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.

Comparison of the effects of triglyceride variability and exposure estimate on clinical prognosis in diabetic patients.

BACKGROUND: Hypertriglyceridemia is an important feature of dyslipidemia in type 1 and type 2 diabetic patients and associated with the development of atherosclerotic cardiovascular disease. Recently, variability of lipid profile has been suggested as a residual risk factor for cardiovascular disease. This study compared the clinical impact of serum triglyceride variability, and their cumulative exposure estimates on cardiovascular prognosis in diabetic patients. METHODS: A total of 25,933 diabetic patients who had serum triglyceride levels measured at least 3 times and did not have underlying malignancy, myocardial infarction (MI), and stroke during the initial 3 years (modeling phase) were selected from three tertiary hospitals. They were divided into a high/low group depending on their coefficient of variation (CV) and cumulative exposure estimate (CEE). Incidence of major adverse event (MAE), a composite of all-cause death, MI, and stroke during the following 5 years were compared between groups by multivariable analysis after propensity score matching. RESULTS: Although there was a slight difference, both the high CV group and the high CEE group had a higher cardiovascular risk profile including male-dominance, smoking, alcohol, dyslipidemia, and chronic kidney disease compared to the low groups. After the propensity score matching, the high CV group showed higher MAE incidence compared to the low CV group (9.1% vs 7.7%, p = 0.01). In contrast, there was no significant difference of MAE incidence between the high CEE group and the low CEE group (8.6% vs 9.1%, p = 0.44). After the multivariable analysis with further adjustment for potential residual confounding factors, the high CV was suggested as an independent risk predictor for MAE (HR 1.19 [95% CI 1.03-1.37]). CONCLUSION: Visit-to-visit variability of triglyceride rather than their cumulative exposure is more strongly related to the incidence of MAE in diabetic patients.

Percutaneous Bone-Anchored Hearing Implant: Is It Clinically Useful in Korean?

BACKGROUND: The aim of this study is to investigate the clinical effectiveness of Ponto in Korea, a recently released percutaneous bone-anchored hearing implant. METHODS: 16 patients with single-sided deafness (SSD) and mixed or conductive hearing loss who underwent Ponto implantation from December 2018 to September 2020 were enrolled in the study. Puretone audiometry, the Korean version of the Hearing in Noise Test (K-HINT), sound localization test (SLT), and Pupillometry were performed pre- and three months post-operation. Standardized questionnaires, the Hearing Handicap Inventory for the Elderly (HHIE) and Speech, Spatial and Qualities of Hearing Scale (SSQ), were administered. RESULTS: The mean age of subjects was 55.5 (range, 48-67) years. Four males and 12 females participated in the study. The mean puretone average was 73.17 dB hearing level (HL) before surgery and significantly improved to 36.72 dB HL three months after surgery. The mean word recognition score improved from 26.0% to 90.75% after implantation. In the case of K-HINT, there was a significant difference in summation (Z = -2.250, P = 0.024) and head shadow effects (Z = -3.103, P = 0.002). There was no significant difference in root mean square error degree (RMSE) and hemifield identification scores for SLT testing. Pupillometry was performed to measure listening effort and the results revealed that the degree of pupillary dilatation decreased under the condition of quiet, 0 dB signal to noise ratio (SNR) and 3 dB SNR. The total score for HHIE decreased significantly (Z = -3.130, P = 0.002) while the SSQ score increased significantly (Z = -2.216, P = 0.027). CONCLUSIONS: The Ponto bone-anchored hearing system showed significant clinical benefit in Korean patients with conductive and mixed hearing loss and SSD.

Removal and Repositioning of a Piston Wire Prosthesis That Entered the Vestibule Secondary to Trauma in a Patient Who Underwent Stapedotomy.

