Guidelines of the French Society of Otorhinolaryngology (SFORL) for teleconsultation in patients with vertigo during the COVID-19 pandemic.

OBJECTIVES: In the context of the SARS-CoV-2 pandemic, patients may have been dissuaded from seeking consultation, thus exposing themselves to a risk of loss of chance. This guide aims to define how teleconsultation can assist in assessing vertiginous adults or children, and to gather the information needed to provide quick medical care. METHODS: These recommendations rely on the authors' experience as well as on literature. A survey on otoneurologic approach via telemedicine has been conducted based on a literature search until March 2020. RESULTS: The first clinical assessment of the vertiginous patient via teleconsultation can only be successful if the following conditions are met: initial contact to verify the feasibility of the assessment at a distance, the presence of a caregiver in order to assist the patient, the possibility of making video recordings. Medical history via telemedicine, as in a face-to-face assessment, allows to assess the characteristics, duration, frequency, and potential triggering factors of the vertigo, in both children and adults. During teleconsultation, the following tests can be carried out: oculomotricity evaluation, assessment of balance, simple neurological tests, checking for positional vertigo/nystagmus and, eventually to perform canalith-repositioning procedures. In children, the following should be searched for: history of hearing or visual impairment, a context of fever or trauma, otorrhea, signs of meningeal irritation. CONCLUSION: The neurotologic telemedicine relies on the accuracy of the clinical assessment, which is based on history taking and a few simple tests, encouraging the development of a decision-making algorithm adapted for teleconsultation. However, the latter has its limitations during an emergency examination of a new patient presenting vertigo, and, at least in some cases, cannot replace a face-to-face consultation. Teleconsultation is often adapted for follow-up consultations of previously selected vertiginous patients during face-to-face assessment.

Vestibular impact of Friedreich ataxia in early onset patients.

BACKGROUND: Friedreich ataxia (FRDA) is the most frequent form of inherited ataxias. Vestibular and auditory assessments are not commonly part of the check up for these patients despite hearing and balance complaints. Screening of vestibular and auditory function was performed in a large group of young patients with genetically confirmed FRDA. METHODS: Our study included 43 patients (7-24 years of age). A complete vestibular assessment was performed including the canals function evaluation at 3 head velocities (bithermal caloric test, earth vertical axis rotation (EVAR) and head impulse test (HIT)) and otolith function evaluation (cervical vestibular evoked myogenic potentials). Information regarding the hearing evaluation of the patients were also retrieved including impedance tympanometry, distortion product otoacoustic emissions (DPOAEs), air and bone conduction audiometry and auditory brainstem response (ABR). RESULTS: Vestibular responses were impaired for canal responses (only at high and middle head velocities) and vestibulospinal otolithic responses. Abnormal neural conduction in the central auditory pathways was frequently observed. Oculomotor abnormalities were frequent, mostly hypermetric saccades and gaze instability. Inhibition of the vestibulo-ocular reflex by fixation was normal. CONCLUSIONS: We show that Friedreich ataxia, even at onset, frequently associate saccadic intrusions, abnormal ABRs and decreased vestibulo-ocular and vestibulospinal responses progressing over time. These sensory impairments combined with ataxia further impair patient's autonomy. These vestibular, auditory and visual impairments could be used as markers of the severity and progression of the disease. Adding vestibular and auditory testing to Friedreich patient's evaluation may help physicians improve patient's management.

[Vestibular neuronitis in a teenager with sickle cell disease. Treatment is urgent]. / Syndrome vestibulaire chez une adolescente drépanocytaire SC. Le traitement est urgent.

Vestibular syndrome is not frequently described in patients with sickle cell disease. We report the case of a teenager with sickle cell disease who had a vestibular syndrome with vertigo that successfully responded to exchange transfusion. We discuss guidelines and review the literature in view of this case report. Sensorineural disorders should be considered as stroke syndromes. They require urgent treatment consisting of exchange transfusion or maintaining optimal hydration associated with blood withdrawal. Treatment of vestibular syndrome in sickle cell disease is urgent.

Potential value of vestibular evoked myogenic potentials in paediatric neuropathies.

PURPOSE: Showing the interest of vestibular evoked myogenic potentials in paediatric neurological vestibulospinal pathology detection and followup. MATERIALS AND METHODS: The vestibular evoked myogenic potentials testing apparatus presented is now commonly used in ENT clinics for patients from 1 month of age. Our system and protocol permits control to evoke and select the best EMG level and makes possible a comparison of data from different sides or level of stimulation or different sessions. Normal vestibular evoked myogenic potentials latencies obtained with tone bursts were remarkably stable (P: 13 +/- 0.8 ms, N: 19.6 +/- 1.6 ms). The reported case illustrates abnormal vestibular evoked myogenic potentials latencies in neuropathy. RESULTS: A 6 y.o. child with progressive imbalance was referred to the ENT department for vestibular functional evaluation. Abnormally long latencies in the vestibular evoked myogenic potentials and neurological examination oriented the diagnosis towards Guillain-Barre syndrome and immediate referral to a neurology department. Vestibular evoked myogenic potentials also helped to monitor the neurological recovery. CONCLUSION: The present case shows the potential value of vestibular evoked myogenic potentials in diagnosis and evaluation of descending brainstem pathways in neuropathies like Guillain-Barre syndrome in complement to neurological evaluation.

