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Objective To investigate the cause of inner ear disturbances in cases of middle ear cholesteatoma with labyrinthine fistula. Setting University hospital. Study design Prospective case study. Patients Eight patients who were scheduled to undergo surgery for middle ear cholesteatoma with labyrinthine fistula were included in this study. Intervention Imaging analysis was performed using a 3-dimensional fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging sequence. Main outcome measures Three-dimensional FLAIR findings were compared with clinical symptoms associated with inner ear disturbance and surgical observations of the fistula. Results Three-dimensional FLAIR in 6 patients revealed areas of high signal intensity in the inner ears on the affected sides and areas with increased signal after the administration of gadolinium, especially in cases accompanied by acute sensorineural hearing loss. These images were considered to be indicative of breakdown of the blood-labyrinth barrier due to middle ear cholesteatoma. This finding was also present in a patient with no clinical symptoms of inner ear disturbances. Conclusion Three-dimensional FLAIR images of the inner ear are valuable in evaluating labyrinthine fistula in patients with cholesteatoma. Future studies are needed to better understand the role of 3-dimensional FLAIR in predicting the severity of inner ear disturbance.

Purpose: To compare the signal increase in cochlear lymph fluid on three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) in patients with sudden sensorineural hearing loss (SNHL) between regular contrast 3D-FLAIR (FL) and heavily T2-weighted 3D-FLAIR (HF). Methods: Twenty-five patients with unilateral sudden SNHL and eight healthy volunteers were included. Patients were divided into two groups: the mild group consisted of 9 patients, with an average hearing level of 60 dB or less; the severe group consisted of 16 patients, with an average hearing level of more than 60 dB. All patients and healthy volunteers underwent magnetic resonance (MR) cisternography for anatomical reference of the fluid space with FL and HF at 3 T. The region of interest (ROI) was manually drawn on the mid-modiolar section of the MR cisternography around the cochlea. The ROI for noise was drawn within the air space. ROIs were copied onto the FL and HF images. The contrast-to-noise ratio (CNR) between the affected and non-affected ear was measured in the patient group and the CNR between the right and left ear was also measured in the control group. Differences in the CNR on FL and HF images among the three groups were tested by one-way analysis of variance (ANOVA). Results: There was a statistically significant difference in mean CNR on HF among the three groups (P < 0.001). Furthermore, based on pairwise comparisons, there was a statistically significant difference between them in mean CNR on HF (P < 0.05). There was no statistically significant difference in mean CNR on FL among the three groups (P = 0.074). Conclusions: HF is more sensitive to signal alterations in cochleae with sudden SNHL than FL.

Pathologic third window lesions, such as superior semicircular canal dehiscence syndrome (SCDS) or large vestibular aqueduct syndrome (LVAS), cause several auditory and vestibular symptoms, which might affect perilymphatic pressure and induce endolymphatic hydrops (EH). In this study, the existence of EH in subjects with SCDS or LVAS was investigated using contrast-enhanced magnetic resonance imaging (MRI).Case series at university hospital.Seventeen ears from nine subjects who were diagnosed as having SCDS (five ears from three cases) or LVAS (12 ears from six cases) were studied. Ears were evaluated by 3-T MRI performed 4 hours after intravenous injection of gadodiamide hydrate. Imaging data concerning the degree of EH in the cochlea and the vestibule were compared with clinical symptoms and hearing levels for all ears.All ears showed air-bone gaps at low frequencies on pure tone audiometry. None of the subjects with SCDS had episodes of acute sensorineural hearing loss (SNHL) or vestibular symptoms, except for one patient who complained of head vibration induced by loud noise. Conversely, five of six subjects with LVAS had episodes of acute SNHL or vestibular symptoms. Four of five ears with SCDS showed severe EH in the cochlea, and two ears showed mild EH in the vestibule. All ears with LVAS showed mild to severe EH in both the cochlea and vestibule.The present study demonstrated the existence of EH in ears with pathologic third window lesions, which might affect patients' auditory or vestibular symptoms.4 Laryngoscope, 126:1446-1450, 2016.

PURPOSE We measured the volume of the endolymphatic space by extending a previously proposed less observer-dependent method of area quantification and compared volume measurements obtained using long (18 min, Image A) and short (8 min, Image B) scan times. METHODS We performed MR imaging of 40 ears in 20 patients with clinically suspected endolymphatic hydrops 4 hours after intravenous administration of single-dose gadolinium-based contrast material (IV-SD-GBCM). Two observers separately measured the ratio of the volume of the endolymph to that of total lymph in the cochlea and vestibule by extending the area ratio measurement method previously reported (Image A and B). The correlation between the values by Image A and B was calculated. RESULTS We observed a strong linear correlation between Images A and B in the cochlear images; the Pearson's correlation coefficient (r) was 0.928 for Observer A and 0.926 for Observer B (P < 0.001, for all). A strong linear correlation was also observed in the vestibular images; the Pearson's correlation coefficient (r) was 0.962 for Observer A and 0.968 for Observer B (P < 0.001, for all). CONCLUSION Measurement of endolymphatic volume after IV-SD-GBCM may be feasible using an MR imaging protocol with a reduced scan time of 8 min. This method might facilitate greater use of endolymphatic hydrops imaging in clinical applications.

