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.

A case of restitution ad integrum in Gorham-Stout disease.

Gorham-Stout disease (or "vanishing bone" disease) is a rare mono or polyostotic disease of unknown etiology, characterized by intraosseous angiomatous proliferation leading to bone resorption. We report the case of a 17-year-old woman presenting with symptomatic osteolytic lesions of the frontal vault. Imaging was suggestive of Gorham-Stout disease without argument for other diagnoses. An unusual evolution of the "vanishing bone" lesions was observed on the scan after one year, with a full recovery of the lytic lesions. This report shows for the first time a spontaneous restitutio ad integrum of bone matrix in Gorham-Stout disease.

The Narrowed Internal Auditory Canal: A Distinct Etiology of Pediatric Vestibular Paroxysmia.

Vestibular paroxysmia (VP) is a disorder encountered in the pediatric population that etiology has been attributed to neurovascular cross-compression syndrome (NVCC). The purpose of this study was to report a new probable pathological condition, the narrowed internal auditory canal (IAC), which appears to be involved in the development of a clinical picture of VP in the pediatric population. A retrospective descriptive comparative study was conducted to compare clinical, electrophysiological, radiological, and therapeutic outcomes in both etiologies. Overall, 16 pediatric patients suffering from VP were included and divided into two groups: patients with narrowed internal auditory (Group 1) were compared to those with NVCC syndrome (Group 2). Patients in both groups were similar in terms of auditory complaints, as well as hearing, vestibular, and electrophysiological status. A narrowed IAC was encountered in the adolescent age category and females, especially those with rapid growth. The diagnosis requires a careful analysis of the shape and diameters of the IAC. Radiologic measurements in the axial plane do not seem to be sufficient to confirm the diagnosis, and, therefore, an analysis of diameters in the coronal plane is required. Treatment with sodium-channel blockers drugs showed promising results not only by relieving vertigo but also by normalizing the electrophysiological findings. In conclusion, a narrowed IAC can be considered in patients suffering from VP.

High Heterogeneity of Temporal Bone CT Aspects in Osteogenesis Imperfecta Is Not Linked to Hearing Loss.

OBJECTIVES: To determine whether temporal bone computed tomography (CT) features are linked to the presence and type of hearing loss in osteogenesis imperfecta (OI) when considering hearing-impaired OI patients and normally hearing (NH) OI ones. A secondary objective was to assess whether other factors influence CT features in a large sample: age, type of mutation, or bone mineral density (BMD). METHODS: A total of 41 adults with OI underwent CTs and pure-tone audiometry in 82 ears. Hearing thresholds were normal in 64 out of 82 ears, and most had not been operated on for stapedectomy or stapedotomy. Ossicle density, footplates, oval and round windows, retrofenestral peri- and endolabyrinths, and temporal pneumatization were analyzed twice by an experienced radiologist. CT features were compared to hearing, age, collagen mutations, and bone mineral density. RESULTS: Unexpectedly a high prevalence of footplate, ossicle, and otic capsule anomalies was observed, even in NH ears. Footplate hypodensity or thickening was mostly found in ears without conductive hearing loss. There were significantly more retrofenestral anomalies or window obstruction in ears with a sensorineural hearing loss component than in ears without. Age was significantly higher in ears with middle layer hypodensity than in ears without. Patients with mutations were expected to have reduced collagen quantity and had significantly more footplate or retrofenestral anomalies than those with qualitative mutations. BMD was significantly higher in ears without temporal hyperpneumatization. CONCLUSION: Temporal bone CT features in OI are present in a large proportion of patients, had they hearing loss or not, and might be determined more by collagen mutation type than by age or BMD.

Persistent Positional Vertigo in a Patient with Partial "Auto-Plugged" Superior Semicircular Canal Dehiscence: A Case Study.

A 73-year-old female was referred to our department for persistent left anterior benign paroxysmal positional vertigo refractory to multiple repositioning procedures and training physiotherapist exercises. The audiovestibular assessment and high resonance computed tomography of the petrosal bone confirmed the presence of a 5.4 mm large paucisymptomatic left anterior semicircular canal dehiscence. The connection between the 2 apparently distinct neurotological entities in the same patient was further sustained by additional imagery. T2-weighted and 3-dimensional labyrinthine sequences confirmed the presence of a partially "auto-plugged" superior semicircular canal dehiscence which progressively entrapped greater otolith particles proximal to the cupula of the superior semicircular canal.