Familial CHARGE syndrome because of CHD7 mutation: clinical intra- and interfamilial variability.

CHARGE syndrome (OMIM #214800) is a multiple malformation syndrome with distinctive diagnostic criteria, usually because of CHD7 (chromodomain helicase DNA binding 7) haploinsufficiency. Familial occurrence of CHARGE syndrome is rare. We report six patients from two Caucasian families (both with one parent and two children) affected by mild to severe CHARGE syndrome. Direct sequencing of the CHD7 gene was performed in these two unrelated families. A mutation in exon 8 (c.2501C>T - p.S834F) in first chromodomain was found in family A and a nonsense mutation in exon 2 (c.469C>T - p.R157X) in family B. Both mutations are de novo in the parents. In family A, the elder son had bilateral cleft lip and palate, esophageal atresia with fistula, complex heart defect and vertebral abnormalities, while the younger had a posterior coloboma. Their mother had asymptomatic vestibular dysfunction and retinal coloboma, identified after the molecular diagnosis of her children. In family B, both affected children had severe expression of CHARGE syndrome. The father carrying the mutation only had asymmetric anomaly of the pinnae. These familial reports describe the intrafamilial variability of CHARGE syndrome, and underline the presence of CHD7 mutations in patients who do not fit the 'classical clinical criteria' for CHARGE syndrome.

[Vertigo in children]. / Les vertiges chez l'enfant

Vertigo and dizziness in children, when these symptoms are recognized, worry physician and children's families and urge them, before doing a good otological, neurological and vestibular clinical examination, on a prescription of CT scan or an NMR which are unnecessary test in most of the cases and expensive tests. A better knowledge of the clinical signs of vestibular deficit and of the most frequent origins of vertigo and dizziness in children (migraine equivalent, ophthalmological disorders, benign paroxysmal idiopathic pediatric vertigo, temporal bone fracture) permits a better and more adapted procedure for diagnosis and therapy.

Vestibular anomalies in CHARGE syndrome: investigations on and consequences for postural development.

UNLABELLED: Recently, vestibular anomalies have been described as a frequent feature in children with coloboma-heart-atresia-retarded-genital-ear (CHARGE) syndrome. They are likely to play an important role in the psychomotor retardation affecting these children. In order to test this hypothesis, we prospectively performed complete vestibular investigations in a series of 17 CHARGE syndrome patients including inner ear CT scan and functional vestibular evaluation of both canal and otolith functions. These results were correlated with the postural anomalies observed during the children's development and showed that vestibular dysfunction is a constant feature in CHARGE syndrome and has very good sensitivity for confirming the diagnosis. Anomalies of semicircular canals were frequently found (94%), easily detectable on CT scan and associated with no response on canal function evaluation. They were considered as partly responsible for the retardation of postural stages. Vestibular functional tests were consistently abnormal but allowed detection of residual otolith function in most patients (94%). All children of this series had an atypical pattern of postural behaviour that we consider to be related to their vestibular anomalies. Residual otolith function seems to have a positive influence for postural development. CONCLUSION: Vestibular investigations are valuable for diagnosis, developmental assessment, and adaptation of specific rehabilitation programmes in CHARGE syndrome patients.

Vertigo is an underestimated symptom of ocular disorders: dizzy children do not always need MRI.

Vertigo, instability, dizziness, or equilibrium disorders are not usually considered as consequences of ophthalmologic problems. We present data indicating that ocular disorders can be responsible for these symptoms in children. In a population of 523 pediatric patients with vertigo or disequilibrium and referred for vestibular testing in our otolaryngology department during a 5-year period, 27 children presented with normal vestibular and somatic neurologic examinations but with ophthalmologic disorders (vergence insufficiency or latent strabismus with binocular vision in 70% and anisometropia in 41%). These patients represented 24% of all vergence insufficiencies detected and 4% of all orthoptic examinations performed in the pediatric ophthalmology department. These ocular abnormalities were considered to be the initial cause of the problems. In two thirds of these patients the symptoms were completely resolved by simple ophthalmologic treatment. No other additional tests, such as magnetic resonance imaging, were required. Therefore we propose that every child complaining of vertigo or dizziness but with normal clinical somatic neurologic and vestibular examinations should have a complete ophthalmologic examination before additional, more costly, investigations. This should lead to better screening and more appropriate care of ocular disorders in children and avoid unnecessary magnetic resonance imaging.

