Mucociliary transport so critical in nasal, paranasal sinus, and middle ear physiology is impaired in chronic sinsusitis and otitis media by factors such as increased mucus viscoelasticity, decreased ciliary area, and primary or secondary ciliary immotility. We reviewed the pathophysiology of primary ciliary dyskinesia, otitis media with effusion, chronic sinusitis, and allergic rhinitis in terms of mucociliary transport. Subjects with primary ciliary dyskinesia may experience recurrent middle ear infection, chronic airway infection, predominantly lower-lobe bronchiectasis, male sterility, or situs inversus. Primary ciliary dyskinesia is sometimes difficult to diagnose in cases without situs inversus. Nasal nitric oxide concentration in such patients decreases, although why is unclear. Mutations may involve dynein arm intermediate chain 1 (DNAI1) or dynein arm heavy chain 5 (DNAH5). Mucociliary clearance decreases more in those with otitis media with effusion than in those without, due in part to increased middle ear effusion viscosity. Prognosis is poor in subjects with viscous effusion, which is difficult to clear from the middle ear via the mucociliary system. An understanding of anatomic paranasal sinus variations is thus extremely important in chronic sinusitis when endoscopic sinus surgery is attempted, although recent advances in computed tomography (CT) have enabled paranasal sinus drainage pathways to be delineated more clearly than ever before.
Usher syndrome (USH) is an autosomal recessive disorder characterized by hearing loss and retinitis pigmentosa. USH can be classified into 3 clinical subtypes (USH type 1-3: USH1-3) on the basis of the severity and progression of hearing loss and the presence or absence of vestibular dysfunction. We conducted a mutation analysis of USH2A, one of the disease-causing genes of USH2, and identified c.8559-2A>G and p.Trp3150X in a heterozygous state in a USH patient. Though USH2 is characterized by non-progressive moderate-to-severe hearing loss and normal vestibular dysfunction, the patient showed atypical USH2 phenotype-rapidly progressive hearing loss. In atypical patients, environmental factors or modifier genes are presumed to influence the clinical findings. Because the patient had no history of noise exposure, ototoxic medication, or ultraviolet exposure, modifier genes were likely to have influenced the atypical phenotype with USH2A mutations. Considering MYO7A, CDH23, and USH3A as modifier genes, we conducted a mutation analysis of these genes. We identified 16, 44, and 2 sequence alterations in MYO7A, CDH23, and USH3A, respectively, none of which was presumed to be a mutation. Though we could not identify the causes of the atypical phenotype, we considered it very important in the expansion of the genetic analysis of USH that the causes of atypical USH patients should be identified.
We studied 677 cases with idiopathic sudden sensorineural hearing loss (ISSNHL) treated with at least intravenous batroxobin, oral betamethasone, and hyperbaric oxygen therapy. The 677 patients received at least intravenous batroxobin from 2005 to 2008. Overall “cure” was 30.7%, 44.2% for Grade 1, 34.4% for Grade 2, 32.2% for Grade 3, and 8.1% for Grade 4. Within the twelve days between onset and treatment start, “cure” was 38.2%. Hearing loss severity and the duration both significantly influenced hearing recovery.
Epistaxis studied in 318 subjects from 2005 to 2007 was observed more frequently in men (61.3%) than in women (38.7%), and decreased in summer. The bleeding point was observed most often in areas other than Kiesselbach's (57.5%). We stopped bleeding in 235 subjects (73.9%) using electrocoagulation. It is important to identify the bleeding point using fiberscopy and to implement electrocoagulation as soon as possible. The chief complication was hypertension. We thus suggest the importance of controlling hypertension in preventing epistaxis.
A 33-year-old man with no history of trauma or surgery was seen for a posterior ethmoidal sinus cyst with intracranial development, causing headaches. He also had anterior cranial fossa bone defects. Computed tomography and magnetic resonance imaging confirmed a cyst in the left posterior ethmoidal sinus, necessitating endoscopic sinus surgery under general anesthesia, to open the cystic lesion and drain mucus secretion. The postoperative course in the 14 months since surgery has been uneventful.
Fungal sinusitis is fairly common and often incidentally diagnosed in X-ray or computed tomography imaging (CT). Most cases are largely asymptomatic and noninvasive. In invasive cases, subjects are frequently immunocompromised hosts suffering from diabetes mellitus, using steroids or immunosuppressive drugs, or aged. The invasive fungus destroys tissues, muscles, and bones, occasionally even causing cranial and orbital complications. Such fatal fungal sinusitis requires intensive, appropriative treatment if subjects are to survive. Antifungal drugs have recently been reported to be effective against invasive fungal sinusitis. We present two cases of invasive aspergillus sinusitis showing orbital apex syndrome, both of which were improved using antifungal drugs.
Malignant fibrous histiocytosis (MFH), the most common soft-tissue sarcoma, appears commonly in adult proximal limb muscles and retroperitoneum, but rarely in the nasal sinus. We report a case with a good outcome thanks to cyberknife therapy of maxillary sinus MFH relapsing frequently after surgical and radiation therapy. An 80-years-old man was seen for tumors filling the right nasal cavity and from the maxillary sinus to the nasal cavity found in computed tomography (CT). We partially excised the right maxilla. Pathological tissue inspection showed malignant fibrous histiocytosis. Despite postoperative 72 Gy radiation, the tumor relapsed twice more, after which we used a cyberknife in therapy, eliminating the tumor. No tumor has recurred in the 18 months since.
