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Hizli, Omer/0000-0001-6822-2679; (Bingol) Hizli, Pelin/0000-0001-8919-3125 WOS: 000383312600004 PubMed: 26860231 Objective: To observe any changes in stria vascularis and cochlear hair cells in patients with syphilis. Materials and methods: We examined 13 human temporal bone samples from 8 patients with syphilis (our syphilis group), as well as 12 histopathologically normal samples from 9 age-matched patients without syphilis (our control group). We compared, between the two groups, the mean area of the stria vascularis (measured with conventional light microscopy connected to a personal computer) and the mean percentage of cochlear hair cell loss (obtained from cytocochleograms). Results: In our syphilis group, only 1 (7.7%) of the 13 samples had precipitate in the endolymphatic or perilymphatic spaces; 8 (61.5%) of the samples revealed the presence of endolymphatic hydrops (4 cochlear, 4 saccular). The mean area of the stria vascularis did not significantly differ, in any turn of the cochlea, between the 2 groups (P > 0.1). However, we did find significant differences between the 2 groups in the mean percentage of outer hair cells in the apical turn (P < 0.026) and in the mean percentage of inner hair cells in the basal (P = 0.001), middle (P = 0.004), and apical (P = 0.018) turns. In 7 samples in our syphilis group, we observed either complete loss of the organ of Corti or a flattened organ of Cord without any cells in addition to the absence of both outer and inner hair cells. Conclusion: In this study, syphilis led either to complete loss of the organ of Corti or to significant loss of cochlear hair cells, in addition to cochleosaccular hydrops. But the area of the stria vascularis did not change. (C) 2016 Elsevier Ireland Ltd. All rights reserved. NIH (NIDCD)United States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute on Deafness & Other Communication Disorders (NIDCD) [U24 DC011968-01]; International Hearing Foundation; Starkey Foundation; 5M Lions International; Scientific and Technological Research Council of Turkey (TUBITAK) ScholarshipTurkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK) This project was funded by NIH (NIDCD), grant number U24 DC011968-01; the International Hearing Foundation; the Starkey Foundation; and the 5M Lions International. Omer Hizh and Serdar Kaya received the Scientific and Technological Research Council of Turkey (TUBITAK) Scholarship.

Monsanto, Rafael/0000-0002-9124-593X; Hizli, Omer/0000-0001-6822-2679; da Costa Monsanto, Rafael/0000-0002-9124-593X; (Bingol) Hizli, Pelin/0000-0001-8919-3125 WOS: 000381264100021 PubMed: 26573155 To the best of our knowledge, histopathologic studies of syphilitic ears have generally focused on hydropic changes; so far, no such studies have investigated peripheral vestibular otopathology using differential interference contrast microscopy, in patients with syphilis. For this study, we examined 13 human temporal bone samples from 8 patients with a history of syphilis. Using conventional light microscopy, we performed qualitative histopathologic assessment. In addition, using differential interference contrast microscopy, we performed type I and type II vestibular hair cell counts on each vestibular sense organ with minimal autolysis; in which the neuroepithelium was oriented perpendicular to the plane of section. We then compared vestibular hair cell densities (cells per 0.01 mmA(2) surface area) in the syphilis group vs. the control group. In the syphilis group, we observed precipitate in the endolymphatic or perilymphatic spaces in 1 (7.7 %) of the samples and endolymphatic hydrops in eight (61.5 %) of the samples. Hydrops involved the cochlea (four samples) and/or saccule (four samples). In addition, the syphilis group experienced a significant loss of type II vestibular hair cells in the maculae of the utricle and saccule, and in the cristae of the lateral and posterior semicircular canals, as compared with the control group (P < 0.05). NIH (NIDCD)United States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute on Deafness & Other Communication Disorders (NIDCD) [U24 DC011968-01]; International Hearing Foundation; Starkey Hearing Foundation; 5M Lions International; Scientific and Technological Research Council of Turkey (TUBITAK)Turkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK) This project was funded by NIH (NIDCD), Grant number U24 DC011968-01; the International Hearing Foundation; the Starkey Hearing Foundation; and the 5M Lions International. Omer Hizli and Serdar Kaya received the Scientific and Technological Research Council of Turkey (TUBITAK) Scholarship

