Clinical Portrait of Cochlear Implantation in Patients With CHARGE Syndrome.

OBJECTIVE: To examine the clinical characteristics and auditory performance of patients with CHARGE syndrome following cochlear implantation (CI), as well as the prognostic factors affecting auditory outcomes. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary academic center. METHODS: A retrospective chart review was performed in patients with CHARGE syndrome who underwent CI from 2007 to 2022. The category of auditory performance (CAP) score was used to assess the CI outcomes, and factors that may affect the speech outcomes were also evaluated. RESULTS: In 14 children with CHARGE syndrome, 22 CIs were performed, 6 unilaterally and 8 bilaterally. The mean age at CI was 25.9 months (range: 10-62). All patients had ear abnormalities and developmental delays, and cochlear nerve deficiency (CND) was present in all ears. At the last follow-up (mean: 49.6 months), the mean CAP score improved significantly compared to the preoperative measure (from 0.36 ± 0.81 to 3.21 ± 1.70, P = .001), with 6 patients (42.9%) achieving a CAP score of 4 points or higher. However, between the unilateral and bilateral CI groups, the final CAP score or change in CAP score was similar. Factors including age, coloboma, and CND did not significantly affect speech outcomes (all P > .05). CONCLUSION: Even though CHARGE syndrome features challenging anomalies, CI can be conducted safely and can offer effective contribution to significant speech improvement. Patients with CHARGE syndrome should be given the opportunity to undergo CI to maximize their audiological progress.

Audiologic Outcomes After Vestibulotomy in Patients With Congenital Absence of the Oval Window.

OBJECTIVE: To examine the clinical features and surgical outcomes in patients with congenital absence of the oval window (CAOW), and to investigate the potential factors that affect audiologic results. STUDY DESIGN: A retrospective chart review. SETTING: A tertiary academic center. PATIENTS AND INTERVENTION: A total of 17 ears among 16 patients were confirmed to have CAOW. Among them, 13 ears underwent vestibulotomy for hearing reconstruction. Clinical parameters associated with the hearing outcomes were analyzed. MAIN OUTCOME MEASURES: A mean air-bone gap (ABG) after 6-month and long-term follow-up was compared with preoperative measurements. RESULTS: Intraoperative findings showed that anomalies of the malleus or incus were observed in 11 ears (64.7%), stapes anomalies were present in all ears (100%), and facial nerve anomalies were present in 10 ears (58.8%). Because of unfavorable facial nerve anomalies, hearing reconstruction was aborted in four cases (23.5%). In the hearing reconstruction group, the mean ABG at 6 months postoperation was significantly reduced after compared with the preoperative value (44.0 ± 8.4 dB versus 58.8 ± 9.1 dB, p = 0.006). After dividing ears into a success subgroup (ABG ≤ 30 dB, seven ears) and non-success subgroup (ABG > 30 dB, six ears), the use of a drill during vestibulotomy was significantly related to a poor hearing outcome (100% versus 16.7%, p = 0.015). The long-term follow-up result (mean, 60 mo) revealed no deterioration compared with the 6-month postoperative result. Five ears (29.4%) underwent revision surgery, and three of them showed ABG improvements. No serious complications were reported. CONCLUSION: Vestibulotomy is an effective and safe option for hearing restoration in patients with CAOW, particularly when the use of a drill is not required. The long-term audiologic outcome is also reliable.

Cochlear implantation in patients with Charcot-Marie-Tooth disease: two cases with a review of the literature.

PURPOSE: To report two cases of bilateral cochlear implantation (CI) in Charcot-Marie-Tooth disease (CMT) patients with novel mutations. Furthermore, we conducted a detailed literature review on the profile and outcomes of CI in this uncommon clinical circumstance. CASE PRESENTATION: Case 1 involved a 25-year-old woman who was referred for sudden hearing loss (HL) in her left ear and had a 7-year history of HL in her right ear. She was diagnosed with CMT type 1 with a thymidine phosphorylase gene mutation. CI was performed on her left side because her hearing gradually worsened to deafness in both ears. At 3 months post-operation, her speech discrimination score without lip-reading improved from 0 to 100%. She underwent a second CI on her right ear 6 months after her first CI. Two years from her first operation, the speech discrimination score was 100%. Case 2 received her first CI on her right ear at the age of nine for her bilateral HL. She was diagnosed with CMT type 2 with a Twinkle mitochondrial DNA helicase gene mutation. Preoperatively, the speech discrimination score in both ear-aided conditions was 70%. At the 7-year post-operation follow-up, the speech discrimination score was 76%. A second CI was performed due to decreasing hearing ability in her left ear. The speech discrimination score showed 100% at 7 months after the second CI. CONCLUSIONS: CI is an effective hearing rehabilitation option for CMT patients with severe-to-profound SNHL. Neuro-otologists should consider CI as a treatment option, even though hearing loss in CMT is associated with auditory neuropathy spectrum disease (ANSD).

