It is well known that Ménière’s disease is associated with endolymphatic hydrops (EH). Before the development of delayed contrast-enhanced MRI for the inner ear, EH could not be directly evaluated. Consequently, its presence was inferred using physiological examinations, referred to as endolymphatic hydrops estimation tests.
Among the various tests developed for this purpose, we have proposed the furosemide loading cervical vestibular evoked myogenic potential (FVEMP) test. This is because VEMP reflects the function of the saccule, which is the second most frequent organ in EH. This practical test evaluates changes in cVEMP amplitude before and after furosemide administration. A result is considered positive when there is an improvement rate of ≥14.2% or when no response is observed before administration but a significant response appears afterward.
The sensitivity and specificity of FVEMP for diagnosing Ménière’s disease are 0.706 and 0.810, respectively. This test can assess potential EH in the unaffected ear of unilateral Meniere’s disease (with normal hearing) and evaluate the presence of EH in ears with profound deafness.
The results of FVEMP are not associated with the degree of EH in the cochlea but are correlated with the degree of EH in the vestibule observed on inner ear contrast-enhanced MRI. When EH in either the cochlea or vestibule, or both, is defined as positive on inner ear contrast-enhanced MRI, the concordance with FVEMP demonstrates substantial agreement, with a Kappa coefficient of 0.644.
Inner ear contrast-enhanced MRI is undoubtedly a groundbreaking tool for the diagnosis and management of Meniere’s disease. However, it cannot be performed at all facilities, and contraindications exist for certain cases. FVEMP may serve as a useful and objective method for estimating the presence of endolymphatic hydrops.
When examining patients with vertigo, it is essential to differentiate between central and peripheral causes. We report on a patient who had been treated as a case of benign paroxysmal positional vertigo (BPPV), but was eventually diagnosed as having cerebellar nodulus infarction. The patient visited our hospital complaining of rotatory vertigo, and we diagnosed and treated the patient as a case of BPPV. He was able to walk, but the dizziness changed from rotatory vertigo persisted. Computed tomography angiography (CTA) revealed obstruction of the right vertebral artery, and the patient was eventually diagnosed as having cerebellar nodulus infarction. Infarction in the lower cerebellum is characterized by vertigo without cerebellar ataxia, and the clinical picture may resemble that of vestibular neuritis or BPPV. We should be aware of cases of cerebellar infarction in which nystagmus can mimic that in BPPV.
We evaluated the postoperative hearing results of 103 patients who underwent type IV tympanoplasty between 1998 and 2021. Using the postoperative hearing result assessment criteria proposed by the Japan Otological Society in 2010, the hearing was evaluated at least 1 year after the surgery. The success rate of hearing improvement in the cases was 51.5%.
Good hearing results after type IV tympanoplasty were obtained in 80% of patients with non-inflammatory diseases such as ossicular chain dislocation or middle ear abnormalities. Good hearing results were also obtained in 75% of patients with congenital cholesteatoma, 51.2% of patients with otitis media, and 43.9% of patients with acquired cholesteatoma.
When the postoperative hearing results were analyzed according to the type of material used for the ossiculoplasty, the success rate in terms of hearing improvement was the highest in patients in whom a ceramic total ossicular replacement prosthesis (TORP) had been used, followed by patients in whom cartilage and bone autografts had been used. However, while the ceramic TORP was useful in patients who had undergone type IV tympanoplasty, caution is needed when a ceramic TORP is used, owing to the risk of development of perilymphatic fistula as a complication.
In fact, we encountered two rare cases of traumatic perilymphatic fistula induced by intrusion of the ceramic TORP into the vestibulum.
One of the two patients had tympanosclerosis with a brittle stapedial footplate; the other had a recurrent cholesteatoma and had undergone myringoplasty using a thick tragal cartilage graft, which accidentally pressed indirectly onto the brittle footplate.
In both cases, the axis of the ceramic TORP intruded into the oval window, resulting in the formation of a traumatic perilymphatic fistula manifesting as severe dizziness in the patients. These cases lend support to our contention that caution is needed when performing type IV tympanoplasty using a ceramic TORP, owing the risk of formation of a perilymphatic fistula and severe dizziness with the use of a rigid ceramic TORP.
