Inner ear malformations constitute about 20% of congenital sensorineural hearing loss. In this review article an updated classification of cochlear malformations is provided. Incomplete partition and cochlear hypoplasia cases are each divided further into three groups. There are two main difficulties in the surgery of inner ear malformations; gusher and facial nerve abnormalities. Radiological features of malformations necessary to identify these problems preoperatively are discussed. Facial nerve abnormalities that may occur are described. Two different types of cerebrospinal fluid leakage are defined and necessary measures to prevent leakage are described. Standard and modified surgical approaches to overcome the described problems are described with literature findings. Finally meningitis which may occur with and without cochlear implantation in this special group of patients is emphasized. This is common in incomplete partition type I patients and is usually due to a fistula in one of the windows (usually oval window) which occurs as a result of cerebrospinal fluid pressure. This is a medical emergency leading to potential meningitis and measures that should be taken to stop the leak as soon as possible are described.

Cochlear implantation in inner ear malformations – a review article

Importance Sensorineural hearing loss is the third leading cause of years lived with disability worldwide. Cochlear implants may provide a viable alternative to hearing aids for this type of hearing loss. The Coverage and Analysis Group at the Centers for Medicare & Medicaid Services was interested in an evaluation of recently published literature on this topic. In addition, this meta-analysis is to our knowledge the first to evaluate quality-of-life (QOL) outcomes in adults with cochlear implants. Objective To evaluate the communication-related outcomes and health-related QOL outcomes after unilateral or bilateral cochlear implantation in adults with sensorineural hearing loss. Data Sources MEDLINE, Cochrane Central Register of Controlled Trials, Scopus, and previous reports from January 1, 2004, through May 31, 2012. Study Selection Published studies of adult patients undergoing unilateral or bilateral procedures with multichannel cochlear implants and assessments using open-set sentence tests, multisyllable word tests, or QOL measures. Data Extraction Five researchers extracted information on population characteristics, outcomes of interest, and study design and assessed the studies for risk of bias. Discrepancies were resolved by consensus. Results A total of 42 studies met the inclusion criteria. Most unilateral implant studies showed a statistically significant improvement in mean speech scores as measured by open-set sentence or multisyllable word tests; meta-analysis revealed a significant improvement in QOL after unilateral implantation. Results from studies assessing bilateral implantation showed improvement in communication-related outcomes compared with unilateral implantation and additional improvements in sound localization compared with unilateral device use or implantation only. Based on a few studies, the QOL outcomes varied across tests after bilateral implantation. Conclusions and Relevance Unilateral cochlear implants provide improved hearing and significantly improve QOL, and improvements in sound localization are noted for bilateral implantation. Future studies of longer duration, higher-quality reporting, and large databases or registries of patients with long-term follow-up data are needed to yield stronger evidence.

https://jamanetwork.com/journals/jamaotolaryngology/articlepdf/1655350/ooa120120_265_272.pdf

Cochlear implantation in adults: a systematic review and meta-analysis.

Objective: To review the literature in order to identify outcome measures used in research on adults with hearing loss (HL) as part of the ICF Core Sets development project, and to describe study a ...

The ICF Core Sets for hearing loss--researcher perspective. Part I: Systematic review of outcome measures identified in audiological research.

Objectives/Hypothesis
To investigate the impact of hearing aid and cochlear implant use on quality of life in adults.

Study Design
Prospective observational cohort study.

Methods
One hundred thirteen adults aged ≥50 years with postlingual hearing loss receiving routine clinical care at a tertiary academic medical center were evaluated with the Medical Outcome Study Short Form-36 before and 6 and 12 months after intervention with hearing aids or cochlear implants. Change in score was assessed using linear mixed effect models adjusted for age, gender, education, and history of hypertension, diabetes, and smoking.