Proposal for a Unitary Anatomo-Clinical and Radiological Classification of Third Mobile Window Abnormalities.

Introduction: An increased number of otic capsule dehiscence (OCD) variants relying on the third window pathomechanism have been reported lately. Therefore, a characterization of the anatomical structures involved and an accurate radiological description of the third window (TW) interface location have become essential for improving the diagnosis and appropriate therapeutic modalities. The purpose of this article is to propose a classification based on clinical, anatomical, and radiological data of third mobile window abnormalities (TMWA) and to discuss the alleged pathomechanism in lesser-known clinical variants. Materials and Methods: The imaging records of 259 patients who underwent, over the last 6 years, a high-resolution CT (HRCT) of the petrosal bone for conductive hearing loss were analyzed retrospectively. Patients with degenerative, traumatic, or chronic infectious petrosal bone pathology were excluded. As cases with a clinical presentation similar to those of a TW syndrome have recently been described in the literature but without these being confirmed radiologically, we thought it necessary to be integrated in a separated branch of this classification as "CT - TMWA." The same goes for certain intralabyrinthine pathologies also recently reported in the literature, which mimic to some extent the symptoms of a TW pathology. Therefore, we suggest to call them intralabyrinthine TW-like abnormalities. Results: Temporal bone HRCT and, in some cases, 3T MRI of 97 patients presenting symptomatic or pauci-symptomatic, single or multiple, unilateral or bilateral OCD were used to develop this classification. According to the topography and anatomical structures involved at the site of the interface of the TW, a third-type classification of OCD is proposed. Conclusions: A classification reuniting all types of TMWA as the one proposed in this article would allow for a better systematization and understanding of this complex pathology and possibly paves the way for innovative therapeutic approaches. To encompass all clinical and radiological variants of TMWA reported in the literature so far, TMWAs have been conventionally divided into two major subgroups: Extralabyrinthine (or "true" OCD with three subtypes) and Intralabyrinthine (in which an additional mobile window-like mechanism is highly suspected) or TMWA-like subtype. Along these subgroups, clinical forms of OCD with multiple localization (multiple OCD) and those that, despite the fact that they have obvious characteristics of OCD have a negative CT scan (or CT - TMWA), were also included.

A new LRP6 variant and Camurati-Engelmann-like disease.

INTRODUCTION: Camurati-Engelmann disease is a rare autosomal dominant bone dysplasia belonging to the group of craniotubular hyperostoses. Genetic analysis classically shows mutation on TGFß1 gene. CASE REPORT: A young woman was hospitalized with intense pain in lower limbs, associated to radiographic hyperostosis and sclerosis of the long bones. RESULTS: Mutation on LRP6 has recently been associated to high bone mass. In this case report, a rare missense variant on LRP6 gene was associated to radiographic features of Camurati-Engelmann. CONCLUSIONS: More studies should be conducted to assess the pathological role of this variant in Camurati-Engelmann-like disease.

A Message from a Narrowed Internal Auditory Canal in a Patient with a Hyperpneumatized Petrous Bone.

Hyperpneumatization of the temporal bone (HPTB) is a rare finding, and its clinical impact is poorly described in literature. We report the case of a 34-year-old woman with no previous otologic condition, complaining of paroxysmal vertigo and right pulsatile tinnitus shortly after a first scuba diving session. Clinical neurotologic assessment found a right vestibular impairment. Cranial tomodensitometry showed a bilateral hyperpneumatization of the petrosal air cell system. Brain magnetic resonance imaging to rule out any retrocochlear pathology was normal, although the cochleo-vestibular nerve (CVN) appeared to be over lengthened in a narrowed internal auditory canal (IAC), especially on the right-hand side. Pain was alleviated by administration of a low-dose anti-epileptic drug. The clinical, neurological, and radiological findings evoked a right vestibulopathy generated by a limited compression of the CVN; HPTB appeared to contribute to the narrowing of the IAC, especially on the right-hand side.

Comparison of Cone-Beam Computed Tomography and Multislice Computed Tomography in the Assessment of Extremity Fractures.

