Organized hematoma of the sphenoid sinus with epistaxis.

Few reports have been made on organized hematoma (OH) originating in the sphenoid sinus. We report the case of a 24-year-old man who presented to the hospital with consistent epistaxis for 1 month. Preoperative computed tomography and magnetic resonance imaging (MRI) detected an expansive heterogeneous mass with minimal bone destruction and hypointense peripheral rim in T2-weighted images of the left sphenoid sinus as a solitary lesion. An OH was suspected first based on the history of repeated epistaxis and characteristic imaging findings, although quite rare. Preoperative vascular embolism was not performed because its effects on the ocular artery should be considered. With endoscopic sinus surgery under hypotensive anesthesia, the mass was carefully and successfully removed without bleeding and diagnosed as OH based on postoperative pathological examination. When solitary lesions are found in the sphenoid sinus with epistaxis, this disease should be differentiated. If it increases, complications, such as visual impairment, frequently occur, and control of intraoperative bleeding is sometimes difficult. Not only missing the characteristic imaging findings, especially MRI with contrast administration but also evaluating the anatomical relationship between OH and other important organs is important for a successful intervention of sphenoid sinus OH.

Distribution of glucocorticoid receptors and 11ß-hydroxysteroid dehydrogenase isoforms in the human inner ear.

HYPOTHESIS: Glucocorticoids (GCs) are widely used as a therapeutic modality for the inner ear disorders including Ménière's disease (MD). The concentration of GCs in the target cells is known to be regulated by 11ß-hydroxysteroid dehydrogenase (11ß-HSD), an enzyme complex responsible for the conversion of hormonally active cortisol into inactive cortisone. There is no morphologic indication of glucocorticoid receptors (GRs) and 11ß-HSD isoforms (11ß-HSD1 and 2) in human inner ear. OBJECTIVES: The objectives of this study are to determine whether GRs and the isoforms of 11ß-HSD are present in human inner ear tissues and to reveal their precise distribution. STUDY DESIGN: This study investigated the expression of GRs and 11ß-HSD isoforms (11ß-HSD1 and 2) in the human inner ear. METHODS: In humans, immunostaining of GRs, 11ß-HSD1, and 11ß-HSD2 was performed in the stria vascularis (SV) and the vestibular tissues, whereas in the cochlear tissues except for the SV, only GRs were investigated. RESULTS: Immunoreactivity of GRs was detected in the SV, outer hair cells, inner hair cell, spiral ligament, Reissner's membrane, vestibular hair cells, vestibular nerve, transitional cells, and dark cells of the crista ampullaris. 11ß-HSD1 was observed in the SV, the apical area of the vestibular hair cells, the transitional cells, and the dark cells. However, no immunoreactivity of 11ß-HSD2 was observed. CONCLUSION: Those data indicate that different local steroid regulation by GRs and the isoforms of 11ß-HSD is present in various parts of the human inner ear tissues and that the tissues are a direct therapeutic target of glucocorticoids in the inner ear diseases.

What factors are associated with good performance in children with cochlear implants? From the outcome of various language development tests, research on sensory and communicative disorders project in Japan: nagasaki experience.

OBJECTIVES: We conducted multi-directional language development tests as a part of the Research on Sensory and Communicative Disorders (RSVD) in Japan. This report discusses findings as well as factors that led to better results in children with severe-profound hearing loss. METHODS: We evaluated multiple language development tests in 33 Japanese children with cochlear implants (32 patients) and hearing aid (1 patient), including 1) Test for question and answer interaction development, 2) Word fluency test, 3) Japanese version of the Peabody picture vocabulary test-revised, 4) The standardized comprehension test of abstract words, 5) The screening test of reading and writing for Japanese primary school children, 6) The syntactic processing test of aphasia, 7) Criterion-referenced testing (CRT) for Japanese language and mathematics, 8) Pervasive development disorders ASJ rating scales, and 9) Raven's colored progressive matrices. Furthermore, we investigated the factors believed to account for the better performances in these tests. The first group, group A, consisted of 14 children with higher scores in all tests than the national average for children with hearing difficulty. The second group, group B, included 19 children that scored below the national average in any of the tests. RESULTS: Overall, the results show that 76.2% of the scores obtained by the children in these tests exceeded the national average scores of children with hearing difficulty. The children who finished above average on all tests had undergone a longer period of regular habilitation in our rehabilitation center, had their implants earlier in life, were exposed to more auditory verbal/oral communication in their education at affiliated institutions, and were more likely to have been integrated in a regular kindergarten before moving on to elementary school. CONCLUSION: In this study, we suggest that taking the above four factors into consideration will have an affect on the language development of children with severe-profound hearing loss.

