A Case of Intralabyrinthine Schwannoma Removal and Sequential BonebridgeTM Implantation

Intralabyrinthine schwannoma (ILS) is a rare benign tumor that arises within the membranous labyrinthine. Since hearing loss is inevitable after tumor removal in most cases, an appropriate rehabilitation method should be considered. A 41-year-old male, who was diagnosed with ILS underwent tumor removal via translabyrinthine approach, has subsequently experienced right-side deafness as a result. Seventeen months after the tumor removal, a surgery for hearing rehabilitation was performed. Since cochlear implantation was not doable due to cochlear ossification, an active transcutaneous bone conduction implant (BonebridgeTM , MEDEL) was placed at the sinodural angle. To the best of our knowledge, this is the first report describing hearing rehabilitation with BonebridgeTM implantation after ILS removal.

Round Window Stimulation of the Cochlea

Mixed hearing loss associated with a sensorineural component and an impaired conductive mechanism for sound from the external ear canal to the cochlea represents a challenge for rehabilitation using either surgery or traditional hearing amplification. Direct stimulations of the ossicular chain and the round window (RW) membrane have allowed an improved hearing in this population. The authors review the developments in basic and clinical research that have allowed the exploration of new routes for inner ear stimulation. Similar changes occur in the electrophysiological measures in response to auditory stimulation through the traditional route and direct mechanical stimulation of the RW. The latter has proven to be very effective as a means of hearing rehabilitation in a group of patients with significant difficulties with hearing and communication.

Hearing Rehabilitation with Cochlear Implants after CyberKnife Radiosurgery of Vestibular Schwannoma: A Report Based on Four Clinical Cases

Severe sensorineural hearing loss can be a symptom of the benign tumor vestibular schwannoma (VS). The treatment of VS with non-invasive stereotactic radiosurgery (SRS) offers a high local tumor control rate and an innovative possibility of sequential hearing rehabilitation with cochlear implantation. This study evaluated the feasibility, complications, and auditory outcomes of such a therapeutic approach. Three males and one female (mean age 65.3 ± 9.4 years) scheduled for cochlear implantation and diagnosed with sporadic VS classified as T1 or T2 (according to Samii) were enrolled in this study. All patients had progressive hearing loss qualifying them for cochlear implantation. First, the tumor was treated using CyberKnife SRS. Next, sequential auditory rehabilitation with a cochlear implant (CI) was performed. Clinical outcomes and surgical feasibility were analyzed, and audiological results were evaluated using pure tone audiometry and speech recognition tests. All patients exhibited open-set speech understanding. The mean word recognition score (at 65 dB SPL, Freiburg Monosyllabic Test, FMT) improved after cochlear implantation in all four patients from 5.0 ± 10% (with hearing aid) preoperatively to 60.0 ± 22.7% six months postoperatively. Our results suggest that in patients with profound hearing loss caused by sporadic vestibular schwannoma, the tumor removal with SRS followed by cochlear implantation is an effective method of auditory rehabilitation.

Caring for Persons With Dementia in Audiology

While hearing loss is highly prevalent among patients with dementia, it frequently goes unidentified and unmanaged. It has been a commonly-held belief that older adults with dementia are unable to benefit from hearing rehabilitation, but recent evidence shows that many individuals with dementia can successfully use amplification, helping to improve communication, social interaction and quality of life for these individuals and their caregivers. This presentation will describe how modifications to practice led to successful outcomes for the majority of patients of a geriatric audiology clinic with co-morbid hearing loss and cognitive impairment. In a study of hearing aid use in 67 patients with these comorbidities, over 90% used hearing aids successfully with measurable benefit for both patients and caregivers. Furthermore, we will discuss approaches to improving communication for LTC residents with dementia and hearing loss through the support of audiologists during remote visits with physicians and families during the pandemic.

