Temporal Bone Magnetic Resonance Imaging Research Articles

Diagnostic performance of multishot echo-planar imaging (RESOLVE) and non-echo-planar imaging (HASTE) diffusion-weighted imaging in cholesteatoma with an emphasis on signal intensity ratio measurement.

To evaluate the diagnostic efficacy of multishot echo-planar imaging (EPI) [RESOLVE (RS)] and non-EPI (HASTE) diffusion-weighted imaging (DWI) in detecting cholesteatoma (CHO), and to explore the role of signal intensity (SI) ratio measurements in addressing diagnostic challenges. We analyzed RS-EPI and non-EPI DWI images from 154 patients who had undergone microscopic middle ear surgery, with pathological confirmation of their diagnoses. Two radiologists, referred to as Reader A and Reader B, independently reviewed the images without prior knowledge of the outcomes. Their evaluation focused on lesion location, T1-weighted (T1W) signal characteristics, and contrast enhancement in temporal bone magnetic resonance imaging. Key parameters included lesion hyperintensity, size, SI, SI ratio, and susceptibility artifact scores across both imaging modalities. Of the patients, 62.3% (96/154) were diagnosed with CHO, whereas 37.7% (58/154) were found to have non-CHO conditions. In RS-EPI DWI, Reader A achieved 89.6% sensitivity, 79.3% specificity, 87.8% positive predictive value (PPV), and 82.1% negative predictive value (NPV). Non-EPI DWI presented similar results with sensitivities of 89.6%, specificities of 86.2%, PPVs of 91.5%, and NPVs of 83.3%. Reader B's results for RS-EPI DWI were 82.3% sensitivity, 84.5% specificity, 89.8% PPV, and 74.2% NPV, whereas, for non-EPI DWI, they were 86.5% sensitivity, 89.7% specificity, 93.3% PPV, and 80% NPV. The interobserver agreement was excellent (RS-EPI, κ: 0.84; non-EPI, κ: 0.91). The SI ratio measurements were consistently higher in non-EPI DWI (Reader A: 2.51, Reader B: 2.46) for the CHO group compared with RS-EPI. The SI ratio cut-off (>1.98) effectively differentiated hyperintense lesions between CHO and non-CHO groups, demonstrating 82.9% sensitivity and 100% specificity, with an area under the curve of 0.901 (95% confidence interval: 0.815-0.956; P < 0.001). Susceptibility artifact scores averaged 1.18 ± 0.7 (Reader A) and 1.04 ± 0.41 (Reader B) in RS-EPI, with non-EPI DWI recording a mean score of 0. Both RS-EPI and non-EPI DWI exhibited high diagnostic accuracy for CHO. While RS-EPI DWI cannot replace non-EPI DWI, their combined use improves sensitivity. SI ratio measurement in non-EPI DWI was particularly beneficial in complex diagnostic scenarios. This study refines CHO diagnostic protocols by showcasing the diagnostic capabilities of both RS-EPI and non-EPI DWI and highlighting the utility of SI measurements as a diagnostic tool. These findings may reduce false positives and aid in more accurate treatment planning, offering substantial insights for clinicians in managing CHO.

High Prevalence of Cochlear Nerve Deficiency in Pediatric Patients With Cochlear Aperture Stenosis.

