Carotid body tumors (CBTs) are hypervascular tumors with multiple feeding arteries and a unique orientation at the carotid bifurcation. CBT has been recognized as a malignant tumor since the 2017 WHO classification 4th edition (ICD-O code: 8692/3). Although resection surgery potentially offers radical cure for this tumor, complete resection is challenging. Multiple feeding arteries and adhesions of the tumor to the carotid arterial wall pose difficulties in CBT resection surgery. The effectiveness of preoperative embolization remains controversial due to the differing protocols among institutions. However, accurate embolization and resection immediately after embolization could reduce the volume of blood loss and operation time in CBT surgery. Most patients with CBT harbor germline mutations, such as SDHB and SDHD. Conversely, the concept of “hereditary paraganglioma-pheochromocytoma syndrome (HPPS)” has been proposed to describe patients with familial paraganglioma. Therefore, analyses of gene alterations are needed in patients with CBT.
Patients with tinnitus often complain suffering from insomnia and other sleep disorders; however, the science underlying the relationship between tinnitus and sleep is not yet clear. We conducted a questionnaire survey using the Pittsburgh Sleep Quality Index (PSQ-I), Tinnitus Handicap Inventory (THI), Hospital Anxiety and Depression Scale (HADS), and Dizziness Handicap Inventory (DHI) to study the relationship between sleep and tinnitus. A total of 34 patients with tinnitus received sleep hygiene guidance in addition to treatment; 22 patients had sleep disorders at the time of initiation of therapy for tinnitus. Patients with sleep disorders had higher prevalence rates of tinnitus and dizziness than patients without sleep disorders. The PSQ-I, THI and DHI scores improved significantly after the subjects had practiced sleep hygiene. Some of the patients showed pronounced improvement of the tinnitus, in addition to improved sleep. Although further investigation is warranted, this study indicates the possibility that treatment of sleep disorders may improve tinnitus.
Boi-Ohgi-To is considered to regulate water metabolism, and to potentially to contribute to improving the condition in patients with Meniere’s disease. We studied the efficacy of the herb medicine “Boi-Ohgi-To” prescribed for Meniere’s disease. Boi-Ohgi-To was given orally 3 times a day at the total dose of 7.5 g for 6 months. Data of a total of 14 patients were analyzed (5 men and 9 women; age 42–79 (median age 67) years). We excluded the patient who reported increased frequency of micturition as a side effect, and analyzed the clinical effect of the herbal medicine in the remaining 13 patients. The improvement rate of dizziness/vertigo was 69% (9/13) and that of the hearing acuity was 30% (3/10) and the rate of overall improvement was 69% (9/13).
We concluded that Boi-Ohgi-To is one of the useful therapeutic options for Meniere’s disease.
In the canal wall up technique (CWUT) for removal of cholesteatoma, keeping the consistency of the cholesteatoma matrix is very important. To keep the consistency of the matrix, we begin by pushing the bottom of the cholesteatoma from the mastoid antrum towards the external auditory canal. After detaching the matrix from the underlying mucosa or bone, we finally cut the epithelium at the point of its entrance from the external auditory canal into the middle ear. This method combined with scutumplasty and cartilage tympanoplasty can also prevent deformity of the external auditory canal and reduce the risk of recurrent cholesteatoma, by minimizing lateral tympanoplasty.
In this study, we classified a total 42 patients with pars flaccida cholesteatoma and 37 patients with pars tensa cholesteatoma who underwent primary surgery at Teikyo University School of Medicine, Mizonokuchi Hospital, between January 2015 and July 2019, according to the Classification and Staging of Cholesteatoma proposed by the Japanese Otological Society in 2015.
Among the 42 ears with pars flaccida cholesteatoma, 14 (33.3%) were classified as showed stage I, 20 (47.7%) as stage II, and 8 (19%) as stage III, with no case (0%) of stage IV. Among the 37 ears with pars tensa cholesteatoma, 4 (10.8%) were classified as stage I, 23 (62.2%) as stage II, and 10 (27%) as stage III, with no case (0%) of stage IV. The successful outcome rate was 100% in the stage I cases, 85% in the stage II cases, and 75% in the stage III cases of the pars flaccida cholesteatoma group, and 100% in the stage I cases, 69.6% in the stage II cases, and 50% in the stage III cases of the pars tensa cholesteatoma group. The residual and recurrent disease rate was 0% in the pars flaccida cholesteatoma group, and 2.7% in the pars tensa cholesteatoma group.
