Although multiple functions of Cav1.3 in adult rat cochlea have been explored, its role in developing stria vascularis (SV) of Sprague-Dawley (SD) rats has rarely been reported.

Primary ciliary dyskinesia (PCD) is a rare genetic disorder that affects the respiratory and auditory systems. This study aims to assess the prevalence, type, and severity of bilateral hearing loss (HL) in PCD and Kartagener syndrome (KS) patients, examining age-related differences and chronic impacts of otologic pathologies.

Younger adults frequently utilize personal music systems (PMSs) for extended periods for leisure. It has been reported in the literature that hearing abilities are affected in such individuals. However, its effect on auditory processing abilities and the vestibular system remains unclear. Hence, the present study was carried out to investigate the audiological and vestibular functioning in young adults who use PMSs regularly.

The aim of this study is to compare patients who develop facial nerve stimulation (FNS) after cochlear implantation (CI) and are managed with a triphasic stimulation pulse pattern (TPP) to those who do not develop FNS regarding the behavioral mapping parameters including the most comfortable loudness level (MCL) charge and amplitude, and the threshold level (THR), as well as the electrophysiological mapping parameters including phase duration (PD) and impedance level.

Gadolinium-enhanced magnetic resonance imaging (MRI) is the gold standard for diagnosing vestibular schwannoma (VS). This study aimed to compare the incidence of VS among patients with low-frequency, high-frequency, and flat-type sudden sensorineural hearing loss (SSNHL) and to assess the effectiveness of MRI for each SSNHL type.

Vestibular schwannoma (VS) management decisions are made within multidisciplinary meetings (MDMs). The improved accuracy of volumetric compared to linear tumor measurements is well-recognized, but current volumetric evaluation methods are too time-intensive. The aim was to determine if the availability of fully automated volumetric tumor measures during MDM preparation resulted in different radiological outcomes compared to a standard approach with linear dimensions, and whether this impacted the clinical management decisions.

Endoscopes play an important role in otologic surgery. Baby shampoos, known for their hypoallergenic, anti-irritant, and antifogging properties, are increasingly being used. The purpose of this study was to evaluate the safety and ototoxic effects of baby shampoo in an animal model using both electrophysiological and histological methods.

Acute mastoiditis (AM), a complication of acute otitis media, remains a concern despite medical advancements and often leads to severe complications such as cerebral sinus vein thrombosis (CSVT). This study aimed to characterize the clinical, microbiological, and hematological aspects of CSVT secondary to AM in children while assessing the necessity of thrombophilia evaluation in these patients.

A port-wine stain (PWS) or nevus flammeus is a congenital capillary malformation that often affects the skin of the head and neck region. Little is known about neuro-otological manifestations associated with this birthmark. We describe 2 patients with a hemifacial PWS and sensorineural hearing loss, caused by involvement of the internal auditory canal (IAC) and inner ear structures. Case one had a history of sudden vestibular hypofunction, followed by high-frequency sensorineural hearing loss 2 years later. In the second case, the exact onset of the high-frequency hearing loss could not be determined. In both patients, magnetic resonance imaging (MRI) showed dural enhancement of the IAC and a loss of T2 signal intensity of the ipsilateral labyrinth. This report shows that a PWS of the head and neck region may be associated with dural thickening within the IAC and secondary inner ear dysfunction. In patients with this capillary malformation suffering from hearing impairment or balance problems, MRI is warranted to detect involvement of the ipsilateral dura and labyrinth.

Debates and research about otosclerosis and stapes surgery have continued since the time it was described, and there is still no consensus on many issues. The aim of this article is to report the controversies in the etiology, pathophysiology, diagnosis, imaging, treatment, and management of otosclerosis and address the points of consensus of experts.

We report a case of a 74-year-old patient with Arnold-Chiari syndrome (type 1) who, due to the bilateral profound hearing loss, was qualified for cochlear implantation. The difficult anatomy of the temporal bone necessitated special preparation for the procedure. The use of neuronavigation, in combination with facial nerve monitoring, enabled a safe surgical approach to the cochlea and ensured accurate placement of the implant.

The vast majority of chondrosarcomas of the skull (CS) are located at the skull base and represent locally aggressive malignant tumors that account for 0.15% of all intracranial neoplasms. Complete surgical resection with wide surgical margins is currently the main treatment strategy, but can be hard to achieve due to the complex anatomy of the head and neck. The jugular foramen, situated in the floor of the posterior fossa and posterolaterally to the petro-occipital suture, is a remarkably rare location for CS. A case of primary CS of the jugular foramen in a 65-year-old patient is reported, presenting with otalgia, pulsatile tinnitus, and mild hearing loss in the left ear, accompanied by peripheral facial nerve paresis. Radiographic imaging showed a mass in the left mastoid, middle ear and jugular fossa with bone destruction of the jugular fossa and mastoid, while magnetic resonance imaging showed additional involvement of the petroclival fissure, hypoglossal canal, jugular bulb, and sigmoid sinus. The tumor was resected with wide margins through a Fisch infratemporal fossa approach type A, followed by radiotherapy. Results of the immunophenotyping along with histological features primarily matched moderately differentiated chondrosarcoma. The patient is recurrence free at 6 months after treatment. Middle ear discharge with facial palsy as a first manifestation of the CS has not been described to date. The unique localization, treatment details and histopathologic data are relevant in expanding the current level of knowledge on the subject.