Otosclerosis is a common cause of adult-onset hearing impairment, and stapedotomy is often performed as surgical treatment. Several studies have reported the complications of stapedotomy surgery; piston wire prosthesis (PWP) disruption or dislocation secondary to indirect force attributable to head trauma is described in many patients. Most PWPs that get displaced are slanted or are completely dislodged from the stapedotomy site and lodged within the middle ear. PWP dislocation into the vestibule is extremely rare. A 65-year-old woman who was involved in a traffic accident underwent computed tomography, which revealed a right-sided PWP in the vestibule. Two weeks after the accident, we observed conductive hearing loss associated with a large air-bone gap (ABG, 47 dB) accompanied by spontaneous nystagmus directed to the right without any change in nystagmus following changes in head or body position. She underwent endoscopic exploratory tympanotomy under general anesthesia, 23 days after the injury. We gently pulled the PWP from the vestibule and repositioned it at its original site with a length of 5.2 mm on the long process of the incus. Pure tone audiometry performed 8 months postoperatively showed a decrease in the ABG from 47 to 10 dB without any complications.

Effects of Sequentially Combined Arytenoid Adduction and Injection Laryngoplasty in Patients With Unilateral Vocal Fold Paralysis.

OBJECTIVES: Arytenoid adduction (AA) and injection laryngoplasty (IL) are major surgical options for the treatment of unilateral vocal fold paralysis (UVFP). AA is a laryngeal framework surgery and IL is a soft-tissue augmentation procedure. Therefore, the effect of each intervention will not be substitutive but complementary to the other. METHODS: Patients who received AA and IL were enrolled (N = 43). Mean age was 60.1 ± 12.7 years. Objective and subjective voice parameters including maximum phonation time (MPT), jitter, shimmer, noise to harmonic ratio (NHR), grade of dysphonia (G), and voice handicap index (VHI)-30 were collected preoperatively and 6 months postoperatively. AA and IL were sequentially performed with time interval; 28 (65.1%) patients received IL first followed by AA (IL+AA group) and 15 (34.9%) had AA followed by IL (AA+IL group). Time interval between first and second procedures was 9.9 ± 14.6 months. RESULTS: MPT, jitter, shimmer, NHR, G and VHI-30 significantly improved by both first and second procedures (P < 0.001). When we evaluated IL+AA group and AA+IL group separately, the final outcomes of MPT, jitter, G, and VHI-30 between the two groups were not significantly different. When the overall effects of IL and AA were compared, MPT significantly improved with AA than with IL (P < 0.001). CONCLUSION: In patients with unilateral vocal fold paralysis, sequential AA and IL (or IL and AA) provided additional improvement of subjective and objective voice parameters. Final outcomes of the two combined procedures resulted in similar degree of voice improvement regardless of the order of procedure.

Validation of inner ear MRI in patients with Ménière's disease by comparing endolymphatic hydrops from histopathologic specimens.

Intravenous gadolinium-enhanced inner-ear magnetic resonance imaging (IV-Gd inner-ear MRI) has been used to visualize endolymphatic hydrops (EH) in clinical diagnosis of Ménière's disease (MD). However, lack of histological validation has led to several concerns regarding how best to interpret the resulting images. Here, we compared hydropic changes in temporal bone specimens with the results of IV-Gd inner-ear MRI in patients with MD. Histopathologic images of temporal bones from 37 patients with MD and 10 healthy controls were collected from the National Temporal Bone Bank of the Massachusetts Eye and Ear Infirmary in the United States. The EH ratios in the vestibule and cochlea were calculated from temporal bones using the methods used for IV-Gd inner-ear MRI, and the degree to which the saccular and utricular hydrops contributed to vestibular hydrops was measured. The presence of hydropic change in each semicircular canal was assessed using temporal bone images and compared with IV-Gd inner-ear MRI scans of 74 patients with MD. Based on human temporal bone imagery, the EH ratios in the cochlea and the vestibule on the affected side were 0.314 and 0.757, respectively. In the healthy control group, the ratio was 0.064 for the cochlea and 0.289 for the vestibule; these values were significantly different from those for the affected side of MD patients. The values for the affected ear were similar to the ratios from the IV-Gd inner-ear MRI scans in MD patients. In the vestibule, saccular hydrops were more common than utricular hydrops. The average EH ratios in the saccule and utricle were 0.513 and 0.242, respectively. No significant hydropic change from each of three semicircular canals was evident in temporal bone histopathology. However, herniation of otolithic organs (saccule or utricle) into the lateral semicircular canal was found in 44.4% of the patients, with saccular herniation (24.8%) more common than utricular herniation (16.7%). Although IV-Gd inner-ear MRI might not reflect fully the results of actual histopathology due to the limited resolution of MRI and image-processing techniques, the measured EH ratios from temporal bone specimens and IV-Gd inner-ear MRI scans were similar. Hydropic change in the three semicircular canals was not significant at either the ampullated or nonampullated end. Canal invasion of vestibular hydrops seen on MRI also appeared in temporal bone histopathology, and saccular invasion was dominant.

