Speech perception was tested using CI2004 and 57S monosyllable lists in 50 patients wearing cochlear implants, to examine the effects of the characteristics of these two tests on patients’ scores. The mean score was significantly higher for 57S (66.9%) than for CI2004 (54.5%). When scores for monosyllables that were common in the two tests were extracted and compared, no significant difference was observed between the two tests, with mean scores of 60.7% and 66.0% for CI2004 and 57S, respectively. In terms of monosyllables that were not common, a significant difference between mean scores was observed: 37.6% and 70.4% for CI2004 and 57S, respectively. This indicates that the types of monosyllables adopted in the tests affect the scores, preventing direct comparison of scores obtained using different word lists. In addition, multiple common kana monosyllables lacked correlation, suggesting distortions in the speech data. Considering the lack of a standardized assessment for patients with cochlear implants in Japan, the development of guidelines for efficacy assessment by the societies would be preferable.

The second-generation acoustic reflectometer (spectral gradient acoustic reflectometer; SG-AR) can be used as an alternative method for detecting middle-ear fluid. This study aimed to assess the applicability of the second-generation EarCheck^® for diagnosing acute otitis media (AOM) and otitis media with effusion (OME), as compared to the first-generation acoustic otoscope. A total of 167 children (178 ears) aged ≦10 years, were examined using two different acoustic reflectometers. SG-AR level was normal 1.14 ± 0.35 (mean ± SD), OME 2.88 ± 1.15, AOM 2.33 ± 1.15, and significant difference (p < 0.001) was observed when comparing OME with normal and AOM with normal. Using SG-AR level ≧2 as a criterion for an abnormal finding, the sensitivity and specificity for diagnosing OME were 93% and 86%, respectively. Using the same criterion, the sensitivity and specificity for diagnosing AOM were 75% and 86%, respectively. The SG-AR does not require a seal in the external auditory canal; so, reading can be obtained from an uncooperative child. SG-AR use cannot replace otoscopy and pneumatic otoscopy. Moreover, SG-AR is not useful in differentiating AOM from OME. It is a diagnostic aid with a performance similar to that of tympanometry, and the two technologies complement each other.

This paper examines the efficacy of using biocompatible ceramic bone graft substitutes, APACERAM^® P and T, in the repair of a lateralized tympanic membrane, when the normal ossicular chain is missing. Earlier, we would treat all patients with a lateralized tympanic membrane by repositioning the membrane to its normal position. This procedure was successful in patients with a normal ossicular chain. However, our results were consistently poor in patients without a malleus handle.

We currently treat patients with a normal ossicular chain with a type I tympanoplasty, which involves creating a new tympanic membrane underlying the malleus handle. In contrast, patients without a malleus handle and a lateralized tympanic membrane are treated with APACERAM^® P or T, where a cartilage is attached to lengthen the columella.

We examined 11 ears of 11 patients who underwent different surgical procedures for a lateralized tympanic membrane from April 2014 at Yamagata University Hospital and compared the surgical results. Three out of the 11 patients had a normal ossicular chain and were treated with a type I tympanoplasty; 1 patient without a normal ossicular chain had a new tympanic membrane constructed using the incus as a columella at the normal position; and 7 patients without a malleus handle and a lateralized tympanic membrane were treated with APACERAM^® P or T.

All three patients with a normal ossicular chain had a postoperative air-bone gap within 15 dB. The patient in whom the incus was used as a columella had a relateralized tympanic membrane and an induced large air-bone gap of over 50 dB. In the remaining seven patients, who were all treated with APACERAM^® P or T, the surgery was classified as successful, based on the achievement of at least one out of the following three postoperative success criteria of reduced air-bone gap, hearing gain, or increased hearing level as described by the JOS. Specifically, 3 out of 7 patients had a postoperative bone-air gap of 15 dB or lower; 5 out of 7 had a hearing gain of more than 15 dB; and 2 out of 7 had a postoperative hearing level of 30 dB or lower. In conclusion, APACERAM^® P and T used with cartilage were shown to be effective materials for the treatment of a lateralized tympanic membrane in patients without a normal ossicular chain.

