Cytomegalovirus (CMV) is a herpesvirus. Infection of an immunocompetent host is associated with no or only mild symptoms, but after the initial infection, the virus remains latent in the host’s body and coexists with the host for life, a characteristic common to all the herpes genus viruses. The clinical problems with CMV infection are intrauterine infection and infection/reactivation in patients with immunodeficiency. Intrauterine CMV infection is known to cause one of the TORCH syndromes and is the most frequent of all congenital viral infections. Even if the infant is asymptomatic at birth, delayed onset of disorders such as hearing impairment and mental retardation are not uncommon. Hearing impairment is a frequently associated neurological symptom with intrauterine infection, and CMV infection is reported as a frequent cause of congenital severe hearing loss. In this paper, we discuss the epidemiology, mechanism, treatment, and prevention of hearing impairment associated with intrauterine CMV infection.
The patient, a 65-year-old man who presented with a history of dizziness 2 years after suffering a pontine hemorrhage, was referred to our department for a thorough examination.
Examination of the nystagmus revealed pendulum-like eye movements, regardless of the gaze or head position. A head CT obtained at the time of the pontine hemorrhage showed a high-density area almost in the pontine midline, and T1-weighted MRI showed a high-intensity area in the periphery and a low-intensity area within the same area.
Because of the development of abnormal eye movements 2 years after the pontine hemorrhage, we suspected ocular myoclonus and performed electro-oculography, pharyngolaryngoscopy, and MRI. Electro-oculography showed pendulum-like eye movements with both horizontal and vertical components. Pharyngolaryngoscopy revealed myoclonus of the soft palate and larynx synchronized with the rhythm of the eye movements. T2-weighted MRI showed a high-signal area to the right of the medulla oblongata that was not seen in the images obtained two years earlier, and enlargement of the right anterior medulla oblongata, and we considered the possibility of hypertrophic olivary degeneration. Considering the MRI findings along with the finding of abnormal eye movements with myoclonus in multiple areas synchronized with the eye movements developing after pontine hemorrhage, we diagnosed the patient as having ocular myoclonus.
We initiated treatment with clonazepam and gabapentin, but the abnormal eye movements and subjective symptoms failed to improve. The symptoms showed neither improvement nor worsening until 3 years later, and the follow-up was terminated.
Ocular myoclonus, a type of abnormal ocular oscillation, is a 1.5- to 5-Hz vertical eye movement that presents as a sustained periodic pendulum-like oscillation of the eye and includes horizontal and rotatory components. It is often accompanied by myoclonus of the soft palate and may be accompanied by extensive involuntary movements of the tongue, facial muscles, pharynx, larynx, and diaphragm. It usually appears after a latency of 2–48 months after brainstem or cerebellar hemorrhage.
A 72-year-old man visited our hospital with the complaints of right-sided otorrhea and hearing loss. Otoscopy revealed a large central perforation of the tympanic membrane with granulation tissue at the promontory. A bacterial smear test and culture of his ear discharge were negative for acid-fast bacilli. Histopathological examination of a biopsy specimen obtained from the granulation tissue revealed only necrotic tissue. Thirty-seven days after his first visit, the patient presented with cough and high fever and showed a positive result of the T-SPOT®.TB blood test for tuberculosis. Based on the observation of a diffuse infiltrative opacities on chest CT and a positive sputum culture test for the Mycobacterium tuberculosis, the patient was diagnosed as having pulmonary tuberculosis. He was also simultaneously diagnosed as having tuberculous otitis media based on a positive result of PCR test of the otorrhea fluid. The patient was started on anti-tuberculosis chemotherapy with isoniazid, rifampicin, ethambutol, and pyrazinamide.
Two years after his first visit to our hospital, we perfomed myringoplasty using a full-thickness tragal cartilage and periosteal pedicle graft to avoid graft necrosis caused by potentially poor wound healing secondary to recurrent tuberculosis. His postoperative course was good and the tympanic membrane showed rapid epithelialization.
Epistaxis is a common symptom encountered in the field of otorhinolaryngology. Hemostasis is easy in patients with bleeding from the Kiesselbach’s area, but often difficult in those with bleeding from the posterior nasal cavity or olfactory cleft. In addition, presence of a deviated nasal septum makes hemostasis more difficult. We immediately undertake to perform hemostasis under general anesthesia in patients in whom hemostasis at the outpatient department proves difficult.
