The cardinal symptoms of Meniere’s disease are episodic vertigo, fluctuating hearing loss and tinnitus. Meniere’s disease runs a chronic course and is often associated with endolymphatic hydrops (EH) in the inner ear. The etiology of the disease is unknown. Using fluid-attenuated inversion recovery (FLAIR) or more advanced techniques with contrast-enhanced magnetic resonance imaging (MRI), we evaluated EH under several conditions in patients with symptoms attributable to Meniere’s disease or inner ear symptoms. To deliver the contrast agent, we used the intratympanic (IT) and/or the intravenous (IV) approach. Potentially higher concentrations of the contrast agent can be introduced into the perilymph via the IT approach than via the IV approach. However, use of the IT approach needs approval of the ethics committee, while use of the IV approach is more acceptable in the clinical setting. In American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria, EH was observed more frequently in patients with Definite Meniere’s disease than in those with Possible Meniere’s disease. Meanwhile, EH was also observed in both the cochlea and the vestibule in patients with atypical Meniere’s disease. Of 56 ears (36 patients) of patients with cochlear Meniere’s disease, 38 showed EH in the cochlea and 44 showed EH in the vestibule. Of 56 ears (28 patients) of patients with vestibular Meniere’s disease, 29 showed EH in the cochlea and 47 showed EH in the vestibule. The vestibular Meniere’s disease group showed significant vestibular predominance in the distribution of EH. In 30 patients with tinnitus as the major complaint, EH in the cochlea was present in 14 of 25 symptomatic ears (56%). Significant EH was present in 7 of 14 ears and mild EH in the remaining 7 ears. EH was observed more frequently in patients with fluctuating tinnitus than in patients with stable tinnitus. Inner-ear contrast-enhanced MRI may be useful to identify EH in its early stages.
We report two cases of intracranial hypotension. In one case, MRI showed typical findings, including diffuse thickening, enhancement of the dura mater, and pseudocyst at the level of C6 and 7 resulting from the leakage of CSF. In the other case, treatment with an epidural blood patch (EBP) improved his symptom, almost completely, although no typical findings were pointed out in MRI. The patient presented with bilateral mild hearing loss and abnormal eye movements before the application of the EBP, while the symptoms disappeared after the EBP application. Not all cases show typical findings, therefore, obtaining a proper history, including of any head injury, is important for the diagnosis of intracranial hypotension.
We conducted a retrospective epidemiological survey to clarify the current status of otitis media with effusion (hereafter, OME) at the time of primary cleft palate surgery at our otorhinolaryngology department. We selected a total of 227 patients (118 boys [52%], 109 girls [48%], mean age: 1.89±0.4 years old) who had undergone primary cleft palate surgery at the oral surgery department of our hospital during the 5-year period from December 2006 to November 2011. At our hospital, two-stage surgery is adopted for palatoplasty, and the primary surgery is usually performed when a patient is around one and half years old. Basically, all the patients undergo medical examination at the otorhinolaryngology department before palatoplasty, and any patients detected to have OME are subjected to myringotomy or ventilating tube insertion at our department. The prevalence of OME during the primary cleft palate surgery was about 60%. No relation was observed between the presence or absence of OME and the presence or absence of any factors, such as the type of cleft palate, gender, height, weight, or associated malformation. The morbidity of OME was significantly higher in the patients who had consulted a previous otorhinolaryngologist before visiting our department. Thus, the effectiveness of preoperative diagnosis of myringotomy was demonstrated. There were many cases of myringotomy, while cases of ventilating tube insertion accounted for only 16%, less than that reported from many other facilities. Since our department has conventionally limited medical treatment of OME to myringotomy, we actually had a low rate of it this procedure. The pros and cons of tube insertion are controversial, therefore, further consideration would be necessary to identify the factors influencing the occurrence of OME and to decide or validate the treatment course.
We report a case of multiple cranial neuropathy caused by nasopharyngeal necrosis occurring long after radiotherapy for nasopharyngeal carcinoma. The patient was a 63-year-old male with the chief complaint of headache. He had received chemoradiotherapy for nasopharyngeal carcinoma 27 years earlier and for paranasal sinus cancer 19 years earlier. Endoscopy revealed nasopharyngeal necrosis, and a contrast-enhanced CT of the neck revealed a low-density area across a wide region from the skull base to the nasopharynx. The patient was also found to have paralysis of the left glossopharyngeal nerve, right vagus nerve, left accessory nerve, and the hypoglossal nerves bilaterally. Although systemic administration of antimicrobial agents and steroids halted the progression of the necrosis, the cranial neuropathy remained. Treatment with appropriate radiation doses is necessary to mitigate the adverse events of radiotherapy, and adequate consideration must be given to radiation exposure, particularly in cases of relapse and cases such as this one, in which adjacent areas are also involved.