The purpose of this study was to evaluate the postoperative outcome of tympanoplasty using a survival analysis and to identify prognostic factors which influence the clinical results using a multivariate Cox proportional hazards regression analysis based on the survival analysis.

This retrospective study included 117 cases of pars tensa cholesteatoma treated with a one stage canal wall reconstruction tympanoplasty from 1991 to 2017. The follow-up period of the patients was more than one year, and the median follow-up period was 7 years.

Disease-free cases were defined as the cases in which patients did not undergo unplanned 2^nd stage operations and also maintained a postoperative air-bone gap within 20 dB.

The results were as follows: the five year survival rates of disease-free cases were 91.0% in the type of ossicular chain repair “On”; 79.2% in the types “Osi,” “Osm,” and “Ost”; and 58.8% in the types “Ofm” and “Oft,” and the 10-year survival rates were 65.0% in the types “Osi,” “Osm,” and “Ost” and 50.6% in the types “Ofm” and “Oft.”

The condition of the reconstructed middle ear in which ventilation and drainage functions are maintained through the Eustachian tube was defined as the “health of the reconstructed middle ear.” In a multivariate analysis of factors affecting the reconstructed middle ear health, adhesive otitis (AO) was a significantly promoting factor while “cutting tensor tympani tendon” was a significantly suppressing factor.

Based on the abovementioned results, it was considered important to secure wide ventilation and drainage space between the mesotympanum and epitympanum even after cutting the tensor tympani tendon when treating pers tensa cholesteatoma, especially for the type with AO complication.

Congenital cholesteatoma, a cholesteatoma that occurs in the middle ear without attachment to the tympanic membrane, is typically diagnosed at birth. We report three cases of spontaneous resolution of congenital cholesteatoma. We encountered 16 cases of congenital cholesteatoma (16 ears) between January 2010 and January 2019. We investigated the characteristics of congenital cholesteatoma showing spontaneous resolution and diagnosed this lesion at an early age (1–2 years) in three patients who showed no abnormalities of the ossicular chain. Early surgical treatment before disease progression is recommended for the treatment of congenital cholesteatoma. However, children (1–2 years) with no abnormalities of the ossicular chain can be observed with close monitoring of disease progression because this lesion is known to spontaneously resolve in a few cases. In addition, all our patients were diagnosed incidentally. It is reasonable to conclude that the cholesteatoma could have spontaneously resolved before diagnosis in a few patients.

Bezold’s abscess is a neck abscess that was first described by Friedrich Bezold as a complication of mastoiditis in 1881. Since the advent of antibiotics, cases of Bezold’s abscess caused by suppurative otitis media have diminished significantly and those occurring as a rare complication of cholesteatoma and chronic otitis media have increased. We present a rare case of left ear canal stenosis complicated by middle ear cholesteatoma developing into Bezold’s abscess. A 62-year-old man visited the emergency department of our hospital with complaints of fever and anorexia. His bilateral external ear canals were almost obliterated, resulting from chronic external otitis. The left postauricular region and posterior part of the neck were markedly swollen. Based on the physical examination findings and computed tomography scan of the temporal bone and neck, he was diagnosed with Bezold’s abscess. After admission, the patient underwent emergent drainage of the left neck abscess through a neck incision, and was treated with intravenous antibiotics. After approximately a month, he underwent left radical tympano-mastoidectomy and meatopasty. The left external ear canal showed bony stenosis, and cholesteatoma was found filling the interior side of his left canal, tympanic cavity, antrum, and mastoid tip. There was no sign of recurrence during the 80-month follow-up period.

Cogan syndrome is a rare disease characterized by non-syphilitic keratitis, vestibular dysfunction, and sensorineural hearing loss. It is a type of vasculitis syndrome and is considered to have an autoimmune origin. Moreover, systemic vasculitis complicates approximately 10%–20% of cases. We report a case of Cogan syndrome in a patient undergoing treatment for eosinophilic otitis media.

A 64-year-old woman, diagnosed with bronchial asthma in her 20s, was dependent on prednisolone (PSL) therapy ever since. Mepolizumab treatment was also started at the age of 62 years.

