Isolated Sensorineural Hearing Loss as Initial Presentation of Recurrent Medulloblastoma: Neuroimaging and Audiologic Co
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Correspondence
Isolated Sensorineural Hearing Loss as Initial Presentation of Recurrent Medulloblastoma: Neuroimaging and Audiologic Correlates C. S. Amene · L. A. Yeh-Nayre · J. R. Crawford
Received: 13 August 2012 / Accepted: 5 November 2012 © Springer-Verlag Berlin Heidelberg 2012
Introduction Medulloblastoma represents 13 % of all childhood primary brain tumors ages 0–14 years and is the most common malignant tumor of childhood with an incidence of 0.51 per 100,000 person per year [1]. Children diagnosed with average-risk medulloblastoma have been reported by several groups to have 5-year survivals greater than 85 % following maximum surgical resection, craniospinal radiation, and adjuvant chemotherapy [2, 3]. Patterns of medulloblastoma recurrence may be both local as well as distant and are frequently discovered by worsening symptomatology or by findings on routine neuroimaging. We report a case of a young adult with multiple recurrent medulloblastoma presenting with isolated right-sided sensorineural hearing loss confirmed by audiometry as the only clinical feature of disease recurrence. Magnetic resonance imaging (MRI) revealed restricted diffusion of the cranial nerve VIII (CNVIII) complex that correlated with contrast-enhancing metastatic disease. This unique pattern of medulloblastoma recurrence is important to recognize and highlights
Assist. Prof. J. R. Crawford, MD, MS () The Department of Neurosciences, Division of Child Neurology, University of California San Diego, Rady Childrens Hospital San Diego, 8010 Frost Street Suite 400, San Diego, CA 92123, USA e-mail: [email protected] C. S. Amene, MD The Department of Neurosurgery, University of California San Diego, Rady Childrens Hospital San Diego, San Diego, CA 92123, USA L. A. Yeh-Nayre, MSN, PNP · Assist. Prof. J. R. Crawford, MD, MS The Department of Pediatrics, University of California San Diego, Rady Childrens Hospital San Diego, San Diego, CA 92123, USA
the importance of diffusion-weighted imaging (DWI) in the routine disease surveillance of patients diagnosed with medulloblastoma. Case Report A 21-year-old male diagnosed with medulloblastoma at the age of 13 years presented on routine follow-up with a chief complaint of tinnitus and decreased hearing in the right ear more than a 2-week period. His treatment at initial diagnosis included craniospinal radiation and standard chemotherapy (vincristine, cisplatin, etoposide, and cyclophosphamide). At the age of 17 years, he had a recurrence characterized by diffuse leptomeningeal disseminated disease and was treated with a variety of chemotherapy and biologics that resulted in stable disease until the current presentation. On examination, he had decreased hearing to both finger rub and whisper in the right ear without vertigo or facial weakness that was a change from his previous baseline neurologic examination of minor difficulties with tandem straight-line testing. An audiogram was performed (Fig. 1) that showed a significant right-sided sensorineural hearing loss across multiple fre
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