Refine
Has Fulltext
- yes (41)
Is part of the Bibliography
- yes (41)
Year of publication
Document Type
- Journal article (41)
Keywords
- nanoparticles (4)
- auditory pathway (3)
- cochlear implantation (3)
- toxicity (3)
- zinc oxide (3)
- Fabry disease (2)
- cochlea (2)
- cochlear implant (2)
- cytotoxicity (2)
- differentiation potential (2)
- discriminant analysis (2)
- epithelial-mesenchymal transition (2)
- fpVCT (2)
- genotoxicity (2)
- head and neck squamous cell carcinoma (2)
- hearing loss (2)
- human adipose-derived stromal cells (2)
- neural stem cells (2)
- neurogenesis (2)
- neurosphere (2)
- principal component analysis (2)
- stem cells (2)
- temporal bone (2)
- zinc oxide nanoparticles (2)
- 3D analysis (1)
- ASC (1)
- App-Entwicklung (1)
- BMP-6 (1)
- CDL (1)
- CIS+ (1)
- CT (1)
- Candida albicans (1)
- Chancen (1)
- Cochlear duct length (1)
- Cochlear planning software (1)
- FTIR spectroscopy (1)
- Head and neck cancers (1)
- Interleukin (1)
- Laser Doppler vibrometer (1)
- Literaturrecherche (1)
- MOC fibers (1)
- MRI (1)
- MSCT (1)
- Meniere’s disease (1)
- OPUS (1)
- Risiken (1)
- T cell activation (1)
- T cell subpopulations (1)
- TGF-ß3 (1)
- Tuberculosis (1)
- Umfrage (1)
- Univted-States (1)
- VEMP (1)
- VSOP (1)
- ZnO (1)
- ZnO-NP (1)
- active middle-ear implant (1)
- adaptive directional microphone setting (1)
- adenotonsillectromy (1)
- adipose-derived stromal cells (1)
- apps (1)
- asbestos (1)
- aspiration (1)
- atraumatic surgery (1)
- bone conduction implant (1)
- cancers and neoplasms (1)
- cardiac dysfunction (1)
- cardiomyopathy (1)
- case-control study (1)
- cavity (1)
- cell proliferation (1)
- central auditory pathway (1)
- children (1)
- chondrogenic differentiation (1)
- chronic kidney disease (1)
- chronic rhinosinusitis (1)
- cigarette smoking (1)
- cisplatin resistance (1)
- cochlear implants (1)
- cochlear nucleus (1)
- coding strategy (1)
- computed tomography (1)
- conductive hearing loss (1)
- confocal Raman imaging (1)
- couplers (1)
- deep neck infection (1)
- development (1)
- dysphagia (1)
- electric acoustic stimulation (EAS) (1)
- electromagnetic navigation (1)
- electromyographic (1)
- endochondral ossification (1)
- epithelial barrier (1)
- extrapulmonary tuberculosis (1)
- facial nerve (1)
- fibrin (1)
- fiducial registration error (1)
- fine structure processing (1)
- fixation (1)
- flexible CO2 laser (1)
- floating mass transducer (1)
- focus of infection (1)
- formaldehyde (1)
- formalin (1)
- hardwood dust (1)
- head and neck (1)
- hearing (1)
- hearing aids (1)
- hearing preservation (1)
- high-sensitivity FEES (1)
- human plasma (1)
- immunomodulation (1)
- incus (1)
- inferior colliculus (1)
- insecticides (1)
- interelectrode-distance (1)
- iron oxide nanoparticles (1)
- irradiation (1)
- jugular paraganglioma (1)
- laryngeal tuberculosis (1)
- laser surgery (1)
- lateral neck abscesses (1)
- lateral skull base (1)
- lipids (1)
- long-term exposure (1)
- lymph node tuberculosis (1)
- lysosomal storage disorder (1)
- malignant tumors (1)
- maxillary sinus (1)
- mediastinitis (1)
- mesenchymal stem cells (1)
- microbiology (1)
- microbiome (1)
- microtubules (1)
- middle ear implant (1)
- middle-ear surgery (1)
- mixed hearing loss (1)
- molecular diagnostics (1)
- monotoring (1)
- motor neuropathy (1)
- multivariate data analysis (1)
- music (1)
- narrow band imaging (NBI) (1)
- nasal cancer (1)
- nasal lavage (1)
- nasal mucosal barrier function (1)
- nasal polyps (1)
- nasal snuff (1)
- nasal spray (1)
- nasopharyngeal carcinoma (1)
- neural progenitor cells (1)
- neural stem cell potential (1)