Verrucous carcinoma is categorized as part of well-differentiated squamous cell carcinoma, the most common carcinoma of the oral cavity and pharynx. This condition which must be diagnosed by both clinical findings and pathological results is generally treated by surgical resection. We report 5 cases of verrucous carcinoma of the oral cavity and pharynx. We treat T1 and T2 carcinoma using photodynamic therapy (PDT) or resection, and T3 and T4 carcinoma using resection and free flap reconstruction. In the 10 to 17 months since treatment, none of our 5 subjects has experienced recurrence or distant metastasis.
Pitfalls in the videoendoscopic evaluation of swallowing disorders were studied in 101 cases in which videoendoscopic evaluation was immediately followed by videofluoroscopic evaluation. Nine of the 101 (9%) could not be diagnosed using videoendoscopy alone. These consisted of 2 subjects with esophageal cancer (2%) and 1 each of esophageal achalasia (1%), ossification of the anterior longitudinal ligament (1%), osteophyte of the cervical spine (1%), thyroid tumor (1%), lateral pharyngeal diverticulum (1%), postoperative complication due to anterior cervical fusion (1%), and Plummer-Vinson syndrome (1%). Videoendoscopy alone could not diagnose all cases of swallowing disorders in the pharyngeal and esophageal phases. It is thus important to be aware of the potential limitations of these tools in outpatient clinic use.
Medullary thyroid carcinoma (MTC), a relatively rare neuroendocrine tumor originating in parafollicular C cells, releases calcitonin and carcinoembryonic antigen (CEA). A 52-year-old female woman with MTC had idiopathic abnormally elevated serum CEA for 2 years. Fluoro-deoxy-glucose positron emission tomography (FDG-PET) identified an intensive signal mass in the right thyroid lobe. Sporadic MTC was clinically diagnosed from typically high serum CEA and calcitonin. Following total thyroidectomy and D1 and deep cervical lymph node dissection, the woman remains free of distant metastasis and recurrence in the three years since surgery. MTC in our case grew very slowly. The ratio of calcitonin to CEA was 14.7. FDG-PET is useful for detecting thyroid tumors and for follow-up if recurrence is suspected. The calcitonin- to -CEA ratio is a valuable parameter in clinical prognosis of this malignant tumor as reported in previous studies.
Deep neck infection, which is serious and potentially life-threatening, has not decreased in frequency despite advances in antibiotics treatment. An 84-year-old woman with poorly controlled diabetes admitted April 25th 2008, for sore throat and a high fever was found in neck computed tomography (CT) to have a deep neck abscess. Despite good postoperative progress, her fever continued. Further careful examinations, in pelvic CT showed a retroperitoneal abscess, which was then treated and controlled with an intravenous antibiotics drip. We suspect that the deep neck infection had spread hematogeneously. Such deep infections in a compromised host must therefore be treated and controlled carefully.
We report a rare case of chopstick penetration of the parapharyngeal space. A 48-year-old man who fell down while holding chopsticks was injured in the left temporal region, after which he reported temporal and jaw pain and trismus. The first examination found a small laceration on the temporal skin but no visible foreign body. Computed tomography (CT) showed a slender low-density material in the infratemporal fossa extending to the parapharyngeal space. After general anesthesia was administered the laceration was opened and a fragment of broken chopstick was removed. His postoperative clinical course was uneventful and pain and trismus subsided completely. Given the potentially useful clinical and radiological features of wooden foreign body penetration of the head and neck, we review their ramifications bibliographically.
We report a case of Kawasaki disease with high fever and cervical lymphadenitis. A 4-year-old boy admitted for high fever and severe pain of neck had cervical lymphadenopathy, including a fever of over 38°C, and slightly swollen tonsils. Computed tomography (CT) later indicated a retropharyngeal cellulitis. Based on these findings, we treated the boy with antibiotics alone until skin changes typical of Kawasaki disease, reddening and rashes on the palms and soles, appeared. Kawasaki disease should therefore be considered a possibility in children with cervical lymphadenopathy, presenting symptoms such as high fever resistant to antibiotics.
A placebo-controlled randomized double-blind parallel-group comparison study of dexamethasone cipecilate (NS-126C) steroid nasal spray, conducted in those with perennial allergic rhinitis, clarified efficacy and safety of drug administration at 400 μg once a day for 12 weeks. Of the 165 subjects in the Full Analysis Set (FAS) (NS-126C: 133; placebo: 32), total nasal symptom scores for sneezing, rhinorrhea, and nasal congestion in the NS-126C group improved significantly over the placebo group throughout the study with efficacy sustained for 12 weeks (p<0.001 to 0.024). Individual Scores for sneezing, rhinorrhea, and nasal congestion also improved more significantly in the NS-126C group over the placebo group throughout the study. A total quality of life (QOL) study score based on the Japan Rhinoconjunctivitis Quality of Life Questionnaire (JRQLQ) was significantly higher in the NS-126C group than in the placebo group (p=0.001 to 0.017) throughout the study. There was no significant difference in adverse event or drug reaction incidences between groups. Neither adverse events with an increase in incidence and no delayed adverse events due to long-term administration was seen. Plasma cortisol was unchanged over time, indicating the absence of an effect on adrenal cortical function. No abnormality was detected on the nasal mucociliary function test. Nasal discomfort, pharyngeal discomfort, and positive reaction to the nasal fungal test were observed in one patient per group as local adverse drug reactions. Findings thus showed that NS-126C is a safe steroid nasal spray with long-acting efficacy improving QOL in patients with allergic rhinitis.