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Objective: An intact cochlear nerve is necessary for successful cochlear implantation (CI). Although the promontory stimulation test (PST) using a promontory stimulator (PS) and a transtympanic needle electrode is invasive, it is still commonly used to verify cochlear nerve function. PSs are currently unavailable because they are no longer manufactured; however, considering that PST continues to be beneficial in certain situations, alternative equipment is needed. The PNS-7000® (PNS) was developed as a neurologic instrument for stimulating the peripheral nerves. This study investigated the usefulness of the ear canal stimulation test (ECST) using PNS with a silver ball ear canal electrode, which is a new noninvasive alternative technique to the PST., Methods: ECST was performed from November 2013 to December 2018 using PS and PNS for patients with severe to profound sensorineural hearing loss. The electrical threshold, most comfortable loudness level, uncomfortable loudness level, dynamic range, and gap detection were measured in the ECST. The results of these measured PNS items were compared with PS., Results: ECST was performed in 61 ears of 35 patients (age, 59.9 ± 20.1 years) using PS and PNS. The sound sensation was elicited in 51 (83.6%) and 52 (85.2%) ears with PS and PNS, respectively. All items excluding GAP were measured in 46 (75%) and 43 (70%) ears at 50 and 100 Hz, respectively. GAP was measured in 33 ears by the ascending and descending methods using PS and PNS. Spearman's rank-order correlation coefficient revealed a significant positive linear correlation between the PS and PNS results in all measurements. No significant difference was found between the PS and PNS thresholds in all measured items., Conclusions: PNS is a useful instrument for performing ECST as a new alternative to PS. ECST using a silver ball electrode is a less invasive and easier test than PST., Competing Interests: Declaration of Competing Interest The authors have no financial conflicts of interest to declare., (Copyright © 2023. Published by Elsevier B.V.)

Objective: We aimed to evaluate the relationship between hearing ability and cognitive domains and determine how the relationship changes after 6 months of introducing a hearing aid., Methods: We conducted a 6-month hearing aid lending study between September 2014 and March 2019, including 59 older participants who visited the Memory Clinic at the National Center for Geriatrics and Gerontology. The hearing level was assessed using pure tone audiometry. Speech intelligibility was measured using the monosyllabic word discrimination score. We assessed the relationship between hearing ability and cognitive domains using the Mini-Mental State Examination (MMSE) total score and four subscale scores (orientation, memory, attention, and language). Differences in the cognitive function between baseline (pre-) and 6 months later (post-) after introducing a hearing aid were also assessed., Results: The pre-orientation score was significantly associated with the pure-tone average (p = 0.013), and the pre-language score was significantly associated with speech intelligibility (p = 0.006) after adjusting for confounders. None of the MMSE subscale scores were significantly different between pre- and post-scores, however, an expectation of improvement with continuous hearing aid use was implied in the attention domain., Conclusion: We found a significant association between hearing ability and cognitive domains in individuals whose cognitive functions were not considered healthy. The presence of a potential relationship between cognitive domains, hearing ability, and auditory compensation is suggested., (Copyright © 2022 Japanese Society of Otorhinolaryngology-Head and Neck Surgery, Inc. Published by Elsevier B.V. All rights reserved.)

Objective: Hearing loss is a risk factor for cognitive impairment, and the use of a hearing aid (HA) may prevent cognitive decline alongside hearing loss. We aimed to elucidate the prevalence of self-reported HA usage in Japanese community-dwelling elders with hearing loss, and the effect of hearing and HA on cognitive impairment., Methods: A total of 1193 participants, who had audiometric defined hearing loss and were aged 60 years or over, had their cumulative 3260 observations followed up for 10 years from a large cohort of a Japanese study. Association between hearing (pure-tone average threshold level at 500, 1000, 2000, and 4000 Hz from the better hearing ear: PTABHE) and HA usage with cognitive impairment (total score of Mini-Mental State Estimation was under 27 or diagnosed as dementia) was analyzed using generalized estimating equations., Results: The HA usage rate of the 1193 community-dwelling elders with hearing loss was 6% during the first involvement. The majority (59.2%) of HA users always used an HA. HA usage rate was 0.7% for the mild hearing loss group and 32.4% for the moderate or greater hearing loss group in the latest participating wave. PTABHE was significantly associated with cognitive impairment (odds ratio for every 10 dB 1.36; 95% CI 1.21-1.53, p<0.0001) after adjusting for age, sex, education, depressed mood, smoking status, alcohol intake, income, activity, obesity, histories of hypertension, dyslipidemia, ischemic heart disease, diabetes, stroke, ear disease, and occupational noise exposure. PTABHE was also significantly associated with cognitive impairment in the mild hearing loss group (odds ratio for every 10 dB 1.34; 95% CI 1.05-1.72, p = 0.020) and moderate hearing loss group (odds ratio for every 10 dB 1.82; 95% CI 1.27-2.61, p = 0.001). HA use showed a significant suppressive effect on cognitive impairment in those with moderate hearing loss who always use an HA (odds ratio 0.54; 95% CI 0.30-1.00, p = 0.049)., Conclusion: The prevalence of HA usage among Japanese community-dwelling elders with hearing loss is consistent, at around 10%. The hearing level remained a primary risk factor for cognitive impairment among elders with hearing loss after adjusting for several confounding factors. Regular HA use may have a protective effect on cognitive impairment in those with moderate hearing loss., Competing Interests: Declaration of Competing Interest None., (Copyright © 2021. Published by Elsevier B.V.)

