Intraoperative EABR Testing Predicts Strength of Cochlear Implant Stimulation Optimized After Long-Term Use in Pediatric Malformation Ears.

OBJECTIVE: This study focused on the intensities of cochlear implant (CI) stimulation in pediatric CI users with inner ear malformation or cochlear nerve deficiency (CND). In this population, CI programming is difficult because a large intensity of CI stimulation is required to achieve sufficient hearing, but the excess CI stimuli often induce facial nerve stimulation. We aimed to assess whether the results of intraoperative electrically evoked auditory brainstem responses (EABRs) testing predict maximum current levels of CI stimuli (cC levels) optimized by a behavioral-based method after long-term CI use. STUDY DESIGN: A retrospective case review. SETTING: A tertiary referral CI center. PATIENTS: A total of 116 ears with malformations (malformation group) and 63 control ears (control group) from patients younger than 18 years who received CI. The malformation group comprised 23 ears with a common cavity (CC), 26 with incomplete partition type 1 (IP-1), 26 with incomplete partition type 2 (IP-2), and 41 with CND. INTERVENTIONS: Diagnostic. MAIN OUTCOME MEASURES: Correlation between intraoperative EABR results and cC levels determined by the behavioral-based CI programming after long-term CI use. RESULTS: The CC, IP-1, and CND ears required significantly larger cC levels than the IP-2 ears and control groups. However, the cC levels increased to reach the plateau 1 year after surgery in all groups. Among the malformation group, 79 ears underwent intraoperative EABR testing. Greater than 80% of the CC, IP-1, and IP-2 ears and 54.8% of the CND ears exhibited evoked wave V (eV) and were included in the eV-positive category. Myogenic responses but no eV were observed in 18.2, 15.0, and 35.5% of the CC, IP-1, and CND ears, defined as the myogenic category. No eV or myogenic response was elicited in 9.7% of the CND ears. We focused on minimum current levels that elicited eV (eV levels) in the eV-positive category and maximum current levels that did not elicit any myogenic responses (myogenic levels) in the myogenic category. A significant relationship was detected between the eV levels and the cC levels. When analyzed in each malformation type, the eV levels significantly correlate with the cC levels in the CC and CND ears but not in the IP-1 and IP-2 ears, probably because of slight variation within the IP-1 group and the small number of the IP-2 group. The myogenic category did not show a significant relationship between the myogenic levels and cC levels, but the cC levels were similar to or smaller than the myogenic levels in most ears. CONCLUSIONS: This study confirmed that intraoperative EABR testing helps predict the optimal cC levels in malformation ears. EABR-based CI programming immediately after cochlear implantation, followed by behavioral-based CI programming, may allow us to achieve early postoperative optimization of CI maps even in young children with severe malformations.

Management of Already Inserted Ventilation Tubes During Pediatric Cochlear Implantation: To Remove or Leave the Tube?