We consider that our method of pushing the bottom of cholesteatoma from the mastoid antrum towards the external auditory canal is quite efficient for keeping the consistency of the matrix and minimizing lateral tympanoplasty to reduce the risk of recurrent of cholesteatoma.
We report the case of a patient with otitis media and ANCA-associated vasculitis (OMAAV), who presented with rapidly progressive bilateral deafness. A 64-year-old woman visited an ENT clinic with the chief complaint of right ear pain. She was diagnosed as having right acute otitis media and left otitis media with effusion, and treated with antibiotics and myringotomies. Twenty-seven days later, she was transported to our hospital by ambulance with headache, fever, vertigo and hearing loss, and pure tone audiometry revealed bilateral deafness. The right eardrum was normal, whereas the left tympanic membrane was perforated, and discharge was observed from the left middle ear cavity. Blood tests showed elevated levels of inflammatory markers, positive serology for MPO-ANCA and a negative test for serum PR3-ANCA. Contrast-enhanced MRI showed findings consistent with hypertrophic pachymeningitis and no enhancement of the inner ear. The hypertrophic pachymeningitis improved with steroid pulse therapy, but the hearing did not improve. During tapering of the prednisolone dose, the patient underwent cochlear implantation and speech hearing was restored. Because of the rapid progression of her bilateral deafness, it was difficult to diagnose OMAAV prior to the occurrence of deafness. Although prolonged treatment is generally required for OMAAV, early cochlear implantation could improve the effect by shorting the hearing loss period.
In this report, we describe the case a 68-year-old man who developed buccal herpes zoster during the course of steroid treatment for sudden deafness, followed by the development of peripheral-like facial nerve palsy. The patient developed right hearing loss on day 1, and his right audiogram showed a hearing threshold of 88.8 dB on day 8. Since head magnetic resonance imaging (MRI) showed no abnormalities, he was hospitalized for steroid therapy. On day 11, he developed herpes zoster of the left buccal region, for which he was started on treatment with valacyclovir. Right facial nerve palsy became apparent on day 17. Since the facial paralysis appeared on the side contralateral to the rash, it was inconsistent with Ramsay Hunt syndrome. Therefore, head MRI was performed, which lead to the diagnosis of pontine infarction. Cerebrospinal fluid examination on day 19 showed a slight increase in the number of cells as well as an increase in the protein and sugar levels, the clinical findings being suggestive of varicella zoster virus (VZV) meningitis. The VZV-index on day 27, calculated from the total IgG and anti-VZV-IgG levels in the blood and cerebrospinal fluid, was 2.04 (>2.0), indicative of cerebral infarction caused by VZV vasculopathy. Cerebrospinal fluid examination revealed no abnormalities on day 32, and the patient was discharged from the hospital.
In this patient, the facial paralysis appeared on the side opposite to the rash. Since the typical rash of Ramsay Hunt syndrome appears on the pinna or the pharynx, other diseases, such as cerebral infarction or mononeuritis multiplex, should have been suspected even if the rash had appeared on the same side. Paralysis of the forehead is usually mild in cases of central facial paralysis, but in this case, the forehead was also paralyzed because of involvement of the entire right facial nerve nucleus. The risk of development of cerebrovascular disease secondary to VZV meningitis, so-called VZV vasculopathy, is 1.3 times higher during the first 3 months after the onset of facial herpes zoster. Nuclear facial nerve paralysis should be considered in the differential diagnosis, even if movements of the forehead are only affected unilaterally.
We report the case of a patient with a dural arteriovenous fistula in a high jugular bulb. A 33-year-old woman visited our hospital with a one-month history of right pulsatile tinnitus. Her right eardrum was dark-red in color, with swelling of the small blood vessels. A pure tone audiometry showed normal hearing. Temporal-bone CT revealed a high jugular bulb on the right side, which was then considered as the cause of the pulsatile tinnitus. Since the tinnitus did not improve with the conservative treatment, a head MRA/contrast-enhanced MRI examination was performed for further evaluation. The MRI findings suggested the presence of a dural arteriovenous fistula in the expanded right jugular bulb. Angiography was performed by the brain surgeon, which revealed a dural arteriovenous fistula at the right transverse-sigmoid sinus fed by a large number of branches derived from the right external carotid artery, internal carotid artery, and vertebral artery. There was no stenosis in the right transverse-sigmoid sinus, and it functioned as a normal venous return pathway. Based on the above, the patient was diagnosed as having a Borden Type 1 arteriovenous fistula, and it was decided to follow up the patient. Because a high jugular bulb could be associated with other vascular abnormalities and temporal bone tumors, head MRI/MRA is useful for the diagnosis. Dural arteriovenous fistulas may show progression, and strict follow-up is recommended even in the absence of any proactive treatment.