Cochlear nerve deficiency (CND) is a common radiologic finding among unilateral sensorineural hearing loss (USNHL) patients. It is generally detected with magnetic resonance imaging (MRI), which is associated with higher cost, less availability, and possible need for sedation. Therefore, identifying computed tomography (CT) findings, such as cochlear aperture stenosis (CAS), that can reliably predict CND is valuable. Our study aimed to determine the prevalence of CND in pediatric patients with CT-diagnosed CAS. Retrospective study. Tertiary care center. We included pediatric patients diagnosed with CAS on temporal bone CT and with available temporal bone MRI. For each patient, an otolaryngologist and a pediatric neuroradiologist measured the cochlear aperture width on CT to confirm CAS (cochlear aperture < 1.4 mm) and assessed the status of the cochlear nerve on MRI. Fifty-five patients, representing 65 ears, had CAS on CT measurement. Median cochlear aperture width in CAS ears was 0.70 mm (interquartilerange [IQR]: 0.40-1.05 mm) versus 2.00 mm in non-CAS ears (IQR: 1.80-2.30 mm, P < .001). CND was found in 98.5% (n = 64/65) of CAS ears, while a normal cochlear nerve was found in 1.5% (n = 1/65) of CAS ears. CND is highly prevalent among pediatric patients with CAS. This suggests that MRI may not be needed to assess for CND in USNHL patients with CAS, as initial CT may provide sufficient information to determine cochlear implant candidacy. We recommend thoughtful shared decision-making with parents of USNHL patients when determining whether to pursue MRI in the setting of a CAS diagnosis.

특발성 안면신경 마비 예후 예측 도구로서 측두골 자기공명영상의 유용성

Background: In facial palsy patients, electroneuronography (ENoG) was used for the diagnosis of the prognosis of facial palsy. This study aimed to determine whether temporal bone magnetic reso-nance imaging (TBMR) was useful for diagnosing and predicting prognosis in facial palsy. Materials and Methods: Among the patients diagnosed with facial palsy, 112 patients who underwent TBMR were included. These patients were divided into three groups according to the enhancement region of the facial nerve in TBMR. Fifty-five patients showed contrast enhancement in the meatal or labyrinthine segment (group 1), 36 patients showed enhancement in the geniculate ganglion (group 2), and 21 patients showed enhancement in the tympanic or mastoid segment (group 3). The initial facial palsy symptoms and prognosis were compared between the three groups. Results: In group 1, 26 patients showed House-Brackmann grade (H-B grade) IV, V initially, and 13 patients and 12 patients showed H-B grade IV, V initially in group 2 and group 3, respectively. Moreover, the initial ENoG results (%) between the three groups did not significantly differ. Patients who took more than 6 months to recover after systemic steroid therapy were 5 patients in group 1, 3 patients in group 2, and 2 patients in group 3. Conclusions: In TBMR of the patients with facial nerve palsy, the most contrast enhancement parts of the facial nerve were the meatal and labyrinthine segments. Enhancement of those segments showed severe initial symptoms clinically. In addition, most of them recovered within 6 months. However, there was no statistical significance, so further studies are needed for more cases and ob-servation periods.

Single Sequence Magnetic Resonance Imaging in Serous Otitis Media.

We evaluated the usability of short-term (approximately 3 minutes) T2 sequence temporal bone magnetic resonance imaging (MRI) in the diagnosis of serous otitis media (SOM) in our study. A prospective study. Otoscopic examination and audiometry-tympanometry were performed on all 73 patients included in the study. All patients underwent short-term T2 sequence temporal bone MRI before the paracentesis procedure. The mean age of 73 patients (30 femaleand 43 male) was 7.78 ± 3.01 (3 to 17 years). A total of 134 ear paracentesis operations were performed. As a result of the intraoperative paracentesis procedure, 107 Type B tympanogram and 13 Type C tympanogram were found out of 120 ears that had fluid in the middle ear. Out of 14 ears without fluid flow in the middle ear, five were found to be Type B tympanogram and nine to be Type C tympanogram. The sensitivity of the type B tympanogram in the diagnosis of SOMwas 89.1%, the specificity was 64.2%, the positive predictive value was 95.5%, and the negative predictive value was 40.9%. The sensitivity and specificity of short-term T2 sequence MRI in diagnosing SOM were found to be 100% and 100%. Among the available methods, the short-term T2 sequence temporal MRI is the most effective method for evaluating fluid in mastoid cells.

