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.

The French Cochlear Implant Registry (EPIIC): Perception and language results in infants with cochlear implantation under the age of 24 months.

OBJECTIVE: Multi-centre study of the National French Registry (EPIIC) of patients with cochlear implants, focusing on infants who were operated-on under the age of 24 months between 2012 and 2016. PATIENTS AND METHODS: A total of 615 profoundly deaf infants, who received cochlear implants (CIs) before their second birthday, were included in the registry by different CI centers. Epidemiological, surgical, speech therapy and school, follow-up data were included in the registry, 12, 24, 36 and 48 months thereafter. The following parameters were studied: type of implantation (uni- or bilateral), complications, cause of deafness, category of auditory perception (CAP), Open-set word recognition score (OSW), speech intelligibility rating, lexical comprehension with EVIP (Peabody), communication mode and type of schooling. Bilateral simultaneous CI (BiCI) and unilateral CI (UniCI) groups were compared. RESULTS: There were 744 implantations. The explantation-reimplantation rate, within the four-year follow-up, was just 3.6%. Mean implantation age was 16.0 months, and similar in the two groups (BiCI/UniCI). A total of 51% of children had their first implant between 12 and 18 months, and 15% before 12 months. Implantation was unilateral in 52% of cases. Fifty-six percent of the bilateral procedures were sequential, with a mean delay of 16.8 months for the second implantation. The cause of deafness was unknown in 52% of cases. Of the 48% (297/615) of attributed cases, 32% had clear genetic causes. The remaining deafness was due to cytomegalovirus (CMV, 8%), inner-ear malformation (5%) and meningitis (3%). The main complications were from infections (47%) and internal device failure (25%). Four years post-operation, 84% of the UniCI and 75% of BiCl groups had a CAP≥5, and 83% of UniCl and 100% BiCI had OSW≥80%. Furthermore 74% of UniCI and 77% of BiCI communicated orally and 85% of UniCI and 90% of BiCI integrated into mainstream schooling. CONCLUSION: The French Registry of cochlear implants (EPIIC) is the only such national registry in the world. Our analysis illustrates the immediate benefits of, either single or double, cochlear implantation for language, perception skills and schooling.

The French Cochlear Implant Registry (EPIIC): Cochlear implant candidacy assessment of off-label indications.

OBJECTIVE: Evaluate in France the outcomes of cochlear implantation outside the selection criteria, off-label. MATERIAL AND METHODS: This is a prospective cohort study including adults and children having received a cochlear implant (CI) in an off-label indication, that is outside the criteria established by the "Haute Autorité de santé (HAS)" in 2012. The data was collected from the "EPIIC" registry on recipients who received CIs in France between 2011 and 2014. Speech audiometry was performed at 60dB preimplantation and after one year of CI use, as well as an evaluation of the scores of the quality of life with the APHAB questionnaire, the scores for CAP and the professional/academic status in pre- and post-implantation conditions. Major and minor complications at surgery have been recorded. RESULTS: In total, 590 patients (447 adults and 143 children) with an off-label indication for CIs were included in this study from the EPIIC registry (11.7% of the whole cohort of EPIIC). For adults, the median percentage of comprehension using monosyllabic word lists was 41% in preimplantation condition versus 53% after one year of CI use (P<0.001) and 60% versus 71% in dissyllabic word lists (P<0.001). The CAP scores were 5 versus 6 in pre- and post-implantation conditions respectively (P<0.001) and the APHAB scores were statistically lower after implantation (P<0.001). In the children cohort, the median percentage of comprehension using monosyllabic word lists was 51% in preimplantation condition and 65% after CI (P<0.001), and 48% versus 82% (P<0.001) for dissyllabic word lists. The CAP scores were 5 versus 7 respectively in pre- and post-CI conditions (P<0.001). Thirty-two minor complications (5.4%) and 17 major complications (2.8%) were reported in our panel of off-label indication patients. CONCLUSION: These results suggest that a revision of the cochlear implantation candidacy criteria is necessary to allow more patients with severe or asymmetric hearing loss to benefit from a CI when there is an impact on quality of life despite the use of an optimal hearing aid.

The French National Cochlear Implant Registry (EPIIC): Cochlear explantation and reimplantation.

