[This corrects the article DOI: 10.1371/journal.pone.
View Article and Find Full Text PDFUsher syndrome (USH) is the most common cause of deafblindness. USH is autosomal recessively inherited and characterized by rod-cone dystrophy or retinitis pigmentosa (RP), often accompanied by sensorineural hearing loss. Variants in >15 genes have been identified as causative for clinically and genetically distinct subtypes.
View Article and Find Full Text PDFThe aim of this article is to determine the efficacy of otoendoscopy during microscopic cholesteatoma surgery on residual cholesteatoma rates postoperatively. The medical records of patients (aged 4-90) with primary acquired cholesteatoma who underwent microscopic cholesteatoma surgery (exclusively transcanal approach or canal wall-up tympano-mastoidectomy) with subsequent otoendoscopic examination (80 ears) for intraoperative cholesteatoma residues were retrospectively reviewed. All cases with mixed microscopic/endoscopic, fully endoscopic, or fully microscopic dissection were excluded, as well as cases where a canal wall-down technique was used.
View Article and Find Full Text PDFEur Arch Otorhinolaryngol
October 2024
We report a case of a severe ear infection in a 35-year-old man treated with ixekizumab for psoriasis. Ixekizumab is a humanized monoclonal antibody that selectively prevents the interaction between interleukin 17 A and its receptor. Biologicals like ixekizumab are used to achieve symptom relief in autoimmune diseases including psoriasis.
View Article and Find Full Text PDFInt J Pediatr Otorhinolaryngol
July 2024
Objectives: Increased neonatal referral rate of conductive hearing loss (CHL) related to otitis media with effusion (OME) following universal neonatal hearing screening (UNHS) may cause an unnecessary clinical, emotional, and financial burden. This study analyzes the long-term, audiological, and medical characteristics of CHL associated with OME in neonates in order to establish a standardized protocol following technology-driven improvements in detection and referral rates in UNHS.
Methods: A retrospective study of all neonates with OME-related CHL referred to the University Hospital of Leuven (Belgium) after failing UNHS with the MAICO devices between January 1, 2013 and December 31, 2021 was performed.
Background: A combined vestibular (VI) and cochlear implant (CI) device, also known as the vestibulocochlear implant (VCI), was previously developed to restore both vestibular and auditory function. A new refined prototype is currently being investigated. This prototype allows for concurrent multichannel vestibular and cochlear stimulation.
View Article and Find Full Text PDFPurpose: Vestibular implant electrode positioning close to the afferent nerve fibers is considered to be key for effective and selective electrical stimulation. However, accurate positioning of vestibular implant electrodes inside the semicircular canal ampullae is challenging due to the inability to visualize the target during the surgical procedure. This study investigates the accuracy of a new surgical protocol with real-time fluoroscopy and intraoperative CT imaging, which facilitates electrode positioning during vestibular implant surgery.
View Article and Find Full Text PDFObjective: We aim to compare the modified Veau-Wardill-Kilner push-back technique (VWK) and the Sommerlad intravelar veloplasty (Sommerlad IVVP) in terms of middle ear outcomes and oronasal fistulae frequency in three years old children.
Methods: For this retrospective cohort study, data were collected and anonymized from consecutive patients with cleft palate (with or without cleft lip) who underwent surgery in our hospital between January 2008 and December 2018. Patients with syndromic diagnoses and patients who underwent surgical treatment elsewhere were excluded.
Cochlear implantation initiates an inflammatory cascade in which both acute insertion trauma and chronic foreign body reaction lead to intracochlear fibrosis and loss of residual hearing. Several strategies have been proposed to attenuate the local reactive process after implantation, including intracochlear drug delivery. The present study gives an overview of what is being investigated in the field of inner ear therapeutics and cochlear implant surgery.
View Article and Find Full Text PDFObjectives: To assess the incidence and onset of cochlear obliteration after translabyrinthine and retrosigmoid vestibular schwannoma surgery.
Materials And Methods: We retrospectively identified a consecutive series of eighty ears in eighty vestibular schwannoma patients who were treated via a translabyrinthine or retrosigmoid approach by a single neuro-otological surgical team in a tertiary referral center from May 2011 to January 2018. Postoperative, high- resolution T2-weighted turbo spin echo three-dimensional magnetic resonance (MR) images of the posterior fossa were evaluated at the level of the membranous labyrinth and internal auditory canal.
Eur Arch Otorhinolaryngol
August 2020
Purpose: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making.
Methods: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered.
Facial nerve schwannomas are rare benign neoplasms. We report a case of a 60-year-old woman who initially presented with vestibular complaints. Magnetic resonance imaging (MRI) revealed a facial nerve schwannoma centered on the right geniculate ganglion extending in the labyrinthine segment.
View Article and Find Full Text PDFLipoid proteinosis is a rare cause of voice problems. Hoarseness is often the first clinical manifestation of this disorder and can present years before any other symptom. Therefore, it is very important as an otorhinolaryngologist to be familiar with the main characteristics of this disease.
View Article and Find Full Text PDFBackground: Laryngeal involvement in inflammatory bowel disease is rare. Only 12 cases of laryngeal involvement in Crohn disease have been reported until now. Moreover, only one case of laryngeal manifestations in ulcerative colitis has been reported so far.
View Article and Find Full Text PDFThe 22q11 deletion syndrome (22q11DS), the most frequent microdeletion syndrome in humans, presents with a large variety of abnormalities. A common abnormality is hearing impairment. The exact pathophysiological explanation of the observed hearing loss remains largely unknown.
View Article and Find Full Text PDFBackground: Tracheal autotransplantation is a reconstructive technique that allows for organ-sparing treatment of selected patients with advanced cricoid cartilage chondrosarcoma and T2 or T3 laryngeal squamous cell carcinoma (SCC) (unilateral T2 with impaired vocal fold mobility; T3 with subglottic extension and/or arytenoid cartilage fixation). This study evaluated the functional and oncologic outcomes of an optimized autotransplant technique that the authors have been using since 2003.
Methods: The study retrospectively reviewed the charts of all patients who underwent tracheal autotransplantation at the authors' center between 2003 and 2015.