- neuronal maturation (1)
- neuronal oscillations (1)
- obstructive sleep apnoea (1)
- occupational exposures (1)
- organic solvents (1)
- otology (1)
- outer hair cell (OHC) (1)
- oxidative DNA damage (1)
- paediatrics (1)
- paranasal sinuses (1)
- partial deafness treatment (1)
- pegylated insulin-like growth factor 1 (1)
- pitch (1)
- polyurethane (1)
- posttranslational modifications (1)
- professional image enhancement technique (PIET) (1)
- questionnaire (1)
- raman spectroscopy (1)
- real-time PCR (1)
- regenerative capacity (1)
- renal dysfunction (1)
- retrospective (1)
- risk factors (1)
- saccotomy (1)
- salivary gland neoplasia (1)
- salivary gland tumor (1)
- salivary gland tumors (1)
- salivary glands (1)
- secondary reconstruction (1)
- silver (1)
- single-sided deafness (1)
- sinonasal cancer (1)
- smoking (1)
- speech perception (1)
- surgery (1)
- surgical drainage (1)
- surgical management of paraganglioma (1)
- surgical oncology (1)
- tight junction (1)
- tight junctions (1)
- tissue preparation (1)
- tracheotomy (1)
- tubulin (1)
- tubulin-binding cofactors (1)
- tympanic paraganglioma (1)
- tympanojugular paraganglioma (1)
- vertigo (1)
- vestibular aqueduct (VA) (1)
- vibroplasty (1)
- wood dust (1)
- wound fluid (1)
Institute
- Klinik und Poliklinik für Hals-, Nasen- und Ohrenkrankheiten, plastische und ästhetische Operationen (40)
- Institut für diagnostische und interventionelle Neuroradiologie (ehem. Abteilung für Neuroradiologie) (6)
- Lehrstuhl für Tissue Engineering und Regenerative Medizin (4)
- Pathologisches Institut (3)
- Medizinische Klinik und Poliklinik I (2)
- Augenklinik und Poliklinik (1)
- Institut für Klinische Neurobiologie (1)
- Institut für Virologie und Immunbiologie (1)
- Klinik und Poliklinik für Strahlentherapie (1)
- Lehrstuhl für Orthopädie (1)
Background
Fabry disease (FD) is an X-linked recessive hereditary lysosomal storage disorder which results in the accumulation of globotriaosylceramid (Gb3) in tissues of kidney and heart as well as central and peripheral nervous system.
Besides prominent renal and cardiac organ involvement, cochlear symptoms like high-frequency hearing loss and tinnitus are frequently found with yet no comprehensive data available in the literature.
Objective
To examine hearing loss in patients with FD depending on cardiac and renal function.
Material and methods
Single-center study with 68 FD patients enrolled between 2012 and 2016 at the Department of Oto-Rhino-Laryngology, Plastic, Aesthetic and Reconstructive Head and Neck Surgery of the University of Würzburg. Every subject underwent an oto-rhino-laryngological examination as well as behavioral, electrophysiological and electroacoustical audiological testing. High-frequency thresholds were evaluated by using a modified PTA\(_{6}\) (0.5, 1, 2, 4, 6, 8) and HF-PTA (6, 8 kHz). Renal function was measured by eGFR, cardiac impairment was graduated by NYHA class.
Results
Sensorineural hearing loss was detected in 58.8% of the cohort, which occurred typically in sudden episodes and affected especially high frequencies. Hearing loss is asymmetric, beginning unilaterally and affecting the contralateral ear later. Tinnitus was reported by 41.2%. Renal and cardiac impairment influenced the severity of hearing loss (p < 0.05).
Conclusions
High frequency hearing loss is a common problem in patients with FD. Although not life-threatening, it can seriously reduce quality of life and should be taken into account in diagnosis and therapy. Optimized extensive hearing assessment including higher frequency thresholds should be used.