Lambert-Eaton myasthenic syndrome (LEMS) is an immunological disease known to be associated with malignant disease, and is characterized by muscle weakness and autonomic neuropathy. Small cell lung cancer is one of the most important causes of this condition. We encountered a case of LEMS associated with small cell lung cancer presenting with dry mouth. A 65-year-old man visited to our hospital with the complaints of dry mouth, muscle weakness of the lower limbs and gait disturbance. The patient showed significant hyposalivation, however, the diagnosis of Sjogren’s syndrome could not be established based on the results of minor salivary gland biopsy and blood tests. Chest CT showed a nodular lesion in the left lung and swollen lymph nodes in the mediastinum. In regard to the neurological findings, the patient showed hyporeflexia, and lower limb weakness. Therefore, we suspected the possibility of LEMS associated with lung cancer. Video-assisted thoracic surgery was performed, and small cell lung cancer was diagnosed based on histopathological examination of the resected specimen. Five courses of chemotherapy (cisplatin+etoposide) and concurrent radiotherapy at a total dose of 45 Gy were administered, which resulted in a complete response. Autonomic neuropathy such as that associated with LEMS should be borne in mind in the differential diagnosis of dry mouth.
Amyloidosis is a metabolic disorder where insoluble fibrous protein aggregates, so-called amyloid proteins, are deposited in the extracellular space in a variety of organs and tissues, leading to organ- or tissue-specific dysfunctions. Herein, we report a rare case of laryngeal amyloidosis. A 54-year-old woman was referred to our department with a 9-month history of hoarseness. Laryngeal endoscopic examination revealed orange-colored uneven edematous swelling of the left false cord, with intact vocal cord mobility. An endoscopic biopsy was performed under local anesthesia. Histopathologically, the specimen showed submucosal deposition of amyloid, with positive Congo red staining. Immunohistochemically, the lesion was positive for immunoglobulin λ light chain, but negative for amyloid A. There were no other abnormal findings on systemic examination. The patient was diagnosed as having localized AL-type laryngeal amyloidosis, and eventually, debulking surgery was performed using a CO2 laser via a direct laryngoscope. The postoperative clinical course was uneventful. The lesion macroscopically disappeared, and the voice quality improved. The clinical characteristics, pathogenesis, diagnosis and treatment of amyloidosis were bibliographically reviewed.
Between 2005 and 2010, we treated 30 cases of Reinke’s edema by surgery. The subjects were 9 men and 22 women aged 33 to 83 years (mean: 57.5 years). Besides the 5 patients whose smoking history was not clear, 22 patients (88%) were smokers and 3 patients (12%) were non-smokers. Among the 20 patients who answered a questionnaire, the voice condition finally improved in 13 patients (65%), while it improved by 1 week after the surgery in 5 patients (25%). In cases of Reinke’s edema, we must be careful in making the right choice of operation, and in addition to perfoming careful operation, must also try to motivate the patients for smoking cessation.
Large-cell neuroendocrine carcinomas (LCNECs) represent one subgroup of large-cell lung cancers. We report a rare case of LCNEC with metastasis to the thyroid gland. A 64-year-old woman with primary lung cancer was treated by surgery and chemotherapy. Histopathology of the resected specimen revealed the diagnosis of LCNEC. Metastases to the brain and endotracheal space occurring several months later were treated by radiotherapy and/or chemotherapy. Two years after the lung surgery, the patient presented with a thyroid tumor suspected to be a metastasis arising from the LCNEC, and a total thyroidectomy was performed. Histopathology of the thyroid tumor revealed features consistent with LCNEC. Until now, about one and half years after the surgery, there has been no evidence of tumor recurrence in the cervical region. The patient is under close follow up.
Liposarcoma, the second most commom soft-tissue sarcoma in adults, arises predominantly in the extremities or retroperitoneum, less frequently in the head and neck region, and rarely in the neck. We report a case of myxoid-type liposarcoma of the neck. A 50-year-old man with a few years’ history of an enlarging painless swelling in the frontal-medial aspect of the neck, measuring 48×40mm in size at the present examination, was found on computed tomography (CT) to have a mass extending between the thyrohyoid muscle and the platysma. We extirpated the tumor with the overlying skin. Histological examination confirmed the diagnosis of myxoid-type liposarcoma. The tumor was completely covered with velamen, and the surgical margin was negative for tumor. No adjuvant therapy has been given, and the patient remains recurrence-free 27 months after surgery.
Synovial sarcomas account for 5% of all pediatric soft tissue sarcomas, and primarily arise in the extremities. We report a case of synovial sarcoma arising in the right infra-auricular region. A 29-year-old woman presented with a 2-months’ history of a growing and slight painful tumor in the infra-auricular region. Pleomorphic adenoma arising from the lower pole of the parotid gland was suspected based on the findings of MRI and fine needle aspiration cytology of the tumor, and the tumor was resected. However, additional dissection was necessitated by the final diagnosis of synovial sarcoma by histopathological examination of the resected tumor. The SYT fusion gene from chromosomal translocation was detected by fluorescence in situ hybridization. Eight months later, a recurrent tumor was detected in the almost same region, and further dissection was performed with preservation of the mandibular branch of the facial nerve. The patient was administered postoperative radiotherapy at the dose of 60Gy, even though it was possible to resect the tumor with an adequate safety margin.
