The basic components of the epithelial, perineural and perivascular basement membranes in the inner ear have been well-documented in several animal models and in the human inner ear. The von Willebrand A domain-related protein (WARP) is an extracellular matrix molecule with restricted expression in cartilage, and a subset of basement membranes in peripheral nerves, muscle and central nervous system vasculature. It has been suggested that WARP have an important role in maintaining the blood-brain barrier. To date no studies on WARP distribution have been performed in the inner that is equipped with an intricate vasculature network. In the present study we determined the distribution of WARP by immunocytochemistry in the human inner ear using auditory and vestibular endorgans microdissected from human temporal bones obtained at autopsy. All subjects (n=5, ages 55-87 years old) had documented normal auditory and vestibular function. We also determined the WARP immunolocalization in the mouse inner ear. WARP-immunoreactivity localized to the vasculature throughout the stroma of the cristae ampullaris, the maculae utricle and saccule in the human and mouse. In the human and mouse inner ear, WARP-immunoreactivity delineated blood vessels located in the stria vascularis, spiral ligament, sub-basilar region, stromal tissue, and the spiral and vestibular ganglia. The distinct localization of WARP in the inner ear vasculature suggests an important role in maintaining its integrity. In addition, WARP allows delineation of microvessels in the inner ear allowing the study of vascular pathology in the development of otological diseases.

A 49-YEAR-OLD MAN WITH A HISTORY OF SCHIzoaffective disorder and bipolar disorder presented with nasal airway obstruction, years of intermittent epistaxis, and decreased libido. He denied visual changes, headaches, ear infections, taste disturbance, and anosmia. Physical examination revealed a large bulge in his nasopharynx that effectively reduced his nasopharyngeal airway (Figure 1). He was also found to have gynecomastia and decreased testicular size. Magnetic resonance imaging of his brain demonstrated a normal pituitary gland and a large midline nasopharyngeal mass (Figure 2). An endoscopic nasopharyngeal biopsy was then performed.Initial histopathologic examination demonstrated sheets of small to medium-sized, round, blue cells arranged in a solid pattern. The sheets of blue cells were located deeper to the normal-appearing nasopharyngeal mucosa, which was evident on the periphery of the biopsy specimen (Figure 3). Additional stains were diffusely positive for prolactin hormone (Figure 4).