The journal of international advanced otology最新文献

Background: Superficial siderosis (SS) is a chronic condition characterized by progressive hemosiderin accumulation in the central nervous system (CNS) due to chronic subarachnoid hemorrhage. Common clinical features include progressive bilateral sensorineural hearing loss (SNHL), cerebellar ataxia and myelopathy. The aim of the study was to analyze the clinical presentation, management, and outcomes of patients affected by SS with bilateral severe-to-profound hearing loss and treated with cochlear implantation (CI), with a particular focus on the course of hearing benefit.

Methods: A retrospective monocentric review was performed, including patients affected by CNS SS and profound SNHL that underwent CI between January 2012 and December 2021. Outcomes were assessed by comparing pre- and post-operative tonal and vocal hearing thresholds together with verbal perception tests.

Results: A total of 4 patients were identified, with a mean follow-up time of 48.25 months. All patients showed significant improvement of hearing threshold and verbal perception in the first 6 months after surgery (preoperative mean aided PTA of 58.3 dB with a mean open-set speech perception score of 20.3% versus postoperative mean PTA in bimodal stimulation of 41.1 dB and open-set speech perception score of 60.1%). At the last follow-up evaluation, despite progressive deterioration of auditory performances, all patients were users and maintained subjective benefit from CIs.

Conclusion: Patients affected by SS who underwent CI showed a significant improvement in auditory and auditory-verbal performances in the first post-operative period. Despite a progressive decline of hearing benefit due to retrocochlear disease progression, CI represents valid support for lip-reading and spatial orientation even in the advanced stages of the disease.

Background: 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.

Methods: We analyzed 755 patients diagnosed with SSNHL at a single tertiary center between January 2014 and March 2020. All underwent temporal bone MRI. Logistic regression was used to determine the associations of SSNHL types with VS. Additionally, we conducted a correlation analysis to examine the relationship between word recognition scores (WRS) and the size of tumors measured on MRI at the time of diagnosis.

Results: Magnetic resonance imaging identified VS in 23 of 755 cases (3.0%). Of the patients, 123 (16.3%) had low-frequency SSNHL; none of these patients had VS. The incidence of VS differed significantly between the low-frequency SSNHL group and other SSNHL types (P < .001). A significant negative correlation was found between initial tumor size and WRS (r2 = 0.2905, P=.008).

Conclusion: The prevalence of VS was rare in patients with low-frequency SSNHL. The pattern of hearing loss may provide insights into the likelihood of tumor presence, thereby aiding in more effective audiometric screening and decision-making regarding MRI for detecting VS.

Background: 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.

Methods: Forty participants between 18 and 25 years of age were divided into 2 groups. Group 1 included 20 regular PMSs users from 2 to 3 years, and group 2 comprised 20 participants who were non-regular PMSs users. Detailed audiological evaluations were carried out on 15 participants in each group, and vestibular evaluations were carried out on all the participants.

Results: It was observed that the extended high-frequency hearing thresholds and otoacoustic emissions were affected in the regular PMSs users. The gap in noise test and vestibular evoked myogenic potential testing revealed that temporal resolution abilities and vestibular system functioning are also compromised among regular PMS users.

Conclusion: Thus, this study highlights the subtle vestibular and auditory impairments that PMS may produce in young adults, as well as the significance of a battery of tests to detect them.

Background: 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.

Methods: This monocentric, retrospective study included 712 patients with BP, divided into 103 patients with HbA1c ≥ 6.5% and 609 patients with HbA1c < 6.5%. Receiver operating characteristic curve analysis was used to evaluate the main factors affecting HbA1c levels. Propensity score matching (PSM) was further utilized to avoid selection bias and disproportionate distributions of confounding factors between the 2 groups. The House-Brackmann (H-B) facial nerve grading system was employed to assess the severity of facial motor dysfunction.

Results: Analysis showed that high HbA1c patients were older, had higher body mass index, less frequently suffered from dysgeusia, and more often had hypertension (P < .05). According to the area under the curve, age had the greatest impact on HbA1c levels (95% CI=0.748-0.803, P <.001). After PSM 1 : 1 matching, there was no statistical difference in initial H-B grade between the 2 groups, but there was a statistical difference in final H-B grade (P = .023), indicating a worse prognosis for patients with BP in the high HbA1c group.

Conclusion: The study, after controlling for confounding factors, showed that patients with BP and high HbA1c have a worse prognosis, suggesting that controlling blood glucose levels has a positive significance for the recovery of patients with BP.

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.

Background: 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.

Methods: Sprague-Dawley rats from postnatal 0 to 21 days (p0-p21) were utilized for the experimental model and classified into 4 groups by age randomly: p0, p7, p14, and p21. p3 SD rats were employed to culture primary marginal cells (MCs). The quantitative reverse transcription polymerase chain reaction (qRT-PCR) and immunofluorescence technique were employed to identify Cav1.3 in vivo. Immunocytochemistry was applied to explore the expression of Cav1.3 in vitro.

Results: Quantitative reverse transcription polymerase chain reaction showed that the Cav1.3 calcium channel gene (CACNA1D) was measured in the cochlear lateral wall (STV), which included SV and spiral ligament. Immunofluorescence photographs revealed that Cav1.3 was intensively expressed in the SV, weakly expressed in the spiral ligament and spiral prominence. In the SV, immunofluorescence labeling of Cav1.3 was present in MCs, intermediate cells, and basal cells. Immunocytochemistry confirmed that Cav1.3 was expressed in the cytomembrane of the MCs. Moreover, the expression of Cav1.3 was increased in the developmental STV at both the protein and mRNA levels.

Conclusion: Cav1.3 was mainly present in the cytomembrane of MCs in the SV of postnatal SD rats. Additionally, Cav1.3 protein and mRNA increased with development in the cochlear lateral wall of SD rats, including in the SV. The alteration of Cav1.3 expression may influence the homeostasis of ions and benefit the normal function and development of the cochlea.