Bilateral cochlear implantation for children in nagasaki, Japan.

OBJECTIVES: The number of patients with bilateral cochlear implant (CI) has gradually increased as patients and/or parents recognize its effectiveness. The purpose of this report is to evaluate the efficacy of 29 bilateral CI out of 169 pediatric CI users, who received auditory-verbal/oral habilitation at our hearing center. METHODS: We evaluated the audiological abilities 29 Japanese children with bilateral CIs including wearing threshold, word recognition score, speech discrimination score at 1 m from front speaker (SP), 1 m from second CI side SP, speech discrimination score under the noise (S/N ratio=80 dB sound pressure level [SPL]/70 dB SPL, 10 dB) at 1 m from front SP, word recognition score under the noise (S/N ratio=80 dB SPL/70 dB SPL, 10 dB) at 1 m from front SP. RESULTS: Binaural hearing using bilateral CI is better than first CI in all speech understanding tests. Especially, there were significant differences between the results of first CI and bilateral CI on SDS at 70 dB SPL (P=0.02), SDS at 1 m from second CI side SP at 60 dB SPL (P=0.02), word recognition score (WRS) at 1 m from second CI side SP at 60 dB SPL (P=0.02), speech discrimination score (SDS) at 1 m from front SP under the noise (S/N=80/70; P=0.01) and WRS at 1 m from front SP under the noise (S/N=80/70; P=0.002). At every age, a second CI is very effective. However, the results of under 9 years old were better than of over 9 years old on the mean SDS under the noise (S/N=80/70) on second CI (P=0.04). About use of a hearing aid (HA) in their opposite side of first CI, on the WRS and SDS under the noise, there were significant differences between the group of over 3 years and the group of under 10 months of HA non user before second CI. CONCLUSION: These results may show important binaural effectiveness such as binaural summation and head shadow effect. Bilateral CI is very useful medical intervention for many children with severe-to-profound hearing loss in Japan as well as elsewhere.

Water channel proteins in the inner ear and their link to hearing impairment and deafness.

The inner ear is a fluid-filled sensory organ that transforms mechanical stimuli into the senses of hearing and balance. These neurosensory functions depend on the strict regulation of the volume of the two major extracellular fluid domains of the inner ear, the perilymph and the endolymph. Water channel proteins, or aquaporins (AQPs), are molecular candidates for the precise regulation of perilymph and endolymph volume. Eight AQP subtypes have been identified in the membranous labyrinth of the inner ear. Similar AQP subtypes are also expressed in the kidney, where they function in whole-body water regulation. In the inner ear, AQP subtypes are ubiquitously expressed in distinct cell types, suggesting that AQPs have an important physiological role in the volume regulation of perilymph and endolymph. Furthermore, disturbed AQP function may have pathophysiological relevance and may turn AQPs into therapeutic targets for the treatment of inner ear diseases. In this review, we present the currently available knowledge regarding the expression and function of AQPs in the inner ear. We give special consideration to AQP subtypes AQP2, AQP4 and AQP5, which have been studied most extensively. The potential functions of AQP2 and AQP5 in the resorption and secretion of endolymph and of AQP4 in the equilibration of cell volume are described. The pathophysiological implications of these AQP subtypes for inner ear diseases, that appear to involve impaired fluid regulation, such as Menière's disease and Sjögren's syndrome, are discussed.

Distribution of glucocorticoid receptors and 11 beta-hydroxysteroid dehydrogenase isoforms in the rat inner ear.

