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Mutations in human SLC26A4 are a common cause of hearing loss associated with enlarged vestibular aqueducts (EVA). SLC26A4 encodes pendrin, an anion-base exchanger expressed in inner ear epithelial cells that secretes HCO3- into endolymph. Studies of Slc26a4-null mice indicate that pendrin is essential for inner ear development, but have not revealed(More)
Hereditary hearing loss is one of the most common birth defects, yet the majority of genes required for audition is thought to remain unidentified. Ethylnitrosourea (ENU)-mutagenesis has been a valuable approach for generating new animal models of deafness and discovering previously unrecognized gene functions. Here we report on the characterization of a(More)
Loss-of-function mutations of SLC26A4/pendrin are among the most prevalent causes of deafness. Deafness and vestibular dysfunction in the corresponding mouse model, Slc26a4(-/-), are associated with an enlargement and acidification of the membranous labyrinth. Here we relate the onset of expression of the HCO(3) (-) transporter pendrin to the luminal pH and(More)
Mutations of SLC26A4 cause an enlarged vestibular aqueduct, nonsyndromic deafness, and deafness as part of Pendred syndrome. SLC26A4 encodes pendrin, an anion exchanger located in the cochlea, thyroid, and kidney. The goal of the present study was to determine whether developmental delays, possibly mediated by systemic or local hypothyroidism, contribute to(More)
Mutations of SLC26A4 are among the most prevalent causes of hereditary deafness. Deafness in the corresponding mouse model, Slc26a4(-/-), results from an abnormally enlarged cochlear lumen. The goal of this study was to determine whether the cochlear enlargement originates with defective cochlear fluid transport or with a malfunction of fluid transport in(More)
CONCLUSION Cochlear implantation (CI) remains a safe procedure with a low complication rate. Nevertheless, advances in surgical techniques and the optimization of treatment modalities would further reduce complications. OBJECTIVE To assess the complications of CI and describe the management of each complication encountered at our hospital. PATIENTS AND(More)
We have evaluated the particle sizes, zeta potentials and hydrodynamic radii of zinc oxide (ZnO) nanoparticles in various aqueous conditions such as deionized water, phosphate buffered saline and Minimum Essential Media Eagle. In order to study the size and surface chemistry effect in colloidal behaviors, different size of 20 and 70 nm ZnO nanoparticles(More)
BACKGROUND/OBJECTIVES Childhood episodic vertigo has been reported to be associated with migraine or childhood periodic syndromes such as benign paroxysmal vertigo of childhood. There is discrete evidence that unexpected recurrent vertigo is associated with a high level of depression and anxiety in adults. However, only a few studies describe the frequency(More)
Several members of the SLC26 gene family have highly-restricted expression patterns in the auditory and vestibular periphery and mutations in mice of at least two of these (SLC26A4 and SLC26A5) lead to deficits in hearing and/or balance. A previous report pointed to SLC26A7 as a candidate gene important for cochlear function. In the present study, inner(More)
OBJECTIVE Several studies suggested the possible relationship between decreased bone mineral density (BMD) of the temporal bone and hearing loss, primarily of the sensorineural type. The aim of the present study is to determine the relationship between BMD and hearing loss and to evaluate the systemic Ca(2+) and vitamin D status with relation to hearing(More)