Evaluation of the ear ossicles with photon-counting detector CT.

Recently, computed tomography with photon-counting detector (PCD-CT) has been developed to enable high-resolution imaging at a lower radiation dose. PCD-CT employs a photon-counting detector that can measure the number of incident X-ray photons and their energy. The newly released PCD-CT (NAEOTOM Alpha, Siemens Healthineers, Forchheim, Germany) has been in clinical use at our institution since December 2022. The PCD-CT offers several advantages over current state-of-the-art energy-integrating detector CT (EID-CT). The PCD-CT does not require septa to create a detector channel, while EID-CT does. Therefore, downsizing the anode to achieve higher resolution does not affect the dose efficiency of the PCD-CT. CT is an indispensable modality for evaluating ear ossicles. The ear ossicles and joints are clearly depicted by PCD-CT. In particular, the anterior and posterior legs of the stapes, which are sometimes unclear on conventional CT scans, can be clearly visualized. We present cases of congenital anomalies of the ossicular chain, ossicular chain dislocation, tympanosclerosis, and cholesteatoma in which PCD-CT was useful. This short article reports the usefulness of PCD-CT in the 3D visualization of the ear ossicles.

Cochlear Implantation in the Poorer-Hearing Ear Is a Reasonable Choice.

Choosing the optimal side for cochlear implantation (CI) remains a major challenge because of the lack of evidence. We investigated the choice of the surgery side for CI (i.e., the better- or poorer-hearing ear) in patients with asymmetric hearing. Audiological records of 74 adults with a unilateral hearing aid who had undergone surgery at Okayama University Hospital were reviewed. The definition of 'better-hearing ear' was the aided ear, and the unaided ear was considered the poorer-hearing ear. We performed a multiple regression analysis to identify potential predictors of speech recognition performance after unilateral CI in the patients. Fifty-two patients underwent CI in the poorer-hearing ear. The post-Ci bimodal hearing rate was far higher in the poorer-ear group (77.8% vs. 22.2%). A multivariate analysis revealed that prelingual hearing loss and the patient's age at CI significantly affected the speech recognition outcome (beta coefficients: 24.6 and -0.33, 95% confidence intervals [11.75-37.45] and [-0.58 to -0.09], respectively), but the CI surgery side did not (-6.76, [-14.92-1.39]). Unilateral CI in the poorer-hearing ear may therefore be a reasonable choice for adult patients with postlingual severe hearing loss, providing a greater opportunity for postoperative bimodal hearing.

Revision cochlear implant surgery in adults and children is a safe and effective technique: a retrospective single faculty study in Okayama University Hospital, Japan.

BACKGROUND: Cochlear implant (CI) surgery is a safe surgical technique, although some patients require revision CI surgery. AIMS/OBJECTIVES: This study investigated the cause and underlying reason of revision CI surgery as well as hearing outcomes in a single institution. PATIENTS AND METHODS: This retrospective study evaluated patients who underwent CI surgery between April 2006 to March 2022 (n = 351). Sex, aetiology of hearing loss (HL), age and period from initial CI surgery to reimplantation, cause of revision, and related factors were examined. RESULTS: Twelve patients (8 males, 4 females) received CI reimplantation. The revision surgery rate was 2.59% (3.15% children, 1.69% adults); the period from initial surgery to reoperation was 8.60 ± 6.56 years for 9 children with congenital HL and 15.27 ± 5.72 years for 3 adults with progressive HL. Device failure was the most common cause (n = 8), followed by infections (n = 2), advanced facial irritation symptoms (n = 1), and electrode slip-out (n = 1). Mean preoperative and postoperative CI thresholds were 44.0 ± 9.46 dBnHL and 39.19 ± 8.89 dBnHL (p < .068), respectively. CONCLUSION AND SIGNIFICANCE: Caregiver education, surgical technique advances, flap design, and extensive antibiotic use may decrease the revision surgery rate. The lack of post-revision deterioration of the hearing threshold contributed to well-being in patients with CI.

Detailed clinical features and genotype-phenotype correlation in an OTOF-related hearing loss cohort in Japan.

Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype-phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype-phenotype correlation. Detailed clinical information was available for 64 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.6%) showed a "typical" phenotype; prelingual and severe-to-profound hearing loss. Forty-seven patients (73.4%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85-90% of the patients showed a hearing level of 20-39 dB with cochlear implant and a Categories of Auditory Performance (CAP) scale level 6 or better. Although truncating mutations and p.Arg1939Gln were clearly related to severe phenotype, almost half of the patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.His513Arg, p.Ile1573Thr and p.Glu1910Lys showed "true" auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype-phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype-phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients.

Cochlin-tomoprotein (CTP) detection test identified perilymph leakage preoperatively in revision stapes surgery.

Perilymphatic fistula (PLF) is defined as an abnormal leakage between perilymph from the labyrinth to the middle ear. Symptoms include hearing loss, tinnitus, and vertigo. The standard mode of PLF detection is intraoperative visualization of perilymph leakage and fistula, which ostensibly confirms the existence of PLF. Other possible methods of diagnosis include confirmation of pneumolabyrinth via diagnostic imaging. Recently, a cochlin-tomoprotein (CTP) detection test has been developed that allows definitive diagnosis of PLF-related hearing loss. We report the case of a 45-year-old man who presented with right-sided tinnitus, hearing loss, and dizziness 30 years after stapes surgery. Middle ear lavage was performed after myringotomy. A preoperative diagnosis of PLF was reached using the CTP detection test. Intraoperative observations included a necrotic long process of the incus, displaced wire piston, and fibrous tissue in the oval window. Perilymph leakage was not evident. The oval window was closed with fascia, and vertigo disappeared within 2 weeks postoperatively. When PLF is suspected after stapes surgery, the CTP detection test can be a useful, highly sensitive, and less invasive method for preoperative diagnosis.

Syntactic development in Japanese hearing-impaired children.

OBJECTIVES: This study examined syntactic development of auditory comprehension of sentences in Japanese-speaking school-age children with and without hearing impairment. METHODS: In total, 592 preschool and school-age children (421 normal-hearing and 171 hearing-impaired) were included in this cross-sectional observation study conducted using the Syntactic Processing Test for Aphasia for Japanese language users. Linear regression analysis was used to determine the estimated age at which each syntactic structure was acquired. RESULTS: Acquisition of syntactic structures was observed in hearing-impaired and normal-hearing children. Basic word order sentences of agent-object-verb and the goal benefactive construction were acquired at preschool age (earlier group), whereas reverse word order sentences of object-agent-verb, source benefactive construction, passive voice, and relative clauses were acquired at school age (later group). The results showed that many hearing-impaired children may not acquire Japanese grammatical structures until the age of 12 years. CONCLUSIONS: Adequate screening for language development for school-age hearing-impaired children is required for an effective intervention.

Orbital emphysema causing syncope.

Orbital emphysema is an abnormal condition in which air is present within the orbit. We report a rare case of a 19-year-old man who suffered syncopic attacks caused by sniffles following orbital emphysema as a result of trauma. Treating rhinitis is important in patients with orbital emphysema, and patients with cardiac disorders in addition to those with this condition must be warned about the risks of sniffles, sneezing, or nose blowing.

Satellite tumors surrounding primary pleomorphic adenomas of the parotid gland.

The occasional local recurrence of benign pleomorphic adenoma (PA) has generally been attributed to the vulnerability of the tumor capsule. Although some reports have also noted the presence of satellite tumors associated with PA recurrence, only few reports have focused on this issue. We paid special attention to the satellite lesions apart from the main tumors and discussed their frequency, origin, nature and the ways of treating them. A total of 108 specimens of primary parotid gland PA resected at the Okayama Saiseikai General Hospital from 1988 to 2008 were microscopically reviewed. Four (3.7%) patients displayed a main mass with satellite tumors in a single parotid gland. The immunohistochemical analysis of p53 and Ki-67 index showed no distinct difference between PAs with satellite tumors and those without. Satellite tumors surrounding the main mass of parotid PA is relatively rare. In most cases, such satellite tumors will arise from capsular perforation of the primary tumor cells. Preoperative evaluation to recognize the existence of satellite tumors would be important and capsular dissection should be discouraged. We could not find any evidence suggesting that primary PA with satellite tumors could be more biologically aggressive than those without.