Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. Methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent

Incorporating Over-the-Counter Hearing Aids into Private Audiology Practice

Over-the-counter (OTC) hearing aids are a new category of hearing aid devices designed to help adults with mild to moderate sensorineural hearing loss. This new category of devices was created with the aim to provide increased access to hearing aids for millions of Americans. Their advent has created significant change for hearing healthcare within the United States as they can be purchased without consulting an audiologist or medical professional. As a result of these changes, private audiology practice owners must choose how they will react. Moving forward they must decide if they will integrate OTC hearing aids into their practices, and if they do, how that integration would work for both the practice owner/audiologist and the consumer/patient. The advantages and disadvantages of incorporating OTC hearing aids into private audiology practice are discussed along with three different models of integration that could be utilized. The hybrid model is proposed as the most advantageous option. Little to no scholarly research on OTC hearing aids currently exists. As such, directions for future research and other needs are considered to better understand the impact that these devices will certainly have

A European perspective on auditory processing disorder-current knowledge and future research focus

Current notions of \u201chearing impairment,\u201d as reflected in clinical audiological practice, do not acknowledge the needs of individuals who have normal hearing pure tone sensitivity but who experience auditory processing difficulties in everyday life that are indexed by reduced performance in other more sophisticated audiometric tests such as speech audiometry in noise or complex non-speech sound perception. This disorder, defined as \u201cAuditory Processing Disorder\u201d (APD) or \u201cCentral Auditory Processing Disorder\u201d is classified in the current tenth version of the International Classification of diseases as H93.25 and in the forthcoming beta eleventh version. APDs may have detrimental effects on the affected individual, with low esteem, anxiety, and depression, and symptoms may remain into adulthood. These disorders may interfere with learning per se and with communication, social, emotional, and academic-work aspects of life. The objective of the present paper is to define a baseline European APD consensus formulated by experienced clinicians and researchers in this specific field of human auditory science. A secondary aim is to identify issues that future research needs to address in order to further clarify the nature of APD and thus assist in optimumdiagnosis and evidence-based management. This European consensus presents the main symptoms, conditions, and specific medical history elements that should lead to auditory processing evaluation. Consensus on definition of the disorder, optimum diagnostic pathway, and appropriate management are highlighted alongside a perspective on future research focus

Subtyping somatic tinnitus: a cross-sectional UK cohort study of demographic, clinical and audiological characteristics

Somatic tinnitus is the ability to modulate the psychoacoustic features of tinnitus by somatic manoeuvres. The condition is still not fully understood and further identification of this subtype is essential, particularly for the purpose of establishing protocols for both its diagnosis and treatment. This study aimed to investigate the characteristics of somatic tinnitus within a large UK cohort using a largely unselected sample. We believe this to be relatively unique in comparison to current literature on the topic. This was investigated by using a total of 608 participant assessments from a set of recognised tinnitus and audiology measures. Results from a set of chi-square tests of association found that amongst the individuals with somatic tinnitus, a higher proportion had pulsatile tinnitus (different from heartbeat), were under the age of 40, reported variation in the loudness of their tinnitus and reported temporomandibular joint (TMJ) disorder. The same pattern of results was confirmed using a multivariate analysis of the data based on logistic regression. These findings have strong implications towards the profiling of somatic tinnitus as a distinct subtype of general tinnitus

A Vowel Analysis of the Northwestern University-Children\u27s Perception of Speech Evaluation Tool

In an analysis of the speech perception evaluation tool, the Northwestern University – Children’s Perception of Speech test, the goal was to determine whether the foil words and the target word were phonemically balanced across each page of test Book A, as it corresponds to the target words presented in Test Form 1 and Test Form 2 independently. Based on vowel sounds alone, variation exists in the vowels that appear on a test page on the majority of pages. The corresponding formant frequencies, at all three resonance levels for both the average adult male speaker and the average adult female speaker, revealed that the target word could be easily distinguished from the foil words on the premise of percent differences calculated between the formants of the target vowel and the foil vowels. For the population of children with hearing impairments, especially those with limited or no access to the high frequencies, the NU-CHIPS evaluation tool may not be the best indicator of the child’s speech perception ability due to significant vowel variations

Experienced hearing aid users' perspectives of assessment and communication within audiology: a qualitative study using digital methods.

