Yuko Kataoka

Ménière's disease in childhood

We report 3 rare cases of Ménières disease in children. In Case 1 and 3, vertigo, hearing loss and tinnitus recovered soon after medical therapy. In Case 2, however, vertigo recurred and the hearing level on the right side markedly deteriorated. The equal-loudness contours on three-dimensional audiogram showed that right-sided aggravated hearing loss fluctuated for 4 years at middle-and low-frequencies despite medication. Finally intratympanic injection of gentamicin sulfate was performed. The patient has had no definitive spell of vertigo after gentamicin therapy. At our department, the incidence of Ménières disease in pediatric patients with vertigo was 2.9%.

Expression of inflammatory mediators in the otitis media induced by Helicobacter pylori antigen in mice

Helicobacter pylori is a Gram‐negative bacterium that is recognized as one of the key factors in gastric diseases such as gastritis, peptic ulcer and gastric cancer. Recent studies have shown relationships between H. pylori and extra‐digestive diseases, and the presence of H. pylori in the middle ear and upper respiratory tract has been reported. However, the role of H. pylori in middle ear disease remains unclear. The present study demonstrated that H. pylori whole‐cell protein directly induces macrophage migration inhibitory factor, macrophage inflammatory protein 2, interleukin 1β and tumor necrosis factor α in middle ear epithelium in mice, and severe proliferation of inflammatory cells was observed in middle ear cavity inoculated with H. pylori whole‐cell protein. In addition, trans‐tympanic injection of macrophage migration inhibitory factor up‐regulated expression of macrophage inflammatory protein 2 in the middle ear. These findings indicate that H. pylori infection causes immunological inflammation in middle ear epithelium, and H. pylori may play a significant role in otitis media.

Monosyllable speech perception of Japanese hearing aid users with prelingual hearing loss: implications for surgical indication of cochlear implant.

OBJECTIVE The monosyllable speech perception ability after years of educational intervention was compared between prelingually deafened pediatric hearing aid users and their cochlear implant counterparts. DESIGN An open-set monosyllabic speech perception test was conducted on all subjects. The test required subjects to indicate a corresponding Japanese character to that spoken by the examiner. Fifty-two subjects with prelingual hearing impairment (47 hearing aid users and 5 cochlear implant users) were examined. RESULTS Hearing aid users with average pure-tone thresholds less than 90 dB HL demonstrated generally better monosyllable perception than 70%, which was equivalent or better performance than that of the cochlear implant group. Widely dispersed speech perception was observed within the 90-99 dB HL hearing-aid user group with most subjects demonstrating less than 50% speech perception. In the cluster of >100 dB HL, few cases demonstrated more than 50% in speech perception. The perception ability of the vowel part of each mora within the cochlear implant group was 100% and corresponding to that of hearing aid users with moderate and severe hearing loss. CONCLUSION Hearing ability among cochlear implant users can be comparable with that of hearing aid users with average unaided pure-tone thresholds of 90 dB HL, after monosyllabic speech perception testing was performed.

Expression of toll-like receptors in chronic otitis media and cholesteatoma

OBJECTIVE Otitis media is one of the most common infectious diseases, especially in young children. Multiple factors affect the onset or development of otitis media. Human toll-like receptors recognize associated patterns and play a critical role in innate immune mechanisms. Toll-like receptors are considered to be important factors for clearance of infection and resolution of inflammation in otitis media. The purpose of this study was to evaluate the histological expression of toll-like receptor 2, which recognizes many kinds of pathogen-associated molecular patterns, and toll-like receptor 4, which recognizes lipopolysaccharide on Gram-negative bacteria, in tissue samples from patients with chronic otitis media and middle ear cholesteatoma. METHODS Human middle ear tissue samples from 12 patients with chronic otitis media (n=7) and acquired middle ear cholesteatoma (n=5) were examined. Normal control middle ear samples without any inflammation were also included (n=7). The expressions of toll-like receptors 2 and 4 in middle ear tissues were examined immunohistochemically. RESULTS Only one normal control middle ear sample showed weak expression of toll-like receptor 2, and toll-like receptor 4 was not observed in all control samples. On the other hand, both toll-like receptors 2 and 4 were markedly expressed in chronic otitis media and cholesteatoma. There was a significant difference between chronic otitis media and normal controls in the expressions of both toll-like receptors. Significant up-regulation of toll-like receptors 2 and 4 was observed in cholesteatoma as compared with control samples. CONCLUSIONS Toll-like receptors 2 and 4 were strongly expressed in chronic otitis media and middle ear cholesteatoma. These findings suggest that toll-like receptors may play a principal role in human chronic otitis media and cholesteatoma.