Features of Audio-Vestibular Deficit and 3D-FLAIR Temporal Bone MRI in Patients with Herpes Zoster Oticus.

Herpes zoster oticus (HZO) is characterized by otalgia and erythematous vesicles in the auricle or external auditory canal. Ramsay Hunt syndrome (RHS) can be diagnosed when facial nerve palsy is accompanied by these symptoms of HZO, and in this case, audio-vestibular symptoms such as hearing loss or dizziness often develop. Recently, 3D-fluid-attenuated inversion recovery sequence (3D-FLAIR) magnetic resonance imaging (MRI) has been introduced in order to evaluate the inner ear structure pathology. The purpose of this study was to investigate the audio-vestibular characteristics in correlation with temporal bone MRI findings in HZO patients. From September 2018 to June 2022, 18 patients with HZO participated in the study. Thirteen patients (77%) showed high-signal intensity in the inner ear structures in 4 h post-contrast 3D-FLAIR images. In a bithermal caloric test, the lateral semicircular canal showed high signal intensity in 4 h post-contrast 3D-FLAIR images in 75% of patients with abnormal canal paresis. While the cochlea showed high signal intensity in 4 h post-contrast 3D-FLAIR images in 75% of patients with hearing loss, the vestibulo-cochlear nerve showed enhancement in post-contrast T1-weighted images in only 33% of patients with hearing loss. The present study demonstrates that audio-vestibular deficits are well-correlated with increased signal intensity of the inner ear endorgans in 4 h post contrast 3D-FLAIR MRI.

Unusual Neurological Manifestation of Langerhans Cell Histiocytosis in an Adult.

Langerhans Cell Histiocytosis is a rare disorder characterized by an abnormal proliferation of Langerhans cells in one or multiple organs. It rarely presents with a central vestibular involvement. We report a case of a multisystem Langerhans Cell Histiocytosis with mucosal, hepatic, and neurological involvement, presenting with dizziness and balance disorders. A 39-year-old woman with a histopathologically confirmed mucosal palatal Langerhans Cell Histiocytosis presented with a history of dizziness for a year. Vestibular examination revealed a saccadic eyes pursuit, an up beating spontaneous nystagmus, a bilateral gaze nystagmus and a prolonged positional nystagmus, in the supine roll test. Pure tone audiometry showed a slight left sensorineural hearing loss at the 8000Hz frequency. Computed tomography (CT) scan showed a bilateral maxillary sinus fullness and a peripheral osteosclerosis of the surrounding bony walls. Hepatic magnetic resonance imaging (MRI) showed a typical hepatic involvement with a hepatomegaly with countless cysts. Temporal bone CT scan and MRI were normal. Cerebral MRI showed an hyperintense nodular signal at T2 FLAIR weighted images lateral to the right pons, at the level of the left middle cerebellar peduncle and at the left mesencephalon. Balance disorders can rarely present a sign of a degenerative neurological cerebellar involvement. Such a rare manifestation can present in different neurological disorders such as Langerhans' cell Histiocytosis.

Cochlear implantation for post-meningitis deafness with cochlear ossification: diagnosis and surgical strategy

Background Cochlear ossification (CO) after meningitis can cause profound sensorineural hearing loss (SNHL). Cochlear implantation (CI) is the ideal treatment strategy for CO. Aims To explore the strategy for CI in patients with CO after meningitis. Materials and Methods In this retrospective study, the medical records of patients diagnosed with profound SNHL due to CO after meningitis and who underwent CI in our department between September 2010 and September 2021 were collected and reviewed. Their imaging and surgical findings were analyzed. Results The data of 26 patients with unilateral CI were reviewed. All patients underwent preoperative temporal high-resolution computed tomography (HRCT) and 22 patients magnetic resonance imaging (MRI). The sensitivity of HRCT was 61.5% (10/26), whereas that of MRI was 81.8% (18/22). Combined HRCT and MRI achieved a detection rate of 92.3% (24/26). Twenty-two and four patients underwent complete and partial electrode implantations, respectively. Conclusions and Significance Preoperative temporal bone HRCT and MRI are essential for determining whether a patient is suitable for CI and surgical planning. A false-negative diagnosis is possible when diagnosing CO, but combined HRCT and MRI can improve the positive rate of preoperative diagnosis of CO post meningitis. Early CI is recommended.

