Yumi Ohta

Diagnosis of the subtype and affected ear of benign paroxysmal positional vertigo using a questionnaire

Abstract Conclusion: The prediction of subtype and the affected ear of benign paroxysmal positional vertigo (BPPV) derived from the answers to our questionnaire can support the definitive diagnosis of BPPV. Objectives: We examined to what extent the diagnosis of subtype and the affected ear of BPPV judged from answers to a questionnaire agreed with the diagnosis decided by the results of the positional nystagmus test. Methods: We asked the following questions: ‘What kind of head movements induce vertigo?’ and ‘How long does the vertigo continue?’. As for the affected ear, we asked which ear was lower during stronger vertigo when induced in a supine position or during sleep. Results: The percentages of correct diagnosis speculated by the combined answers were 69% in posterior canal-type BPPV, 48% in BPPV with geotropic nystagmus, and 39% in BPPV with apogeotropic nystagmus. The percentage of correct diagnoses of the affected ear was more than 80%.

Psychological condition in patients with intractable Meniere's disease

Abstract Conclusions: Physicians should consider additional treatment strategies for Menieres disease patients with a long history of disease and hearing loss in the secondary affected ear and also provide psychological support regarding future progressive bilateral hearing loss. Objectives: To treat intractable Menieres disease patients effectively, we need to understand the psychological condition of each patient. We examined the state of neurosis and depression in patients and correlated this with demographic and background information. Methods: Between 1998 and 2009, we enrolled 207 patients with intractable Menieres disease in this prospective study. We used the Cornell Medical Index and the Self-rating Depression Scale to evaluate their psychological condition. We also obtained demographic and background information relating to sex, age, duration of disease, vertigo frequency, hearing level in bilateral sides, and plasma vasopressin level. Results: Neurosis and depression was diagnosed in 40.1% and 60.4%, respectively, of patients with intractable Menieres disease. Our results showed that surgical treatment significantly improved vertigo and hearing ability in patients with no psychological symptoms compared with those exhibiting psychological symptoms. Patients with a longer duration and worse hearing level in the secondary affected ear had a significantly higher incidence of mental illness than those with a shorter duration and better level of hearing.

Stapes surgery in Japanese patients with osteogenesis imperfecta.

Osteogenesis imperfecta (OI) is a heterogenous connective tissue disorder. The classical triad of symptoms involves a conductive and/or sensorineural hearing impairment together with a tendency to spontaneous bone fractures and blue sclerae. Between 1993-2004, primary stapes surgery was performed on 14 ears of 11 OI patients who presented with conductive and/or mixed hearing loss. Pathological findings included atrophy and/or fractures of the stapedial crura in combination with thickening and fixation of the stapes footplate and hypervascularity of the promontory mucosa. All the patients with stapes surgery had significant hearing gain and bone conduction thresholds did not differ significantly in any of the cases; the mean postoperative air-bone gap at the main speech frequency range was within 10 dB in 13/14 (93%) and within 20 dB in 14/14 (100%). Hearing results following stapes surgery in patients with otosclerosis during the same time interval (n = 132) did not differ significantly. These data indicate that stapes surgery in OI can be performed safely with comparable functional predictability as in otosclerosis.

Chronic otitis media with cholesteatoma with canal fistula and bone conduction threshold after tympanoplasty with mastoidectomy.

Objective To understand the third mobile window effect of chronic otitis media with cholesteatoma with inner ear fistula on the bone conduction threshold, we examined changes in the bone conduction audiogram after tympanoplasty with mastoidectomy for chronic otitis media with cholesteatoma with canal fistula. Study Design Retrospective case review. Setting Tertiary referral center. Patients According to the intraoperative classification of Dornhoffer and Milewski, we focused especially on Type IIa (anatomic bony fistula with no perilymph leak). We checked the bone conduction threshold at least 3 times: just before, just after, and 6 months after surgery in 20 ears with Type IIa lateral semicircular canal fistula. Intervention Tympanoplasty with mastoidectomy. Main Outcome Measure Bone conduction thresholds before and after tympanoplasty with mastoidectomy. Results Compared with the preoperative bone conduction threshold, 6 cases were better, 12 cases were unchanged, and 2 cases were worse within the first postoperative week. Finally, 1 case was better, 15 cases were unchanged, and 4 cases were worse at the sixth postoperative month. Patients with a better bone conduction threshold in the low-tone frequencies immediately after surgery had a tendency to show no preoperative fistula symptoms. Postoperative spontaneous nystagmus had a tendency to be observed in patients with a worse bone conduction threshold in the high-tone frequencies. Conclusion The better bone conduction threshold at low-tone frequencies immediately after tympanoplasty with mastoidectomy and no preoperative fistula symptoms might imply the third mobile window theory. The worse bone conduction threshold in high-tone frequencies with spontaneous nystagmus after surgery might indicate inner ear damage.