This study aims to determine the frequency and causes of cochlear explants with re-implantation (ERI) after 5 years' follow up of the patients included in the French national EPIIC (étude post-inscription des implants cochléaires) registry tracking patients with cochlear implantation. This multicenter, descriptive prospective study was conducted on 5051 patients enrolled in the EPIIC database between January 2012 and December 2016. Ninety-five patients (1.9%) received a primary implant and an ERI during the study. Of these, four benefitted from two ERIs. The number of ERIs was significantly higher in the pediatric population than among adults. The explantation and reimplantation were performed simultaneously in 86% of cases. The reasons for explantation were: in 46.4% of cases linked to a malfunction of the implant, and in 39.3% of cases for medical or surgical reasons. The number of electrodes inserted was significantly higher after the ERI than after the first implantation. There was just one post-ERI infection for these 95 explanted and re-implanted patients. As well as explantation with reimplantation rarely being necessary, it generally presents no major surgical difficulty and in most cases it allows a better integration than in the first implantation.

The French Cochlear Implant Registry (EPIIC): Cochlear implantation complications.

OBJECTIVES: The aim of this study was to evaluate peri- and post-operative complications related to cochlear implantations. We searched for risk factors predicting these complications and analyzed the complications in the youngest and most elderly. STUDY DESIGN: Retrospective analysis of cochlear implant patients. MATERIALS AND METHODS: All patients who underwent cochlear implantation in France between January 2012 and December 2016 were anonymized and registered in the EPIIC database. This population included 3483 adults and 2245 children. Their demographic and surgical data and their incidence of peri- or post-operative complications, including their severity, whether major or minor, were all indicated. RESULTS: The global complication rate was 6.84%. The risk of complication was higher in initial implantation versus reimplantation (P<0.0001). The risk was also higher for bilateral implantation versus unilateral (P<0.0001). Complications were more frequent for patients with cochlear malformation (P=0.002). There was no difference in complication rates across age groups; babies under 1 year old, and the elderly over 80 and even over 90, did not have more complications than the rest of the population. Patients treated in the daily care unit had no more complications than those who were hospitalized for one night or more (P=0.64). CONCLUSION: Cochlear implantation is a safe technique with a low incidence of complications. The absence of increased risk in patients at the extremes of the age spectrum justifies offering this solution to all, without age limitation.

The French National Cochlear Implant Registry (EPIIC): Bilateral cochlear implantation.

OBJECTIVES: Assessment of the incidence and results of bilateral cochlear implantation in adults and children in France. MATERIALS AND METHODS: Multicenter retrospective study of data in the French national registry of cochlear implantations from January 1st 2012 to December 31st 2016. Functional results from CAP (Category of Auditory Performance) questionnaires and speech audiometry tests, with mono- and di-syllabic word-lists, were compared before and after implantation. Speech audiometry tests were carried out against a noisy background, except before simultaneous implantations. RESULTS: Nine hundred and forty two bilateral cochlear implantations were performed during this period, that is, 16.4% of all cochlear implantations. Five hundred and eighty eight bilateral implantations were performed sequentially. 59% of the bilateral implantations were performed in children. Bilateral implants significantly improved CAP scores in all cases (P<0.001). Auditory performance, with the two types of word-list, were significantly improved after simultaneous implantation (P<0.01). After sequential implantation, the speech discrimination score, already very good with the first implant, reached 63±26% [0-100] with monosyllabic word lists, and 72±28% [0-100] with dissyllabic words. There were more complications due to surgery in bilateral cases than in the entire population of cochlear recipients (9.1% vs 6.4%, P<0.02). CONCLUSION: Hearing is significantly improved by simultaneous cochlear implantation. For sequential implantation, at one year, when auditory results were already excellent from the first implant, in the bimodal condition CAP scores were significantly improved, although there was no further change in speech audiometry in noise.

Consensus statement. Corticosteroid therapy in ENT in the context of the COVID-19 pandemic.

This consensus statement about the indications and modalities of corticosteroid treatment in the context of the COVID-19 pandemic was jointly written by experts from the French Association of Otology and Oto-Neurology (AFON) and from the French Society of Otorhinolaryngology, Head and Neck Surgery (SFORL). There is currently not enough data in favour of danger or benefit from corticosteroids in COVID-19, so until this matter is resolved it is advisable to limit their indications to the most serious clinical pictures for which it is well established that this type of treatment has a positive impact on the progression of symptoms. In Grade V and VI Bell's palsy according to the House-Brackmann grading system, a week's course of oral corticosteroids is recommended. Corticosteroid therapy is also recommended in cases of sudden hearing loss of more than 60dB, either in the form of intratympanic injections or a week's course of oral medication. In rhinology, there is no indication for systemic corticosteroid therapy in the current situation. However, patients are advised to continue with their local corticosteroid therapy in the form of a nasal spray or by inhalation. Treatments with corticosteroid nasal sprays can still be prescribed if there is no alternative. Finally, systemic or local corticosteroid therapy is not indicated for bacterial ENT infections.

