Writing in the European Annals of Otorhinolaryngology Head & Neck Diseases. Dos and Don'ts!

Too many articles are still rejected by scientific medical journals due to lack of preparation of the manuscript and of knowledge of the modern editorial rules that govern scientific medical writing. Therefore, the editorial board of the European Annals of Otorhinolaryngology Heads & Neck Diseases summarized studies published by its members since 2020 in the columns of the scientific journal of the French Society of Otorhinolaryngology and the International Francophone Society of Otorhinolaryngology and data from the PubMed indexed literature dedicated to scientific medical writing in otolaryngology in the 21st century. The authors hope that this review, in the form of a list of "Dos and Don'ts", will provide authors with a practical guide facilitating publication of rigorous, reproducible and transparent scientific studies, in accordance with the movement toward better science that society as a whole has been fighting for since the beginning of this century.

Bilateral vestibulopathy and disabling vertigo: A CARE case series.

INTRODUCTION: The term bilateral vestibulopathy (BV) was recently defined by the Bárány Society. Loss of otolith function was not included in their criteria. Although spontaneous progression to complete bilateral impairment of vestibular function is expected, it is unlikely that patients with advanced BV will continue to present episodes of intense vertigo. Here, following CARE case report guidelines, we report the case of patients meeting the criteria for BV and still disabled by vertigo. CASE SERIES: Three patients evaluated in our department meeting the Bárány criteria for definite BV but still complaining of disabling rotatory vertigo were included. All underwent clinical and instrumental vestibular examination. The observations are reported. CONCLUSION: In case of BV, the conservation of a stable otolithic reference frame could allow patients to optimize postural strategy. It would be useful to revisit a classification of BV by stages, by introducing an evaluation of otolithic function and postural control for possible subsequent vestibular implantation.

A new genetic variant causing auditory neuropathy: A CARE case report.

INTRODUCTION: Auditory neuropathy refers to impaired synchronization of the auditory signal along the cochlear nerve. The present study, following CARE case report guidelines, describes a case of auditory neuropathy secondary to a genetic variant not previously described. OBSERVATION: An 18-year-old patient was followed for multiple learning disorder. His main complaint was speech comprehension, especially in noise. Auditory neuropathy was diagnosed on electrophysiological criteria, linked to a 2.66Mb deletion on the short arm of chromosome 16, at 16p13.11p12.3 (15,492,317-18,162,167, according to the hg19 version of the human reference genome). Adapted speech therapy sessions with auditory training for intelligibility in noise and a hearing aid with high-frequency microphone were prescribed. At 6months, the patient reported improvement in understanding speech in noise. CONCLUSION: The involvement of this 16p13.11 deletion in the patient's symptomatology was not obvious, in a probable context of incomplete penetrance and variable expression. Early diagnosis of auditory neuropathy allowed implementation of better adapted multidisciplinary specialized management.

Guidelines of the French Society of Otorhinolaryngology-Head and Neck Surgery (SFORL) and the French Society of Audiology (SFA) for Speech-in-Noise Testing in Adults.

OBJECTIVES: This document presents the fundamentals of speech audiometry in noise, general requirements for implementation and criteria for choice among the tests available in French according to the health-professional's needs. MATERIAL AND METHODS: The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of doctors, audiologists and audioprosthetists from all over France. They are graded A, B, C or expert opinion according to decreasing level of scientific evidence. RESULTS: Eight tests of speech audiometry in noise can be used in France. CONCLUSION: To be complete, evaluation of hearing status requires testing understanding of speech in noise. The examination must begin with a minimum of two measurements familiarizing the subject with the test procedure. For initial diagnosis, adaptive procedures establishing the 50% speech reception threshold (SRT50) in noise are to be preferred in order to obtain a rapid and standardized measurement of perception of speech in noise. When the aim is to measure real-life speech comprehension, tests based on sentences, cocktail-party noise and free-field stimulation are to be preferred. Prosthetic gain is evaluated exclusively in free field. This is the only way to evaluate the contribution of binaurality and to measure perception in noise in an environment as close as possible to real life. In order to avoid acoustic interference in free field, at least five loudspeakers should be used, in particular for evaluating the effectiveness of directional microphones, CROS devices enabling sounds picked up in the damaged ear to be rerouted to the functional ear, or bimodal fitting (i.e., when hearing is enabled by two modalities: for example, hearing aid for one ear, cochlear implant for the other).

