Comprehensive Etiologic Analyses in Pediatric Cochlear Implantees and the Clinical Implications.

Cochlear implantation is the treatment of choice for children with profound sensorineural hearing impairment (SNHI), yet the outcomes of cochlear implants (CI) vary significantly across individuals. To investigate the CI outcomes in pediatric patients with SNHI due to various etiologies, we prospectively recruited children who underwent CI surgery at two tertiary referral CI centers from 2010 to 2021. All patients underwent comprehensive history taking, next generation sequencing (NGS)-based genetic examinations, and imaging studies. The CI outcomes were evaluated using Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores. Of the 160 pediatric cochlear implantees (76 females and 84 males) included in this study, comprehensive etiological work-up helped achieve clinical diagnoses in 83.1% (133/160) of the patients, with genetic factors being the leading cause (61.3%). Imaging studies identified certain findings in 31 additional patients (19.3%). Four patients (2.5%) were identified with congenital cytomegalovirus infection (cCMV), and 27 patients (16.9%) remained with unknown etiologies. Pathogenic variants in the four predominant non-syndromic SNHI genes (i.e., SLC26A4, GJB2, MYO15A, and OTOF) were associated with favorable CI outcomes (Chi-square test, p = 0.023), whereas cochlear nerve deficiency (CND) on imaging studies was associated with unfavorable CI outcomes (Chi-square test, p < 0.001). Our results demonstrated a clear correlation between the etiologies and CI outcomes, underscoring the importance of thorough etiological work-up preoperatively in pediatric CI candidates.

Data for pressure ulcers and skin infections after cochlear implantation.

This article contains data concerning the research article entitled "Pressure ulcers and skin infections after cochlear implantation: A delayed yet serious issue" (Hui-Shan Hsieh, Chee-Yee Lee, Hung-Pin Wu, Ming-Ying Zhuo, and Chung-Feng Hwang) [1]. This data article reports the causes of skin flap pressure ulcer over the antenna site and protocol for the clinical managements. The patients with cochlear implant (n = 315) were enrolled. We used the National Pressure Ulcer Advisory Panel (NPUAP) pressure injury staging system to grade injury severity in all patients. The data included in this article are as follows: the clinical characteristics of patients, baselines variables between groups with and without pressure ulcer, the severity of skin flap reactions based on the NPUAP pressure injury system and corresponding interventions, related clinical details of patients with pressure ulcer, This article will be valuable for routine clinical practice as serving as a paradigm.

Management Options for Patients With Nasopharyngeal Carcinoma Undergoing Cochlear Implantation.

OBJECTIVES: Cochlear implantation (CI) has been successfully used to restore hearing in patients with severe to profound hearing loss. In addition to hearing loss, irradiated patients with nasopharyngeal carcinoma (NPC) also often have chronic otitis media and Eustachian tube (ET) dysfunction. These features may result in difficulties in CI. We conducted this study to review the management options for irradiated patients with NPC undergoing CI. Furthermore, we investigated the results of CI in irradiated patients with NPC with chronic otitis media and obstructive ET dysfunction, accordingly to different treatment strategies. METHODS: We retrospectively reviewed the data of patients with NPC who underwent CI in our hospital between 2006 and 2020. All subjects were found to be eligible according to the following inclusion criteria: had NPC, received irradiation, underwent cochlear implant surgery, and had chronic otitis media with obstructive ET dysfunction. RESULTS: A total of 9 patients with obstructive ET dysfunction and chronic otitis media underwent cochlear implant surgery. Seven patients with an inflamed ETs underwent a 2-stage operation. Two patients with obliterated ETs, we performed a single-stage CI surgery. Only 1 patient had otitis media effusion after the surgery. CONCLUSION: With the proper management of chronic otitis media and ET dysfunction, cochlear implant is an effective treatment for irradiated patients with NPC when conventional hearing aids failed.

Pressure ulcers and skin infections after cochlear implantation: A delayed yet serious issue.

OBJECTIVES: Skin flap infection is one of the most common complications of cochlear implantation (CI). We identified the causes of skin flap pressure ulcer over the antenna site and proposed wound management strategies. METHODS: A total of 250 consecutive pediatric patients who underwent CI to treat profound hearing loss were retrospectively assessed. Data on demographic characteristics, the cause of skin infection, and the time of onset were obtained. RESULTS: Seventeen patients (17/250, 6.8%) had a total of 23 skin pressure injuries in the area covering the antenna. We used the National Pressure Ulcer Advisory Panel pressure injury staging system to grade injury severity. Twelve patients had 16 (16/23, 69.6.%) stage 1 pressure injuries; the skin reaction resolved after the patients stopped wearing the device for a brief period, loosened the magnet to relieve pressure on the coil, and received topical antibiotics. Five patients with six (6/23, 26.1%) stage 2 pressure injuries and one (1/23, 4.3%) stage 3 injury, were treated with oral antibiotics. The patient with the stage 3 injury was instructed not to wear the external device for 10-14 days. The incidence of skin reactions associated with the ESPrit speech processor (0/17, 0%) was significantly lower than that associated with the Freedom (2/17, 11.8%), N5 (8/17, 47.1%), and N6 (7/17, 41.1%; p < 0.05) processors. Pressure injuries were more common in younger children (≤7 years, 100%) than in older children (>7 years, 0%; p < 0.05) most likely due to their thinner scalps. CONCLUSIONS: Early detection and treatment can prevent implant-threatening infections, particularly in younger children. We believe that better antenna designs will reduce this complication.