Inner-ear obliteration in ulcerative colitis patients with sensorineural hearing loss

OBJECTIVE Systemic autoimmune diseases, including ulcerative colitis, may involve the inner ear. Several ulcerative colitis cases presenting with sensorineural hearing loss have been reported. We report the T2-weighted, three-dimensional, inner-ear magnetic resonance imaging findings in the inner ears of two such patients. METHODS Case reports and a review of the literature concerning autoimmune disease and sensorineural hearing loss are presented. RESULTS We describe two cases of ulcerative colitis with sensorineural hearing loss in which three-dimensional magnetic resonance imaging revealed obliteration of the inner ear. Those inner ears with obliteration had severe hearing loss, and responded poorly to steroid therapy. CONCLUSION To our knowledge, there has been no previous published report of the T2-weighted, inner-ear magnetic resonance imaging findings of cases of ulcerative colitis with sensorineural hearing loss. This paper represents the first published report in the world literature of inner-ear obliteration in such patients. Three-dimensional magnetic resonance imaging is beneficial in elucidating the pathophysiology of the inner-ear involvement seen in ulcerative colitis.

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.

Does the introduction of newborn hearing screening improve vocabulary development in hearing-impaired children? A population-based study in Japan

OBJECTIVE Permanent hearing impairment has a life-long impact on children and its early identification is important for language development. A newborn hearing screening (NHS) program has started in Okayama Prefecture, Japan, in 1999 to detect hearing impairment immediately after birth. We aim to examine the effect of this screening program on vocabulary development in pre-school children in a before and after comparative study design. METHODS A total of 107 5-year-old children who graduated from Okayama Kanariya Gakuen (an auditory center for hearing-impaired children) between 1998 and 2011 were enrolled in this study. The pre-NHS group (n=40) was defined as those who graduated between 1998 and 2003, while the post-NHS group (n=67) was defined as those who graduated between 2004 and 2011. The primary outcome was receptive vocabulary, which was assessed by the Picture Vocabulary Test [score <18 (low) vs. score ≥18 (high)]. The secondary outcome was productive vocabulary, or the number of productive words, which was assessed by an original checklist [<1773 words (low) vs. ≥1773 (high)]. We calculated odds ratios and 95% confidence intervals for vocabulary development and compared both groups. RESULTS The adjusted Picture Vocabulary Test score and number of productive words were significantly higher (p<0.01) in the post-NHS group than the pre-NHS group. Odds ratios were 2.63 (95% confidence interval: 1.17-5.89) for receptive vocabulary and 4.17 (95% confidence interval: 1.69-10.29) for productive vocabulary. CONCLUSIONS The introduction of NHS in Okayama Prefecture significantly improved both receptive and productive vocabulary development in hearing-impaired children.

