Spasmodic dysphonia (SD) is a focal dystonia affecting the laryngeal muscles, causing involuntary spasms during phonation. First described over 150 years ago, SD is categorized into three types: adductor SD (strained voice due to excessive vocal fold closure), abductor SD (breathy voice due to vocal fold opening), and mixed type. Epidemiological studies in Japan estimate SD prevalence between 0.29 and 7.0 per 100,000, with a higher incidence in females and typical onset in the 30s.

Although the exact etiology remains unclear, neurological factors are implicated. Imaging studies suggest abnormalities in the basal ganglia, thalamus, and sensorimotor cortex, and genetic predisposition is considered significant. Mutations in dystonia-related genes (e.g., DYT1, DYT25) have been linked to SD, suggesting genetic overlap with other dystonias.

Diagnosis is based on clinical history and laryngeal examination, with specific phonetic triggers differentiating SD from other voice disorders such as essential voice tremor and muscle tension dysphonia. Sensory tricks, where voice symptoms temporarily improve with certain maneuvers, are characteristic of SD.

Botulinum toxin injection into the laryngeal muscles is the first-line treatment, offering temporary symptom relief with high efficacy. However, repeated injections are necessary, and some patients experience side effects such as breathiness and swallowing difficulties. Surgical interventions, including selective laryngeal nerve denervation, thyroarytenoid muscle myectomy, and type 2 thyroplasty with titanium bridges, have been explored as alternative treatments. Advances in neuroimaging and genetics continue to provide insights into SD pathophysiology, paving the way for potential novel therapies. Further research is needed to refine treatment strategies and improve long-term outcomes for SD patients.

Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal clonal proliferation of Langerhans cells, which are antigen-presenting cells derived from the bone marrow, along with inflammatory cell infiltration. The disease can present as a solitary lesion or multisystem lesions, reflecting a diverse clinical spectrum. LCH lesions in the head and neck region are relatively common, accounting for approximately 70% of all the cases, and these patients are often evaluated by otolaryngologists.

We present a case report of a pediatric patient with recurrent otorrhea as the primary complaint, who was initially suspected as having cholesteatomatous otitis media. She underwent surgical intervention based on this suspicion, but the intraoperative findings raised concerns of an inflammatory disorder, which led to the performance of a biopsy, which confirmed the diagnosis of LCH. Based on subsequent evaluation, the disease was categorized as showing multisystem involvement with invasion of risk organs. Complete remission was achieved and has been maintained with chemotherapy.

This report highlights the importance of considering LCH in the differential diagnosis of temporal bone lesions and also discusses the clinical manifestations of LCH originating from the temporal bone.

High jugular bulb refers to the condition in which the jugular bulb protrudes in the direction of the head, and cases where high jugular bulb protrudes in a notch-like shape toward the posterior part of the internal auditory canal are labelled as having a jugular bulb diverticulum. Jugular bulb diverticulum is rare, with few published case reports, but almost all published reports are of cases diagnosed after the onset of hearing loss or vertigo. The following mechanisms of inner ear damage caused by a jugular bulb diverticulum have been speculated: (1) secondary endolymphatic hydrops due to compression of the vestibular aqueduct and endolymphatic sac; (2) compression of the cochlear nerve and blood vessels in the internal auditory canal; and (3) direct compression of the cochlea, vestibule, and utricular labyrinthine membrane.

We report the case of a 38-year-old female patient who visited our hospital with the chief complaint of transient ear symptoms associated with postural changes. At first, because the ear symptoms associated with postural changes, it was suspected that the patient had a patulous eustachian tube, but the eustachian tube function was subsequently confirmed as being normal. Eventually, the diagnosis of a high jugular bulb diverticulum was made based on the findings of CT and MRI. We suspect that the ear symptoms were caused by the transmission of venous pressure or intracranial pressure to the endolymph via the vestibular aqueduct.

Reactivation of varicella-zoster virus (VZV) can cause a wide variety of neurological syndromes. We report the case of a 65-year-old man with aseptic meningitis and multiple cranial neuropathies developing as a result of VZV reactivation.

