Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.

Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls (P = 0.001); half were previously unknown (AF < 0.000002). MYO6, MYO7A, PTPRQ, and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1N321I/+ mice, carrying the TMC1:p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy.

Developing a serious game for gaze stability rehabilitation in children with vestibular hypofunction.

BACKGROUND: Children with vestibular hypofunction (VH) may have gaze instability, balance disorders, and delayed postural-motor development. Gaze stabilization exercises (GSE) are designed to improve dynamic visual acuity (DVA). We aimed to assess the acceptability of a serious game prototype called Kid Gaze Rehab (KGR) designed to implement GSE training in children with VH, combined with traditional vestibular rehabilitation. Effects on DVA and motor performance were also analyzed. METHODS: Twelve children (6 to 9 years old) were included. Sessions were held at the hospital twice a week, for 5 weeks. An adapted French version of The Child Simulator Sickness Questionnaire (SSQ) and the Face Scale Pain-Revised (FPS-R) were used to assess pain in the cervical region and undesirable side effects after each session. Vestibular and motor function parameters (active and passive DVA and Movement Assessment Battery for Children-Second Edition, MABC-2) were assessed before and after the training. RESULTS: All children included completed the 10 sessions. The FPS-R visual analog scale and SSQ showed good cervical tolerance and no oculomotor or vegetative adverse effects nor spatial disorientation. After training, active DVA scores were significantly improved for the right, left, and up directions (p < 0.05). Passive DVA scores were significantly improved for the left and down directions (p < 0.01 and p < 0.05, respectively). MABC-2 scores were improved in the balance and ball skill sections (p < 0.05). CONCLUSION: An innovative pediatric training method, the use of a dedicated serious game for gaze stabilization was well-tolerated as a complement to conventional vestibular rehabilitation in children with VH. Moreover, both DVA and motor performance were found to improve in the study sample. Although replication studies are still needed, serious game-based training in children with VH could represent a promising rehabilitation approach for years to come. TRIAL REGISTRATION: The study was conducted in accordance with the Declaration of Helsinki and approved by an Institutional Review Board (local ethics committee, CPP Sud-Est IV, ID 2013-799). The study protocol was registered on ClinicalTrials.gov (NCT04353115).

Online digital audiometry vs. conventional audiometry: a multi-centre comparative clinical study.

OBJECTIVE: The primary objective of the current study was the validation of a cloud-centralized audiometry system for clinical practice. DESIGN: A cross-sectional study design was used. STUDY SAMPLE: A convenience sample of patients (>10 years old) booked for follow-up appointments were invited to participate. Participants completed both conventional and online digital audiometry in a standard sound treated clinic space during a single clinic visit; tests were completed in random order. Data for both ears were included. Patients were from one of three audiological practices. RESULTS: A total of 41 participants completed both audiometric tests. Validation study results showed that the mean difference between the two audiometric test results remained within 5 dB HL for both air and bone conduction thresholds at all tested frequencies. CONCLUSIONS: Online digital audiometry has been demonstrated as a clinically accurate method for hearing assessment.

Early grammar-building in French-speaking deaf children with cochlear implants: A follow-up corpus study.

