Rosai-Dorfman disease associated with neurosensorial hearing loss in two siblings.
Rosai-Dorfman disease (RDD) is an uncommon pathologic condition of unknown ethiology with an idiopathic proliferation of the hystiocytes. It is generally presented with massive bilateral hypertrophy of the cervical lymph nodes. But other lymph nodes may also be involved. Approximately, 30% of these patients have extra nodal mass or lesion with different signs or symptoms depending on localization. We present two male siblings with Rosai-Dorfman disease who have classical cervical lymphadenopathy associated with progressive neurosensorial hearing loss and dural-based intracranial lesions.
Yetiser
Sertac
S
Department of ORL & HNS, Gulhane Medical School, 06018 Etlik, Ankara, Turkey. syetiser@yahoo.com
Cekin
Engin
E
Tosun
Fuat
F
Yildirim
Altan
A
D000158:Acoustic Impedance Tests; D001300:Audiometry, Evoked Response; D001301:Audiometry, Pure-Tone; D001927:Brain Diseases / Q000150:complications*; D002648:Child; D004388:Dura Mater / Q000473:pathology; D016057:Evoked Potentials, Auditory, Brain Stem; D006319:Hearing Loss, Sensorineural / Q000150:complications* / Q000175:diagnosis; D015618:Histiocytosis, Sinus / Q000150:complications*; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology*; D008206:Lymphatic Diseases / Q000150:complications*; D008279:Magnetic Resonance Imaging; D008297:Male; D010256:Paranasal Sinuses / Q000473:pathology