Speech reception after cochlear implantation for Cogan's syndrome: Case series following CARE guidelines.

INTRODUCTION: Cogan's syndrome is a rare form of vasculitis mainly affecting young subjects of whatever gender, associating cochleovestibular and ophthalmological damage. Despite medical treatment, auditory prognosis is uncertain, with 50-60% of patients showing irreversible severe to profound hearing loss, thus being candidates for cochlear implantation. Following CARE guidelines, we report 10 cases of cochlear implantation in Cogan's syndrome, with assessment of speech reception threshold and maximum intelligibility after a minimum 1 year's experience with the implant. CASE SERIES: Ten patients from 4 centers received cochlear implants (14 implants) for Cogan's syndrome between 2005 and 2020. After 1 year's experience, there was significant improvement in speech reception threshold (P=0.0002) and maximum intelligibility (P=0.0002). CONCLUSION-DISCUSSION: Audiovestibular signs associated with ophthalmological manifestations should suggest Cogan's syndrome. Hearing impairment is usually irreversible. Cochlear implantation may be necessary and improves hearing performance.

Auditory brainstem response: Key parameters for good-quality recording.

Auditory brainstem response (ABR) is widely used in ENT to investigate hearing loss. This test evaluates the response of the ascending auditory pathway, from cochlea to mesencephalon, following auditory stimulation. It provides precise analysis of waves numbered I to V according to location on the auditory pathway, in terms of amplitude, latency and inter-wave interval. Good-quality assessment requires familiarity with the parameters to be used and the factors likely to modify response. We describe the procedure for ABR examination and the recorded responses, with particular attention to factors influencing response to which the examiner must be vigilant. These factors are related to the individual (age, gender, hearing loss, body temperature, drug treatments), transducer (air or bone conduction), stimulation parameters (type, polarity, intensity, calibration, duration, cadence, number of clicks, background noise) and acquisition parameters (analysis window, scale, electrodes). We also briefly describe the clinical applications of this examination.

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).

Training in temporal bone drilling.

Acquiring surgical experience in the operating room is increasingly difficult. Simulation of temporal bone drilling is therefore essential, and more and more widely used. The aim of this review is to clarify the limitations of classical surgical training, and to describe the different types of simulation available for temporal bone drilling. Systematic Medline search used the terms: "temporal bone" and training and surgery; "temporal bone" and training and drilling. Seventy-one of the 467 articles identified were relevant for this review. Various temporal bone simulators have been created to get around the limitations (ethical, financial, cultural, working time) of temporal bone drilling. They can be classified as cadaver, animal, physical or virtual models. The main advantages of physical and virtual prototyping are their ease of access, the possibility of repeating gestures on a standardised model, and the absence of ethical issues. Validation is essential before these simulators can be included in the curriculum, to ensure efficacy and thus improve patient safety in the operating room.

Hearing loss in inherited metabolic disorders: A systematic review.

Inherited metabolic disorders (IMDs) have been observed in individuals with hearing loss (HL), but IMDs are rarely the cause of syndromic HL. With early diagnosis, management of HL is more effective and cortical reorganization is possible with hearing aids or cochlear implants. This review describes relationships between IMDs and HL in terms of incidence, etiology of HL, pathophysiology, and treatment. Forty types of IMDs are described in the literature, mainly in case reports. Management and prognosis are noted where existing. We also describe IMDs with HL given age of occurrence of HL. Reviewing the main IMDs that are associated with HL may provide an additional clinical tool with which to better diagnose syndromic HL.

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 Adult [Pré-print]

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. 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. Eight tests of speech audiometry in noise can be used in France. 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).

Otosclerosis surgery under local anesthesia with sedation: Assessment of quality of life and stress.