Direction-changing spontaneous nystagmus in cerebellopontine angle tumour

The most common symptoms of tumours involving the cerebellopontine angle (CPA) are unilateral sensorineural hearing loss, dizziness, and asymmetric tinnitus. While the clinical manifestations have been well documented in previous studies, the nystagmus findings in these patients have not been thoroughly investigated yet. This study aimed to investigate the incidence of direction-changing spontaneous nystagmus in patients with CPA tumours, evaluate their radiologic characteristics, and gain insight into the mechanisms underlying nystagmus. Direction-changing spontaneous nystagmus was observed in 6 out of 83 patients (7%) with CPA tumours during the 7-year period. Temporal bone magnetic resonance imaging findings revealed the presence of an intrameatal mass in CPA tumours in all six patients with direction-changing spontaneous nystagmus. Vestibular schwannomas were confined within the internal auditory meatus in four patients, and petroclival meningiomas extended into the internal auditory meatus in two patients. The mechanism of direction-changing spontaneous nystagmus may be explained as paroxysmal secondary central hyperactivity in the vestibular nucleus due to the long-standing pressure effect in the vestibular nerve by tumours, or by ephaptic discharges in the vestibular nerve.

Clinical usefulness of labyrinthine three-dimensional fluid-attenuated inversion recovery magnetic resonance images in idiopathic sudden sensorineural hearing loss.

Literature on the use of current magnetic resonance imaging (MRI) for patients with idiopathic sudden sensorineural hearing loss (ISSNHL) is reviewed, emphasizing the role of three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) MRI. The discussion focuses on the diagnostic role of temporal bone MRI using 3D-FLAIR and the relationship between MRI findings, clinical symptoms, and hearing outcome. The currently suggested MRI protocol for SSNHL includes a 3D T2-weighted steady-state free procession sequence or its equivalent, pre and postcontrast T1-weighted, and pre and postcontrast 3D-FLAIR sequences. The 3D-FLAIR image identifies an underlying labyrinthine condition in 24-57% of patients with ISSNHL, contributing to understanding the pathophysiologic mechanisms (e.g., labyrinthitis or labyrinthine hemorrhage). Recent studies demonstrated consistent results that initial hearing loss could be related to the signal change on the 3D-FLAIR image. Various results on 3D-FLAIR image value prediction for the final hearing outcome were shown. 3D-FLAIR MRI application identifies an underlying labyrinthine condition. Abnormal MRI findings correlate with initial hearing loss and accompanying symptoms and hearing outcome. Performing temporal bone MRI with 3D-FLAIR sequence may clarify probable ISSNHL pathophysiology, improve diagnostic accuracy, provide prognostic information to physicians, and possibly guide toward a more specific treatment.

Construction of an MRI-based decision tree to differentiate autoimmune and autoinflammatory inner ear disease from chronic otitis media with sensorineural hearing loss