Contribution of bone conduction click-evoked auditory brainstem responses to diagnosis of hearing loss in infants in France.

INTRODUCTION: Neonatal hearing screening in France involves confirmation by a childhood hearing expert centre in case of suspected hearing loss. Although click-evoked air-conduction auditory brainstem responses (AC-ABR) are the gold standard in France, there are no guidelines for bone-conduction ABRs (BC-ABR). The present study assessed the interest of associating click-evoked BC- and AC-ABRs for diagnostic confirmation in neonatal hearing screening. MATERIALS AND METHODS: A retrospective study included 59 infant ears with conductive hearing loss referred to the centre of Lyon, France. Objective hearing thresholds were compared between click-evoked BC- and AC-ABRs on a method previously validated in a normal-hearing population. RESULTS: There was a significant difference in mean threshold between AC-ABR (53.27±1.189 dBnHL) and BC-ABR (28.1±0.935 dBnHL) (P<0.001). AC thresholds ranged from 40 to 60 dBnHL while BC thresholds exceeded 40 dBnHL in only 9 ears. CONCLUSION: Using BC-ABRs could reduce the false-positive rate in neonatal bilateral permanent hearing loss screening, in complement to AC-ABRs using the same stimulus. Click-evoked BC-ABR could be contributive whenever conductive hearing loss is suspected, in complement to AC-ABR, without unreasonably increasing examination time.

Audiovestibular neuropathy in an immunocompetent man with cryptococcal meningitis.

INTRODUCTION: Cryptococcus spp. is a fungus responsible for 600,000 deaths per year worldwide, mainly in immunosuppressed subjects. However, 20% of cases occur in immunocompetent subjects. Neuropathic disorders involving the auditory nerve have been reported, but vestibular disorders have never been described in detail. We report the case of an immunocompetent man, who presented audiovestibular disorders leading to a diagnosis of cryptococcal meningitis. CASE REPORT: A 39-year-old man was referred for balance disorders and right sensorineural hearing loss. He presented right vestibulo-saccular impairment and bilateral absence of auditory brainstem responses. Brain MRI was suggestive of cryptococcal meningitis. A cystic lesion in the right flocculus compressed the vestibulocochlear nerve. During monthly follow-up, pure tone audiometry gradually improved and speech audiometry in silence returned to normal. Partial resynchronization of the auditory afferent pathways was observed only on the contralateral side to vestibulocochlear nerve compression, while complete recovery of saccular function was observed. DISCUSSION: Cryptococcal meningitis in immunocompetent subjects may be accompanied by lesions of the auditory and vestibular afferent pathways. Recovery of hearing and balance was observed in response to medical treatment and early vestibular rehabilitation.

Vestibular impairment in cochlear implanted children presenting enlarged vestibular aqueduct and enlarged endolymphatic sac.

Enlarged vestibular aqueduct (EVA) is a common finding in tomodensitometry. When cranial MRI is performed, enlarged endolymphatic sac (EES) can also be found. Profound hearing loss is a common finding in these patients but a few studies have investigated vestibular function after cochlear implantation (CI) in EVA and EES patients. Our main objective was to find out whether in EVA children candidates to CI, a higher endolymphatic sac (ES) volume was predictive for higher rates of postsurgical vestibular complications. METHODS: We retrospectively included EVA children who benefited from CI, during the last 2 years. Two groups were constituted according to the presence or not of a vestibular impairment (decrease in the VOR gain on the VHIT test on one of the semicircular canals and/or a loss of cVEMPs) 6 months after CI. Endolymphatic volume of both VA and ES was measured for each patient. RESULTS: Fifteen patients were included. The mean endolymph volume was significantly higher in the impaired group (0.40 cm3 ± 0.23, range 0.08-0.70) than in the non-impaired group (0.11 cm3 ± 0.07, range 0.04-0.29; p = 0.029). Four children of the impaired group were followed during one year. At the end of vestibular rehabilitation, all children recovered a lateral canal function and a saccular function. CONCLUSION: In EVA children, a combined EES appears to increase the risk of severe post CI vestibular impairment. To minimize this risk prior CI surgery, besides tomodensitometry, MRI measurement of the ES volume should be systematically performed.

High-frequency semicircular canal deficit affects postural control in hearing-impaired children.

