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Gorham–Stout Disease of the Skull Base With Hearing Loss: Dramatic Recovery and Antiangiogenic Therapy
Author(s) -
Nozawa Akifumi,
Ozeki Michio,
Kuze Bunya,
Asano Takahiko,
Matsuoka Kentaro,
Fukao Toshiyuki
Publication year - 2016
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.25886
Subject(s) - medicine , hearing loss , skull , etiology , cerebrospinal fluid , rhinorrhea , surgery , pathology , audiology
Gorham–Stout disease (GSD) is a rare disorder of unknown etiology. We present a 6‐year‐old male with GSD involving the skull base who presented with recurrent cerebrospinal fluid (CSF) rhinorrhea, severe hearing loss, and facial palsy secondary to cerebellar herniation into the internal auditory canal. After 2 months of treatment with pegylated interferon (IFN) α‐2b (50 μg/week), his hearing recovered dramatically. Two years later, new bone formation appeared radiologically and IFN was switched to sirolimus. One year after the switch, CSF rhinorrhea disappeared. Antiangiogenic therapy might inhibit proliferation of vascular endothelial cells in osteolytic lesions and lead to new bone formation.