Hearing loss is a risk factor for the progression of dementia. Cognitive improvement is occasionally found after removal of cerumen impaction. Because patients with dementia do not usually complain about cerumen impaction, detection methods are important. The present study aimed to investigate whether cerumen impaction is observable using brain magnetic resonance imaging.Participants were six patients whose hearing level improved 15 dB or more unilaterally or bilaterally after the removal of cerumen impaction. A radiologist who was blind to the impaction side and whether magnetic resonance imaging scans were taken before or after impaction removal classified cerumen impaction as positive, negative or unclear.Three ears classified as impaction positive and five ears classified as impaction negative corresponded accurately to the presence or absence of cerumen impaction. Among four ears classified as unclear, two did and two did not have cerumen impaction.Careful examination of the external ear canal on brain magnetic resonance imaging can be used to detect cerumen impaction.

alpha-Tectorin and beta-tectorin are major noncollagenous proteins of the tectorial membrane, which plays a crucial role in the reception of sonic signals in the cochlea. Midkine and pleiotrophin are closely related proteins that serve as growth factors and cytokines. In mice doubly deficient in the midkine gene and pleiotrophin gene, expression of beta-tectorin mRNA was nearly abolished in the cochlea on day 1 and 7 after birth. Expression of alpha-tectorin mRNA was unaffected in the double knockout mice, and expression of beta-tectorin mRNA was not altered in mice deficient in only the midkine or pleiotrophin gene. In newborn wild-type mice, both midkine and pleiotrophin were expressed in the greater epithelial ridge of the cochlea, in which beta-tectorin mRNA was strongly expressed. These results indicate that either midkine or pleiotrophin is required for significant expression of beta-tectorin. In agreement with the view that beta-tectorin is essential for normal auditory function, mice doubly deficient in both midkine and pleiotrophin genes exhibited very severe auditory deficits. We observed that mice deficient in either midkine or pleiotrophin gene were also impaired in their auditory response, but the level of the deficit was generally low or moderate. The present finding illustrates the importance of growth factor expression in the cochlea for auditory function.

The appropriate cutoff Hounsfield unit (HU) value for the diagnosis of otosclerosis was determined and the correlation between the bone conduction threshold and the findings of computed tomography (CT) densitometry investigated. CT images, 0.5-mm thick, were evaluated in 24 ears with otosclerosis and 19 control ears. Eight regions of interest were set around the otic capsule. The mean HU values in the area anterior to the oval window (A-OW) and anterior to the internal auditory canal (A-IAC) were significantly lower in otosclerosis than in controls. Based on receiver operating characteristic (ROC) analysis, the cutoff HU value in A-OW was determined to be 2,187.3 HU. The mean HU value in retrofenestral otosclerosis was significantly lower in the area A-OW, A-IAC and around the cochlea than in controls. Based on ROC analysis, the cutoff HU value in the latter was determined to be 2,045 HU. A statistically significant correlation was found between the density of the area A-OW and the hearing level at 500 and 1,000 Hz, and between the density of the area around the cochlea and the hearing level at most frequencies. These results suggest the semi-automated diagnosis of otosclerosis may be possible.

We investigated whether an acyclic polyisoprenoid antiulcer drug, geranylgeranylacetone (GGA), induces the expression of HSP70 in the rat cochlea. Immunoblotting revealed upregulation of HSP70 in the cochlea at 12 h after transtympanic (local) or oral (systemic) administration of GGA, and this increased at 24 h after administration. Positive immunohistochemical staining of HSP70 was observed in the hair cells, the spiral ganglion, the stria vascularis, the spiral ligament, and the perivascular portion of modiolar vessels. We therefore subsequently studied the effects of GGA as an HSP-inducer on inner ear trauma due to inflammation. Damage to the lateral wall due to inflammation induced by lipopolysaccharide inoculation was protected against by pretreatment with GGA, as assessed physiologically by measurement of cochlear blood flow and morphologically by electron microscopy. The results of the present study suggest that GGA can protect the cochlea against other injuries including those induced by noise, ototoxic drugs, and ischemia by upregulating HSP70.

