Multidisciplinary Care for Moebius Syndrome and Related Disorders: Building a Management Protocol.

Moebius syndrome is a collection of orofacial anomalies with highly variable features affecting many different systems but characterised by bilateral facial palsy and absent eye abduction. We largely regard Moebius syndrome as a diagnosis of exclusion. Lack of awareness and knowledge means that children often fall between services, leading to treatment delays and difficulty interfacing with social care and schools, with long-term impact on physical health and psychosocial development.

Experience from the First Paediatric Vestibular and Balance Clinic in a Multiracial Asian Setting.

Objective: Paediatric vestibular and balance services have recently gained attention across the globe. We present our one-year experience exploring the aetiology of paediatric vestibular disorders in a recently established Paediatric vestibular and balance clinic.

Methods: Children and adolescents under 18 referred to the Paediatric Vestibular and Balance Clinic for evaluation were included.

The pioneers of vestibular physiology in the 19th century.

It was known from ancient times that vertigo was a malady and that the inner ears of animals contained an intricate network of structures named the labyrinth, whose function was unknown. The flourishing of human vestibular anatomy in the Renaissance period still adhered to age-old notions of traditional spiritual philosophy. In the post-Renaissance period, when science was being redefined and challenging these traditional thoughts, vestibular physiology was born.

Suppression head impulse test in children-experiences in a tertiary paediatric vestibular centre.

The suppression head impulse paradigm (SHIMP) involves suppression of the vestibulo-ocular reflex (VOR) and anticompensatory saccades generated thereof. SHIMP is gaining importance to understand vestibular compensation with its different parameters (VOR gain/peak saccadic velocity PSV/latency of saccades). SHIMP studies are emerging in adults, but pediatric studies have hardly been performed.

Cochleovestibular Phenotype in a Rare Genetic MED13L Mutation.

The gene MED13 participates in transcription. The MED13L gene is a paralog of MED13 that is involved in developmental gene expression. Mutations in the gene have been shown to result in a heterogenous phenotype affecting several physiological systems.

Modernising vestibular assessment.

Background: There is a high prevalence of dizziness, vertigo and balance symptoms in the general population. Symptoms can be generated by many inner-ear vestibular disorders and there are several diagnostic tests available that can help identify the site of the vestibular lesion. There is little consensus on what diagnostic tests are appropriate, with diagnostics either not completed or minimally performed, leading to missed diagnosis, unsatisfactory results for patients and costs to healthcare systems.

X-Linked Gusher Disease DFNX2 in Children, a Rare Inner Ear Dysplasia with Mixed Hearing and Vestibular Loss.

Conductive hearing losses are typically present in disorders of the external/middle ear. However, there is a rare group of inner ear conditions called third windows that can also generate a conductive hearing loss. This is due to an abnormal connection between the middle and the inner ear or between the inner ear and the cranial cavity.

Fifteen-minute consultation: The dizzy child.

Vestibular disorders are often overlooked in children and may cause significant morbidity. About a third of children presenting with problems in balance show a vestibular pathology and the overall prevalence of paediatric vertigo is about 5%. Appropriate diagnosis and holistic management can have a significantly positive impact on a child's quality of life and can be very rewarding.

Vestibular Function in Children With Von Hippel-Lindau Disease.

Von Hippel-Lindau disease (VHL) is a rare autosomal dominant disorder. It is caused by a mutation in the tumor suppressor gene localized at 3p25-26. Endolymphatic sac tumors (ELSTs) are rare low-grade adenocarcinomas which can occur sporadically but are more commonly found in association with VHL disease.

Audiovestibular Quantification in Rare Third Window Disorders in Children.

Third window disorders are structural abnormalities in the bony otic capsule that establish a connection between the middle/inner ear or the inner ear/cranial cavity. Investigated extensively in adults, they have hardly been studied in children. This study is a retrospective study of children (aged 5-17 years) diagnosed with rare third window disorders (third window disorders reported rarely or not reported in children) in a tertiary pediatric vestibular unit in the United Kingdom.

Double-Blind Randomized Trial on the Efficacy of the Forced Prolonged Position for Treatment of Lateral Canal Benign Paroxysmal Positional Vertigo.

Objectives/hypothesis: The need for class I and II studies on the efficacy of liberatory maneuvers in the treatment of lateral canal benign paroxysmal positional vertigo (LC-BPPV) motivated the present double-blind randomized trial on the short-term efficacy of the forced prolonged position (FPP).

Study Design: Double-blind, randomized controlled trial.

Methods: Two hundred twenty-one patients with unilateral LC-BPPV met the inclusion criteria for a multicentric study.

Alexander Crum Brown: A Forgotten Pioneer in Vestibular Sciences.

Although vestibular anatomy was described in the Renaissance period, research in vestibular physiology began in the 1820s and was spearheaded by Purkinje and Flourens. This was subsequently expanded by Ménière, Helmholtz, Goltz, Mach, Breuer, Ewald, and Hogyes, who are regarded as the early pioneers in research on vestibular physiology in the 19th century. The relationship of endolymphatic flow and semicircular canal function is termed the .

Functional and Objective Audiovestibular Evaluation of Children With Apparent Semicircular Canal Dehiscence-A Case Series in a Pediatric Vestibular Center.

Semicircular canal dehiscence is a bony abnormality in the otic capsule especially involving the superior semicircular canal. Since its identification in 1998, there is significant research regarding the pathology in the adult population. This condition generates a third window effect that is well-described in the literature.