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.

Episodic ataxia type 2: unusual aspects in clinical and genetic presentation. Special emphasis in childhood.

OBJECTIVE: To describe aspects in clinical and genetic presentation in five patients with episodic ataxia type 2 (EA2). METHODS: CACNA1A gene screening identified a mutation in three probands and in two of their children. RESULTS: The three probands had attacks of imbalance, associated with dizziness/vertigo and/or headache. Two of them had independent migraine attacks. Interictal oculomotor examination revealed a gaze evoked nystagmus and central oculomotor signs. Two probands had a history of strabismus. All responded well to acetazolamide. Two children were found to have both clinical and genetic abnormalities. At 23 months, one child started to have short attacks of imbalance mimicking benign paroxysmal vertigo of childhood. Then, the frequency and duration of his attacks increased and some were associated with headache. The other child developed permanent imbalance with falls at the age of 2 years, strabismus, hyperactivity and slight to moderate cognitive deficiency. When aged 10 years, this was further complicated by episodic ataxia. Genetic analysis revealed three novel mutations in the calcium channel gene CACNA1A (chromosome 19p13). The two children had the same genetic abnormality as their parents. CONCLUSION: EA2 may present with still unknown genetic mutations in adults, and with large and various phenotypes in children, such as short attacks of imbalance or permanent imbalance, cognitive deficiency, and possibly strabismus and hyperactivity.

Impact of cochlear implantation on peripheral vestibular function in adults.

OBJECTIVE: Cochlear implantation may have a detrimental effect on vestibular function and residual hearing. Our goal was to investigate the impact of cochlear implantation on peripheral vestibular function and the symptomatology that ensues. MATERIAL AND METHODS: A prospective observational study included all adults undergoing cochlear implantation by the same operator between July 2014 and December 2015, with pre- and postoperative (4 months) neurovestibular balance examination comprising a questionnaire and clinical tests [head impulse test (HIT), head-shaking test (HST), skull vibration test (SVT)] and instrumental tests [caloric test of the lateral semicircular canal and cervical vestibular-evoked myogenic potentials (cVEMP)]. RESULTS: Twenty-two patients were included, with a mean age of 62 years and sex-ratio of 1.2. Before implantation, 50% of subjects (n=11) reported at least one episode of vertigo associated with balance disorder during their life. After implantation, there were 11 cases of vertigo but only one patient described persistent discomfort related to vertigo 4 months after surgery. Patients with impaired vestibular function after 4 months, taking all symptoms together, were all aged more than 75 years. HIT was abnormal in 18% of cases before implantation and in 59% after (P=NS). HST showed nystagmus in one patient both before and after surgery. Only 18% of patients showed nystagmus induced by SVT before surgery, increasing to one-third after surgery (P=NS). Caloric test of the lateral canal showed hypofunction in 50% of cases before surgery, including 10% of cases with areflexia. This rate increased after surgery to 58%, with 18% areflexia (P=NS). cVEMPs were not detected in 68% of cases before implantation and this rate increased to 86% after surgery (P=NS). There were no significant associations (P>0.05) between test results and symptoms. CONCLUSIONS: In the medium term, although older subjects more frequently presented vestibular disorder, cochlear implantation induced little vertigo or balance disorder, sometimes even improving vestibular function. However, vestibular disorders were frequent preoperatively and increased postoperatively. We tested vestibular function on different stimulation frequencies and yet found no correlation between postoperative test results and postoperative vertigo.

Delayed endolymphatic hydrops. Special emphasis on nystagmus associated with episodes and contribution of chemical labyrinthectomy.

OBJECTIVES: The main objective was to describe spontaneous nystagmus characteristics during an episode of delayed endolymphatic hydrops (DEH), including an initial vertical upbeating nystagmus in one patient. The secondary objective was to highlight the contribution of chemical labyrinthectomy. METHODS: Episodic vertigo after a prolonged period of time of sensorineural hearing loss (profound or total) in one ear characterized ipsilateral DEH and was associated with the development of hearing loss in the opposite ear in contralateral DEH. RESULTS: Ten patients met the criteria for DEH: 7 ipsilateral and 3 contralateral. Three (all ipsilateral DEH) were examined during a vertigo episode. Two patients had a typical horizontal-torsional nystagmus beating contralaterally to the hearing loss. One patient showed atypic initial vertical upbeating nystagmus with a slight torsional component, which secondarily became horizontal-torsional beating contralaterally to the hearing loss. Four patients had disabling vertigo with unilateral total deafness (ipsilateral DEH), successfully treated by 1-3 transtympanic gentamycin (Gentalline®) injections. CONCLUSION: Nystagmus direction during vertigo episodes varies, and may initially present as vertical upbeating nystagmus, which, to our knowledge, has not been previously reported in DEH or Menière's disease. This nystagmus might reflect an inhibition of the superior semicircular canal (on the hearing-impaired side), suggesting incipient hydrops in this canal. Chemical labyrinthectomy is a simple and effective procedure in unilateral DEH, especially as the patient often suffers from total deafness.