OBJECTIVE: Ventilation tube (VT) insertion is usually recommended before cochlear implantation (CI) in pediatric cochlear implant candidates with recurrent acute otitis media (AOM) or chronic otitis media with effusion (OME). However, there is no consensus on whether the VT is beneficial even after CI, that is, whether the tube should be removed or left in place during CI. This study aimed to assess the effect of tube placement after CI, especially on the incidence of post-CI AOM, in pediatric cochlear implant recipients who had undergone VT insertion before CI because of recurrent AOM or chronic OME. STUDY DESIGN: A retrospective medical record review. SETTING: A tertiary referral cochlear implant center. PATIENTS: This study recruited 58 consecutive ears of children who underwent VT insertion followed by CI at age 7 years or younger between 2004 and 2021. Before October 2018, we removed the VT simultaneously with CI (removed group, 39 ears), while since then, the tube has remained in place during CI (retained group, 19 ears). INTERVENTION: Therapeutic. MAIN OUTCOME MEASURE: The primary outcome was the proportion of ears that developed AOM at post-CI 6 months in the removed and retained groups. RESULTS: The age at CI was significantly higher in the removed group than in the retained group (mean [standard deviation]: the removed group, 2.9 [1.2] yr; the retained group: 1.5 [0.8] yr; p < 0.001). The removed group showed a significantly higher proportion of ears with post-CI AOM (8 of 39 ears; 20.5%) than the retained group (none of 19 ears; 0%) 6 months after CI ( p = 0.044). The AOM-free proportion at post-CI 12 months was 76.9% in the removed group and 83.3% in the retained group, demonstrating no significant difference ( p = 0.49), probably because the VT was spontaneously extruded in the retained group at a median of 6.5 months after CI. Throughout the study period, 17 ears (13 from the removed group) were affected by post-CI AOM. Of these, three ears in the removed group and two in the retained group after spontaneous extrusion of the VT were hospitalized and treated with intravenous antibiotics for AOM that had failed to respond to oral antibiotic therapy. Only one ear in the removed group required an explanation of the infected implant. None suffered from chronic perforation of the tympanic membrane or secondary cholesteatoma after VT insertion or meningitis associated with post-CI AOM. CONCLUSION: Our results suggest that in CI for children who already have a VT because of a recurrent AOM or chronic OME, retaining the tube in position, rather than removing the tube, may decrease the incidence of AOM at least within 6 months after CI, during which most cochlear implant device infection was reported in the pediatric population.

A multicenter study to investigate the positive rate of SARS-CoV-2 in middle ear and mastoid specimens from otologic surgery patients.

OBJECTIVE: Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), a novel coronavirus, causes coronavirus disease 2019 (COVID-19). Otologic surgeries with drilling by powered instruments induce significant aerosols, which may induce SARS-CoV-2 transmission to medical staff if SARS-CoV-2 exists in the middle ear and mastoid cavity. During a COVID-19 pandemic, therefore, confirming a negative COVID-19 test prior to otologic surgery is recommended. However, previous coronavirus studies demonstrated that coronavirus was detected in the middle ear in some patients even though the polymerase chain reaction (PCR) test using their nasopharyngeal swab was negative. This study aimed to elucidate the probability of a positive SARS-CoV-2 PCR test in the middle ear or mastoid specimens from otologic surgery patients in whom SARS-CoV-2 was not detected by preoperative PCR test using a nasopharyngeal swab. METHODS: We conducted a prospective, multicenter clinical study. Between April 2020 and December 2021, during the COVID-19 pandemic, 251 ears of the 228 participants who underwent otologic surgery were included in this study. All participants had no symptoms suggesting COVID-19 or close contact with a confirmed COVID-19 patient two weeks prior to the surgery. They were also negative in the SARS-CoV-2 PCR tests using a nasopharyngeal swab before surgery. We collected mucosa, granulation, bone dust with mucosa or fluid from the middle ear or mastoid for the SARS-CoV-2 PCR tests during each otologic surgery. RESULTS: The median age of the participants at surgery was 31.5 years old. Mastoidectomy using a powered instrument was conducted in 180 of 251 otologic surgeries (71.8%). According to intraoperative findings, active inflammation in the middle ear or mastoid cavities was evident in 20 otologic surgeries (8.0%), while minor inflammation was observed in 77 (30.7%). All SARS-CoV-2 PCR tests of otologic specimens showed a negative result. No patient suffered from COVID-19 within two months after otologic surgery. Furthermore, no hospital-acquired infections associated with otologic surgery occurred in our institutions CONCLUSIONS: Our results showed that PCR testing did not detect SARS-CoV-2 in middle ear and mastoid specimens, suggesting that the risk of transmission of SARS-CoV-2 is not high in otologic surgeries even using powered instruments when both clinical and laboratory tests are confirmed to be negative for COVID-19.

Etiology of hearing loss affects auditory skill development and vocabulary development in pediatric cochlear implantation cases.