At the age of 50 years, she was diagnosed with eosinophilic otitis media and was subsequently referred to our department at the age of 62 years. Despite the absence of otorrhea during the course of the disease, the bone conduction threshold persistently increased, and despite PSL administration, her hearing loss progressed. She developed scleritis at the age of 63 years and was treated for this condition at another hospital.

Because there was a notable time lapse between the development of the inner ear disorder and the onset of ocular symptoms, a diagnosis of atypical Cogan syndrome was made. The clinical course of this case was documented, and the relationship between Cogan syndrome and vasculitis was examined.

A 30-year-old pregnant woman complained of left otorrhea and otalgia for almost one month. She was diagnosed with left acute otitis media and treated with peroral cefditoren pivoxil (CDTR-PI) by a nearby doctor. At one point, her condition got better, but it became worse again. At 36 weeks of pregnancy, she presented with disturbance of consciousness, headache, nausea, and vomiting and was admitted to our hospital as an emergency patient. Meningeal irritation sign was positive in the patient, and her left tympanic membrane showed swelling and severe otorrhea. She was diagnosed with bacterial meningitis secondary to left acute otitis media. Because she was in her late pregnancy, we operated on her for left mastoidectomy and myringotomy; ventilation tube induction was conducted secondary to an urgent caesarean section. After operations, she received the administration of meropenem (MEPM), vancomycin (VCM), and corticosteroids. A few days later, bacteria test of the spinal fluid indicated mucoid type Streptococcus pneumoniae involvement, so we changed the antibiotics to penicillin-G (PCG) from MEPM. After a 14-day intravenous antimicrobial administration in total, her meningitis healed without leaving neurologic aftereffects. She and her child were both healthy.

In this case, continuous administration of CDTR-PI in primary care was a concern. Mucoid type Streptococcus pneumoniae is mostly resistant against cephem antibiotics in Japan. It is important for general otorhinolaryngologists, who take primary care of patients with otitis media, to understand about acute otitis media occurring due to mucoid type Streptococcus pneumoniae.

Because the chorda tympani nerve (CTN) runs through the tympanic cavity, ear surgeons often encounter the CTN during middle ear surgery, which can cause postoperative taste disturbance. We reported two cases in which the CTN was unintentionally sectioned during surgery, and the stumps of the injured nerve were re-adapted to each other, resulting in recovery of taste function. In case 1, tympanoplasty was performed in the left ear with tympanosclerosis and the right ear with cholesteatoma, and the CTN was sectioned once during surgery on both sides. The stumps were then re-adapted, and both symptoms and findings of electrogustometry (EGM) improved after the surgeries. In case 2, after stapes surgery for right otosclerosis, a piston repeatedly fell off two times in 9 years, and stapedotomy was performed three times. The right CTN was sectioned during the initial surgery, and re-adaptation was performed. In the second and third revision surgeries, regeneration of the right CTN was microscopically confirmed, and both findings of EGM and symptoms improved. Even if the CTN is unintentionally sectioned during surgery, re-adaptation of the nerve ends may allow regeneration of the chorda tympani nerve and restoration of taste function.

Facial nerve paralysis rarely occurs because of any facial nerve tumor; therefore, no treatment guidelines have been established. Here, we report a rare case of intratemporal facial neurofibroma, which was treated with transmastoid decompression surgery. A 47-year-old woman presented with left facial paralysis. Examination did not reveal vertigo or hearing loss. Although high-dose corticosteroids were administered as the primary treatment, her facial movement did not improve for 2 months. Computed tomography showed enlargement of the mastoid segment in the facial canal, and magnetic resonance imaging showed a relevant gadolinium enhancement, indicating facial nerve schwannoma. Total resection followed by nerve grafting would typically be recommended; however, transmastoid decompression and biopsy were performed in this case. The histopathological diagnosis was neurofibroma. After 1.5 years postoperatively, her facial paralysis had recovered to House–Brackmann grade 2. This case suggested that surgical decompression could be an alternative to total resection with nerve grafting to treat facial nerve paralysis due to benign facial nerve tumors.