[Horizontal and anterior canal benign paroxysmal positional vertigo].

Benign paroxysmal positional vertigo (BPPV) is suspected when the patient complains of brief episodes of vertigo induced by changes in the head position. The direction of the positional nystagmus during the positional maneuvers is essential to affirm the canal involved. There is a consensus for the diagnosis of a PC-BPPV and HC-BPPV (geotropic and ageotropic form) as well as for the treatment of a PC-BPPV and a geotropic HC-BPPC. However, there is still a debate for the diagnosis of an AC-BPPV and the treatment of an ageotropic HC-BPPV and an AC-BPPV.

Otologic disorders causing dizziness, including surgery for vestibular disorders.

This chapter will focus on vertigo/dizziness due to inner-ear malformations, labyrinthine fistula, otosclerosis, infectious processes, and autoimmune inner-ear disorders. Inner-ear malformation due to dehiscence of the superior semicircular canal is the most recently described inner-ear malformation. Vertigo/dizziness is typically induced by sound and pressure stimuli and can be associated with auditory symptoms (conductive or mixed hearing loss). Labyrinthine fistula, except after surgery for otosclerosis, in the context of trauma or chronic otitis media with cholesteatoma, still remains a challenging disorder due to multiple uncertainties regarding diagnostic and management strategies. Otosclerosis typically manifests with auditory symptoms and conductive or mixed hearing loss on audiometry. Vertigo/dizziness is rare in nonoperated otosclerosis and should draw clinical attention to an inner-ear malformation. Computed tomography scan confirms otosclerosis in most cases and should rule out an inner-ear malformation, avoiding needless middle-ear surgical exploration. Labyrinth involvement after an infectious process is unilateral when it complicates a middle-ear infection but can be bilateral after meningitis. Labyrinth involvement due to an inflammatory disease is a challenging issue, particularly when restricted to the inner ear. The diagnosis relies on the bilateral and rapid aggravation of audiovestibular symptoms that will not respond to conventional therapy but to immunosuppressive drugs.

Creutzfeldt-Jakob disease after extracranial dura mater embolization for a nasopharyngeal angiofibroma.

We report a 25-year-old man with Creutzfeldt-Jakob disease (CJD) who had received dura mater embolization in the external carotid artery for a nasopharyngeal angiofibroma 90 months earlier. The patient was heterozygotous (Met/Val) at codon 129. This case suggests that dura mater embolization can be responsible for the CJD.

[Post-traumatic syringomyelobulbia and inferior vertical nystagmus]. / Syringomyélobulbie post-traumatique et nystagmus vertical inférieur.

A case of post-traumatic cervico-thoracic syringomyelia was complicated, 3 years after the injury, by a bulbar extension manifested by a downbeat vertical nystagmus which became a source of disabling oscillopsia. The syringobulbia was visualized at MRI. The usefulness of syringostomy in such a case is discussed.

[Attical fixation of the malleus head associated with obstruction of the round window niche by a high jugular bulb]. / Blocage attical de la tête du marteau associé à une obstruction de la fenêtre ronde par le golfe de la jugulaire.

OBJECTIVE: To report the case of a 52 year-old-woman presenting with a left mixed hearing loss. Surgical exploration revealed an attical fixation of the malleus head associated with obliteration of the round window by a high jugular bulb. METHOD: Surgical exploration revealed this rare association. Attical fixation of the malleus head was treated by a partial prosthesis. The mobility of the chain was normal at the end of the surgical exploration but it was not possible to search for the round window sign because the niche of the round window was obliterated by an high Jugular bulb. RESULTS: Audiogram was unchanged even if CT-scan confirm the good position of the partial prosthesis. CONCLUSION: Surgical treatment of an attical fixation of the malleus head do not seem to be necessary if it is associated with a simultaneous obliteration of the round window. Indeed, obstruction of the round window by a high Jugular bulb can caused a conductive hearing loss.

[Benign paroxysmal positional vertigo of the horizontal canal. Clinical features in 25 patients]. / Le vertige positionnel paroxystique bénin du canal horizontal.