We analyzed the data of a total of 60 patients with epistaxis in whom hemostasis was accomplished under general anesthesia at Hokuto Hospital between January 2013 and April 2022 in this study. The analyzed data included the age, sex, month of onset, comorbidity, number of days from onset to hemostasis, number of days from the first visit to hemostasis, site of bleeding, hemostasis method used, presence of rebleeding, and treatment used for rebleeding.
The median age of the patients was 58 years, and there were 41 male patients (68%) and 19 female patients (32%). The number of patients was the largest in December. Hypertension was the most common comorbidity (32 [53%]/60 patients). The median number of days from onset to hemostasis and to the first visit were 2.5 days and 0.5 days, respectively. The posterior nasal cavity was the most common site of bleeding (43 patients, 72%). Endoscopic electrocoagulation was performed in 32 patients (53%), and endoscopic electrocoagulation with sphenopalatine artery coagulation in 28 patients (47%). Septoplasty was added in 12 patients (20%). Bleeding recurred in only 2 patients (3%) after the hemostasis.
The rebleeding rate following treatment by the method described in the present study was lower than that by gauze compression under local anesthesia reported in the literature. Immediate hemostasis by endoscopic electrocoagulation and/or sphenopalatine artery coagulation under general anesthesia is an excellent method to control severe bleeding from the posterior nasal cavity or olfactory cleft.
Maxillary external dental fistula is a chronic suppurative inflammatory disease of the tooth root, leading to fistula formation between the affected tooth and the facial or neck skin. More than half of the patients initially consult the department of otorhinolaryngology, surgery or dermatology, rather than the dental department, because of the absence of dental symptoms or because the lesions are distant from the affected tooth. The therapy is extraction of causative tooth (or teeth) and managing the inflammation. It is sometimes difficult to diagnose this condition because the patients may altogether fail to recognize the dental issue as a source of their condition. Herein, we report a rare case of a left maxillary external dental fistula in a 74-year-old female patient who presented with swelling of the base of the nasal wing and was initially suspected as having a maxillary malignant tumor. However, subsequent CT and histopathological examinations revealed the diagnosis of a maxillary external dental fistula. The causative tooth was the left maxillary canine, and the roots of two neighboring teeth were also involved. The patient was initiated on therapy with a antibiotics (amoxycillin) and the three involved teeth were extracted. The patient showed a satisfactory clinical response to the treatment. Recently, among dentists, conservative therapy for the teeth, without extraction, is becoming more popular for this disease. With the increasing number of elderly patients with teeth problems, it is necessary for otorhinolaryngologists to differentiate such dental diseases from other diseases such as malignant tumors and to collaborate with dentists to ensure effective dental therapy.
Dentigerous cyst is a developmental cyst that arises from the epithelium of the tooth germ. While otolaryngologists often encounter patients with radicular cysts, which causes maxillary sinusitis, there are some differences in pathology between dentigerous cysts and radicular cysts. A typical finding of a dentigerous cyst is the inclusion of the crown of a tooth within the cyst. Various symptoms might appear depending on the site and size of cyst. Surgical treatment is undertaken for patients with a facial deformity, sinusitis, and eye symptoms, and curative surgery is selected because of reports of malignant transformation in some cases. Conventionally, the external incision approach has been the standard surgical method. However, an increasing number of reports of successful resection by the endoscopic transnasal approach have been published in recent years.
We received referral for a case of a dentigerous cyst occupying the maxillary sinus, and the chief complaint of the patient was excessive lacrimation. The findings in this case were typical, including presence of the crown of a tooth within the cyst and the double wall sign on CT. Therefore, we could rapidly diagnose the cyst as a dentigerous, not a radicular, cyst. We planned surgical treatment by endoscopic endonasal resection with the endoscopic modified medial maxillectomy (EMMM) approach, because the tooth was on the lateral side of the maxilla. Finally, the dentigerous cyst was successfully resected with the tooth, cyst wall, and the mucosal epithelium lining the cyst wall, and there has been no recurrence.
This was a case of a dentigerous cyst that occupied the maxillary sinus which was completely resected by the EMMM approach. The EMMM approach appears to be useful as a complete resection approach for dentigerous cysts.