BACKGROUND: One of the most consistent findings reported in the paediatric cochlear implant (CI) literature is the heterogeneity of language performance observed more in grammatical morphology than in lexicon or pragmatics. As most of the corpus studies addressing these issues have been conducted in English, it is unclear whether their results can be generalized to other languages. In particular, little is known about languages known for their grammatical complexity, such as French. AIMS: The aim of this corpus study was to compare the productive use of function words (FWs) and some agreement features (AGRs) in children with CIs and children with typical development (TD) matched for mean length of utterance in words (MLUwords ), a general index of grammatical complexity, and auditory experience, as measured by hearing age (HA) and chronological age (CA), respectively. METHODS & PROCEDURES: Natural speech samples from 116 monolingual French-speaking children, including 40 children with CIs followed longitudinally and 76 TD children, were collected. FWs and AGRs were analysed using a Part of Speech Tagger (POS-T) from the Child Language Data Exchange System (CHILDES). OUTCOMES & RESULTS: The two groups differed by 3 years for HA and CA. No effect of family socio-economic status (SES) was found in the CI group. Stepwise regression analyses showed that the two groups did not share the same predictors of MLUwords : plurals and determiners predicted MLUwords in children with CIs, at 2 and 3 years of HA, whereas feminine markers and subject-pronouns were found to best predict MLUwords in TD children at 2 and 3 years of CA. Structural equation models (SEMs), a combination of confirmatory factor analysis (CFA) and path analysis, yielded a different hierarchical structure of grammatical relations (GRs). Selective difficulties affecting verbal clitics and other pronominal forms were found specifically in the CI group (object-pronouns, reflexive, relative and past participles). Dependency grammar analysis confirmed these contrasting developmental profiles in multiword utterances, such as preposition/nouns, subject/verbs, and verb/determiner/nouns. CONCLUSIONS & IMPLICATIONS: Atypical grammatical patterns in children with CIs reflect a specific architecture of syntactic dependencies of FWs underpinning morphological complexity and syntactic connectivity. Clinical implications are discussed for assessment and intervention planning. WHAT THIS PAPER ADDS: What is already known on this subject The productive use of FWs has been identified as a particular area of weakness in children with CIs compared with TD children. In addition, heterogenous grammatical performance has often been found after 1-3 years of CI use, regardless of demographic factors such as age at implantation, duration of deafness or SES. What this paper adds to existing knowledge Assessing the early building of FWs and AGRs in children with CIs helps to understand the syntactic complexity and hierarchical structure of their language. Since most corpus studies on grammatical morphology have been conducted in English, it is not clear whether their difficulties can be generalized to other languages. The French language has a system of FWs and inflections that determine the morphophonological properties of nominal and verbal forms. Early grammar learning in children with CIs born with profound deafness were compared with the two groups of TD children matched both for duration of auditory experience (i.e., HA of CI children, CA of TD children) and for MLUwords . We found a similar profile between groups at 2 years but not at 3 years for HA and CA. The two groups do not share the same predictors of MLUwords : namely, plurals and determiners for CI children versus feminine markers and subject pronouns for TD children. They show a different syntactic organization of GRs. Children with CIs struggle with selective difficulties affecting verbal clitics and pronominal forms (object-pronouns, reflexive, relative and past participles). Consistent with theories of morphophonological richness and syntactic connectivity, our results support the distributional learning hypothesis of language acquisition that infants and toddlers are sensitive to FWs and AGRs at an early age. Specific components of syntactic organization are disrupted in children with CIs. What are the potential or actual clinical implications of this work? This work has potential clinical implications because it unravels the limitations of morphophonological processing in children with CIs. Its results highlight a specific difficulty in learning FWs and AGRs in a verbal inflectional morphology context.

Speech-in-Noise Audiometry in Adults: A Review of the Available Tests for French Speakers.

BACKGROUND: Difficulty understanding speech in background noise is the reason of consultation for most people who seek help for their hearing. With the increased use of speech-in-noise (SpIN) testing, audiologists and otologists are expected to evidence disabilities in a greater number of patients with sensorineural hearing loss. The purpose of this study is to list validated available SpIN tests for the French-speaking population. SUMMARY: A review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. PubMed and Scopus databases were searched. Search strategies used a combination of 4 keywords: speech, audiometry, noise, and French. There were 10 validated SpIN tests dedicated to the Francophone adult population at the time of the review. Some tests use digits triplets as speech stimuli and were originally designed for hearing screening. The others were given a broader range of indications covering diagnostic or research purposes, determination of functional capacities and fitness for duty, as well as assessment of hearing amplification benefit. KEY MESSAGES: As there is a SpIN test for almost any type of clinical or rehabilitation needs, both the accuracy and duration should be considered for choosing one or the other. In an effort to meet the needs of a rapidly aging population, fast adaptive procedures can be favored to screen large groups in order to limit the risk of ignoring the early signs of forthcoming presbycusis and to provide appropriate audiological counseling.

