Primary ciliary dyskinesia (PCD) is a hereditary disorder with structural and/or functional impairment of the cilia of the whole body. PCD is characterized by chronic sinusitis, bronchiectasis, male infertility and ectopic pregnancy due to impaired ciliary motility. The prevalence of PCD is estimated at 1 in 20,000 live births. Cases with situs inversus are termed “Kartagener’s syndrome” and usually these diagnoses are not difficult to make. However, in cases without situs inversus, the diagnosis can be very difficult. As such, it is most likely that PCD without situs inversus is underdiagnosed at the present time. The diagnosis of PCD is made when the patients have characteristic clinical features and (1) structural abnormalities of the cilia observed with electron microscopy and/or (2) presence of mutations in one of the genes associated with PCD. Electron microscopic diagnosis is not easy because ultrastructural ciliary defects can also be found in a few cilia among the healthy populations. Measuring levels of nasal nitric oxide (NO) concentration is a very useful tool in screening for PCD, because low NO values are a feature of PCD. Hand-held devices using tidal breathing are as useful as stationary devices. The sensitivity and specificity of nasal NO levels of <250 ppb for diagnosing PCD were 97% and 90%, respectively. Some other features can help to diagnose PCD. Frontal sinuses and sphenoid sinuses are hypoplastic. Having a combination of rhinosinusitis, middle ear and lung diseases increases the probability of PCD. The effectiveness of endonasal sinus surgery to treat rhinosinusitis associated with PCD is controversial.
Hypertrophic pachymeningitis (HP) is a rare disease and patients with HP present with headache and multiple cranial nerve palsy. We report herein on a case of HP with hearing loss and facial palsy. A 61-year-old woman presented with headache, right hearing loss, right facial palsy and numbness of the right chin. She was first diagnosed as having severe acute otitis media. Most of the symptoms persisted after treatment with antibiotics and corticosteroids. Ga-DTPA-enhanced magnetic resonance imaging (MRI) revealed diffuse thickening of the dura mater consistent with HP. HP is caused by several diseases such as infection, collagen disease, vasculitis syndrome, IgG4 related disease, and metastatic cancer. In this case, HP was caused by metastatic rectal cancer to the cranial bone and dura mater. The facial palsy and chin numbness improved after the chemoradiotherapy, whereas hearing loss did not. HP should be taken into consideration when multiple cranial nerve palsy are observed. Ga-DTPA-enhanced MRI is useful in the diagnosis of HP.
Sarcoidosis is an inflammatory multisystem disorder of unknown cause and is a form of sarcoidosis that affects the nervous system, seen in approximately 5-7% of patients with systemic sarcoidosis. This report describes a case of neurosarcoidosis in a 69-year-old woman who suffered from vertigo and bilateral sensorineural hearing loss. Routine chest X-ray showed a pulmonary hilar lesion in the left lung. A chest CT for close inspection revealed left hilar and mediastinal lymphadenopathy, leading to broncho-alveolar-lavage (BAL) with a biopsy. Although the biopsy was negative, the BAL studies were strongly positive for lymphocytosis and a high CD4/CD8 ratio. In addition, the ACE level was high in the serum test. The patient was therefore given a possible diagnosis of neurosarcoidosis. High dose intravenous methylprednisolone pulse therapy was administrated for three courses and then tapered to the maintenance dosage. Vertigo and bilateral hearing loss were dramatically improved after the pulse therapy, but left high frequency hearing loss did not change. In this case, an inner ear disorder was suspected for the source of these symptoms with neuro-otological examinations and brain MRI finding which showed no abnormal lesion. Neurosarcoidosis must be considered when evaluating patients with atypical hearing loss and vestibulopathy.
We report herein on a 19-year-old female with Langerhans cell histiocytosis (LCH) of the temporal bone. She complained of right ear fullness and showed conductive hearing loss of the right ear. CT imaging showed a tumor in the temporal bone and multiple nodules in the lung, and LCH was diagnosed by biopsy of the temporal bone tumor. After a series of remission-induction therapy of B1, but not A and maintenance therapy of B2 and C on the basis of the JLSG-02 protocol of the Japan LCH Study Group, LCH lesions in both organs disappeared and the defect of the temporal bone was replaced with new bone formation.