Objective: Understanding the relationships among aging, cognitive function, and olfaction may be useful for diagnosing olfactory decline in older adults. Olfactory function declines in the early stage of neurodegenerative diseases, including Alzheimer's and Parkinson's diseases. Aging and cognitive impairment are associated with olfactory decline. Moreover, the assessment of hyposmia and anosmia is paramount to the diagnosis of neurodegenerative diseases. We aimed to assess the relationships among aging, cognitive function, and olfaction in patients with olfactory impairment., Methods: This observational study included 141 patients with olfactory deterioration who presented with dementia, mild cognitive impairment (MCI), age-related hyposmia, or postviral olfactory dysfunction (PVOD). The patients underwent T&T olfactometry, a self-administered odor questionnaire (SAOQ), a visual analog scale (VAS), and a Mini-Mental State Examination., Results: T&T odor recognition thresholds decreased with aging (p < 0.01) and cognitive impairment (p < 0.08). The average T&T recognition thresholds were 5.1,4.6,4.2, and 3.7 in dementia, MCI, age-related hyposmia, and PVOD, respectively. Moreover, the average differences between the detection and recognition thresholds were 3.7, 2.8, 2.3, and 2.0 in dementia, MCI, age-related hyposmia, and PVOD, respectively. Hyposmia with dementia presented the highest recognition thresholds (p < 0.05) and the largest differences between the T&T detection and recognition thresholds, compared with age-related hyposmia and PVOD (p < 0.05). Hyposmia with dementia had the highest SAOQ and VAS scores compared with the other groups (p < 0.05)., Conclusion: The possibility of dementia should be investigated in patients with hyposmia, including those with high T&T recognition thresholds, a large difference between the T&T detection and recognition thresholds, and high SAOQ and VAS scores., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020. Published by Elsevier B.V.)

Objective: Involvement in the tracheostomy procedure for COVID-19 patients can lead to a feeling of fear in medical staff. To address concerns over infection, we gathered and analyzed experiences with tracheostomy in the COVID-19 patient population from all over Japan., Methods: The data for health-care workers involved in tracheostomies for COVID-19-infected patients were gathered from academic medical centers or their affiliated hospitals from all over Japan., Results: Tracheostomies have been performed in 35 COVID-19 patients with a total of 91 surgeons, 49 anesthesiologists, and 49 surgical staff members involved. Twenty-eight (80%) patients underwent surgery more than 22 days after the development of COVID-19-related symptoms (11: 22-28 days and 17: ≥29 days). Thirty (85.7%) patients underwent surgery ≥ 15 days after intubation (14: 15-21 days, 6: 22-28 days, and 10: ≥29 days). Among the total of 189 health-care workers involved in the tracheostomy procedures, 25 used a powered air-purifying respirator (PAPR) and 164 used a N95 mask and eye protection. As a result, no transmission to staff occurred during the 2 weeks of follow-up after surgery., Conclusion: No one involved in tracheostomy procedures were found to have been infected with COVID-19 in this Japanese study. The reason is thought to be that the timing of the surgery was quite late after the infections, and the surgery was performed using appropriate PPE and surgical procedure. The indications for and timing of tracheostomy for severe COVID-19 patients should be decided through multidisciplinary discussion., Competing Interests: Declaration of Competing Interest We declare that we have no conflicts of interest., (Copyright © 2021. Published by Elsevier B.V.)