Eosinophilic chronic rhinosinusitis (ECRS) is a refractory and recurrent disease and its prevalence has been increasing. ECRS recurrence is known to be associated with the early development of frontal recess polyps after endoscopic sinus surgery (ESS). We found it challenging to address recurrent frontal sinus opacification early after surgery at our department. Consequently, in August 2018, we modified our technique for opening the frontal sinus drainage pathway (FSDP), introduced the building block concept, and changed the surgical instruments used for opening the FSDP. Our present study included 32 patients with ECRS who underwent type IV ESS at our department between March 2016 and October 2020; the patients were divided into two groups: group A and group B, comprising patients who underwent ESS before and after August 2018, respectively. To evaluate the effects of the changes, we retrospectively compared the operative time, degree of patency of the frontal recess, computed tomography (CT) score improvement rate (IR), and peripheral blood eosinophil percentage between the two groups.
Group A showed no significant difference in the degree of patency in the frontal recess or peripheral blood eosinophil percentage before and after the surgery, whereas group B showed significant improvement of both. Furthermore, group B showed a significantly higher IR of all the sinuses, including the posterior ethmoid and sphenoid sinuses than group A, although the IRs of the frontal, anterior ethmoid, and maxillary sinuses did not differ statistically significantly between the two groups.
Despite the significantly improved frontal recess patency in group B, no significant differences in the IR of the anterior ethmoid and frontal sinuses were observed between the two groups. This could be attributable to persistent sinus opacification due to inadequate removal of the lamina in the ethmoid sinus. Improvement of total sinus opacification in patients with ECRS may reduce postoperative peripheral eosinophilia. Future studies with long-term follow-ups are warranted to further explore this possibility.
Pyriform sinus fistula (PSF) is a congenital fistula. It usually manifests as acute suppurative thyroiditis or neck abscess, and the diagnosis is often made in patients presenting with these infections. Patients with a previous history of infection are candidates for surgical treatment. Our patient was a 34-year-old man who presented to another doctor with swelling and pain of the anterior neck. He was initially diagnosed as having subacute thyroiditis and treated with a corticosteroid. However, he presented at a later date with a neck abscess, based on which he was diagnosed as having a PFS and referred to our department. We treated his PFS by surgical resection; the postoperative course was favorable, and there has been no recurrence. Sometimes, it is difficult to identify PFS due to the presence of inflammatory scarring. In the case reported herein, we accomplished complete surgical resection of the PSF with some careful prior preparation, including asking the patient to drink Pyoktanin blue solution before the operation, using a laryngoscope and gastroscope to observe the hypopharynx, introducing a thin tube into the PSF, and so on. These ingenuities are very useful to the success of operation for PFS.
Ever since the onset of the COVID-19 pandemic in December 2019, several variants of the severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2) have arisen. Although the alpha, beta, and delta variants mainly caused lower respiratory syndromes, the omicron variant caused upper airway symptoms like fever, sore throat, and odynophagia. After the omicron variant began to spread, it was reported that diffuse necrotic erosive lesions in acute laryngitis could be an indicator of infection with this variant. We report two cases of acute laryngitis caused by COVID-19.
One patient was a 40-year-old woman who presented with odynophagia and difficulty in swallowing. Flexible fiberoptic laryngoscopy revealed erosions and necrotic lesions on the posterior surface of the epiglottis and bilateral arytenoids. The patient was diagnosed as having COVID-19 using an antigen test. She was hospitalized and given intravenous fluids and analgesics. On the third hospital day, she began to eat and was discharged from the hospital.
The other patient was an 80-year-old man who presented with fever, odynophagia, and difficulty in swallowing. Flexible fiberoptic laryngoscopy revealed diffuse erosions along with edematous necrotic lesions on the posterior surface of the epiglottis, subglottis, and bilateral arytenoids. Saliva retention was observed at the hypopharynx. After COVID-19 was diagnosed based on an antigen test, the patient was hospitalized and treated with remdesivir, dexamethasone, and intravenous fluids. He began to eat on the third day and was discharged from the hospital on the fifth day. We diagnosed both cases of laryngitis as being due to the omicron variant based on the laryngeal findings and the status of the epidemic at that time.
Laryngitis caused by the omicron variant should be suspected from the characteristic laryngoscopic findings. We should collect information about the current status of the epidemic to suppress the spread of damage caused by the pandemic.
Small cell carcinoma arises most commonly in the lungs and rarely in the larynx. Since no therapeutic strategy for small cell carcinoma of the larynx has been established yet, chemotherapy, radiotherapy, and/or surgery are selected by reference to the principles of treatment of small cell carcinoma of the lung. The prognosis of small cell carcinomas has been reported to be poor because of the propensity of these tumors to show rapid progression and metastasize early. We report a patient with primary small cell carcinoma of the larynx who was treated by surgery and survived for more than 5 years after the surgery.