BACKGROUND: Cochlear implantation (CI) is an effective treatment for severe-to-profound hearing loss patients and is currently used as the standard therapeutic option worldwide. However, the outcomes of CI vary among patients. AIMS/OBJECTIVES: This study aimed to clarify the clinical features for each etiological group as well as the effects of etiology on CI outcomes. MATERIALS AND METHODS: We collected clinical information for 308 pediatric cochlear implant cases, including the etiology, hearing thresholds, age at CI, early auditory skill development, total development, monosyllable perception, speech intelligibility and vocabulary development in school age, and compared them for each etiology group. RESULTS: Among the 308 CI children registered for this survey, the most common etiology of hearing loss was genetic causes. The genetic etiology group showed the most favorable development after CI followed by the unknown etiology group, syndromic hearing loss group, congenital CMV infection group, inner ear malformation group, and cochlear nerve deficiency group. CONCLUSIONS AND SIGNIFICANCE: Our results clearly indicated that the etiology of HL affects not only early auditory skill development, but also vocabulary development in school age. The results of the present study will aid in more appropriate CI outcome assessment and in more appropriate intervention or habilitation programs.

Prognostic impact of incisional or excisional biopsy of cervical lymph node metastases of solid tumors.

OBJECTIVES: In performing an open biopsy of a neck mass, an incisional biopsy may increase the risk of cancer cell seeding and dissemination that, ultimately, worsens a patient's survival. The aim of this study was to compare the impact of incisional and excisional biopsies of cervical lymph node metastases of solid tumors on patients' survival. METHODS: A retrospective review was made of patients with cervical metastases of solid tumors who underwent an open biopsy for a diagnosis between 2005 and 2015. Sixty-four patients met the criteria out of 524 open biopsy cases undertaken during the period. Survival analyses were estimated from 33 cases whose initial symptoms were the presence of a neck mass, using two modes of biopsy: excisional and incisional. RESULTS: The 2-year overall survival rates in incisional and excisional biopsy groups were 65% and 43%, respectively, and 2-year disease-specific survival rates were 74% and 43%, respectively. The differences were not significant. For lung cancer or head and neck cancer subgroups, survival differences between incisional and excisional biopsy groups were also not significant. CONCLUSIONS: A carefully targeted physical examination and performing a fine needle aspiration are essential to establish a diagnosis for the etiology of an unknown neck mass. In performing an open biopsy, the effect of an incisional biopsy on patients' survival was no worse than that of an excisional biopsy, despite the latter being theoretically preferable.

Facial flushing after alcohol intake as a predictor for a high risk of synchronous or metachronous cancer of the upper gastrointestinal tract.

BACKGROUND: In Japan, there has been a lot of reports showing an association between facial flushing after light alcohol consumption and heterozygosity for inactive aldehyde dehydrogenase-2 (ALDH2). Persons with inactive ALDH2 may have a higher risk of alcohol-related oral, pharyngeal and esophageal cancers, compared with those with wild-type ALDH2. The purpose of this study was to examine whether flushers with oral or pharyngeal squamous cell carcinoma have an increased risk of synchronous or metachronous cancer of the upper gastrointestinal (UGI) tract. METHODS: A retrospective study was performed by medical chart review and through a questionnaire sent to 285 patients treated for oral and pharyngeal cancer. Responses were obtained from 150 patients (52.6%), who were classified as flushers or non-flushers, smokers (≥20 pack-year; 1 pack-years = number of cigarettes/20 per day) or non-smokers, and drinkers (≥14 units of alcohol consumption per week; 1 unit = 22 g) or non-drinkers. Relationships of these factors with occurrence of second primary cancers (SPCs) in the UGI tract were investigated. RESULTS: In Kaplan-Meier analysis, there was a significantly higher rate of SPC at 5 years in flushers and drinkers, but no relationship with smoking. In multivariate analyses, a history of flushing was significantly associated with SPC in the UGI tract (HR 2.64, 95% CI 1.25-5.52, P = 0.0109), but not with smoking or alcohol consumption. CONCLUSIONS: A simple interview on history of facial flushing after alcohol intake can be useful for identifying patients at high risk for synchronous or metachronous cancers of the UGI tract.