Autoimmune and autoinflammatory inner ear diseases (AIED/AID) are characterized by the symptom of sensorineural hearing loss (SNHL). To date, standardized diagnostic tools for AIED/AID are lacking, and clinically differentiating AIED/AID from chronic otitis media (COM) with SNHL is challenging. This retrospective study aimed to construct a magnetic resonance imaging (MRI)-based decision tree using classification and regression tree (CART) analysis to distinguish AIED/AID from COM. In total, 67 patients were enrolled between January 2004 and October 2019, comprising AIED/AID (n = 18), COM (n = 24), and control groups (n = 25). All patients underwent 3 T temporal bone MRI, including post-contrast T1-weighted images (postT1WI) and post-contrast FLAIR images (postFLAIR). Two radiologists evaluated the presence of otomastoid effusion and inner ear contrast-enhancement on MRI. A CART decision tree model was constructed using MRI features to differentiate AIED/AID from COM and control groups, and diagnostic performance was analyzed. High-intensity bilateral effusion (61.1%) and inner ear enhancement (postFLAIR, 93.8%; postT1WI, 61.1%) were the most common findings in the AIED/AID group. We constructed two CART decision tree models; the first used effusion amount as the first partitioning node and postT1WI-inner ear enhancement as the second node, whereas the second comprised two partitioning nodes with the degree of postFLAIR-enhancement of the inner ear. The first and second models enabled distinction of AIED/AID from COM with high specificity (100% and 94.3%, respectively). The amount of effusion and the degree of inner ear enhancement on MRI may facilitate the distinction between AIED/AID and COM with SNHL using decision tree models, thereby contributing to early diagnosis and intervention.

Mastoid effusion on temporal bone MRI in patients with Bell\u2019s palsy and Ramsay Hunt syndrome

This study aimed to investigate the incidence of mastoid effusion on temporal bone magnetic resonance imaging (MRI) in patients with Bell’s palsy (BP) and Ramsay Hunt syndrome (RHS), and evaluate the usefulness of mastoid effusion in early differential diagnosis between BP and RHS. The incidence of mastoid effusion on 3.0 T—temporal bone MRI, which was conducted within 10 days after the onset of acute facial nerve palsy, was compared between 131 patients with BP and 33 patients with RHS. Findings of mastoid cavity on temporal bone MRI were classified into three groups as normal mastoid, mastoid effusion, and sclerotic change, and the incidence of ipsilesional mastoid effusion was significantly higher in RHS than BP (P < 0.001). Tympanic membrane was normal in 7 of 14 RHS patients with mastoid effusion, and injected without middle ear effusion in 7 patients. This study highlights significantly higher incidence of ipsilesional mastoid effusion in RHS than BP, and suggests that the presence of mastoid effusion may provide additional information for differential diagnosis between RHS and BP.

The Diagnostic Efficacy of MRI in the Evaluation of the Enlarged Vestibular Aqueduct in Children with Hearing Loss.

The aim of our study is to evaluate the diagnostic effectiveness of magnetic resonance imaging (MRI) compared to computed tomography (CT) in the detection of enlarged vestibular aqueduct (EVA) in childhood. One hundred twenty-three children who underwent temporal bone CT and MRI examinations for hearing loss between 2013 and 2020 were evaluated retrospectively. All CT and MRI images were examined by two pediatric radiologists, according to the Valvassori and Cincinnati criteria for EVA. Imaging findings on CT and MRI of the vestibular aqueduct were recorded. Two pediatric radiologists performed the measurements for EVA on CT and MRI. In addition, an otolaryngologist performed the measurements independently. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of MRI compared to CT were calculated to detect EVA. The difference between the measurements on CT and MRI was investigated. The inter-observer agreement was evaluated for measurements. The mean age of 123 children (65 boys and 58 girls) was 50.18±50.40 months. Two hundred forty-six ears were evaluated in 123 children. On CT images, EVA was present in 28 (11.3%) of 246 ears according to Cincinnati criteria and 27 (10.9%) of 246 ears according to Valvassori criteria, respectively. While sensitivity, specificity, PPD, and NPD rates of MRI were 100%, 99%, 92.8%, and 100%, respectively, for Cincinnati criteria, for Valvassori criteria, they were 100%, 97.3%, 77.7%, and 100%, respectively. According to the visual evaluation performed without using measurement, the enlarged appearance of the vestibular aqueduct was significant for the diagnosis of EVA (p<0.001), while the absence of this appearance was significant for the exclusion of EVA (p<0.001). There was no significant difference between the measurements on CT and MRI. There was a perfect correlation between the observers for measurements. MRI can be used as an initial imaging technique in children with suspicion of EVA to reduce radiation exposure.