We performed laryngotracheal separation (LTS) surgery in 27 patients with severe motor and intellectual disabilities (SMID) over a period of 8 years. Of these, seven developed serious respiratory complications after the operation. Herein, we present four of the cases with severe respiratory complications. Case 1: A 9-year-old boy who developed severe breathing difficulty with laryngomalacia immediately after LTS: The condition was attributed to narrowing of the lumen of the trachea by the adjacent brachiocephalic artery. Brachiocephalic artery ligation was performed, which resulted in expansion of the lumen of the trachea. Case 2: A 9-year-old boy who developed severe breathing difficulty with airway obstruction and difficulty in expectoration three years after the LTS operation: He developed altered sensorium, which persists until date. Case 3: A 9-year-old boy who developed severe airway obstruction with difficulty in expectoration four months after the LTS operation: He developed altered sensorium and died of heart failure six months later. Case 4: A 5-year-old girl who developed pneumonia due to respiratory syncytial virus infection: she died sixteen 16 days later. We need to improve the operating method of LTS in cases with a distance of less than 20 mm between the back of the sternum and the anterior aspect of the vertebral body. The method is desirable as it does not involve lifting of the trachea and avoidance of narrowing of the tracheal lumen by the adjacent brachiocephalic artery. Thus, LTS surgery might result in serious respiratory complications, e.g., airway obstruction with difficulty in expectoration and severe pneumonia. Sufficient explanation about the risk of respiratory complications must be provided to the family and caregiver prior to undertaking LTS surgery. Furthermore, after LTS surgery, multiple approaches to respiratory care, including chest physiotherapy and breathing exercises are necessary.
We report 10 patients of styloid process syndrome who were admitted to our hospital. The patients, consisting of 6 men and 4 women, ranged in age from 28 to 80 years old, with an average age of 62.9 years. The chief complaints were pain in the throat (3 cases), neck pain (2 cases), lower jaw pain (1 case), giddiness (1 case), sense of incongruity in the throat (2 cases), and asymptomatic (1 case). The length of the styloid process was measured using computed tomography (CT). The length of the right styloid process was distributed in the range of 30 to 75 mm, with an average length of 49.5 mm, while that of the left styloid process was distributed in the range of 30 to 75 mm, with an average length of 46.5 mm. Surgery was performed in 4 patients, and was successful in all 4. The symptoms of styloid process syndrome are non-specific, which potentially delays accurate diagnosis. Therefore, styloid process syndrome is a condition that should always be borne in mind in routine clinical practice.
Objective: The aim of this study was to evaluate the toxicity of neoadjuvant chemotherapy with docetaxel, cisplatin and 5-fluorouracil (TPF) in patients with squamous cell carcinoma of head and neck. Materials and Methods: We treated 28 patients (25 men and 3 women) with squamous cell carcinoma of the oral cavity, larynx, oropharynx, nasopharynx, or hypopharynx. The median age was 56.6 years (range, 46-80 years). Patients received two cycles of neoadjuvant TPF therapy (docetaxel 50 mg/m2, cisplatin 60 mg/m2, 5-fluorouracil 600 mg/m2). The interval between the 1st and the 2nd cycles of neoadjuvant TPF was two to three weeks. The white blood cell count, red blood cell count, Hb, platelet count, AST, ALT, total protein, BUN, and serum Cr were compared between before and during the 1st and 2nd cycles of neoadjuvant TPF therapy. The incidences of anorexia, stomatitis and diarrhea were compared between the 1st cycle and the 2nd cycle of TPF therapy. Results: There were statistically significant decreases of the white blood cell count (P<0.0001), red blood cell count (P<0.0001), Hb (P<0.0001), platelet count (P<0.0001), serum total protein (P<0.0001), and BUN (P=0.0046) during the 1st cycle and 2nd cycle of therapy as compared with the levels before the start of the chemotherapy. Furthermore, even more significant decreases of the red blood cell count (P<0.01) and Hb (P<0.01) were observed during the 2nd cycle of therapy as compared with those during the 1st cycle of therapy. There were no significant differences in the incidences of anorexia (P=0.44), stomatitis (P=0.37), and diarrhea (P=0.07) between the 1st and 2nd cycle of therapy. Conclusion: The 2nd cycle neoadjuvant TPF can be safely administered like the 1st, if caution is observed against a decrease of the red blood cell count and Hb.
Eleven reports of autopsies related to Menière’s disease or Menière’s vertigo were collected. These patients had passed away with fatal diseases other than Menière’s disease, but showed Menière’s symptoms while alive. The reports on labyrinthine lesions were manifold as follows: labyrinthine hemorrhage, labyrinthitis, labyrinthitis with meningitis, sclerosis of the ossicular chain, sclerosis around the oval window, sclerosis of the round window, and impacted cerumen. These findings later gave rise to various theories, at least in part, for the pathogeneses of Menière’s disease. In addition, cases with acoustic tumor and other brain tumors were also included in the early reports.