特発性低髄圧症候群は, 腰椎穿刺, 腰椎麻酔, 脳外科手術などの誘因がなく, 何らかの原因で髄液量が減少し, 頭蓋内圧が低下し, 起立性頭痛を特徴とする疾患である。本症候群はしばしば難聴, 耳鳴, めまいを合併し, 突発性難聴や低音障害型感音難聴との鑑別が重要となる。今回我々は, 両側変動性感音難聴をきたした特発性低髄圧症候群の一症例を経験したので報告する。症例は, 46歳女性, 初発症状は, 後頭部痛, 全身倦怠感, 左難聴, 左耳閉感であり, 経過中に回転性めまいあり, 頭・頸部痛も増強したため精査のため当科紹介され入院となった。純音聴力検査では両側低音部感音難聴を示し, 眼振は認めなかった。造影MRIでは肥厚した硬膜の増強を認めた。脳槽シンチでは頸椎領域より2ヵ所の髄液漏出を認めた。難聴や頭・頸部痛はステロイド投与では改善を認めず, 起立時に増悪する頭・頸部痛, 難聴, 耳鳴があり, 臥位で改善した。難聴感は発症後2ヶ月近くたって消失したが, その他の症状は硬膜外自家血パッチ術を施行し軽快した。術後2年にわたり経過観察しているが頭痛や難聴悪化は認めていない。

The purpose of this study was to elucidate the influence of otitis media on blood flow in the lateral wall of the cochlea by means of a model of endotoxin-induced otitis media. The cochlear blood flow (CBF) following lipopolysaccharide inoculation into the middle ear cavities of rats was measured by laser-Doppler flowmetry and compared with that of untreated ears. After this evaluation, the influence on CBF of concomitant use of a nitric oxide synthase inhibitor was also investigated. The first day after inoculation, the CBF of treated ears decreased significantly. This decrease recovered gradually between the 7th and 14th days. With concomitant use of a nitric oxide synthase inhibitor, the decrease in CBF was prevented to some extent. The results showed a functional influence upon CBF by endotoxin-induced otitis media. The significance of prophylactic use of the drug is also discussed in regard to the effect on CBF following otitis media.

This study was performed to elucidate the contribution of the stapedial artery (SA)--which has been considered to be independent of the vertebrobasilar system--to blood flow in the ear by observing ear blood flow in Sprague-Dawley rats. A laser-Doppler (LD) probe was positioned on the bony wall of the cochlear basal turn after removal of the middle ear mucosa. The LD output was measured while either or both the SA and the anterior inferior cerebellar artery (AICA) were occluded. The LD output decreased to 70.6+/-2.5% (mean+/-S.D.) of the baseline value following SA occlusion, and to 58.0+/-7.8% following AICA occlusion in 12 animals. The rebound phenomenon of blood flow, which is a type of autoregulation, was only observed after releasing AICA occlusion. Simultaneous occlusion of the SA and AICA decreased the LD output to 27.0+/-5.5% of the baseline value. The role of the contribution of the SA and AICA to the LD output is discussed.

We studied the damage to the cochlear lateral wall induced by otitis media and the therapeutic effects of intratympanic administration of steroid and nitric oxide (NO) synthase inhibitor. In Sprague-Dawley rats, right middle ear cavities were inoculated with lipopolysaccharide, followed after 30 min by intratympanic administration of dexamethasone, NOS-inhibitor or PBS. Twenty-four hours after lipopolysaccharide inoculation, cochlear blood flow was measured by laser-Doppler flowmetry. Prostaglandin E(1) was topically applied to the round window membrane of the right ear and changes in cochlear blood flow were calculated. Changes of cochlear blood flow were significantly different among the three groups. Increases in cochlear blood flow following PGE(1) application were higher in the group that received NOS-inhibitor. Electron microscopic examination revealed that changes in the stria vascularis were less severe in rats treated with dexamethasone or NOS-inhibitor. Our results show the effectiveness of intratympanic dexamethasone or NOS-inhibitor in treating cochlear lateral wall damage caused by otitis media.

A rare case of recurrent pleomorphic adenoma of temporal bone in a 67-year-old woman is described. The primary tumor originated in the superficial lobe of the right parotid gland, and was treated with surgery 10 times over 30 years, followed by radiotherapy of 50 Gy. The secondary tumor appeared 32 months after radiotherapy in the right temporal bone, extending to the posterior cranial f ossa and foramen magnum. Right partial temporal bone resection and lateral suboccipital craniotomy were performed on this metastasizing pleomorphic adenoma. Metastasizing pleomorphic adenoma is a very unusual tumor, which appears more likely in cases after incomplete resection. While some of recurred pleomorphic adenoma are pathologically benign, their behavior may become metastatic or clinically malignant in the long run as in the case descrebed here.

The degree of hearing loss and audiogram shape vary in patients with perilymphatic fistula. The cause of variability is not well known. To determine the effect of perilymphatic fistula on cochlear function, we studied the effect of round window membrane rupture on cochlear blood flow (CBF) by placing a laser-Doppler probe to the basal turn in 20 guinea pigs. In 10, endocochlear potential (EP) was measured by inserting a glass microelectrode through the stria vascularis of the basal turn. During 60 minutes following round window membrane rupture, CBF decrease of more than 15% was observed in 9 of 20 animals, and EP decrease of more than 15% was observed in 5 of 10. A significant correlation was observed between the degree of CBF reduction and EP reduction. In 3 animals in which CBF decreased after round window membrane rupture, temporal bones were studied histologically. One cochlea showed endolymphatic hydrops and another cochlea showed significant hemorrhage in the scala tympani after rupture. These results suggested that CBF impairment is associated with cochlear dysfunction in perilymphatic fistula.