Simplification of syllable structure in childhood apraxia of speech: a 2-year follow-up French case study.

Although Childhood Apraxia of Speech (CAS) has been extensively investigated in the clinical literature, most of the findings regarding impairments in the production of syllable structure, recorded within this population, have been mainly focused on English. The main purpose of this two-year follow-up case study was, therefore, to examine whether syllable complexity may be considered as a robust indicator in CAS and whether it can explain the persistence of errors and, if so, at what age. This was tested in a boy followed up annually from age 5 to 7 who was administered a narrative task. Data analyses used the Phon program to estimate accuracies of different syllabic structures, phones, singleton and cluster consonants. Overall, the findings suggest that this child experienced difficulty producing syllabic structure commensurately with the level of complexity of the target structures. Notably, the presence of syllable planning/sequencing deficit found in French data clearly supports the hypotheses according to which (a) there is a relationship between the level of complexity of syllabic structures and their simplification and (b) the persistence of errors on the most complex syllables remains, becoming a robust indicator for identifying CAS from other speech disorders. Further cross-language investigations on syllable complexity in CAS are needed to design better assessments and to plan efficient intervention.

Reliability of the Language ENvironment Analysis system (LENA™) in European French.

In this study, we examined the accuracy of the Language ENvironment Analysis (LENA) system in European French. LENA is a digital recording device with software that facilitates the collection and analysis of audio recordings from young children, providing automated measures of the speech overheard and produced by the child. Eighteen native French-speaking children, who were divided into six age groups ranging from 3 to 48 months old, were recorded about 10-16 h per day, three days a week. A total of 324 samples (six 10-min chunks of recordings) were selected and then transcribed according to the CHAT format. Simple and mixed linear models between the LENA and human adult word count (AWC) and child vocalization count (CVC) estimates were performed, to determine to what extent the automatic and the human methods agreed. Both the AWC and CVC estimates were very reliable (r = .64 and .71, respectively) for the 324 samples. When controlling the random factors of participants and recordings, 1 h was sufficient to obtain a reliable sample. It was, however, found that two age groups (7-12 months and 13-18 months) had a significant effect on the AWC data and that the second day of recording had a significant effect on the CVC data. When noise-related factors were added to the model, only a significant effect of signal-to-noise ratio was found on the AWC data. All of these findings and their clinical implications are discussed, providing strong support for the reliability of LENA in French.

Drug-induced hearing loss: Listening to the latest advances.

Sensorineural hearing loss (SNHL) is the most common type of hearing loss. Causes include degenerative changes in the sensory hair cells, their synapses and/or the cochlear nerve. As human inner ear hair cells have no capacity for regeneration, their destruction is irreversible and leads to permanent hearing loss. SNHL can be genetically inherited or acquired through ageing, exposure to noise or ototoxic drugs. Ototoxicity generally refers to damage to the structures and functions of the inner ear following exposure to specific drugs. Ototoxicity can be multifactorial, causing damage to cochlear hair cells or cells with homeostatic functions that modulate cochlear hair cell function. Clinical strategies to limit ototoxicity include identifying patients at risk, monitoring drug concentrations, performing serial hearing assessments and switching to less ototoxic therapy. This review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, using the PubMed® database. The search terms "ototoxicity", "hearing loss" and "drugs" were combined. We included studies published between September 2013 and June 2023, and focused on medicines and drugs used in hospitals. The review highlighted a number of articles reporting the main drug classes potentially involved: namely, immunosuppressants, antimalarials, vaccines, antibiotics, antineoplastic agents, diuretics, nonsteroidal anti-inflammatory drugs and analgesics. The presumed ototoxic mechanisms were described, together with the therapeutic and preventive options developed over the last ten years.

Sudden Sensorineural Hearing Loss after COVID-19 Vaccination: A Review of the Available Evidence through the Prism of Causality Assessment.

