Otology & Neurotology最新文献

Background: The erosive effect of chronic otitis media impairs the middle ear's ossicles. Alloplastic materials or autologous incus can be used for ossicular repair. Perichondrial roll is simple to use and saves considerable operative time and cost, especially in developing countries where ossicular prosthesis and bone substitutes are not affordable for the majority of patients.

Objective: To evaluate the use of the perichondrial roll in the reconstruction of the long process of the incus.

Subjects and methods: This study involved 62 patients diagnosed with chronic suppurative otitis media characterized by central perforation, presenting with dry and quiescent ears, and exhibiting an eroded long process of the incus, all of whom underwent tympanoplasty using perichondrial roll and bone pate for reconstruction of the long process of the incus. Audiological evaluations were done for all patients, including preoperative and postoperative evaluation of the air-bone gap and the average pure tone threshold.

Results: The mean air pure tone threshold was 21.6 dB after surgery and 41.7 dB before. The air pure tone thresholds after surgery improved significantly compared with those before (P<0.01). Before surgery, the air-bone gap was 29.6 dB at 0.5 kHz, 30.5 dB at 1 kHz, and 23.9 dB at 2 kHz. At the completion of 6 months of follow-up, the air-bone gap was 6.9 dB at 0.5 kHz, 7.1 dB at 1 kHz, and 7.3 dB at 2 kHz. The air-bone gap revealed a significant improvement in hearing (P<0.001).

Conclusion: Perichondrial roll for bridging the gap between the long process of incus and stapes head seems to be a useful and effective method in ossiculoplasty.

Purpose: To investigate changes in hearing sensitivity post cochlear implantation with MED-EL and Advanced Bionics (AB) lateral wall electrode arrays, with a focus on characterizing delayed onset hearing loss (doHL) and concurrent electrode impedance changes.

Methods: A retrospective review of 96 adult cochlear implant (CI) recipients (101 ears; MED-EL: 51, AB: 50). Hearing sensitivity was assessed using the low-frequency pure tone average (LFPTA; 125, 250, 500 Hz). Immediate decrements in hearing sensitivity were considered separately from delayed decrements. The incidence, degree, and timing of doHL were characterized. Electrode impedances were considered for individuals with doHL compared with those with stable hearing.

Results: Median immediate hearing loss was 19 dB across manufacturers. At initial activation, 73% of subjects had functional (LFPTA <80 dB HL) hearing. When all subjects with variable postoperative time periods were included, the incidence of doHL was 27% with a median degree of 24 dB. The median time doHL occurred was 76 weeks. Most (79%) subjects were categorized with gradual decrements rather than precipitous. No MED-EL subjects with doHL experienced concurrent impedance changes; 3 AB subjects did. Incidence of doHL rose to 31% and 44% when considering the subset of subjects with at least 1 year and 2 years of postoperative follow-up, respectively.

Conclusion: DoHL that was precipitous in nature was observed less frequently than previously reported. Although precipitous hearing loss tended to occur shortly after surgery, most instances of doHL occurred later than anticipated. The absence of concurrent impedance changes for recipients of MED-EL electrode arrays is consistent with previous reports.

Objective: Cochlear implant (CI) non-use and minimal-use are important, but sparsely investigated parameters. This study investigates the risk of becoming a CI non-user or a minimal-user in a post-lingual deafness adult population.

Study design: Retrospective cohort study.

Setting: Tertiary medical institution.

Materials and methods: All adult patients (18 years or above) who received CI surgery at a single center from 2010 to 2022 (n=599) were invited to participate in a questionnaire study concerning the CI usage after surgery. The questionnaire information was combined with data from medical records. Non-use was defined as never/rarely usage of the device. Minimal-users were patients who reported <4 hours of daily use, and patients considering ceasing to use their device, and not included in the non-use group.

Results: In total, 472 (78.8%) patients replied to the questionnaire regarding CI usage. Hereof, 20 patients (4.2%) were non-users and 26 patients (5.5%) were minimal-users with mean follow-up times of 8.4±3.4 years. Rehabilitation was provided for 97% of the participants by a speech-language pathologist for 6 to 12 months. Abnormal cochlear anatomy was associated with CI non-use (odds ratio=5.7, 95% confidence interval [1.1-30.8], P=0.04). No additional correlations were found between non-users, minimal-users, and CI users.

Conclusion: The CI non-user rate was 4.2% and these patients experienced lower QoL compared with CI users. In addition, 5.5% of the patients were minimal-users. In adults with abnormal cochlear anatomy, an increased risk of non-use was identified, reflecting the need for thorough information regarding surgical outcomes in this specific subgroup of patients.

Objective: Sudden sensorineural hearing loss (SSNHL) is a condition with largely unknown etiology, though both vascular and genetic components have been implicated. The X-ray repair cross-complementing group 1 (XRCC1) gene, involved in DNA repair and oxidative stress response, has been linked to ischemic stroke and noise-induced hearing loss. This study aimed to investigate the association between XRCC1 single nucleotide polymorphisms (SNPs) and SSNHL risk in Taiwan.

