Diagnostic accuracy and usability of the EMBalance decision support system for vestibular disorders in primary care: proof of concept randomised controlled study results.

Background: Dizziness and imbalance are common symptoms that are often inadequately diagnosed or managed, due to a lack of dedicated specialists. Decision Support Systems (DSS) may support first-line physicians to diagnose and manage these patients based on personalised data.

Aim: To examine the diagnostic accuracy and application of the EMBalance DSS for diagnosis and management of common vestibular disorders in primary care.

Central mimics of benign paroxysmal positional vertigo: an illustrative case series.

Benign paroxysmal positional vertigo (BPPV) is the most common peripheral vestibular disorder that is diagnosed based solely on clinical findings. Rarely, central lesions can present with positional vertigo and nystagmus, mimicking BPPV. Recognised red flags that may help distinguish central mimics from BPPV include the presence of additional neurological symptoms and signs, atypical nystagmus patterns, and the absence of a sustained response to repositioning manoeuvres.

Results of a multicentre randomised controlled trial of cochlear-sparing intensity-modulated radiotherapy versus conventional radiotherapy in patients with parotid cancer (COSTAR; CRUK/08/004).

Purpose: About 40-60% of patients treated with post-operative radiotherapy for parotid cancer experience ipsilateral sensorineural hearing loss. Intensity-modulated radiotherapy (IMRT) can reduce radiation dose to the cochlea. COSTAR, a phase III trial, investigated the role of cochlear-sparing IMRT (CS-IMRT) in reducing hearing loss.

Correction to: The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management.

The following information was inadvertently omitted in the original publication.

The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management.

Cerebellar ataxia, areflexia, pes cavus, optic atrophy and sensorineural hearing impairment (CAPOS) is a rare clinically distinct syndrome caused by a single dominant missense mutation, c.2452G>A, p.Glu818Lys, in ATP1A3, encoding the neuron-specific alpha subunit of the Na+/K+-ATPase α3.

Visually Induced Dizziness in Children and Validation of the Pediatric Visually Induced Dizziness Questionnaire.

Aims: To develop and validate the Pediatric Visually Induced Dizziness Questionnaire (PVID) and quantify the presence and severity of visually induced dizziness (ViD), i.e., symptoms induced by visual motion stimuli including crowds and scrolling computer screens in children.

EMBalance - validation of a decision support system in the early diagnostic evaluation and management plan formulation of balance disorders in primary care: study protocol of a feasibility randomised controlled trial.

Background: Balance problems are caused by multiple factors and often lead to falls and related fractures, bringing large socio-economic costs. The complexity of balance control mechanisms, the lack of medical expertise, and the absence of specialised equipment contribute to the delayed or incorrect diagnosis and management ofthese patients. Advances in computer science have allowed the development of computer systems that support clinical diagnosis and treatment decisions based on individualised patient data.

The Pediatric Vestibular Symptom Questionnaire: A Validation Study.

Objective: To develop and validate the Pediatric Vestibular Symptom Questionnaire (PVSQ) and quantify subjective vestibular symptom (ie, dizziness, unsteadiness) severity in children.

Study Design: One hundred sixty-eight healthy children (female, n = 91) and 56 children with postconcussion dizziness or a vestibular disorder (female, n = 32), between ages 6 and 17 years, were included. The PVSQ contains questions regarding vestibular symptom frequency during the previous month.

A detailed clinical and molecular survey of subjects with nonsyndromic USH2A retinopathy reveals an allelic hierarchy of disease-causing variants.

Defects in USH2A cause both isolated retinal disease and Usher syndrome (ie, retinal disease and deafness). To gain insights into isolated/nonsyndromic USH2A retinopathy, we screened USH2A in 186 probands with recessive retinal disease and no hearing complaint in childhood (discovery cohort) and in 84 probands with recessive retinal disease (replication cohort). Detailed phenotyping, including retinal imaging and audiological assessment, was performed in individuals with two likely disease-causing USH2A variants.

Mobile telephone use effects on perception of verticality.

Low-level radiofrequency (RF) signals may produce disorientation and nausea. In experiment I, we assessed mobile phone effects on graviception in nine symptomatic subjects after mobile telephone use and 21 controls. The mobile handset was strapped to each ear for 30 min in pulsed emission, continuous RF emission, or no emission test mode, respectively.

Vestibular involvement in peripheral neuropathy: a review.

Objective: This paper reviews the current literature on involvement of the vestibular division of the eighth cranial nerve in peripheral neuropathies. The literature abounds with references to auditory neuropathy, which is frequently related to more generalized neuropathies, but there is a marked paucity of work regarding vestibular neuropathy. A brief overview of neuropathies and the anatomy of the vestibulocochlear nerve provide the background for a review of the literature of vestibular nerve involvement in a range of neuropathies.

