Nasal mucociliary clearance and hearing loss in pseudoexfoliation syndrome.

Eur Arch Otorhinolaryngol

Otorhinolaryngology Department, Faculty of Medicine, Van Yüzüncü Yıl University, Van, Turkey.

Published: June 2021

Purpose: To evaluate nasal mucociliary clearance (NMC) and hearing loss in patients with pseudoexfoliation (PEX) syndrome.

Methods: The study included 36 patients with PEX syndrome as the study group, and 39 healthy individuals as the control group. The duration of NMC was measured using the saccharine test. Audiometry was applied at 250, 500, 1000, 2000, 4000, 6000 and 8000 Hz frequencies to determine hearing thresholds and prevalence of hearing loss.

Results: No statistically significant difference was determined between the groups in respect of age and gender (p = 0.23 and p = 0.36, respectively). In the saccharine test, the mean NMC time was 18.41 ± 8.53 min for the study group and 11.69 ± 4.96 min for the control group. The mean NMC time of study group was significantly longer than that of the controls (p < 0.001). In the comparisons of the two groups in terms of the prevalence of hearing loss, no statistically significant difference was found at any of the evaluated frequencies (p > 0.05, for all). There was also no statically significant difference between groups in relation to hearing thresholds at these frequencies (p > 0.05, for all).

Conclusion: The NMC time was significantly prolonged in patients with PEX syndrome. This result indicates that nasal mucosa cilia, which have similar elastic and mobile structures as the iris, are damaged in PEX syndrome, which is an infiltrative disease increasing the rigidity of affected tissues. In upper respiratory tract infections of unknown cause, PEX syndrome should be considered among the etiological factors. In this study, no significant relationship was detected between PEX and hearing loss.

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00405-020-06461-2DOI Listing

Publication Analysis

Top Keywords

pex syndrome
16
hearing loss
12
study group
12
nmc time
12
nasal mucociliary
8
mucociliary clearance
8
patients pex
8
control group
8
saccharine test
8
hearing thresholds
8

Similar Publications

Purpose: To compare changes in angle morphology, anterior chamber depth (ACD) and refractive prediction error (PE) after phacoemulsification between pseudoexfoliative (PEX) and non-PEX eyes.

Methods: Prospective case-control study of eyes submitted to cataract surgery. Biometric data and angle parameters - Anterior Chamber Angle (ACA), Angle Opening Distance (AOD), Scleral Spur Angles (SSA) and Trabecular Iris Space Area (TISA) - were measured preoperatively and 1-month postoperatively through swept-source anterior segment optical coherence tomography.

View Article and Find Full Text PDF

Susceptibility to pseudoexfoliation linked to intronic variant rs4926246 in CACNA1A: Evidence from an Indian population study.

Biochim Biophys Acta Gene Regul Mech

January 2025

School of Biological Sciences, National Institute of Science Education and Research (NISER) Bhubaneswar, P.O. Bhimpur-Padanpur, Jatni, Khurda, Odisha 752050, India; Homi Bhabha National Institute (HBNI), Training School Complex, Anushaktinagar, Mumbai 400094, India. Electronic address:

Pseudoexfoliation (PEX) is an age-related, complex systemic disorder of protein aggregopathy. It is characterized by the extracellular fibril depositions, termed PEX fibrils, initially observed in various organ tissues during pseudoexfoliation syndrome (PEXS) and with significant prominence in the eye during advanced pseudoexfoliation glaucoma (PEXG). The study explores the association between CACNA1 A (calcium channel, voltage-dependent, P/Q type, alpha 1 A subunit) variants and PEX in an Indian population.

View Article and Find Full Text PDF

Zellweger spectrum disorder presenting with opsoclonus-myoclonus-ataxia syndrome: a case report on immunotherapy.

Acta Neurol Belg

January 2025

Department of Pediatrics, Neurology Unit, University of Health Sciences, Ankara Etlik City Hospital, Ankara, Turkey.

Introduction: Zellweger spectrum disorder (ZSD) refers to a group of autosomal recessive genetic disorders that affect multiple organ systems and are predominantly caused by pathogenic variants in PEX genes. ZSD present a wide clinical spectrum, ranging from the most severe form, Zellweger syndrome, to the mildest form, Heimler syndrome.

Case Report: A 14-month-old male patient was brought to our clinic with recent-onset ocular tremors and unsteady gait.

View Article and Find Full Text PDF

Objectives This study aimed to identify the etiology and the direction of dislocation of the natural crystalline lens or intraocular lens (IOL) in IOL intrascleral fixation surgery and to determine the change in intraocular pressure (IOP) after surgery. Methods We retrospectively investigated the diagnosis, direction of lens and IOL dislocation, and IOP before and after surgery (preoperatively and one day, one week, and one month postoperatively) in 236 eyes from 228 patients who underwent IOL intrascleral fixation at Chiba University Hospital between February 2015 and September 2020. Results IOL intrascleral fixation was performed in 48 (20.

View Article and Find Full Text PDF

Zellweger syndrome; identification of mutations in and gene in Saudi families.

Ann Med

December 2025

Department of Basic Medical Sciences, College of Medicine & Center for Genetics and Inherited Diseases, Taibah University Medina, Medina, Saudi Arabia.

Background: Peroxisome biogenesis disorders (PBD) affect multiple organ systems. It is characterized by neurological dysfunction, hypotonia, ocular anomalies, craniofacial abnormalities, and absence of peroxisomes in fibroblasts. PBDs are associated with mutations in any of fourteen different genes, which are involved in peroxisome biogenesis.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!