We evaluated the postoperative hearing results of 33 patients who underwent a type IV tympanoplasty between January 2001 and March 2010 at Tokyo Medical University Hospital.
A total of 33 ears in 18 male and 15 female patients were studied with ages ranging from 8 to 77 years. The patients were followed up for at least 6 months after surgery. The surgical techniques were Type IV-c in 32 ears, and IV-i in 1 ear.
In the case of a non-inflammatory disease, such as malformation of the middle ear, traumatic lesion of the ossicular chain and congenital cholesteatoma, successful hearing results were attained in 92.9% of the patients. In the case of an inflammatory disease, such as acquired cholesteatoma and otitis media, successful hearing results were attained in 36.8%.
The success rate was 87.5%, when an autograft cartilage was used for an ossiculoplasty and 43.8%, when bone was used.
We evaluated the risk factors for refractory otitis media in children and factors related to the clinical efficacy of antimicrobial treatment using tebipenem pivoxil (TBPM-PI). Multivariate logistic regression analysis revealed that “group living” (adjusted odds ratio; 6.2, 95%CI; 1.6–24.7, P=0.010), “age under 2 years old” (adjusted odds ratio; 4.2, 95%CI; 1.5–11.3, P=0.005), and “concomitant rhinosinusitis” (adjusted odds ratio; 2.9, 95%CI; 1.1–8.1, P=0.039) were significant risk factors for refractory otitis media. In 96 cases of acute otitis media (AOM) with the risk factors of refractoriness, TBPM-PI showed clinical efficacies with 100% for patients with one of the three risk factors and with 85% for patients with two or more of the risk factors. Multivariate logistic regression analysis revealed that “concomitant rhinosinusitis” (adjusted odds ratio; 4.4, 95%CI; 1.5–13.1, P=0.008) was a significant factor related with the poor clinical efficacy of TBPM-PI treatment for AOM.
Based on the results clinicians providing medical care for pediatric AOM should keep the risk factors of refractoriness in mind when selecting an appropriate treatment strategy.
Meningiomas account for 10–15% of all intracranial tumors, but less than 1–2% of them extend extracranially. They are benign tumors that originate in the meningothelial cells of the arachnoid granulations overlying the convexity of the brain and the skull base. We herein report on a case of meningioma that extended into the nasal cavity from the cavernous sinus, treated with surgical excision after Gamma-Knife irradiation. With the use of the Gamma-Knife procedure, the tumor was resected with less bleeding. In addition, Gamma-Knife irradiation increases the granulation that blocks the leakage of spinal fluid if there are bone defects.
A 24-year-old male with severe sore throat, poor oral intake and pharyngolaryngeal edema was referred to our hospital. After making a diagnosis of infectious mononucleosis based on the laboratory data and a physical examination, we started to treat the patient without antibiotics. Following intravenous drip infusion and inhalation therapy, the symptoms ameliorated. However, the pain in the left side of the pharynx again deteriorated, and swelling of the left soft palate developed. We diagnosed the patient as having a secondary peritonsillar abscess. The pus was drained with an incision of the left lateral wall of the pharynx and the symptoms improved. There are various kinds of reports related to complications of infectious mononucleosis, however a case of peritonsillar abscess is rare. Although antibiotic medication for infectious mononucleosis is not generally recommended, we must examine the prescription of antibiotics carefully depending on each case, considering the possibility of aggravation of bacterial infection as secondary abscess formation following infectious mononucleosis.
We experienced four cases of laryngeal paralysis associated with due to reactivation of the varicella-zoster virus (VZV). In all cases, the titers of the EIA antibody (IgG) were markedly increased. All patients were treated with an anti-viral agent and steroid. After treatment, there were no residual signs of the disease in all cases. In cases of unilateral cranial nerve palsy in which vesicles are either present or absent, herpes zoster should be considered and early treatment with anti-viral agent and steroid should be performed.
