Otology & Neurotology最新文献

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.

Objective: This study hypothesized that the expression levels of eosinophil extracellular traps (EETs) in middle ear lavage fluid and serum would correlate with various clinical parameters in otitis media associated with eosinophilic granulomatosis with polyangiitis (EGPA) and eosinophilic otitis media (EOM). The aim of this study was to evaluate the potential utility of EETs as diagnostic and monitoring tools for assessing disease activity and therapeutic response.

Study design: Exploratory research.

Setting: Two tertiary referral centers.

Patients: EGPA and EOM patients were eligible for inclusion. Patients with chronic otitis media (COM) were examined as controls.

Intervention: The middle ear lavage and blood samples were obtained from patients with EGPA, EOM, and COM.

Main outcome measures: The levels of cell-free deoxyribonucleic acid (DNA), citrullinated-histone H3 (cit-H3)-DNA complex, and eosinophil peroxidase (EPO)-DNA complex as EET remnants were quantified using an enzyme-linked immunosorbent assay.

Results: Patients with EGPA and EOM showed significantly higher levels of EET remnants in the middle ear lavage compared with controls. In patients with EGPA and EOM, the levels of EET remnants in the middle ear lavage at post-treatment were significantly lower compared with those at pretreatment. Furthermore, the levels of EET remnants in the middle ear lavage were positively correlated with Lund-Mackay scores and temporal bone CT scores. It is noteworthy that EET remnants in the serum of patients with EGPA were found to be elevated and were positively correlated with peripheral blood eosinophil fraction and serum immunoglobulin E levels.

Conclusions: These preliminary findings suggest that EET remnants in the middle ear lavage may be potentially useful as biomarkers for the diagnosis and disease activity of otologic lesions in EGPA and EOM. It seems acceptable to measure the levels of EET remnants using blood samples in patients with EGPA.

Objective: To optimize the procedure of auditory steady-state responses (ASSR) for monitoring hearing function during middle ear surgery.

Study design: A prospective study.

Setting: Tertiary hospital.

Patients: A total of 80 patients with conductive hearing loss who underwent middle ear surgeries were included in the study to analyze the effectiveness of the modified ASSR. Another 6 patients with unilateral sudden deafness and contralateral normal hearing were included to evaluate the interaural attenuation value of the sound field speaker.

Interventions: Changing the placement of reference electrode to avoid disrupting the surgical process, and applying contralateral masking to improve ASSR threshold accuracy.

Main outcome measures: Preoperative pure-tone audiometry (PTA) and intraoperative ASSR threshold were collected. The correlation coefficients between ASSR threshold from different electrode placements and between ASSR and PTA from the same patient were analyzed.

Results: High correlation coefficients (0.6232 to 0.8766) were observed for mastoid and mandibular electrode placements across all frequencies. Without contralateral masking, ASSR thresholds correlated well with PTA thresholds at 1, 2, and 4 kHz, except for 500 Hz (r = 0.2747). For patients with binaural PTA differences >25 dB, contralateral masking significantly improved the 500 Hz correlation coefficient to 0.5981 and increased the mean correlation index across all frequencies from 0.6111 to 0.7621.

Conclusions: Changing the placement of the reference electrode is feasible, and applying contralateral masking is necessary for patients with binaural PTA differences exceeding 25 dB.

Objectives: Hearing performance among cochlear implant (CI) users is associated with the degree of experience with using the device, but different measures of experience are available. Here, we compared 3 different measures of experience: the duration of time elapsed since activation (CI duration), the hours of device use per day (median datalogging hours), and the total cumulative device use (TDU).

Design: A retrospective analysis of 119 adult CI users (53 female) who underwent postoperative electrophysiological testing for cortical auditory evoked potentials (CAEPs). CI experience was compared between users with present and absent CAEP responses. We also examined associations between CAEP latencies and amplitudes and the degree of CI experience. For a subset of users, postoperative speech perception test scores were available, and associations with CI experience were also investigated.

Results: Significant associations were found between the presence of CAEP responses and both median datalogging hours and TDU, but not CI duration. No significant correlations were observed between CI experience and the amplitudes or latencies of CAEP waveform components. A significant correlation was observed between both median datalogging hours and TDU with speech perception in quiet test scores.

Conclusions: These findings suggest that with respect to central auditory processing and hearing outcomes, median datalogging hours and TDU are superior measures of the degree of experience compared with CI duration.