Guidelines (short version) of the French Society of Otorhinolaryngology (SFORL) on pediatric cochlear implant indications.

OBJECTIVES: The authors present the guidelines of the French Society of Otorhinolaryngology - Head and Neck Surgery (Société française d'oto-rhino-laryngologie et de chirurgie de la face et du cou - SFORL) on the indications for cochlear implantation in children. METHODS: A multidisciplinary work group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. They were then read over by an editorial group independent of the work group. The guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence. RESULTS: The SFORL recommends that children with bilateral severe/profound hearing loss be offered bilateral cochlear implantation, with surgery before 12months of age. In sequential bilateral cochlear implantation in children with severe/profound hearing loss, it is recommended to reduce the interval between the two implants, preferably to less than 18months. The SFORL recommends encouraging children with unilateral cochlear implants to wear contralateral hearing aids when residual hearing is present, and recommends assessing perception with hearing-in-noise tests. It is recommended that the surgical technique should try to preserve the residual functional structures of the inner ear as much as possible.

French Society of ENT (SFORL) guidelines. Indications for cochlear implantation in adults.

The authors present the guidelines of the French Society of ENT and Head and Neck Surgery (SFORL) regarding indications for cochlear implantation in adults. After a literature review by a multidisciplinary workgroup, guidelines were drawn up based on retrieved articles and group-members' experience, then read over by an independent reading group to edit the final version. Guidelines were graded A, B, C or "expert opinion" according to decreasing level of evidence. There is no upper age limit to cochlear implantation in the absence of proven dementia and if autonomy is at least partial. Bilateral implantation may be proposed if unilateral implantation fails to provide sufficiently good spatial localization, speech perception in noise and quality of life, and should be preceded by binaural hearing assessment. Rehabilitation by acoustic and electrical stimulation may be proposed when low-frequency hearing persists. Quality of life should be assessed before and after implantation.

Pediatric cochlear reimplantation: Decision-tree efficacy.

OBJECTIVES: The context leading to pediatric cochlear reimplantation (CreI) can be complex. The objectives of this study were to define initial CreI indications, analyze final diagnosis and draw up a decision-tree. METHODS: A retrospective study included patients undergoing CreI between 2005 and 2015. Demographic characteristics, CreI circumstances and technical reports were collected. Circumstances indicating CreI were classified in 3 groups: performance decrement, suspected device failure, or medical. After CreI, final diagnoses were classified in 2 groups: confirmed failure (DFail) or medical (DMed). RESULTS: 69 out of 734 cochlear implantation surgeries were for CreI (8%). Manufacturers' reports were available in 64 cases (93%). Two principal causes were found: trauma and infection. Initial indications were: performance decrement: 27%; device failure: 56%; and medical: 17%. Final diagnoses were: DFail: 72%; and DMed: 28%. Initial indication and final diagnosis were similar in 86% of cases. The majority of the 14% initial indication errors belonged to the "performance decrement" group. Traumatic causes correlated with risk of initial indication error (P=0.039). CONCLUSION: Apart from spontaneous device failure, the two causes of CreI were infection and trauma. Using the present decision algorithm, half of the complex cases were resolved after CreI.

Atypical failure after cochlear implantation in children.

We report a case of intermittent dysfunction in a 10-years-old boy, implanted with MedEL(®) cochlear implant. Few weeks after the surgery the boy described short and intermittent episodes of implant dysfunction with rapid return to a normal function. No evidence for any electric or neural dysfunction was found. After few weeks, a clinical link was discovered to episodes of sneeze or nose blowing. Clinical and surgical implications are discussed.

Biallelic nonsense mutations in the otogelin-like gene (OTOGL) in a child affected by mild to moderate hearing impairment.

Hearing impairment is characterized by great genetic heterogeneity. We report the identification, by whole exome sequencing, of two different nonsense mutations (c.1558C>T; p.Gln520 and c.2773C>T; p.Arg925) in the otogelin-like gene (OTOGL), in a child affected by mild to moderate isolated deafness. Parental genotypes allowed us to conclude that these mutations are present in the compound heterozygous state in the patient. In addition, our clinical data establish that the tectorial membrane and/or the outer hair cells are defective in this form of deafness.

Cochlear implant in the carotid canal. Case report and literature review.