Objectives: Primary squamous cell carcinoma (SCC) of the thyroid is a rare disease. It usually presents with locally advanced disease and has an overall poor prognosis. In this study, we investigated the characteristics and outcomes of patients with SCC of the thyroid, and reported our experience with chemotherapy with lenvatinib in the treatment of SCC of the thyroid., Methods: The management outcome of 10 patients who had SCC of the thyroid between January 2000 and 2015 at Kyushu University Hospital or associated facilities was reviewed., Results: There were 3 males and 7 females, ranging in age from 53 to 77 years. Extent of disease was staged as follows: stage IVA, 3 cases; stage IVB, 3 cases; stage IVC, 4 cases. Only tracheostomy was applied for 2 cases, surgical resection, such as total thyroidectomy and neck dissection, for the other 8 cases. Radiotherapy following surgical treatment was applied for 9 cases. Four patients started on oral lenvatinib due to recurrent or progressive SCC of the thyroid. The one year actuarial survival rate of patients was 22.7%. There was no 2-year survivor of all patients., Conclusions: Treatment should primarily be targeted at surgical resection with negative margins in patients with resectable disease. Lenvatinib may show promise to potentially extend survival., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objective: To examine endolymphatic hydrops (EH) using magnetic resonance imaging (MRI) in patients with definite Ménière's disease (MD) and those with nonotological diseases., Methods: We studied 32 patients with unilateral MD, 10 patients with bilateral MD and 21 patients with control ears who had other benign diseases not associated with hearing or vestibular dysfunction. The mean age of the subjects was 54.0 years (range 27-74) in the MD group and 56.1 years (range 24-79) in the control group. Using MRI, the degree of EH was classified as none, mild and significant in the cochlea and vestibule separately. The ratio of the area of endolymphatic space to the vestibular fluid space was calculated for the vestibule. The duration of MD was defined as the months between the first attack of MD and the MRI study., Results: EH was present in the cochlea of 45/52 affected ears of patients with MD (87%) and in 16/42 control ears (38%). Significant cochlear hydrops was present in 37/52 affected ears (71%) and in 4/42 control ears (10%). EH in the vestibule was present in 49/52 affected ears (94%) and in 3/42 control ears (7%). Significant vestibular hydrops was present in 40/52 affected ears (77%) and in none of the 42 control ears. There was no relationship between the degree of EH and its duration. Using a cut off value for the relative size of EH in the vestibule of 41.9%, the test had a sensitivity of 88.5% and a specificity of 100% to diagnose definite MD., Conclusion: Cochlear EH was occasionally observed in control ears on MRI, as in normal temporal bone specimens. The presence or absence and degree of vestibular EH were significantly different between ears with MD and control ears. EH in the vestibule might be a specific predictor of definite MD., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Olfactory neuroblastoma (ONB) is a relatively rare nasal or paranasal malignant tumor. This tumor is rarely accompanied by paraneoplastic syndromes such as syndrome of inappropriate antidiuretic hormone secretion (SIADH). Here, we report a 31-year-old female with histologically confirmed ONB who had been diagnosed with SIADH three years prior. She was treated with surgery followed by concurrent chemoradiotherapy. SIADH resolved immediately after surgical tumor resection. Immunohistochemically, both biopsy and resected specimens from the nasal cavity had been negative for ADH. Although extremely rare, ONB may be associated with SIADH, and the possibility of this cancer should be taken into account during the follow-up of idiopathic SIADH., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Objective: The first revision of "Japanese Clinical Practice Guideline for Head and Neck Cancer" was made in 2013 by the clinical practice guideline committee of Japan Society for Head and Neck Cancer, in response to the revision of the TNM classification., Methods: 34 CQs (Clinical Questions) were newly adopted to describe the diagnosis and treatment methods currently considered most appropriate, and offered recommendation grade made by the consensus of the committee. A comprehensive literature search was performed for studies published between 2001 and 2012 using PubMed. Qualified studies were analyzed and the results were evaluated, consolidated and codified by all the committee members., Results: Elective neck dissection (ND) does contribute to improvement in survival and should be performed for patients with high-risk tongue cancer. At present, no research has clearly demonstrated the utility of superselective arterial infusion chemotherapy. However, depending on the site and stage of the cancer, combination with radiotherapy may be useful for preserving organ function or improving survival rate. Concurrent CDDP chemotherapy and adjuvant radiotherapy contributes to improvement of survival rate as an adjuvant therapy for advanced squamous cell carcinoma of the head and neck in patients at high risk of recurrence. The anti-EGFR antibody cetuximab (Cmab) has an additive effect with radiotherapy. However, the indication must be carefully considered since this treatment has not been compared with the standard treatment of chemoradiotherapy. Cmab has been shown to have an additive effect with chemotherapy (CDDP/5-FU) in patients with unresectable metastatic or recurrent cancer. Preoperative and postoperative oral care may reduce the risk of postoperative complications such as surgical wound infection and pneumonia in head and neck cancers. Rehabilitation soon after ND for cervical lymph node metastasis is recommended for maintaining and restoring shoulder function., Conclusions: In this article, we described most relevant guidelines and CQs for the diagnosis and treatment of head and neck cancer in Japan. These guidelines are not intended to govern therapies that are not shown here, but rather aim to be used as a guide in searching for the most appropriate treatment for individual patient., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Combined small cell carcinoma (SCC) and squamous cell carcinoma (SqCC) of the oropharynx is extremely rare and shows an aggressive clinical course. There are only 5 reported cases of combined SCC and SqCC in the English language literature. Here, we report a 59-year-old male presenting with a right tonsillar mass. The mass was biopsied, and the histological findings showed a proliferation of small-sized tumor cells with scant cytoplasm. Immunohistochemically, the tumor cells were positive for neuroendocrine markers (synaptophysin, chromogranin A, and CD56). Our first diagnosis was tonsillar small cell carcinoma. We treated the patient with concurrent chemoradiotherapy together with cisplatin followed by surgery. The resected tonsillar specimen showed a residual tumor composed of SCC and SqCC, and lymph nodes showed metastatic tumor cells of the SCC component. Immunohistochemically, the SCC component was positive for all neuroendocrine markers and p16; on the other hand, the SqCC component was positive for p40, p63, p16, and EGFR. Fluorescence in situ hybridization revealed that neither component showed any EGFR gene copy number gain. The patient was treated with adjuvant chemotherapy consisting of irinotecan and cisplatin. Liver and bone metastases developed, resulting in the death of the patient. We discuss the present case and review similar cases. Most cases of combined SCC and SqCC occur regardless of p16 status, and a therapeutic strategy has yet to be determined. Further examination of this kind of combined tumor is necessary., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Objective: The aim of this study was to investigate the clinical features and prognosis of patients with squamous cell carcinoma (SCC) associated with sinonasal inverted papilloma (IP)., Methods: The medical records of 95 patients who were diagnosed with IP or SCC associated with IP were retrospectively reviewed. Out of 95 patients, 15 were diagnosed with SCC associated with IP. The clinical characteristics, treatment modalities, and survival outcomes of the patients were analyzed., Results: The incidence of SCC associated with IP was 15.8%. Although differential diagnosis between IP and SCC associated with IP is difficult, epistaxis may be the specific symptom in SCC associated with IP cases. The 3-year disease-specific survival rate was higher in cases with T1, 2 and 3 than in cases with T4. There was no significant difference in survival rate between maxillary sinus and other primary sites. On the other hand, there was a significant difference in survival rate between the microscopic SCC with IP cases and the other cases. In addition, the patients with <70 years old better than those with >70 years old with a 3-year disease free survival of 80% versus 0%., Conclusions: Some T4 patients were found to have a highly aggressive disease. Therefore, complete surgical resection followed by chemo-radiation therapy is the recommended treatment for patients with T4 disease to control of the primary tumor site., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Objective: To document the clinical features and associated pure-tone audiometry data in patients with enlargement of the vestibular aqueduct (EVA), and to identify risk factors for fluctuating hearing loss (HL) and vertigo/dizziness in EVA patients., Methods: In this nationwide survey in Japan, a first survey sheet was mailed to 662 board-certified otolaryngology departments to identify the ones treating EVA patients. A second survey sheet, which contained solicited clinical information and the results of the hearing tests, was mailed to all facilities that reported treating EVA cases. We analyzed clinical information, including age at the time of the most recent evaluation, gender, EVA side, age at onset, initial symptoms, precipitating factors, and etiology from survey responses, and assessed 4-frequency (500, 1000, 2000, and 4000Hz) pure-tone average (PTA) from accompanying pure-tone audiometry data. A multivariate logistic regression analysis was utilized to identify the possible risk factors for fluctuating HL and vertigo/dizziness., Results: In total, 513 hospitals (response rate, 77.5%) responded to the first survey, and 113 reported treating patients with EVA. Seventy-nine out of the 113 hospitals (response rate 69.9%) responded to the second survey, and the data of 380 EVA patients were registered and analyzed. Of the 380 patients, 221 (58.2%) were female, suggesting female preponderance. The patient age ranged from 0 to 73 years (mean, 16.7 years; median, 13 years; interquartile range, 6-24 years). EVA was bilateral in 91.1% of the patients (346/380). The most prevalent initial symptom was HL (341/380), followed by vertigo/dizziness/imbalance (34/380). Sudden HL occurred secondary to head trauma in 5.3% of the patients and upper respiratory infection in 5.0%. Pure-tone audiometry showed profound HL (PTA >91dB) in 316 (52.0%) of the 608 ears in the 304 patients tested, and asymmetric HL, defined as >10dB, in 147 (48.4%) of the 304 patients. The mean PTA was 83.7dB (median, 91.3dB; interquartile range, 71.3-103.8dB), and the severity in PTA did not correlate with age. Multivariate logistic regression identified age ≥10 years (compared to age of 0-9 years), bilateral HL (compared to unilateral HL/normal hearing), a history of head trauma, and Pendred syndrome (compared to the other EVA-associated disorders) as significant risk factors for fluctuating HL and/or vertigo/dizziness., Conclusion: The present nationwide survey of 380 EVA patients provided a more precise description of the clinical features, including risk factors for fluctuating HL and vertigo/dizziness., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Objective: Eyelid carcinomas are rare, and the management strategy of regional lymph node metastasis linked to eyelid carcinomas has not been standardized to date. The aim of the present study was to analyze the patterns of regional metastasis and to assess the optimal extent of surgical treatment for lymph node metastasis of eyelid carcinoma., Methods: This study was a retrospective review of patient data from a single institution. From a series of 268 eyelid carcinomas, we selected the 21 patients with lymph node metastasis, and we analyzed the patterns of lymphatic spread, approach to treatment and outcomes., Results: The most common histological type of eyelid carcinoma with regional metastasis was sebaceous carcinoma (17/21, 81.0%). Submandibular area metastases were seen only in the patients with the primary tumor originating in the medial half of the eyelid, but parotid area metastases were seen in both the patients whose tumors had a medial-half origin and those with a lateral-half origin. Although 11 of the 16 patients with parotid-area metastases underwent a tumorectomy or superficial parotidectomy (which resulted in four cases of recurrence in the parotid area), none of the five patients who underwent a total parotidectomy developed parotid-area recurrence. The incidence of regional recurrence of the patients who received adjuvant radiotherapy (14.3%) was lower than that of the patients without adjuvant radiotherapy (57.1%)., Conclusion: Continued surveillance and optimal management of regional lymph node metastases are important for the control and survival of eyelid carcinomas., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Objective: The treatment of head and neck squamous cell carcinoma (HNSCC) with N3 (>6cm) lymph nodes remains difficult, and the best treatment strategy has not been elucidated. The aim of this study was to evaluate the outcomes of various treatment modalities., Methods: Sixty-nine patients with HNSCC and N3 neck disease treated with definitive therapy in our institute between 1987 and 2013 were included in the analysis. We compared the clinical outcomes of radiotherapy (RT) alone, chemoradiotherapy (CRT) and surgery with or without induction chemotherapy (ICT)., Results: The overall survival (OS) at three years for the patients with N3 neck disease was 41%. The three-year OS rates of patients treated with definitive surgery and definitive CRT were 41% and 48%, respectively. There were no significant differences between these two treatments (P=0.82). The OS of patients who received ICT followed by definitive therapy was significantly better than that of patients who did not (P<0.001). The most common recurrence pattern was distant metastases. The rate of distant metastases was 61% of all treatment failures (20/33)., Conclusion: The high rate of distant metastases in patients with N3 neck disease suggests that prevention of distant metastases can improve survival. Based on this study, we consider that ICT may play an important role in the treatment of N3 neck disease., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