Activation of NLRP3 inflammasome in human middle ear cholesteatoma and chronic otitis media

Abstract Conclusions: The nucleotide-binding oligomerization domain-like receptor protein 3 (NLRP3) inflammasome plays an important role in the pathogenesis of middle ear diseases. Modulation of inflammasome-mediated inflammation may be a novel therapeutic strategy for cholesteatoma and chronic otitis media. Objective: NLRP3 inflammasome is a critical molecule mediating interleukin (IL)-1β responses. However, the expression of NLRP3 in the pathogenesis of cholesteatoma and chronic otitis media has not been fully examined. This study sought to assess the expression of NLRP3, ASC (apoptosis-associated speck-like protein containing a caspase recruitment domain and a pyrin domain), and caspase-1 in middle ear tissues in patients with cholesteatoma or chronic otitis media. Methods: Middle ear tissue samples were obtained from patients with cholesteatoma or chronic otitis media. Control middle ear samples were collected during cochlear implant surgery of patients without middle ear inflammation. The expression of NLRP3, ASC, and caspase-1 were examined by reverse transcription polymerase chain reaction (RT-PCR) assay and immunohistochemical study. Results: The levels of mRNA of NLRP3, ASC, and caspase-1 were significantly elevated in cholesteatoma and chronic otitis media as compared with that of normal controls. The proteins of NLRP3, ASC, and caspase-1 were observed in infiltrating inflammatory cells in cholesteatoma and chronic otitis media.

Impact of early intervention on comprehensive language and academic achievement in Japanese hearing-impaired children with cochlear implants

OBJECTIVES Early hearing detection and intervention (EHDI) is critical for achievement of age-appropriate speech perception and language development in hearing-impaired children. It has been 15 years since newborn hearing screening (NHS) was introduced in Japan, and its effectiveness for language development in hearing-impaired children has been extensively studied. Moreover, after over 20 years of cochlear implantation in Japan, many of the prelingual cochlear implant (CI) users have reached school age, and the effect of CI on language development have also been assessed. To identify prognostic factors for language development, audiological/language test scores and demographic factors were compared among prelingual severe-to-profound hearing-impaired children with CI divided into subgroups according to age at first hearing aid (HA) use and whether they received NHS. METHODS Prelingual severe-to-profound deafened children from the Research on Sensory and Communicative Disorders (RSCD) project who met the inclusion criteria were divided into groups according to the age (in months) of HA commencement (before 6 months: group A, after 7 months: group B), and the presence or absence of NHS (groups C and D). Language development and socio-economic data were obtained from audiological/language tests and a questionnaire completed by caregivers, respectively. RESULTS In total, 210 children from the RSCD project participated in this study. Group A (n=49) showed significantly higher scores on comprehensive vocabulary and academic achievement (p<0.05) than group B (n=161), with no difference in demographics except for significantly older age in group B. No differences in language scores were observed between group C (n=71) and group D (n=129), although participants of group D was significantly older and had used CIs longer (p<0.05). CONCLUSIONS Early use of HAs until the CI operation may result in better language perception and academic achievement among CI users with prelingual deafness. A long-term follow-up is required to assess the usefulness of NHS for language development.

Steroid-dependent sensorineural hearing loss in a patient with Charcot-Marie-Tooth disease showing auditory neuropathy.

Charcot-Marie-Tooth disease (CMT) is the most common form of hereditary sensorimotor neuropathy and sometimes involves disorders of the peripheral auditory system. We present a case of steroid-dependent auditory neuropathy associated with CMT, in which the patient experienced 3 episodes of acute exacerbation of hearing loss and successful rescue of hearing by prednisolone. An 8-year-old boy was referred to the otolaryngology department at the University Hospital. He had been diagnosed with CMT type 1 (demyelinating type) at the Child Neurology Department and was suffering from mild hearing loss due to auditory neuropathy. An audiological diagnosis of auditory neuropathy was confirmed by auditory brainstem response and distortion-product otoacoustic emissions. At 9 years and 0 months old, 9 years and 2 months old, and 10 years and 0 months old, he had experienced acute exacerbations of hearing loss, each of which was successfully rescued by intravenous or oral prednisolone within 2 weeks. Steroid-responsive cases of CMT have been reported, but this is the first case report of steroid-responsive sensorineural hearing loss in CMT. The present case may have implications for the mechanisms of action of glucocorticoids in the treatment of sensorineural hearing loss.