11ß-hydroxysteroid dehydrogenase (11ß-HSD) is an enzyme complex responsible for the conversion of hormonally active cortisol to inactive cortisone, and two isoforms of the enzyme (11ß-HSD1 and 11ß-HSD2) have been cloned and characterized. An immunohistochemical study was performed to determine the precise distribution of glucocorticoid receptors (GRs) and the isoforms of 11ß-HSD in the rat (postnatal day 1, 4, 10, and adult). Immunoreactivity of GRs was detected in the stria vascularis (SV), the outer hair cells (OHCs), the inner hair cells (IHCs), the spiral ligament (SLig), the spiral limbus (SLib), the spiral ganglion cells (SGCs), Reissner's membrane (RM), the cochlear nerve (CN), the vestibular hair cells (VHCs), the dark cells (DCs), and the vestibular nerve (VN) in the rats. Immunostaining of 11ß-HSD1 was observed in almost all the tissues in the cochlea and the vestibule except SLig, SLib, SGCs, CN, VHCs, and VN during all developmental stages, whereas, immunoreactivity of 11ß-HSD2 was not detected in any of the inner ear tissues. A polymerase chain reaction (PCR) study was also performed on GRs, 11ß-HSD1, and 11ß-HSD2 in the OC, SV and vestibule of the postnatal rats, and revealed that mRNAs were detected in all those and tissues in all the developmental days of postnatal days 1, 4, and 10. This data indicates that expression of GRs and 11ß-HSD isoforms in the inner ear is tissue and age-specific, and that different local steroid regulation by GRs and the isoforms of 11ß-HSD is present in each part of the inner ear.

Influence of effortful swallow on pharyngeal pressure: evaluation using a high-resolution manometry.

OBJECTIVE: To evaluate the effect of effortful swallow on pharyngeal pressure while swallowing saliva and water using a novel high-resolution manometry (HRM) system. STUDY DESIGN: Case series with planned data collection. SETTING: Nagasaki University Hospital. SUBJECTS AND METHODS: Eighteen asymptomatic Japanese adult volunteers were studied. A solid-state HRM assembly with 36 circumferential sensors spaced 1 cm apart was positioned from the velopharynx to the upper esophagus to record pressures while swallowing. The maximum values of the pressure while swallowing saliva (dry swallowing) and 5 mL of water were measured at the velopharynx, meso-hypopharynx, and upper esophageal sphincter (UES) with and without effortful swallows. RESULTS: The maximum values of dry swallowing pressures (mm Hg) at the velopharynx, meso-hypopharynx, and UES were significantly higher with effortful swallow (155.7 ± 59.7, 256.7 ± 78.7, and 276.5 ± 87.5, mean ± standard deviation) than without it (115.3 ± 60.8, 172.9 ± 57.0, and 195.8 ± 61.3). Those of water swallowing pressures were also statistically higher with effortful swallow (169.3 ± 69.1, 236.6 ± 77.2, and 267.3 ± 79.1) than without it (119.2 ± 59.7, 189.5 ± 70.7, and 221.3 ± 72.7). CONCLUSION: The present results provide quantitative evidence of effortful swallow as well as physiological information. It also is hoped to be an aid to future clinical and investigative studies.

Adenosquamous carcinoma arising in a thyroglossal duct cyst: report of a case.

A 61-year-old Japanese woman had an 11-year history of an enlarging anterior neck mass that was diagnosed otolaryngologically as a thyroglossal duct cyst. Preoperatively, fine-needle aspiration cytology suggested a neoplastic lesion, but no obvious malignancy was clinically evident. Sistrunk's operation was performed. An adenosquamous carcinoma arising in a thyroglossal duct cyst was found on pathological examination. Carcinoma arising in a thyroglossal duct cyst is rare, and only approximately 200 cases have been reported, most of which were papillary carcinoma. Only 21 cases of squamous cell carcinoma arising in a thyroglossal duct cyst have been reported. The present case is the first report of adenosquamous carcinoma arising in a thyroglossal duct cyst.

Influence of head rotation on upper esophageal sphincter pressure evaluated by high-resolution manometry system.