Sudden sensorineural hearing loss (SSNHL), a rare audiological condition that accounts for 1% of all cases of sensorineural hearing loss, can cause permanent hearing damage. Soon after the launch of global COVID-19 vaccination campaigns, the World Health Organization released a signal detection about SSNHL cases following administration of various COVID-19 vaccines. Post-marketing studies have been conducted in different countries using either pharmacovigilance or medico-administrative databases to investigate SSNHL as a potential adverse effect of COVID-19 vaccines. Here, we examine the advantages and limitations of each type of post-marketing study available. While pharmacoepidemiological studies highlight the potential association between drug exposure and the event, pharmacovigilance approaches enable causality assessment. The latter objective can only be achieved if an expert evaluation is provided using internationally validated diagnostic criteria. For a rare adverse event such as SSNHL, case information and quantification of hearing loss are mandatory for assessing seriousness, severity, delay onset, differential diagnoses, corrective treatment, recovery, as well as functional sequelae. Appropriate methodology should be adopted depending on whether the target objective is to assess a global or individual risk.

Vestibular Impairment and Postural Development in Children With Bilateral Profound Hearing Loss.

Importance: Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing. Objectives: To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL. Design, Setting, and Participants: This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023. Main Outcomes and Measures: The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay. Results: A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment. Conclusions and Relevance: This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.

Guidelines of the French Society of Otorhinolaryngology and Head and Neck Surgery (SFORL) for vestibular rehabilitation in children with vestibular dysfunction. A systematic review.

INTRODUCTION: The consequence of complete or partial uncompensated vestibular dysfunction in children is usually balance disorders, with the risk of falls and increased fatigue, particularly during tasks requiring postural control. The aim of these recommendations is to establish guidelines for vestibular rehabilitation (VR) in children with vestibular impairment. MATERIAL AND METHODS: The guidelines were developed based on a systematic review of the international literature, validated by a multidisciplinary group of French-speaking otorhinolaryngologists, scientists, and physiotherapists. They are classified as grade A, B, C, or expert opinion according to a decreasing level of scientific evidence. RESULTS: A PubMed search of studies published between January 1990 and December 2021 was carried out using the keywords "vestibular," "rehabilitation," and "children". After filtering and reviewing the articles, a total of 10 publications were included to establish the recommendations. CONCLUSION: It is recommended that a vestibular assessment be carried out before VR, including a study of vestibulo-ocular reflex, otolithic function, and postural control. In cases of vestibular dysfunction, physiotherapy treatment is recommended from an early age to train different aspects of postural control, including anticipatory and reactive postural adjustments. VR adapted to the pediatric population is recommended for children whose vestibular dysfunction leads to functional disorders or symptoms of vertigo for those who have suffered head trauma. It is recommended that children with bilateral vestibular impairment be treated using gaze stabilization exercises for adaptation and substitution. Optokinetic stimulation and virtual reality are not recommended for children and young adolescents.

Cervical vestibular evoked myogenic potentials in healthy children: Normative values for bone and air conduction.

Objectives: To characterize cervical vestibular evoked myogenic potentials (c-VEMPs) in bone conduction (BC) and air conduction (AC) in healthy children, to compare the responses to adults and to provide normative values according to age and sex. Design: Observational study in a large cohort of healthy children (n = 118) and adults (n = 41). The c-VEMPs were normalized with the individual EMG traces, the amplitude ratios were modeled with the Royston-Wright method. Results: In children, the amplitude ratios of AC and BC c-VEMP were correlated (r = 0.6, p < 0.001) and their medians were not significantly different (p = 0.05). The amplitude ratio was higher in men than in women for AC (p = 0.04) and BC (p = 0.03). Children had significantly higher amplitude ratios than adults for AC (p = 0.01) and BC (p < 0.001). Normative values for children are shown. Amplitude ratio is age-dependent for AC more than for BC. Confidence limits of interaural amplitude ratio asymmetries were less than 32%. Thresholds were not different between AC and BC (88 ± 5 and 86 ± 6 dB nHL, p = 0.99). Mean latencies for AC and BC were for P-wave 13.0 and 13.2 msec and for N-wave 19.3 and 19.4 msec. Conclusion: The present study provides age- and sex-specific normative data for c-VEMP for children (6 months to 15 years of age) for AC and BC stimulation. Up to the age of 15 years, c-VEMP responses can be obtained equally well with both stimulation modes. Thus, BC represents a valid alternative for vestibular otolith testing, especially in case of air conduction disorders.