The Japan Society for Head and Neck Surgery (JSHNS) started a board certification system for head and neck surgeons in 2010. To become certified, the following qualification and experiences are required: (1) board certification as otorhinolaryngologist, (2) 2 years of clinical experience in a board-certified training facility, (3) clinical care of 100 patients with head and neck cancer under the supervision of board-certified faculty and (4) surgical experience in 50 major head and neck surgical procedures, including 20 neck dissections, under the supervision of board-certified faculty. The following scientific activities are also required during the preceding 5 years: (1) two clinical papers on head and neck cancers presented at major scientific meetings, (2) one clinical paper on head and neck cancer published in a major journal, (3) attendance at two annual meetings of JSHNS and (4) enrolment in three educational programs approved by JSHNS. The qualifying examination consists of multiple choice tests and oral examinations. A total of 151 head and neck surgeons were certified in 2010 followed by 43 in 2011 and 34 in 2012, while the membership of JSHNS dramatically increased from 1201 in 2007 to 1748 in 2013. Although the board certification system for head and neck surgeons was started only recently, it has encouraged many residents and fellows as well as established head and neck surgeons. We believe that this system will contribute to further advancement in the clinical practice for head and neck cancers in Japan., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Cricoid cartilage fractures usually occur concurrently with disorders of laryngeal function. In, particular, displaced cricoid lamina fractures can affect arytenoid movement. However, functional, recovery may require proper repositioning of the cricoid lamina, which is associated with a high rate of, complications. Here we present a case in which an isolated cricoid cartilage fracture with arytenoid, immobility due to displacement of the fracture in the cricoarytenoid joint space was successfully, treated. Our findings suggest that a combination of external approaches with temporary, cricothyrotomy and wide suturing of the entire cricoid framework has the potential to improve, arytenoid movement and prevent associated complications., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Objectives: Microsurgery is difficult to perform in necks that have been previously irradiated and operated upon because of the limited availability of recipient vessels. The objective of this study was to clarify the feasibility and safety of performing microsurgery in necks that are scarred and fibrous owing to previous treatment., Methods: Twenty patients whose necks were previously irradiated and operated upon and who underwent free tissue transfer were included in this study. All patients had been previously administered an average of 60.7 (range, 30-95)Gy of radiotherapy. Thirteen patients had undergone hemilateral neck dissections, 5 patients had undergone bilateral neck dissections, 8 patients had undergone (pharyngo)laryngectomies, and 10 patients had undergone prior flap transfer. The success rate of microsurgery and the selection of recipient vessels were examined., Results: All recipient vessels could be adopted in the neck field without vessel grafting. One patient developed necrosis of the flap, which was salvaged with retransfer of another flap after trimming the same cervical vessels. For the remaining 19 patients, free tissue transfers were successful., Conclusions: Suitable recipient vessels are residual and available even in the previously irradiated and operated neck field. When performed properly, free tissue transfer in the previously treated neck is not as risky a surgery as was generally believed., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Visualization of endolymphatic hydrops has been performed using magnetic resonance imaging (MRI) after intratympanic or intravenous gadolinium (Gd) injection. Our recent findings indicate that just as the prevalence of asymptomatic glaucoma is greater than that of symptomatic glaucoma, there are also many cases of asymptomatic endolymphatic hydrops. It is assumed that the asymptomatic endolymphatic hydrops that precedes Ménière's disease is found more frequently using MRI than with other techniques. Gd in the inner ear moves into the cerebrospinal fluid (CSF) via the internal auditory meatus. Gd enhancement is also recognized in the ocular fluid after the intravenous Gd administration. In this paper, the relationships between CSF, ocular fluid and inner ear fluid are reviewed. The central nervous system, eye and inner ear contain specialized extracellular fluids that are essential for maintaining their function: CSF, ocular fluid consisting of vitreous humor and aqueous humor, and inner ear fluid consisting of perilymph and endolymph. Abnormal accumulation of or pressure elevation in these fluids is associated with hydrocephalus, glaucoma and Ménière's disease, respectively. The dura mater and the arachnoid membrane of the optic nerve canal and inner ear meatus are very close to the eye and the inner ear, respectively. It has been reported that low CSF pressure is associated with glaucoma and endolymphatic hydrops. In glaucoma and Ménière's disease, nerve damage to ganglion cells rather than damage of the sensory cells is directly associated with progression of the disease. Retinal ganglion cells in glaucoma and spiral ganglion cells in Ménière's disease are targets of the abnormal accumulation of, or increased pressure in, the extracellular fluid, just as neurons are damaged in hydrocephalus. Studies on hydrocephalus, glaucoma and Ménière's disease as a group may deepen our understanding of each disease., (Copyright © 2011. Published by Elsevier Ireland Ltd.)

