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).

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.

[Management of vertigo and dizziness]. / Vertiges et troubles de l'équilibre : démarche diagnostique.

Balance disorders presenting with symptoms of dizziness and vertigo are due to various diseases. Clinical approach gives the opportunity to identify emergency situations and most common causes, among them the first one being the benign paroxysmal positional vertigo. Oculomotor assessment is pertinent as major clinical orientation, particularly between peripheral and central diseases. These clinical findings support the respective indication of modern imaging and/or vestibular tests, focused on the direction of presupposed diagnosis. On elderly the risk of falls and their complications needs a specific evaluation.

Evaluation of the impact of hearing loss in adults: Validation of a quality of life questionnaire.

OBJECTIVES: The impact of hearing loss and of auditory rehabilitation (hearing aid, cochlear implant) on quality of life is a crucial issue. Commonly used questionnaires to assess quality of life in these patients (Nijmegen, APHAB, GBI) are time-consuming, difficult for patients to fill out, and show poor sensitivity to small improvements or deterioration. The objective of the present study was to validate a dedicated quality of life scale for hearing-impaired adults with or without auditory rehabilitation. MATERIAL AND METHODS: ERSA (Évaluation du Retentissement de la Surdité chez l'Adulte: Evaluation of the Impact of Hearing Loss in Adults) is a self-administered questionnaire. It is divided into 4 domains, each comprising 5 questions graded from 1 to 10. The questions are simple, and formulated so patients will answer according to how they feel at the actual time of the session. Test-retest reliability was measured in 38 patients. Internal coherence and validity against the APHAB questionnaire as gold standard and in relation to hearing performance were measured in 122 patients at auditory assessment. Sensitivity to change in hearing was measured in 36 cochlear implant patients, before and 6 or 12 months after implantation. RESULTS: Test-retest reliability was very satisfactory (ρ=0.88). Internal coherence was good for all questions. External validity, comparing ERSA to APHAB scores in the same non-implanted hearing-impaired patients, was good (ρ=0.52). Additionally, ERSA scores correlated with hearing performance in adverse conditions (monosyllabic words: ρ=0.22; sentences in noise: ρ=0.19). In patients tested before and after cochlear implantation, improvement in hearing performance in silence and in noise correlated with an improvement in ERSA score (ρ=0.37 to 0.59, depending on the test), but not to GBI score. CONCLUSION: The ERSA questionnaire is easy and quick to use, reliable, and sensitive to change in hearing performance after cochlear implantation.

French Society of ENT (SFORL) guidelines (short version): Audiometry in adults and children.

INTRODUCTION: French Society of ENT (SFORL) good practice guidelines for audiometric examination in adults and children. METHODS: A multidisciplinary working group performed a review of the scientific literature. Guidelines were drawn up, reviewed by an independent reading group, and finalized in a consensus meeting. RESULTS: Audiometry should be performed in an acoustically controlled environment (<30dBA); audiometer calibration should be regularly checked; and patient-specific masking rules should be systematically applied. It should be ensured that masking is not overmasking. Adult pure-tone audiometry data should be interpreted taking account of clinical data, speech audiometry and impedancemetry. In case of discrepancies between clinical and pure-tone and speech audiometry data, objective auditory tests should be perform. In children aged 2 years or younger, subjective audiometry should be associated to behavioral audiometry adapted to the child's age. In suspected hearing impairment, behavioral audiometry should be systematically supplemented by objective hearing tests to determine and confirm the level and type of hearing impairment.

[Neurofibromatosis type 2]. / La neurofibromatose de type 2.

INTRODUCTION: Neurofibromatosis 2 (NF2) is a rare autosomal dominant disease whose hallmark is the development of bilateral vestibular schwannomas. STATE OF THE ART: Other features of NF2 include schwannomas, meningiomas, ependymomas, localized along the central nervous system, schwannomas of the peripheral nerves, cutaneous and ophthalmological manifestations. NF2 can be diagnosed in patients without bilateral vestibular schwannoma with sets of diagnostic criteria. Disease phenotype is variable among patients. Main negative prognostic factors are a young age at onset of symptoms and a high number of tumors at diagnosis. NF2 tumor suppressor gene encodes Merlin/Schwannomin, and is also involved in most sporadic schwannomas and meningiomas. Its functions remains largely unknown. PERSPECTIVES AND CONCLUSIONS: Treatment and follow of NF2 patients up require oto-neurosurgical teams experienced in NF2. Yearly and life time surveillance is mandatory. A clinical screening protocol is suggested. Classically, only symptomatic lesions are to be treated. Some authors advocate an early proactive strategy against vestibular schwannoma in order to preserve hearing. When a treatment is advisable, surgery remains the treatment of choice for tumors. Auditory brainstem implant must be taken into account in hearing rehabilitation.