We monitored the progression of hearing loss in a juvenile case with MYO15A gene variants for over 6 years. The patient was a 13-year-old girl who presented with hearing loss in February 2020. Except for her father, who had 35-dB c5-dip at 4000 Hz in the left ear, no other family members had hearing loss. She did not undergo newborn hearing screening. A high-tone sensorineural hearing loss was incidentally identified in a medical checkup conducted after elementary school entrance at the age of 6 years. The hearing loss advanced gradually, and she experienced severe hearing loss in April 2019. On genetic testing, we identified the c.9415_9418del and c.10263C>G variants of the MYO15A gene. MYO15A gene mutations cause non-syndromic sensorineural hearing loss with an autosomal recessive inheritance pattern. Many previously reported cases of this gene mutation showed congenital hearing loss or high-tone hearing loss, whereas some showed progressive sensorineural hearing loss. In our case, a hearing aid and digital support system effectively managed the condition.

We report a case of presumed inner ear hemorrhage and acute sensorineural hearing loss, which are extremely rare complications of multiple myeloma. A 60-year-old man complained of sudden hearing loss in the right ear, tinnitus, and dizziness. Pure tone audiometry showed moderate sensorineural hearing loss, which was not recovered from after steroid therapy. Two months after onset, hearing loss recurred in both ears. Hemorrhagic diathesis was also observed in the brain, oral and nasal mucosa during the same period. Blood analysis and bone marrow biopsy resulted in the diagnosis of multiple myeloma. The right ear presented profound deafness and showed no recovery after chemotherapy. In contrast, the left ear presented worsening of hearing loss, which gradually improved to normal levels in 4 months. MRI showed a high signal intensity on the non-enhanced T1-weighted image in bilateral inner ears and no enhancement after gadolinium injection, suggesting inner ear hemorrhage. Inner ear hemorrhage was most likely due to hemorrhagic diathesis and blood hyperviscosity.

Skull base osteomyelitis, developing from malignant external otitis or acute otitis media, is occasionally fatal. Although long-term antibiotics are needed, indicators for the termination of antibiotic therapy have not been established. In this study, computed tomography (CT) of four patients with skull base osteomyelitis revealed bony destruction of the skull base. All patients received long-term intravenous and oral antimicrobials. The bony destruction gradually improved in all cases. The antimicrobial treatment was then stopped, and there has been no recurrence to date. Thus, improvement of skull base bony destruction could serve as an indicator for the termination of antibiotic therapy.

Tinnitus is a common condition with a prevalence of 20%, and it is pulsatile in approximately 4% of tinnitus patients. Pulsatile tinnitus may be associated with vascular wall anomalies of major vessels in the temporal bone, such as the sigmoid sinus, jugular bulb, and internal carotid artery. High-resolution computed tomography detects sigmoid sinus wall anomalies, including sigmoid sinus diverticulum and dehiscence, in approximately 20% of patients with pulsatile tinnitus. Herein, we report a case of sigmoid sinus wall dehiscence treated with sigmoid sinus wall reconstruction.

A 49-year-old woman presented with a complaint of right pulsatile tinnitus. The total score of the tinnitus handicap inventory (THI) was 100, indicating severe psychological distress. The baseline of the acoustic reflex testing showed a waveform synchronized with the rhythm of her heartbeat. Computed tomography revealed an ipsilaterally prominent sigmoid sinus and the absence of a normal thin layer of the mastoid cortex over the sigmoid sinus. The patient underwent mastoidectomy via the transtemporal approach, which revealed a bony defect of the transverse-sigmoid sinus. The sigmoid sinus wall was reconstructed using the temporal fascia and bone pate. Her symptoms of pulsatile tinnitus improved significantly after the surgery, and the waveform on the acoustic reflex testing disappeared. The total THI score declined to 30, one month postoperatively. The patient remained free of recurrence of pulsatile tinnitus for 6 months postoperatively.