Natural history and retinal structure in patients with Usher syndrome type 1 owing to MYO7A mutation.

Purpose: To evaluate the phenotypic variability and natural history of ocular disease in a cohort of 28 individuals with MYO7A-related disease. Mutations in the MYO7A gene are the most common cause of Usher syndrome type 1, characterized by profound congenital deafness, vestibular arreflexia, and progressive retinal degeneration.

Design: Retrospective case series.

Peripheral vestibular dysfunction is prevalent in older adults experiencing multiple non-syncopal falls versus age-matched non-fallers: a pilot study.

Background: vestibular disorders are common in the general population, increasing with age. However, it is unknown whether older adults who fall have a higher proportion of vestibular impairment compared with age-matched older adult non-fallers.

Objective: to identify whether a greater proportion of older adult fallers have a peripheral vestibular impairment compared with age-matched healthy controls.

Screening for duplications, deletions and a common intronic mutation detects 35% of second mutations in patients with USH2A monoallelic mutations on Sanger sequencing.

Background: Usher Syndrome is the leading cause of inherited deaf-blindness. It is divided into three subtypes, of which the most common is Usher type 2, and the USH2A gene accounts for 75-80% of cases. Despite recent sequencing strategies, in our cohort a significant proportion of individuals with Usher type 2 have just one heterozygous disease-causing mutation in USH2A, or no convincing disease-causing mutations across nine Usher genes.

Patient-reported speech in noise difficulties and hyperacusis symptoms and correlation with test results.

Objectives/hypothesis: To compare self-reported symptoms of difficulty hearing speech in noise and hyperacusis in adults with auditory processing disorders (APDs) and normal controls; and to compare self-reported symptoms to objective test results (speech in babble test, transient evoked otoacoustic emission [TEOAE] suppression test using contralateral noise).

Study Design: A prospective case-control pilot study.

Methods: Twenty-two participants were recruited in the study: 10 patients with reported hearing difficulty, normal audiometry, and a clinical diagnosis of APD; and 12 normal age-matched controls with no reported hearing difficulty.

Patient-reported auditory functions after stroke of the central auditory pathway.

Background And Purpose: Auditory functional limitations experienced by patients after stroke of the central auditory pathways remain underinvestigated. Purpose- To measure patient-reported hearing difficulties in everyday life in nonaphasic patients with stroke of the auditory brain versus normal control subjects. To examine how hearing difficulties correlate with auditory tests and site of lesion in individual cases.

Comprehensive sequence analysis of nine Usher syndrome genes in the UK National Collaborative Usher Study.

Background: Usher syndrome (USH) is an autosomal recessive disorder comprising retinitis pigmentosa, hearing loss and, in some cases, vestibular dysfunction. It is clinically and genetically heterogeneous with three distinctive clinical types (I-III) and nine Usher genes identified. This study is a comprehensive clinical and genetic analysis of 172 Usher patients and evaluates the contribution of digenic inheritance.

Mutations in the USH1C gene associated with sector retinitis pigmentosa and hearing loss.

Purpose: To determine the molecular cause of sector retinitis pigmentosa and hearing loss in two affected siblings.

Methods: Direct DNA sequencing of the USH1C gene was performed in two affected siblings. Putative pathogenic sequence changes were assayed in their parent's chromosomes and in control chromosomes.

Alteration in auditory function during the ovarian cycle.

This study investigates whether physiological variations in ovarian hormones during the ovarian cycle (OC) are associated with changes in auditory function. Sixteen women with normal hearing underwent auditory tests and simultaneous measurements of the hormone levels four times during OC. The auditory tests included recording of otoacoustic emissions (OAEs), the medial olivocochlear (MOC) suppression and auditory brainstem responses (ABRs).

Nonsense mutation in TMEM126A causing autosomal recessive optic atrophy and auditory neuropathy.

Purpose: To define the phenotype and elucidate the molecular basis for an autosomal recessively inherited optic atrophy and auditory neuropathy in a consanguineous family with two affected children.

Methods: Family members underwent detailed ophthalmologic, electrophysiological, and audiological assessments. An autozygosity mapping strategy using high-density single nucleotide polymorphism microarrays and microsatellite markers was used to detect regions of genome homozygosity that might contain the disease gene.

Computer-based auditory training (CBAT): benefits for children with language- and reading-related learning difficulties.

This article reviews the evidence for computer-based auditory training (CBAT) in children with language, reading, and related learning difficulties, and evaluates the extent it can benefit children with auditory processing disorder (APD). Searches were confined to studies published between 2000 and 2008, and they are rated according to the level of evidence hierarchy proposed by the American Speech-Language Hearing Association (ASHA) in 2004. We identified 16 studies of two commercially available CBAT programs (13 studies of Fast ForWord (FFW) and three studies of Earobics) and five further outcome studies of other non-speech and simple speech sounds training, available for children with language, learning, and reading difficulties.