Bone Conduction Cervical Vestibular Evoked Myogenic Potentials as an Alternative in Children with Middle Ear Effusion.

OBJECTIVE: To compare the amplitude ratio and P-wave latency of cervical vestibular evoked myogenic potentials (c-VEMPs) for bone conduction (BC) and air conduction (AC) stimulation in children with otitis media with effusion (OME). MATERIAL AND METHODS: This is an observational study of a cohort of 27 children and 46 ears with OME. The c-VEMP amplitude ratio and P-wave latency were compared between BC and AC in children with OME and healthy age-matched children. RESULTS: The c-VEMP response rate in children with OME was 100% when using BC stimulation and 11% when using AC stimulation. The amplitude ratio for BC was significantly higher in the OME group than the age-matched healthy control group (p = 0.004). When focusing on ears with an AC c-VEMP response (n = 5), there was a significant difference in the amplitude ratio between the AC and BC stimulation modes, but there was no significant difference in the AC results between the OME group and the age-matched control group. CONCLUSIONS: BC stimulation allows for reliable vestibular otolith testing in children with middle ear effusion. Given the high prevalence of OME in children, clinicians should be aware that recording c-VEMPs with AC stimulation may lead to misinterpretation of otolith dysfunction in pediatric settings.

Age-Related Dichotic Listening Skills in Impaired and Non-Impaired Readers: A Comparative Study.

Dichotic listening is the high-level auditory process which enables the perception of different verbal stimuli delivered simultaneously to the right and left ears (binaural integration), as well as the perception of a verbal stimulus presented to one ear while ignoring a different stimulus in the other ear (binaural separation). Deficits in central auditory processing have been reported in children with learning disabilities. The present study aimed to compare dichotic listening performances in right-handed impaired readers (IR) and non-impaired readers (non-IR) according to age. For this, a cross-sectional study was conducted in 120 IR (56 males and 64 females) divided into five age groups and 120 non-IR (63 male and 57 female) matched on chronological age (8 to 9 years; 9 to 10 years; 10 to 12 years; 12 to 18 years; adult). They were tested for binaural integration and binaural separation, allowing for the calculation of dichotic aptitude (DA), ear prevalence (EP), and attentional shift index (ASI). A series of ANOVAs showed an effect of age and of the reading group for all the dichotic-related measures, except for EP. Binaural separation scores were lower in IR who also showed more intrusive responses compared to non-IR. These intrusive responses, which were more frequent on the right ear for IR, decreased with age in both groups. Overall, these results suggest that dichotic listening scores improve with age as the central auditory pathways mature. However, whatever the age, performances are lower in IR than in non-IR. This might be explained by an incomplete maturation of the auditory pathways in IR; an early start for long-term follow-up and auditory training is suggested.

The Magnitude of Contralateral Suppression of Otoacoustic Emissions Is Ear- and Age-Dependent.