OBJECTIVES: The present study aimed to analyse the impact of vestibulo-ocular reflex (VOR) gain deficit on postural control (PC) in children with sensorineural hearing loss. METHODS: A retrospective study included 55 children with sensorineural hearing loss between 4 and 17 years of age. The Video Head Impulse Test (vHIT®) was used to assess semicircular canal function. PC was assessed on the Balance Quest® posturographic platform testing sensory organisation. RESULTS: Spatial and temporal postural parameters (area and mean speed of centre of pressure oscillation, spectral power index) in the OKN-U condition (optokinetic with unstable platform) were significantly better in children with than without VOR deficit. CONCLUSION: Posturographic exploration in the OKN-U condition can screen for semicircular canal deficit in hearing-impaired children. VOR deficit also seems to predict PC deficit.

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.

Telemedicine in Audiology. Best practice recommendations from the French Society of Audiology (SFA) and the French Society of Otorhinolaryngology-Head and Neck Surgery (SFORL).

OBJECTIVES: Access to diagnosis and treatments for auditory disorders and related pathologies has regressed in France during the COVID-19 pandemic, posing a risk to the patient's chance of recovery. This best practice recommendations guide aims to list the existing technological solutions for the remote examination of a patient with hearing complaint, and to outline their benefits and, where applicable, their limitations. METHODS: The recommendations were developed both from the clinical experience of the medical experts who drafted the guide, and from an extensive review of the literature dealing with clinical practice recommendations for tele-audiology. Tele-audiometry solutions were identified on the basis of a search engine query carried out in April 2020, prior to verification of their availability on the European market. RESULTS: Video otoscopy solutions allow for the teletransmission of images compatible with a high-quality diagnosis, either by connecting via internet to a tele-health platform or using a smartphone or a tablet with an iOS or Android operating system. Using the same telecommunication methods, it is possible to remotely conduct a pure-tone audiometry test in accordance with standard practice, a speech-in-quiet or a speech-in-noise audiometry test, as well as objective measures of hearing. Clinical and paraclinical examinations can be accessed by the physician to be interpreted on a deferred basis (asynchronous tele-audiology). Examinations can also be conducted in real time in a patient, at any age of life, as long as a caregiver can be present during the installation of the transducers or the acoumetry. Tele-audiology solutions also find application in the remote training of future healthcare professionals involved in the management of deafness and hearing impairment. CONCLUSION: Under French law, tele-otoscopy is a medical procedure that is either a tele-expertise (asynchronous adive) or a teleconsultation act (synchronous advice). Subjective and objective evaluation of the patient's hearing functions can be done remotely provided that the listed precautions are respected.

Paradoxical and labile medial olivocochlear functioning as a potential marker of auditory processing disorder in a child with learning disabilities.

INTRODUCTION: The medial olivocochlear system (MOCS) is composed of fibres projecting directly onto outer hair cells and plays a role in improving the signal-to-noise ratio. The MOCS can be evaluated by measuring suppression of the otoacoustic emissions evoked by contralateral acoustic stimulation. Dyslexic children present an increased probability of auditory processing disorder (APD). These children may present paradoxical MOCS dysfunction. CASE REPORT: We report the case of a dyslexic child with APD, who was severely disabled in a noisy environment. Audiometric tests were normal, and the central auditory assessment showed labile MOCS functioning that was not only ineffective, but also potentially deleterious, possibly accounting for this child's hearing impairment in a noisy environment. DISCUSSION: This case illustrates the importance of audiological assessment and objective investigation of MOCS function in children with a learning disability, especially with hearing difficulties in the presence of noise, in whom auditory training can be beneficial.

Unilateral cochleovestibular nerve compression syndrome in a patient with bilateral IAC osteoma.

INTRODUCTION: Osteomas of the internal auditory canal are rarely reported in the literature. Patients may complain of disabling symptoms of dizziness, hearing loss, and vestibular dysfunction. We report the case of a patient with bilateral osteomas of the internal auditory canal (IAC) associated with bilateral neurovascular compression mainly affecting the right cochleovestibular nerve (VIII) and right anterior inferior cerebellar artery (AICA). OBSERVATION: This 75-year-old woman patient complained of disabling paroxysmal vertigo, typewriter tinnitus and hearing loss of the right ear. Temporal bone computed tomography showed bilateral osteoma arising from the posterior superior wall of the IAC. MRI sequences of the right VIII demonstrated compression by the right AICA against the inferior wall of the narrowed IAC. Treatment with oxcarbazepine allowed marked and lasting improvement of the patient's symptoms. CONCLUSION: To our knowledge, this is the first description of an ipsilateral neurovascular compression syndrome of the VIII secondary to the presence of an osteoma narrowing the IAC.

