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Progressive sensorineural hearing impairment in maternally inherited diabetes mellitus and deafness (MIDD).
Hendrickx JJ, Mudde AH, 't Hart LM, Huygen PL, Cremers CW. Hendrickx JJ, et al. Otol Neurotol. 2006 Sep;27(6):802-8. doi: 10.1097/01.mao.0000224091.02506.a0. Otol Neurotol. 2006. PMID: 16788417
Vestibular and brainstem-evoked response audiometry test results were normal. All the patients achieved the maximum speech recognition score, as was expected based on their pure-tone average at 1, 2, and 4 kHz. ...
Vestibular and brainstem-evoked response audiometry test results were normal. All the patients achieved the maximum speech recognition sc …
Progressive sensorineural hearing impairment in professional fishery divers.
Haraguchi H, Ohgaki T, Okubo J, Noguchi Y, Sugimoto T, Komatsuzaki A. Haraguchi H, et al. Ann Otol Rhinol Laryngol. 1999 Dec;108(12):1165-9. doi: 10.1177/000348949910801212. Ann Otol Rhinol Laryngol. 1999. PMID: 10605922
A prospective study was performed to determine whether sensorineural hearing acuity in the same divers deteriorated faster than in a normal population as they continued diving. After an observation period of approximately 5 years, audiometric examination was performed on a …
A prospective study was performed to determine whether sensorineural hearing acuity in the same divers deteriorated faster than in a normal …
Expanding the phenotype spectrum associated with pathogenic variants in the COL2A1 and COL11A1 genes.
Čopíková J, Paděrová J, Románková V, Havlovicová M, Balaščáková M, Zelinová M, Vejvalková Š, Simandlová M, Štěpánková J, Hořínová V, Kantorová E, Křečková G, Pospíšilová J, Boday A, Meszarosová AU, Turnovec M, Votýpka P, Lišková P, Kremlíková Pourová R. Čopíková J, et al. Ann Hum Genet. 2020 Sep;84(5):380-392. doi: 10.1111/ahg.12386. Epub 2020 May 19. Ann Hum Genet. 2020. PMID: 32427345
In nine families, the COL2A1 disease-causing variant arose de novo. Phenotypically, we observed myopia (95%) and retinal detachment (47%), joint hyperflexibility (92%), midface retrusion (84%), cleft palate (53%), and various degrees of hearing impairment (50%). ...In the …
In nine families, the COL2A1 disease-causing variant arose de novo. Phenotypically, we observed myopia (95%) and retinal detachment ( …
Immunologic alterations in patients with sensorineural hearing disorders.
Mayot D, Béné MC, Dron K, Perrin C, Faure GC. Mayot D, et al. Clin Immunol Immunopathol. 1993 Jul;68(1):41-5. doi: 10.1006/clin.1993.1092. Clin Immunol Immunopathol. 1993. PMID: 8513592
We report a study performed in 57 individuals with sudden deafness (n = 17, group 1) or progressive sensorineural hearing impairment (n = 40, group 2). A severe depletion in CD3+ and CD4+ peripheral lymphocytes was observed in group 1 and a mark …
We report a study performed in 57 individuals with sudden deafness (n = 17, group 1) or progressive sensorineural hearing …
Progressive sensorineural hearing impairment in systemic vasculitides.
Berrettini S, Ferri C, Ravecca F, LaCivita L, Bruschini L, Riente L, Mosca M, Sellari-Franceschini S. Berrettini S, et al. Semin Arthritis Rheum. 1998 Apr;27(5):301-18. doi: 10.1016/s0049-0172(98)80051-6. Semin Arthritis Rheum. 1998. PMID: 9572712
Those patients with progressive hearing impairment were selected and studied carefully. RESULTS: A PSNHL was observed in 14 patients. The hearing loss was bilateral and asymmetrical in most subjects. ...
Those patients with progressive hearing impairment were selected and studied carefully. RESULTS: A PSNHL was observed in 14 patients. …
A Dutch family with progressive sensorineural hearing impairment linked to the DFNA2 region.
Ensink RJ, Huygen PL, Van Hauwe P, Coucke P, Cremers CW, Van Camp G. Ensink RJ, et al. Eur Arch Otorhinolaryngol. 2000;257(2):62-7. doi: 10.1007/pl00007511. Eur Arch Otorhinolaryngol. 2000. PMID: 10784363
We studied a Dutch family with DFNA2-linked progressive sensorineural hearing impairment (SNHI). Recent audiograms were obtained from 18 of the affected persons (age 7-81 years) and were used in a gene-linkage analysis. ...Linkage analysis was carried …
We studied a Dutch family with DFNA2-linked progressive sensorineural hearing impairment (SNHI). Recent audiogra …
Cochlear implantation in a patient with Epstein syndrome.
Nishiyama N, Kawano A, Kawaguchi S, Shirai K, Suzuki M. Nishiyama N, et al. Auris Nasus Larynx. 2013 Aug;40(4):409-12. doi: 10.1016/j.anl.2012.07.005. Epub 2012 Jul 31. Auris Nasus Larynx. 2013. PMID: 22854055
We report a case of a patient with Epstein syndrome in whom bilateral profound hearing impairment developed and who had undergone cochlear implantation 9 years previously. Prior to this, the patient showed progressive sensorineural hearing impairment a …
We report a case of a patient with Epstein syndrome in whom bilateral profound hearing impairment developed and who had undergone cochlear i …
Pediatric cochlear implant candidacy issues.
Osberger MJ, Chute PM, Pope ML, Kessler KS, Carotta CC, Firszt JB, Zimmerman-Phillips S. Osberger MJ, et al. Am J Otol. 1991;12 Suppl:80-8. Am J Otol. 1991. PMID: 2069195
Children with progressive sensorineural hearing impairment represent a special challenge to the audiologist and the otologist. ...By the same token however, there appears to be no advantage to waiting an inordinate amount of time after the loss of func …
Children with progressive sensorineural hearing impairment represent a special challenge to the audiologist and …