Audio-visual speech perception in prelingually deafened Japanese children following sequential bilateral cochlear implantation.

OBJECTIVES: An effect of audio-visual (AV) integration is observed when the auditory and visual stimuli are incongruent (the McGurk effect). In general, AV integration is helpful especially in subjects wearing hearing aids or cochlear implants (CIs). However, the influence of AV integration on spoken word recognition in individuals with bilateral CIs (Bi-CIs) has not been fully investigated so far. In this study, we investigated AV integration in children with Bi-CIs. METHODS: The study sample included thirty one prelingually deafened children who underwent sequential bilateral cochlear implantation. We assessed their responses to congruent and incongruent AV stimuli with three CI-listening modes: only the 1st CI, only the 2nd CI, and Bi-CIs. The responses were assessed in the whole group as well as in two sub-groups: a proficient group (syllable intelligibility ≥80% with the 1st CI) and a non-proficient group (syllable intelligibility < 80% with the 1st CI). RESULTS: We found evidence of the McGurk effect in each of the three CI-listening modes. AV integration responses were observed in a subset of incongruent AV stimuli, and the patterns observed with the 1st CI and with Bi-CIs were similar. In the proficient group, the responses with the 2nd CI were not significantly different from those with the 1st CI whereas in the non-proficient group the responses with the 2nd CI were driven by visual stimuli more than those with the 1st CI. CONCLUSION: Our results suggested that prelingually deafened Japanese children who underwent sequential bilateral cochlear implantation exhibit AV integration abilities, both in monaural listening as well as in binaural listening. We also observed a higher influence of visual stimuli on speech perception with the 2nd CI in the non-proficient group, suggesting that Bi-CIs listeners with poorer speech recognition rely on visual information more compared to the proficient subjects to compensate for poorer auditory input. Nevertheless, poorer quality auditory input with the 2nd CI did not interfere with AV integration with binaural listening (with Bi-CIs). Overall, the findings of this study might be used to inform future research to identify the best strategies for speech training using AV integration effectively in prelingually deafened children.

Prognostic impact of salvage treatment on hearing recovery in patients with sudden sensorineural hearing loss refractory to systemic corticosteroids: A retrospective observational study.

OBJECTIVE: To determine the prognostic factors for hearing recovery in patients with sudden sensorineural hearing loss (SSHL) refractory to systemic corticosteroids following salvage treatment. METHODS: This is a retrospective observational study at nine tertiary referral hospitals. A total of 120 patients with sudden deafness refractory to systemic corticosteroids were enrolled. The patients were randomly assigned to receive topical application of recombinant human IGF-1 or intratympanic injection of dexamethasone as salvage treatment. Multiple regression analysis was performed to identify determinants of hearing recovery using pure tone audiometry results at 8 weeks after treatment. Clinical predictors that were evaluated included age, sex, pretreatment hearing level, presence of vertiginous symptoms, days to study entry from symptom onset and salvage treatment assignment (IGF-1 vs. dexamethasone). RESULTS: The linear regression model identified age (P=0.001), pretreatment hearing level (P<0.001), days to study entry from symptom onset (P=0.011) and treatment assignment (P=0.033) at 8 weeks after treatment as significant variables influencing the recovery of pure tone audiometry average thresholds. Younger age (<60 years), early initiation of salvage treatment and treatment with topical IGF-1 therapy had significant effects on hearing recovery. CONCLUSION: The results indicate that early initiation and choice of treatment modalities for salvage treatment may be important for the prognosis of patients with refractory SSHL. The positive effect of topical IGF-1 therapy on hearing recovery indicates its utility as salvage treatment.

