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Understanding primary ciliary dyskinesia.
Pediatr Pulmonol
October 2024
Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA.
Primary ciliary dyskinesia (PCD) is a rare, inherited disease characterized by impaired motile ciliary function leading to chronic sinopulmonary disease, persistent middle ear effusions, laterality defects, and subfertility. Over fifty PCD-associated genes have also been identified, which have provided new insights into the processes involved into ciliary assembly, structure, and function. Historically, the diagnosis of PCD was based on the presence of ultrastructural defects in the ciliary axoneme but with identification of a growing number of disease-associated genes, genetic testing has become a first-line diagnostic tool.
View Article and Find Full Text PDFHistologic and ultrastructural study of intracranial Gaucheroma causing deafness in a patient with Gaucher disease type 3: Effects of substrate reduction therapy.
Mol Genet Metab Rep
September 2024
Pathology and Laboratory Medicine, Children's Hospital Los Angeles, University of Southern California, Los Angeles, CA, USA.
Hearing loss is frequently associated with Gaucher disease (GD). Gaucher cells are enlarged reticuloendothelial cells containing glucocerebroside in the lysosomes due to deficiency of the glucocerebrosidase. Gaucheromas consist of accumulated Gaucher cells.
View Article and Find Full Text PDFPlanar Cell Polarity in the Multiciliated Epithelial Lining of the Mouse Eustachian Tube.
Laryngoscope
August 2024
Department of Otolaryngology-Head and Neck Surgery, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, China.
Objectives: Planar cell polarity (PCP) signaling, essential for uniform alignment and directional beating of motile cilia, has been investigated in multiciliated epithelia. As a complex structure connecting the middle ear to the nasopharynx, the eustachian tube (ET) is important in the onset of ear-nose-throat diseases. However, PCP signaling, including the orientation that is important for ciliary motility and clearance function in the ET, has not been studied.
View Article and Find Full Text PDFAssessment of chorda tympani function after endoscopic and microscopic middle ear surgery.
Eur Arch Otorhinolaryngol
June 2023
Otorhinolaryngology Department, Faculty of Medicine, Mansoura University, Mansoura, 35511, Egypt.
Development of the Hair Cells of the Human Cochlea: A Scanning Electron Microscopic Study.
J Microsc Ultrastruct
March 2022
Department of Anatomy, Maulana Azad Medical College, New Delhi, India.
Introduction: In the mammalian auditory system, the cochlea is the first to attain structural and functional maturity. Although ultrastructural details of the developing cochlea of lower animals have been elucidated in the last few decades, comprehensive studies on human cochlea are lacking.
Materials And Methods: In the present investigation we studied the development and maturation of the hair cells of ten human fetal cochlea from gestational weeks (GW) 12 to 37 by scanning electron microscopy.
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