Objective: NF2-related schwannomatosis (NF2-SWN) is an autosomal dominant genetic disorder characterized by the development of schwannomas, meningiomas, and spinal ependymomas. Treatment with bevacizumab, a monoclonal antibody against VEGF, has been shown to result in decreased vestibular schwannoma size and hearing improvement in ~50% of NF2-SWN patients. It is unknown whether the same degree of benefit is seen in younger patients compared with older patients. The objective of this study is to determine the association between age and bevacizumab treatment outcomes in NF2-SWN.

Study design: Retrospective cohort study.

Setting: Tertiary referral center.

Patients: Thirty-seven patients with NF2-SWN.

Interventions: Bevacizumab.

Main outcome measures: Change in tumor size of 20% or more.

Results: This study includes 37 patients with NF2-SWN who were treated with bevacizumab at our institution between 2014 and 2024. They were divided into 2 groups: 22 adults over the age of 25 (26 to 71 y) and 15 adolescent and young adult (AYA) patients under the age of 25 (12 to 24 y). The median treatment duration was 2.1 years. A significantly higher proportion of AYA schwannomas (37.5%, n=9) exhibited radiographic tumor progression during the treatment period compared with those of the older patient group (11.9%, n=5) ( P =0.026), despite similar pre-treatment growth rates. There was no significant difference in the proportion of older and younger patients with hearing decline, improvement, or stability ( P >0.05).

Conclusions: AYA patients were significantly more likely to exhibit progression of tumor growth during bevacizumab treatment compared with older patients, though no significant differences were detected in hearing outcomes.

Objectives: The purpose of this study was to quantitatively evaluate the mucosa, cartilage, Ostmann's fatty tissue (OF), and important surrounding muscles of the eustachian tube (ET) using 3 Tesla magnetic resonance imaging (MRI), and then to compare the results between healthy ears and those with a patulous eustachian tube (PET).

Study design: Retrospective review.

Patients: Sixty-one normal subjects (control group) and 28 patients with patulous ET (patulous group).

Methods: We used oblique axial T2-weighted imaging (T2WI), the oblique axial plane T2 spc_short time inversion recovery (T2_spc_stir), the oblique parasagittal T2WI, and the oblique short axis T2WI sequences and localization methods to examine the mucosa and cartilage of the ET, the surrounding muscles, and OF. The measured structures included the following: (1) mucosa and cartilage of the ET, (2) the tensor veli palatini muscle (TVPM), (3) the levator veli palatini muscle (LVPM), and (4) the OF.

Results: There were no significant differences between the patient and control groups in terms of age, sex, mucosa, and LVPM ( P > 0.05). In the PET group, bilateral ET cartilage and TVPM thickness of the caudal areas of the ET were significantly smaller than in the control group ( P < 0.05). OF and the narrowest part of the OF were significantly smaller in the PET group ( P < 0.05). Statistically significant differences were found in thickness of the LVPM, OF, and the narrowest part of the OF according to sex ( P < 0.05); it was thicker in the male participants.

Conclusions: MRI evaluation of the ET provided detailed anatomic information about its structure and the surrounding tissues. Abnormal thicknesses of the cartilage, TVPM, and OF were specific imaging features of PET patients.

Objective: To examine the incidence, predictors, and clinical impact of iatrogenic dural tears and cerebrospinal fluid (CSF) leaks during and after the middle cranial fossa (MCF) repair of superior canal dehiscence syndrome (SCDS).

Study design: Case-control study.

Setting: Tertiary referral center.

Patients: Subjects with SCDS electing to undergo surgery.

Interventions: MCF repair of SCD.

Main outcome measures: Identification of intraoperative dural microtears and larger iatrogenic CSF leaks, as well as postoperative CSF leaks; analysis of predictive factors; and evaluation of symptomatology and audiometric outcomes.

Results: Among 426 MCF repairs, larger iatrogenic intraoperative leaks requiring lumbar drain placement occurred in 4% (n=17) of cases, and clinically significant postoperative CSF leaks occurred in 2.1% (n=9). In addition, 76 cases of pinpoint dural microtears were observed intraoperatively, all of which were successfully repaired at the time of surgery. Older age (aOR: 1.05 per year, 95% CI: 1.02-1.07), systemic hypertension (aOR: 1.90, 95% CI: 1.09-3.31), and radiographic tegmen dehiscence (aOR: 2.14, 95% CI: 1.27-3.61) independently predicted leak risk. Compared with controls, cases with CSF leak had a mean increase of 14 operative minutes (adj. β 0.24h, 95% CI: 0.09-0.39), and a 0.8-day longer hospital stay (aIRR: 1.25, 95% CI: 1.10-1.43).