OBJECTIVES: The aims of this study were to evaluate the agreement between cone-beam computed tomography (CBCT) and multislice computed tomography for the characterization of extremity fractures and to compare image quality, radiation dose, and patient tolerance. METHODS: Thirty-six patients with suspected fracture affecting distal extremities or who required preoperative fracture assessment were enrolled prospectively. Each patient underwent CBCT and multislice computed tomography the same day. Both examinations were evaluated independently twice by 2 trained radiologists using the Müller AO classification for fracture characterization. RESULTS: Cohen κ coefficient for agreement between the imaging techniques was almost perfect for fracture characterization, κ = 0.94 [95% confidence interval, 0.91-0.98]. There was substantial to almost perfect agreement for secondary findings. Cone-beam computed tomography was well tolerated and significantly less irradiant and had better subjective image. CONCLUSIONS: An excellent agreement between both imaging techniques was found. This confirms the ability of CBCT to assess fractures and its potential in the management of patients with distal limb trauma.

Cone beam versus multi-detector computed tomography for detecting hearing loss.

OBJECTIVE: To determine whether the diagnostic value of cone-beam computed tomography (CBCT) is equivalent or superior to that of multi-detector computed tomography (MDCT) for the radiological assessment of conductive hearing loss with intact tympanic membrane in adults. Evaluation of inter- and intraobserver variability and measurement of the radiation dosimetry associated with each technique were secondary objectives. STUDY DESIGN: Prospective, single-center study. METHODS: Ten adults were included from April to June 2013. All patients underwent MDCT and CBCT with reconstruction of temporal bones. Two radiologists with ENT experience reviewed the results twice. Diagnostic agreement between MDCT and CBCT and inter- and intraobserver agreement was evaluated with the kappa statistic. Comparisons of dosimetry were evaluated by calculating the ratio of the CT dose index (CTDI) between MDCT and CBCT. RESULTS: Diagnostic agreement between MDCT and CBCT was satisfactory (kappa = 0.69). Inter- and intraobserver agreement was also acceptable, and the average ratio of the CTDI of MDCT and CBCT was 4.01. CONCLUSION: CBCT is a reliable method that uses a low dose of radiation to investigate conductive hearing loss with intact tympanic membrane in adults. Its relevance and potential superiority to MDCT in diagnosing middle ear pathologies such as otosclerosis remain to be demonstrated, but the preliminary data are promising.

Stenting the Superior Petrosal Sinus in a Patient With Symptomatic Superior Semicircular Canal Dehiscence.

Patients presenting superior semicircular canal dehiscence (SSCD) can experience symptoms such as conductive hearing loss, pulsatile tinnitus, autophony, and pressure-induced vertigo. Decreased cervical vestibular-evoked myogenic potentials (cVEMPs) thresholds and high-resolution computed tomography (HRCT) of the petrous bone are essential for diagnosis of SSCD syndrome. We report the case of a 43-year-old man suffering from constant right pulsatile tinnitus, intermittent autophony, and unsteadiness induced by physical exercise. An SSCD by the superior petrosal sinus (SPS) was confirmed on the right side by axial HRCT of the temporal bone reformatted in the plane of Pöschl and ipsilateral abnormally low elicited cVEMPs. Treatment options were discussed with the patient since the pulsatile tinnitus progressively became debilitating. Two options were considered: surgery or a new endovascular treatment; the patient chose the latter option. After stenting the right SPS, the intensity of the pulsatile tinnitus dramatically decreased. As there was no complication the patient was discharged at Day 1. The other symptoms improved progressively. By the 60-day follow-up visit the patient only reported a slight tinnitus worsened by physical exercise. Angiographic follow-up at 5 months confirmed the patency of the SPS. Stenting the SPS in patients with SSCD by the SPS appears to be an alternative to the existing surgical treatments.

[Allergic fungal rhinosinusitis: A diagnosis to evoke]. / Rhinosinusite fongique allergique : un diagnostic à évoquer.

Among fungal infections of the nasal sinuses, allergic fungal rhinosinusitis is a recently described, relatively rare and little known pathology. Its diagnosis is based on: clinical criteria (it occurs in young immunocompetent patients, often associated with bilateral nasosinusal polyposis); pathological criteria (allergic mucin: eosinophilic mucus without invasion of the sinusal mucosa and observation of fungal hyphae on surgical samples); biological criteria (hypereosinophilia, total and specific antifungal IgE); and radiological criteria. The combination of tomodensitometry (heterogeneous hyperdense and diffuse filling of nasosinusal cavities with expanded borders and a distended appearance of the bony wall), and MRI scan (extensive character and lesional signal [hypo and asignal T1 and T2], and cerebriform aspect of the fungal process) strongly suggests the diagnosis. It is important to distinguish the most typical forms, as well as unusual forms of allergic fungal rhinosinusitis. Main differential diagnoses are represented by other forms of fungal nasosinusal infections with different treatment and prognosis.

