Article
Health Care Sciences & Services
Jianan Li, Shuoshuo Kang, Haiqiao Du, Shuwei Wang, Dandan Wang, Mengyu Liu, Shiming Yang
Summary: The selection of cochlear implant electrodes is different for cochleae with enlarged vestibular aqueduct (EVA) compared to those with a normal structure. EVA ears often have an incomplete partition type II (IP II) malformation, which may result in a smaller basal turn of the cochlea.
JOURNAL OF PERSONALIZED MEDICINE
(2022)
Article
Clinical Neurology
Sarah E. Hodge, Nicholas J. Thompson, Lisa R. Park, Kevin D. Brown
Summary: EVA is the most common congenital malformation of the inner ear and is associated with hearing loss progression. Research shows that the majority of children with EVA reach cochlear implant candidacy before the age of 12, but only 60% of them actually receive implants.
OTOLOGY & NEUROTOLOGY
(2021)
Review
Otorhinolaryngology
Asma Alahmadi, Yassin Abdelsamad, Marzouqi Salamah, Saad Alenzi, Khalid M. Badr, Saeed Alghamdi, Abdulrahman Alsanosi
Summary: This study reviewed the surgical and clinical outcomes of cochlear implantation among patients with enlarged vestibular aqueduct (EVA). The results showed improvement in audiometric and speech performance after CI in patients with EVA. However, many patients experienced intra- or postoperative complications, indicating the need for further research.
EUROPEAN ARCHIVES OF OTO-RHINO-LARYNGOLOGY
(2022)
Article
Otorhinolaryngology
F. Forli, F. Lazzerini, G. Auletta, L. Bruschini, S. Berrettini
Summary: This study aimed to analyse the clinical and audiological features of a cohort of Caucasian patients with NSEVA/PDS, genetic assessment and inner ear morphology. The results showed a significant correlation between SLC26A4 mutations and the presence of PDS, as well as differences in severity of hearing loss between PDS and NSEVA patients.
EUROPEAN ARCHIVES OF OTO-RHINO-LARYNGOLOGY
(2021)
Review
Pediatrics
Davide Brotto, Marzia Ariano, Mose Sozzi, Roberta Cenedese, Eva Muraro, Flavia Sorrentino, Patrizia Trevisi
Summary: About 20% of children with congenital hearing loss have malformations of the inner ear. The morphology and function of the anterior part of the labyrinth have been extensively studied, as hearing loss can significantly impact a child's overall development. While a chance for hearing rehabilitation is now available for most of them, the anomalies range from lack of inner ear development to specific isolated structures. However, little is known about vestibular anomalies and dysfunctions in children with inner ear malformations.
FRONTIERS IN PEDIATRICS
(2023)
Article
Neurosciences
Emanuele Bernardinelli, Sebastian Roesch, Edi Simoni, Angela Marino, Gerd Rasp, Laura Astolfi, Antonio Sarikas, Silvia Dossena
Summary: POU3F4 gene mutations are the main cause of X-linked deafness, resulting in hearing loss through impaired transcriptional activity and subcellular distribution. Using RNA sequencing and genetic analysis, researchers found that POU3F4 regulates the transcriptional levels of the amino acid transporter gene SLC6A20, providing insights into the mechanism of POU3F4-related deafness.
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2022)
Article
Medicine, Research & Experimental
Nathan Farrokhian, Armine Kocharyan, Jeremy Ruthberg, Robin Piper, Alejandro Rivas, Maroun Semaan, Todd Otteson, Nauman F. Manzoor
Summary: This study found that male gender, increasing operculum size, higher air pure tone average at diagnosis, and presence of incomplete partition type II were associated with increased risk for progressive hearing loss in EVA patients. Stratified risk groups based on nomogram scores showed that the high-risk group had significantly higher progression probabilities at 1, 3, and 9 years. The model demonstrated a discriminative ability with a C-index of 0.79.
Article
Otorhinolaryngology
Luan Viet Tran, Vu Anh Duong, Saim Lokman
Summary: This study identified a prevalence of 5.7% of inner ear malformations in cochlear implant recipients, with incomplete partition and cochlear hypoplasia being the main types. Intraoperative complications included cerebrospinal fluid gusher and inability to identify the round window.
INDIAN JOURNAL OF OTOLOGY
(2021)
Article
Clinical Neurology
Vladislav Razskazovskiy, Andrew A. Mccall, Barton F. Branstetter
Summary: Using high-resolution, multiplanar oblique reformats, CT scans of asymptomatic patients showed that the prevalence of cochlear-facial dehiscence is similar to postmortem studies, while jugular bulb-vestibular aqueduct dehiscence is a common incidental finding.
AMERICAN JOURNAL OF NEURORADIOLOGY
(2023)
Article
Medicine, Research & Experimental
Julia Perry, Erica Sher, Kosuke Kawai, Shelby Redfield, Tieqi Sun, Margaret Kenna
Summary: This study aimed to investigate the association between newborn hearing screening (NBHS) results and enlarged vestibular aqueduct (EVA)-related hearing loss (HL). The study found that almost half of the patients with EVA passed their NBHS. The results emphasize the importance of regular hearing monitoring for children who are initially not suspected of having hearing loss.
Review
Medicine, Research & Experimental
Liliya Benchetrit, Nicolette Jabbour, Swathi Appachi, Yi-Chun Liu, Michael S. Cohen, Samantha Anne
Summary: The study describes outcomes of cochlear implantation in children with enlarged vestibular aqueduct and incomplete partition type 2. The results show significant improvement in speech perception, auditory, and language performance among these children, with outcomes comparable to control children without inner ear malformations.
Article
Otorhinolaryngology
P. Reynard, E. Ionescu, C. A. Joly, A. Ltaief-Boudrigua, A. Coudert, H. Thai-Van
Summary: In children with EVA undergoing CI surgery, those with a larger endolymphatic sac volume are more likely to experience postoperative vestibular complications. Therefore, MRI measurement of the ES volume should be performed systematically to minimize this risk before CI surgery.
INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY
(2021)
Article
Radiology, Nuclear Medicine & Medical Imaging
Haroon S. Saeed, Azita Rajai, Rachel Dixon, Tejas Kapadia, Iain A. Bruce, Stavros Stivaros
Summary: Morphological features of enlarged endolymphatic duct and sac can serve as imaging biomarkers for genotype and hearing loss phenotype. Measurements of length, width, and signal heterogeneity of the duct and sac showed good reproducibility, indicating their potential as clinical predictors.
BRITISH JOURNAL OF RADIOLOGY
(2023)
Article
Pediatrics
Davide Brotto, Marzia Ariano
Summary: This case report presents a patient initially diagnosed with conductive hearing loss due to recurrent otitis, then juvenile otosclerosis, who was ultimately diagnosed with bilateral ossicular chain fixation and enlarged vestibular aqueduct using cone-beam CT.
Review
Audiology & Speech-Language Pathology
Marie-Louise Uhre Hansen, Eva Rye Rasmussen, Per Caye-Thomasen, Kristianna Mey
Summary: This study investigated the effects of cochlear implantation in pediatric patients with Pendred Syndrome or non-syndromic enlarged vestibular aqueduct (NSEVA), including improvement in hearing and speech perception outcomes, as well as surgical complications. A systematic review of the literature was performed, and 55 studies were included for analysis. The results showed significant improvements in audiometric outcomes and auditory performance/speech intelligibility, with a decrease in implantation age over the past two decades. The most common surgical complication reported was perilymph gusher/oozing.