Genetic epidemiology focuses on the genetic determinants in the etiology of disease among populations and seeks to elucidate the role of genetic factors and their interaction with environmental factors in disease occurrence. In recent years, genetic epidemiological research has become more focused on complex diseases, and human genome analysis technology has made remarkable advances. Age-related hearing impairment (ARHI) is a complex trait, which results from a multitude of confounding intrinsic and extrinsic factors. Although the number of genetic investigations of ARHI is increasing at a surprising rate, the etiology of ARHI is not firmly established. In this article, we review (1) the methodological strategies used to analyze genetic factors that contribute to human ARHI, (2) several representative investigations, and (3) specific genetic risk factors for human ARHI identified in previous work., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Intracochlear schwannomas (ICSs) are rare tumors. The diagnosis of ICS is based on high-resolution magnetic resonance imaging (MRI), which should be used for the accurate determination of the location of tumors. Recent advancements in imaging technologies and software enable the precise regional diagnosis of ICS. We experienced a case of intracochlear schwannoma with a diagnosis of progressive hearing loss and tinnitus. Audiometry revealed severe hearing loss in the right ear with remaining low-frequency hearing. MRI showed an abnormal lesion in the cochlea. Three-dimensional real inversion recovery (3D rIR) and constructive interference in steady state (CISS) MRI revealed the size and shape of the tumor via identification of the cochlear nerve and cochlear fluid space. CISS and 3D rIR sequences provide useful information regarding the boundaries of tumors and the tissues that surround them., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Objective: The aim of this study was to evaluate the immunohistochemical TS expression in patients with maxillary sinus SCC., Methods: The value of immunohistochemical TS expression as a predictive indicator for 5-FU efficacy was retrospectively examined in 47 patients with maxillary sinus SCC., Results: Of the 47 patients, 29 (62%) showed complete response for 5-FU based chemoradiotherapy. Seventeen of 19 (89%) TS low cases showed a complete response, whereas 12 of 28 (43%) TS high cases showed complete response for 5-FU based chemoradiotherapy. Low TS patients had significantly better response rates compared with high TS patients., Conclusion: These findings suggest that TS expression affects the chemotherapeutic effect of 5-FU in patients with maxillary sinus SCC and the assessment of TS expression level might be useful both in the management and in the treatment of maxillary sinus SCC., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Objective: To investigate relevance and characteristics of gastroesophageal reflux (GER) in adult patients with otitis media with effusion (OME) of unknown etiology who attended private clinics., Materials and Methods: A total of 186 adults with OME of unknown etiology (OME group) and 156 adults without OME (control group) were asked to answer a questionnaire specific for the diagnosis of GER disease. Pepsinogen (PG) levels in the middle-ear effusions (MEEs) of the OME group were measured using a chemiluminescence enzyme immunoassay kit. Distributions of PG concentrations by age or body mass index (BMI) in the OME group were analyzed. Patients with high PG levels received proton pump inhibitors (PPIs) and their responses were evaluated., Results: Symptoms of GER were reported by significantly more patients in the OME group than in the control group (43.0% vs. 12.8%). Patients with GER symptoms tended to have higher MEE PG concentrations than those without symptoms. PG levels did not show a significant difference by age. However, high PG levels were less found in patients over 60 years old with high BMI >25. This tendency was not observed in patients under 60 years old. PG levels decreased in seven out of ten patients with high PG concentrations after PPI therapy, corresponding with palliation of GER-related symptoms. Two patients had high MEE bilirubin concentration, and OME resolved in these patients after instruction about lifestyle factors related to GER, including sleeping position., Conclusions: GER symptoms were more prevalent than expected in patients with OME of unknown etiology. BMI might affect GER-related OME, especially in elderly patients. Instruction about lifestyle factors related to GER, especially in patients who do not respond to PPI therapy, may be effective for patients with intractable OME., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Objective: It has become clear through epidemiological analysis that the incidence of cancers of the lung, liver, colon, and rectum are increasing in Japan every year. However, there have been few epidemiological analyses of nasopharyngeal carcinoma (NPC) in Japan. The aim of this study was to analyze the epidemiology and current incidence of NPC in the Chubu region of Japan during the period from 1996 to 2005., Methods: Takeshita et al. conducted a similar investigation in the Chubu region 10 years ago, and, as a result, this is a comparative study. The Chubu region is the central region of Japanese main island. We researched NPC patients treated in hospitals in each prefecture over a 10-year period (1996-2005) using a questionnaire., Results: A total of 525 cases (male:385, female:134, unknown:6) were analyzed epidemiologically, histologically, serologically, and clinically in this study. The incidence per 10(5) population per year was 0.29. For the period of 1986-1995, the age-standardized incidence of NPC was 0.28 per 10(5) persons per year in Takeshita's report. There was no significant difference between the two periods. The ages of the patients ranged from 13 to 90 years. The mean age of was 55.2 years. On the basis of the World Health Organization (WHO) histological criteria, 36% of the patients were classified as WHO I, 27% as WHO II, and 37% as WHO III. Carcinoma was located in the posterosuperior region in 56%, lateral in 41%, and inferior in 3%. Tumor staging showed that 6% to belonged to stage I, 25% to stage II, 31% to stage III, and 38% to stage IV. A neck mass was present in 52% of the patients, ear symptoms in 48%, nasal symptoms in 27%, headaches in 10%, pharyngeal symptoms in 9%, ophthalmologic symptoms in 9%, and cranial neurological symptoms in 9%. The positive rates of serum titers of the antibodies to Epstein-Barr virus (EBV)-related antigens were calculated. The positive rate of anti-EBV-viral capsid antigen (VCA) immunoglobulin (Ig) G titers was 58.6%, that of anti-EBV-VCA IgA titers was 53.6%, and that of EBNA was 81%. The five-year survival rate for all patients was 67.6%, and that for those in stage I, II, III, and IV was 75%, 84%, 69%, and 53%, respectively. The five-year survival rate for stage IV was significantly lower than those for the other stages (P<0.05)., Conclusion: The age-standardized annual incidence of NPC in our survey was 0.29 per 10(5) persons per year, being relatively low and stable., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Objectives: Midkine and Pleiotrophin are low molecular weight basic proteins with closely related structures and serve as growth/differentiation factors. They have been reported to be expressed in the cochlea during the embryonic and perinatal periods. In the present study, we focused on the roles of midkine and pleiotrophin in the stria vascularis and investigated morphological changes using mice deficient in these genes., Methods: Midkine knockout, pleiotrophin knockout, and double knockout mice were used and compared to wild-type mice. Auditory brain stem responses (ABRs) and cochlear blood flows were measured in each type of mice. Pathological changes in the stria vascularis were examined by light microscopy, including immunohistochemical staining with anti-Kir4.1 antibody, and electron microscopy., Results: Hearing thresholds examined by ABRs were significantly higher in midkine knockout and pleiotrophin knockout mice than in wild-type mice. Double knockout mice showed higher thresholds compared to midkine knockout and pleiotrophin knockout mice. Blood flow in the lateral walls did not significantly differ and light microscopy examination showed an almost normal appearance of the stria vascularis in these knockout mice. However, the expression of Kir4.1 was weak in the knockout mice and severe vacuolar degeneration was observed by electron microscopy in the intermediate cells of the double knockout mice., Conclusion: The present study demonstrates that midkine and pleiotrophin play some roles for the morphological maintenance of intermediate cell in the stria vascularis., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Objectives: Arterial sclerosis contributes to inadequate blood supply to multiple organs, suggesting that general atherosclerosis may play an important role in the inner ear. Since noise is a major etiology for hearing loss, the aim of this study was to evaluate both the respective and the combined effects of arterial sclerosis and occupational noise exposure on hearing after accounting for age in middle-aged and elderly men., Methods: The evaluation was conducted using 773 subjects from a population-based sample of 1189 men, aged 40-83 years. The impact of carotid atherosclerosis (CA) or retinal arteriolosclerosis (RA) on hearing was assessed according to history of occupational noise exposure (Noise) obtained in a questionnaire. Differences in the mean pure-tone thresholds at each frequency, between the CA (+) and CA (-) groups or between the RA (+) and RA (-) groups, based on noise exposure were compared using the general linear model (GLM) Procedure in SAS, with adjustments for age. Then, the main effect of CA or RA, and the interactive effect of noise and either CA or RA on pure-tone threshold at seven frequencies were analyzed using an analysis of covariance (ANCOVA), after adjusting for age., Results: In the Noise (+) group, a statistically significant deterioration in hearing was found in the CA (+) group compared with the CA (-) group at 500 and 1000 Hz. The results in RA were significant at even lower frequencies than in CA. In the results from ANCOVA, the significant main effect of CA was shown in the pure-tone threshold at 8000 Hz, but not in the analysis of RA. A significant interactive effect of either CA or RA and Noise was observed in hearing at the range from 125 to 1000 Hz., Conclusions: The present study suggests that the impact of arterial sclerosis on hearing is limited but significantly hazardous in middle-aged and elderly men, and that arterial sclerosis exacerbates the deleterious effects of noise on hearing. Early recognition of arterial sclerosis might be contributory to the hearing prognosis after middle age, especially for noise-exposed men., (Copyright 2010 Elsevier Ireland Ltd. All rights reserved.)