Bevacizumab is a molecular-targeted agent that targets vascular endothelial growth factor and is used in the treatment of some types of cancers. Rare cases of nasal septal perforation occurring after bevacizumab therapy have been reported. Herein, we report a case of nasal mucosal adhesions that developed after bevacizumab administration.
The patient was a 62-year-old woman with ovarian cancer, who had received treatment with TCB (paclitaxel, carboplatin and bevacizumab) two years ago. She was referred to our hospital complaining of nasal obstruction. Examination revealed nasal mucosal adhesions; the mucosa lining the inferior turbinates on either side was adherent to the mucosa lining the nasal septum. No perforation of the nasal septum was recognized. Laboratory examination revealed negative test results for markers of ANCA-associated vasculitis, infectious disease, and autoimmune disease. The patient had no previous history of nasal operation or trauma. We dissected the adhesions and obtained biopsy specimens from the nasal mucosa under general anesthesia for the purpose of diagnosis and appropriate treatment.
Silicon seats were placed in the nasal cavity and the patient was administered tranilast and steroid collunarium after the operation to prevent readhesion. Biopsy revealed vascular hyperplasia and an abundant fibrous stroma.
There has been increased in the number of reports of nasal perforation in patients treatment with bevacizumab recently. An observational study of the findings in the nose after chemotherapy and bevacizumab in 47 cancer patients (including colon cancer, ovarian cancer, breast cancer, lung cancer, renal cancer, and cervical cancer) revealed some abnormal findings in 96%, color change in 57%, erosions in 30%, and perforation in 2% of the patients. There are no reports to date of nasal adhesions developing after bevacizumab administration, but we believe that the nasal mucosal adhesions in our case could have been caused by bevacizumab.
A schwannoma is a benign tumor that arises from the Schwann cells surrounding the axons of the peripheral nerves. Nasal and sinonasal schwannomas are extremely rare and account for only 1% of all schwannomas. Schwannomas are essentially treated by surgical removal of the tumor, including the base. We managed a patient with a schwannoma that originated from a paranasal sinus and extended to the skull base. The patient, a woman in her 70s, presented with discomfort in her right eye due to a right ethmoid sinus tumor. The intranasal findings showed a smooth-surfaced, hard mass in the right ethmoid sinus that compressed the mucosa of the middle nasal passage. CT and MRI showed that the tumor had eroded the skull base and medial wall of the orbit, thinning the bone, extending to the frontal sinus, and deviating the nasal septum; however, no apparent bony destruction was noted. A biopsy was performed under local anesthesia, and the histopathological diagnosis was suspected schwannoma. The operation was performed via an endoscopic approach. As a preliminary step prior to tumor removal, the left ethmoid sinus was opened widely, a right medial maxillectomy was performed, and the nasal septum was separated caudal to the tumor. After completion of the segmental resection, the tumor and dura were carefully dissected, and the base of the tumor was removed. Because the dura was partially damaged, the spinal fluid leak was closed using free fat (bath plug closure method) and a vascularized nasoseptal flap. The histopathological findings comfirmed the mass as a schwannoma.
Leiomyosarcoma is a tumor that rarely arises from the thyroid gland. Therefore, diagnosis of leiomyosarcoma of the thyroid may be difficult, and the treatment remains controversial. We report the case of a 73-year-old male patient who presented with the complaints of a growing mass in the front of the neck and dysphagia. Imaging studies showed a solid mass in the right lobe of the thyroid, without regional or distant metastasis. Cytology was diagnostic for a malignant tumor of the thyroid, although the precise histopathological diagnosis remained unknown. We performed hemithyroidectomy and regional neck lymph node dissection. Immunohistochemical examination of the specimen showed positive staining for actin and desmin, while the results were negative for other markers such as pan-cytokeratin, epithelial membrane antigen, S-100, chromogranin A, synaptophysin, GFAP, CD31, CD34, SS18-SSX, and SOX10; based on the findings, the tumor was diagnosed as a leiomyosarcoma. Postoperative adjuvant radiotherapy at 60 Gy was undertaken, because histopathology revealed invasiveness of the tumor and a positive surgical margin. At the 6-month follow-up after surgery, no recurrence was detected and the patient remains alive. Differential diagnosis of thyroid leiomyosarcoma is important and includes other malignancies of the gland. The prognosis of this disease is poor and there is no consensus regarding the treatment.
