Literature DB >> 3625027

Pendred's syndrome with episodic vertigo, tinnitus and vomiting and normal bithermal caloric responses.

V K Das.   

Abstract

A case of early-onset bilateral sensori-neural deafness with episodic objective vertigo, tinnitus and vomiting, suggestive of Menière's syndrome, was found to have a diffuse goitre. A Perchlorate discharge test was positive, indicating an organification defect diagnostic of Pendred's syndrome. The case was found to be euthyroid both clinically and on investigations. Tone decay was absent and recruitment was found, thus indicating a cochlear site of lesion. Bithermal caloric tests and clinical tests of vestibular function were within normal limits. X-rays, including petrous tomography, did not show any abnormalities.

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Year:  1987        PMID: 3625027     DOI: 10.1017/s0022215100102592

Source DB:  PubMed          Journal:  J Laryngol Otol        ISSN: 0022-2151            Impact factor:   1.469


  4 in total

Review 1.  Pendred syndrome.

Authors:  W Reardon; R C Trembath
Journal:  J Med Genet       Date:  1996-12       Impact factor: 6.318

Review 2.  Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models.

Authors:  Andrew J Griffith; Philine Wangemann
Journal:  Hear Res       Date:  2011-06-06       Impact factor: 3.208

3.  Expression pattern of the mouse ortholog of the Pendred's syndrome gene (Pds) suggests a key role for pendrin in the inner ear.

Authors:  L A Everett; H Morsli; D K Wu; E D Green
Journal:  Proc Natl Acad Sci U S A       Date:  1999-08-17       Impact factor: 11.205

4.  Localization and functional studies of pendrin in the mouse inner ear provide insight about the etiology of deafness in pendred syndrome.

Authors:  Ines E Royaux; Inna A Belyantseva; Tao Wu; Bechara Kachar; Lorraine A Everett; Daniel C Marcus; Eric D Green
Journal:  J Assoc Res Otolaryngol       Date:  2003-09
  4 in total

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