A Case of Neurosarcoidosis with Labyrinthine Involvement

Sarcoidosis is a chronic granulomatous disease of unknown aetiology, which may involve any organ system. It most commonly occurs in adults with childhood involvement being rare. Central nervous system involvement is seen in up to 25% and typically involves meningeal disease resulting in multiple cranial neuropathies. Other common clinical findings include seizures, headache, dementia, and pituitary dysfunction. Imaging plays a central role in the diagnosis with typical findings including pachymeningeal and leptomeningeal enhancing lesions. Other imaging findings include lacunar and major territory infarcts, hypothalamic and infundibular thickening, hydrocephalus, and cranial nerve enhancement. We present a case of an eight-year-old male patient with progressive headache, visual disturbance, unilateral sensory hearing loss, and multiple cranial neuropathies. Imaging findings demonstrated the classic pachymeningeal and leptomeningeal enhancement along much of the skull base, as well as enhancement of the right and left second and eighth cranial nerves. Extensive inflammatory changes were noted in the temporal bones and paranasal sinuses. There was also enhancement of the right and left labyrinths. Sinus biopsy confirmed sarcoidosis. We present the first case to our knowledge of sarcoid labyrinthitis. 1. Introduction Sarcoidosis is a chronic granulomatous disease of unknown aetiology with the presence of noncaseating granulomas [1]. It may involve any organ system, although the lungs and lymphatic system are among the more common sites. Sarcoidosis typically affects adults, with bimodal peaks in the third decade and sixth decade [1]. It is uncommon in childhood. There is an increased risk in people of African ethnicity [2], although this seems to be more prevalent in those living in northern climates. Isolated neurosarcoidosis is rare [3]. In postmortem series of patients with sarcoidosis, central nervous system involvement is seen in 14–25% [4–7]. Symptomatic cases are however reported with a much lower frequency (3%–5%) [8, 9]. This suggests that CNS involvement is more common than clinically apparent. Depending on the organ systems involved, clinical findings may vary widely. With central nervous system involvement, symptoms include those of cranial neuropathy, particularly the facial nerve and the optic nerve. Other symptoms include seizures, headache, dementia, weakness, parasthesia, and pituitary/hypothalamic dysfunction [10]. 2. Case Report We present a case of an eight-year-old male with a progressive history of headache, neck pain, and