or tinnitus. Physical examination revealedamassobstructing theright external auditory canal and House-Brackmann grade 2 facial nerve weakness on theright. Audiogram revealed right-sided moderate to profound mixed hearing loss. Com-puted tomography revealeda soft-tissuemassemanating fromthemiddleear involving thefacial canal with surrounding bony erosion, including dehiscence of the tegmen, cochlea,bony labyrinth, lateral semicircular canal, and jugular foramen.Thepatientunderwent trans-temporalmastoidectomyanddebulking.However, owing to themass’s vascularity and sig-nificant bleeding, the operation was aborted, and she was sent for embolization (Figure,A).Magnetic resonance imagingduringembolization revealed intenseenhancement in theright middle ear extending from the epitympanum to the hypotympanumwith extensioninto the right external auditory canal (Figure, B). She returned to the operating room, andthe vascular mass was found to extend inferiorly to the jugular bulb and carotid and ante-riorly into the eustachian tube. Themasswas adherent to the underlying bone and had in-vaded the facial canal but not the nerve itself. Bothmalleus and incuswere eroded. Immu-nohistochemical stains of the specimen indicated cells positive for synaptophysin with asustentacular pattern of cells positive for S-100 (Figure, C and D).Intraoperative photographA Axial MRI imageBHistopathologic imageC Histopathologic imageDFigure. A, Mastoidectomy, visualization of vascular mass. B, T1-weighted axial magnetic resonance image(MRI) showing enhancement of right middle ear extending to external auditory canal. C, Hematoxylin-eosin,original magnification ×40. Clusters of cells surrounded by vascular tissue. D, Synaptophysin stain, originalmagnification ×40. Nests of strongly staining neuroendocrine cells separated by vasculature.WHAT IS YOURDIAGNOSIS?A. Middle ear paragangliomaB. Facial nerve hemangiomaC. Facial nerve schwannomaD. Middle ear adenomaClinical Review& EducationClinical Challenge | PATHOLOGYjamaotolaryngology.com (Reprinted) JAMAOtolaryngology–Head&Neck Surgery May 2017 Volume 143, Number 5 515© 2017 American Medical Association. All rights reserved.Downloaded From: https://jamanetwork.com/ by a Henry Ford Health System User  on 12/19/2019DiagnosisA.Middle ear paragangliomaDiscussionMiddleearparagangliomasareparasympatheticneoplasmsofnaturallyoccurringparaganglionicbodies.Paragangliomasareslow-growing, in-vasive,vascularizedneoplasmsarisingfromeitherthemiddleearorthejugular bulb, with a small percentage derived from the facial nerve.1Middleearparagangliomaspredominate inwomen,usuallypresentinginthefifthandsixthdecadeswithbiphasicpeaks inthefourthandsev-enthdecades.2,3Thoughmostgounreported,theincidenceofheadandneckparagangliomashasbeenestimatedat 1per 100000.2,4Mostofthesearesporadic,although10%to30%arefamilialandassociatedwithmutations in the succinatedehydrogenase complex.3,5The hallmark presentation for middle ear paraganglioma in-cludes pulsatile tinnitus (81%-90%), hearing loss (77%-81%), andaural fullness (12%-71%).6,7 They often abut or invade nearby cra-nial nerves, with patients occasionally presenting with facial pare-sis (0%-3%), dysphasia, hoarseness, and even shoulder or tongueweakness.6,7 Physical examination may reveal a fleshy redmass inthe middle ear that blanches secondary to pressure from pneu-maticotoscopy (Brownsign).Biopsy is contraindicatedowing to themass’s vascularity and possible catecholamine secretion.Parasympathetic paragangliomas in general have a low inci-dence of catecholamine secretion, with only 1% to 3% of patientspresenting with symptoms of catecholamine excess.3,8 Patientsshould nevertheless be questioned regarding symptoms of cat-echolamine secretion such as palpitations, headaches, and hyper-tension. While all suspicions for middle ear paraganglioma shouldbe tested for urinemetanephrine and vanillylmandelic acid preop-eratively,positive results shouldprompt investigation forpheochro-mocytomaowingto the low incidenceof functionalglomustumors.2Serum epinephrine and norepinephrine levels are also useful in di-agnosis,asparasympatheticparagangliomas lacktheenzymepheny-lethanolamine N-methyltransferase, which converts norepineph-rine to epinephrine in their sympathetic counterparts.Computed tomographywith contrast classically demonstratesa homogeneous mass with intense enhancement, and is particu-larly sensitive for signs of bony involvement. Magnetic resonanceimaging with gadolinium enhances soft-tissue detail, resulting in alower false-negative rate9 and better delineating dural and neuro-vascular involvement.Magnetic resonance