BACKGROUND: Numerous complications have been described following cochlear implantation. Most of them are post-operative and benign, but some are intra-operative and can be more difficult to manage. METHODS: Case report of a pediatric case of misplaced cochlear electrode in the carotid canal and literature review. RESULTS AND CONCLUSIONS: Post-operative CT-scan allowed the diagnosis. The misplaced cochlear implant in the carotid canal was successfully removed and a successful re-implantation followed immediately. Anatomy of the interval between the cochlea and the carotid canal is reviewed, together with information regarding the neural telemetry response. In each case, specific anatomical landmarks must be identified to perform the cochleostomy in the right position. If not, or if surgery proves itself difficult, the surgeon should intra-operatively control the position of the electrode.

Phenotype and genotype in females with POU3F4 mutations.

X-linked deafness is a rare cause of hereditary isolated hearing impairment estimated as at least 1% or 2% of the non-syndromic hearing loss. To date, four loci for DFN have been identified and only one gene, POU3F4 responsible for DFN3, has been cloned. In males, DFN3 is characterized by a progressive deafness associated with perilymphatic gusher at stapes surgery and with a characteristic inner ear malformation. The phenotype of eight independent females carrying POU3F4 anomalies is defined, and a late-onset hearing loss is found in three patients. Only one has an inner ear malformation. No genotype/phenotype correlation is identified.

Cochlear implantation in children with internal ear malformations.

OBJECTIVE: To evaluate surgical aspects and results of cochlear implantation in inner ear malformations. STUDY DESIGN: Retrospective cohort study. SETTING: Ear, nose, and throat department of a tertiary referral hospital. PATIENTS: Out of 260 implanted children, 18 (6.9%) had inner ear malformations: complex cochleovestibular malformation (n = 11), common cavity (n = 1), and enlarged vestibular aqueduct (EVA) (n = 6). Deafness was progressive in 12 cases (G1) and congenital in 6 cases (G2). Genetics lead to diagnosis in 12 of 13 cases: PSD mutation (n = 11), Waardenburg syndrome (n = 1), negative (1). Mean age at implant was 7.8 years. Mean follow-up period was 48 months. MAIN OUTCOME MEASURES: Medical and surgical outcomes were reported. Closed (CSW) and open (OSW) set word perception and level of speech production were evaluated each year. The results were compared pre- and postoperatively and between the two groups. RESULTS: Gusher at surgery was observed in 50% of cases, with a persistent leak in one case. No facial injury or infectious complications were observed. At 12 months, 83% of the population had achieved more than 75% recognition in CSW, versus 16% before implant (p = 0.001). After 2 years, 64% of patients had more than 50% recognition in OSW. Good oral language was seen in 76% at 2 years and 100% at 3 years, versus 55% before implant (respectively, p > 0.05 and p = 0.03). At 1 year after implant, 83% of the G1 and 20% of the G2 achieved more than 50% recognition in OSW (p = 0.02). After 24 months, 83% of G1 and 40% of G2 had more than 50% in OSW (p > 0.05). Before implant, 75% in G1 and 0% in G2 had good oral language (p = 0.01). At 1 year, 83% in G1 and 16% in G2 had good oral language (p = 0.02). At 2 years, 100% in G1 and 20% in G2 had good oral language (p = 0.02). One child in G1 had no improvement after implantation. CONCLUSIONS: No major complication was seen. Perceptive and linguistic results were variable and depended on the type of the deafness. In progressive deafness, the perceptive and linguistic result are expected to be good. In congenital deafness, the results are more variable.

Screening of SLC26A4 (PDS) gene in Pendred's syndrome: a large spectrum of mutations in France and phenotypic heterogeneity.

Sensorineural hearing defect and goiter are common features of Pendred's syndrome. The clinical diagnosis of Pendred's syndrome remains difficult because of the lack of sensitivity and specificity of the thyroid signs. The identification of PDS as the causative gene allowed molecular screening and enabled a re-evaluation of the syndrome to identify potential diagnostic characteristics. This report presents the clinical and genotypic findings of 30 French families, for whom a diagnosis of Pendred's syndrome had been made. Twenty-seven families had at least one mutated allele. Twenty-eight different mutations were identified, 11 of which had never been previously reported. The main clinical characteristics were: early hearing loss, fluctuation in terms of during deafness evolution, and the presence of an enlarged vestibular aqueduct.

Evolution of the bacteriologic features of persistent acute otitis media compared with acute otitis media: a 15-year study.

