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).

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).

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.

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.

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.

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.

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.

Acquired medial external auditory canal stenosis, anterior tympanomeatal angle blunting, and lateralized tympanic membrane: Nosology, diagnosis, and treatment.

OBJECTIVES: To analyze the etiologies, auditory consequences, diagnostic tools and therapeutic results of three often confused pathologies: acquired fibrous stenosis of the medial part of the external auditory canal (EAC), fibrous anterior tympanomeatal angle blunting, and lateralized tympanic membrane. MATERIAL AND METHODS: Retrospective study of 18 cases operated on over a 16-year period (14 patients: 7 female, 7 male; aged 11-64 years): 8 cases of medial EAC stenosis, 3 of blunting, and 7 of tympanic membrane lateralization. RESULTS: In all 3 pathologies, otoscopic and radiologic diagnosis was easily established, so that they could not be confused. All 3 induced>33dB conductive hearing loss. Medial EAC stenosis was secondary to chronic inflammation of the EAC, aggravated by surgery in 5 cases. Blunting was secondary to surgery altering the anterior tympanic annulus. Tympanic membrane lateralization was secondary to prior surgery without inflammatory process. Underlying EAC cholesteatoma was found in 3 cases of medial stenosis and in 1 case of blunting. Surgical results were disappointing in medial stenosis, with 62.5% recurrence and mean functional gain of 9dB, and in blunting, with 66.7% recurrence and mean functional gain of 6dB; auditory results were, however, good in these 2 pathologies when there was no recurrence of fibrosis. Results were significantly better in lateralized tympanic membrane, with 28.6% recurrence and mean functional gain of 16dB. CONCLUSION: The good results obtained in tympanic membrane lateralization seem to justify surgery in patients bothered by their hearing loss. The indication is more questionable in cases of medial fibrous stenosis and blunting, although significant auditory improvement is achieved in case of surgical success.

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

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