Objective: The fatty acid-binding protein 2 (FABP2) is involved in the transport and metabolism of fatty acids. The FABP2 gene has been proposed as a candidate gene for diabetes and obesity. This study evaluates the hearing impairment risk in the Ala54Thr polymorphism of FABP2 in middle-aged and elderly Japanese., Methods: Our sample population comprised 1428 community-dwelling Japanese aged 40-86 years (mean+/-standard deviation [SD]: 63.1+/-9.8) who participated in the Study of Aging between 2004 and 2006. An average hearing threshold level greater than 25 dB in the better ear for frequencies 500, 1000, 2000 and 4000 Hz was defined as hearing impairment. Data were analyzed by means of a multiple logistic regression with adjustment for potential confounders., Results: The per-allele odds ratio for hearing impairment risk was 1.262 (95% confidence interval [CI]: 1.012-1.574) in model 1, adjusting for age, sex, history of ear disease, and history of occupational noise exposure; and 1.259 (CI: 1.009-1.571) in model 2, which adjusted for diabetes, body mass index and the histories of heart disease and hypertension, as well as the moderators in model 1. A significant adverse effect of the Thr54 variant on hearing was observed and the effect was independent of both diabetes and obesity in the present analyses., Conclusions: This study demonstrated that the Ala54Thr polymorphism of FABP2 was associated with a risk of hearing impairment in middle-aged and elderly people. The results might support caloric restriction theory indirectly, but additional researches are desired., (Copyright 2010 Elsevier Ireland Ltd. All rights reserved.)

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., (Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.)