Although there have been many studies on the round window membrane (RWM), little information has been reported about changes in the membrane associated with aging. We have undertaken morphological studies of RWMs using young (7-8 weeks old) and aged (27-29 months old) C57BL/6 mice. The RWM was thinner in mice from the aged group compared with that of the young group. The cell density in the epithelial and inner layers was also reduced in the aged group. In the middle layer of the RWM in aged mice, transmission electron microscopy revealed many degenerated short and thick elastic fibers. Confocal laser microscopy using fluorescein isothiocyanate(FITC)-wheat germ agglutinin (WGA) staining was used to identify WGA-positive fibers in the middle layer of the RWM, which changed in a similar manner to the fibers in aging skin.

Meniere's disease (MD) is characterized by episodic vertigo, fluctuating hearing loss and tinnitus. Vestibular migraine (VM) is a relatively new disorder that is characterized by episodic vertigo or dizziness, coexisting migraine and absence of hearing loss. It is occasionally difficult to distinguish between VM and vestibular MD with headache. Because endolymphatic hydrops (EH) is a characteristic sign of MD, we attempted to evaluate endolymphatic space size in both diseases. Endolymphatic space size in the vestibule and the cochlea was evaluated in seven patients with VM and in seven age- and sex-matched patients with vestibular MD. For visualization of the endolymphatic space, 3T magnetic resonance imaging was taken 4 h after intravenous injection of gadolinium contrast agents using three-dimensional fluid-attenuated inversion recovery and HYbriD of reversed image of positive endolymph signal and native image of positive perilymph signal techniques. In the vestibule of VM patients, EH was not observed, with the exception of two patients with unilateral or bilateral EH. In contrast, in the vestibule of patients with vestibular MD, all patients had significant EH, bilaterally or unilaterally. These results indicate that endolymphatic space size is significantly different between patients with VM and vestibular MD.

Age-related hearing impairment (ARHI) is a complex, multifactorial disorder that is attributable to confounding intrinsic and extrinsic factors. The degree of impairment shows substantial variation between individuals, as is also observed in the senescence of other functions. This individual variation would seem to refute the stereotypical view that hearing deterioration with age is inevitable and may indicate that there is ample scope for preventive intervention. Genetic predisposition could account for a sizable proportion of interindividual variation. Over the past decade or so, tremendous progress has been made through research into the genetics of various forms of hearing impairment, including ARHI and our knowledge of the complex mechanisms of auditory function has increased substantially. Here, we give an overview of recent investigations aimed at identifying the genetic risk factors involved in ARHI and of what we currently know about its pathophysiology. This review is divided into the following sections: (i) genes causing monogenic hearing impairment with phenotypic similarities to ARHI; (ii) genes involved in oxidative stress, biologic stress responses, and mitochondrial dysfunction; and (iii) candidate genes for senescence, other geriatric diseases, and neurodegeneration. Progress and prospects in genetic research are discussed.

To establish a methodology for magnetic resonance imaging (MRI) assessment in the diagnosis of cholesteatoma using signal intensity on BLADE diffusion-weighted MRI (BLADE-DWI) and apparent diffusion coefficient (ADC) mapping.Retrospective case series.University hospital.Participants comprised 29 patients who underwent middle ear surgery and in whom preoperative differential diagnosis between cholesteatoma and other middle ear diseases was difficult using local and computed tomographic findings and required BLADE-DWI.Signal intensity ratio (SIR) between the affected region of the middle ear and the pons measured by BLADE-DWI and on ADC maps was evaluated numerically. SIR in an area located near the target lesion in each case was used as a control. Values were compared between both cases in which cholesteatoma was histopathologically confirmed (cholesteatoma group) and cases in which cholesteatoma was excluded on histopathologic examination (noncholesteatoma group).Imaging and histopathologic findings.SIR on BLADE-DWI was significantly higher in the cholesteatoma group than in the noncholesteatoma group, although both groups showed significantly higher SIR in the target lesion than in the control area. Moreover, SIR on ADC maps was significantly lower in the cholesteatoma group than in the noncholesteatoma group. The clear cutoff value of SIR on ADC maps was 1.5.The combination of BLADE-DWI and ADC mapping offers a useful imaging tool for accurate detection of middle ear cholesteatoma. Use of SIR can numerically differentiate between cholesteatoma and noncholesteatoma.