Reconsidering olfactory bulb magnetic resonance patterns in Kallmann syndrome.

OBJECTIVE: The aim of this retrospective study was to perform magnetic resonance imaging assessment of olfactory pathway and skull base abnormalities in Kallmann syndrome (KS) patients with hypogonadotropic hypogonadism and olfaction disorder. METHODS: Magnetic resonance brain patterns were retrospectively studied in 19 patients clinically classified as KS. Qualitative assessment of olfactory bulb region comprised bulb atrophy and rectus and medial orbital gyrus ptosis; quantitative assessment measured olfactory fossa depth and width, sulcus depth and ethmoid angle. Results were compared to an age- and sex-matched control population (n=19) with no impairment in the region of interest. Sixteen of the 19 KS patients were genetically screened for mutations associated with KS. RESULTS: On the above qualitative criteria, 15 of the 19 patients presented either unilateral (n=2) or bilateral (n=13) olfactory bulb agenesis; 16 showed tract agenesis and 16 showed gyrus malformation (ptosis or absence). On the quantitative criteria, 18 of the 19 patients showed abnormal sulcus depth and/or olfactory fossa malformation and/or abnormal ethmoid angle. CONCLUSION: The presence of malformation abnormalities in the olfactory fossae of 18 of the 19 patients appears to be a key factor for etiological diagnosis of hypogonadotropic hypogonadism, and should enable targeted study of genes involved in KS.

Superior Semicircular Canal Ampullae Dehiscence As Part of the Spectrum of the Third Window Abnormalities: A Case Study.

A 60-year-old man was referred to the ENT department for intense episodic vertigo triggered by loud sounds. Pure tone audiometry and otoneurological assessment, including videonystagmography using auditory stimulation and cervical vestibular evoked myogenic potential measures, conducted to the hypothesis of a third window syndrome in the left ear. Results from the high-resolution computed tomography of the petrous bone confirmed the hypothesis and revealed the presence of a submillimeter semicircular canal dehiscence, located between the left lateral and superior semicircular canal ampullae on the left side.

Cone Beam CT Versus Multislice CT: Radiologic Diagnostic Agreement in the Postoperative Assessment of Cochlear Implantation.

OBJECTIVE: To evaluate the diagnostic concordance between multislice computed tomography (MSCT) and cone beam computed tomography (CBCT) in the early postoperative assessment of patients after cochlear implantation. STUDY DESIGN: Prospective, randomized, single-center, interventional, pilot study on the diagnostic performance of a medical device. SETTING: Tertiary referral center. PATIENTS: Patients aged over 18 years requiring a computed tomographic (CT) scan after cochlear implant surgery. INTERVENTIONS: Nine patients were implanted with electrode arrays from three different manufacturers, including one bilateral. High-resolution MSCT and CBCT were then performed, and two experienced radiologists blinded to the imaging modality evaluated the randomized images, twice. MAIN OUTCOME MEASURES: Concordance between MSCT and CBCT for assessing the scalar position (tympani or vestibuli) of the electrodes. Secondary outcome measures were also studied: length of the intracochlear electrode array, percentage of implanted cochlea, number of intracochlear electrodes, and radiation doses. RESULTS: There was a good agreement between both CT scanners in determining the scalar position and estimating the number of implanted electrodes and percentage of implanted cochlea. CBCT had a lower radiation exposure. CONCLUSIONS: The CBCT appears to be a useful tool for postoperative assessment of cochlear implanted adult patients and is comparable to the conventional scanner in determining the scalar position, with lower radiation exposure.

Keep an Ear Out for Francisella tularensis: Otomastoiditis Cases after Canyoneering.

We report here three unusual cases of otomastoiditis due to Francisella tularensis, complicated by cervical abscesses and persistent hearing loss, plus facial paralysis for one patient. Intriguingly, the three patients had practiced canyoneering independently in the same French river, between 2009 and 2014, several days before clinical symptoms onset. The results point out that fresh water exposure may be a potential contamination route for tularemia. Besides, due to the frequent complications and sequelae, we believe that F. tularensis should be considered as a possible etiology in case of otitis media, failure of the conventional antibiotic treatment, and suspicious exposure of the bacteria.