[HTML][HTML] Nonsyndromic Hearing Loss and Deafness, DFNA3–RETIRED CHAPTER, FOR HISTORICAL REFERENCE ONLY

RJH Smith, PT Ranum - 2016 - europepmc.org
Nonsyndromic Hearing Loss and Deafness, DFNA3 – RETIRED CHAPTER, FOR HISTORICAL
REFERENCE ONLY - Abstract - Europe PMC Sign in | Create an account https://orcid.org …

AudioGene: refining the natural history of KCNQ4, GSDME, WFS1, and COCH-associated hearing loss

RK Thorpe, WD Walls, R Corrigan, A Schaefer, K Wang… - Human genetics, 2022 - Springer
Autosomal dominant non-syndromic hearing loss (ADNSHL) displays gene-specific
progression of hearing loss, which is amenable to sequential audioprofiling. We sought to …

Exome sequencing utility in defining the genetic landscape of hearing loss and novel‐gene discovery in Iran

M Mohseni, M Babanejad, KT Booth, P Jamali… - Clinical …, 2021 - Wiley Online Library
Hearing loss (HL) is one of the most common sensory defects affecting more than 466
million individuals worldwide. It is clinically and genetically heterogeneous with over 120 …

DFNA5 (GSDME) c.991-15_991-13delTTC: Founder Mutation or Mutational Hotspot?

KT Booth, H Azaiez, RJH Smith - International journal of molecular …, 2020 - mdpi.com
Deafness due to mutations in the DFNA5 gene is caused by the aberrant splicing of exon 8,
which results in a constitutively active truncated protein. In a large family of European …

Genome-first approach to rare EYA4 variants and cardio-auditory phenotypes in adults

S Ahmadmehrabi, B Li, J Park, B Devkota, M Vujkovic… - Human Genetics, 2021 - Springer
While newborns and children with hearing loss are routinely offered genetic testing, adults
are rarely clinically tested for a genetic etiology. One clinically actionable result from genetic …

A Big Data Perspective on the Genomics of Hearing Loss.

B Vona, M Müller, S Dofek, M Holderried… - Laryngo-Rhino …, 2019 - europepmc.org
The completion of the human genome, the most fundamental example of big data in science
and medicine, is the remarkable product of multidisciplinary collaboration and is regarded …

Computational analysis based on audioprofiles: A new possibility for patient stratification in office-based otology

O Weininger, A Warnecke, A Lesinski-Schiedat… - Audiology …, 2019 - mdpi.com
Genetic contribution to progressive hearing loss in adults is underestimated. Established
machine learning-based software could offer a rapid supportive tool to stratify patients with …

A Genome-First Approach to Rare Variants in Dominant Postlingual Hearing Loss Genes in a Large Adult Population

S Ahmadmehrabi, B Li, D Hui, J Park… - … –Head and Neck …, 2022 - journals.sagepub.com
Objective To investigate the importance of rare variants in adult-onset hearing loss. Study
Design Genomic association study. Setting Large biobank from tertiary care center. Methods …

[HTML][HTML] Big data in der Diagnostik genetischer Schwerhörigkeit

B Vona, M Müller, S Dofek, M Holderried… - Laryngo-Rhino …, 2019 - thieme-connect.com
Die vollständige Sequenzierung des menschlichen Genoms demonstriert als ein
grundlegendes Beispiel eindrucksvoll die Entstehung einer großen Datenmenge (engl.: big …

AGTD-The AudioGene Translational Dashboard; a Hybrid Machine Learning and Visualization Interface for Genetic Diagnosis of Autosomal Dominant Non-Syndromic …

B DeSollar - 2024 - search.proquest.com
Abstract Autosomal Dominant Non-Syndromic Hearing Loss (ADNSHL) presents a major
genetic diagnosis challenge due to the complex heterogeneity of the molecular basis of the …