The temporal relationship between speech auditory brainstem responses and the acoustic pattern of the phoneme /ba/ in normal-hearing adults.

OBJECTIVE: To investigate the temporal relationship between speech auditory brainstem responses and acoustic pattern of the phoneme /ba/. METHODS: Speech elicited auditory brainstem responses (Speech ABR) to /ba/ were recorded in 23 normal-hearing subjects. Effect of stimulus intensity was assessed on Speech ABR components latencies in 11 subjects. The effect of different transducers on electromagnetic leakage was also measured. RESULTS: Speech ABR showed a reproducible onset response (OR) 6ms after stimulus onset. The frequency following response (FFR) waveform mimicked the 500Hz low pass filtered temporal waveform of phoneme /ba/ with a latency shift of 14.6ms. In addition, the OR and FFR latencies decreased with increasing stimulus intensity, with a greater rate for FFR (-1.4ms/10dB) than for OR (-0.6ms/10dB). CONCLUSIONS: A close relationship was found between the pattern of the acoustic stimulus and the FFR temporal structure. Furthermore, differences in latency behaviour suggest different generation mechanisms for FFR and OR. SIGNIFICANCE: The results provided further insight into the temporal encoding of basic speech stimulus at the brainstem level in humans.

[Tinnitus and temporomandibular joint: State of the art]. / Acouphènes et articulation temporo-mandibulaire : état des connaissances.

Tinnitus has been described in temporomandibular joint dysfunction for a long time. Yet, other disorders, such as hearing loss, stress, anxiety and depression, play a major role in the pathophysiology of tinnitus. Temporomandibular joint dysfunctions seem to increase the risk of tinnitus in patients with other predisposing factors. Especially somatosensory tinnitus, which is characterized by sound modulations with neck or mandible movements, is frequently associated with temporomandibular joint dysfunction, but it is not pathognomonic of such a disorder. In such cases, functional therapy of the temporomandibular joint should be part of the multidisciplinary rehabilitation of patients with tinnitus.

[Auditory neuropathy: clinical presentation of seven cases and review of the literature]. / Neuropathie auditive: clinique et revue de la littérature. A propos de 7 observations.

INTRODUCTION: Auditory Neuropathy (AN) is defined as a sensorineural hearing loss characterized by normal cochlear haircell function (assessed by recordable Otoacoustic Emissions) and absent or abnormal auditory brainstem evoked potentials (ABR) corroborated with absence of middle ear reflexes. PATIENTS AND METHODS: We report five cases with AN. We also report two others cases in which the presentation was different but suggestive of AN. For the majority of patients, the hearing loss had been detected during childhood. Hearing assessment of these patients included appropriate behavioral audiometric techniques (Pure Tone Audiometry - PTA, and speech audiometry), objective measures of middle ear function, acoustic reflex studies, Otoacoustic Emissions (OAE) and Auditory Brainstem Responses (ABR). RESULTS: Pure tone audiometry revealed mild-to-profound hearing loss. In patients with recordable PTA thresholds were less degraded than speech intelligibility. In all patients, tympanogram and OtoAcoustic Emissions were normal. The stapedius reflex and Auditory Brainstem Responses were absent or very degraded. CONCLUSIONS: AN can be diagnosed by the combined use of pure tone audiometry, speech audiometry, and objectives measures with the recording of OAE and ABR responses. Neonatal hearing loss OAE screening can miss babies with AN. The sooner the diagnosis is established the more successful the treatment, new opportunities being afforded by cochlear implantation.

Menière's disease: pathophysiology and treatment.