TMPRSS3 Gene Variants With Implications for Auditory Treatment and Counseling

Objective: To identify and report novel variants in the TMPRSS3 gene and their clinical manifestations related to hearing loss as well as intervention outcomes. This information will be helpful for genetic counseling and treatment planning for these patients.Methods: Literature review of previously reported TMPRSS3 variants was conducted. Reported variants and associated clinical information was compiled. Additionally, cohort data from 18 patients, and their families, with a positive result for TMPRSS3-associated hearing loss were analyzed. Genetic testing included sequencing and copy number variation (CNV) analysis of TMPRSS3 and the Laboratory for Molecular Medicine’s OtoGenome-v1, -v2, or -v3 panels. Clinical data regarding patient hearing rehabilitation was interpreted along with their genetic testing results and in the context of previously reported cochlear implant outcomes in individuals with TMPRSS3 variants.Results: There have been 87 previously reported TMPRSS3 variants associated with non-syndromic hearing loss in more than 20 ancestral groups worldwide. Here we report occurrences of known variants as well as one novel variant: deletion of Exons 1–5 and 13 identified from our cohort of 18 patients. The hearing impairment in many of these families was consistent with that of previously reported patients with TMPRSS3 variants (i.e., typical down-sloping audiogram). Four patients from our cohort underwent cochlear implantation.Conclusion: Bi-allelic variants of TMPRSS3 are associated with down-sloping hearing loss regardless of ancestry. The outcome following cochlear implantation in patients with variants of TMPRSS3 is excellent. Therefore, cochlear implantation is strongly recommended for hearing rehabilitation in these patients.

Mythbusters! The Truth about Common Misconceptions in Cochlear Implantation

Cochlear implantation (CI) is the preferred method of hearing rehabilitation when patients cannot perform well with traditional amplification. Unfortunately, there are still significant misconceptions around this life-changing intervention. The goal of this article is to address some of the most common myths around CI surgery. After reading this article, the learner will be able to explain the utility of CI in patients with residual hearing and recognize that insurance coverage is widespread. The reader will be able to list common risks associated with this well-tolerated procedure including anesthetic risk and the risk of vestibular dysfunction. Additionally, the reader will be able to identify the significant positive impact of CI on patients' quality of life. Finally, the reader will identify that many patients can safely have an MRI scan after implantation, including nearly all contemporary recipients.

The Noise Level Measurements of Children’s Sound Books

Purpose: It was intended to measure the sound intensity of children's sound books and to compare them with the standards of Korea and the International Standards Organization (ISO).Methods: The loudness of 15 children’s sound books was measured at a distance of 25 cm (child’s arm length), and 2.5 cm length of external auditory canal. Measurements taken three times with each book were performed, and the overall sound intensity of the sound books and the sound intensity of each button were measured and compared.Results: Compared with the Korean standard, all the buttons of all books exceeded the standard for the sound volume of the children’s book measured at a distance of 2.5 cm. When comparing the maximum sound intensity measured at a distance of 25 cm with 85 LAmax, a total of 168 buttons of these, 25 (14.88%) were recorded as exceeding the maximum loudness. According to the standards of the ISO, all buttons in all books were below the standard 85 LAeq.Conclusion: Several children’s books are loud enough to cause noise-induced hearing loss, especially when they are placed close to the ear. Strict standards for sound children’s books are required, and it is important to put a warning on the cover of the book. It is expected that the sound intensity of the children’s sound book presented in this study can be referenced when referring to the loudness during hearing rehabilitation in children.

Hearing Aid Selection: Closed- versus Open-canal Fitting Hearing Aids

To select hearing aid is an essential process for successful hearing rehabilitation. The purpose of this study is to review hearing aid selection considerations between receiver in-the-canal (RIC) and custom hearing aid (CHA) in order to guide appropriate selection of the hearing aid. This study discussed three key factors in the hearing aid selection including physical, acoustic and electroacoustic characteristics and other aspects. Advantages of RIC types are comfort to wear, reduction of the occlusion effect, presence of directional microphones, on-site fit, easy connectivity with other devices, and use of rechargeable batteries. On the other hand, the CHA types have their advantage in terms of being comfort to wear with masks, proper insertion and placement, reduction of the acoustic feedback, good approximation of frequency response curve, improvement of speech in noise perception, expanded hearing aid candidacy with varying hearing thresholds, and easy telephone use. We concluded that appropriate selection of the hearing aid would contribute to successful hearing rehabilitation, if considering physical, psycho-social, and acoustical characteristics.