The diagnosis of aseptic meningitis due to VZV reactivation was made based on an elevated value of the V-Index. Although the patient was initiated on treatment with aciclovir and a steroid, palsy of cranial nerves IX and X was found to still persist at 2 months after discharge.

In patients presenting with lower cranial nerve neuropathy, the ideal approach is to exclude central nervous system disease, head and neck tumors, and prior surgery, then submit a blood specimen for determination of the serum VZV antibody titers, and then carefully observe the patient for appearance of a skin rash or of enanthema by daily laryngoscopy to obtain an early diagnosis and ensure start of antiviral drug and high-dose steroid therapy at an early stage of the disease.

Nasopharyngeal carcinoma typically presents with neurological complications, notably trigeminal and abducens nerve palsy. However, facial nerve palsy is a rare occurrence, with only a limited number of reports until date. Herein, we report the case of an 80-year-old male patient with nasopharyngeal carcinoma who first presented with symptoms of facial nerve paralysis. Initially, he presented with right eye discomfort and had received treatment for Bell’s palsy before he was referred to our department. Clinical examination revealed a Yanagihara paralysis score of zero, and otoscopic examination revealed tympanic membrane pulsation with effusion. Imaging examinations confirmed extensive bone destruction and neoplastic infiltration from the nasopharynx to the skull base. Endoscopic examination revealed ulceration around the Eustachian tube, and histopathology confirmed the diagnosis of nasopharyngeal carcinoma (adenocarcinoma). The facial paralysis was hypothesized as having been caused by tumor extension through anatomical structures (Eustachian tube, carotid artery duct, or sinus of Morgagni). This case emphasizes the importance of otological evaluation in the differential diagnosis of patients presenting with facial paralysis.

Mucosal contact point headache is disease characterized by the occurrence of headache and facial pain triggered by nasal mucosal contact. We report the case of a 45-year-old woman who presented to us with the complaints of left-sided headache and a nasal obstruction on the left side. At her first visit to our hospital, we made the diagnosis of allergic rhinitis and prescribed oral medicines and a nasal spray. However, 2 days later, she presented with recurrent left nasal pain. Sinus CT revealed a huge left concha bullosa that was in contact with the nasal septum. We placed gauze impregnated with adrenaline and lidocaine at the nasal contact point, which led to significant resolution of the nasal pain and headache. Therefore, the patient was suspected as having mucosal contact point headache. We performed endoscopic nasal surgery and separated the concha bullosa from the nasal septum to avoid contact between the two. After the surgery, both the headache and facial pain disappeared within a week.

The nasal turbinate mucosa often comes in contact with the nasal septum, but rarely does this trigger a headache. The mechanism by which it triggers a headache only in some cases is unknown. There are few reports of this disease yet in the literature, and it cannot be diagnosed unless the physician has a high index of suspicion.

This disease may be overlooked unless an otolaryngologist recognizes it: it is important to suspect this disease in patients complaining of a headache in whom contact between the nasal mucosa and nasal tubinate is recognized.

In recent years, the prevalence of syphilis has become a social concern, and the number of patients has been increasing throughout Japan. Our hospital is in the center of Osaka, and over the past 6 years, we have diagnosed 10 cases of syphilis at our department. Of the 10 patients, 9 were male and 1 was female, with a median age of 30.5 years. The chief complaints were persistent tonsillitis/pharyngitis in 7 cases, induration of the upper lip in 1 case, and left cervical swelling in 2 cases. One case with a chief complaint of cervical swelling had a suspicious mass lesion in the left oropharynx, which required to be differentiated from malignant lymphoma and oropharyngeal cancer.

Resistance of T. pallidum, the causative bacterium of syphilis, to penicillin has not been observed until date, and syphilis can be completely cured if treated appropriately. However, its progression has irreversible effects on the body. Diagnosis is difficult because of the incubation period and the variety of symptoms. It is important to consider the possibility that commonly encountered symptoms such as tonsillitis/pharyngitis, lymph node swelling, and lip induration may be caused by syphilis, and to perform a screening test when findings specific to syphilis are observed.