Objectives: An aim of this study was to assess the predictive power of an otorhinolaryngological examination of the upper airway to identify risk factors of obstructive sleep apnea syndrome (OSAS) in the patients., Methods: We examined 141 consecutive patients with OSAS. The morphological features were assessed by the designated otorhinolaryngologist while the subjects were sitting relaxedly with tidal breathing. The bilateral nasal resistance was measured using the active anterior rhinomanometry during daytime wakefulness., Results: The body mass index (BMI), fauces's narrowness, neck circumference, lowest oxygen saturation, tonsil size and modified Mallampati grade (MMP) showed the statistically significant correlations with the apnea-hypopnea index (AHI) of an index of apnoeseverity, however, the age, Epworth sleepiness scale (ESS), nasal resistance and retroglossal space were not significantly associated with the AHI., Conclusions: The upper airway morphology significantly associated with AHI are fauces's narrowness, tonsil size, and MMP, but not nasal resistance and retroglossal space.

Objectives: The aim of this study was to clarify the interaction of lateral and supine sleeping positions with upper airway morphology in patients with obstructive sleep apnea syndrome (OSAS)., Patients and Methods: Thirty-one patients with OSAS, whose apnea/hypopnea index (AHI: number of episodes of apnea or hypopnea per hour) was over 15, were enrolled in this study. Subjects were divided in two groups according to positional effects on their AHI. In six patients, a lateral posture decreased the AHI by 50% and more (responders); in the remaining 25, lateral positioning decreased the AHI by less than 50% or even increased the AHI (nonresponders). AHI and body mass index (BMI) of the responders tended to be lower and their mean age was younger than those of nonresponders, but these differences were not statistically significant. We compared the upper airway morphology between the responders and the nonresponders regarding the tonsil size, tongue position (modified Mallanpati grade, reflecting the space between the tongue and soft palate) and the width of the fauces and retroglossal space. In addition, we compared nasal resistance between the groups using active rhinomanometry., Results: The width of the fauces was significantly greater (P=0.041) among the responders than among the nonresponders. However, the other parameters were not consistently different between the two, and these differences were not statistically significant either., Conclusions: The distance between the fauces was the sole morphological feature to distinguish the responders and the nonresponders to the positional therapy in patients with OSAS. Lateral positioning during sleep might be a recommended sleep hygiene for OSAS patients with wide fauces.

A 51-year-old man had progressive hearing loss over more than 15 years. He had bilateral sensorineural hearing loss (SNHL). Computed tomography (CT) showed extensive bilateral demineralization of the cochlear capsule, which is characteristic of diffuse cochlear otosclerosis. Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) of magnetic resonance imaging before enhancement revealed high signals in the cochlea and vestibule. Postcontrast 3D-FLAIR revealed enhancement of the basal turn of the left cochlea. This is the first published case of the breakdown of the blood-labyrinth barrier in a patient with cochlear otosclerosis. Our findings suggest that the breakdown of the blood-labyrinth barrier is associated with a part of SNHL in cochlear otosclerosis.

Objective: This study aimed to investigate how the symptoms of ear fullness, tinnitus and otoacoustic emissions (OAE) change in relation to the recovery course of pure tone audiometry thresholds (PTA) in sudden deafness (SD)., Methods: This study analyzed follow-up data on ear fullness, tinnitus and otoacoustic emissions of eight SD patients with good hearing improvement (Group A) and eight SD patients with poor hearing improvement (Group B) in an attempt to elucidate the behavior of these symptoms in their recovery course. This study was done until there was no change in the PTA for more than 1 week and hearing recovery was no longer expected., Results: All patients from both groups had ear fullness and tinnitus in association with the onset of SD. However, these symptoms improved only in Group A. showing a significant relationship between PTA recovery and the improvement of ear fullness annoyance (P<0.05), presence of tinnitus (P<0.01), improvement in tinnitus loudness (P<0.01) and in tinnitus annoyance (P<0.01). No patients (Group A or B) had OAE responses at their first examination. In Group A, OAE responses appeared simultaneously with improvement of hearing levels in five patients (63%) and it appeared later than hearing levels improvement in the other three patients (37%) from Group A. No patient from Group B showed OAE response on follow-up., Conclusion: SD patients with good hearing improvement (Group A) tended to have OAE responses and the sensations of the ear fullness and tinnitus improved almost simultaneously with hearing level improvement. Their PTA improvement occurred primarily in the low to mid frequencies, with high frequencies showing less recovery. When hearing recovery was not full, OAEs did not reappear for these frequencies. Patients with poor hearing improvement tended to have absent OAEs and persistent ear fullness and tinnitus.

Objective: Ectopic gastric mucosa of the cervical esophagus, referred to as inlet patch (IP), is considered to be a common development abnormality of the esophagus. We report here a case of hypopharyngeal squamous cell carcinoma bordering on IP of the cervical esophagus., Methods: A 44-year-old female underwent partial pharyngectomy, total laryngectomy, cervical esophagectomy, and bilateral neck dissection under the diagnosis of hypopharyngeal carcinoma., Results: Resected specimens of the hypopharynx revealed a well-differentiated squamous cell carcinoma in the ulcerative tumorous lesion (3.5 cm x 0.5 cm). A brown patch (2 cm x 1.5 cm) bordering the anal aspect of the tumor comprised ectopic gastric mucosa of fundic type epithelium. Immunohistochemistry revealed the surface mucosal cells of this lesion were strongly positive for human gastric mucin (HGM) and the fundic cells were positive for gastrin., Conclusion: The hypopharyngeal squamous cell carcinoma likely developed in association with chronic irritation due to gastric acid from the IP. To our knowledge, this is the first reported case of squamous cell carcinoma related to IP.

