Ewa Raglan

Tinnitus after head injury: evidence from otoacoustic emissions

OBJECTIVE Tinnitus may be caused by a lesion or dysfunction at any level of the auditory system. This study explores cochlear mechanics using otoacoustic emissions in patients with tinnitus after head injury, in whom there seems to be evidence to support dysfunction within the CNS. METHODS The study included 20 patients with tinnitus and other auditory symptoms, such as hyperacusis and difficulty in listening in background noise, after head injury, in the presence of an “intact” auditory periphery (normal or near normal audiometric thresholds). They were compared with 20 normal subjects and 12 subjects with head injury, but without tinnitus, who had similar audiometric thresholds. In all subjects otoacoustic emissions, including transient click-evoked (TEOAEs) and spontaneous otoacoustic emissions (SOAEs), were recorded, and a test of efferent medial olivocochlear suppression, consisting of recording of TEOAEs under contralateral stimulation, was performed. RESULTS A significantly higher prevalence of SOAEs (100%), higher TEOAE response amplitudes, and reduced medial olivocochlear suppression in patients with tinnitus in comparison with subjects without tinnitus have been found. CONCLUSION These findings have been interpreted to be an extracochlear phenomenon, in which the reduction in central efferent suppression of cochlear mechanics, leading to an increase in cochlear amplifier gain, was subsequent to head injury. Auditory symptoms in these patients seemed to constitute the “disinhibition syndrome”.

Homozygosity for piebaldism with a proven KIT mutation resulting in depigmentation of the skin and hair, deafness, developmental delay and autism spectrum disorder.

North West Thames Regional Genetics Service (Kennedy Galton Centre), Northwick Park Hospital, NWLH NHS Trust, Harrow, Departments of Paediatrics, Community Paediatrics, Hillingdon Hospital, Uxbridge, Middlesex, Departments of Ophthalmology, Audiological Medicine, Great Ormond Street Hospital, London, UK and Molecular Genetics Laboratory, University of Munich, Munich, Germany Correspondence to Angela F. Brady, FRCP, PhD, North West Thames Regional Genetics Service (Kennedy Galton Centre), Northwick Park Hospital, NWLH NHS Trust, Harrow HA1 3UJ, UK Tel: + 44 20 8869 2795; fax: + 44 20 8869 3106; e-mail: a.brady@imperial.ac.uk

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. Methods 169 healthy (female n = 89; recruited from mainstream schools, London, UK) and 114 children with a primary migraine, concussion, or vestibular disorder diagnosis (female n = 62), aged 6–17 years, were included. Children with primary migraine were recruited from mainstream schools while children with concussion or vestibular disorder were recruited from tertiary balance centers in London, UK, and Pittsburgh, PA, USA. Children completed the PVID, which assesses the frequency of dizziness and unsteadiness experienced in specific environmental situations, and Strength and Difficulties Questionnaire (SDQ), a brief behavioral screening instrument. Results The PVID showed high internal consistency (11 items; α = 0.90). A significant between-group difference was noted with higher (i.e., worse) PVID scores for patients vs. healthy participants (U = 2,436.5, z = −10.719, p < 0.001); a significant difference was noted between individual patient groups [χ2(2) = 11.014, p = 0.004] but post hoc analysis showed no significant pairwise comparisons. The optimal cut-off score for discriminating between individuals with and without abnormal ViD levels was 0.45 out of 3 (sensitivity 83%, specificity 75%). Self-rated emotional (U = 2,730.0, z = −6.169) and hyperactivity (U = 3,445.0, z = −4.506) SDQ subscale as well as informant (U = 188.5, z = −3.916) and self-rated (U = 3,178.5, z = −5.083) total scores were significantly worse for patients compared to healthy participants (p < 0.001). Conclusion ViD is common in children with a primary concussion, migraine, or vestibular diagnosis. The PVID is a valid measure for identifying the presence of ViD in children and should be used to identify and quantify these symptoms, which require specific management incorporating exposure to optokinetic stimuli.

Vestibular function in children with auditory neuropathy spectrum disorder.

OBJECTIVE Children with auditory neuropathy spectrum disorder (ANSD) account for about 10% of paediatric patients referred for cochlear implantation. Vestibulopathy may be associated with ANSD, and may have implications when formulating management plans in this patient group. We wanted to determine the incidence and predictive factors for vestibulopathy in this patient group to guide vestibular testing in this patient population, and give insight to the aetiology of ANSD. METHODS We reviewed the outcomes of vestibular function testing in a cohort of paediatric patients with ANSD. RESULTS Probable or definite vestibulopathy was seen in 42% of patients who were tested. Vestibulopathy was associated with medical co-morbidities, but was not associated with imaging findings. CONCLUSIONS Vestibulopathy is relatively prevalent in this patient group, and should be considered when planning the investigation and management of children with ANSD.

Vestibular dysfunction in Apert Syndrome

Abstract Objectives: Apert syndrome affects the craniofacial structures resulting in craniosynostosis, craniofacial anomalies and syndactyly. Although many characteristic features are recognized, vestibular dysfunction is a little known feature that can often be present. This can cause a delay in the attainment of gross motor milestones and impair motor function. We aimed to examine this cohort to review the incidence of vestibular hypofunction and to assess the effect on motor function. Study design: We performed a retrospective analysis of 55 Apert syndrome patients who had undergone audiological review at Great Ormond Street Hospital for Children. Results: 50.9% of patients experienced balance difficulties; 18.1% of patients (all of whom had delayed motor milestones) underwent clinical examination and vestibular assessment. Results showed only 20% of patients had bilateral vestibular function present; 30% of patients had unilateral and 50% bilateral vestibular hypofunction. Two patients who initially presented with imbalance and vestibular hypofunction experienced significant improvement in symptoms on re-examination following vestibular physiotherapy. Conclusion: Vestibular dysfunction is a feature of Apert syndrome. Although the imbalance may be attributable to mechanical problems arising from syndactyly, it is important to recognize that a vestibular component can coexist. This is not well recognized, poorly understood and scarcely treated. We aim to increase awareness of vestibular dysfunction in Apert syndrome and advocate a high index of suspicion, particularly in the presence of motor milestone delay. Early detection, thorough vestibular assessment and prompt initiation of vestibular physiotherapy can aid vestibular compensation, rehabilitating patients and maximizing their functional attainment.