This report describes a case of acoustic neuroma in a patient who underwent contralateral cochlear implantation. A 78-year-old female presented with an acoustic neuroma on the left side and was referred to our department for possible cochlear implantation. She had moderate-to-severe bilateral hearing loss. She had experienced idiopathic sudden sensorineural hearing loss in her right ear over 20 years ago, and her left ear’s hearing level deteriorated gradually following gamma knife therapy for the neuroma. Initial examination showed pure tone audiometry average thresholds of 78.3 dB and 66.7 dB on the right and left ear, respectively, with 25% and 35% speech discrimination rates, respectively. Using bilateral hearing aids, her speech discrimination rate was 45%. Preoperative computed tomography (CT) and magnetic resonance imaging (MRI) scans were unremarkable, except for acoustic neuroma. At 79, she underwent right cochlear implantation using a Cochlear^® CI632 implant with full insertion. Postoperatively, she experienced short-term dizziness, but otherwise, her recovery was uneventful. Two years post-implantation, her implantation threshold was 36.7 dB, and her speech discrimination rates were 76% for words and 95% for sentences using CI-2004, the Japanese speech discrimination test. MRI could detect the tumor without being influenced by the artifacts from the implant.

Contralateral cochlear implantation may be recommended in cases of hemilateral acoustic neuroma with bilateral severe to profound hearing loss, even if the contralateral hearing loss has been longstanding.

Pemphigus is an autoimmune bullous disease characterized by lesions on the skin and mucous membranes. Characteristic clinical findings include painful and intractable erosions and ulcers in the oral cavity, but when lesions are not present on the skin but only on the oral mucosa, diagnosis is often difficult. Although laryngeal lesions are believed to be uncommon as the initial manifestation of pemphigus vulgaris, it is necessary to keep in mind the possibility.

We report a rare case of pemphigus vulgaris diagnosed in a patient who initially presented with recurrent laryngeal edema, followed subsequently by the appearance of mucosal lesions. The patient, a 75-year-old man, presented to the otorhinolaryngologist with a 2-month history of pharyngeal pain. Examination revealed only laryngeal edema, which improved after steroid administration. During the subsequent course, however, oral mucosal lesions appeared, which led to the diagnosis of pemphigus and treatment appropriate for this disease, and the patient was successfully treated without becoming severely ill.

In this case, there were no oral mucosal lesions or skin rash at the time of the initial examination, which made the diagnosis difficult. In the case of pemphigus vulgaris of the mucocutaneous type, since findings may initially appear only in the otorhinolaryngological field, it is important to keep in mind not only otorhinolaryngological diseases, but also systemic diseases and to seek dermatology referral for diagnosis and treatment when the disease follows an atypical course.

For case of locally advanced nasopharyngeal carcinoma, the benefit of induction chemotherapy prior to concurrent chemoradiotherapy has been reported. In Japan, cisplatin, fluorouracil, plus docetaxel (TPF) is commonly used for induction chemotherapy in patient with nasopharyngeal carcinoma, but adverse effects such as leukopenia are often a problem. Gemcitabine plus cisplatin (GP) is the standard for induction chemotherapy overseas. However, gemcitabine is not covered by insurance in Japan for head and neck cancer, in spite of its effectiveness. We report the case of a patient with nasopharyngeal carcinoma who was treated with GP under self-paid medical treatment and showed good results of treatment.

The patient was a 36-year-old man of Chinese nationality. Computed tomography (CT) performed in his home country showed a nasopharyngeal tumor, and he was referred to our hospital at his own request, as he wished to receive treatment at our hospital. We diagnosed him as having nasopharyngeal carcinoma (cT3N2M0 Stage III). We proposed administration of induction chemotherapy prior to concurrent chemoradiotherapy to improve the treatment outcome. We presented the patient with both options for induction therapy: TPF, the regimen commonly used in Japan, and GP, which is not covered by insurance in Japan but is standard overseas, and the patient requested GP as he was informed that this treatment was associated with a smaller number of adverse events. Three cycles could be completed without any major adverse effects and concurrent chemoradiotherapy was administered smoothly after induction therapy with GP. One year has passed since the completion of the radiation therapy and there has been no evidence of metastasis or recurrence.

Induction therapy with GP may be easier to complete, with fewer adverse events than induction treatment with TPF.

If gemcitabine were to obtain approval for the treatment of head and neck cancer in Japan, it could become the preferred regimen for induction chemotherapy.