Detection of subclinical recurrence or second primary cancer using (18) F-FDG PET/CT in patients treated curatively for head and neck squamous cell carcinoma.

BACKGROUND: The efficacy of posttreatment surveillance (18) F-fluorodeoxyglucose positron emission tomography ((18) F-FDG PET)/CT was evaluated in patients with head and neck squamous cell carcinoma (HNSCC). METHODS: The subjects were 158 patients with HNSCC who underwent PET/CT after definitive treatment. PET/CT detection of subclinical recurrence or a second primary cancer and the effect of timing of PET/CT scans on survival were analyzed. RESULTS: Recurrence or a second primary cancer occurred in 70 patients, and 67% of these cases were detected by PET/CT. Detection rates were 17%, 9%, 5%, and 5% in the first, second, third, and fourth scans at 4, 9, 15, and 21 months, respectively. In multivariate analysis, patients who underwent early first scans had significantly better disease-specific (hazard ratio [HR] = 0.37; p = .031) and overall (HR = 0.45; p = .040) survival compared with those who underwent late first scans. CONCLUSION: Earlier detection of subclinical lesions by surveillance PET/CT within 4 months after treatment may improve survival in patients with HNSCC. © 2015 Wiley Periodicals, Inc. Head Neck 38: E511-E518, 2016.

Audio-visual integration during speech perception in prelingually deafened Japanese children revealed by the McGurk effect.

OBJECTIVE: To investigate the McGurk effect in profoundly deafened Japanese children with cochlear implants (CI) and in normal-hearing children. This was done to identify how children with profound deafness using CI established audiovisual integration during the speech acquisition period. METHODS: Twenty-four prelingually deafened children with CI and 12 age-matched normal-hearing children participated in this study. Responses to audiovisual stimuli were compared between deafened and normal-hearing controls. Additionally, responses of the children with CI younger than 6 years of age were compared with those of the children with CI at least 6 years of age at the time of the test. RESULTS: Responses to stimuli combining auditory labials and visual non-labials were significantly different between deafened children with CI and normal-hearing controls (p<0.05). Additionally, the McGurk effect tended to be more induced in deafened children older than 6 years of age than in their younger counterparts. CONCLUSIONS: The McGurk effect was more significantly induced in prelingually deafened Japanese children with CI than in normal-hearing, age-matched Japanese children. Despite having good speech-perception skills and auditory input through their CI, from early childhood, deafened children may use more visual information in speech perception than normal-hearing children. As children using CI need to communicate based on insufficient speech signals coded by CI, additional activities of higher-order brain function may be necessary to compensate for the incomplete auditory input. This study provided information on the influence of deafness on the development of audiovisual integration related to speech, which could contribute to our further understanding of the strategies used in spoken language communication by prelingually deafened children.

Bilateral duplication of the internal auditory canal: a case with successful cochlear implantation.

We report a case of bilateral duplication of the internal auditory canal (IAC). An 11-month-old girl with congenital profound hearing loss was referred to our hospital. Imaging evaluations revealed bilateral IAC duplication, which contained the facial and cochleovestibular nerves in one canal, but no nerves in the other. She underwent cochlear implantation. At 5 months after surgery her hearing thresholds with the cochlear implant are 40 and 45dB at 2000 and 4000Hz, respectively. Bilateral duplicated IAC is extremely rare, with seven cases reported in the literature. This case represents a previously unreported type of IAC duplication, and is the first case in which cochlear implantation was successfully performed.

A case of angiosarcoma arising from internal jugular vein.