Objective: Otoacoustic emissions (OAEs) are useful clinical tools that can be used as a test to reflect hearing, especially cochlear function. When OAEs are recorded, the sound energy is conducted inwards and outwards through the middle ear system, but the degree to which the middle ear condition affects the OAEs level remains obscure. The objectives of the present study were to estimate the effect of the condition of the middle ear expressed by multifrequency tympanometry on the distortion product otoacoustic emissions (DPOAEs) adjusting for age and the corresponding pure-tone thresholds., Subjects and Methods: The evaluation was conducted using 1,043 subjects without a history of ear disease or occupational noise exposure out of a population-based sample of 2,259 adults aged 40-82 years. Multifrequency tympanometry, DPOAEs, and pure-tone audiometry were administered. The resonance frequency (RF), static admittance (SA) and tympanometric peak pressure (PP) were taken as variables representing middle ear function. Subjects were categorized into three groups of variables < or = 5th percentile, between 5th and 95th percentile, and > or = 95th percentile. The signal-to-noise ratio in DPOAE levels were compared among three groups adjusting for age and the pure-tone threshold level., Results: Both abnormally high and low RF exerted a negative effect on DPOAEs. Smaller RF values were related to smaller DPOAEs for the frequency around 1000 Hz, and greater RF values were related to smaller DPOAEs for the frequency around 4000 Hz. The tendency was similar between genders. Abnormally high SA had a negative influence on DPOAEs, while abnormally low SA had no significant effect on either gender. Smaller PP values were significantly related to smaller DPOAEs., Conclusions: We interpreted the findings of the present study as verification of the effect of the condition of the middle ear on DPOAEs after adjustment for age and the pure-tone threshold level, although ears out of normal range in tympanometric variables might conceivably include not only middle ear dysfunction but also inner ear abnormality. Tympanometric assessment must always be taken into account when OAEs are analyzed for an estimation of the cochlea function.

Nasopharyngeal actinomycosis is extremely rare, and to our knowledge, only seven cases have previously been reported. Diagnosis of actinomycosis is made by clinical finding, observation of the bacteria and histopathological examination. Treatment for actinomycosis is surgical debridement and administration of antibioticus, especially penicillin for several weeks with good prognosis. We report a case of nasopharyngeal actinomycosis, which lead to otitis media with effusion. Endoscopic surgery and prolonged penicillin administration for 2 months were effective for treatment of actinomycosis in the nasopharynx.

Objective: To understand the defense status of glandular tissue in the human adult larynx, we examined the glandular distribution, concentration and the ratio of the mucous glands in the subglottic and supraglottic larynx., Methods: One-hundred and seven adult larynges were examined. The area of the mucosa and glands were measured using an image analyzing system. The correlations of the concentration as well as the ratio of the mucous glands with age were analyzed., Results: The ratio of the areas occupied by the glandular acini of the subglottis was almost equal to that of infants as we reported earlier. The concentration of the laryngeal glands decreased in higher-aged adults at the level of the supraglottis, but not at the level of the subglottis., Conclusions: In comparison to infants, the ratio of laryngeal glands of the human subglottic larynx is rather constant in human adults. However, the supraglottic larynx seems to be influenced by increasing age. The changes deserved in the laryngeal glands predominantly occur in the supraglottic larynx.

Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm that occurs in the soft tissues, especially in the upper airways. We report two cases of laryngeal EMP, both of which were treated by surgical resection, one with and one without combination radiotherapy. A 39-year-old male was diagnosed with a laryngeal tumor localized in the left arytenoid region in October 1999. Biopsy showed the tumor to be a plasmacytoma. He underwent a direct laryngoscopy and resection of the tumor with a KTP laser. Consequently, he received radiotherapy to a total dose of 60 Gy. He has remained clear, with no local recurrence during 6 years of follow-up. A 59-year-old male was diagnosed with an epiglottic tumor in December 1989. He underwent a direct laryngoscopy and removal of the tumor. Histopathological study of the tumor showed a diffuse proliferation of plasmacytoid cells. He has remained diseasefree during 15 years of follow-up.

Objective: Adenoid cystic carcinoma (ACC) of the head and neck grows slowly with structural differentiation, however, this tumor nevertheless also shows a highly invasive potential and a high recurrence rate. Extracellular matrices have been suggested to play an important role in the differentiation and growth of ACC. The aim of this study is to understand the roles of laminin 5 basement membrane (BM) components, including collagen IV and laminin gamma2 for the high degree of invasiveness and the recurrence of ACC., Methods: Collagen IV and laminin gamma2 were immunohistochemically localized in tissue sections from nine patients with ACCs., Results: Cribriform structures with pseudocysts were preserved in small invading tumor nests, and the expression of both of collagen IV and laminin gamma2 was observed in the inner border of the pseudocysts and the surrounding area of the tumor nests. In areas of perineural invasion, the BM components continued to be expressed around the long tumor nests. Recurrent tumors consisted of multiple small nests with a few tumor cell layers, and the expression of the BM components was observed on both the inside of the inner tumor cells and the outside of the outer tumor cells, which was an obviously different appearance from that of the primary tumor., Conclusion: ACC appears to possibly grow and invade using the laminin 5 BM matrices while also preserving their differentiated architecture. The laminin 5 BM matrices might play an important role not only in the differentiation and growth, but also in the invasion and recurrence of ACC of the head and neck.

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.

In Japan, mumps vaccination is optional and the mumps were increased accompanied with prevalent waves according to the Infections Agents Surveillance Report (IASR). The aim of this study was to clarify that there was relevance for increase of mumps epidemic and mumps deafness. The Acute Profound Deafness Research Committee of the Japanese Ministry of Health and Welfare (reorganized to the Ministry of Health, Labour and Welfare in 2001) conducted a nationwide epidemiological survey to determine the number of patients treated for mumps deafness in 1987, 1993 and 2001. Based on its findings, the annual numbers of mumps deafness cases was estimated to be 300 in 1987, 400 in 1993 and 650 in 2001, which correlated with the overall incidence of mumps in those years. Because the majority of cases exhibited severe or profound sensorineural hearing loss that usually did not recover, rapid improvement of mumps vaccine coverage is strongly recommended.

The aim of this study is to analyze dysphagic symptoms of a patient with Huntington's disease (HD) who had having difficulty in swallowing. The patient was a 66-year-old female with HD. Inspection of self-feeding at bedside and videofluorographic swallowing assessment were performed. The features at self-feeding were the tendency of rapid eating, inability for smooth transportation of food to oral cavity, weak lip closure, which resulted in falling of food and eating it again. The videofluorography indicated clumsy tongue movement and postural instability by chorea which caused discoordination between oral and pharyngeal stage. Those ended in spill of liquid to the pharynx and retention of bolus in the oral cavity and vallecula, and aspiration did not occur. Pudding was carefully chewed because of the patient's alertness to the examination. The cognitive disturbance and choreic movement caused dysphagia at the preparatory and oral stages, and chorea also produced the discoordication between the oral and pharyngeal stage. The change of the shape of cups and stable posture were advised to lessen the chance of her aspiration.