[Auditory brainstem implant: indications and results]. / Implant auditif du tronc cérébral: indications et résultats.

OBJECTIVES: To summarize the indications and evaluate the Auditory Brainstem Implant (ABI) performances in neurofibromatosis type 2 (NF2) and other otologics indications, as postmeningitis ossified cochlea. MATERIAL AND METHODS: Main and first indication of ABI is NF2. Emergent indications are bilateral total ossified cochlea, vestibular schwannoma with controlateral lesions, cochlear nerve aplasia or inner ear's malformations. The pre-operative evaluation includes clinical, radiological, lipreading, and psychological status. A translabyrinthine or retrosigmoid approach is performed, depending on tumoral or not tumoral status. The auditory perception with the ABI is evaluated by testing, the words recognition in open-set lists, and the speech understanding with usual sentences. RESULTS: In NF2 patients, best results are obtained in cases of smaller vestibular schwannoma and none, or short term, auditory deprivation. Negative prognostic factors are duration of total hearing loss (>10 years), tumor size (>30 mm), difficulties in electrode array placement, complications during post-operative course and number of active electrodes (<10). In cases of postmeningitis total deafness with totally ossified cochlea, results demonstrate a good benefit reaching these obtained with cochlear implant in post-meningitis deafness. CONCLUSION: These results show a clear benefit of ABI in NF2 patients, with or without previous tumor removal, in case of small tumor with a short duration of hearing loss. In case of postmeningitis ossified cochlea, results potentially reach those of cochlear implants.

[Osteomyelitis of the skull base due to otologic or sinus infections. 5 cases]. / Ostéomyélite de la base du crâne d'origine otosinusienne. Etude d'une série de cinq cas récents.

INTRODUCTION: Skull base osteomyelitis is an uncommon but severe condition generally secondary to necrotizing otitis externa. The aim of this study was to determine the patients demographics, clinical and radiological findings, and outcomes of this condition. MATERIAL AND METHODS: We prospectively follow 5 patients with a diagnosis of skull base osteomyelitis between 2004 and 2005. Clinical, biologic and imaging data were collected, and also follow-up during treatment. RESULTS: The five patients were men and the average age at presentation was 75 years (63-89). All except one had a previous diabetes mellitus, with a more or less recent clinical story of otitis externa. All of them suffered of headaches and cranial nerve(s) deficits, particularly facial nerve. Diagnosis was made on MRI with gadolinium injection and fat saturation, and Pseudomonas aeruginosa identified as pathogen in all cases. One patient deceased of cranial nerves palsy complication. In 4 cases we found previously a too short or non adapted treatment of necrotizing otitis externa. CONCLUSIONS: In the setting of headache, cranial nerves deficit and abnormal skull base imaging on MRI with Fat sat, osteomyelitis should be consider as the likely diagnosis. This complication should be avoided by optimal management of necrotizing otitis externa.

[Middle ear overpressure with Meniett in Menière disease: indications, results at short and middle terms in 53 cases]. / Traitement pressionnel par Meniett au cours de la Maladie de Menière. Indications, tolérance et résultats à court et moyen termes dans 53 cas.

OBJECTIVES: To expose the results obtained at short and middle terms and the tolerance of over pressure treatment with Meniett in Menière disease. MATERIALS AND METHODS: A pressure treatment using Meniett device was initiated for 53 patients suffering of unilateral or bilateral active Menière's disease, despite medical treatment and physiotherapy. RESULTS: Retrospective analysis of the data demonstrate i) no complications due to the tympanostomy tube, ii) with a 4 months follow up after the treatment, a total control or reduction of vertigo in respectively 65% and 24.5% of the initially treated patients. CONCLUSION: The overpressure treatment with Meniett has been well tolerated. It can be repeated in case of recurrence of the symptoms. It is an interesting alternative for the treatment of Menière disease.

Diagnostic and therapeutic strategy in Menière's disease. Guidelines of the French Otorhinolaryngology-Head and Neck Surgery Society (SFORL).