Hizli, Omer/0000-0001-6822-2679; (Bingol) Hizli, Pelin/0000-0001-8919-3125 WOS: 000377194300031 PubMed: 27050650 Hypothesis: We hypothesized that, in archived human temporal bone samples from patients with systemic lupus erythematosus (SLE), a pathologic condition exists in the stria vascularis and cochlear hair cells. Background: Sensorineural hearing loss is a common feature in SLE patients. However, the pathophysiologic mechanism of cochlear dysfunction is unclear. Methods: We examined 15 temporal bone samples from 8 SLE patients, along with 17 samples from 10 age-matched healthy control patients. The samples were serially sectioned in the horizontal plane and stained with hematoxylin and eosin. We determined the area of the stria vascularis in a midmodiolar section of each cochlear turn. Then, we made cytocochleograms and calculated the percentage of missing inner and outer hair cells. Results: The area of the stria vascularis in our SLE group was significantly smaller than in our control group. The number of remaining inner hair cells in our SLE group was smaller than in our control group; however, the difference did not reach statistical significance. The loss of outer hair cells in our SLE group was significantly higher than in our control group. There was a tendency toward a positive correlation between the loss of cochlear hair cells and the duration of SLE. Conclusion: The stria vascularis and cochlear hair cells are affected in SLE patients. Our findings could provide the histopathologic basis for the cochlear dysfunction, including sensorineural hearing loss, experienced by SLE patients. National Institute on Deafness and Other Communication Disorders (NIDCD)United States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute on Deafness & Other Communication Disorders (NIDCD) [U24 DC011968-01]; International Hearing Foundation; Starkey Hearing Foundation; 5M Lions International; Scientific and Technological Research Council of Turkey (TUBITAK)Turkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK) This project was funded by the National Institute on Deafness and Other Communication Disorders (NIDCD), grant number U24 DC011968-01; the International Hearing Foundation; the Starkey Hearing Foundation; the 5M Lions International; and the Scientific and Technological Research Council of Turkey (TUBITAK) Scholarship.

Bart-Pumphrey syndrome (BPS) is an autosomal-dominant disorder characterized by hearing loss, leukonychia, knuckle pads and palmoplantar keratoderma. Two mutations in the extracellular domain of GBJ2 are resposible for this syndrome. To date, less than 10 case reports or clinical series about BPS have been published in the literature. Hearing loss and knuckle pads are the more commonly seen findings of this syndrome. Three generations and six family members with variable findings of knuckle pads, leukonychia, hearing loss and palmoplantar hyperkeratosis were presented in this report. We want to emphasize that dermatogists must be alert during the evaluation of these findings because some findings of this disorder may be vague or absent.

Hizli, Omer/0000-0001-6822-2679 WOS: 000368020200021 PubMed: 26571410 Hypothesis: We hypothesized that a pathologic condition exists in vestibular hair cells in human temporal bones from patients with systemic lupus erythematosus (SLE). Background: A significant association between sensorineural hearing loss and autoimmune disease has been reported. Patients with SLE also frequently have vestibular symptoms whose pathophysiologic mechanism is unclear. Methods: We examined 15 temporal bone samples from 8 patients with SLE, along with 21 samples from 17 age-matched healthy control patients. The samples were serially sectioned in the horizontal plane and stained with hematoxylin and eosin. Using differential interference contrast microscopy, we counted the number of type I and type II hair cells in the saccular macula, the utricular macula, and the cristae of the three semicircular canals; then, we calculated the hair cell density (cells per 0.01 mm(2)). Results: The mean density of type I hair cells in our SLE group was significantly lower than in our control group in the saccular macula, in the utricular macula, and in the superior, lateral, and posterior semicircular canals. But in all five vestibular sensory epithelia, the mean density of type II hair cells did not significantly differ between our two groups. In our SLE group, the mean density of vestibular hair cells did not significantly correlate with the patient's age at death or with the duration of SLE. Conclusion: Type I hair cells in peripheral vestibular organs are affected in patients with SLE. Our findings could provide a pathologic basis for the difficulty with balance experienced by patients with SLE. National Institute on Deafness and Other Communication Disorders (NIDCD)United States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute on Deafness & Other Communication Disorders (NIDCD) [U24 DC011968-01]; International Hearing Foundation; Starkey Hearing Foundation; 5M Lions International; Scientific and Technological Research Council of Turkey (TUBITAK)Turkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK) This project was funded by the National Institute on Deafness and Other Communication Disorders (NIDCD), grant number U24 DC011968-01; the International Hearing Foundation; the Starkey Hearing Foundation; the 5M Lions International; and the Scientific and Technological Research Council of Turkey (TUBITAK).