Design: Prospective case-control study.

Setting: Tertiary academic medical center.

Materials and methods: A total of 276 patients with SSNHL and 293 healthy controls were enrolled. Three XRCC1 SNPs (rs1799782, rs25489, and rs25487) were genotyped using TaqMan assays. All SNPs were tested for Hardy-Weinberg equilibrium. Associations with SSNHL risk were analyzed under dominant and recessive models using multivariate logistic regression. Clinical predictors of recovery were also evaluated.

Results: The TT genotype of XRCC1 rs1799782 was significantly associated with increased SSNHL risk compared with the CC genotype [adjusted odds ratio (aOR)=2.005; 95% CI=1.13-3.62; P=0.0164]. This association persisted under the recessive model (TT vs. CC+CT) (aOR=1.983; 95% CI=1.15-3.49; P=0.0134). No significant associations were observed for rs25489 and rs25487. High-tone (aOR=6.42; P = 0.0043) and flat-type (aOR=4.12; P=0.0071) audiogram patterns and longer treatment delay were linked to unfavorable clinical outcomes. XRCC1 genotypes were not predictive of treatment response.

Conclusions: The TT genotype of XRCC1 rs1799782 is significantly associated with increased SSNHL susceptibility in the Taiwanese population.

Objective: Auditory nerve monitoring through electrically evoked compound action potentials (eCAP) can be used to assess cochlear neural integrity during vestibular schwannoma (VS) resection. Herein, we report on 5 cochlear implant (CI) patients who underwent VS resection with present eCAP responses both intraoperatively and postoperatively. Patients either did not perceive any sound through their CI or rapidly adapted to electrical stimulation, suggesting that the presence of eCAP does not guarantee auditory nerve function in patients with VS resection.

Patients: Five patients who underwent simultaneous VS translabyrinthine resection and cochlear implantation.

Interventions: Simultaneous cochlear implantation and translabyrinthine microsurgical VS resection.

Main outcome measure: Electrically evoked compound action potential.

Results: All patients had eCAP responses present during their VS resection and for at least 50 days post-surgery. Three of the 5 patients did not perceive any sound with their cochlear implant. This shows that the presence of an eCAP alone does not guarantee a functional auditory nerve or auditory perception. The other 2 patients could hear non-speech sounds, but these sounds quickly became inaudible, indicating that their auditory nerve lacked the ability to integrate the electrical stimulus over time.

Conclusions: The presence of an eCAP response does not guarantee auditory sensation through a CI. Although eCAP measurements provide insight into the function of the distal end of the nerve, it does not ascertain integrity or function of the larger auditory nerve pathway. Therefore, eCAPs should not be used in isolation to monitor auditory nerve function during VS resection.

Hypothesis significant: Cochlear implant (CI) insertion trauma, as evidenced by fracture of the osseous spiral lamina (OSL), is associated with localized "dead zones" and focal spiral ganglion neuron (SGN) loss.

Background: Hearing and structure preservation approaches to CI insertion aim to minimize trauma and preserve residual SGNs. In cases of significant insertion trauma, peripheral axons running through the bony OSL inherently become damaged if the OSL is fractured. The current investigation sought to determine if the relative location of OSL fracture was associated with focal areas of SGN loss.

Methods: Five adult ears from the Mass Eye and Ear Otopathology Laboratory were identified with OSL fractures. When available, contralateral ears were also analyzed for comparison. Digitized e-Slides were used to create 3D cochlear reconstructions, and a coordinate system relative to the round window allowed for % distance along Rosenthal canal (RC) assignment of SGNs and OSL fracture locations. Abrupt changes in SGN density, defined as a drop >50% within a 5% segment along RC were used as criteria for a significant and focal SGN loss. Insertion trauma in the form of electrode translocation and spiral ligament (SL) perforation was also characterized.

Results: OSL fracture lengths ranged from approximately 72 to 109 degrees about the modiolus, with a median fracture length of 75 degrees. Total SGN counts ranged from 16,810 to 22,260. Abrupt and focal drops of >50% in SGN density occurred in all 5 temporal bones with OSL fracture. In 3 of the cases, there were areas of localized "dead zones" at or immediately adjacent to the OSL fracture site, whereas in the remaining 2 cases, drops were seen in various areas across the cochlea. The case with the longest fracture demonstrated nearly a complete loss of neurons apical to the deepest fracture location. Ears with OSL fractures demonstrate a higher number of "dead zones" than the contralateral ears used for comparison, with median values of 3 and 2, respectively. Cases with an OSL fracture, electrode translocation, and SL perforation demonstrated a notably higher number of "dead zones" (median = 3) as compared with ears with only 1 or 2 CI insertional trauma injuries (median = 2).

Conclusion: In temporal bones from CI patients, OSL fractures explain some-but not all-of the abrupt SGN loss observed following traumatic CI insertions. Distinct regions of SGN density observed across the length of these cochlea indicate multifactorial processes likely contribute to relative SGN "dead zones."