OBJECTIVES: The authors present the guidelines of the French Otorhinolaryngology-Head and Neck Surgery Society (Société française d'oto-rhino-laryngologie et de chirurgie de la face et du cou: SFORL) for diagnostic and therapeutic strategy in Menière's disease. METHODS: A work group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, then read over by an editorial group independent of the work group. The guidelines were graded according to the literature analysis and recommendations grading guide published by the French National Agency for Accreditation and Evaluation in Health (January 2000). RESULTS: Menière's disease is diagnosed in the presence of the association of four classical clinical items and after eliminating differential diagnoses on MRI. In case of partial presentation, objective audiovestibular tests are recommended. Therapy comprises medical treatment and surgery, either conservative or sacrificing vestibular function. Medical treatment is based on lifestyle improvement, betahistine, diuretics or transtympanic injection of corticosteroids or gentamicin. The main surgical treatments, in order of increasing aggressiveness, are endolymphatic sac surgery, vestibular neurotomy and labyrinthectomy.

[Performances and complications of cochlear implant in 134 adult patients implanted since 1990]. / Performances et complications de l'implant cochléaire chez 134 patients adultes implantés depuis 1990.

OBJECTIVE: The aim of this study was to analyse performance of cochlear implants in a retrospective series of adults with postlingually in order to search for predictive factors and identify complications. METHODS: Between 1990 and 2003, 134 adult patients were implanted: 129 patients on one side and 5 patients on both sides. Hearing benefit at 6, 12, 24 and 36 months and performance at 12 months were analyzed as function of the etiology, age at implantation, lipreading and speech coding strategy. RESULTS: Compared to performances before implantation, cochlear implants provided significant and rapid improvement at 6 months (p<0.0001). The results were not correlated with age at implantation, etiology or lipreading ability before implantation. Performances were worse in patients with meningitis (p<0.01), but the percentage of improvement was similar between the different etiologies. Major complications were rare: one case of regressive postoperative facial nerve weakness. CONCLUSION: Cochlear implants provide significant and rapid improvement, with low morbidity. Results are not correlated with age at implantation, etiology or lipreading ability.

Benefit of contralateral hearing aid in adult cochlear implant bearers.

OBJECTIVES: The present study assessed the interest of a contralateral hearing aid (HA) in adult cochlear implant (CI) bearers. MATERIAL AND METHODS: The study recruited 10 French-speaking adult HA bearers with postlingual bilateral hearing loss, fitted for at least 2 years with a unilateral CI after loss of benefit from HA in one ear but continuing to use their contralateral HA: 4 male, 6 female; mean age, 58 years. All had regularly used bilateral HAs prior to CI. Audiometric assessment comprised: (1) individual ear hearing assessment on pure-tone audiometry and speech discrimination; and (2) free-field testing without aid, with CI only, with HA only and with CI plus HA, on pure-tone audiometry and speech discrimination with quiet background and on speech discrimination in noise. RESULTS: Speech discrimination was significantly improved in the bimodal condition (CI plus HA) as compared to CI alone, on all tests. In quiet, discrimination for disyllabic words was>50% in 7 cases with HA alone, in 2 cases with CI alone and in 1 case in with HA+CI. Under 0dB signal-to-noise ratio, discrimination was>50% in 1 case with HA alone, in 3 cases with CI alone and in 6 cases with HA+CI. CONCLUSION: The present results showed benefit in auditory perception in quiet and in noise with bimodal stimulation. When there is residual hearing in the non-implanted ear, a HA should be fitted; and in progressive bilateral hearing loss, CI should be suggested when HA benefit decreases in one ear.

[Labyrinthine hemorrhage caused by anticoagulant therapy]. / Hémorragie labyrinthique liée aux anticoagulants.

OBJECTIVE: To report a case of labyrinthine hemorrhage with a sudden sensorineural hearing loss and vertigo. This is a rare mechanism of inner ear damage, which may be considered in patients with anticoagulant therapy. MATERIAL AND METHODS: We report a case of sudden sensorineural hearing loss and vertigo in a 73-year-old male on anticoagulant therapy for arrhythmia. An audiovestibular and imaging work-up was performed. RESULTS: Audiometry showed a left profound hearing loss. Videonystagmography demonstrated a left labyrinthine deficit. Hemostasis assessment showed an INR at 7.5 MR imaging evidenced a high intralabyrinthine signal on T1-weighted images without gadolinium and on FLAIR T2-weighted images, suggesting labyrinthine hemorrhage. At clinical follow-up the vertigo regressed but the hearing function was not recovered. CONCLUSION: Labyrinthine hemorrhage should be evoked in the event of sudden sensorineural hearing loss in patients with anticoagulant therapy. MRI is a useful diagnostic tool in this case.

[Vestibular dysfunction after cochlear implantation: a national multicenter clinical study]. / Vertiges et troubles de l'equilibre après implantation cochléaire. Analyse préliminaire des données du PHRC national: "Diffusion de la technique des implants cochléaires: etude d'impact des conséquences médicales, sociales et economiques".