OBJECTIVES: The main study endpoint was tolerance of stapedotomy under local anesthesia with sedation and under general anesthesia using stress and quality of life assessment questionnaires. Secondary endpoints comprised operative time and functional results. MATERIAL AND METHOD: In a consecutive series of stapedotomy patients operated on over a 12-month period, quality of life and perioperative stress were analysed by 3 questionnaires: the Glasgow Benefit Inventory, Cohen's perceived stress scale and the Post-traumatic stress disorder checklist scale. Questionnaire responses and audiometric data were compared between groups treated under local anesthesia with sedation and under general anesthesia. RESULTS: Twenty-two patients were included in the local anesthesia with sedation group and 6 in the general anesthesia group. There was no difference between the groups for quality of life, onset of post-traumatic stress, or perceived pre- and postoperative stress. There was also no difference in operative time. The audiometric data confirmed the reliability of stapedotomy. Stapedotomy under local anesthesia with sedation improved air conduction with≤10dB air-bone gap (ABG), comparable to results under general anesthesia. The rate of ABG≤10dB was 71.4%; no labyrinthisation was observed. CONCLUSION: Under local anesthesia with sedation, stapedotomy was well tolerated without increasing the stress associated with otosclerosis surgery. By correcting hearing loss, the procedure improves quality of life.

Vestibular evoked myogenic potentials.

Vestibular Evoked Myogenic Potentials (VEMP) are commonly recorded in patients experiencing vertigo or chronic instability. This test evaluates the patient's otolith function and is often combined with both Videonystagmography and Video Head Impulse Test. VEMP is a simple, reproducible test, in the absence of any pre-existing conductive hearing loss. Cervical VEMP explore both saccular function and the inferior vestibular nerve, whereas ocular VEMP assess utricular function and the superior vestibular nerve. In combination with previously described tests, VEMP allows characterization of vertigo and provides support for the diagnosis of superior semicircular canal dehiscence syndrome, Menière's disease, vestibular neuritis, vestibular schwannoma or idiopathic bilateral vestibulopathy. A good knowledge of these electrophysiological tests is essential in order to precisely assess the presence or absence of vestibular function impairment. We describe the test recording technique and the most common pitfalls in interpretation of the results. We then outline the results observed in various diseases impacting vestibular function.

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.

Emotional prosody perception in presbycusis patients after auditory rehabilitation.

OBJECTIVE: Perception of emotion plays a major role in social interaction. Studies have shown that hearing loss and aging degrade emotional recognition. The main aim of the present study was to evaluate the benefit of first-time hearing aids (HA) for emotional prosody perception in presbycusis patients. Secondary objectives comprised comparison with normal-hearing subjects, and assessment of the impact of demographic and audiologic factors. METHODS: To assess HA impact, 29 subjects with presbycusis were included. They were tested without HA and 1 month after starting to use HA. A test with emotional hearing stimuli (Montreal Affective Voice test: MAV) was performed at various intensities (50, 65 and 80dB SPL). Patients' experience was evaluated on the Profile of Emotional Competence questionnaire, before and after HA fitting. Results were compared with those of 29 normal-hearing subjects. RESULTS: Auditory rehabilitation did not significantly improve MAV results (P>0.005), or subjective questionnaire results (P>0.005). Scores remained lower than those of normal-hearing subjects (P<0.001). MAV results, before and after HA, showed significant correlation with pure-tone average (r=-0.88, P<0.001) and age (r=0.44, P=0.018). The older the presbycusis patient and the more severe the hearing loss, the greater the difficulty in recognising emotional prosody. CONCLUSION: Despite hearing rehabilitation, presbycusis patients' results remained poorer than in normal-hearing subjects.

Stapes surgery for otosclerosis under local anaesthesia with sedation.

The stapes surgery surgical technique has now been clearly standardized, ensuring a reliable and reproducible procedure with a satisfactory success rate. The possibility of performing this surgery under local anaesthesia with sedation requires very good collaboration between surgeons and anaesthetists. The patient is informed about the various steps of the operation to ensure that he or she is reassured both before and during the procedure. Local anaesthesia with sedation constitutes an alternative in patients afraid of general anaesthesia. Sudden onset of dizziness reported by the patient during the operation after piston placement may be due to an excessively long piston, which may need to be adjusted. We describe the technique used in our centre. In the literature, there is no difference in terms of audiometric performance and dizziness between patients operated under local anaesthesia with sedation or general anaesthesia.

Influence of surgical technique on residual cholesteatoma location and prevalence.