Does endolymphatic sac decompression surgery prevent bilateral development of unilateral Ménière disease

The aim of the study was to elucidate whether endolymphatic sac decompression surgery (ESDS) has the potential to prevent unilateral Ménière disease (MD) from becoming bilateral.

POU4F3 mutation screening in Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis identified novel variants associated with autosomal dominant hearing loss

A variant in a transcription factor gene, POU4F3, is responsible for autosomal dominant nonsyndromic hereditary hearing loss, DFNA15. To date, 14 variants, including a whole deletion of POU4F3, have been reported to cause HL in various ethnic groups. In the present study, genetic screening for POU4F3 variants was carried out for a large series of Japanese hearing loss (HL) patients to clarify the prevalence and clinical characteristics of DFNA15 in the Japanese population. Massively parallel DNA sequencing of 68 target candidate genes was utilized in 2,549 unrelated Japanese HL patients (probands) to identify genomic variations responsible for HL. The detailed clinical features in patients with POU4F3 variants were collected from medical charts and analyzed. Novel 12 POU4F3 likely pathogenic variants (six missense variants, three frameshift variants, and three nonsense variants) were successfully identified in 15 probands (2.5%) among 602 families exhibiting autosomal dominant HL, whereas no variants were detected in the other 1,947 probands with autosomal recessive or inheritance pattern unknown HL. To obtain the audiovestibular configuration of the patients harboring POU4F3 variants, we collected audiograms and vestibular symptoms of the probands and their affected family members. Audiovestibular phenotypes in a total of 24 individuals from the 15 families possessing variants were characterized by progressive HL, with a large variation in the onset age and severity with or without vestibular symptoms observed. Pure-tone audiograms indicated the most prevalent configuration as mid-frequency HL type followed by high-frequency HL type, with asymmetry observed in approximately 20% of affected individuals. Analysis of the relationship between age and pure-tone average suggested that individuals with truncating variants showed earlier onset and slower progression of HL than did those with non-truncating variants. The present study showed that variants in POU4F3 were a common cause of autosomal dominant HL.

Intercellular Communication between Keratinocytes and Fibroblasts Induces Local Osteoclast Differentiation: a Mechanism Underlying Cholesteatoma-Induced Bone Destruction

ABSTRACT Bone homeostasis is maintained by a balance in activity between bone-resorbing osteoclasts and bone-forming osteoblasts. Shifting the balance toward bone resorption causes osteolytic bone diseases such as rheumatoid arthritis and periodontitis. Osteoclast differentiation is regulated by receptor activator of nuclear factor κB ligand (RANKL), which, under some pathological conditions, is produced by T and B lymphocytes and synoviocytes. However, the mechanism underlying bone destruction in other diseases is little understood. Bone destruction caused by cholesteatoma, an epidermal cyst in the middle ear resulting from hyperproliferation of keratinizing squamous epithelium, can lead to lethal complications. In this study, we succeeded in generating a model for cholesteatoma, epidermal cyst-like tissue, which has the potential for inducing osteoclastogenesis in mice. Furthermore, an in vitro coculture system composed of keratinocytes, fibroblasts, and osteoclast precursors was used to demonstrate that keratinocytes stimulate osteoclast differentiation through the induction of RANKL in fibroblasts. Thus, this study demonstrates that intercellular communication between keratinocytes and fibroblasts is involved in the differentiation and function of osteoclasts, which may provide the molecular basis of a new therapeutic strategy for cholesteatoma-induced bone destruction.

