The number of inner hair cells in the cochlea is about 3000, compared with as few as 20 for cochlear implant (CI) electrodes. However, CI users can understand speech rather well. This finding suggests that our brain may play a substantial role in understanding spoken language coded by CI. We used functional brain imaging to examine how the brain manages to perceive speech in CI users. We subtracted brain activities of normal individuals from those of CI users during listening to speech, and found increased activities in various cortical areas of CI users, including the areas that are not usually activated during speech perception. These findings show that more data processing has to be done in the brain to decode the fewer and artificial signals delivered by CI. We know that the plasticity of the brain has a strict time limitation. For acquisition of spoken language, the most sensitive period of the brain appears to be from 0 to 3 years of age. This developmental plasticity decreases gradually with age, and is lost almost completely after puberty. The human brain develops dramatically after birth. The weight of the brain of a newborn baby is about 400 grams but at the age of 13 years the brain weight has almost reached adult values of around 1300 grams. The rapid growth of the brain is not due to an increase in the number of neurons, but an increase in the synapses that connect them together with development of the myelin sheath that envelopes the nerve fibers and blood vessels that supply the neural structures with glucose and oxygen. The neural networks for verbal activity are established during this period. The brain is considered to respond flexibly to changes in the input. Sign language significantly activates the temporal cortex in congenitally deaf native signers. The temporal cortex, expected to be activated by speech, is also activated by visual input. It has also been revealed that in congenitally blind subjects, brail reading with the fingers significantly activated the visual cortex. We know that CI users do not enjoy music due to technical limitation of the CI processor in coding pitch. However they enjoy singing a song a cappella utilizing the pitch sensation located laryngeal muscles. It may be important to develop a viewpoint from which we can see not only the peripheral organs that code speech, but also the brain that decodes the auditory input signals and processes their meaning.
We encountered a 60-year-old woman with initial symptoms of dizziness diagnosed as meningeal relapse of an ovarian primary, from which meningeal carcinomatosis is very rare. Plain magnetic resonance imaging (MRI) of the brain at a local hospital showed no abnormalities. Although she was treated with oral medicine, her condition remained unimproved and she subsequently complained of right-sided hearing loss. She then visited our hospital. A physical examination on the first visit showed no abnormal nystagmus or cerebellar ataxia, but gadolinium-enhanced MRI of the brain revealed meningeal carcinomatosis. She subsequently showed rapid development of bilateral deafness and died 38 days after admission, despite radiotherapy. Diagnosis of meningeal carcinomatosis requires contrast-enhanced MRI rather than plain MRI. In the literature, dizziness complicated by meningeal carcinomatosis has mainly appeared as drift while walking and has generally proven intractable. No specific findings of nystagmus have been noted, appearing in some cases, but not in others. Conversely, hearing loss resulting from meningeal carcinomatosis usually develops rapidly and results in bilateral deafness. In conclusion, meningeal carcinomatosis is very rare in the natural history of ovarian cancer. In cases showing intractable dizziness and progressive hearing loss, particularly bilateral progressive deafness, meningeal carcinomatosis should be suspected. Contrast-enhanced MRI should be performed aggressively in suspicious cases.
Recently there have been cases of vertigo patients with Superior Semicircular Canal Dehiscence Syndrome. In this present report, we describe a 40 y.o. female patient with vertigo after nose-blowing. Although a vertigo patient with nose-blowing is often diagnosed as having a perilymph fistula, our patient was diagnosed as having left Superior Semicircular Canal Dehiscence Syndrome by interview, clinical course findings and CT findings. She did not suffer hearing loss, tinnitus nor any abnormality in the electric nystamography test. The eustachian tube function test revealed the stenosed type. Advice to be careful when blowing her nose helped her vertigo disappear especially as she did not wish to have any operative procedures.
The eccrine porocarcinoma is a rare malignant tumor derived from the sweat glands that is most commonly found on the leg. In the head and neck region the incidence of this tumor is rare, so we report herein on a rare case of eccrine porocarcinoma occurring on the auricle. A 74-year-old woman visited our hospital complaining of a mass on the left auricle. A CT scan showed with enhanced imaging that the mass that had not infiltrated the external auditory meatus. We removed the lesion surgically. Histological examination showed that the tumor was an eccrine porocarcinoma. At the 1-year postoperative follow-up there has been no evidence of recurrence.
Osteoradionecrosis of the temporal bone can occur after radiotherapy for head and neck cancer and brain tumors, such as parotid cancer and upper pharyngeal cancer. It sometimes causes external auditory canal cholesteatoma. We report herein on a 17-year-old female who received chemoradiation therapy for a brain tumor at the age of 10 years. Several years later, ear wax and debris began to accumulate in both external auditory canals. She also lost hearing in both ears. A CT scan showed extensive lytic change of both temporal bones. The semicircular canals of the right ear were also eroded. Pure tone audiometry showed moderate mixed hearing loss of the right ear and moderate conductive hearing loss of the left ear. We diagnosed an external auditory canal cholesteatoma caused by osteoradionecrosis extending to the middle ear. Although there has been no progression of her temporal bone lysis on imaging, the bone conduction hearing level of both ears had gradually deteriorated. She was treated conservatively by cleaning out the crust and debris, and systemic administration of a corticosteroid when the bone conduction hearing level worsened. Careful follow-up over a long time is needed to prevent serious complications.