Although this is the first report of a patient with sigmoid sinus wall dehiscence treated with sigmoid sinus wall reconstruction in Japan, a high success rate of wall reconstruction technique in terms of the resolution of pulsatile tinnitus has been reported in other countries. Otolaryngologists should be aware of the sigmoid sinus anomalies as one of the etiologies of pulsatile tinnitus.

Subtotal petrosectomy, with obliteration of the middle ear and mastoid and closure of the external auditory canal, is indicated in complicated and recurrent diseases, primarily those of the middle ear and mastoid, which do not extend deep into the petrous apex or the internal auditory canal. The indications for this procedure are recurrent infection such as chronic otitis media, middle ear cholesteatoma, and osteoradionecrosis, and middle-ear tumors such as tympanomastoid paraganglioma and facial nerve schwannoma. This procedure is common in Europe and the United States of America. However, it is rarely performed in Japan, and only a few cases have been reported. This paper reports a case of a semicircular canal fistula with a cavity problem that was treated by subtotal petrosectomy.

A 76-year-old female patient underwent radical mastoidectomy for middle-ear cholesteatoma 25 years ago and had chronic inflammation of the open cavity after the surgery. A traumatic semicircular canal fistula occurred while she was receiving a local treatment for the cavity problem, and it caused severe vertigo. She was transferred to our hospital as fistula closure was needed. However, if the chronic inflammation persists, the fistula could relapse. Further, there was a possibility of the occurrence of otitis interna. Therefore, to eliminate the cavity problem, a subtotal petrosectomy was performed in addition to fistula closure. After the procedure, the vertigo disappeared, and she was relieved from chronic otorrhea. Constant local treatment was no longer needed and her quality of life improved greatly. No complications were reported during a 1-year follow-up.

Subtotal petrosectomy is an effective solution against chronic and recurrent infection in the middle-ear and mastoid, with no realistic chance of reconstruction of the conductive apparatus.

Small cell carcinoma of the temporal bone is extremely rare (only two cases have been reported). A 69-year-old man presented with right-sided facial nerve palsy and narrowing of the ear canal, which was treated with intravenous steroids and antibiotics. However, the facial nerve palsy did not improve, and a temporal bone computed tomography (CT) was performed which led to the suspicion of cholesteatoma otitis media. The patient had suffered from otitis media for many years. On his first visit to our hospital, no ear discharge, otalgia, or dizziness was observed. An audiological examination revealed severe mixed hearing loss on the right side.

Temporal bone CT showed a wide soft tissue shadow, covering the right tympanic cavity, mastoid bone, and ear canal. Bone destruction was observed in the posterior ear wall, posterior cranial fossa, and middle cranial fossa. The regions covered by the shadow displayed isointense signals on both T1-weighted and T2-weighted magnetic resonance imaging (MRI) and high-intensity signals on diffusion-weighted imaging. Contrast-enhanced MRI showed relatively clear enhancement in the above-mentioned regions, and the entire mass was uniformly imaged. On diffusion-weighted imaging, the area of high signal intensity exhibited enhancement.

A biopsy was performed via transcortical mastoidectomy, and the patient was diagnosed with small cell carcinoma. Histological analysis showed diffuse proliferation of small, round, atypical cells. The pathological diagnosis was confirmed by immunohistochemistry, which revealed positivity for synaptophysin, CD56, and CAM5.2, and negativity for cytokeratin-AE1/AE3, leukocyte common antigen, CD3, CD20, CD99, S-100, and thyroid transcription factor-1.

Chemoradiotherapy (CDDP + VP-16 combined with 66 Gy radiotherapy) was administered for localized small-cell lung cancer. Grade 2 enteritis and grade 4 neutropenia occurred but disappeared after supportive therapy. The patient was originally scheduled for 4 courses of chemotherapy but abandoned it after 3 courses. Regarding local findings, swelling of the right ear canal was alleviated, and the tympanic membrane could be identified. The patient experienced nausea and a headache about 3 weeks after discharge from the hospital. He was diagnosed with cancerous meningitis and hydrocephalus via contrast-enhanced head MRI, and his general condition gradually worsened. Unfortunately, he died 6 months after his first visit.