OBJECTIVES: This study aimed to quantify the effects of head rotation on upper esophageal sphincter (UES) pressure in healthy subjects using a novel high-resolution manometry (HRM) system. STUDY DESIGN: Prospective study. SETTING: Nagasaki University Hospital. SUBJECTS AND METHODS: Eighteen asymptomatic Japanese male adult volunteers were studied. A solid-state HRM was positioned to record resting UES pressure. After endoscopically confirming on which side of the pyriform sinus the manometric sensor was positioned within the hypopharynx, we measured the maximum and mean values of the resting UES pressure and the length of the zone of the UES along the esophagus with the patients in the following positions: 1) neutral and straightforward head position (NSF), 2) turning the head in the direction of the side in which the sensor was positioned (HSS), and 3) turning the head in the opposite direction of the side with sensor (HOS). RESULTS: The maximum and mean values of the resting UES pressure were statistically higher in HSS than in NSF (P = 0.0001 and P < 0.0001, respectively), and were statistically lower in HOS than in NSF (P < 0.0001 and P < 0.0001, respectively). The length of the zone of the UES was statistically shorter in HOS than in NSF (P < 0.0001), but there was no significant difference in resting UES pressure along the esophagus between HSS and NSF (P = 0.3024). CONCLUSION: The present study provided us with physiological information regarding normal UES pressure in relation to head rotation. This data will be of aid to future clinical and investigative swallowing studies. Additionally, the current study provides evidence of the safety and usefulness of the head rotation maneuver for dysphagic patients.

Evaluation of swallowing pressure in a patient with amyotrophic lateral sclerosis before and after cricopharyngeal myotomy using high-resolution manometry system.

BACKGROUND: This study aimed to evaluate the pharyngeal pressure of a patient with, amyotrophic lateral sclerosis (ALS) before and after cricopharyngeal myotomy by high-resolution manometry (HRM) system. METHODS: A 60-year-old man with ALS underwent cricopharyngeal myotomy for his intractable, aspiration. The swallowing pressure along the velopharynx and upper esophagus was measured using, the HRM 1 month before and 3 months after the surgery. RESULTS: Before cricopharyngeal myotomy, the maximum values of the resting UES pressure, the dry swallowing, pressures in the velopharyngeal muscle zone and in the UES zone were 89, 95, and 171 mmHg, respectively. After surgery, the maximum values of both the resting pressure and dry swallowing, pressure in the UES zone remarkably decreased to 21 and 75 mmHg, respectively. CONCLUSIONS: This is the first report showing the effect of cricopharyngeal myotomy by demonstrating, the difference in the swallowing pressure along the velopharynx and upper esophagus before and after, the surgery in an ALS patient using this HRM system.

Spatial anatomy of the nasofrontal recess on the CT using multiplanar reconstruction technique.

OBJECTIVE: To clarify the spatial orientation relevant to the endoscopic sinus surgery (ESS) for the frontal sinus. METHODS: Various dimensions of structures around the frontal recess were measured on 256 CT images of 128 Japanese patients using the multiplanar reconstruction technique. Measurements done in this study were: the distance from the nostril to the narrowest point of the frontal recess (D1), and the distance from the narrowest point of the frontal recess to the bony wall of the anterior skull base on an extension of the same line (D2). The smallest anterior-posterior (D3) and right-left (D4) diameters of the "route to the frontal sinus," a site containing the narrowest airspace in the frontal recess, being surrounded by the posterior edge of maxillary bone anteriorly, by the bony wall of the anterior skull base posteriorly, by the middle turbinate medially, and by the medial orbital bony wall laterally, respectively, on the parasagittal and coronal planes. The angle between the line connecting the nostril to the lowest portion of the frontal process of the maxilla and frontal bone, and the line connecting the lowest portion of the frontal process to the narrowest portion of the frontal recess (A1). RESULTS: The mean values D1-D4 were 55.9, 9.4, 6.9 and 8.2mm. In 154 of 256 sides, A1 ranged from 129.5 degrees to 175.7 degrees. CONCLUSION: The present study provides important information about the spatial anatomy of the nasofrontal recess, which is essential for avoiding complications of an ESS for the frontal sinus.

Subjective visual vertical test in patients with chronic dizziness without abnormal findings in routine vestibular function tests.