Patients with MPO-ANCA related angitis sometimes experience the onset of sonsorineural hearing loss and facial palsy. The main aim of this study was to investigate the relationship between the inflammation in the inner ear and sensorineural hearing loss, or between the inflammation of the facial nerve and facial nerve palsy in patients with MPO-ANCA related angitis. From 2007 to 2011, the inner ear was evaluated in 16 ears from 8 patients with MPO-ANCA related angitis with 3D-FLAIR MRI. In 12 ears of the 16 ears the onset of sensorineural hearing loss was noted, and the signal intensity ratio (SIR) in the cochlea was 0.62 +/- 0.15 on pre-enhancement 3D-FLAIR MRI, and 0.97 +/- 0.5 on post-enhancement. These SIR values were significantly higher than the SIR of non-sensorineural hearing loss patients. The onset of facial palsy was noted in 9 of 16 sides and the facial nerve SIR was 0.83 +/- 0.23 on post-contrast 3D-FLAIR MRI. The SIR in the facial nerve of facial nerve palsy patients was higher than non-facial palsy patients. The conclusion is that sensorineural hearing loss and facial palsy in patients with MPO-ANCA related angitis occurred due to strong inflammation in the cochlea or facial nerve. 3D-FLAIR MRI made this pathology clear.

We describe a case report of a patient with Meniere's disease whose changes in endolymphatic hydrops were observed using magnetic resonance imaging (MRI). Gadolinium was injected intratympanically through the tympanic membrane, and MRI scans performed with a 3-T MRI unit revealed endolymphatic hydrops inside the perilymphatic space filled with gadolinium. We evaluated the relationship between the image findings and hearing level. The correlation between the degree of endolymphatic hydrops observed by MRI and hearing level in patients with Meniere's disease offers a promising new method to study the progression of Meniere's disease.

In this report, we describe a highly unusual case of glandular malignant peripheral nerve sheath tumor presenting as a neck mass in a previously healthy 29-year-old man. Grossly, the tumor was found to arise from a swollen peripheral nerve trunk. The tumor was largely composed of spindle cells that demonstrated marked nuclear pleomorphism and numerous abnormal mitotic figures. In addition, histologically malignant glandular structures lined by simple nonciliated columnar cells with goblet cells were found clustered in the center of the tumor. Examination of the swollen peripheral nerve trunk revealed the presence of a plexiform neurofibroma. The spindle cells were positive for S100. The glands were negative for S100 but positive for keratin, epithelial membrane antigen, and neuroendocrine markers (somatostatin, chromogranin, Leu-7, and calcitonin). This patient was subsequently diagnosed as having von Recklinghausen disease and died of tumor metastasis to the lungs 34 months after the presentation. To our knowledge, only 3 similar cases have been previously described in the literature.

Most cases of neoplasms arising from the palate can be treated by partial excision of the palate, reconstruction with local flaps and a palatal prosthesis. Several methods of reconstruction with free flaps have also been reported to correct surgical defects of the palate.We report a case of adenoid cystic carcinoma in the palate, which required a total palatectomy. A preoperative CT examination revealed that the tumor had spread extensively and destroyed anteriorly the alveolar process of the maxilla and the pterygoid process of the sphenoid bone posteriorly. The defect of the palate was corrected with a fibular free flap. Blood loss during the operation was over 6000ml, however, the patient has been well after being discharged.Functional reconstruction of the palate is a complicated problem, and the quality of life of patients should always be taken into consideration when treating an aggressive neoplasm.

SUMMARY: By optimizing the inversion time of a 3D inversion-recovery turbo spin-echo sequence at 3T, we obtained separate images of endolymphatic and perilymphatic space 24 hours after intratympanic administration of gadolinium contrast material. In patients with Ménière disease, endolymphatic hydrops were detected not only in the cochlea but also in the vestibule. Fusion of the 2 types of images visualized the entire fluid space of the labyrinth and the spatial relationship of the 2 spaces.

Objectives To characterize glycoconjugate expression in normal human eustachian tubes and study the alterations in glycoconjugate expression found in eustachian tubes with otitis media. Study design Using lectin histochemistry, alterations in glycoconjugates were studied in three normal temporal bones, in four temporal bones with mucoid otitis media (MOM), and in five with serous otitis media (SOM). Methods Sections of previously processed temporal bones were decelloidinized, and then incubated with seven biotinylated lectins--WGA, SNA, MAA, BPA, PNA, UEA-1, and LcH--that reflect seven carbohydrate residues of glycoconjugates, respectively: GlcNAc/NeuNAc, NeuNAc alpha(2-6)GalNAc, NeuNAc alpha(2-3)GalNAc, Gal beta(1-3) GalNAc, L-fucose, and alpha-mannose residues. Control sections were incubated with inhibitory carbohydrates or without biotinylated lectins. Results In the normal temporal bones, five carbohydrate residues in goblet cells and cilia of the eustachian tube demonstrated moderate to strong activity--NeuNAc alpha(2-6)GalNAc, NeuNAc alpha(2-3)GalNAc, GalNAc, Gal beta(1-3)GalNAc, and L-fucose. Two residues demonstrated weak activity--GlcNAc/NeuNAc and alpha-mannose. Temporal bones with MOM revealed increases in sialic acid and alpha-mannose, and a decrease in L-fucose. Residues of carbohydrates in the cilia of bones with SOM were notably decreased, especially for GalNAc, Gal beta(1-3)GalNAc, and NeuNAc alpha(2-6)GalNAc. Conclusions Glycoconjugates in the normal human eustachian tube are rich in GalNAc, Gal beta(1-3)GalNAc, L-fucose, and NeuNAc alpha(2-3/2-6) GalNAc, but low in alpha-mannose and sialic acid. Eustachian tubes from cases with SOM or MOM demonstrated alterations in glycoconjugate expression in cilia and goblet cells, which may reflect disorder of the carbohydrate metabolism during otitis media, especially in SOM.