The angiomyolipoma is thought to be a form of hamartoma, with most reported arising in the kidneys. Cases have also been described in the liver, skin, generative organ and oral cavity. However an angiomyolipoma at other sites is rare, and to our knowledge, only 17 cases have been described in the nasal cavity. It is easy to develop apoplexy if enlarged. We report herein on a rare case of angiomyolipoma of the nasal vestibule that did not cause apoplexy and ache. A 75-year-old female presented with slowly progressive left nasal obstruction. An intranasal examination showed a soft and smooth mass in the left anterior nares. CT scan revealed a mass, the inside of which was uniform, in the left nasal vestibule without bony destruction. MRI almost showed water intensity. The patient had no clinical signs of tuberous sclerosis and hypertension. As for the preoperative diagnosis, a mural cyst was most strongly suspected but a nasal cavity tumor could not be ruled out, either. The tumor was removed through the vestibule using a transoral approach under general anesthesia. The tumor was solid and needed pathological diagnosis, which revealed a final diagnosis of angiomyolipoma. Histopathologically, the tumor was composed of mature fat cells, vascular spaces and bundles of mature smooth muscle cells. There has been no recurrence for 16 months after the surgery.
Idiopathic nasopharyngeal stenosis is a rare disease entity and a standard treatment has not been established. A 71-year-old woman visited our hospital with bilateral nasal obstruction. Nasal fiberscopy showed severe nasopharyngeal stenosis. Muscles including the palatal tensor Levator veli palatine muscle and surrounding submucosal connective tissue looked intact on MRI. Inflammatory findings were not observed with pathological examination of biopsied tissue from the nasopharynx. Nasopharyngoplasty using a pedicled musculomucosal flap harvested from the buccal region was performed. Recurrence of the nasopharyngeal stenosis has not been observed in a 1-year follow-up.
Chordoma is a relatively rare malignant tumor of the skull base and sacrum thought to originate from embryonic remnants of the notochord. Chordomas arising from the skull base typically occur in the clivus, and extraosseous chordoma of the nasopharynx is extremely rare. A 34-year-old woman was diagnosed as having a nasopharyngeal tumor on April, 2009. Computed tomography and magnetic resonance imaging revealed a tumor in the nasopharynx not extending into the intracranial region. FNAC examination revealed findings suggestive of pleomorphic adenoma. Based on a suspected diagnosis of pleomorphic adenoma of the nasopharynx, transnasal endoscopic surgery using CO2 laser was performed. Histological examination of the resected specimen revealed the diagnosis of chordoma. There has been no evidence of recurrence until the present, 3 years since the operation.
Between July 2004 and December 2007, 15 patients with sinonasal papilloma were surgically treated at the Department of Otolaryngology, Saitama Medical Center, Jichi Medical University. All patients underwent endonasal sinus surgery (ESS) under general anesthesia. In preoperative imaging diagnosis, MRI was useful for evaluating the extent of tumor growth, and CT was useful for determining the presence or absence of osteolysis. In principle, ESS consisted of opening of the ethmoidal sinus, followed by resection of the membranous portion containing the lesion, and opening of the maxillary sinus. When the tumor base was located in the ethmoidal/maxillary sinus, extensive resection of the tumor and the surrounding normal mucosa was performed. When resection via the membranous portion alone was impossible due to tumor growth anterior to the maxillary sinus, after confirmation of the opening of the nasolacrimal duct, the inferior nasal meatus was opened, as much as possible, and manipulation via the inferior nasal meatus was also performed. The nasolacrimal duct, including the opening, was preserved. A microdebrider was used in all patients. No recurrence has been observed to the present in 11 of 14 patients who underwent total resection of the tumor. The other 3 patients with recurrence underwent ESS again under local anesthesia and have shown no recurrence to the present. In remaining 1 patient, preoperative CT showed osteolysis, and squamous cell carcinoma as a complication was suspected. However, since the endonasal biopsy at the outpatient clinic did not facilitate a definite diagnosis, ESS was performed mainly for pathological examination, and a diagnosis of squamous cell carcinoma was made. Subsequently, this patient underwent cranial base surgery in another institution. Since importance has been attached to functional surgery in recent years, ESS for sinonasal papilloma meets the demands of the times.