Primary angiosarcoma is a rare disease with a poor prognosis. It most commonly arises in the head and neck region; localization in the deep soft tissue of the neck is extremely rare. We herein present a case of angiosarcoma derived from the right internal jugular vein. A 79-year-old man presented with a 1-month history of a growing right neck mass. Computed tomography, magnetic resonance imaging, positron emission tomography-computed tomography, and fine-needle aspiration cytology revealed a malignant tumor of unknown origin. Right neck dissection was performed for both diagnosis and therapy. Immunostaining of the resected tumor cells revealed positivity for CD31, CD34, factor VIII-related antigen, and D2-40, which allowed for a definitive diagnosis of angiosarcoma. Postoperative radiotherapy (66Gy) was performed on the right neck, including the surgical bed and upper mediastinum. The patient was followed up for 10 months with no recurrence. Only six cases of angiosarcoma arising in the deep soft tissue of the neck have been reported in the English-language literature. The present report is the first to describe angiosarcoma arising from the internal jugular vein.

SLC26A4 p.Thr410Met homozygous mutation in a patient with a cystic cochlea and an enlarged vestibular aqueduct showing characteristic features of incomplete partition type I and II.

Mutations of SLC26A4 are associated with incomplete partition type II (IP-II) and isolated enlargement of the vestibular aqueduct (EVA). We experienced a congenitally deaf 6-year-old boy with a rare p.Thr410Met homozygous mutation in SLC26A4 who underwent bilateral cochlear implantation. He had bilateral inner ear malformation, in which the dilated vestibule and EVA were identical to those in IP-II, but the cochlea lacking a bony modiolus resembled that in incomplete partition type I. These results suggest that homozygous mutations in SLC26A4 are always associated with EVA, while the severity of cochlear malformation may vary depending on the type of SLC26A4 mutation.

A randomized controlled clinical trial of topical insulin-like growth factor-1 therapy for sudden deafness refractory to systemic corticosteroid treatment.

BACKGROUND: To date, no therapeutic option has been established for sudden deafness refractory to systemic corticosteroids. This study aimed to examine the efficacy and safety of topical insulin-like growth factor-1 (IGF-1) therapy in comparison to intratympanic corticosteroid therapy. METHODS: We randomly assigned patients with sudden deafness refractory to systemic corticosteroids to receive either gelatin hydrogels impregnated with IGF-1 in the middle ear (62 patients) or four intratympanic injections with dexamethasone (Dex; 58 patients). The primary outcome was the proportion of patients showing hearing improvement (10 decibels or greater in pure-tone average hearing thresholds) 8 weeks after treatment. The secondary outcomes included the change in pure-tone average hearing thresholds over time and the incidence of adverse events. RESULTS: In the IGF-1 group, 66.7% (95% confidence interval [CI], 52.9-78.6%) of the patients showed hearing improvement compared to 53.6% (95% CI, 39.7-67.0%) of the patients in the Dex group (P = 0.109). The difference in changes in pure-tone average hearing thresholds over time between the two treatments was statistically significant (P = 0.003). No serious adverse events were observed in either treatment group. Tympanic membrane perforation did not persist in any patient in the IGF-1 group, but did persist in 15.5% (95% CI, 7.3-27.4%) of the patients in the Dex group (P = 0.001). CONCLUSIONS: The positive effect of topical IGF-1 application on hearing levels and its favorable safety profile suggest utility for topical IGF-1 therapy in patients with sudden deafness. TRIAL REGISTRATION: UMIN Clinical Trials Registry Number UMIN000004366, October 30th, 2010.

Risk factors for retropharyngeal cellulitis in Kawasaki disease.

OBJECTIVE: Kawasaki disease (KD) is an acute multisystemic vasculitis of unknown etiology that occurs in infants and children. Retropharyngeal cellulitis has been reported as a rare manifestation of KD. This study investigated the frequency and characteristics of patients with KD manifesting as retropharyngeal soft-tissue swelling. METHODS: We retrospectively reviewed 277 patients, with a mean age of 1 year and an age range of 7 months to 12 years, in whom KD had been diagnosed between 2005 and 2011. RESULTS: In 10 patients (3.6%), contrast-enhanced computed tomography (CECT) showed low-density lesions without ring enhancement in the retropharyngeal spaces. These patients presented initially with fever and cervical lymphadenopathy, and were initially treated by their pediatricians for suppurative lymphadenitis (seven patients) or retropharyngeal abscess (three patients). KD was finally diagnosed either after antibiotics had been ineffective or when other symptoms characteristic of KD emerged. CONCLUSION: Low-density lesions in the retropharyngeal space were identified by CECT in 3.6% of the KD patients. Early diagnosis of KD is essential because coronary artery lesions develop in 50% of untreated patients. If a child presents with fever, cervical lymphadenopathy, and swelling of the retropharyngeal space, KD should be included in the differential diagnoses.