OBJECTIVES: To review the clinical features of benign paroxysmal positional vertigo of the horizontal canal (HC-BPPV). PATIENTS AND METHODS: Retrospective study (September 1999 to March 2001) of 25 patients with HC-BPPV. Patients complained of positional vertigo associated with direction changing horizontal positional nystagmus, either geotropic or ageotropic. RESULTS: The horizontal nystagmus was triggered in all patients when rolled to either side in a supine position (head raised 30 degrees) and in 14 patients by the Dix Hallpike manoeuvre. The nystagmus was geotropic in 15 patients and ageotropic in 10. It varies in time in 4 patients with one change in 3 (from geotropic to ageotropic in 2 and ageotropic to geotropic in 1) and 4 changes in 10 days in another patient. In one patient with head trauma, the HC-BPPV was currently associated with ipsilateral posterior canal benign paroxysmal positional vertigo (PC-BPPV), and its characteristic rotatory-up beat nystagmus. Interestingly, 4 patients had a previous history of ipsilateral PC-BPPV and in 5 others an ipsilateral PC-BPPV occurred after the HC-BPPV. Although spontaneous recovery of HC-BPPV was common in most patients, a 270 degrees "barbecue rotation" or a "prolonged position on the healthy side" was effective in some patients. CONCLUSION: Patients with positional vertigo should undergo Dix Hallpike positioning and supine lateral head turns as this latter manoeuvre is more efficient to diagnose an HC-BPPV. Both the transformation of the positional horizontal nystagmus (geotropic-ageotropic) and the association with a PC-BPPV support the diagnosis of an HC-BPPV.

[Cochlear otosclerosis and pseudo fourth turn of the cochlea]. / Otospongiose cochléaire et pseudo-quatrième tour de cochlée.

OBJECTIVE: To describe different sequences on magnetic resonance imaging (MRI) in otosclerosis with peri-cochlear involvement. METHOD: MRI T1 and T2 sequences with T1 gadolinium injection and computed tomography (CT) scans with millimetric slices on axial and coronal views were obtained. The diagnosis of bilateral otosclerosis was confirmed by surgical exploration. RESULTS: On the CT scan, there was a fourth turn of the cochlea which appeared on the MRI T1 sequence with an intermediate signal and on the T2 sequences with a high intensity signal. After gadolinium injection, there was signal enhancement, suggestive of active otospongiosis. On the CT scan, there was another lesion in front of the cochlea with endosteal involvement. This was no however visible on the MRI, even after gadolinium infusion, in accordance with inactive otospongiosis. DISCUSSION: We reviewed the literature concerning MRI and results in otosclerosis. CONCLUSION: MRI of the labyrinth with T1 sequences and gadolinium injection can be contributive to the diagnosis of otosclerosis to differentiate inactive from inactive otospongiosis. However, prospective studies must be conducted to confirm this hypothesis.

[Failure of regain full function after surgery for otosclerosis: causes, diagnosis and treatment]. / Echecs fonctionnels dans la chirurgie de l'otospongiose: causes, diagnostic et traitement.

OBJECTIVE: The aim of this study was to evaluate the causes, the diagnosis, the treatment and the results obtained by revision surgery, in cases of stapedectomy failures. MATERIALS AND METHODS: Retrospective study of 50 recent cases operated on between January 1997 and December 2001. DIAGNOSIS OF THE FAILURE: All reoperated patients had clinical and audiological assessment; CT scan with virtual endoscopy. RESULTS: The mean time of onset of failure was 23 months. The mean preoperative air bone gap was 25.5 dB. Otoscopy revealed a retraction pocket caused by poor eustachian tube function in 9 cases. CT scan proved to be very effective at differentiating the cause of the failure. OPERATIVE FINDINGS: In 11 cases the prosthesis was too short, in 8 cases the prosthesis had migrated out of the hole of stapedotomy and in 6 cases the piston was fixed in the stapedotomy hole. A partial or complete lysis of the long process of the incus was frequently associated, but in 9 cases it was the only cause of the failure. In all the cases when the piston was displaced, the stapedotomy was found to be covered by a thin mucous membrane, avoiding labyrinthine fistula. In 3 cases, the failure was due to recurrent otosclerosis. In 5 cases the failure was due to a local anomaly at the level of the oval window niche, 2 cases of failure were due to a malleus ankylosis. In 5 cases fibrous adhesion was found between the incus and the mucosa of the promontory. In one case a reparative granuloma was found at the level of the oval window. TREATMENT: In cases of partial lysis of the long process of the incus, a new prosthesis was placed in a 0.4 mm diameter stapedotomy, performed using a KTP laser. A 0.4 mm diameter piston was extended 0.2 mm below stapedotomy to avoid a new extrusion. Indeed some prosthesis extrusion could be due to increased movements of the ossicular chain in cases of eustachian tube dysfunction. In cases of complete lysis of the long process of the incus, or in cases of a very short long process of the incus, a piston was put in the stapedotomy and attached to the malleus manubrium. The results of revision stapedotomy were favorable in the absence of associated fibrous tissue adhesion or local malformation. The air bone gap was found to be less than 10 dB in 40 cases and between 10 and 20 dB in 8 cases. An impairment of the air bone gap was found in 2 cases. No case of bone conduction impairment was found in this series.

