Cochlear Implantation in Patients With Osteolytic Labyrinthitis: A Case Series.

OBJECTIVE: To describe the rare process of osteolytic labyrinthitis, previously referred to as labyrinthine sequestrum, which involves progressive obliteration of the bony and membranous labyrinth with eventual supplantation with soft tissue and, in some cases, bony sequestrum. PATIENTS: Three patients with diverse presentations of osteolytic labyrinthitis from two tertiary care academic medical centers. INTERVENTIONS: Case series report analyzing the relevant clinical, radiologic, pathologic, and surgical data on our patients with osteolytic labyrinthitis and comparing these index cases to the existing literature. MAIN OUTCOME MEASURES: We describe the varying image findings seen in osteolytic labyrinthitis on computed tomography and magnetic resonance imaging. Also, we report successful surgical intervention and hearing rehabilitation with cochlear implantation in patients with osteolytic labyrinthitis. RESULTS: Our three patients presented with profound sudden sensorineural hearing loss and vertigo consistent with labyrinthitis. None of the three patients had a history of chronic otitis media. Imaging workup revealed varying degrees of erosion to the otic capsule bone demonstrating the spectrum of disease seen in osteolytic labyrinthitis. Although two cases showed osteolytic changes to the semicircular canals and vestibule, the first case revealed frank bony sequestrum within the obliterated labyrinth. The three cases were taken for surgical debridement and cochlear implantation. CONCLUSIONS: We propose the new term, osteolytic labyrinthitis-previously referred to as labyrinthine sequestrum-to describe the rare spectrum of disease characterized by destruction of the osseous and membranous labyrinth and potential supplantation with bony sequestrum. Cochlear implantation is a viable option in selected patients with osteolytic labyrinthitis.

Signal Alteration of the Inner Ear on High-Resolution Three-Dimensional Constructive Interference in Steady State Sequence in Patients with Ménière's Disease and Labyrinthitis.

INTRODUCTION: The aim of this study is to evaluate signal alteration in the inner ear using three-dimensional (3D)-constructive interference in steady state (CISS) sequence in patients with Ménière's disease and labyrinthitis and its correlation with clinical and audiological parameters. METHODS: The medical records of the department of otorhinolaryngology were searched for patients with Ménière's disease or labyrinthitis who underwent MRI with 3D-CISS sequence. Blinded analysis of these patients and of MRI from control subjects without middle or inner ear symptoms was performed to detect any signal asymmetry of the inner ear structures. The results were correlated with clinical symptoms and results of audiological and vestibular tests. RESULTS: Fifty-eight patients with definite Ménière's disease and 5 patients with labyrinthitis as well as 41 control exams were included. A separate analysis was performed for patients with probable Ménière's disease (n = 68). A total of 172 3D-CISS sequences were analyzed by 2 blinded independent neuroradiologists. A CISS-hypointense signal of the inner ear structures was found in 3 patients with definite Ménière's disease (5.2%), in 4 patients with probable Ménière's disease (5.9%), and 2 patients with labyrinthitis (40%). No CISS hypointensity was found in the control group. Although no significant difference in symptoms or audiological test results was found between patients with and without this signal change, the side of hypointensity was frequently correlated with the symptomatic side and with hearing impairment. DISCUSSION/CONCLUSION: CISS hypointensity of the inner ear structures was evident in patients with clinical conditions other than vestibular schwannoma - more frequently in labyrinthitis than in Ménière's disease. This signal alteration was frequently encountered on the same symptomatic side as that of the pathological audiology tests, but it is not a predictor for hearing or vestibular impairment.

Etiology-Specific Mineralization Patterns in Patients with Labyrinthitis Ossificans.