Changes in Revision Cochlear Implantation and Device Failure Profiles.

OBJECTIVES: As cochlear implantation (CI) experiences rapid innovations and its indications expand, the characteristics of revision CI (RCI) are evolving. This study investigated changes in the RCI profile and explored their clinical implications. METHODS: A retrospective chart review was conducted of all CIs performed at a tertiary medical institution between October 2001 and January 2023. The rates of and reasons for RCI were evaluated in relation to the manufacturer and device model. Kaplan-Meier analysis was employed to examine cumulative and device survival curves. Cumulative and device survival rates were additionally analyzed based on age group, period of primary CI, and manufacturer. A Cox proportional hazards model was employed to evaluate the association between RCI and the device manufacturer. RESULTS: Among 1,430 CIs, 73 (5.1%) required RCI. The predominant reason for RCI was device failure (40 of 73 RCIs [54.8%]), with an overall device failure rate of 2.8%. This was followed by flap-associated problems and migration (nine of 73 RCIs each [12.3%]). Flap retention issues emerged as a new cause in three cases (two involving the CI 632 and one involving the SYNCHRONY 2 implant), and six instances of electrode tip fold-over arose (four for the CI 600 series and two for the CI 500 series). The overall 10-year cumulative and device survival rates were 93.4% and 95.8%, respectively. After excluding models with recall issues, significant differences in cumulative (P =0.010) and device (P =0.001) survival rates were observed across manufacturers. CONCLUSION: While the overall CI survival rate is stable, device failure persists as the predominant reason for RCI. Moreover, the types of complications leading to revision (including issues with flap retention and electrode tip fold-over) have shifted, particularly for newer implant models. Given the clinical importance of device failure and subsequent reoperation, clinicians should remain informed about and responsive to these trends.

Endoscopic Debridement of Post-Radiation Nasopharyngeal Necrosis: The Effects of Resurfacing With a Vascularized Flap.

OBJECTIVES: Post-radiation nasopharyngeal necrosis (PRNN) is a serious complication that severely impacts the quality of life and survival of nasopharyngeal carcinoma patients. Endoscopic debridement is considered the first-line treatment for PRNN. This study aimed to analyze clinical outcomes, focusing on the mucosal resurfacing status and the effectiveness of salvage operations. METHODS: Twenty-seven patients who underwent endoscopic debridement were retrospectively analyzed. The patients were divided into two groups according to the initial surgical modality: debridement with a nasoseptal flap (NSF; n=21) and debridement only (no NSF; n=6). Clinical features, postoperative mucosal status, internal carotid artery (ICA) rupture, survival, and final mucosal status were evaluated. The NSF group was categorized according to flap viability to analyze risk factors for flap failure. RESULTS: Regardless of the initial modality, most patients experienced symptom improvement (96.0% for headache and 100% for foul odor); however, complete cranial nerve palsy did not improve in any patients. In the NSF group, complete healing was observed in 66.7%, while all patients in the no-NSF group underwent salvage surgery because none maintained complete healing. In the NSF group, 19.0% of patients required salvage surgery. After the last operation, favorable symptom improvement was noted (100% for headache and 90.0% for foul odor), and 77.8% had completely healed mucosa, whereas only 14.8% and 7.4% had partial healing and persistent necrotic mucosal status. The necrotic or uncovered NSF subgroup showed statistically non-significant tendencies for old age, advanced necrosis stage, advanced T stage, ICA involvement, high frequency and dose of radiation therapy, diabetes mellitus, and underlying comorbidities. Two ICA ruptures and three deaths occurred. CONCLUSION: Resurfacing the nasopharynx with NSF after endoscopic debridement showed better outcomes than debridement only for PRNN treatment. Despite initial NSF failure, additional resurfacing reconstructive surgery offers advantages in symptom mitigation, quality of life, and survival.

Thyroid Gland Flap for Prevention of Pharyngocutaneous Fistula After Total Laryngectomy.