[Intrapetrous cholesteatoma]. / Les cholestéatomes intra-pétreux.

Intrapetrous cholesteatomas correspond to lesions extending beyond the classical limits of impairment at the level of the middle ear. This paper analyzes 31 cases of which only 2 are definitely primary, the other 29 probably being secondary. In 5 cases, a cholesteatoma had been previously removed by an open or semi-open technique. Such cholesteatomas, that are found at all ages (from 12 to 74), affect both sexes equally. They are easy to diagnose when the symptomatology combines a history of otitis, damage to facial motoricity, mixed deafness or anacusis and a tympanic aspect of cholesteatoma. They are much more difficult to diagnose in the absence of any facial deficit, of major deafness, and even more so if the tympanum is closed. CT-scanning and MRI now enable a precise study of the nature of the complaint and its extension. Surgical treatment requires full mastery of all the techniques of otoneurosurgery, the procedure depending very much upon the seat and extent of the intrapetrous cholesteatoma.

[Significance of lasers in surgery of cerebellopontine angle tumors]. / Intérêt du laser dans la chirurgie des tumeurs de l'angle ponto-cérébelleux.

The use of the laser in otology has demonstrated its effectiveness and safety. The aim of this paper is to show its application in otoneurosurgery. After comparing the advantages and disadvantages of various lasers (Yag, CO2, KTP), we soon opted for the KTP laser, which we have since used exclusively in otology and otoneurosurgery. 25 patients with cerebello-pontine angletumours were operated using the KTP laser (22 neuromas and 3 meningiomas). Although in the absence of rigourous methodology it is difficult to assert the superiority of the laser over the classical techniques of resection, the results seem better since we have been using the laser for otoneurosurgery, especially for facial function. In any case, the laser is merely an instrument, which it is necessary to learn to manipulate; it does allow lesions to be vaporized without traction, and has a haemostatic action. It should never be used directly in contact with nervous structures, especially nerves. It seems to us to be most helpful in dealing with large and very vascular tumours.

[Cholesteatoma and labyrinthine fistula]. / Cholestéatome et fistule labyrinthique.

43 cases of large labyrinthic fistulas caused by a cholesteatoma of the middle ear were studied (33 External Semi-circular Canal fistulas, 4 External Semi-circular Canal amputations with opening of the vestibule, 3 of the promontory, 2 of the oval window, 1 of the Semi-circular Canal). The audiometric, preoperative, radiological data was analyzed. The radiological exam is often disappointing for the average-sized fistulas. Preoperative labyrinthization is moderate for External Semi-circular Canal fistulas and even for certain vestibular amputations; it is often incomplete in the other locations. Systematic exeresis of the cholesteatoma matrix is recommended by the authors, at least for the External Semi-circular Canal fistulas as it is not generally accompanied by aggravation of the bony curve. In the other locations, surprising auditory preservations were observed. In conclusion, the presence of External Semi-circular Canal fistulas should not be a counter-indication for carrying out a ossiculoplasty.

Cholesterol granuloma of the middle ear invading the cochlea.

OBJECTIVES: To report a second case of cholesterol granuloma of the middle ear invading the cochlea. MATERIAL AND METHODS: A 54-year-old woman, who had undergone right-side tympanoplasty with stapedectomy, complained of intermittent right-side otorrhea associated with cophosis. Otomicroscopy found anterior eardrum perforation with mucopurulent effusion. Computed tomography (CT) showed a lesion filling the tympanic cavity, exposing the tympanic facial nerve, with destruction of the ossicles; the vestibule and cochlea were ossified except for the basal turn. The petrous apex was normal. Magnetic resonance imaging (MRI) showed invasion of the cavity and basal turn of the cochlea by a mass in heterogeneous hypersignal on T-1 weighted images, non-enhanced by gadolinium injection, and hyperintensity on T2-weighted images. Cholesterol granuloma of the middle ear was suspected, with surgery indicated due to the facial nerve exposure and cochlear invasion. RESULTS: A brownish-yellow compressive mass invading the basal turn of the cochlea, suggestive of cholesterol granuloma, was removed. Histologic examination confirmed diagnosis. CONCLUSION: Direct invasion of the otic capsule by cholesterol granuloma is extremely unusual. Surgery is indicated in such cases, to avoid onset of neurologic complications.