Tympanoplasty With or Without Balloon Eustachian Tuboplasty for Chronic Suppurative Otitis Media With Obstructive Eustachian Tube Dysfunction.

OBJECTIVE: To further elucidate the role of balloon Eustachian tuboplasty (BET) in tympanoplasty, we conducted a study to compare the outcomes of tympanoplasty with and without BET for the treatment of chronic suppurative otitis media (CSOM) with obstructive Eustachian tube dysfunction (OETD). STUDY DESIGN: Case control study. SETTING: Tertiary referral center. PATIENTS: A total of 70 ears diagnosed with CSOM (tubotympanic type) and OETD were included in this study. Thirty-five patients were prospectively enrolled for BET and tympanomastoidectomy between February 2018 and June 2019. Thirty-five control subjects were matched by sex and age and retrospectively enrolled for tympanomastoidectomy between July 2016 and January 2018. INTERVENTIONS: BET, tympanomastoidectomy. MAIN OUTCOME MEASURES: The graft take rate, hearing levels, and Eustachian tube function test results. RESULTS: The graft take success rate was higher in the BET group (80.0%; 28/35) than in the control group (68.6%; 24/35). However, the difference was not statistically significant. The average air-bone gap (ABG) improvement was 10.93 ±â€Š7.70 dB in the BET group and 7.11 ±â€Š8.08 dB in the control group, with a statistically significant between-group difference (p = 0.033). CONCLUSIONS: Our findings suggest that BET can objectively and subjectively improve the Eustachian tube function, with a slight but significant improvement in ABG despite the lack of a clinically significant improvement overall. However, it does not affect the graft take rate. In summary, BET could be used as an adjunctive procedure in the treatment of CSOM with OETD.

Genetic Epidemiology and Clinical Features of Hereditary Hearing Impairment in the Taiwanese Population.

Hereditary hearing impairment (HHI) is a common but heterogeneous clinical entity caused by mutations in a plethora of deafness genes. Research over the past few decades has shown that the genetic epidemiology of HHI varies significantly across populations. In this study, we used different genetic examination strategies to address the genetic causes of HHI in a large Taiwanese cohort composed of >5000 hearing-impaired families. We also analyzed the clinical features associated with specific genetic mutations. Our results demonstrated that next-generation sequencing-based examination strategies could achieve genetic diagnosis in approximately half of the families. Common deafness-associated genes in the Taiwanese patients assessed, in the order of prevalence, included GJB2, SLC26A4, OTOF, MYO15A, and MTRNR1, which were similar to those found in other populations. However, the Taiwanese patients had some unique mutations in these genes. These findings may have important clinical implications for refining molecular diagnostics, facilitating genetic counseling, and enabling precision medicine for the management of HHI.

Effects of Minimally Invasive Surgery for Patients With OSA on Quality of Life.

OBJECTIVE: To retrospectively study the effects of minimally invasive single-stage multilevel surgery (MISS MLS) for treating patients with obstructive sleep apnea/hypopnea syndrome (OSA) on quality of life (QOL). METHODS: All OSA patients with multilevel obstruction who failed or refused continuous positive airway pressure (CPAP) treatment and then underwent Pillar implants and temperature-controlled radiofrequency of the base of tongue (RFBOT) were enrolled. The subjective symptoms and QOL (SF-36 Taiwan Standard Version 1.0) parameters were collected preoperatively and a minimum of 3 months postoperatively. Postoperative morbidity and complications were also recorded. RESULTS: Thirty-seven patients (30 men, 7 women; mean age = 46.8 years; mean BMI = 23.9 kg/m2) were enrolled. The mean Epworth Sleepiness Scale changed from 12 ± 4.3 to 10.6 ± 4.3 ( P = .004, Wilcoxon signed-rank test). The mean snoring visual analog scale reduced from 9.5 ± 1.3 to 4.0 ± 2.0 ( P < .0001). The mean score of SF-36 increased from 65.5 ± 19.6 to 75.2 ± 16.7 ( P = .001). The SF-36 subscales showed statistically significant changes, especially in bodily pain, role-emotional, and general health. CONCLUSION: The study demonstrated that MISS MLS was effective on QOL in selected patients for treating OSA patients who are unresponsive to conservative OSA therapy.