Although pulmonary infections caused by Pseudomonas aeruginosa can hardly be eradicated in patients with cystic fibrosis (CF, the most common genetic disease among Caucasians), these patients are mainly treated with intravenous and nebulized tobramycin. Long-term treatment with tobramycin, however, may induce ototoxic effects. We assessed the clinical histories and postmortem temporal bones of six patients with CF for signs of this ototoxicity. Four bones showed typical manifestations of ototoxicity induced by aminoglycosides (AGs): loss of hair cells in the lower turns, and degeneration of ganglion cells. Six bones revealed no loss or scattered loss of hair cells, however, degeneration of the spiral ganglion cells was observed. This suggests that degeneration of the spiral ganglion may occur as a primary manifestation in some cases of ototoxicity due to aminoglycosides. Recent reports have shown that trophic factors (neurotrophins and acidic fibroblast growth factor) interacting with hair cells and the spiral ganglion protect the inner ear from damage. It may be that disturbances in supply of such trophic factors caused degeneration of ganglion cells without loss of hair cells in the cases we studied.

We report a case of advanced small cell carcinoma in the larynx, which was treated with Irinotecan hydrochloride (CPT-11) chemotherapy. The patient was free of disease for 4 years after treatment. Several chemotherapeutic agents for small cell carcinoma have been proposed; however, median survival time has been miserable, especially in advanced cases. For the cure of the aggressive lethal behavior of this disease, chemotherapy with CPT-11 might be effective to improve median survival of patients with small cell carcinomas of the larynx.

Pleomorphic adenoma of the larynx is a rare disorder, and until recently has been treated mainly by approaches involving pharyngotomy. We encountered a case of pleomorphic adenoma originating from the laryngeal surface of the epiglottis and removed it using a YAG laser through a suspension laryngoscope. This case was complicated by delayed-onset bilateral vocal-cord paralysis, the causes of which are also discussed.

PURPOSE Many inner ear disorders, including Meniere's disease, are believed to be based on endolymphatic hydrops. We evaluated a newly proposed method for semi-quantification of endolymphatic size in patients with suspected endolymphatic hydrops that uses 2 kinds of processed magnetic resonance (MR) images. METHODS Twenty-four consecutive patients underwent heavily T2-weighted (hT2W) MR cisternography (MRC), hT2W 3-dimensional (3D) fluid-attenuated inversion recovery (FLAIR) with inversion time of 2250 ms (positive perilymph image, PPI), and hT2W-3D-IR with inversion time of 2050 ms (positive endolymph image, PEI) 4 hours after intravenous administration of single-dose gadolinium-based contrast material (IV-SD-GBCM). Two images were generated using 2 new methods to process PPI, PEI, and MRC. Three radiologists contoured the cochlea and vestibule on MRC, copied regions of interest (ROIs) onto the 2 kinds of generated images, and semi-quantitatively measured the size of the endolymph for the cochlea and vestibule by setting a threshold pixel value. RESULTS Each observer noted a strong linear correlation between endolymphatic size of both the cochlea and vestibule of the 2 kinds of generated images. The Pearson correlation coefficients (r) were 0.783, 0.734, and 0.800 in the cochlea and 0.924, 0.930, and 0.933 in the vestibule (P

To analyze the factors affecting the fenestration of the footplate in stapes surgery and to evaluate the effectiveness of Fisch's reversal steps stapedotomy.Retrospective study.A total of 191 patients with otosclerosis in whom 230 primary stapes surgeries were performed by 1 surgeon in 3 tertiary hospitals.Evaluation of factors affecting the fenestration of the footplate in stapes surgery by logistic regression analysis.Stapedotomy (small hole in stapes footplate) was performed in 148 ears (64.3%). Partial stapedectomy (half removal of stapes footplate) was performed in 65 ears (28.3%). Total stapedectomy (total removal of stapes footplate) was performed in 17 ears (7.4%). Stapedotomy could be performed in 72.1% of cases (75/104) in which the stapes suprastructure was removed after insertion of the prosthesis and in 57.9% of cases (73/126) in which the stapes suprastructure was removed before the insertion. Stapedotomy could be performed in 65.4% of cases (117/179) in which a 0.6-mm-thick Schuknecht-type Teflon wire piston was used and in 60.8% (31/51) in which a whole Teflon piston was used. Multivariate analysis of factors affect stapedotomy using logistic regression analysis showed that the surgical order of the removal of the stapes suprastructure and insertion of the prosthesis were the most important 2 factors.Fisch's reversal steps technique was useful in performing stapedotomy in all otosclerosis patients. Manipulation of the prosthesis when crimping it to the incus is also important. The prosthesis should be crimped onto the incus and inserted in the footplate opening gently and atraumatically.