The Potential Esthetic Effect of Endonasal Septoplasty on the Cartilaginous Dorsum in Asian Population.

Importance: Septoplasty is used to correct nasal obstructions caused by a deviated septum. In some patients, septal deviation is also associated with external nasal deformity, which suggests that proper septoplasty can improve external deformities as well. However, objective studies of the esthetic outcome of septoplasty are limited. Objective: To investigate the potential esthetic outcome of correction of septal in addition to its functional purpose of treating nasal obstructions. Design, Setting, and Participants: Retrospective chart analysis of prospectively collected data was conducted for patients who underwent septoplasty performed by a single surgeon at our clinic. Ninety-five patients who had preoperative and postoperative medical photographs were enrolled. Main Outcomes and Measures: We measured the mid-vault dorsum angle (MDA) from a bird's eye view to evaluate the objective esthetic outcome. The nasofrontal angle and the nasolabial angle were also included in our analysis. Differences in preoperative anthropometric measurements and application of specific surgical techniques were additionally analyzed. Results: The mean age of the participants was 39.1 years (male: 85.2%, female: 14.8%). The average preoperative MDA was 3.41° ± 3.05°, and the postoperative MDA was 1.88° ± 1.09° (p < 0.001). In the severe dorsum deviation group (MDA >4°, N = 31), angle measure improvement was prominent (3.82° ± 2.97°, p < 0.001). The correlation analysis revealed that a higher preoperative MDA had a significant correlation with a higher angle improvement postoperatively (r = 0.7, p < 0.001); this outcome was also observed in the severe dorsal deviation group (r = 0.6, p = 0.003). In addition, we found a positive correlation between the bony septal deviation angle on computed tomography and preoperative MDA (r = 0.51, p = 0.003). Conclusions and Relevance: In some cases with significant external dorsum deviation, septoplasty via an endonasal approach can yield positive esthetic outcomes in addition to functional improvements. This finding will benefit patient counseling and also provide important surgical concepts in rhinoplasty with an emphasis on a gradual surgical approach to correcting external nasal deformity.

Serum creatinine level at 1-month posttransplant can independently predict long-term graft survival and functional status.

Background: After the year 2000, kidney transplants with high immunologic risk and deceased donors increased rapidly in Korea. At the same time, the medical community developed special pretransplant and early posttransplantation management protocols. Our team evaluated the effect of early graft stabilization on long-term graft survival and functional status using databases from a high-volume kidney transplantation center. Methods: We included 1,895 kidney transplant patients from a total of 1,976 performed between 2005 and 2018. Early graft failure within 1 month (n=9), loss to follow-up (n=2), pediatric recipient or donor (n=37), and combined organ transplantation (n=33) cases were excluded. We grouped the cases at 1-month posttransplantation by serum creatinine quantiles (1.0 mg%, 1.23 mg%, and 1.52 mg%). Results: After an average of 95 months of follow-up (maximum 189 months), the high-serum creatinine group (4th quantile) showed significantly poorer graft survival than other groups (1st to 3rd quantile) (P<0.05). In multiple Cox regression analysis, a high serum creatinine level (4th quantile) at 1-month posttransplant is an independent risk factor for graft failure with a hazard ratio of 1.799 (P=0.013). The quantile group by serum creatinine shows a persistent, significant difference of functional graft status (glomerular filtration rate by Modification of Diet in Renal Disease method) among quantile groups beyond ten years posttransplant. Conclusions: Serum creatinine level at 1-month posttransplant is a strong independent predictor of graft survival and functional graft status beyond ten years posttransplant.