The role of computed tomography and magnetic resonance imaging for preoperative pediatric cochlear implantation work-up in academic institutions

Objective The goal of the study is to investigate the association of pertinent preoperative temporal bone computed tomography (CT) and brain magnetic resonance imaging (MRI) results and intraoperative surgical findings and complications of pediatric cochlear implantation reported in academic settings. Methods This is a retrospective review of cochlear implant patients who received a pre-operative temporal bone CT and MRI of the brain between 2005 and 2012 at academic pediatric otolaryngology practices within children’s hospitals in the United States and France. Scans were reviewed in a double-blind fashion and compared to intraoperative findings. Results 91 children were analyzed (mean age 5.54 +/− 0.58 years). A small facial recess identified on CT was associated with difficult insertion of electrodes (P = 0.0003). A prominent sigmoid sinus noted on CT was associated of difficult insertion of electrodes (P = 0.01), iatrogenic tegmen dehiscence (P = 0.005), as well as difficult round window access (P = 0.025). No specific CT finding was found to be associated with external auditory canal injury, perilymphatic gusher, or iatrogenic facial nerve injury. MRI brain and internal auditory canal findings were not predictive of surgical outcomes. Conclusions Preoperative CT and MRI remain an important planning tool for pediatric cochlear implantation, particularly in academic institutions. The findings of our study demonstrate that a detailed assessment of both preoperative CT and MRI are valuable for teaching and surgical planning.

The Importance of the Temporal Bone 3T MR Imaging in the Diagnosis of Menière's Disease.

The aim of this study was to evaluate endolymphatic hydrops using the 3T temporal bone magnetic resonance imaging (MRI), performed according to the chosen protocol, and determine whether it could be applied as an objective diagnostic tool for Menière's disease. 105 participants diagnosed with probable (n = 50) and definite (n = 55) Menière's disease were included in this prospective study at Vilnius University Hospital, Santaros Clinics. Audiometry, vestibular function tests, videonystagmography, and computer posturography were performed before MRI. The 3T MRI with gadolinium contrast was performed to evaluate the endolymphatic hydrops. Imaging protocol consisted of 3D-FLAIR and 3D T2DRIVE sequences. Vestibular endolymphatic sac was interpreted as enlarged if occupied more than 50% of the vestibular area. 78.1% of subjects had abnormal MRI findings other than hydrops, and it was more than 90% (50/55) of patients in the definite MD group (p < 0.001). Changes in caloric test were observed in 63.8% of subjects in general, and in 76.4% of patients with a definite Menière's disease. The side of the endolymphatic hydrops observed on MR imaging corresponded to the clinical diagnosis of the Menière's disease based on the results of audiometry (p < 0.001) and unilateral weakness (p < 0.001). Endolymphatic hydrops on MRI and directional preponderance in caloric test were two independent predictors of the definite Menière's disease. Temporal bone 3T MRI with gadolinium contrast is clinically superior to confirm the diagnosis of Menière's disease. Grade II endolymphatic hydrops on MRI, directional preponderance, and unilateral weakness on caloric test were independent predictors for the definite Menière's disease.