Vertigo and dizziness are the most common complications of cochlear implantation. Data of a multicentric study about clinical aspects and cost utility were collected for vertigo and dizziness: incidence, clinical and treatment were analyzed among 469 adults and children. Results demonstrated that 16% of adults and 3% of children experienced dizziness postoperatively. In a few cases a specific mechanism as perilymphatic fistula was identified. In other cases the mechanism underlying delayed vertigo remains speculative and endolymphatic hydrops was suggested.

Hearing restoration with auditory brainstem implants after radiosurgery for neurofibromatosis type 2.

The auditory brainstem implant (ABI) is designed to restore useful auditory sensations in patients with neurofibromatosis Type 2 (NF2). The implantation is usually performed at the time of tumor removal in patients who do not undergo radiation treatment. The authors evaluated the performance of ABIs in three patients with NF2 in whom vestibular schwannoma continued to grow after radiation treatment. These three patients with NF2 received a 21-channel ABI; a translabyrinthine approach was used for both the tumor removal and the ABI placement. The interval between radiosurgery and the tumor removal plus device implantation ranged from 2 to 11 years. In all cases, the tumor was growing and the patients presented with total deafness. The mean number of active electrodes in these three patients was equivalent to the average results reported in other patients who received ABIs. The patients in this study used the ABI regularly for everyday life and obtained useful levels of environmental sound recognition. It is concluded that hearing function can be rehabilitated using ABIs in patients with NF2, even if radiosurgery fails to control the tumor growth.

Extraaxial primitive neuroectodermal tumor mimicking a vestibular schwannoma: diagnostic and therapeutic difficulties. Report of two cases.

Extraaxial cerebellopontine angle (CPA) medulloblastomas and other primitive neuroectodermal tumors (PNETs) are rare tumors. The authors report on two patients with PNETs who presented with progressive audiovestibular symptoms. In each case magnetic resonance (MR) imaging revealed an extraaxial lesion that filled the internal auditory meatus and exhibited the neuroimaging features of a vestibular schwannoma (VS). No high signal intensity was apparent in either the brainstem or adjacent cerebellum on T2-weighted MR images. Surgery with maximum resection (total in one case and subtotal in the other) was performed, followed by craniospinal radiotherapy. One year postoperatively, both patients were free from tumor. A CPA PNET mimicking a VS is a rare entity, the diagnosis of which is important because its treatment differs dramatically from that of VS, including prescribed surgery followed by conventional craniospinal radiotherapy.

Benefits of cochlear implantation in elderly patients.

Cochlear implantation in elderly patients is a questionable subject. The purpose of this study was to evaluate the procedure and its outcome, the postoperative course, and the audiologic and social benefits of cochlear implantation in this population. Twenty-seven patients older than 60 years were compared with a control group of 15 adult patients. This retrospective study analyzed data concerning the outcome of the procedure, postoperative course, postoperative orthophonic test results, and answers of a questionnaire assessing the changes in communication, perception, and social outcomes. The procedure was uneventful in both groups. Minor complications were not more frequent in elderly patients. Orthophonic test results were comparable in both groups. At 12 months, 83% of the elderly patients had an open-set speech discrimination score above 60%. The benefits of cochlear implantation in terms of the quality of life are not statistically different with younger patients. Cost-utility analysis might support these findings.

Hypertrophic neuropathy of the facial nerve.

Hypertrophic neuropathy is a peripheral nerve lesion that is histologically characterized by onion bulb formations around axons. This histologic picture, which is usually seen in generalized hypertrophic neuropathies, can occasionally be observed in single nerves as localized hypertrophic neuropathy. Cranial involvement of such localized hypertrophic neuropathy represents a very rare entity; only a few cases have been reported in the literature. We report the history of a progressive facial paralysis with a tumorous enlargement of the seventh cranial nerve that was clinically suspected of being a schwannoma. Pathological examination permitted the diagnosis of hypertrophic neuropathy.

[Sudden bilateral hearing loss disclosing meningeal carcinomatosis]. / Surdité brusque bilatérale révélatrice d'une carcinomatose méningée.

We report the case of a patient presenting a sudden bilateral hearing loss. Four years before, a bladder carcinoma was resected and a chemotherapy was started. Gadolinium-enhanced magnetic resonance images revealed enhancement of both acoustic nerves. Cerebrospinal fluid analysis showed malignant cells consistent with the initial bladder cancer. Meningeal metastases from bladder carcinoma are extremely rare. Systemic chemotherapy and its low meningeal diffusion may enhance the incidence of this complication. Bilateral hearing loss is a rare initial manifestation of meningeal carcinomatosis.