Results
A significant increase in Mental Component Summary score was observed in both hearing aid and cochlear implant users from baseline to 12 months, with cochlear implant users increasing nearly twice that of hearing aid users (hearing aid: 2.49 [95% confidence interval: 0.11, 4.88], P = .041; cochlear implant: 4.20 [95% confidence interval: 1.85, 6.55], P < .001). The most substantial increases were observed in individuals with the lowest baseline scores. There was no significant difference in physical component summary score from baseline to 12 months.

Conclusions
Treatment of hearing loss with hearing aids and cochlear implants results in significant increases in mental health quality of life. The majority of the increase is observed by 6 months post-treatment, and we observed differential effects of treatment depending on the level of baseline quality of life score with the greatest gains observed in those with the lowest scores.

Level of Evidence
2b. Laryngoscope, 126:2110–2115, 2016

/pdf/quality-of-life-after-intervention-with-a-cochlear-implant-dub4dm9d2a.pdf

Quality of life after intervention with a cochlear implant or hearing aid.

Vitamin K (VK) is an essential cofactor for the post-translational conversion of peptide-bound glutamate to γ-carboxyglutamate. The resultant vitamin K-dependent proteins are known or postulated to possess a variety of biological functions, chiefly in the maintenance of hemostasis. The vitamin K cycle is a cellular pathway that drives γ-carboxylation and recycling of VK via γ-carboxyglutamyl carboxylase (GGCX) and vitamin K epoxide reductase (VKOR), respectively. In this review, we show how novel molecular biological approaches are providing new insights into the pathophysiological mechanisms caused by rare mutations of both GGCX and VKOR. We also discuss how other protein regulators influence the intermediary metabolism of VK, first through intestinal absorption and second through a pathway that converts some dietary phylloquinone to menadione, which is prenylated to menaquinone-4 (MK-4) in target tissues by UBIAD1. The contribution of MK-4 synthesis to VK functions is yet to be revealed.

Key Pathways and Regulators of Vitamin K Function and Intermediary Metabolism.

Objective: The purpose of this study is to present normative data on the Chinese version of the international outcome inventory for hearing aids (IOI-HA) and examine the effective-ness of the hearing aid service. Design: Cross-sectional study. The IOI-HA was administered in a paper and pencil format. Study sample: The IOI-HA and self-designed questionnaire were mailed to 1502 experienced hearing-aid users who received their hearing aids from 14 hearing service centers in mainland China. In all, 1049 completed responses were included in the final analysis, and descriptive statistics were reported. Results: The mean scores of each item of the IOI-HA ranged from 3.55 to 4.16. The IOI-HA scores showed a skewed distribution, with the maximum frequency for a score of 4 or 5. The total IOI-HA scores ranged from 9 to 35, and the mean total score was 26.3. The results compared favorably with the previous reports, and slightly higher than the published norms, indicating a high level of self-reported outcome...

International outcome inventory for hearing aids (IOI-HA): results from the Chinese version.

The role of microRNA-21-5p (miR-21-5p) in sepsis-induced acute kidney injury (AKI) has been seldom discussed. Therefore, the objective of this present study was to investigate the mechanism of endothelial progenitor cells-derived exosomes (EPCs-exos) in sepsis-induced AKI via miR-21-5p/runt-related transcription factor 1 (RUNX1) axis. miR-21-5p was downregulated and RUNX1 was upregulated in the kidney of cecal ligation and puncture (CLP) rats, and miR-21-5p targeted RUNX1. Elevation of miR-21-5p improved renal function and renal tissue pathological damage, attenuated serum inflammatory response, as well as reduced apoptosis and oxidative stress response in renal tissues, and regulated endothelial glycocalyx damage marker proteins syndecan-1 and heparanase-1 in CLP rats. Overexpression of RUNX1 abolished the impacts of elevated miR-21-5p in CLP rats. Also, EPCs-exos upregulated miR-21-5p expression, and functioned similar to elevation of miR-21-5p for CLP rats. Downregulating miR-21-5p partially reversed the effects of EPCs-exos on sepsis-induced AKI. Collectively, our study suggests that EPCs release miR-21-5p-containing exosomes to alleviate sepsis-induced AKI through RUNX1 silencing.