Objectives:Evaluate vascular activity in ears with otosclerosis by intraoperative measurement of blood flow using laser-Doppler flowmetry, and compare the data with densitometry on computed tomography (CT).Methods:Thirty-nine ears from patients who underwent surgery for otosclerosis were assessed in the present study. The subjects were divided into the fenestral (29 ears) and the retrofenestral (10 ears) groups by CT. Ratios of bone density in the area anterior to the oval window (AOW) or the promontory (PT) near the round window niche to that of the basis labyrinthine otic capsule were calculated on CT images. Measurements of blood flow were performed with a laser-Doppler flowmeter at the two corresponding areas evaluated by CT.Results:In both groups, relatively young subjects with high blood flow values in the AOW or PT had rather low ratios of bone density in the corresponding areas. In contrast, old subjects with low ratios of bone density showed low blood flow values in the same areas. In the retrofe...

Objectives:Human temporal studies have shown that endolymphatic hydrops (EH) is accompanied by otosclerosis, which is a risk factor for complications after stapes surgery. Recently, visualization of EH has become possible using magnetic resonance imaging (MRI) with contrast agents. The presence of EH in cases with otosclerosis was investigated by MRI to prevent complications following stapes surgery.Methods:Sixteen ears from nine cases diagnosed as having otosclerosis were evaluated by 3-Tesla MRI examinations taken 4 h after intravenous gadolinium injection. More than half of the ears showed mixed hearing loss and were suspected to have cochlear otosclerosis. Imaging data were compared with clinical findings in all ears and postoperative symptoms in ears undergoing surgery.Results:Varying degrees of EH were observed in 14 of the 16 ears. Episodes of acute sensorineural hearing loss or vertigo occurred in 4 ears with severe EH in the cochlea and 3 ears with severe EH in the vestibules. Overall, 12 of 14 e...

Objective Acute low-tone sensorineural hearing loss (ALHL) has been reported to be associated with endolymphatic hydrops (EHs). However, evaluation of the size of the endolymphatic space has not been reported. We attempted to visualize EH in ALHL using magnetic resonance imaging (MRI). Study design Prospective diagnostic study. Setting University hospital. Methods We evaluated 25 ears of 25 unilateral ALHL patients. Three-tesla MRI was obtained 24 hours after intratympanic injection of gadolinium (Gd) (n = 5) or 4 hours after intravenous injection of Gd (n = 20). A radiologist blinded to the patients' clinical data classified the degree of EH in the vestibule and cochlea into 3 groups: none, mild, and significant. Results On the affected sides, cochlear EH was recognized in 23 ears (92%) and was classified as significant EH (n = 15) or mild EH (n = 8); vestibular EH was detected in 22 ears (88%), classified as significant EH (n = 16) or mild EH (n = 6). Cochlear EH was more frequently observed in the affected ear than in the contralateral ear (90% versus 40%, p Conclusion In ALHL, EH was observed not only in the cochlea but also in the vestibule as in Meniere's disease.

Objectives/Hypothesis Endothelin-1 is a potent vasoconstrictor peptide that is widely distributed throughout the mammalian body including the spiral modiolar artery, vestibule, and cochlea. This study aimed to investigate the association between the Lys198Asn (G/T) polymorphism (rs5370) of the endothelin-1 gene and sudden sensorineural hearing loss (SSNHL). Study Design Case-control study. Methods Seventy-two SSNHL patients (mean age, 58.3 ± 14.0 years) were compared with 2,159 controls included in a community-based study of aging. Multiple logistic regression was used to obtain odds ratios (ORs) for SSNHL. In subgroup analysis, patients with SSNHL who visited to the hospital within the first month of onset were selected to assess audiometric features according to genotype. Pure-tone averages at 250, 500, 1,000, 2,000, and 4,000 Hz were calculated in the affected ear. Results Under the recessive genetic model, after adjustment for age, sex, histories of hypertension, dyslipidemia and diabetes, the crude and adjusted ORs for SSNHL risk were 2.209 (95% confidence interval [CI]: 1.140-4.281) and 2.173 (95% CI: 1.086-4.348), respectively. No significant ORs were observed under the additive and dominant models. The severity of SSNHL differed significantly between genotypes. The mean pure-tone averages at the initial visit were 78.6, 66.4, and 57.8 dB for the GG, GT, and TT genotypes, respectively (P = .034). Conclusions Our study indicates that the recessive genotype was significantly associated with increased SSNHL risk; however, the severity was lower in these individuals than it was in those with the wild-type genotype. Endothelin-1 may be implicated in SSNHL. Level of Evidence 3b Laryngoscope, 123:E59–E65, 2013

Eleven patients with suspected Ménière's disease received intratympanic (IT) administration of gadolinium (gadopentetate dimeglumine; Gd) prior to acquisition of 3-dimensional (3D) fluid-attenuated inversion recovery (FLAIR), 3D real inversion recovery (IR), and fast T₁ mapping by dual flip angle 3D gradient echo (FT₁-map) imaging sequences to evaluate the degree of perilymph enhancement. Though 3-dimensional FLAIR could detect lower concentrations of gadolinium than 3D real IR, in 2 patients, poor enhancement still prevented visualization of the endolymphatic space using 3D FLAIR. We could predict poor contrast enhancement in these 2 patients using the FT₁-map technique.