Menière's disease is defined by the association of 4 symptoms: vertigo attacks, fluctuating hearing loss, tinnitus and an auricular plenitude sensation. The pathophysiology is commonly explained by a distension of membranous labyrinth by the endolymph, equally called endolymphatic hydrops. Recent studies also tend to relate the disease to immune mechanisms. The treatment is medical in the majority of patients but there is no international consensus on the management of the different stages of Menière's disease. Regarding the lack of clinical studies clearly demonstrating the effectiveness of a certain therapy or another. the recommendations are usually based on the empirical experience of practitioners and on the observation of a marked amelioration at 2 years of treatment in the majority of patients. The treatment of the acute phase of Menière's disease is basically symptomatic. Vestibular suppressant drugs have a well-established record in controlling acute attacks of vertigo. Most have variable anticholinergic, anti-emetic and vestibular sedative effects. If necessary, the administration of benzodiazepines will help to alleviate anxiety. Long term management of Menière's disease includes a low salt diet, the use of diuretics in the post-crisis phase, and the very common use in Europe of histaminergic agents. Corticosteroids are used in bilateral forms of Menière's disease, particularly if an autoimmune basis is suspected. All authors insist on the interest and the importance of regular follow-up, especially with regard to the psychological status and responsiveness to treatment of the patient. Surgical indications are rare and the least invasive procedures are used first. The choice of the procedure should take into consideration the need to preserve the auditory function of the patient.

Relationship between NRT measurements and behavioral levels in children with the Nucleus 24 cochlear implant may change over time: preliminary report.

OBJECTIVE: Response from spiral ganglion cells to electrical stimulation via the Nucleus 24 cochlear implant can be measured using the neural response telemetry system. The purpose of this study was to assess, in children, the correlation between the neural response threshold and the behavioral levels used for cochlear implant programming process. METHODS: The neural response telemetry test was administered to 23 children (mean age at implantation: 4 years) with the Nucleus 24 cochlear implant. Four intra-cochlear electrodes (electrodes 5, 10, 15 and 20) were tested. The neural response threshold at 3, 6, 9 and 12 months post-implantation was compared with the behavioral threshold and the maximum comfort level estimated during the same periods: a Pearson's correlation test was performed for each tested electrode. RESULTS: On apical electrodes, the correlation with the behavioral threshold remained significant from 3 to 12 months post-implantation (r ranging from 0.696 to 0.909, P<0.05), and the correlation with the maximum comfort level was also significant throughout the study period, except on electrode 15 at 9 months (tendency to significance). On basal and intermediate electrodes, statistical correlations were found only at some points of time; nonetheless, at 12 months post-implantation, a significant correlation with behavioral levels could be clearly demonstrated both on electrode 15 (r=0.914--0.778, P<0.05) and on electrode 10 (r=0.845--0.720, P<0.05). CONCLUSIONS: This preliminary study suggests that the correlation between the neural response threshold and behavioral levels may improve from the base towards the apex of the cochlea. However, a significant correlation can be demonstrated for all tested electrodes at 12 months post-implantation. During the first months post-implantation care must be exercised when interpreting neural response telemetry measurements: a positive test does not necessarily mean that the stimulus delivered to the acoustic nerve will be centrally processed with the result of an auditory perception.

[Binaural cochlear implantation: general concept, surgical technique and electrophysiology]. / Implantation cochléaire binaurale.

Cochlear implant have been proven to be safe and cost-effective in deafened adults. Generally, a single cochlear implants is proposed providing monaural audition. Some teams have published interesting results in patients wearing two cochlear implants. The cost of the surgical procedure is greatly increased for bilateral implantation. In an attempt to provide patients with binaural hearing and a better chance to improve their capacity for frequency discrimination without an excessive increase in cost, we developed an new concept and design for binaural cochlear implants. The aim of the present study was to establish the surgical technique for binaural cochlear implantation with a single internal receptor-stimulator. We report descriptive data on surgical procedures performed in two cadavers and in two deafened adults. The special implant is composed of a single receptor-stimulator and two electrode arrays: a short one implanted into the ipsilateral ear and a long one in the contralateral ear. A specially designed tool for endoscopic facial lifting was used to create subcutaneous tunnels from the ipsilateral to the contralateral ear. Insertion into cochleas was unremarkable. Additional time for surgery (compared with mean operative time for routine adult surgery in our unit), and surgical details are discussed. Binaural cochlear implantation is feasible, easy and not very time consuming. Studies are in progress to determine patient benefit. Obtaining auditory brainstem evoked potentials electrically in patients with binaural cochlear implants is possible and provides an interesting mode for in vivo analysis of the long-term effect of profound/total deafness on neural transmission. In our patients, results have demonstrated that neural response properties in auditory pathways are negatively related to duration of deafness.