OBJECTIVES: To define the epidemiologic features of persistent acute otitis media (PAOM) and modifications of these features during the past 15 years and to investigate for possible differences in bacterial resistance between acute otitis media (AOM) and PAOM. DESIGN: Retrospective patient series. SETTING: Academic tertiary care center. PATIENTS AND METHODS: Persistent acute otitis media was defined as AOM lasting longer than 3 weeks despite 1 or several courses of antibiotic therapy, with the persistence of clinical and otoscopic signs of AOM. From 1982 to 1997, 475 children with PAOM were seen in our department. Every patient had 1 or several specimens of aspirations or swabs of spontaneous otorrhea (or both). Microbiologic characteristics of the isolated strains (including antibiotic susceptibility) were analyzed. Four successive series of specimens were analyzed-group 1: from October 1, 1982, to June 30, 1986 (136 patients); group 2: from January 1, 1987, to December 31, 1989 (165 patients); group 3: from January 1, 1992, to April 30, 1993 (73 patients); and group 4: from January 1, 1994, to January 31, 1997 (101 patients). During the same study periods, the bacteriologic results of patients with AOM in the same geographic region were recorded. MAIN OUTCOME MEASURES: A longitudinal comparison between the groups of patients with PAOM and a cross-comparison within each group between patients with PAOM and those with AOM. RESULTS: Obtaining repeated and multiple specimens from patients with PAOM led to a progressive decrease in the rate of sterile specimens, from 35.3% (group 1, 48 patients) to 14.9% (group 4, 15 patients) (P<.01). During this period, the prevalence of Streptococcus pneumoniae increased in patients with positive culture results, from 18.2% (group 1, 16 of 88 patients) to 44.2% (group 4, 38 of 86 patients) (P<.001). These strains rapidly and dramatically became resistant to penicillin (amoxicillin) (0% through 1989, 76.2% [16 of 21 patients] in 1993, and 97.4% [37 of 38 patients] in 1996) (P = .01). The overall prevalence of Haemophilus influenzae remained stable (between 31.4% [27 of 86 patients] and 45.4% [40 of 88 patients]), but the proportion of beta-lactamase-producing strains increased from 30.0% (group 1, 12 patients) to 55.6% (group 4, 15 patients) (P=.04). The prevalences of Pseudomonas aeruginosa and Staphylococcus aureus did not vary significantly (from 23.1% [group 2, 30 patients] to 10.7% [group 3, 6 patients] and from 10.2% [group 1, 9 patients] to 4.6% [group 4, 4 patients], respectively). Comparing data from patients with PAOM with those with AOM revealed that the increased resistance of H influenzae and, in particular, of S pneumoniae was more rapid and more marked in patients with PAOM than in those with AOM (highest rate of resistance in AOM: 36.0% [271 of 753 specimens] and 50.6% [398 of 787 specimens] for H influenzae and S pneumoniae, respectively; P<.001 for S pneumoniae). CONCLUSIONS: The increase in bacterial resistance frequently encountered during otitis media is even more marked in patients with PAOM. The identification of the organism is essential when the otitis does not resolve, especially in patients with PAOM. Obtaining repeated specimens helps to decrease the rate of sterile cultures.

Head and neck infantile myofibromatosis--a report of three cases.

Infantile myofibromatosis (IM) is a proliferative disorder characterized by the development of single or multiple nodular lesions arising from cutaneous, subcutaneous, muscular, bone or visceral structures. This proliferation may occur at any anatomical site, but in 30% of the cases it involves the head and neck. We report here three cases of head and neck IM occurring in young children and presenting as solitary lesions. The clinical heterogeneity and the misleading histopathological appearances can make the diagnosis difficult. The treatment is surgical but the low rate of recurrence and the possibility of spontaneous tumoral regression may lead to conservative surgery or therapeutic abstention.

[Neuroglial heterotopia. Apropos of 8 cases with non-nasofrontal sites]. / Les hétérotopies neurogliales. A propos de 8 cas de localisations non naso-frontales.

Neuroglial heterotopia, glioma, is an uncommon congenital nervous tissue tumor, usually found in a nasofrontal localization. About 60 cases with an atypical localization, usually in the pterygomaxillary fossa have been reported. We present 8 cases. Symptomatology followed CT-scan or MRI identified localization and was helpful in orienting diagnosis before biopsy. Exeresis was difficult, particularly in deep infratemporal localizations reaching the base of the skull. The main risk is recurrence. Different surgical approaches have been discussed and should be adapted to each case.