Utility of Non-EPI DWI MRI in the Management of Pediatric Cholesteatoma

Abstract Introduction Cholesteatoma in children can be acquired or congenital and is generally extensive. The most commonly used technique for the removal of cholesteatoma is intact canal wall mastoidectomy. This invariably leads to second-look surgery to assess for recurrence or residual cholesteatoma. Magnetic resonance imaging (MRI) with non–echo-planar imaging (non-EPI) diffusion-weighted imaging (DWI) is an accurate noninvasive imaging option that can be used in diagnosing primary cholesteatoma. This can also be used to diagnose residual or recurrent cholesteatoma in patients who have undergone intact canal wall mastoidectomy. Case Report A 7-year-old male patient presented with a 1-week history of foul-smelling discharge from the right ear. On examination, a polypoidal mass with keratinized debris was seen. A diagnosis of cholesteatoma of right ear was made. Imaging with high-resolution computed tomography (HRCT) of the temporal bone and non-EPI DWI MRI confirmed the same. The child underwent an intact canal wall mastoidectomy and was followed up at 2 years with non-EPI DWI MRI to monitor any residual or recurrent cholesteatoma. Conclusion Non-EPI DWI MRI provides a convenient, accurate, and noninvasive method of evaluating and monitoring for residual or recurrent cholesteatoma and thus helps avoid unnecessary second-look surgery.

The relation between idiopathic subjective tinnitus and risk factors for venous and arterial thrombosis

Aim: The aim of this study was to investigate the relationship between tinnitus and the factors that cause the tendency to thrombosis in terms of gender.Material and Methods: Fifty-five consecutive patients who admitted to our clinic with the complaint of idiopathic subjective tinnitus were included in this study. Routine ENT (ear nose throat) examinations and pure tone audiometry test were performed. Prothrombin time (PT), partial thromboplastin time (PTT), total cholesterol, low-density lipoprotein (LDL) cholesterol, high-density lipoprotein (HDL) cholesterol, Triglycerides, antithrombin III (AT III), protein C, protein S, homocysteine, antiphospholipid and anticardiolipin antibodies were examined in blood samples of patients. Temporal bone magnetic resonance imaging (MRI) was performed all patients. The results of the patients were compared in terms of gender and age groups (40 age).Results: The study was completed with 47 patients. Twenty-four of the patients were female and 23 were male. Both genders groups were similar in terms of age and pure tone average. Antiphospholipid and anticardiyolipin antibodies (IgG and IgM) were found as negative in all patients. Homocysteine levels were found to be statistically significantly higher in males than females (p=0.015; respectively 13.38±5.98 µmol/L and 9.87±3.11 µmol/L). AT III and Protein S levels were lower in males than females although this difference was not statistically significant (respectively p=0.07 and p=0.08)Conclusion: In this study, an association was shown between hyperhomocysteinemia and tinnitus, especially in males. Keywords: Tinnitus; tinnitus of vascular origin; thrombosis; gender role.

아놀드 신경에서 발생한 외이도의 신경초종 1예

Neurilemmoma is a benign tumor that originates from the Schwann cell. It frequently occurs in head and neck regions, but neurilemmoma of external auditory canal is extremely rare. Here, we report a very rare case of 36-year-old male with a tumor located in the posterior wall of external auditory canal, preoperatively suspected as neurilemmoma originated from Arnold’s nerve by temporal bone magnetic resonance imaging. The tumor mass was surgically excised and pathologically confirmed as a neurilemmoma (J Clinical Otolaryngol 2018;29:245-249)

Spontaneous Labyrinthine Hemorrhage: A Case Series.

To describe patient characteristics, audiometric outcomes, and magnetic resonance imaging (MRI) signal patterns in patients with suspected labyrinthine hemorrhage. Retrospective review. Tertiary medical center. Radiology database was queried for terms related to labyrinth hemorrhage or labyrinthitis and then selected for patients in which labyrinthine hemorrhage was suspected in the report. Eleven patients were identified and all treated at our institution. Blinded assessment of temporal bone MRI by 2 experienced neuroradiologists was performed and interrater reliability assessed. Patient demographics, medical comorbidities, and audiometric outcomes are described. Of the 11 patients identified, the median patient age was 60 years; 7 were female and 4 male. Ten of 11 patients presented with unilateral sudden sensorineural hearing loss (SNHL), and 8 of 11 had associated vertigo. One patient experienced vertigo without hearing loss. Of those presenting with sudden SNHL, 82% were left with nonserviceable American Academy of Otolaryngology-Head and Neck Surgery class D hearing. Interrater reliability for detecting T1 signal abnormalities was moderate but very good for detecting fluid attenuation inversion recovery (FLAIR) signal abnormalities. Most patients had existing hypertension. Average follow-up was 13.3 months. We present the largest cohort of patients with radiographic diagnosis of labyrinthine hemorrhage using T1 and FLAIR signal abnormalities on MRI. Most patients presented with a profound unilateral sudden SNHL that did not recover. Our findings are consistent with prior reports that abnormal FLAIR signal on MRI is a reliable marker for detecting inner ear injury and can potentially be used as a marker for poor prognosis.

