Skull Base Rep 2011; 1(2): 089-094
DOI: 10.1055/s-0031-1276722
© Thieme Medical Publishers

Ganglioneuroma of the Internal Auditory Canal Presenting as a Vestibular Schwannoma

Kimon Bekelis1 , Duncan A. Meiklejohn2 , Symeon Missios1 , Brent Harris3 , James E. Saunders2 , Kadir Erkmen1
  • 1Section of Neurosurgery, Dartmouth–Hitchcock Medical Center, Lebanon, New Hampshire
  • 2Section of Ear Nose and Throat Surgery, Dartmouth–Hitchcock Medical Center, Lebanon, New Hampshire
  • 3Section of Pathology, Dartmouth–Hitchcock Medical Center, Lebanon, New Hampshire
Weitere Informationen


11. April 2011 (online)


In most series, 90% of cerebellopontine angle tumors are vestibular schwannomas. Meningiomas and epidermoid tumors follow with decreased frequency. Ganglioneuroma is a benign tumor usually found in the retroperitoneum and posterior mediastinum. We report a case of a 21-year-old man with gradual sensorineural hearing loss and a minimally enhancing lesion of the internal auditory canal, which was excised through a middle fossa approach and found histologically to be a ganglioneuroma. Like vestibular schwannomas, these lesions are benign in nature and may be managed in a similar fashion, although the possibility of malignant transformation may support surgical resection over conservative management or radiosurgery. Ganglioneuromas should be considered in patients with atypical radiographic findings for vestibular schwannomas.


  • 1 Geoerger B, Hero B, Harms D, Grebe J, Scheidhauer K, Berthold F. Metabolic activity and clinical features of primary ganglioneuromas.  Cancer. 2001;  91 1905-1913
  • 2 Scheithauer B W, Woodruff J M, Erlandson R A. Ganglioneuroma. In: Tumors of the Peripheral Nervous System. Bethesda, MD: Armed Forces Institute of Pathology; 1999: 259
  • 3 Slattery III W H, Brackmann D E, Hitselberger W. Middle fossa approach for hearing preservation with acoustic neuromas.  Am J Otol. 1997;  18 596-601
  • 4 Abe T, Asano T, Manabe T, Matsuura H, Furuta T, Taguchi K. Trigeminal ganglioneuroma.  Brain Tumor Pathol. 1999;  16 49-53
  • 5 Brouwers F M, Eisenhofer G, Lenders J W, Pacak K. Emergencies caused by pheochromocytoma, neuroblastoma, or ganglioneuroma.  Endocrinol Metab Clin North Am. 2006;  35 699-724 viii
  • 6 Estefano J, Algaba J, Gorostiaga F, Urruticoechea A, Hernández J M. Ganglioneuroma of the middle ear. Apropos of a case.  An Otorrinolaringol Ibero Am. 1992;  19 5-12
  • 7 Kaufman M R, Rhee J S, Fliegelman L J, Costantino P D. Ganglioneuroma of the parapharyngeal space in a pediatric patient.  Otolaryngol Head Neck Surg. 2001;  124 702-704
  • 8 Lonergan G J, Schwab C M, Suarez E S, Carlson C L. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation.  Radiographics. 2002;  22 911-934
  • 9 Ozluoglu L N, Yilmaz I, Cagici C A, Bal N, Erdogan B. Ganglioneuroma of the internal auditory canal: a case report.  Audiol Neurootol. 2007;  12 160-164
  • 10 Radin R, David C L, Goldfarb H, Francis I R. Adrenal and extra-adrenal retroperitoneal ganglioneuroma: imaging findings in 13 adults.  Radiology. 1997;  202 703-707
  • 11 Palmer C A, Townsend J J, Harnsberger H R, Parkin J L, Apfelbaum R I. Cerebellopontine angle glioneuronal hamartoma.  Surg Neurol. 1996;  45 467-469
  • 12 Ambros I M, Zellner A, Roald B et al.. Role of ploidy, chromosome 1p, and Schwann cells in the maturation of neuroblastoma.  N Engl J Med. 1996;  334 1505-1511
  • 13 Miura K, Mineta H, Yokota N, Tsutsui Y. Olfactory neuroblastoma with epithelial and endocrine differentiation transformed into ganglioneuroma after chemoradiotherapy.  Pathol Int. 2001;  51 942-947
  • 14 Ichikawa T, Ohtomo K, Araki T et al.. Ganglioneuroma: computed tomography and magnetic resonance features.  Br J Radiol. 1996;  69 114-121
  • 15 Rha S E, Byun J Y, Jung S E, Chun H J, Lee H G, Lee J M. Neurogenic tumors in the abdomen: tumor types and imaging characteristics.  Radiographics. 2003;  23 29-43
  • 16 Califano L, Zupi A, Mangone G M, Long F. Cervical ganglioneuroma: report of a case.  Otolaryngol Head Neck Surg. 2001;  124 115-116
  • 17 Leonardis M, Sperb D, Alster C, Campisi C, Herter N T. Ganglioneuroma of the neck, masquerading as a goiter.  Eur J Surg Oncol. 2003;  29 929-930
  • 18 Hillman T, Chen D A, Arriaga M A, Quigley M. Facial nerve function and hearing preservation acoustic tumor surgery: does the approach matter?.  Otolaryngol Head Neck Surg. 2010;  142 115-119
  • 19 Irving R M, Jackler R K, Pitts L H. Hearing preservation in patients undergoing vestibular schwannoma surgery: comparison of middle fossa and retrosigmoid approaches.  J Neurosurg. 1998;  88 840-845
  • 20 Stárek I, Mihál V, Novák Z, Pospísilová D, Vomácka J, Vokurka J. Pediatric tumors of the parapharyngeal space. Three case reports and a literature review.  Int J Pediatr Otorhinolaryngol. 2004;  68 601-606
  • 21 Carvalho G A, Matthies C, Osorio E, Samii M. Hamartomas of the internal auditory canal: report of two cases.  Neurosurgery. 2003;  52 944-948 discussion 948-949
  • 22 Gonul E, Yetiser S, Tasar M, Ongoru O. Glioneural hamartoma of the VIIIth nerve.  J Laryngol Otol. 2006;  120 70-73
  • 23 Pucci A, Pucci E, Santini F et al.. A ganglioneuroma with features of a thyroid nodule: intense pain on fine needle biopsy as a diagnostic clue.  Thyroid. 2009;  19 201-204

Kadir ErkmenM.D. 

Section of Neurosurgery, Dartmouth-Hitchcock Medical Center

One Medical Center Drive, Lebanon, NH 03756