The journal of international advanced otology最新文献

Hearing loss is a widespread condition throughout the world. It may affect patients from newborns to the elderly. There are too many reasons for hearing loss, including congenital hearing loss, virus infections, age-related situations, and traumatic situations, which may be related to the immune-mediated system. Fifty percent of hearing loss is related to genetic mutations and defects; genetic causes are highly heterogeneous, so the analysis of new variants are important for diagnosis. We aimed to describe the importance of detected gene variations by using targeted gene panels in the Next-Generation-Sequencing (NGS) platform. Eighty-one hearing loss targeted genes were investigated using Illumina NextSeq550 technology in 100 participants with hearing loss between 2017 and 2022 in our Genetic Diseases Evaluation Center. Targeted genes were performed on 100 patients with hearing loss diagnosis. The total number of detected variants was 77. Forty-seven cases have likely pathogenic/pathogenic variants. Thirty of them have uncertain clinical significance variants, and from the detected variants, 8 are novel. In this research, we highlighted that earlier detection of hearing loss using molecular genetic methods may help us understand the etiology and orient for a better prognosis. Results detected by using the NGS platform can assist and improve the diagnosis. In this study, the diagnostic rate with targeted genes was detected as 35.29%. It has an important role in clinical practice as the recommendation of cochlear implants. Clarifying the genotype and phenotype correlation helps us figure out the etiology of hearing loss and also the worth of genetic counseling in hereditary hearing loss.

This study aimed to compare total endoscopic ear surgery (TEES) and microscopic postauricular canal-wall-down tympanomastoidectomy (CWD) in cholesteatoma surgery in our clinic. This study included 59 patients, of whom 30 and 29 were operated on with CWD in 2016-2018 and TEES in 2019-2021, respectively and compared regarding intraoperative findings, hearing outcomes, long-term outcomes, and recidivism rates between groups. This study excluded patients in stage IV according to the European Academy of Otology and Neurotology/Japan Otological Society Staging System on Middle Ear Cholesteatoma, aged < 18, with congenital cholesteatoma, who underwent revision surgery. Two patients in the TEES group had recidivism (6.9%), with recurrent disease observed in both patients and residual disease in none, whereas 3 patients in the CWD group had recidivism (10%), including recurrent disease in 2 and residual disease in 1 patient. Tympanic membrane perforation occurred in 2 (6.9%) and 1 (3.3%) patients in the TEES and CWD groups, respectively. The 2 groups revealed no significant difference in terms of recidivism and perforation rates (P=1.000, P = .612). The CWD group had a longer mean operation time (225.54 ± 47.86 minutes) than the TEES group (160.55 ± 24.98 minutes) (P < .001). The 2 groups demonstrated no significant difference regarding pre- and postoperative air-bone gap (ABG) and ABG gain (P = .105, P=.329, P=.82, respectively). Total endoscopic ear surgery provides similar results in terms of hearing, recidivism, and long-term outcomes with the microscopic CWD approach. However, the CWD approach is still important, especially in patients in advanced stages.

In cochlear implant recipients, the diagnostic value of magnetic resonance imaging (MRI) scans is reduced by image artifacts. The static magnetic field of a 3.0T scanner is associated with the risk of implant demagnetization. The development of rotatable implant magnets aimed to support the advancement of 3.0T MRI scanners and eliminate the risk of demagnetization of cochlear implant magnets. This study aimed to compare the image artifacts caused by first-t and second-generation rotatable cochlear implant magnets in 3.0T MRI. Three Tesla MRI T2W TSE sequences were performed on 3 subjects with first- and second-generation rotatable cochlear implant magnets. The cochlear implant was fixed to the head at the implantation position by a swim cap. The size of the image artifact was determined in the transverse plane. Intraindividual comparative analyses showed that within the margin of combined uncertainty of 5 mm at a resolution of 2 mm, the cochlear implant-induced image artifacts in all subjects showed for both (first- and second-generation rotatable cochlear implant magnets), the same maximum image artifact dimension of 125 mm. We could show that no difference in image artifact size was detected within the margin of error determined by resolution, localized induced shift of the scan, and reproducibility of the tilt angle of the head relative to the chest in a living subject. Assumed improved magnet attachment can be reached without compromising of the magnet artifact size.