Effects of endolymphatic sac decompression surgery on vertigo and hearing in patients with bilateral Ménière's disease.

Objective The aim of the present study, which involved a 2-year observation period and a nonsurgical control group, was to determine whether endolymphatic sac decompression surgery (ESDS) has the potential to prevent the progression of vertigo and hearing loss in patients with intractable bilateral Ménière’s disease (MD). Study Design Prospective case-control study. Setting Tertiary referral center. Methods Between 1996 and 2008, we subjected 67 patients with intractable bilateral MD to ESDS and local corticosteroid treatment. Another 34 patients with intractable bilateral MD who declined ESDS were treated with the best available medical therapies. All of the patients underwent regular follow-up examinations for at least 2 years. Results Vertigo was resolved in 22 of 34 patients in the non-ESDS group and 60 of 67 patients in the ESDS group (p = 0.055, Fisher’s exact test). Of the 24 patients in the non-ESDS group and 55 patients in the ESDS group in whom the ipsilateral ear (the treated ear) exhibited worse hearing function than the contralateral ear, the hearing level of the former ear was preserved in 13 and 52 patients, respectively (p = 0.007, Fisher’s exact test). Of the 10 patients in the nonsurgical group and 12 patients in the surgical group in whom the ipsilateral ear exhibited better hearing function than the contralateral ear, the hearing level of the former ear was preserved in 2 and 11 patients, respectively (p = 0.035, Fisher’s exact test). Conclusion The present findings suggest that ESDS combined with local corticosteroid treatment can control progressive hearing loss in both ears in patients with bilateral MD at least during the first 2 postoperative years.

Unilateral posterior canal-plugging surgery for intractable bilateral posterior canal-type benign paroxysmal positional vertigo

OBJECTIVE To investigate the effectiveness of unilateral posterior semicircular canal (PSCC)-plugging surgery for patients with intractable bilateral PSCC-type benign paroxysmal positional vertigo (P-BPPV). METHODS From July 2011 to December 2015, we diagnosed 136 patients with P-BPPV. Of these, 3 patients had bilateral P-BPPV, and in 2 of the 3, the condition had been refractory to conservative treatment for more than 1 year. We planned a staged PSCC-plugging surgery for these 2 patients; initially one side was treated, and the contralateral side was treated 6 months later. RESULTS After the first surgery, both patients experienced improvement in symptoms of vertigo and nystagmus on the operated side and no change on the non-operated side. Patients underwent the Epley maneuver for the non-operated side. In one case, the non-operated side was cured. In the other case, although the P-BPPV was not completely resolved, the patient was satisfied with the result of unilateral surgery because he was now able to turn in bed to the operated side without vertigo. Before surgery, he had experienced vertigo when turning even slightly in bed. CONCLUSION We propose that even unilateral PSCC-plugging surgery is effective for some patients with intractable bilateral P-BPPV.

Gadolinium contrast-enhanced MRI reveals cystic lateral semicircular canal contents

Abstract Conclusion: Contrast-enhanced magnetic resonance imaging (MRI) reveals variations in the endolymphatic morphology of the cystic lateral semicircular canal (CLSC) that correlate with inner ear function. This report is the first to suggest a relationship between the morphology and function of this common inner ear malformation in clinical cases. Objectives: This study investigated the radiological and functional findings of a common inner ear malformation using computed tomography (CT), gadolinium contrast-enhanced magnetic resonance imaging (MRI), caloric testing, and cervical and ocular vestibular evoked myogenic potential (VEMP) testing. Method: Four ears in three patients who were radiologically diagnosed with a CLSC and a normal cochlea on high-resolution CT and contrast-enhanced MRI were included. Semicircular canal and vestibular functions were analyzed using the caloric test and cervical and ocular VEMP testing. Results: Unilateral and bilateral cystic canals were found in two and one patients, respectively. In the first patient, the malformed vestibule and cystic space were separate on imaging, and perilymph filled the cystic space. The functional test results were normal. In the second patient, endolymph filled both cystic spaces, and the functional responses were poor. In the third patient, endolymph filled the cystic space, and the ear did not respond during functional testing.