Results 141 to 150 of about 42,755 (252)

Cochlear Implantation in Autoimmune Inner‐Ear Disease: Outcome and Patient‐Reported Benefit

The Laryngoscope, Volume 136, Issue 9, Page 3980-3989, September 2026.
This study demonstrates that patients with secondary autoimmune inner‐ear disease (AIED) achieve significant improvements in speech perception and quality of life following cochlear implantation. Although AIED patients may show slightly lower performance in noisy environments compared to controls, their subjective hearing benefit converges with matched
Merete Hartmann, David Oestreicher, Marc Albert Hüser, Dirk Beutner, Christian Wrobel   +4 more
wiley   +1 more source

Seasonal effects on incidence and outcomes in idiopathic sudden sensorineural hearing loss. [PDF]

Front Neurol
Ghantous J, Barak S, Yehuda T, Israeli O, Hadar A, Salem R, Sichel JY, Perez R, Shaul C.   +8 more
europepmc   +1 more source

Quantifying Soft‐Surgery in Cochlear Implantation: Multimodal Data From 30 International Specialists

The Laryngoscope, Volume 136, Issue 9, Page 3990-3997, September 2026.
Objective data from 30 specialist cochlear implant surgeons showed that surgical handling strongly influences intracochlear mechanical stress during implantation. Surgeons with fewer than 50 lifetime insertions performed significantly worse than more experienced colleagues, while self‐assessment did not reflect objective performance in any group ...
Philipp Aebischer, Georgios Mantokoudis, Marco Caversaccio, Stefan Weder   +3 more
wiley   +1 more source

Prognostic factors for pediatric sudden sensorineural hearing loss: a systematic review and meta-analysis. [PDF]

Front Pediatr
Wu Z, Zhang P, Sun J, Diao M.
europepmc   +1 more source

Disparities in Hearing Screening Practices in Minnesota Elementary Schools

The Laryngoscope, Volume 136, Issue 9, Page 4064-4071, September 2026.
ABSTRACT Objective Identifying and addressing pediatric hearing loss is critical to supporting a child's development. School‐based hearing screening is a mainstay of timely identification of hearing loss. The objectives of this study were to characterize the current hearing screening practices in public, charter, and private elementary schools in ...
Autefeh Sajjadi, Elizabeth Kim, Kathryn S. Marcus, Soorya Todatry, Nicholas Hable, Brianne Roby, Abby C. Meyer, Andrew Redmann, Hannah Herd, Rebecca Maher, Siva Chinnadurai, Amanda Nickel, Asitha Jayawardena   +12 more
wiley   +1 more source

Steroids for the Prevention of Sensorineural Hearing Loss Secondary to Acute Otitis Media: A Systematic Review. [PDF]

Int Arch Otorhinolaryngol
Sampaio AFBS, Monsanto RDC, Jorge JJ, Borges GC.   +3 more
europepmc   +1 more source

Expanding Spectrum of FIG4‐Related Neurological Disorders of Lysosomal Homeostasis: Case Report and Overview of the Potential Genotype–Phenotype Correlations

Clinical Genetics, Volume 110, Issue 3, Page 363-368, September 2026.
FIG4 is essential for lysosomal homeostasis. FIG4‐related disorders present as a continuous spectrum from the juvenile lethality in Yunis‐Varon syndrome to an increased risk of amyotrophic lateral sclerosis (ALS) in adult life. FIG4‐related disorders comprise a novel group of disorders of lysosomal homeostasis and can be classified into severe ...
Pankaj Prasun, Matthew Rasberry
wiley   +1 more source

Novel Postzygotic Variants Associated With Hypomelanosis of Ito Expand the ACTB‐Related Neurocutaneous Disease Spectrum

Clinical Genetics, Volume 110, Issue 3, Page 369-373, September 2026.
We describe a previously unreported phenotype related to postzygotic ACTB variants with hypomelanosis of Ito, characterized by hypopigmentation associated or not with neurodevelopmental features, distinct from Becker presentations, bridging constitutional neurodevelopmental and somatic cutaneous phenotypes.
Estella Castillon, Paul Rollier, Didier Bessis, Laurent Pasquier, Caroline Racine, Alexis Praga, Pierre Vabres, Bertille Bonniaud, Paul Kuentz, Laurence Faivre   +9 more
wiley   +1 more source

USP34 Haploinsufficiency as a Cause of Neurodevelopmental Phenotypes

Clinical Genetics, Volume 110, Issue 3, Page 315-324, September 2026.
Heterozygous loss‐of‐function variants in USP34 cause a novel neurodevelopmental disorder characterized by global developmental delay, speech impairment, autism, hypotonia, craniofacial dysmorphism, and distal limb anomalies. Disrupted Wnt/β‐catenin signaling via reduced Axin stabilization refines gene‐specific contributions within 2p15p16.1 ...
Helena Wigoda, Amjad Khan, Bryce A. Mendelsohn, Noriko Miyake, Nobuhiko Okamoto, Naomichi Matsumoto, Patricia J. C. Knijnenburg, Johanna M. van Hagen, Jiddeke van de Kamp, Quinten Waisfisz, Bryn D. Webb   +10 more
wiley   +1 more source

Diagnostic Yield and Clinical Impact of Comprehensive WES/WGS Testing Beyond Common Genetic Causes in Hereditary Optic Atrophy

Clinical Genetics, Volume 110, Issue 3, Page 336-346, September 2026.
Opticus atrophy—Genetic testing with WES/WGS in 62 patients with optic atrophy provided a genetic diagnosis in 21 patients (33.9%). 42.9% of these involved non‐OPA1 genes, including WFS1, ACO2, NR2F1, UCHL1, CACNA1F, and COQ2, where the genetic diagnosis prompted additional clinical evaluation, surveillance, or therapeutic intervention.
Katrine M. Johannesen, Karen Grønskov, Line Kessel, Sarah Linea von Holstein, Lisbeth Birk Møller, Mette Kjøbæk Gundestrup Andersen, Marianne Søndergaard Khinchi, Steffen Hamann, Marianne Wegener, Mette Bertelsen   +9 more
wiley   +1 more source