Since the physiological background of motion sickness is not entirely clear, it was aimed to examine the physiological differences in groups consisting of individuals susceptible and non-susceptible to motion sickness. Sixty subjects [motion sickness (MS) group: 33 female, 3 male; 28.8 ± 8.1 years; control group: 19 female, 5 male; 24.5 ± 4.3 years] were included in the study. Near visual acuity test on the treadmill in the presence of visual stimulation, pattern visual-evoked potentials, oculomotor tests, and computerized dynamic posturography were applied. Receiver operating characteristic analysis was performed to determine the parameter that provides the excellent discrimination between the groups. The most effective parameter in differentiating the study groups was determined as dynamic visual acuity with 77.8% sensitivity and 95.8% specificity. Significant differences were found in the vestibular (mean ± standard deviation: 0.63 ± 0.17), visual (0.77 ± 0.18), and composite scores (73.11 ± 11.89) of the patients (P=.000) in posturographic evaluation. In the visual-evoked potential examination, a significant decrease was found in the amplitude values between the P100-N145 waves in the binocular (5.0 ± 2.8, P=.002), right eye (7.6 ± 3.2, P=.009) and left eye (7.9 ± 2.9, P=.016) in the symptomatic patients. In binocular oculomotor evaluation, directional asymmetric findings were obtained. It has been shown that the most effective test parameter that distinguishes the MS susceptible and non-susceptible individuals is the dynamic visual acuity value. Based on the results of neuro-physiological tests, it was suggested that a possible visual-vestibular integration disorder in individuals susceptible to motion sickness may affect visual and vestibular performance.

Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive rare disease condition in which audiological deficit is also observed. A 4-year-old male child with PMD underwent an audiological evaluation. The results suggested normal middle ear and outer hair cells functioning, with only peak I of the auditory brainstem response present until 30 dBnHL. Further, the cervical vestibular evoked myogenic potential showed delayed latencies with normal amplitudes. In this case report, we attempt to explain the audio-vestibular test results and correlate them with the pathophysiology. This is the first report on the cervical vestibular myogenic potentials in patients with PMD.

Hemangioma is a common vascular neoplasm that arises in the head and neck regions but is rare in the petrous bone. We report the first case of a solitary cavernous hemangioma in the mastoid antrum. A 68-year-old woman visited our hospital with a complaint of tinnitus without any other symptoms. Tinnitus of the right ear occurred especially when the patient yawned or swallowed. Both tympanic membranes appeared normal on otoscopic examination. On pure-tone audiometry, mild hearing loss up to 25 dB was detected in the right ear. Temporal bone computed tomography revealed a 7.0 mm × 4.5 mm × 5 mm, solitary soft tissue mass in the aditus ad antrum. Excisional biopsy was performed under general anesthesia through the canal wall as in a mastoidectomy. The mass was completely removed without any bleeding or ossicular chain damage. The mass was confirmed as a cavernous hemangioma. During follow-up, the patient's tinnitus and right low-tone hearing loss improved. No solitary hemangioma of the mastoid antrum has been reported previously. Surgical excision of the lesion appears to be proper treatment to achieve pathologic confirmation along with resolution of symptoms.

Extended high-frequency audiometry (HFA) is considered an important tool in the detection of hearing loss. However, the values at extended high frequencies (EHF) in older adults (in both men and women) are associated with considerable uncertainty due to limited reference data. The presented review aimed to analyze hearing thresholds at EHF in adults older than 60 years. A literature search for HFA-related keyword combinations was conducted using the electronic databases PubMed, Scopus, and Web of Science. A total of 1654 records, published in the last 22 years, were identified through this search, of which only 7 articles were ultimately included in the analysis. Multiple studies have shown that significant hearing loss can be observed at EHF in older adults. Hearing thresholds in the frequency range of 9-20 kHz in the elderly varied widely across the studies. Therefore, further research in this field is needed to complete the normative data.

