Objective To investigate a new wide bone-anchored hearing implant considering initial stability, stability over time, implant loss, and skin reaction. Study Design Consecutive, prospective case series. Setting Tertiary referral center. Patients Twenty adult patients were enrolled. All operations were 1-stage, single-incision technique with subcutaneous reduction. Intervention(s) Measurement of implant stability. Main Outcome Measure(s) Implant stability quotient (ISQ) values were recorded using resonance frequency analysis at the time of implantation and at 10 days, 6 weeks, 6 months, and 1 year after surgery. Skin and soft tissue reactions according to Holgers grading system. Results Implant stability quotient measurements revealed a significant increase in ISQ during the first 10 days after operation, and the ISQ values continued to rise throughout the 1-year observation period. No implants were lost. Skin and soft tissue reactions were rare and minor, as no reaction was seen in 93% of the follow-up examinations and no grade 4 reactions occurred. Conclusion The new wide implant showed good stability at surgery. Osseointegration was fast, and implant stability increased throughout the 1-year observation period. No implants were lost. Skin and soft tissue reactions were rare and minor.

Cochlear implantation is considered the best treatment option for patients with severe-to-profound sensorineural hearing loss for whom conventional hearing aids are insufficient. We used a repeated measures longitudinal approach to evaluate speech recognition and patient-reported outcomes after cochlear implantation in an unbiased cohort of Danish adult patients in a prospective cohort study. We assessed 39 recipients before and two times after implantation using a battery of tests that included Dantale I, the Danish Hearing in Noise Test, the Nijmegen Cochlear Implant Questionnaire, and the Speech, Spatial, and Qualities of Hearing Scale. The study group improved significantly on all outcome measures following implantation. On average, Dantale I scores improved by 29 percentage points and Hearing in Noise Test scores improved by 22 percentage points. Most notably, the average Dantale score improved from 26 to 70% in the CI in quiet condition and from 12 to 42% in the cochlear implantation in noise condition when tested monaurally. Dantale demonstrated a significant positive correlation with Nijmegen Cochlear Implant Questionnaire and Speech, Spatial, and Qualities of Hearing Scale scores, while Hearing in Noise Test had no significant correlation with the patient-reported outcome measures. Patients improved significantly at 4 months and marginally improved further at 14 months, indicating that they were approaching a plateau. Our study’s use of audiometric and patient-reported outcome measures provides evidence of the treatment benefits of cochlear implantation in adults, which may help physicians advise patients on treatment decisions and align treatment benefit expectations, as well as serve as a foundation for the development of new cochlear implantation selection criteria.

The purpose of the present study is to explore, demonstrate, and describe the expression of genes related to the solute carrier (SLC) molecules of ion transporters in the human endolymphatic sac. cDNA microarrays and immunohistochemistry were used for analyses of fresh human endolymphatic sac tissue samples. Twelve tissue samples of the human endolymphatic sac were obtained during translabyrinthine surgery for vestibular schwannoma. Microarray technology was used to investigate tissue sample expression of solute carrier family genes, using adjacent dura mater as control. Immunohistochemistry was used for verification of translation of selected genes, as well as localization of the specific protein within the sac. An extensive representation of the SLC family genes were upregulated in the human endolymphatic sac, including SLC26a4 Pendrin, SLC4a1 sodium-bicarbonate transporter, SLC9a2 sodium-hydrogen transporter, SLC12a3 thiazide-sensitive Na-Cl transporter, and SLC34a2 sodium-phosphate transporter. Several important ion transporters of the SLC family are expressed in the human endolymphatic sac, including Pendrin, the thiazide-sensitive Na-Cl transporter, and the Na-phosphate transporter SLC34a2. The data provide a new knowledge base considering the ion-dependent metabolic mechanisms maintaining inner ear homeostasis. More specifically, the results indicate a strong similarity with the ion transportation occurring in the kidney collecting ducts. In addition, the findings prompt a revision of the theories behind contemporary pharmacological treatment of Ménière's disease and may broaden the understanding of the pathogenesis of BPPV.

Objective This is a systematic review of the literature on the spontaneous course of hearing in patients observed with a vestibular schwannoma. Included studies are appraised using the Grading of Recommendations Assessment, Development and Evaluation system. Design PubMed, Embase, Medline, Cochrane library, and CINAHL (Cumulative Index to Nursing and Allied Health Literature) were searched for literature on hearing in patients observed with a vestibular schwannoma. Participants Of 217 evaluated papers, 15 were included, representing a total of 2,142 patients. Main Outcome Measures Hearing according to the AAO-HNS (American Academy of Otorhinolaryngology- Head and Neck Surgery) classification system. Weighted average of the proportion of patients preserving good hearing (>70% discrimination score and pure tone audiometry [PTA] < 30dB) and serviceable hearing (>50% discrimination score and PTA < 50dB) was determined. Results Fifty percent of patients presenting with good hearing at diagnosis had preserved this after a mean of 5 years of observation, whereas serviceable hearing was preserved in 54%. Patients with normal discrimination at diagnosis preserve their hearing very well. Very few studies exist on long-term hearing preservation. Conclusions After 5 years of observation, around half of patients will have preserved good or serviceable hearing. Patients with normal discrimination at diagnosis are more likely to preserve good hearing.

In light of missing systematic reviews in the literature, the objective of this paper is to present the contemporary knowledge on the molecular biology of vestibular schwannomas (VS), based on a systematic literature search. In addition, current and prospected medical therapy based on molecular biology is addressed. A systematic literature search was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The systematic search was performed in the Pubmed and Embase databases. The following were the words searched: acoustic neuroma/vestibular schwannoma, molecular biology, gene, and microRNA. Specific inclusion and exclusion criteria were determined prior to search. The systematic search rendered 486 articles, ultimately yielding 69 included articles, whereas 35 were from relevant references. The occurrence of at least one mutation in the merlin gene was reported to range between 54% and 76%, whereas the loss of heterozygosity (LOH) corresponding to chromosome 22 occurs in 25% to 83% of sporadic VS. Global gene expression studies indicate that a number of genes other than merlin are at play. No high-level methylation of the merlin gene has been found. Several miRNAs are deregulated in tumor tissue, among others let-7d, miR-221, and miR-21. The acquired knowledge on molecular biology has led to several clinical implementations. Lack of the tumor suppressor merlin plays a principal role in the development of VS. Existing knowledge on the molecular biology has led to the first attempts of targeted medical treatment to prevent tumor growth. Future research is likely to introduce potential imaging markers with prognostic value and new targets for medical therapy. Cite this article as: Sass H, Cayé-Thomasen P. Contemporary Molecular Biology of Sporadic Vestibular Schwannomas: A Systematic Review and Clinical Implications. J Int Adv Otol 2018; 14(2): 322-9.