BACKGROUND AND PURPOSE: Our aim was to identify whether specific patterns of ossification in labyrinthitis ossificans are associated with the known risk factors. Labyrinthitis ossificans has been described as sequela of prior temporal bone trauma, prior infection, and other disorders including sickle cell disease. Specific patterns of mineralization in the membranous labyrinth associated with these risk factors has not been previously described. MATERIALS AND METHODS: This was a retrospective study evaluating temporal bone CT scans at our institution from November 2005 to May 2018 in patients with labyrinthitis ossificans. Membranous labyrinthine structures evaluated for ossification included the following: basal, middle, and apical cochlear turns; lateral, posterior, and superior semicircular canals; and the vestibule for both ears in all patients. These structures were assigned a severity score, 0-4, based on degree of mineralization. Clinical records were reviewed for potential labyrinthitis ossificans risk factors. Basic descriptive statistics and a mixed model were used to correlate the degree and patterns of ossification with clinical history. RESULTS: Forty-four patients (58 ears) with labyrinthitis ossificans were identified and evaluated. The most common risk factors were chronic otomastoiditis (n = 18), temporal bone surgery (n = 9), temporal bone trauma (n = 6), sickle cell disease (n = 5), and meningitis (n = 4). For all etiologies, the semicircular canals were most severely affected, and the vestibule was the least. In patients with prior temporal bone surgery, significantly greater mineralization was seen in the basal turn of the cochlea (P = .027), the vestibule (P = .001), and semicircular canals (P < .001-.008). No significant pattern was identified in patients with meningitis, sickle cell disease, or trauma. CONCLUSIONS: Significant patterns of mineralization in labyrinthitis ossificans were observed in patients with prior temporal bone surgery. For all etiologies, the semicircular canals were most severely affected. No significant mineralization pattern was observed in patients with chronic otomastoiditis, meningitis, sickle cell disease, or prior temporal bone trauma.

Conductive hearing loss with a "dry middle ear cleft"-A comprehensive pictorial review with CT.

Conductive hearing loss (CHL) commonly results from middle ear fluid and inflammation (otitis media). Less commonly in patients with CHL, the middle ear cleft is well aerated or 'dry' with absence of soft tissue or fluid clinically and on imaging. There are numerous causes for this but they can be clinically challenging to diagnose. This pictorial review aims to illustrate and discuss the CT features of both common and less common causes of CHL in patients with a "dry middle ear cavity".

Differentiation Between Intralabyrinthine Schwannoma and Contrast-enhancing Labyrinthitis on MRI: Quantitative Analysis of Signal Intensity Characteristics.

OBJECTIVE: Distinguishing intralabyrinthine schwannoma (ILS) from labyrinthitis is crucial in deciding patient management between surgery and medication; however, the clinical and radiological differentiation between ILS and labyrinthitis is difficult, especially when labyrinthitis shows contrast-enhancement mimicking ILS on magnetic resonance imaging (MRI). This study aimed to evaluate the quantitative characteristics of signal intensity on 3T MRI for differentiating ILS from contrast-enhancing labyrinthitis (CEL). STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: Between July 2003 and June 2017, 9 subjects diagnosed with ILS (16 MRI cases) and 9 subjects diagnosed with CEL (9 MRI cases) were enrolled in the study. MAIN OUTCOME MEASURES: Normalized signal intensity on contrast-enhanced T1-weighted image (nCE-T1), normalized signal intensity on T2-weighted image (nT2), and normalized signal intensity on contrast-enhanced fluid-attenuated inversion recovery image (nFLAIR) were measured and compared between ILS and CEL. The diagnostic performance of these values was assessed using receiver operating characteristic (ROC) analysis. RESULTS: In ILS, the nCE-T1 and the nT2 were significantly higher and lower, respectively, than in CEL (all, p < 0.05); and the nFLAIR did not significantly differ (p > 0.05). The maximal nCE-T1 achieved the highest area under the curve (AUC) in differentiating ILS and CEL, followed by the mean nCE-T1, the mean nT2, and the maximal nT2. Combination of the nCE-T1 and the nT2 showed higher AUC than the nCE-T1 alone, but without statistical significance (p = 0.340). CONCLUSIONS: Quantitative measurement of the signal intensity on MRI can be a viable imaging tool for differentiating ILS from CEL.

Indications and radiological findings of acute otitis media and its complications. / Indicaciones y hallazgos radiológicos de la otitis media aguda y sus complicaciones.

Most cases of acute otitis media resolve with antibiotics and imaging is not required. When treatment fails or a complication is suspected, imaging plays a crucial role. Since the introduction of antibiotic treatment, the complication rate has decreased dramatically. Nevertheless, given the critical clinical relevance of complications, the importance of early diagnosis is vital. Our objective was to review the clinical and radiological features of acute otitis media and its complications. They were classified based on their location, as intratemporal or intracranial. Imaging makes it possible to diagnose the complications of acute otitis media and to institute appropriate treatment. Computed tomography is the initial technique of choice and, in most cases, the ultimate. Magnetic resonance is useful for evaluating the inner ear and when accurate evaluation of disease extent or better characterization of intracranial complications is required.