Glycated hemoglobin A1c (HbA1c) is an indicator of blood glucose levels, but the impact of hyperglycemia on Bell's palsy (BP) remains unclear. This study aims to assess the influence of high and low HbA1c levels on the prognosis of patients with BP.

Background: External auditory canal (EAC) squamous cell carcinoma (SCC) is classified as a rare cancer and has a poor prognosis at advanced stages. Mechanical stress has been implicated in external auditory canal squamous cell carcinoma (EACSCC), but the molecular mechanism has not been elucidated. Mechanotransduction is well-known for Yes-associated protein (YAP) signaling. When YAP is translocated to the nucleus, the L1 cell adhesion molecule (L1CAM) is activated as an effector of mechanotransduction. Core fucosylation of L1CAM by Fucosyltransferase 8 (FUT8) has been implicated in the degree of tumor malignancy, modulating cleavage of the extracellular domain of L1CAM. Methods: In this study, an expression analysis of YAP, L1CAM, and FUT8 was performed by stretch assay in vitro. Immunohistochemistry was also performed in human EACSCC and normal skin specimens. Results: The labeling index of FUT8-positive cells exhibited YAP nuclear translocation under stretch stress was significantly higher in a human SCC cell line (HSC1) than in a human keratinocyte cell line. Stretch stress significantly increased the expression levels of full-length L1CAM in HSC1 cells. Moreover, colocalization of FUT8 and L1CAM was demonstrated immunohistochemically in advanced human EACSCC tissues. Conclusion: These results suggested that L1CAM expression is increased under mechanotransduction and may possibly avoid L1CAM cleavage by FUT8 modulation.

Cutaneous angiosarcomas of the scalp are rare and aggressive tumors with non-specific appearances. They rarely affect the retroauricular region, and diagnoses are often difficult and delayed. We present the case of an 87-year-old patient referred for a large, spontaneously appearing, and rapidly progressing retroauricular bruise while on anticoagulants. Initial biopsies were negative, and the cervico-facial computed tomography (CT) scan with contrast injection was inconclusive. Despite stopping the anticoagulants, the lesion persisted and extended, leading to new biopsies that revealed a high-grade cutaneous angiosarcoma. Positron emission tomography-computed tomography (PET-CT) detected homolateral supraclavicular lymph node involvement and pleuropulmonary metastases. Given the patient's deteriorating general condition and the metastatic spread of the disease, exclusive comfort care was decided. With a highly variable clinical presentation, the diagnosis of cutaneous angiosarcoma is often delayed and made at a metastatic stage. Optimal treatment remains complete surgical excision followed by radiotherapy, but this is challenging in very extensive tumors.

Mutations in the TWNK gene were described in patients with Perrault syndrome—an autosomal-recessive disease that includes hearing loss, central auditory and speech disorders, cerebellar ataxia, motor and sensory neuropathy, and ovarian dysfunction. Only around 100 cases of Perrault syndrome have been described to date. Genetically, it caused by biallelic pathologic variants in 1 of 6 genes. A literature review and a case study of Perrault syndrome are given in the article. Two mutations in the TWNK gene were detected in a 13-year-old girl with the phenotype of auditory neuropathy spectrum disorder (ANSD). The nucleotide variant c.1523A>G (p.(Tyr508Cys), NM_021830.5) was previously described; another variant c.1199G>T (p.(Arg400Leu) NM_021830.5) is a new one with an unknown population frequency. The main value of this case is the combination of mutations in the TWNK gene with the phenotype of ANSD, as well as the manifestation of the disease with hearing impairment but without neurological symptoms, unlike what was described in the literature. Specifically, in this case, progression of hearing disorders, ineffective amplification, and limited CI effect were noted. Genetic testing results suggested endocrine system testing, which revealed ovarian dysfunction at a preclinical stage; cerebellar ataxia was also diagnosed. The patient requires further monitoring by a multidisciplinary team.

McCune-Albright syndrome is a disorder of fibrous bone dysplasia complicated by skin pigmentation and endocrine abnormalities. Although temporal bone lesions are rare, surgical treatment is required when external auditory canal (EAC) stenosis develops. However, no consensus has been reached regarding surgical approaches. To safely perform surgery for temporal bone malformations, knowing the exact location of the critical organs in the temporal bone in relation to the bony lesion is important. Otherwise, intraoperative orientation may be lost owing to differences from the normal anatomy. Although image-guided surgery systems (IGSSs) would be useful in surgery for temporal bone malformations, few studies have reported on the use of IGSS in temporal bone surgery. In this report, we describe a case of McCune-Albright syndrome with EAC stenosis that was safely treated using IGSS-assisted temporal bone surgery.