During the period from January 2003 to September 2013, 45 patients (44 males and 1 female, mean age 65 years) were diagnosed as having laryngeal carcinoma at Chubu Rosai hospital. We reviewed the clinical charts of these patients and investigated the treatment outcome and prognostic factor of laryngeal carcinoma. The clinical stage at initial presentation was stage I in 23 patients, stage II in 10 patients, stage III in 6 patients, and stage IV in 6 patients. As initial treatments, operation, radiotherapy (RT), chemoradiotherapy and chemotherapy were done for 7, 28, 9 and 1 patients, respectively. Five-year overall survival rates (OS) and disease-specific survival rates (DSS) for all laryngeal carcinoma patients were 65.4% and 79.0%, respectively. OS and DSS for 34 patients with glottic carcinoma were 71.4% and 84.7%, respectively. OS and DSS for 11 patients with supraglottic carcinoma were 40.0% and 53.3%, respectively. Both the OS and DSS for the glottic carcinoma were significantly higher than those for the supraglottic carcinoma (P=0.011, 0.017). The OS rates for all laryngeal carcinoma patients with stage I, II, III and IV were 92.3%, 40.0%, 33.3% and 40.0%, respectively. The ratio of laryngeal preservation was 71%. Twelve patients died due to laryngeal carcinoma in 6 patients, second primary carcinomas in 3 patients, and other causes in 3 patients. The OS for the patients with stages II and III were poor. Three patients with stage II died of other causes. Two patients with stage III, one of whom rejected surgery, and the other of whom had inoperable general condition, died of laryngeal carcinoma. Therefore the treatment for laryngeal carcinoma was possibly proper. All five patients with normal cord mobility with T2 glottic carcinoma who underwent RT are currently alive with no recurrence. RT may therefore be effective for T2 glottic carcinoma with normal cord mobility.
A 45-year-old woman was referred to our department with sore throat, swelling of the lower left chin and high fever even after four days of oral antibiotics treatment in a private clinic. She had redness and swelling along her left sternocleidomastoid muscle from the lower left ear to the sternum. Her laboratory test showed an elevated white blood cell count and elevated C-reactive protein (CRP), but the results of the test did not satisfy the criteria of disseminated intravascular coagulation. Contrast enhanced computed tomography showed a left deep neck abscess and thrombosis of the left internal jugular vein (IJV) from the level of the transvers process of the second cervical spine to that of venous angle. She did not have mediastinum abscess, pulmonary emboli, and liver and spleen abscess, however, that are often observed in Lemierre’s syndrome. She did not complain of arthralgia in her large joints. Under the diagnosis of pharyngitis with deep neck abscess and thrombophlebitis of the IJV, she was given intravenous ampicillin/sulbactam (ABPC/SBT). On the third day of her admission, she underwent drainage of the left deep neck abscess because of gas production. Peptostreptococcus was detected from a blood culture which was sensitive to ABPC/SBT. Intravenous ABPC/SBT treatment was continued for 35 days until we confirmed the total control of the infection and the IJV thrombosis had stabilized.
The patient experienced less severe symptoms than typical Lemierre’s syndrome cases probably due to early treatment with an appropriate intravenous antibiotic, an atypical pathogen, and onset at an atypically older age.
Charcot presented two clinical lectures at La Salpêtrière in January, 1874 and in November, 1875. These two lectures are recorded as Lectures No.17 and No.18 in his “Oevrtes complètes de J.-M. Charcot, 1894”. Lecture No.17 presented a female patient suffering from Menière’s vertigo, as Charcot called it. The patient showed a left sanguine-purulent aural discharge and had once been under Menière’s care. At the age of 45, she was admitted to La Salpêtrière for six years due to her constant stage of vertigo accompanied with frequent recurrent attacks, and she was shown as a typical grave case of Menière’s vertigo. All conventional treatments were of little avail. She was again presented next year, this time as a successfully cured case. Charcot had long been aware that final total deafness is almost always accompanied with the ameliorations of both Menière’s vertigo and annoying tinnitus. In Lecture No.18, Charcot suggested that this final stage could be artificially produced in order to relieve the patient from agonizing attacks of vertigo by any manner of means. Quinine sulfate was known to paralyze the auditory nerve (as Charcot so said). Charcot therefore made the serious step of using quinine sulfate. His prescription for the female patient mentioned above was one gram divided in two a day for three months, and the patient was freed from her longstanding misery. Charcot showed similar success in another such case.