Evaluation of the Vestibular System and Etiology in Children with Unilateral Sensorineural Hearing Loss.

The aim of this study was to evaluate the vestibular system of children with unilateral sensorineural hearing loss (USNHL), investigate the etiological factors of USNHL and analyze whether a genetic predisposition exists. Thirty-three children aged less than 18 years with USNHL, who visited the ear, nose, and throat (ENT) department between January 2004 and December 2012, were included in this study. Cases with conductive hearing loss were excluded from the study. The patients were subjected to etiologic, genetic, and ophthalmologic evaluation; radiologic imaging; electronystagmography (ENG); and vestibular evoked myogenic potential (VEMP) tests. The control group, which included 25 healthy children (13 males and 12 females), had undergone audiological assessment and were subjected to ENG and VEMP tests. All of the patients had severe-to-profound hearing loss. Mumps immunoglobulin G was positive in 22 (66.7%) of 33 patients. The 35delG mutation was not found in any of the patients. All of the patients underwent temporal computed tomography (CT) and magnetic resonance imaging (MRI). Inner ear anomaly was present in 51.5% of the patients. Overall, 21 of 31 ENG patients had canal paresis in the affected ear. The VEMP response was absent on the affected side in three patients. The n23 latency average of the patient group was longer than that of the control group. Because USNHL causes irreversible problems in children, early diagnosis and auditory rehabilitation are very important. As USNHL is accompanied by inner ear anomaly, children with USNHL should undergo temporal bone CT and MRI. To evaluate the vestibular system, ENG and VEMP are non-invasive and diagnostic tests.

Outcomes of evaluation and testing of 660 individuals with hearing loss in a pediatric genetics of hearing loss clinic.

Hearing loss is a relatively common condition in children, occurring in approximately 2 out of every 1,000 births with approximately 50% of reported diagnoses having a primary genetic etiology. Given the prevalence and genetic component of hearing loss, coupled with a trend toward early diagnosis with the institution of universal newborn hearing screening, The Genetics of Hearing Loss Clinic was established at The Children's Hospital of Philadelphia to manage the diagnosis, testing, and genetic counseling for individuals and families. This paper described a cohort of 660 individuals with a diagnosis of hearing loss evaluated between July 2008 and July 2015 in the Genetics of Hearing Loss Clinic. To elucidate the cause of hearing loss in this cohort for better management and prognostication, testing included single nucleotide polymorphism chromosomal microarray, hearing loss next generation sequencing panel, and additional clinical tests inclusive of thyroid and renal function studies, temporal bone magnetic resonance imaging, and electrocardiogram. Of those evaluated, most had bilateral sensorineural hearing loss, occurring in 489/660 (74%). Additionally, 612/660 (93%) of patients presented with a nonsyndromic form of hearing loss (no other observed clinical findings at the time of exam), of which pathogenic mutations in GJB2 were most prevalent. Of the individuals with syndromic manifestations (48/660), Usher and Waardenburg syndrome were most commonly observed. A family history of hearing loss (first degree relative) was present in 12.6% of families with available information. Through molecular analyses, clinical examination, and laboratory testing, a definitive etiologic diagnosis was established in 157/660 (23.8%) of individuals. © 2016 Wiley Periodicals, Inc.