OBJECTIVE: To explore experienced hearing aid users' perspectives of audiological assessments and the patient-audiologist communication dynamic during clinical interactions. DESIGN: A qualitative study was implemented incorporating both an online focus group and online semi-structured interviews. Sessions were audio-recorded and transcribed verbatim. Iterative-inductive thematic analysis was carried out to identify themes related to assessment and communication within audiology practice. STUDY SAMPLES: Seven experienced hearing aid users took part in an online focus group and 14 participated in online semi-structured interviews (age range: 22 - 86 years; 9 males, 11 females). RESULTS: Themes related to assessment included the unaided and aided testing procedure and relating tests to real world hearing difficulties. Themes related to communication included the importance of deaf aware communication strategies, explanation of test results and patient centred care in audiology. CONCLUSION: To ensure hearing aid services meet the needs of the service users, we should explore user perspectives and proactively adapt service delivery. This approach should be ongoing, in response to advances in hearing aid technology. Within audiology, experienced hearing aid users' value (1) comprehensive, relatable hearing assessment, (2) deaf aware patient-audiologist communication, (3) accessible services and (4) a personalised approach to recommend suitable technology and address patient specific aspects of hearing loss

Factors Related to Self-Identification of Candidacy, Device Selection, and Self-Fitting of Over-The-Counter Hearing Aids

Purpose: The Over-The-Counter (OTC) Hearing Aid Act was introduced in an effort to make hearing aids more accessible and affordable. Implementation of this law will go into effect in 2020. It is assumed that the average consumer will be able to self-navigate an OTC hearing aid fitting. In the OTC hearing aid model consumers are expected to self-diagnose, self-treat, and self manage their hearing loss. The purpose of the present study was to assess how well the average consumer can perform each step in the OTC hearing aid model, and identify factors related to self-identification of candidacy, device selection, and self-fitting of an OTC hearing aid. Method: Participants included 52 adults who were 40 years of age and older, self-reported having trouble hearing and were interested in trying an OTC hearing aid. They had to have owned a smartphone and had no prior hearing aid experience. Data was collected over two tests sessions. During the first session all participants were asked to report their degree of hearing loss, identify if they thought they were at risk for having ear disease, and completed questionnaires related to demographics, health literacy, hearing aid self-efficacy, health locus of control, and technology commitment and usage. Also, participants completed three cognitive tasks and were given a hearing test and administered three cognitive measures: the Reading-SPAN, Digit Symbol Substitution Task, and the Simon task. During the second test session participants were asked to browse three different OTC hearing aids online and select the device they preferred. They were asked to complete a questionnaire regarding potential reasons for why they selected a particular device. The OTC hearing aid they selected was given in its original packaging, and participants were asked to set the device up without any assistance. The Practical Hearing Aid Skills Test- Revised (PHAST-R) along with three questions related to Bluetooth connectivity was used to evaluate the participants’ hearing aid handling skills. Real-ear verification was performed to assess how closely the participant’s settings were to NAL-NL2 prescriptive targets. Last, participants completed the Consumer Ear Disease Risk Assessment (CEDRA) to determine if participants correctly self-identified the risk for ear-disease. Results: Only 38% of participants were able to correctly classify their hearing status in both ears, with pure tone average being a significant predictor of correct hearing status classification. A majority of the participants who misclassified their hearing status had normal hearing, but self-reported they had a hearing loss. Eighty-eight percent of the participants who were identified for being at risk for ear disease misclassified their risk for ear disease. Years of education was inversely related to correctly self-identifying risk for ear disease. Sixty percent of the participants who were flagged by the CEDRA and 30% of normal-hearing participants indicated that they would purchase an OTC hearing aid at the end of the study. Participants’ scores ranged from 45-100% on the PHAST-R and Bluetooth connectivity assessment. The type of the manufacturer’s instructional material was significantly associated with participants’ hearing aid and Bluetooth connectivity skills. For the normal-hearing participants all of the OTC devices attenuated speech, and none of the devices met NAL-NL2 targets in the high frequencies for the hearing-impaired participants. Income status and technology commitment was not predictive of OTC hearing aid device selection and all participants ranked ‘easy to read descriptions’ and ‘website appearance’ as the main factors that influenced their decision to select a device. Conclusions: Most participants were unable to successfully navigate all of the steps in the OTC hearing aid model. Some of the participants who had normal hearing but self-reported a hearing loss and the participants who were at risk for ear disease said they would purchase an OTC hearing aid as a treatment option. Unfortunately, both groups are not the intended user of an OTC hearing aid. Manufacturer instructional material can impact set up and programming of an OTC device. However, users may still run into fitting and programming challenges that will require the assistance of a hearing health care professional

Data-Driven Audiogram Classification for Mobile Audiometry

Recent mobile and automated audiometry technologies have allowed for the democratization of hearing healthcare and enables non-experts to deliver hearing tests. The problem remains that a large number of such users are not trained to interpret audiograms. In this work, we outline the development of a data-driven audiogram classification system designed specifically for the purpose of concisely describing audiograms. More specifically, we present how a training dataset was assembled and the development of the classification system leveraging supervised learning techniques. We show that three practicing audiologists had high intra- and inter-rater agreement over audiogram classification tasks pertaining to audiogram configuration, symmetry and severity. The system proposed here achieves a performance comparable to the state of the art, but is signific