The maturation of the uncrossed medial olivocochlear (UMOC) efferent remains poorly documented to date. The UMOC efferent system allows listeners to not only detect but also to process, recognize, and discriminate auditory stimuli. Its fibers can be explored non-invasively by recording the effect of contralateral acoustic stimulation (CAS), resulting in a decrease in the amplitude of transient evoked otoacoustic emissions (TEOAE). The objective of the present cross-sectional study was to investigate how the effectiveness of this system varies with age in healthy subjects aged 8 years to adulthood. For this purpose, 120 right-handed native French-speaking subjects (57 females and 63 males) were divided into five age groups of 24 subjects each: 8y-10y, 10y-11y6m, 11y6m-13y, 13y-17y, and ≥18y. TEOAE amplitudes with and without CAS were recorded. The equivalent attenuation (EA) was calculated, corresponding to the change in TEOAE amplitude equivalent to the effect generated by CAS. General linear models were performed to control for the effect of ear, sex, and age on EA. No sex effect was found. A stronger EA was consistently found regardless of age group in the right ear compared to the left. In contrast to the right ear, for which, on average, EA remained constant across age groups, an increasingly weaker TEOAE suppression effect with age was found in the left ear, reinforcing the asymmetrical functioning of the UMOC efferent system in favor of the right ear in adulthood. Further studies are needed to investigate the lateralization of the UMOC efferent system and its changes over time in cases of atypical or reversed cortical asymmetries, especially in subjects with specific learning disorders.

Why should multiple dehiscences of the otic capsule be considered before surgically treating patients with superior semicircular canal dehiscence? A radiological monocentric review and a case series.

This review aims to draw attention to the multiple ipsilateral otic capsule dehiscences (OCDs), which may cause therapeutic failure in operated patients. A series of six severely disabled patients with symptoms and signs consistent with a superior semicircular canal dehiscence (SSCD) diagnosis, confirmed by a high-resolution CT scan, is presented here. Five of the patients underwent surgery, and in four of the cases, the postoperative results were poor and/or disappointing. The ethical principles underlying modern medicine encourage medical staff to learn from past experience even when the results are modest despite the accuracy of the treatment applied to a patient. Consequently, we reviewed the radiological records of symptomatic and asymptomatic patients diagnosed or referred to our center for confirmation over the past 5 years to determine the incidence of multiple OCD in this population. Multiple localizations of suspected OCD in the ipsilateral ear did not appear to be rare and were found in 29 of 157 patients (18.47%) in our retrospective review using high-resolution thin-sliced CT scans. The decision to perform surgery for a documented symptomatic superior SSCD should be made with caution only after ruling out concomitant lesser-known variants of OCD in the ipsilateral ear.

The "Near"-Narrowed Internal Auditory Canal Syndrome in Adults: Clinical Aspects, Audio-Vestibular Findings, and Radiological Criteria for Diagnosis.

INTRODUCTION: Vestibular Paroxysmia (VP) refers to short attacks of vertigo, spontaneous or triggered by head movements, and implies the presence of a compressive vascular loop in contact with the cochleovestibular nerve (CVN). Classically, a narrowed internal auditory canal (IAC) corresponds to a diameter of less than 2 mm on CT, usually associated with a hypoplastic CVN on MRI. The aim of this study was to discuss a distinct clinical entity mimicking VP in relation to a "near"-narrowed IAC (NNIAC) and to propose radiological criteria for its diagnosis. METHODS: Radiological measurements of the IAC were compared between three groups: the study group (SG, subjects with a clinical presentation suggestive of VP, but whose MRI of the inner ear and pontocerebellar angle excluded a compressive vascular loop) and two control groups (adult and children) with normal vestibular evaluations and no history of vertigo. RESULTS: 59 subjects (18 M and 41 F) were included in the SG. The main symptoms of NNIAC were positional vertigo, exercise- or rapid head movements-induced vertigo, and dizziness. The statistical analysis in the study group showed that the threshold values for diagnosis were 3.3 mm (in tomodensitometry) and 2.9 mm (in MRI) in coronal sections of IAC. Although a significantly lower mean value for axial IAC diameter was found in SG compared with controls, the statistics did not reveal a threshold due to the large inter-individual variations in IAC measurements in normal subjects. There was no significant difference in IAC diameter between the adult and pediatric controls. CONCLUSIONS: In the present study, we report a new anatomopathological condition that appears to be responsible for a clinical picture very similar-but not identical-to VP in association with the presence of an NNIAC. The diagnosis requires a careful analysis of the IAC's shape and diameters in both axial and coronal planes.