Objective: To review the effects of perioperative steroid delivery on cochlear impedances in human patients.

Data bases reviewed: PubMed, Web of Science, Embase, Ovid, and Cochrane databases were reviewed from inception through March 2025.

Methods: The Preferred Reporting Items for Systematic Reviews (PRISMA) reporting guidelines were used. Studies comparing cochlear impedances among patients who underwent cochlear implantation either with or without perioperative steroids were included. Data were collected on method, timing, and dose of steroid administration and cochlear impedance in the perioperative period.

Results: Twelve studies were included in the review, which described 4 different routes of steroid delivery: intratympanic (3), intracochlear (7), drug-eluting electrodes (1), and intravenous±oral steroids (1). Nine studies revealed a significant association between steroid use during at least one point in the perioperative period to include 2 intracochlear studies and the intravenous±oral steroids study. Intratympanic steroid delivery reduced impedances up to 6 months postoperatively, whereas intracochlear and drug-eluting electrodes had persistent reductions in impedance up to 1 year. There was significant heterogeneity in study outcomes that limited the ability to perform a meta-analysis.

Conclusions: Based on studies meeting inclusion criteria, local delivery of steroids via intratympanic, intracochlear, and drug-eluting formulations appear to lower cochlear impedances for a variable duration postoperatively. Oral steroids do not appear to affect cochlear impedances postoperatively. More studies are necessary to further elucidate the relationship between steroids and cochlear impedance.

Objectives: We present an unusual type I congenital first branchial cleft anomaly (CFBCA) characterized by a distinct sinus tract in the superior wall of the cartilaginous external auditory canal (EAC), classified as the sinus variant of type I congenital first branchial cleft anomalies (CFBCAs). This study aims to characterize its distinctive clinical features and present management approaches for this rare entity.

Methods: Medical records of patients undergoing excision for sinus variant of type I CFBCAs between January 2007 and September 2023 (a 16-y period) were retrospectively analyzed. Clinical characteristics, therapeutic interventions, histopathologic diagnoses, and long-term outcomes were systematically catalogued.

Results: The sinus variant of type I CFBCAs is a rare manifestation of CFBCAs, accounting for 11 of 188 cases (5.9%) in our series. Cardinal clinical features included recurrent periauricular inflammation (9/11 cases, 81.8%), with all cases demonstrating a characteristic concealed sinus orifice at the superior cartilaginous EAC (11/11 cases, 100%). All patients (11/11 cases, 100%) were initially misdiagnosed as lymphadenitis, epidermal cysts, or localized infections. The concealed sinus orifices at the superior cartilaginous EAC were frequently overlooked, with definitive diagnosis established by visual inspection in only 3 patients (3/11 cases, 27.3%). In the remaining 8 patients (8/11 cases, 72.7%), occult sinus orifices were identified through combined palpation and visual inspection. All lesions originated exclusively from the superior cartilaginous EAC (11/11 cases, 100%) and maintained a relative simple relationship with the facial nerve trunk (11/11 cases, 100%). Complete surgical excision of the sinus tract along with any adherent periauricular cysts was determined to be the optimal treatment approach.

Conclusions: Clinicians should maintain a high index of suspicion for the sinus variant of type I CFBCAs when evaluating pediatric patients with recurrent preauricular inflammation. The identification of a characteristic sinus orifice at the superior cartilaginous EAC during focused examination is diagnostic.

Level of evidence: Level 4.

Purpose: Otitis media with effusion (OME) is a prevalent pediatric condition, yet its molecular mechanisms remain incompletely understood. The NLRP3 inflammasome is known to regulate inflammation in various diseases, but its role in OME remains unclear. This study aimed to investigate NLRP3 activation in OME using both a murine model and clinical samples.

Methods: Experimental OME was induced in mice via intratympanic injection of lipopolysaccharide (LPS). On day 3 postinduction, middle ear tissues and lavage fluid were collected. Nlrp3 mRNA expression was assessed by qPCR, while cleaved caspase-1 and mature IL-1β protein levels were evaluated by western blotting. IL-1β levels in lavage fluid and serum were measured via ELISA. Human middle ear effusions (MEE) and matched serum samples were collected from pediatric OME patients, and concentrations of IL-1β and IL-18 were measured and normalized to total protein. To assess the functional role of NLRP3, OME was induced in Nlrp3 knockout ( Nlrp3-/- ) and wild-type (WT) mice, followed by otoscopic and histologic evaluation.

Results: LPS-induced OME mice exhibited increased expression of NLRP3, cleaved caspase-1, and IL-1β. ELISA confirmed elevated IL-1β in middle ear lavage fluid. In human samples, IL-1β and IL-18 were significantly higher in MEE than in serum. Nlrp3-/- mice showed reduced IL-1β production but no significant differences in histopathology or effusion resolution compared with WT.

Conclusion: NLRP3 inflammasome contributes to local inflammation in OME but does not significantly alter disease progression, suggesting involvement of additional inflammatory pathways.