The most common clinical feature of a primary thyroid lymphoma is a rapidly growing thyroid mass, mimicking anaplastic carcinoma, often with dyspnea.
A 77-year-old man presented with a rapidly growing neck mass and dyspnea. Computed tomography (CT) showed diffuse enlargement of the thyroid, with compression of the trachea.
As the patient showed evidence of airway obstruction, taking into account the tumor location of the tumor, we performed cricotracheostomy, and obtained a biopsy specimen from the thyroid for histopathological examination. The lesion was diagnosed as a thyroid lymphoma, and the patient was treated with six courses of R-THP-COP chemotherapy. Following this treatment, he achieved complete remission.
We present a case of cricotracheostomy performed for a patient with a primary thyroid lymphoma. Despite the tumor surrounding the trachea, by selecting cricotracheostomy, we could create an opening in the airway more easily and safely, and even obtained a biopsy specimen.
Elongated styloid processes could be associated with a variety of symptoms, which have been described in detail by W. Eagle.
We present the case of a patient with styloid process syndrome who presented with dizziness. A 42-year-old woman was referred to our hospital with a 4-year history of pharyngeal pain, tinnitus and dizziness during cervical rotation. Although she had been suffering the symptoms for four years, no diagnosis had been made and she was not under clinical follow-up. Her condition became steadily worse.
3D-CT showed bilateral elongated styloid processes compressing the internal jugular vein medially at the level of the first to second cervical vertebra.
Elongated styloid processes cause compression of the internal jugular vein during changes of the head position. Compression of the internal jugular vein causes decrease of the cardiac output and decrease of the cerebral blood flow. It was predicted that the cause of dizziness in these patients is circulatory disturbance, when Nagayama and others reported cases in which dizziness was accompanied by a decrease of the brain blood flow. They described both cases with reduced blood flow in the vertebral artery and those with reduced blood flow in the internal carotid artery. When the blood supply decreases in the vertebral artery, it also decreases in the vertebrobasilar artery, and reduction of blood flow in the vertebrobasilar arteries causes dizziness and disturbance of consciousness as the major symptoms, with nystagmus induced by neck rotation. On the other hand, it is said that impaired carotid artery blood flow mainly causes disturbance of consciousness as the major symptom, and rarely produces dizziness. However, it has been reported that dizziness can occur when the overall cerebral blood flow decreases with decrease in the blood flow to the brainstem.
The patient underwent surgical treatment via the transcervical approach. Postoperative 3D-CT showed that the lengths of the residual styloid processes were within normal range (within 30 mm), and the subjective symptoms of the patient, including dizziness, improved.
Skull base osteomyelitis is an extremely rare disease that usually occurs by direct spread of bacterial or fungal infection from adjacent organ, such as the paranasal sinus or external ear canal. However, some cases without a clear primary focus of infection, such as paranasal sinusitis or malignant otitis externa, have also been reported. In such cases, definitive diagnosis is difficult. We encountered a case of skull base osteomyelitis caused by fungal invasion that spread from the nasopharynx to the skull base, in which the original focus of infection remained unclear.
A 76-year-old man with a history of diabetes mellitus was referred to our hospital with left otalgia and headache. CT examination showed bone erosion of the skull base and slight swelling of the nasopharynx. A biopsy specimen obtained from the nasopharynx revealed inflammation, but no evidence of malignancy. PCR for tuberculosis and bacterial or fungal culture of the collected tissues were negative. Serum β-D glucan was negative. Amoxycillin hydrate was prescribed as empiric therapy. While the headache and left otalgia improved transiently, the patient complained of severe headache again while the antibiotic therapy was still ongoing. In addition, he developed hoarseness caused by left vocal cord paralysis. A repeat serum test at this time revealed a positive result for β-D glucan. We prescribed liposomal amphotericin B, an antifungal agent, and the headache resolved. We made the clinical diagnosis of skull base osteomyelitis caused by invasive fungal infection.
When bone erosion of the skull base with swelling around it is observed, it is important to rule out skull base osteomyelitis caused by fungal infection, besides malignant tumor. Moreover, in some cases, empiric therapy with an antifungal agent(s) is needed despite the absence of pathological or microbiological evidence of fungal infection.