CONCLUSION: The subjective visual vertical (SVV) test can detect abnormality of the otolithic organs and the graviceptive pathways present in a considerable number of patients having dizziness but presenting no abnormal findings in conventional vestibular function tests. OBJECTIVE: To evaluate whether the SVV test can detect dysfunction of the otolithic organs and perception of gravity in patients with dizziness having no abnormal finding on routine tests for the vestibular system. PATIENTS AND METHODS: Forty-four patients who complained of chronic dizziness but had no abnormal finding on routine tests for vestibular system and on brain MRI studies were selected between 2004 and 2006. SVV tests were performed on these patients. Patients with chronic dizziness caused by apparent psychogenic disorders, such as depression, were excluded. RESULTS: Among the 44 patients, 3 showed abnormal tilts of SVV. The latter three patients had deep white matter hyperintensities on their MRI, probably due to aging.

A case with posterior fossa epidermoid cyst showing audiovestibular symptoms caused by insufficiency of anterior inferior cerebellar artery--usefulness of free DICOM image viewing and processing software.

A 58-year-old Japanese man suddenly suffered from vertigo. On physical examination, left-beating horizontal torsional spontaneous nystagmus was observed; the direction did not change with gaze. Other neurotological examinations revealed findings within normal limits except the left side sensorineural hearing loss of approximately 32 dB on average. Diffusion-weighted MRI revealed no infarction in the brain, but demonstrated an epidermoid cyst in the left cerebello-pontine cistern region. Using free digital imaging and communications in medicine (DICOM) image viewing and processing software, it was found that the epidermoid cyst clearly compressed the left anterior inferior cerebellar artery (AICA). Therefore, we speculated that insufficiency of the left AICA caused his audiovestibular symptoms. This new technique used in the present study was considered useful when the site responsible for vertigo is suspected in the cerebello-pontine angle, where anatomic relationships between the nerves and the vessels are complicated.

Oxygen consumption by bacteria: a possible cause of negative middle ear pressure in ears with otitis media.

CONCLUSIONS: Oxygen consumption by bacteria could be a cause of the negative middle ear pressure in ears with otitis media (OM). OBJECTIVE: To determine whether oxygen consumption by bacteria could be a cause for production of negative pressure in ears with OM. MATERIALS AND METHODS: Hermetically sealed bottles containing high dose (group A) and low dose (group B) of Streptococcus pneumoniae with air space and maintained at 37 degrees C in a water bath were connected to a micropressure sensor. The chronological pressure changes were monitored in vitro for 3-13 h and were compared with those in the control bottles containing culture medium only. RESULTS: The pressure of the group A samples showed significantly lower values than that of controls 3 h later (p<0.0001). In group B, the pressure was also significantly lower than that in the control group (p<0.0001). The partial pressures of oxygen measured at the beginning and end of the experiment in the six samples in group B showed significant decrease, while that in the control group showed only a slight decrease (p<0.0019).

Solitary fibrous tumor arising from the sphenoid sinus.

Solitary fibrous tumor (SFT) is an uncommon neoplasm that usually arises from the pleura. To our knowledge, only 30 cases of SFTs in the nasal cavity and paranasal sinuses have been reported in the literature. We describe an SFT that arose from the right sphenoid sinus and extended to the nasal cavity and epipharynx. The tumor was completely removed by endoscopic sinus surgery without complication. The patient is taking an uneventful course without any evidence of recurrence of the disease 8 months after surgery now.

Cochlear implantation in children with congenital cytomegalovirus infection.

OBJECTIVE: To assess the impact of cochlear implantation (CI) on children with cytomegalovirus (CMV)-induced deafness. STUDY DESIGN: Retrospective chart review. PATIENTS: Four children with congenital CMV-related deafness (CMV group) and 17 children with congenital deafness without CMV infection as the cause of deafness (non-CMV group). The age at CI ranged from 2.0 to 3.3 years (mean, 2.6 yr) in the CMV group and from 1.8 to 3.6 years (mean, 2.6 yr) in the non-CMV group. Their follow-up period ranged from 3.0 to 4.3 years (mean, 3.3 yr) in the CMV group and from 1.6 to 4.3 years (mean, 3.3 yr) in the non-CMV group. METHODS: Evaluation and comparison of preoperative and postoperative hearing levels, motor, social, and language development, Infant-Toddler Meaningful Auditory Integration Scale, and Enjoji Scale of Infant Analytical Development between the 2 groups. RESULTS: Within 12 months after CI, the mean score of both language perception and production was poorer in the CMV group than in the non-CMV group, and the difference in the language production was statistically significant. However, 12 months after CI, the language perception and production showed good progress at levels similar to that of the non-CMV group. CONCLUSION: Long-term results of the language perception and production after CI was satisfactory in Japanese children with congenital CMV-related deafness compared with in subjects deafened by other causes. CMV alone, as a cause of deafness, is not likely a contraindication for CI.