A clinical study was conducted of 121 patients with a foreign body in the external auditory canal who were treated at Himeji St. Mary’s Hospital between January 2013 and December 2023, including one case of aural myiasis. The patients ranged in age from 1 to 92 years, but patients under 9 years of age represented the largest subgroup (34 cases, 28.1%). The average and median ages were 41.4 and 46 years, respectively; 75 patients were male and 46 were female. Among the 119 cases, excluding two cases in which the disease side was unknown, the foreign body was located on the left side in 51 cases (42.9%), on the right side in 61 cases (51.3%), and on both sides in 7 cases (5.9%). The foreign bodies included 20 living things (16.5%) and 101 non-living things (83.5%). Hair was the most common foreign body (n = 32; 26.4%), followed by insects (n = 20; 16.5%), sand and pebbles (n = 18; 14.9%), etc. Of the total, 33 cases (27.3%) visited the emergency department outside of business hours, and insects were the most common type of foreign body in these cases (n = 14). Of the 19 cases, except for one case whose onset time is unknown, the event occurred during the nighttime (6:00 pm to 6:00 am of the following day) in 14 cases (73.7%). Extraction of the foreign body was performed without anesthesia in 104 cases (86.0%), under local anesthesia in seven cases (5.8%), and under general anesthesia in 10 cases (8.3%).
In one rare case, a 74-year-old man complaining of an insect having lodged in his right ear while he was asleep was brought to our emergency department in an ambulance. One fly and a number of maggots were observed in the right external auditory canal, and the patients was diagnosed as a case of aural myiasis; the fly was identified as belonging to the Sarcophagidae family.
Extramedullary plasmacytoma is a monoclonal proliferation of plasma cells without bone destruction or intramedullary involvement, being characterized by only soft tissue involvement. Extramedullary plasmacytomas do not present with symptoms of multiple myeloma, but with a mass and accompanying symptoms.
The patient reported herein was a 49-year-old woman who presented to our hospital with the chief complaint of discomfort while swallowing. She was identified as having a mass (maximum diameter 27 mm) in the posterior wall of the nasopharynx. There was no evidence of extension into surrounding tissues or bone destruction. Therefore, surgery was selected as the initial treatment. There are two possible approaches to the nasopharynx: a transnasal approach under endoscopic guidance and a transoral approach. In this case, the base of the tumor was determined as being caudal to the extension of the hard palate, and transoral videolaryngoscopic surgery (TOVS) was performed as the procedure of choice. Because of the positive deep margins, the patient also received postoperative radiation therapy. No recurrence or metastasis has been observed until now, 3 years since the surgery. It is reported that extramedullary plasmacytoma show transformation into multiple myeloma in 17%–33% of patients; therefore, continued and careful follow-up after local treatment is necessary for early detection of transformation to multiple myeloma.
Dupilumab is a humanized anti-human IL-4/13 receptor monoclonal antibody that has been approved for the treatment of chronic rhinosinusitis with nasal polyps (CRSwNP), bronchial asthma, atopic dermatitis, prurigo nodularis, and chronic spontaneous urticaria that respond insufficiently to existing treatments. Some patients with CRSwNP experience repeated relapses while receiving existing treatments, and for such cases, treatment with dupilumab is promising.
In this study, we compared the changes in the nasal polyps and olfactory dysfunction in 7 patients with CRSwNP who were treated with dupilumab. Some of the patients showed improvement of the olfactory dysfunction, and in all cases, the improvement was greater than that achieved with systemic steroid therapy. This difference in the response to the two treatments could possibly be explained by the presence of glucocorticoid receptor beta (GRβ) in the lesions, which attenuates the effects of systemic steroids, but does not the actions of dupilumab.
Dupilumab is a drug that primarily targets nasal polyps and nasal congestion rather than olfactory dysfunction, which limits its approved indications in cases of CRSwNP. As a result, some patients whose symptoms cannot be effectively managed with existing therapies might not meet the criteria for use of the drug, even if they could benefit from dupilumab. In such cases, exploring alternative target diseases such as bronchial asthma could be a viable approach. To facilitate this, it is advisable to collaborate with physicians from other specialties to discuss the potential indications of the drug.