Conclusion: Iatrogenic dural microtears with intraoperative CSF microleak are common (∼18%) but overwhelmingly minor and effectively repaired intraoperatively. Larger intraoperative leaks requiring lumbar drain occurred in 4% of cases, while clinically significant postoperative CSF leaks were rare (2.1%). Older age, systemic hypertension, and tegmen dehiscence are independently associated with increased leak risk, although the overall clinical impact was modest.

Background: Preserving residual low-frequency hearing has become a crucial goal in cochlear implantation, particularly for candidates eligible for electro-acoustic stimulation. Electrode array design is thought to influence the degree of cochlear trauma and hearing preservation, but direct comparisons between slim perimodiolar (PM) and lateral wall (LW) electrodes remain limited.

Aims: To compare hearing preservation outcomes between new generation PM and LW electrodes in adult cochlear implant recipients and identify clinical predictors associated with successful preservation.

Methods: A retrospective cohort study was conducted at the NextSense Cochlear Implant Centre, Sydney, involving 333 adults implanted between 2015 and 2021. All had normal cochlear anatomy and preoperative low-frequency four-frequency pure-tone averages (FFPTA) ≤80 dB HL. Patients received either LW (CI522/622, n=244) or PM (CI532/632, n=89) arrays. Hearing preservation was defined as a postoperative threshold shift ≤20 dB at FFPTA or 250 Hz, or any measurable hearing. Regression and ROC analyses were used to identify predictors.

Results: Hearing preservation rates, defined as a ≤20 dB postoperative shift in the four-frequency pure tone average (0.25, 0.5, 1, and 2 Hz), were similar between groups (36.3% LW vs. 36.4% PM, P =0.992). Both groups experienced significant threshold shifts at 250 Hz (LW: 33.2 dB; PM: 31.2 dB; P <0.0001). Age was a significant negative predictor of hearing preservation in the PM group ( P =0.0079) but not in the LW group. Poorer preoperative FFPTA was positively associated with preservation in both groups.

Conclusions: Hearing preservation is achievable with both PM and LW electrodes, with comparable outcomes. Age and baseline thresholds influenced preservation, highlighting the need for individualised electrode selection.

Objective: To assess how variations in scala tympani (ST) electrode position affect speech-perception performance, controlling for cochlear health and stimulation strategy.

Materials and methods: This retrospective cohort study included 98 postlingually deafened adult cochlear implant (CI) recipients at a tertiary referral center. Twenty-one received lateral wall electrodes (CI624; 20 mm) and 77 received perimodiolar electrodes (CI632; 18.4 mm). All implants were positioned with in the ST and programmed with the Advanced Combination Encoder (ACE) strategy. Cochlear health was quantified using pre-insertion round window electrocochleography-total response (ECochG-TR). Postoperative computed tomography with 3-dimensional reconstruction was used to determine electrode modiolar proximity (wrapping factor, WF) and angular insertion depth (AID). The primary outcome was speech perception at 6 months postactivation using CNC word scores.

Results: ECochGTR demonstrated a strong positive correlation with CNC performance (r = 0.61; 95% CI: 0.42 to 0.84). WF correlated weakly and negatively with CNC scores (r = -0.36; 95% CI: -0.53 to -0.16), indicating better outcomes with tighter modiolar proximity. AID showed a weak positive correlation with CNC performance (r = 0.29; 95% CI: 0.08 to 0.47), with deeper insertions associated with improved scores. A regression model using ECochG-TR alone underestimated performance for tightly wrapped and deeply inserted arrays and overestimated performance for lateral wall and shallower insertions. A multivariate model incorporating ECochG-TR, WF, and AID significantly improved predictive accuracy (R² = 0.51; P = 0.001).

Conclusions: Electrode position within the ST varies considerably, even within the same electrode design; perimodiolar arrays may lie along the lateral wall if over inserted, whereas lateral wall arrays may approach the modiolus depending on cochlear morphology. Incorporating electrode location and cochlear health into predictive models is essential for understanding performance differences between array types. When controlling for ECochG-TR, WF, AID, and programming strategy, perimodiolar electrodes with tighter modiolar proximity outperform lateral wall electrodes at 6 months, emphasizing the importance of these factors in optimizing CI outcomes.