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Year Number of Results
1988 1
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2001 1
2003 1
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2017 3
2019 1
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Page 1
Noonan Syndrome: An Underestimated Cause of Severe to Profound Sensorineural Hearing Impairment. Which Clues to Suspect the Diagnosis?
Ziegler A, Loundon N, Jonard L, Cavé H, Baujat G, Gherbi S, Couloigner V, Marlin S. Ziegler A, et al. Otol Neurotol. 2017 Sep;38(8):1081-1084. doi: 10.1097/MAO.0000000000001509. Otol Neurotol. 2017. PMID: 28742629 Review.
OBJECTIVE: To highlight Noonan syndrome as a clinically recognizable cause of severe to profound sensorineural hearing impairment. STUDY DESIGN: New clinical cases and review. ...RESULTS: Five patients presented with profound sensorineural …
OBJECTIVE: To highlight Noonan syndrome as a clinically recognizable cause of severe to profound sensorineural hearing …
Application of the TEN test to hearing-impaired teenagers with severe-to-profound hearing loss.
Moore BC, Killen T, Munro KJ. Moore BC, et al. Int J Audiol. 2003 Dec;42(8):465-74. doi: 10.3109/14992020309081516. Int J Audiol. 2003. PMID: 14658854
The aim of this study was to assess problems in applying the TEN test to teenagers with longstanding severe-to-profound sensorineural hearing impairment, and to assess the prevalence of dead regions in this population. ...
The aim of this study was to assess problems in applying the TEN test to teenagers with longstanding severe-to-profound sensorineu …
Further evidence of involvement of TMEM132E in autosomal recessive nonsyndromic hearing impairment.
Liaqat K, Hussain S, Bilal M, Nasir A, Acharya A, Ali RH, Nawaz S, Umair M, Schrauwen I, Ahmad W, Leal SM. Liaqat K, et al. J Hum Genet. 2020 Jan;65(2):187-192. doi: 10.1038/s10038-019-0691-4. Epub 2019 Oct 28. J Hum Genet. 2020. PMID: 31656313 Free PMC article.
In the present study, a family of Pakistani origin with prelingual profound sensorineural hearing impairment displaying AR mode of inheritance was investigated via exome and Sanger sequencing. ...
In the present study, a family of Pakistani origin with prelingual profound sensorineural hearing impairment dis …
SOX10 mutation causes Waardenburg syndrome associated with distinctive phenotypic features in an Iranian family: A clue for phenotype-directed genetic analysis.
Jalilian N, Tabatabaiefar MA, Alimadadi H, Noori-Daloii MR. Jalilian N, et al. Int J Pediatr Otorhinolaryngol. 2017 May;96:122-126. doi: 10.1016/j.ijporl.2017.03.016. Epub 2017 Mar 16. Int J Pediatr Otorhinolaryngol. 2017. PMID: 28390600
METHOD: A two-generation pedigree with three affected members and considerable phenotypic heterogeneity was recruited. The proband was a 15-year-old boy, with severe to profound sensorineural hearing impairment, heterochromia iridis, hypoplastic blue e …
METHOD: A two-generation pedigree with three affected members and considerable phenotypic heterogeneity was recruited. The proband was a 15- …
Hearing performance in noise of cochlear implant patients versus severely-profoundly hearing-impaired patients with hearing aids.
Hamzavi J, Franz P, Baumgartner WD, Gstöettner W. Hamzavi J, et al. Audiology. 2001 Jan-Feb;40(1):26-31. doi: 10.3109/00206090109073097. Audiology. 2001. PMID: 11296938
For this purpose, we tested two groups of patients: one that had received cochlear implants (n=22) and a group of subjects with severe-profound sensorineural hearing impairment, fitted with hearing aids (n = 15). ...
For this purpose, we tested two groups of patients: one that had received cochlear implants (n=22) and a group of subjects with severe-pr …
Auditory steady-state responses for children with severe to profound hearing loss.
Swanepoel D, Hugo R, Roode R. Swanepoel D, et al. Arch Otolaryngol Head Neck Surg. 2004 May;130(5):531-5. doi: 10.1001/archotol.130.5.531. Arch Otolaryngol Head Neck Surg. 2004. PMID: 15148172
PATIENTS: A referred sample of 10 patients (20 ears), 5 girls and 5 boys aged 10 to 15 years (mean age, 13 years 4 months), with severe to profound sensorineural hearing impairment. MAIN OUTCOME MEASURES: The difference, and correlation, between 160 pu …
PATIENTS: A referred sample of 10 patients (20 ears), 5 girls and 5 boys aged 10 to 15 years (mean age, 13 years 4 months), with severe to …
Treatment of otological features of the oculoauriculovertebral dysplasia (Goldenhar syndrome).
Skarzyński H, Porowski M, Podskarbi-Fayette R. Skarzyński H, et al. Int J Pediatr Otorhinolaryngol. 2009 Jul;73(7):915-21. doi: 10.1016/j.ijporl.2009.01.015. Epub 2009 Feb 8. Int J Pediatr Otorhinolaryngol. 2009. PMID: 19203801
The degree of hearing loss can range from mild to moderate conductive type in cases of chronic otitis media with effusion and severe to profound sensorineural hearing impairment with malformations of the inner ear in various forms of cochlear hypoplasi …
The degree of hearing loss can range from mild to moderate conductive type in cases of chronic otitis media with effusion and severe to p …
Broadening the phenotype of DFNB28: Mutations in TRIOBP are associated with moderate, stable hereditary hearing impairment.
Wesdorp M, van de Kamp JM, Hensen EF, Schraders M, Oostrik J, Yntema HG, Feenstra I, Admiraal RJC, Kunst HPM, Tekin M, Kanaan M, Kremer H, Pennings RJE. Wesdorp M, et al. Hear Res. 2017 Apr;347:56-62. doi: 10.1016/j.heares.2016.12.017. Epub 2017 Jan 12. Hear Res. 2017. PMID: 28089734
DFNB28 is characterized by prelingual, severe to profound sensorineural hearing impairment (HI). It is associated with mutations in exon 6 and 7 of TRIOBP and has not been reported in the European population. ...
DFNB28 is characterized by prelingual, severe to profound sensorineural hearing impairment (HI). It is associate …
Identification of a novel mutation in POU3F4 for prenatal diagnosis in a Chinese family with X-linked nonsyndromic hearing loss.
Li J, Cheng J, Lu Y, Lu Y, Chen A, Sun Y, Kang D, Zhang X, Dai P, Han D, Yuan H. Li J, et al. J Genet Genomics. 2010 Dec;37(12):787-93. doi: 10.1016/S1673-8527(09)60096-5. J Genet Genomics. 2010. PMID: 21193157
We present the clinical and genetic findings for a Chinese family with X-linked non-syndromic hearing loss in which the affected males showed congenital profound sensorineural hearing impairment. In two affected brothers, the computer tomography of tem …
We present the clinical and genetic findings for a Chinese family with X-linked non-syndromic hearing loss in which the affected males showe …
Ear dominance for nonsense-syllable recognition ability in sensorineural hearing-impaired children: monaural versus binaural amplification.
Hattori H. Hattori H. J Am Acad Audiol. 1993 Sep;4(5):319-30. J Am Acad Audiol. 1993. PMID: 8219299
Subjects were 35 children with bilateral symmetric, moderately severe to profound, sensorineural hearing impairment. Seventeen were fitted with nonalternating monaural amplification and 18 were fitted with binaural or alternating monaural amplification …
Subjects were 35 children with bilateral symmetric, moderately severe to profound, sensorineural hearing impairment …
11 results