Effects of a tongue-holding maneuver during swallowing evaluated by high-resolution manometry.

OBJECTIVE: This study aimed to investigate the effects of a tongue-holding maneuver (THM) during swallowing using a novel high-resolution manometry (HRM) system. STUDY DESIGN: Case series with planned data collection. MATERIALS AND METHODS: Thirty-three asymptomatic Japanese adults were studied. A solid-state HRM assembly with 36 circumferential sensors spaced 1-cm apart was positioned to record pressures from the velopharynx to the upper esophagus at rest and during swallowing. The maximum values of the dry swallowing pressures at the velopharynx, mesohypopharynx, upper esophageal sphincter (UES), and distance from the nostril to each point of maximum values with and without the THM were measured. RESULTS: The distance from the nostril to the UES was statistically shorter when swallowing with the THM than without the THM (paired t test, P=0.009). The maximum pressure at UES was greater when swallowing with the THM than without the THM, although there was no statistically significant difference (paired t test, P=0.051). There was no difference in the pressures and the distance between swallowing with or without the THM at any other site. CONCLUSIONS: These findings suggest that the THM may not have a potential to facilitate compensatory swallowing power when swallowing.

Amplitude and area ratios of summating potential/action potential (SP/AP) in Meniere's disease.

CONCLUSIONS: Our results suggest that summating potential/action potential (SP/AP) area ratio may not necessarily have higher sensitivity in the diagnosis of endolymphatic hydrops of Meniere's disease (MD) than SP/AP amplitude ratio in transtympanic electrocochleography (ECochG). OBJECTIVE: Recent studies suggested that SP/AP area curve ratio was more sensitive to endolymphatic hydrops in comparison with SP/AP amplitude ratio in extratympanic ECochG. The purpose of the present study was to evaluate the utility of the SP/AP area curve ratio in transtympanic ECochG for the diagnosis of MD. PATIENTS AND METHODS: A retrospective chart review of 198 patients (209 ears) was conducted in cases of MD. RESULTS: With regard to SP/AP amplitude ratio, 57.1% in definite cases of MD (group 1), 39.6% in probable cases of MD (group 2), and 50.0% in the cases who had transformed from probable MD to definite MD (group 3) showed abnormally high values, respectively. Abnormally high values were observed in 43.9%, 27.7%, and 30.0% in SP/AP area ratio in groups 1, 2, and 3, respectively, indicating that abnormal values were observed more frequently in the amplitude ratio than in the area ratio in all three groups.

Subjective visual vertical in acute attacks of Ménière's disease.

OBJECTIVES: To investigate whether or not and how often there is otolith dysfunction in an acute attack of Ménière's disease. PATIENTS: Twenty-two definite cases of unilateral Ménière's disease diagnosed in accordance with the 1995 criteria of the American Academy of Otolaryngology-Head and Neck Surgery for Ménière's disease. INTERVENTION: Subjective visual vertical (SVV) test was performed before, at, and after acute attacks on 22 patients with unilateral Ménière's disease who showed normal tilts of SVV before acute attacks. MAIN OUTCOME MEASURE: Abnormal tilts of SVV. RESULTS: Of 22 cases, 14 (63.6%) with unilateral Ménière's disease showed abnormal tilts of SVV in acute attacks. The tilts were toward the side of the affected ear in 13 (92.9%) of them. Abnormal tilts returned to normal within a few weeks after the acute attacks in 12 (85.7%) of the 14 cases with unilateral Ménière's disease. CONCLUSION: Otolith dysfunction occurred in acute attacks in a considerable number of patients with Ménière's disease. Subjective visual vertical can be used as a good tool for the evaluation of otolith dysfunction at acute attacks in patients with Ménière's disease.