[The role of computer and magnetic resonance imaging in patients with deafness]. / Rol' komp'yuternoi i magnitno-rezonansnoi tomografii u patsientov s glukhotoi.

The objective of the present study was to evaluate the potential of CT and MRI for diagnostics of congenital and acquired pathology of the inner ear in the deaf patients. Two groups of the patients were examined. The first group consisted of 75 patients with congenital or acquired deafness etiology. The second group was comprised of 75 patients with deafness associated with acute bacterial meningitis suffered in the preceding period. All the patients were examined by CT and MRI of temporal bones. The results of the study provided a basis for the development of indications for the application of CT and MRI to examine the patients presenting with hearing loss and deafness. CONCLUSION: CT and MRI make it possible to identify individual features of the temporal bone structure significant for the surgical treatment. MRI appears to have an advantage over CT for diagnostics of early obliteration of the cochlea. Both CT and MRI are the optional methods for the examination of the patients with deafness developing after meningitis.

The incidence of tympanogenic labyrinthitis ossificans.

OBJECTIVE: To estimate the incidence of tympanogenic labyrinthitis ossificans. METHODS: The records of patients treated with mastoidectomy for various tympanogenic aetiologies from January 2007 to December 2011 were retrospectively reviewed. Patients whose high-resolution computed tomography scans showed evidence of labyrinthine calcification of the temporal bone were enrolled. Patients with a history of head and neck cancer, meningitis, and otosclerosis, and patients with cochlear implants, were excluded from this study. RESULTS: A total of 195 patients were enrolled in this study; 4 of the patients presented with calcification in the inner ear. Therefore, the incidence of tympanogenic labyrinthitis ossification was 2 per cent. The computed tomography findings revealed: (1) cochlear calcifications of the basal and middle turn in two patients; and (2) vestibular, superior semicircular canal, posterior semicircular canal and lateral semicircular canal calcification in one, four, three and two patients, respectively. CONCLUSION: The incidence of tympanogenic labyrinthitis ossification in patients who had undergone a mastoidectomy was 2 per cent.

Semicircular canal dehiscence: frequency and distribution on temporal bone CT and its relationship with the clinical outcomes.

PURPOSE: In this study, we aimed to investigate the frequency of SCD and its distribution and relationship with clinical outcomes on thin-section CT of the temporal bone. MATERIALS AND METHODS: Digital temporal bone CT images of 850 consecutive patients (1700 temporal bone CTs, 5100 SCs) who presented with a range of complaints such as vertigo, deafness, ear pain, fullness, and discharge between January 2008 and December 2011 were re-evaluated. Axial and oblique coronal reconstruction images of the temporal bone were made with a reconstruction thickness of 0.5mm. Additionally, superior SC was evaluated in two perpendicular planes. RESULTS: Out of 850 patients, 70 had completely normal temporal bone CT. Ninety-three patients had at least one SCD. In the temporal bone-based evaluation, 119 (26 bilateral, 67 unilateral) of 1700 temporal bones (7%) showed dehiscence. The SC-based evaluation revealed 125 SCD (2.5%) in 5100 SCs. The total number and rates of SCD were as follows: superior 103 (82.4%), posterior 13 (10.4%), and lateral nine (7.2%). Twenty of the 93 patients with SCD (21.5%) revealed no other findings on their temporal bone CTs. We determined a significant correlation between vestibular complaints, conductive hearing loss and SCD but there was no correlation between mixed, sensorineural hearing loss and SCD. CONCLUSION: We determined the frequency of SCD in 11% of patients and 7% of temporal bones. With regards to the distribution, the superior SC showed the highest dehiscence rate (82.4%). We found a significant correlation between vestibular symptoms, conductive hearing loss and SCD.

Temporal bone high-resolution computed tomography in non-syndromic unilateral hearing loss in children.