imagesdemonstrateT1-isointense signaling and T2-hyperintense signaling with a possible“salt andpepper”pattern in larger lesionsowing to flowvoids.8Mag-netic resonance angiography can further delineate the relationshipto thecarotidartery, sigmoid sinusand jugularbulb, and identifypo-tential blood supply—often the ascending pharyngeal artery in thecase of jugular bulb paragangliomas. The differential diagnosis fora vascular lesion near the geniculate ganglion includes facial nervehemangioma, which also presents with significant facial nerve pa-resis and bony erosion disproportionate to its size.Histologically, paragangliomas present as nests of chief cells—polygonal cells with round nuclei and granular, eosinophilic cyto-plasm—surrounded by spindle-shaped sustentacular cells in aZellballen pattern.3 Higher-grade tumors have been shown to pro-gressively lose the relationship between the 2 cell types.10 Immu-nohistochemical stainingof chief cells demonstrates positive stain-ing for neuroendocrine markers, such as neuron-specific enolase,synaptophysinand/or chromogranin, andnegative staining forkera-tins. This lack of keratin immunostaining in paragangliomas distin-guishes them frommiddle ear adenomas. Sustentacular cells typi-cally stain positive for S-100.Middle ear paragangliomas are often locally destructive butrarely malignant. Although features such as an increase in mitoticactivity, central necrosis and vascular invasion may increase suspi-cion of malignancy, only metastasis to a lymph node or distant or-gan confirms malignancy.2 Surgical resection is indicated owing totheir progressive growth pattern and involvement ofmajor vesselsand cranial nerves, although radiotherapy presents a growingoption.6While preoperative embolizationmay be helpful in reduc-ing bleeding, our practice has also found the use of carbon dioxidelaser extremely helpful in achieving hemostasis.ARTICLE INFORMATIONAuthor Affiliations:Department ofOtolaryngology–Head and Neck Surgery, WayneState University School of Medicine, Detroit,Michigan (Kandinov); Department ofOtolaryngology–Head and Neck Surgery, HenryFord Health System, Detroit, Michigan (Smith,Ahsan).Corresponding Author: Aron Kandinov, BS,Department of Otolaryngology–Head and NeckSurgery, Wayne State University School ofMedicine, 4201 St Antoine, 5E-UHC, Detroit, MI48201 (akandino@med.wayne.edu).Section Editor: Edward B. Stelow, MD.Published Online: January 5, 2017.doi:10.1001/jamaoto.2016.3222Conflict of Interest Disclosures: All authors havecompleted and submitted the ICMJE Form forDisclosure of Potential Conflicts of Interest. Nodisclosures were reported.REFERENCES1. Bartels LJ, Pennington J, Kamerer DB, BrowarskyI. Primary fallopian canal glomus tumors.Otolaryngol Head Neck Surg. 1990;102(2):101-105.2. McNicol AM. Update on tumours of the adrenalcortex, phaeochromocytoma and extra-adrenalparaganglioma.Histopathology. 2011;58(2):155-168.3. Sweeney AD, CarlsonML, Wanna GB, BennettML. Glomus tympanicum tumors.Otolaryngol ClinNorth Am. 2015;48(2):293-304.4. Oosterwijk JC, Jansen JC, van Schothorst EM,et al. First experiences with genetic counsellingbased on predictive DNA diagnosis in hereditaryglomus tumours (paragangliomas). J Med Genet.1996;33(5):379-383.5. Dannenberg H, DinjensWN, AbbouM, et al.Frequent germ-line succinate dehydrogenasesubunit D genemutations in patients withapparently sporadic parasympatheticparaganglioma. Clin Cancer Res. 2002;8(7):2061-2066.6. Fayad JN, Keles B, Brackmann DE. Jugularforamen tumors.Otol Neurotol. 2010;31(2):299-305.7. CarlsonML, Sweeney AD, Pelosi S, Wanna GB,Glasscock ME III, Haynes DS. Glomus tympanicumOtolaryngol Head Neck Surg. 2015;152(1):136-142.8. Hu K, Persky MS. Multidisciplinary managementof paragangliomas of the head and neck, part 1.Oncology (Williston Park). 2003;17(7):983-993.9. Erickson D, Kudva YC, Ebersold MJ, et al. Benignparagangliomas. J Clin Endocrinol Metab. 2001;86(11):5210-5216.10. Kliewer KE, Cochran AJ. A review of thehistology, ultrastructure, immunohistology, andmolecular biology of extra-adrenal paragangliomas.Arch Pathol Lab Med. 1989;113(11):1209-1218.Clinical Review& Education Clinical Challenge516 JAMAOtolaryngology–Head&Neck Surgery May 2017 Volume 143, Number 5 (Reprinted) jamaotolaryngology.com© 2017 American Medical Association. All rights reserved.Downloaded From: https://jamanetwork.com/ by a Henry Ford Health System User  on 12/19/2019

Middle Ear Mass Causing Vertigo and Facial Nerve Weakness

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Middle Ear Mass Causing Vertigo and Facial Nerve Weakness

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