Several fixation methods have been described to secure the cochlear implant's receiver/stimulator, but the optimal stabilization technique is still being debated. The aim of this study was to compare the conventional technique with suture fixation to the subperiosteal tight pocket technique in terms of revision cochlear implantation rate. A retrospective review was conducted on the medical records of 649 patients who underwent cochlear implantation. The study participants were divided into different groups regarding the applied surgery technique. The relationship between the fixation technique, revision rates, and the cause of revisions related to techniques was investigated. The overall revision rate was 2.9% (19 out of 649). There were 14 (3.5%) and 5 (2%) revision implantations in the subperiosteal tight pocket and conventional technique groups, respectively. The incidence of device failure was 2.5%, and it constituted the primary cause for revision surgery in both groups. Even though patients who had the subperiosteal tight pocket technique had a much higher rate of device failure, the results indicate that there was no significant difference between the groups, as evidenced by a P-value of .12. The conventional and subperiosteal tight pocket techniques can both be safely preferred with low revision rates in patients undergoing cochlear implantation.

Eosinophilic otitis media, first reported in Japan, is a viscous, intractable otitis media often linked to bronchial asthma and chronic rhinosinusitis, characterized by highly viscous middle ear effusion. Its pathological mechanism remains unclear and the condition occasionally does not respond to steroids. It is now recognized as a rare type 2 inflammatory disease and should be treated specifically to enhance quality of life. This systematic review and meta-analysis evaluated the efficacies of biologic treatments. We searched PubMed, SCOPUS, Embase, Web of Science, and Cochrane databases up to September 2023. We retrieved ear examination findings, otitis media-related and symptom scores, air-bone gaps and hearing thresholds, serum eosinophil, and immunoglobulin E (IgE) levels before and after biologic treatments. Biologics treatment significantly improved subjective otitis media-related scores, compared with control group (standard mean difference (SMD) -1.62; 95% confidence interval (CI) [-2.24; -1.01], I2=54%). Additionally, the serum eosinophil counts and IgE levels significantly decreased (SMD -1.40; 95% CI [-1.99; -0.81], I 2=0%) after 6-12 months of biologic treatments, but the hearing thresholds did not significantly change. There were no significant differences between groups treated with dupilumab and groups treated with other biologics. Biologics treatment for eosinophilic otitis media significantly improved subjective otitis media-related scores and decreased serum eosinophil and IgE levels, but no significant changes in hearing threshold. More randomized cohort studies are needed to confirm the efficacies of biologics in patients with refractory eosinophilic otitis media.

Benign paroxysmal positional vertigo (BPPV) is a common vestibulopathy and involves failed dissolution and dislocation of calcium carbonate crystals into the semicircular canal. This causes short-lasting vertigo during changes in head position. Oftentimes, BPPV can be resolved within a single clinic visit, but secondary to many known risk factors, BPPV can recur. This case report follows a patient with extremely frequent recurrences despite a lack of known risk factors. A 55-year-old female experienced BPPV in December 2022, with successful canalith repositioning treatment from otolaryngology. On having a recurrence in March 2023, the patient underwent videonystagmography including caloric testing, and MRI, all of which showed normal findings besides left posterior-canal BPPV. From December 2022 to February 2024, the patient had 13 recurrences, each treated to resolution, confirmed by repeating positional tests and per subjective report for at least 2 weeks following. The incidence of BPPV recurrence is reported higher in females, however, this could not be interpreted as a causative factor. Though many other risk factors are documented in literature, this patient's history, demographics, imaging, and blood tests were all negative. This case report highlights a gap in knowledge of vestibular pathophysiology, as this patient's high rate of recurrence remains unexplained.