OBJECTIVE: The aim of this study was to disclose possible inner ear abnormalities/pathologies by means of high-resolution computed tomography (HRCT) of the temporal bone (TBHRCT) in children with unilateral hearing loss (UHL). METHODS: Retrospective review of audiological evaluation and TBHRCT in 22 children with UHL. RESULTS: Two thirds of the children showed profound hearing loss. Review of HRCT scans identified inner ear malformations/pathologies in 9 (41%) cases and a high jugular bulb (HJB), always dehiscent with the vestibular aqueduct, in another 5 (22%). Inner ear malformations included enlarged vestibular aqueduct, common cavity and cochleovestibular hypoplasia, while labyrinthine ossification was the detected pathology. In 1 child, the common cavity of the right ear was associated with congenital melanocytic naevus of the left eyelid and lipomeningocele. To the best of our knowledge, this condition has never been described. CONCLUSIONS: The aetiology of UHL may be revealed in more than half of patients by means of TBHRCT. Besides common inner ear abnormalities, TBHRCT should be evaluated carefully to rule out HJB, dehiscences, diverticulum or erosion of inner ear structures.

Semicircular canal dehiscence in HR multislice computed tomography: distribution, frequency, and clinical relevance.

The literature about bony defects in the semicircular canal system is highly inconsistent. Therefore, we analyzed a series of 700 high-resolution multislice CT examinations of the temporal bone for semicircular canal dehiscencies. An unselected group of ENT patients with different clinical symptoms and variable age was chosen. We found semicircular canal dehiscence in 9.6% of temporal bones, superior semicircular canal was affected mostly (8%), less common posterior semicircular canal (1.2%); only in 3 cases (0.4%), lateral semicircular canal showed dehiscence. In 60% of SSC dehiscence, we registered bilateral manifestation. The so-called "third mobile window" in semicircular canal dehiscence causes a great variety of clinical symptoms like vertigo, nystagmus, oscillopsies, hearing loss, tinnitus and autophonia. Comparison with anatomic studies shows that CT examination implies the risk of considerable overestimation; this fact emphasizes the important role of clinical and neurophysiological testing.

Acoustic brainstem implant in a post-meningitis deafened child-Lessons learned.

We report a case of a deafened child with advanced labyrinthitis ossificans resulting from meningo-encephalitis. She received a cochlear implant in the right ear, following a drill-out procedure. Post-implant hearing outcomes were satisfactory initially, but deteriorated over time as a result of partial electrode migration. The child subsequently received a left auditory brainstem implantation with improvement of hearing outcomes. Post-operatively, a sub-dural hematoma developed not on the side of the operation but on the opposite side. Simultaneous use of the cochlear implant on one side and the acoustic brainstem on the other, aggravated the non-auditory side effects of the ABI and compromised its potential for optimal hearing results.

Posttraumatic labyrinthitis ossificans with perilymphatic fistulization.

Labyrinthitis ossificans is fibrosis or ossification of the membranous labyrinth. Tympanogenic, meningogenic, and hematogenous etiologies are more common than trauma in the development of labyrinthitis ossificans. We present a case complaining of right-sided hearing loss and symptoms of otitis media and positional vertigo resulting from perilymphatic fistulization. Imaging revealed labyrinthitis ossificans secondary to temporal bone fracture crossing through the otic capsule.

Imaging of inflammatory and infectious diseases in the temporal bone.

Inflammatory and infectious diseases of the temporal bone are a major indication to perform high-resolution CT and MR imaging studies. Such studies allow one to evaluate the extent of the disease in the soft tissues and in the bony structures of the temporal bone. On these same imaging studies the possible extension of the infection to surrounding regions is visualized. In this article a segmental approach is used, focusing on four structures in the temporal bone: the external ear, the otomastoid and petrous apex, the inner ear, and the facial nerve. For each of the four sections imaging findings are described and illustrated, and if relevant a differential diagnostic approach is highlighted.

[Neuroradiological imaging in patients with sensorineural hearing loss prior to cochlear implantation]. / Neuroradiologische Diagnostik bei Patienten mit sensorineuralem Hörverlust vor Cochlea-Implantation.

Cochlear implantation (CI) is an established technique for enabling speech perception in patients suffering from severe bilateral sensorineural hearing loss (SNHL). Thorough preoperative radiological assessment is essential for correctly evaluating the indication for surgery and safely performing cochlear implantation. CT and conventional and functional MRI are available for radiological assessment. Therefore, knowledge of the most frequent congenital syndromal, non-syndromal, and acquired malformations of inner ear structures is fundamental. This article provides information about imaging techniques prior to CI and relevant malformations of the inner ear. Safety aspects for patients with a cochlear implant undergoing MR imaging are also discussed.