Electrically evoked auditory brainstem response-based evaluation of the spatial distribution of auditory neuronal tissue in common cavity deformities.

OBJECTIVE: In a common cavity (CC) deformity, the cochlea and vestibule are confluent to form a single cavity without internal architecture, and distribution of auditory neuronal tissue is unclear. The purposes of this study are to reveal the spatial distribution of auditory neuronal tissue in CC deformity using electrically evoked auditory brainstem response (EABR) during cochlear implantation. STUDY DESIGN: Retrospective case review. SETTING: Cochlear implant (CI) center at a tertiary referral hospital. PATIENTS: Five patients with CC deformity who underwent cochlear implantation and intraoperative EABR testing. MAIN OUTCOME MEASURES: Spatial distribution of electrodes that elicited an evoked wave V (eV) in EABR testing was evaluated in each CC deformity. RESULTS: Electrically evoked auditory brainstem response testing demonstrated that electrodes attached on the inner wall of the anteroinferior cavity of the CC deformity successfully elicited a reproducible eV in all cases, and the latency of each eV was an approximately 4 ms, which is similar to those reported in patients without an inner ear malformation. Interestingly, in Case 1 with the lowest percentage of eV-positive electrodes (31.8%), CI-aided audiometric thresholds were changed, depending on the frequency allocation to eV-positive electrodes in the programming. Cochlear implant-mediated facial nerve stimulation was observed in 3 of 5 cases, and results of EABR testing were useful for optimizing the device program to decrease facial nerve stimulation without sacrificing CI-mediated auditory performance. CONCLUSION: The results of EABR testing suggested that auditory neuronal elements are distributed to the anteroinferior part of CC deformity, mainly around or near the inner wall of the cavity. In cases with CC deformity, EABR testing is useful to achieve the optimal electrode array placement and to adjust programming parameters of the implanted device, which might be essential to maximize CI outcomes and to decrease facial nerve stimulation.

Intra-arterial administration of antibiotics for refractory skull base osteomyelitis.

We report two cases of elderly diabetic men with skull base osteomyelitis (SBO) originating from malignant external otitis (MEO). In both, a devastating infection and neural paralysis deteriorated after conventional therapy, including long-term intravenous administration of culture-directed antibiotics with strict control of blood sugar levels and surgical debridement of infectious granulation tissue. Since poor perfusion of antibiotics in the lesion may be associated with serious nature of MEO/SBO, we administered antibiotics intra-arterially via a retrograde catheter with the tip set at the proximal point of the external carotid artery to increase the tissue drug concentration in the maxillary artery (MA) and ascending pharyngeal artery (APA) supply areas, in which intense inflammation was observed. This intra-arterial administration of antibiotics (IA therapy) followed by long-term intravenous and oral antibiotic treatments eliminated their infection and no recurrence was observed in 2 years follow-up period. Interestingly, CT images of angiography via the catheter demonstrated stronger enhancement in the MA supply area compared to the APA supply area and IA therapy was more effective in the former. These results suggest that IA therapy, which might achieve high antibiotic concentration at the site of infection, is effective in patients with MEO/SBO refractory to conventional treatments.

Predictive value of middle ear aeration before second-stage operation in staged tympanoplasty with soft-wall reconstruction.