Schwannoma is a relatively common disease of the head and neck region, but its occurrence in the palate is rather rare. We report a case of a soft palate schwannoma that enlarged over time to occupy the oral cavity. A 39-year-old man visited a nearby clinic with the chief complaints of pharyngeal pain and dysphagia. Examination revealed a submucosal tumor in the middle of the soft palate. CT and MRI revealed findings suggestive of a schwannoma measuring 42 mm in diameter. But at the time of the patient’s visit, the tumor was growing rapidly. Therefore, a biopsy was performed, which confirmed the diagnosis of a benign schwannoma. However, as there was still a possibility of malignancy clinically, based on the growth rate of the tumor, the patient was referred to our hospital. We immediately performed surgery with total excision of the tumor. Histopathological examination revealed presence of the characteristic Antoni A and Antoni B areas, and the Ki-67 labelling index was 6.7%, confirming the diagnosis of benign schwannoma. The surgical site healed well and the patient was able to resume oral intake. There were no signs of tumor recurrence until the follow-up examination at 1 year after the surgery.
In the case of intraoral schwannoma, there is a possibility of sudden enlargement when the nerve capsule is damaged for some reason. Therefore, preoperative biopsy and incision should be approached with caution. In addition, when malignant transformation is clinically suspected, sufficient preoperative consideration and informed consent from the patients are required to select whether to prioritize functional preservation or complete resection.
We present the case of a 73-year-old male patient with relapsing polychondritis. The patient presented with only the symptom of cough. FDG-PET showed accumulation in the trachea, thyroid cartilage, and ribs. We were not able to obtain a diagnosis of relapsing polychondritis by auricular cartilage biopsy, but were able to confirm the diagnosis by thyroid cartilage biopsy. Treatment could be started early after diagnosis, and the early intervention has prevented any worsening of the symptom. Early diagnosis and treatment are important in case of relapsing polychondritis, because the airway symptoms can be fatal. We think that biopsy from an appropriate site, taking into account the degree of invasiveness, is useful for early diagnosis.
Klebsiella pneumoniae (K. pneumoniae), an oral and intestinal commensal bacterium, is a major cause of community-acquired infections, such as respiratory, urinary, and intra-abdominal infections. In recent years, invasive pathologies caused by hypervirulent K. pneumoniae, a variant that has increasingly been recognized as causing severe and life-threatening infections, including liver abscesses and systemic bloodstream dissemination/sepsis, have been reported.
A 22-year-old Southeast Asian woman was referred to our department with a left cervical swelling that she had noticed about 1 week earlier. Physical examination revealed a large swelling with overlying erythema in the left upper neck that was tender to palpation. Computed tomography (CT) showed a space-occupying lesion with an internal hypodense area in the left neck, suggestive of a cervical lymph node abscess.
Antibiotic therapy was initiated, percutaneous puncture drainage was performed, and K. pneumoniae colonies that tested positive for the string test were detected on pus culture. After three puncture drainages, followed by an incisional drainage, the abscess/neck swelling gradually improved. Hypervirulent K. pneumoniae produces excessive amounts of capsular substances and is associated with a hypermucoviscous phenotype that tests positive in the string test. In cases of a suspected hypervirulent, hypermucoviscous K. pneumoniae-induced abscess, early puncture or incisional drainage should be aggressively considered.
Tonsillectomy is a surgery commonly performed for treating repetitive tonsillitis. Tonsillectomy changes the shape and characteristics of the pharynx. It could also alter the resonating characteristics of the pharynx and affect the voice of the patient. We examined the voice changes in patients undergoing tonsillectomy.
A total of 33 adult patients who underwent tonsillectomy at our institution between July 2020 and December 2021 were included in the study. The subjects consisted of 16 males and 17 females, with a mean age of 33.7 ± 10.7 (mean ± SD) years. We measured the maximum phonation time, acoustic analysis values (Jitter%, Shimmer%, NHR), speaking fundamental frequency, and vocal range, first four formants (F1 to F4) in the sustained vowel /a/, and VHI. We examined whether there were any changes at 3 months after the surgery as compared with the findings prior to the surgery.
The shimmer% and NHR decreased significantly after surgery as compared with the observations prior to surgery. In the female patients, the speaking fundamental frequency tended to increase and the F2 significantly increased after the surgery as compared with that prior to surgery.
Removal of the swollen tonsils reduced the resistance of the supraglottic space and resulted in a lower laryngeal adduction force required for phonation. The decrease of the shimmer% and NHR might be attributed to reduction of the turbulence in the supraglottic space. The change in the shape of the pharynx caused the changes of the speaking fundamental frequency and formants. The changes were thought to be influenced by the size of the patient’s pharyngeal cavity, the volume of the tonsils, and the scarring of the pharyngeal mucosa. It is important to inform patients before the operation about the possibility of changes in their voice after the operation.