/pdf/endothelial-progenitor-cells-derived-exosomal-microrna-21-5p-9dsuxask45.pdf

Endothelial progenitor cells-derived exosomal microRNA-21-5p alleviates sepsis-induced acute kidney injury by inhibiting RUNX1 expression.

Background: Microwave ablation (MWA) is an effective treatment for severe secondary hyperparathyroidism (SHPT), but it can also be used for mild-to-moderate secondary hyperparathyroidism (SHPT). In...

Microwave ablation: an effective treatment for mild-to-moderate secondary hyperparathyroidism in patients undergoing haemodialysis.

Conclusion: This study indicates that Mandarin-speaking subjects acquire significant benefit from a cochlear implant (CI) and audio processor with the fine structure processing (FSP) coding strategy. It can take adult Mandarin CI users time to become accustomed to their CI and appreciate the benefits. Objectives: This study aimed to determine speech perception outcomes in Mandarin-speaking adults who received the FSP coding strategy. Methods: This was a prospective study. Ten adults who were implanted unilaterally with a PULSARCI100, and received an OPUS 1 or OPUS 2 speech processor, were included in this study. The mean age at implantation was 31.1 years. We determined the benefit to postlingually deafened Mandarin-speaking adults with a severe to profound hearing loss using the tests: monosyllables in quiet, sentences in quiet and the Mandarin Hearing in Noise Test (MHINT). Tone perception was evaluated using a tone perception test. A visual analog scale (VAS) was used to score sound quality an...

Cochlear implants with fine structure processing improve speech and tone perception in Mandarin-speaking adults

The aim of this study is to survey and compare the development of auditory skills in young children with Mondini dysplasia and profoundly-deaf young children with radiologically normal inner ears over a period of 3 years after cochlear implantation. A total of 545 young children (age 7 to 36 months) with prelingual, severe to profound hearing loss participated in this study. All children received cochlear implantation. Based on whether or not there was a Mondini dysplasia as diagnosed with CT scanning, the subjects were divided into 2 groups: (A) 514 young children with radiologically normal inner ears and (B) 31 young children with Mondini dysplasia. The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to assess the children's auditory skills that include vocalization changes, spontaneous alerting to sounds in everyday living environments, and the ability to derive meaning from sounds. The assessment was performed prior to surgery and at 1, 3, 6, 9, 12, 24, and 36 months after implant device switch-on. The mean scores for overall auditory skills were not significantly different between groups A and B at pre-surgery, 1, 12, 24, and 36 months post-surgery, but were significantly different at 3, 6, and 9 months post-surgery. The mean scores for all auditory skills in children with Mondini dysplasia showed significant improvement over time. The mean scores for the three subcategories of auditory skills in children with Mondini dysplasia also showed significant differences at pre-surgery, 1, 3, 6, and 9 months, however, there were no significant differences at 12, 24, and 36 months. Overall, the auditory skills of young children with Mondini dysplasia developed rapidly after cochlear implantation, in a similar manner to that of young children with radiologically normal inner ears. Cochlear implantation is an effective intervention for young children with Mondini dysplasia.

/pdf/the-development-of-auditory-skills-in-young-children-with-ig40g033qd.pdf

Sha Liu

Papers

International outcome inventory for hearing aids (IOI-HA): results from the Chinese version.

Endothelial progenitor cells-derived exosomal microRNA-21-5p alleviates sepsis-induced acute kidney injury by inhibiting RUNX1 expression.

Microwave ablation: an effective treatment for mild-to-moderate secondary hyperparathyroidism in patients undergoing haemodialysis.

Cochlear implants with fine structure processing improve speech and tone perception in Mandarin-speaking adults

The development of auditory skills in young children with Mondini dysplasia after cochlear implantation.