CONCLUSION: The extent of middle ear aeration before second-stage canal wall-down (CWD) tympanoplasty was correlated with postoperative middle ear stability. OBJECTIVE: To evaluate middle ear aeration before second-stage CWD tympanoplasty as a predictor of postoperative re-aeration potential and external auditory canal (EAC) stability in staged CWD tympanoplasty with soft-wall reconstruction (SWR). METHODS: Middle ear aeration was evaluated before and at 1 year after the second-stage operation in patients who underwent staged CWD tympanoplasty with SWR for middle ear cholesteatoma. Based on the computed tomography (CT) findings, middle ear aeration was graded as A when the mastoid and tympanic cavities were aerated, B when only the tympanic cavity was aerated, and C in cases with no aeration in the tympanic cavity. We also examined postoperative EAC stability. RESULTS: Forty-one ears were included. In all, 17 of 19 ears (89.5%) with grade A aeration preoperatively maintained grade A aeration postoperatively, while 5 of 18 ears (27.8%) with grade B aeration had grade A aeration, and no ear with grade C aeration had recovered grade A aeration. All ears with grade A aeration preoperatively maintained smooth EACs. EAC retraction requiring additional treatment occurred in five ears with grade B aeration and all ears with grade C aeration.

Clinical features of rapidly progressive bilateral sensorineural hearing loss.

CONCLUSION: Rapidly progressive bilateral sensorineural hearing loss (SNHL) often develops as a symptom of intracranial diseases or systemic vasculitis. For early diagnosis and treatment of these potentially fatal diseases, a history of hearing deterioration within 2 months and associated symptoms may be important. OBJECTIVES: To reveal clinical features and causative diseases for rapidly progressive bilateral SNHL. METHODS: The inclusion criterion was patients with bilateral progressive SNHL, who had experienced difficulty in daily conversation within 4 days to 1 year after the onset of hearing loss awareness. This study was a retrospective evaluation of 12 patients with rapidly progressive bilateral SNHL who visited our hospital between 2007 and 2011. RESULTS: The causative disease for hearing loss was identified in 11 of 12 patients; intracranial lesions including nonbacterial meningitis, meningeal metastasis of lymphoma, and superficial siderosis in 4 patients, systemic vasculitis in 2, auditory neuropathy spectrum disorder in 1, and an isolated inner ear disorder in 4. Relatively rapid hearing deterioration within 2 months showed a significant association in six patients with an intracranial lesion or systemic vasculitis. Moreover, all these six patients complained of dizziness and/or non-cochleovestibular symptoms such as fever, headache, and/or altered mental state in addition to hearing loss.

Evaluation of cortical processing of language by use of positron emission tomography in hearing loss children with congenital cytomegalovirus infection.

OBJECTIVE: To predict cochlear implant efficacy and investigate the cortical processing of the visual component of language in profoundly deafened patients with asymptomatic congenital cytomegalovirus (CMV) infection. METHODS AND CASES: The cortical activity of two children with CMV-related hearing loss was evaluated with fluorodeoxyglucose-positron emission tomography (FDG-PET) with a visual language task before cochlear implantation. Total development and auditory perception ability were assessed one year after implantation. RESULTS: The two children with CMV-related hearing loss showed activation in the auditory association area where no activation was found in the controls, and exhibited nearly identical cortical activation patterns to those seen in patients with profound congenital hearing loss. In contrast, differences in total development in verbal ability and discrimination of sentences between the two cases were revealed one year after implantation. CONCLUSION: These results might indicate that the differences of cortical activities according to hearing abilities could have been influenced by CMV infection that involves higher function of the brain directly and/or affects the cochlea peripherally. Additionally, if CMV infection might have affected only the cochlea, these cortical activation patterns were influenced secondary by the time course of hearing loss characterized by CMV infection, which had varied manifestations. Accurate diagnosis and cochlear implantation at the appropriate time are important for successful speech development, and each patient needs a personalized habilitation program based on their etiology and brain function.