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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Ganglionic hamartoma of the intracanalicular acoustic nerve causing sensorineural hearing loss.

OBJECTIVE: This article highlights the clinical presentation and treatment issues of ganglionic hamartoma of the internal auditory canal and emphasizes the similarity of this lesion to acoustic neuroma regarding its audiologic and radiographic characteristics. STUDY DESIGN: This article is composed of case reports and a literature review. SETTING: The study was performed at a university hospital/tertiary referral center. PATIENT: A patient with biopsy-proven ganglionic hamartoma of the acoustic nerve was studied. INTERVENTION: Intervention consisted of surgical therapy. MAIN OUTCOME MEASURE: The main outcome measure was clinical evaluation. RESULTS: The result was successful removal of lesions with facial nerve preservation. CONCLUSIONS: An intracanalicular ganglionic hamartoma resulted in progressive sensorineural hearing loss and magnetic resonance imaging findings suggestive of small acoustic neuroma. This lesion, composed of an admixture of ganglion cells, fibroadipose-tissue, and normal myelinated axons, although rare, should be added to the differential diagnosis of internal auditory canal lesions.[1]


  1. Ganglionic hamartoma of the intracanalicular acoustic nerve causing sensorineural hearing loss. Hung, T.Y., Litofsky, N.S., Smith, T.W., Megerian, C.A. The American journal of otology. (1997) [Pubmed]
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