Nonsyndromic sensorineural hearing loss

Partial or total loss of hearing that is not associated with other signs and symptoms. Some hereditary cases may be caused by mutations in MITOCHONDRIAL GENES. OMIM: 500008

Also Known As:

Autosomal dominant nonsyndromic hereditary hearing impairment; Deafness, nonsyndromic sensorineural, mitochondrial; Nonsyndromic hereditary hearing impairment

Networked: 32 relevant articles (0 outcomes, 4 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Hearing Loss (Hearing Impairment)
2.	Nonsyndromic Deafness
3.	Aminoglycoside-Induced Deafness

Experts

1.	Sartorato, Edi Lúcia: 3 articles (12/2011 - 10/2009)
2.	Varbanova, Sonya: 2 articles (09/2014 - 06/2012)
3.	Cao, Xin: 2 articles (07/2013 - 09/2011)
4.	Chen, Zhibin: 2 articles (07/2013 - 09/2011)
5.	Lu, Yajie: 2 articles (07/2013 - 09/2011)
6.	Wei, Qinjun: 2 articles (07/2013 - 09/2011)
7.	Xing, Guangqian: 2 articles (07/2013 - 09/2011)
8.	Marques-de-Faria, Antonia Paula: 2 articles (12/2011 - 10/2009)
9.	Pereira, Tânia: 2 articles (12/2011 - 10/2009)
10.	Erbe, Christy B: 2 articles (04/2004 - 07/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Nonsyndromic sensorineural hearing loss:

1.	Connexin 26IBA 06/01/2012 - "In conclusion, this study determines the importance of connexin 26 mutations in Bulgarian children with severe to profound congenital nonsyndromic sensorineural hearing loss, the prevalence of the different mutation variants and their relationship with the ethnical background of the patients. " 06/01/2012 - "Objective of the study is to assess the prevalence of Connexin 26 (GJB2) mutation in patients with congenital nonsyndromic sensorineural hearing loss in Bulgarian population. " 01/01/2009 - "A novel missense mutation in the Connexin 26 gene associated with autosomal recessive nonsyndromic sensorineural hearing loss in a consanguineous Tunisian family." 04/01/2004 - "Mutations in the connexin 26 (Cx26) or gap junction beta 2 gene are the leading cause of hereditary nonsyndromic sensorineural hearing loss in Caucasians. " 07/01/2002 - "GJB2 (connexin 26) variants and nonsyndromic sensorineural hearing loss: a HuGE review."
2.	Mitochondrial DNA (mtDNA)IBA 07/01/2016 - "Our findings in the present study indicate that even in sporadic patients with nonsyndromic sensorineural hearing loss, mitochondrial DNA mutations might also contribute to the clinical phenotype." 07/01/2016 - "Prevalence of mitochondrial DNA mutations in sporadic patients with nonsyndromic sensorineural hearing loss." 02/01/2011 - "GJB2 mutation was frequent in sporadic outpatients and its mutation frequency was significant higher in the prelingual group than in the postlingual group, whereas the mutation of mtDNA A1555G and SLC26A4 was very rare in Chinese sporadic outpatients with nonsyndromic sensorineural hearing loss (NSHL). " 09/01/2003 - "Lack of a modulative factor in locus 8p23 in a Finnish family with nonsyndromic sensorineural hearing loss associated with the 1555A>G mitochondrial DNA mutation." 07/01/2008 - "The identification of the mtDNA A1555G mutation in a large proportion of Chinese probands with nonsyndromic sensorineural hearing loss (NSHL) provides a molecular explanation for the high prevalence of aminoglycoside-induced deafness in China. "
3.	AminoglycosidesIBA 11/01/2009 - "The study was composed of 20 patients with nonsyndromic sensorineural hearing loss without prior use of aminoglycosides and 40 premature and high-risk newborns who used ototoxic drugs, of whom 20 had good hearing and 20 had hearing loss. " 07/01/2009 - "In various regions of the world, patients suffer from nonsyndromic sensorineural hearing loss initiated by aminoglycoside antibiotics. " 05/01/1998 - "A mitochondrial mutation at nucleotide 1555 has been reported to be susceptible to aminoglycoside antibiotics as well as one of the causes of nonsyndromic sensorineural hearing loss. "
4.	ConnexinsIBA 09/01/2014 - "Connexin gene mutations among Ugandan patients with nonsyndromic sensorineural hearing loss." 01/01/2001 - "Mutations in the gene GJB2 encoding connexin 26 (Cx26), a gap junction protein, have been shown to be responsible for a majority of recessive nonsyndromic hereditary hearing impairment in children. "
5.	Ser Transfer RNAIBA 07/01/2009 - "[Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia]." 09/01/2011 - "To further characterize the molecular etiology of hearing loss in the Chinese population, we recruited a total of 135 unrelated patients with nonsyndromic sensorineural hearing loss (NSHL) for mutational screening of GJB2, GJB3, GJB6, SLC26A4, SLC26A5 IVS2-2A>G and mitochondrial 12SrRNA, tRNA(Ser(UCN)) by PCR amplification and direct DNA sequencing. "
6.	Proteins (Proteins, Gene)FDA Link 07/01/2002 - "Mutations of the gap junction beta 2 (GJB2) gene coding for the protein connexin 26 account for up to 50% of nonsyndromic sensorineural hearing loss (NSHL), with specific mutations associated with distinct ethnic groups. " 02/15/2009 - "The GJB2 gene located on chromosome 13q12 and encoding the connexin 26 (Cx26) protein, a transmembrane protein involved in cell-cell attachment of almost all tissues, including the skin, causes autosomal recessive and sometimes dominant nonsyndromic sensorineural hearing loss. "
7.	DNA (Deoxyribonucleic Acid)IBA 10/09/2023 - "Exome sequencing was performed on DNA of three families segregating autosomal recessive moderate to severe nonsyndromic sensorineural hearing loss. " 09/01/2011 - "To further characterize the molecular etiology of hearing loss in the Chinese population, we recruited a total of 135 unrelated patients with nonsyndromic sensorineural hearing loss (NSHL) for mutational screening of GJB2, GJB3, GJB6, SLC26A4, SLC26A5 IVS2-2A>G and mitochondrial 12SrRNA, tRNA(Ser(UCN)) by PCR amplification and direct DNA sequencing. "
8.	Anti-Bacterial Agents (Antibiotics)IBA 07/01/2009 - "In various regions of the world, patients suffer from nonsyndromic sensorineural hearing loss initiated by aminoglycoside antibiotics. " 05/01/1998 - "A mitochondrial mutation at nucleotide 1555 has been reported to be susceptible to aminoglycoside antibiotics as well as one of the causes of nonsyndromic sensorineural hearing loss. "
9.	12S ribosomal RNAIBA 07/04/2013 - "Mutations in GJB2 and mitochondrial DNA (mtDNA) 12S rRNA are the most common molecular etiology for nonsyndromic sensorineural hearing loss (NSHL). " 07/01/2009 - "[Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia]."
10.	Myosin Type IIIBA 05/01/2013 - "Human genetic mutations that affect the N-terminal head-domain of the nonmuscle myosin-II (MyoII) molecule can result in nonsyndromic sensorineural hearing loss but the underlying mechanism is unknown. "

Therapies and Procedures

1.	Ligation 10/01/2009 - "In the present study we investigated the presence of the IVS 1+1 G>A mutation by multiplex ligation-dependent probe amplification in 185 unrelated Brazilian patients with autosomal recessive nonsyndromic sensorineural hearing loss (43 heterozygous patients and 142 without any pathogenic mutation in the GJB2-coding region). "
2.	Cochlear Implantation 09/01/2014 - "To compare the audiologic outcome after cochlear implantation between 2 groups of patients with congenital nonsyndromic sensorineural hearing loss. "
3.	Cochlear Implants (Cochlear Implant) 12/01/2003 - "From 181 children who participated in a nationwide cochlear implant research program, 122 children were identified with congenital nonsyndromic sensorineural hearing loss and invited to participate. "
4.	Genomic Medicine 07/15/2020 - "Diagnostic and therapeutic applications of genomic medicine in progressive, late-onset, nonsyndromic sensorineural hearing loss."