Effect of peer review on accuracy of reported auditory brainstem response thresholds in newborn hearing screening programme referrals

Abstract Objective: The aim of this study was to establish the impact of peer review on estimated ABR thresholds. Study design: The reported ABR thresholds of two groups of 38 babies tested over a period of six months when a system of formal peer review was in place, and another period of six months when it was not in place, were retrospectively analysed by expert clinicians. Results: The modal differences between experts and tester estimated threshold with and without peer review were 5dB (-10 to +20) and 0dB (-10 to +35), respectively. Wilcoxons signed-rank test for paired samples revealed a small but significant difference in estimated thresholds between experts and tester irrespective of whether tester was subjected to peer review on the day of the test or not. Conclusion: Peer review provides opportunities for peer support and continuing professional development. A system of formal peer review is strongly advocated.

Is gross motor delay secondary to bilateral vestibular hypofunction in Jervell and Lange-Nielsen syndrome?

Abstract Jervell and Lange-Nielsen syndrome is a rare autosomal recessive condition characterized by bilateral profound sensorineural hearing loss, and prolonged QT interval that may result in ventricular tachycardia leading to syncope, cardiac arrest or sudden death. Treatment of the arrhythmias is often life-saving as this condition has a high mortality if untreated. We describe 3 cases of Jervell and Lange-Nielsen syndrome, who presented with profound sensorineural hearing loss and delayed gross motor milestones and have evidence of bilateral vestibular hypofunction on vestibular testing. We suggest that vestibular hypofunction is associated with this syndrome and evaluation of vestibular function may increase the diagnostic yield in the asymptomatic stage of this rare, but severe form of long QT syndrome. Clinicians should consider Jervell Lange-Nielsen in the differential diagnosis of ‘non-syndromic’ profound sensorineural hearing loss presenting with vestibular failure, along with Usher syndrome type 1, and morphological malformations of the vestibular apparatus. However, more studies are needed to confirm these findings.

Setting up a paediatric vestibular testing laboratory

This paper describes the vision of networked balance testing centres as set out in the Balance Good Practice Guide published in 2009. It provides examples of room design and enabling works. A suggestion for hardware and software specifications for a supra-specialist balance centre is provided. How to design, evaluate and commission such a laboratory will be covered, including brief descriptions of the qualitative and quantitative balance tests used in assessing vestibular function in children, maintenance and training issues. The appendix provides guidance on making a business case, applying for capital bids, option appraisal, writing equipment specifications, bid evaluation, and procurement.

An audio-vestibular study of 128 children presenting to a specialized paediatric audio-vestibular clinic: Should every child with hearing impairment have vestibular function assessed?

This is a study of the vestibular function of 128 children with a varying degree/type and cause of hearing impairment presenting to a specialized paediatric vestibular service. It shows that there are some syndromic and non-syndromic conditions that may be associated with vestibular dysfunction. Due to the relatively small cohort of children tested with a lesser degree of hearing impairment, the study is unable to confirm the proportional dependence of the presence of vestibular dysfunction with the degree of hearing impairment but it confirms such a trend. However, it supports earlier findings that profound hearing loss due to autosomal recessive inheritance associated with mutation in the Connexin 26 gene is not associated with vestibular dysfunction of the lateral semicircular canal. It further shows that children with profound hearing loss and lack of vestibular function present with a history of gross motor developmental delay. It argues that the referral of a child for vestibular assessment should be based on the knowledge of syndromic/non-syndromic conditions associated frequently with inner ear anomalies as well as the knowledge of the noxious factors that may lead to vestibular dysfunction and also hearing impairment such as meningitis, rubella, birth risk factors and ototoxicity.

The effect of language on maternal knowledge of the child's hearing status.

The aim of this study was to assess whether mothers whose first language is not English are as accurate as English speakers in reporting on their childs hearing. Some Health Trusts are using parental report following an educative process as part of their screening programme for hearing impairment. At present, in Wandsworth, London, UK, where there is a relatively high ethnic minority population, many of whom speak little or no English, the parental educative programme is carried out in English. There are many studies supporting the translation of the educative programme into appropriate minority languages but the authors wanted to assess whether doing so would actually improve the hearing impaired identification rate by the raised awareness programme. The presence or absence of concern in the two groups of mothers, one English speaking and the other non-English speaking, was elicited by direct questioning and its accuracy checked by formal hearing assessment. The results indicate that there is no significant difference in the reliability of maternal observation between the two groups in normal hearing and hearing impaired children. However, two-thirds of non-English speaking mothers of hearing impaired children were not concerned about their childs hearing status, compared to one-fifth of the English speaking mothers. Although the numbers of hearing impaired children are small, and the reason for the high maternal inaccuracy in non-English speakers in this group has not yet been ascertained, it seems reasonable to recommend translation of the parental educative leaflets in Wandsworth into minority languages. This recommendation is supported by other studies.