OBJECTIVES: Pharyngocutaneous fistula (PCF) is the one of the major complications after total laryngectomy (TL), which can result in saliva leakage, rupture of major vessels, and increased hospitalization. Several flaps have been tried to prevent PCF during TL, and they are usually associated with donor site morbidity and uncertain viability in case of previously irradiated patients. This study aimed to report a novel bilateral pedicled thyroid gland flap (TGF) technique for delicate and effective reinforcement of neopharynx suture line after TL. METHODS: Bilateral pedicled TGF was performed for the reinforcement of neopharynx T-shape suture sites after TL in this case series. RESULTS: A total of five cases of the TGF were performed during TL, with four of them being salvage setting following previous chemoradiation therapy (CRT). The well-adapted and viable TGF status was confirmed by post-operative computed tomography scan and normally preserved thyroid hormone levels in all patients. No post-operative infections, hemorrhage, hypothyroidism, hypoparathyroidism, or PCF were reported. CONCLUSION: The TGF is an effective and reliable flap in TL patents and should be considered as a method in neopharynx reconstruction, even in salvage TL following prior CRT.

Therapeutic Effect of Multilevel Surgery on Laryngopharyngeal Reflux in Obstructive Sleep Apnea Patients: Impact on the Reflux Symptom Index and Reflux Finding Score.

OBJECTIVES: Our previous study found that multilevel obstructive sleep apnea (OSA) surgery mitigated laryngopharyngeal reflux (LPR) symptoms in terms of the reflux symptom index (RSI), but no studies have investigated the impact of OSA surgery on laryngoscopic parameters. The aim of this study was to examine the clinical outcome of LPR improvement following OSA surgery, with a focus on both the RSI and the reflux finding score (RFS). METHODS: Prospectively collected data from 28 patients who underwent multilevel OSA surgery from 2017 to 2021 were retrospectively analyzed. Patients were asked to complete the RSI questionnaire and underwent a laryngoscopic examination to evaluate the RFS before and after surgery. Age, height, weight, body mass index (BMI), and polysomnography data before and after surgery were also reviewed. RESULTS: After surgery, the total RSI and RFS decreased significantly from 11.96±8.40 to 7.68±6.82 (P=0.003) and from 6.57±3.49 to 3.21±1.87 (P<0.001). The positive rates of RSI and RFS decreased from 28.6% to 17.9% and 32.1% to 0%, respectively. Significant improvements were found in the RSI subdomains of throat clearing, throat mucus, breathing difficulty, troublesome cough, and heartburn sensation, while all RFS subdomains except granuloma improved significantly. In subgroup analyses, no significant differences were found between subgroups based on age, OSA severity, or BMI. CONCLUSION: OSA surgery has the potential to alleviate both LPR symptoms and laryngoscopic. RESULTS: Additional research integrating more objective techniques and novel treatment strategies is required to better comprehend the clinical impact of OSA surgery on LPR.

A rapid quantification of invasive phenotype in head and neck squamous cell carcinoma: A novel 3D pillar array system.

BACKGROUND: The widely used in vitro invasion assays for head and neck squamous cell carcinoma (HNSCC) are wound healing, transwell, and organotypic assays. However, these are still lab-intensive and time-consuming tasks. For the rapid detection and high throughput screening of invasiveness in 3D condition, we propose a novel spheroid invasion assay using commercially available pillar platform system. MATERIALS AND METHODS: Using the pillar-based spheroid invasion assay, migration and invasion was evaluated in three patient-derived cells (PDCs) of HNSCC. Immunofluorescence of live cells was used for the quantitative measurement of migratory and invaded cells attached to the pillar. Expression of epithelial-mesenchymal transition (EMT)-related gene (snai1/2) was measured by qRT-PCR. We also tested the impact of drug treatments (cisplatin, docetaxel) on the changes in the invasive phenotype. RESULTS: All PDCs successfully formed spheroid at 4 days and can be measured invasiveness within 7 days. Intriguingly, one PDC (#1) obtained from the advanced stage showed robust migration, invasion and higher transcription of snai1/2, compared with the other two PDCs. Furthermore, the invasion ratio of the control spheroids was about 70% while the invasion ratios of drug-treated spheroids were lower than 50%, and the difference showed statistical significance (p < 0.01). CONCLUSION: The presented spheroid invasion assay using pillar array could be useful for the evaluation of cancer cell behavior and physiology in response to diverse therapeutic drugs.