Objective To review the findings of otitis media in adults in relation to supraesophageal reflux of gastrointestinal contents and summarize current concepts. Data sources Literature published in English-language journals from 2001 to the present identified by searching electronic databases (MEDLINE and Web of Science). Study selection Clinical articles that contained the terms reflux, ear, otitis media, and adult and relevant animal studies. Data extraction Findings of searchable case reports and results of animal studies were included. Data synthesis Current findings were reviewed for the following points: 1) proposed effect of reflux, 2) prevalence and characteristics, 3) risk factors, and 4) treatment. Conclusion Published literature concerning reflux and otitis media in adults is limited to clinical case series. Reflux is likely present in a significant number of adult cases with otitis media and may lead to Eustachian tube dysfunction in such subjects. Reflux in adult subjects with otitis media is potentially different from the physiologic events observed in children, but the causal link between them remains unclear. Evaluation of more cases that could be diagnosed as reflux-induced otitis media is necessary for better understanding of the disease entity.

The etiologies of idiopathic sudden sensorineural hearing loss (SSNHL) and Meniere's disease remain unclear. Recently, accumulating evidence has demonstrated that free radicals are related to the pathology of inner ear disease. Because genetic factors may contribute partly to the etiologies of SSNHL and Meniere's disease, we investigated the association between genetic polymorphisms located in genes related to the free-radical process and susceptibility to SSNHL and Meniere's disease. We compared 83 patients affected by SSNHL and 83 patients affected by Meniere's disease with 2048 adults (for SSNHL) and 1946 adults (for Meniere's disease) who participated in the National Institute for Longevity Sciences, Longitudinal Study of Aging. Multiple logistic regression was used to calculate odds ratios (ORs) for SSNHL and Meniere's disease in individuals with polymorphisms in the genes: methionine synthase (MTR; rs1805087); methionine-synthase reductase (MTRR; rs1801394); nitric oxide synthase 3 (NOS3; rs1799983); caveolin 1 (Cav1; rs3840634); melatonin receptor 1B (MTNR1B; rs1387153); NAD(P)H oxidase p22(phox) subunit (NADH/NADPHp22phox; rs4673); and mitochondria 5178 (MT5178; rs28357984). The NOS3 polymorphism was significantly associated with a risk of SSNHL; in addition, the OR for the NOS3 polymorphism and SSNHL risk was 2.108 (CI, 1.343-3.309) with adjustment for age and sex. The Cav1 polymorphism was significantly associated with a risk of Meniere's disease; moreover, the OR for the Cav1 polymorphism and Meniere's disease risk was 1.849 (CI, 1.033-3.310) with adjustment for age and sex. In conclusion, the NOS3 and Cav1 polymorphisms were significantly associated with the risk of SSNHL and Meniere's disease, respectively.

A HYDROPS (HYbriD of Reversed image Of Positive endolymph signal and native image of positive perilymph Signal) image, created by subtracting a positive endolymph image from a positive perilymph image after intravenous injection of single-dose gadodiamide, has been reported to facilitate recognition of endolymphatic hydrops. To further increase the contrast-to-noise ratio (CNR) of HYDROPS images, we multiplied a T2-weighted MR cisternographic image onto the HYDROPS image. Ten patients with suspected Meniere's disease were included. The average CNR of generated images increased to more than 200 times that of HYDROP images.

A substrain of an autoimmune-prone mouse, NZB/kl, was found to show spontaneous elevation of the auditory brainstem response (ABR) threshold with age. Morphological examination of the inner ear in NZB/kl mice with high ABR thresholds revealed pathological changes confined to the stria vascularis, including marked thickening of the capillary basement membrane which contained many foamy structures, and vacuolar degeneration of the intermediate cells. Circular or granular IgM deposits and some IgG deposits were found in the stria vascularis in the mice with high ABR thresholds, suggesting that deposits of immune complexes (mainly IgM antibodies) could cause strial damage that resulted in the ABR threshold elevation. Another substrain of NZB mice, NZB/san, showed lower levels of IgM immune complexes and anti-ss DNA antibodies, and did not develop either inner ear morphological changes or a high ABR threshold. NZB/kl mice may provide a useful animal model for studying the mechanism of autoimmune inner ear disease.