[Inner ear in C.H.A.R.G.E. association]. / L'oreille interne dans l'association C.H.A.R.G.E.

Cochleovestibular dysfunction is one of the major features of C.H.A. R.G.E. association. The inner ear anomalies were studied in a population of 17 children with CHARGE. Temporal bone anomalies were defined with CT scan, hearing loss was evaluated with audiologic procedures appropriate for age, and functional canal and otolith vestibular impairment evaluated with respectively vertical and off vertical axis rotation (OVAR) tests. Temporal bone anomalies appear specific of C.H.A.R.G.E. association. They are characterized by an aplasia of the posterior labyrinth (with an absence of semicircular canals) and a variable degree of anomalies of the anterior labyrinth with sometimes normal cochlea. Hearing loss is variable, often worsened by delayed neurological maturity and frequent association of middle ear effusion. Thus, multiple audiologic evaluations are required at regular intervals. The treatment of middle ear effusion, the association of hearing aid and speech therapy management permit language acquisition in spite of the other multiple sensory handicaps. Vestibular functional evaluation shows a constant canal areflexia but a residual vestibular otolith function (sometimes normal). The deficit of the vestibular function is certainly involved in the delay of posturo-motor development as well as visual deficit and neurological impairment. This can permit an adaption of the physical therapy program for each child to make use of the available sensorial information.

[Direct foreign body footplate trauma: 3 cases]. / Traumatisme direct de la platine par corps étranger, à propos de 3 cas.

3 cases of direct foreign body footplate trauma are described in children. A surgical exploration was decided on history, cochleovestibular signs and/or CT abnormalities. Hearing improved in all cases after surgery.

Motoneuron axon distribution in the cat stapedius muscle.

Stapedius-motoneuron cell bodies in the brainstem are spatially organized according to their acoustic response laterality, as demonstrated by intracellular labeling of physiologically identified motoneurons [Vacher et al., 1989. J. Comp. Neurol. 289, 401-415]. To determine whether a similar functional spatial segregation is present in the muscle, we traced physiologically identified, labeled axons into the stapedius muscle. Ten labeled axons were visible in the facial nerve and five could be traced to endplates within the muscle. These five axons had 39 observed branches (others may have been missed). This indicates an average innervation ratio (> or = 7.8) which is much higher than that obtained from previous estimates of the numbers of stapedius motoneurons and muscle fibers in the cat. One well-labeled stapedius motor axon innervated only a single muscle fiber. In contrast, two labeled axons had over 10 endings and innervated muscle fibers spread over wide areas in the muscle. Two of the axons branched and coursed through two primary stapedius fascicles, indicating that the muscle zones innervated by different primary fascicles are not functionally segregated. In another series of experiments, retrograde tracers were deposited in individual primary nerve fascicles. In every case, labeled stapedius-motoneuron cell bodies were found in each of the physiologically identified stapedius-motoneuron regions in the brainstem. These observations suggest there is little, if any, functional spatial segregation based on separate muscle compartments in the stapedius muscle, despite there being functional spatial segregation in the stapedius-motoneuron pool centrally.

Vestibular function in children with the CHARGE association.

BACKGROUND: Histopathological examinations and computed tomographic scans of the temporal bone in patients with the CHARGE association (a malformative syndrome that includes coloboma, heart disease, choanal atresia, retarded development, genital hypoplasia, and ear anomalies, including hypoplasia of the external ear and hearing loss) have shown an absence of semicircular canals and a Mondini form of cochlear dysplasia. Until recently, no information was available concerning a possible loss of vestibular function, which could be a factor in retarded posturomotor development. To our knowledge, this is the first report of otolith tests done on patients with the CHARGE association. OBJECTIVE: To test residual vestibular function in patients with the CHARGE association. STUDY DESIGN: In 7 patients with the CHARGE association, we made electro-oculographic recordings of vestibulo-ocular responses to earth-vertical and off-vertical axis rotations to evaluate the function of the canal and the otolith-vestibular systems. RESULTS: None of the 7 patients had semicircular canals in the computed tomographic scan, and none had canal vestibulo-ocular responses to earth-vertical axis rotation, but all had normal otolith vestibulo-ocular responses to the off-vertical axis rotation test. CONCLUSIONS: These results support the hypothesis of a residual functional otolith organ in the hypoplastic posterior labyrinth of children with the CHARGE association. The severe delays in psychomotor development presented by these children are more likely a consequence of multiple factors: canal vestibular deficit, visual impairment, and environmental conditions (long hospital stays and breathing and feeding problems). The remaining sensitivity of the otolith system to gravity and linear acceleration forces in these children could be exploited in early education programs to improve their posturomotor development.

Asymmetric otolith vestibulo-ocular responses in children with idiopathic scoliosis.