A 76-year-old man presented to a previous medical facility complaining of pharyngeal pain. A tumorous lesion was found on the left of the epiglottis, and a biopsy of the lesion was performed by laryngeal microsurgery at the previous medical facility. As the histopathology revealed findings suspicious for small cell carcinoma, the patient was referred to our department. PET-CT showed no evidence of distant metastasis, and there was no obvious accumulation in the primary tumor. Based on the findings, the patient was diagnosed as having carcinoma of the epiglottis, cT1N0M0. A transoral videolaryngoscopic surgery (TOVS) was performed to completely resect the epiglottic tumor under general anesthesia.
Postoperative histopathology revealed small tumor cells resembling plasmacytes with ubiquitous subepithelial nuclei and small follicular proliferations, and positive immunohistochemical staining of the tumor cells for chromograninA, synaptophysin, CD56, AE1/AE3, and p40. Based on the findings, the tumor was definitively diagnosed as a neuroendocrine carcinoma, small cell type. The patient showed no evidence of any recurrence for more than 5 years after the surgery even in the absence of any additional treatments. In this case, early detection and surgical intervention in the early stage led to long-term survival of the patient. Surgical treatment is suggested to be effective in patients with primary small cell carcinoma of the larynx when the lesions are in the early stage and limited, without metastasis.
Granular cell tumors are rare benign tumors that can arise in any part of the body. We report a case of granular cell tumor on the right cheek. A 62-year-old man presented with a mass in his right cheek that he had first noticed several years earlier. The mass was elastic- hard, poorly mobile, and was not tender to palpation. The oral mucosa over the mass was intact. CT showed a solid tumor on the right cheek without contrast enhancement or bone destruction. On both T1- and T2- weighted MRI, the mass was visualized as being iso-intense with muscle tissue. A fine-needle aspiration cytology revealed granular cytoplasm and no nuclear atypia, suggestive of a granular cell tumor. We performed resection of the buccal tumor through the gingival approach. The maxillary nerve was identified inside the tumor and preserved. The subcutaneous mass was resected with a safety margin of a few millimeters. Postoperative histopathology confirmed the diagnosis of granular cell tumor. There has been no evidence of recurrence for 2 years. Granular cell tumors are benign tumors of the soft tissues, and in the present case, fine-needle aspiration cytology was useful for the diagnosis. Although the treatment of choice is surgical resection, the extent of resection should be determined taking into consideration both the expected functional and cosmetic outcomes.
We report a case of refractory auricular inflammatory disease.
A 58-year-old female patient visited her local doctor complaining of left ear pain and swelling of the left auricule. She was referred to our department as the symptoms persisted even at 4 weeks after the onset. After 19 days of visiting our department, she was admitted to the respiratory department. After 22 days, the patient developed dyspnea, and after 23 days, she developed wheezing and other signs of airway narrowing, so that an emergency tracheotomy was performed. Clinically, the patient was diagnosed as having relapsing polychondritis (RP) which was classified as severe based on the score of 16 on the Severity Classification for this disease, and the patient was started on steroid therapy.
Histopathology of cricoid and auricular cartilage biopsied at the time of tracheostomy showed inflammation of the auricular perichondrium and auricular cartilage, MRI revealed evidence of polychondritis, and a serologic test for type II collagen antibodies returned positive, confirming the diagnosis.
The symptoms improved promptly in response to the steroid therapy, but the patient was also started on methotrexate (MTX) for treating the residual inflammatory reaction. After the symptoms resolved, the tracheal cannula was removed and the tracheal foramen was closed, and there has been no recurrence for more than one year.
There are many reports of patients with RP developing dyspnea and cardiopulmonary arrest, as well as cases with a long time to diagnosis. Although auricular chondritis usually occurs as a bilateral disease, unilateral auricular chondritis has been reported to occur in 27.5% of cases. A major difference from infectious auricular cellulitis is that the redness and swelling do not extend to the auricle, which has no cartilage.
The auricular cartilage is often the first site of manifestation in cases of RP, and the condition may progress rapidly and become severe in some cases. When erythema and swelling that do not extend to the auricular cartilage are observed, systemic search and a biopsy must be performed proactively while paying attention to the systemic findings, even if the signs are not bilateral.