OBJECTIVE: Compared to canal wall up (CWU) tympanoplasty, canal wall reconstruction (CWR) allows better visualization of cholesteatoma extension. The canal wall up approach provides good functional outcomes, but with higher rates of residual cholesteatoma. The aim of this study was to compare residual cholesteatoma prevalence and location between the two approaches. METHOD: Subjects were adult patients with residual cholesteatoma following CWU or CWR surgery between January 1, 2010 and December 31, 2015. During this period, 94 patients underwent CWU and 71 CWR; 22 presented with residual cholesteatoma: 16 after CWU (R-CWU group) and 6 after CWR (R-CWR group). RESULTS: There was no significant inter-group difference in residual cholesteatoma prevalence: 17% after CWU, 8.4% after CWR. Locations comprised: 13 (81%) in the attic, 9 (56%) in the tympanic cavity and 4 (25%) in the mastoid in the R-CWU group, and 6 (100%) in the attic in the R-CWR group. There were significantly fewer tympanic cavity locations after CWR compared to CWU (P=0.046). CONCLUSION: Residual cholesteatoma prevalence did not significantly differ between the CWU and CWR approaches. The most frequent location was the attic; significantly more locations were in the tympanic cavity with the CWU approach. These findings are important for surgeons and neuro-radiologists during follow-up.

Otosclerosis surgery: Assessment of patient comfort.

OBJECTIVES: To evaluate operative comfort and stress in patients undergoing stapedotomy for otosclerosis under local versus general anesthesia. MATERIAL AND METHODS: Consecutive otosclerosis patients managed over a 9-month period responded to 3 validated questionnaires to assess peri- and post-operative comfort: Glasgow Benefit Inventory, Cohen's Perceived Stress Scale and the Posttraumatic Stress Disorder Checklist Scale. These results and audiometric data were compared between local and general anesthesia groups. RESULTS: Twenty-one patients were included in the local anesthesia group and 7 in the general anesthesia group, after exclusion of patients with history of otosclerosis surgery. There was no significant inter-group difference on Glasgow Benefit Inventory (P=0.38) or Posttraumatic Stress Disorder Checklist Scale (P=0.86). Perceived Stress Scale scores were higher in the general anesthesia group (P=0.038). In total, 67% of patients reported no discomfort under local anesthesia, and 86% were ready to undergo the procedure under local anesthesia again. There were no significant differences in postoperative symptoms, or in air-bone gap≤10dB (local anesthesia 81%, general anesthesia 71%; P=0.156). CONCLUSIONS: Local anesthesia in otosclerosis surgery did not increase stress or postoperative symptoms compared to general anesthesia. Audiometric results were not affected by type of anesthesia.

Cortical auditory responses according to hearing rehabilitation in unilateral hearing loss.

OBJECTIVE: To assess the impact of rehabilitation systems (CROS: Contralateral Routing of Signal; BAHA: Bone-Anchored Hearing Aid; CI: cochlear implant) on cortical auditory evoked potentials (CAEP) and auditory performance in unilateral hearing loss. SUBJECTS AND METHOD: Twenty-one adults with unilateral hearing loss, using CROS (n=6), BAHA (n=6) or CI (n=9), were included. Seven normal-hearing subjects served as controls. CAEPs were recorded for a (/ba/) speech stimulus; for patients, tests were conducted with and without their auditory rehabilitation. Amplitude and latency of the various CAEP components of the global field power (GFP) were measured, and scalp potential fields were mapped. Behavioral assessment used sentence recognition in noise, with and without spatial cues. RESULTS: Only CI induced N1 peak amplitude change (P<0.05). CI and CROS increased polarity inversion amplitude in the contralateral ear, and frontocentral negativity on the scalp potential map. CI improved understanding when speech was presented to the implanted ear and noise to the healthy ear, and vice-versa. CONCLUSION: Cochlear implantation had the greatest impact on CAEP morphology and auditory performance. A longitudinal study could analyze progression of cortical reorganization.

Unilateral vagus nerve lesion revealing clival meningocele.

INTRODUCTION: Cranial nerve lesions can be secondary to a space-occupying lesion of the skull base compressing adjacent nerves. CASE REPORT: We report the case of an 84-year-old man, who presented with rapid and concomitant onset of dysphagia and ipsilateral recurrent laryngeal nerve paralysis, suggesting an isolated lesion of the vagus nerve. MRI revealed a diagnosis of previously unknown clival meningocele. DISCUSSION: Unilateral vagus nerve paralysis constitutes an exceptional mode of presentation of meningocele. Only a few isolated cases of clival meningocele have been reported to date, with no cranial nerve repercussions. The symptomatic management adopted in this case allowed rapid improvement of the patient's disorders.