OBJECTIVE: A suggested cause of idiopathic scoliosis (IS) in children is a disequilibrium in the vestibulospinal control of trunk muscles. We sought a correlation between otolith vestibular dysfunction and IS. METHODS: A recently developed test for evaluation of otolith vestibular function (off-vertical axis rotation, OVAR) was applied to 30 children with IS, 12 control subjects, and 3 with congenital scoliosis as a result of spinal deformities. RESULTS: Of the patients with IS, 67% had significantly greater values of directional preponderance on the OVAR test (a measure of otolith system imbalance) compared with control subjects. Patients with congenital scoliosis showed normal responses on the OVAR test. No correlation was found between the direction of the preponderance and the side of the spine imbalance, or between the directional preponderance and the curve magnitude. The rate of progression of the scoliosis was not significantly correlated with the amplitude of the directional preponderance. CONCLUSION: These results support the hypothesis that central otolith vestibular system disorders lead to a vestibulospinal system imbalance, and may be a factor in the cause of IS.

Canal and otolith vestibulo-ocular reflexes to vertical and off vertical axis rotations in children learning to walk.

In order to determine the characteristics of the vestibulo-ocular reflexes (VORs) as a function of age and posturo-motor development, a group of 26 normal children (6 to 25 months old) were tested at three different stages of posturo-motor control: prior to as well as during the first attempts to walk without support, and during the first year of independent walking. The test consisted of electro-oculographic (EOG) recordings of the VOR responses to horizontal semi-circular canal and otolith stimulations. The canal VOR was elicited in seated subjects by rotatory impulsions about a vertical axis (acceleration and deceleration both at 40 degrees/s2, separated by a rotation at 60 degrees/s velocity). The otolith VOR was elicited by inclining the rotating chair by 9 degrees respect to gravity. For the canal VOR, the time constant and the highest initial slow phase velocity were measured. The otolith VOR was characterized by the amplitude of the modulation and the bias (offset of baseline from zero) of the slow phase velocity averaged over 10 to 20 rotation cycles, for both the horizontal and vertical components of the response. The pooled values of these data show that canal VOR parameters did not vary significantly either with age of the children or with their stage of posturo-motor control. However, the otolith VOR parameters changed during the period of learning to walk: the modulation of the horizontal component increased and the modulation of the vertical component decreased significantly. Thus the ability to walk without support is marked by a significant change in the otolith but not canal responses; since the vestibular sensory organs develop anatomically at the same rate these results indicate that central nervous system processors of canal and otolith information develop independently.

Head coordination in the sagittal plane in toddlers during walking: preliminary results.

In adults, head rotations in the pitch plane are highly synchronized with vertical head translations during different locomotor tasks as walking, running, and hopping. The head is rotating up then down in the pitch plane as stepping movement drives the head down then up, respectively. The purpose of our study was to determine at which period of the motor control development this fine head coordination occurred. The organization of head movements was analyzed in four normal children observed longitudinally from the onset of walking up to 80 weeks of independent walking (IW). The degree of synchronization between vertical head translations and head rotations in the pitch plane was used to define an index of head coordination for 15 to 25 steps per child per session. Our results show that the coordination of head rotations in the pitch plane improves continually in toddlers but does not reach the optimal level during the first year of IW experience. We showed previously that head stabilization in space was achieved during the first weeks of IW. Both head stabilization in space and fine head coordination during walking are considered as directly involved in gaze stabilization, but in toddlers head stabilization in space is completed earlier than head coordination. Head stabilization appears to be a necessary motor control to acquire prior to the development of head movement coordination.

Trunk and head stabilization during the first months of independent walking.

This study measured the rate of acquisition of head and trunk postural control during the two early developmental periods of independent walking, as defined by global gait parameters. Gait parameters were observed longitudinally in four children. The maximum angular deviations of the trunk and head oscillations were computed in the frontal and sagittal planes. These decreased most dramatically during the first 10-15 weeks of independent walking, during the same period when global gait parameters changed rapidly. This head and trunk stabilization may be a fundamental process that help to maintain equilibrium during walking, and may be a necessary step prior to the development of fine posturo-motor control.

Audiological assessment of infants and children with preauricular tags.

A previous study found sensorineural hearing loss in 13% of 178 children with solitary preauricular tags. The present results are quite different. Audiological assessment was performed on 42 children (median age of 2 months) with preauricular tags as the sole anatomical malformation. Tests included behavioral audiometry and transient evoked otoacoustic emissions. Twenty-two subjects were also tested with auditory brainstem recording while under general anesthesia during surgical excision of the tag. None of the children had a sensorineural hearing loss, but 4 had a bilateral or unilateral conductive hearing loss due to secretory otitis media. It is concluded that solitary ear tags are not a high risk factor for sensorineural hearing loss if not associated with a family history of hearing loss.