The odontogenic ghost cell carcinoma (OGCC) is the malignant counterpart of a calcifying cystic odontogenic tumor and dentinogenic ghost cell tumor. We report herein on a case of OGCC of the maxilla. A 31-year-old male visited our department complaining of a 2-year history of left cheek swelling. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a huge maxillary tumor invading both sides of the maxilla. A biopsy was performed, and the lesion was diagnosed as OGCC. After bilateral total maxillectomy, radiotherapy was performed. Neither local recurrence nor distant metastasis has been seen for 14 months since surgery. Previous reports of OGCC are also reviewed.
Granulocytic sarcoma is a rare extramedullary tumor associated with acute myeloid leukemia (AML) or myeloproliferative neoplasmas. It may arise from any site in the body, although involvement of the head and neck region is rare. A 7-year-old male presented with the chief complaint of swelling in the left parotid region. Parotid gland tumor was suspected at the first examination, however, CT and MRI showed that the tumor was located in the left masseter muscle. Peripheral blood examination revealed an increased count of leukocytes and myeloblasts. AML was diagnosed based on bone-marrow examination. Histological examination of an open biopsy from the tumor confirmed leukemia-like cells. Based on the findings, a final diagnosis of granulocytic sarcoma of the masseter muscle with AML was made.
Cancer of the lip is comparatively rare, and it has been reported that this cancer accounts for 1.56-3.0% of malignant tumors of the mouth. Surgical resection is often selected for treatment of lower lip cancer, however, resection may cause not only cosmetic problem but also functional disadvantage from the point of feeding and speech. We performed surgical reconstruction of a lower lip with a fan-shaped flap on a patient following resection of a lower lip squamous cell carcinoma (SCC). The patient was a 76-year old man with a tumor on the lower lip, and was referred to our department by a practicing physician in late September, 2007. The histopathological diagnosis of the tumor biopsy sample was SCC, which was surgically resected. We performed surgical reconstruction with the fan-shaped flap. After the operation, he was satisfied with the cosmetic appearance, and did not complain about any problems related to feeding or speech.
It has been reported that concurrent chemoradiotherapy (CCRT) can result in a superior treatment response and survival outcome compared with radiotherapy alone in patients with squamous cell carcinoma of the head and neck, and it has become the standard of care for locally advanced disease and organ preservation. However, the major limitation to radiotherapy or CCRT is locoregional treatment-related toxicities, particularly oral mucositis (OM). We experienced two cases of pain-uncontrolled OM in which the Traditional Oriental Medicine Hange-shashin-to (TJ-14) was effective. Case 1: A 44-year-old man with nasopharyngeal carcinoma and neck metastases underwent CCRT and suffered from OM of grade 3 according to the Common Terminology Criteria for Adverse Effects (CTCAE). His pain was uncontrolled with a variety of analgesics, so we prescribed TJ-14 for him as a gargle. Even during CCRT, the pain significantly diminished and OM was improved to grade 1. TJ-14 contributed to completion of CCRT and improvement of the patient’s nutrition status. Case 2: A 67-year-old man with unknown primary and neck metastases underwent neck dissection and adjuvant radiotherapy. During adjuvant radiotherapy, he had OM of grade 3 and was unable to eat, so he was hospitalized and was started to have TJ-14. Although his OM remained grade 3 during the therapy, his pain was alleviated, leading to completion of the treatment. TJ-14 can be an effective supportive therapy for OM caused by radiotherapy.
Forty-four cases with stage IV laryngeal carcinoma treated in our department between 1991 and 2009 were clinically analyzed. The cases were equivalent to 16.8% of the total laryngeal cases, and 77.3% of the stage IV cases were of the supraglottic type. The disease-specific 5-year survival rate of all of the stage IV cases was 67.2%. Differences in the prognoses between under T3 and T4 were not statistically significant. Although the differences in the prognoses between surgical and nonsurgical treatment of the larynx were not statistically significant, neck dissection cases had a significantly better prognosis compared to cases without neck dissection. Neither radiotherapy nor chemotherapy had any influence on the prognosis. Therefore, positive treatment is needed even though the stage of the cases is advanced, and especially in cases with neck lymph node metastasis, neck dissection is strongly recommended. For histopathologically high-risk cases in which radiotherapy and chemotherapy were indicated in this study, appropriate additional treatment will be effective in order to prevent prognostic deterioration.
Renal cell carcinoma (RCC) often metastasizes to other organs such as the lungs and bones. However, solitary thyroid metastasis is very rare. We present herein on a case of solitary thyroid metastasis seven years after surgery for RCC of the right kidney. A 76-year-old man presented with an anterior cervical tumor and came to our hospital for examination. He had a history of total nephrectomy for RCC of the right kidney seven years previously. Cervical computed tomography and ultrasound examination revealed tumorous lesions on both lobes of the thyroid; fine-needle aspiration cytology revealed the aspirates to be class II. Adenomatous goiter was suspected, and total thyroidectomy was performed. Thyroid metastasis from RCC was diagnosed given that the histopathological tests showed clear cell carcinoma and thyroglobulin immunostaining was negative. No metastasis to other organs was found in a postoperative examination.