Optimal use of CT imaging in pediatric congenital cholesteatoma.

OBJECTIVE: This study aimed to determine the radiation dose of temporal bone computed tomography (TBCT) to detect pediatric congenital cholesteatoma (CC), and suggests strategy for lowering perioperative radiation dose. METHODS: Fifty-three CC patients followed up for more than 12 months after surgery. Perioperative clinical findings and the effective radiation dose (ERD) of TBCT were investigated. Patients' mean age at initial TBCT was 60 months; the mean follow-up period was 43 months. RESULTS: In 33 (62.3%) of 53 patients, only one TBCT was performed postoperatively. Postoperative CT imaging was performed to evaluate before second-stage ossiculoplasty, due to suspicion of CC recurrence on examinations, or as part of follow-up. When TBCT was performed on more than two occasions after surgery as a part of follow-up, there was no difference in the distribution of stage or type of CC compared to patients subjected to TBCT on less than three occasions (p=0.093 and p=0.744, respectively). During the most-recent follow-up of these 53 cases, there was no CC recurrence. The mean ERD of a single TBCT was 1.2mSv, and the mean cumulative ERD of TBCT was 2mSv per patient. CONCLUSION: Mean and cumulative ERD of TBCT performed in CC cases was not too high to consider radiation hazard. However, efforts to minimize the number of CT scans should be taken basically.

Temporalis myofascial flap coverage for extrusion of internal device after cochlear implantation.

Two pediatric patients with internal device exposure of cochlear implant (CI) were treated successfully using vascularized temporalis myofascial flaps. The visible scarring was minimal, and the CI function was excellent. Although we used temporalis myofascial flaps for late complications of an implanted ear, it can also be used in a primary case to provide flap reinforcement. In conclusion, the temporalis myofascial flap technique is an ultimate surgical option that offers advantages for CI patients with flap-related problems.

Long-term functional and behavioral-emotional outcomes in children with early cochlear implants: Parental testimonies.

OBJECTIVES: (1) To assess the long-term effects of cochlear implantation (CI) on speech perception and school life, and (2) to evaluate behavioral and emotional development, including social skills, post-implantation. METHODS: We telephoned caregivers and asked them a standardized questionnaire. We used the data to explore the daily lives (including school life) of children who had undergone CI before the age of 4 years and who had used the device for >10 years. We also evaluated behavioral/emotional development. RESULTS: Most children used CI devices for virtually all their waking moments and attended mainstream schools. Moreover, more than 75% of them could comprehend common phrases or conversations without lip-reading and carry out a telephone conversation with a person known to them. The mean T-scores for all scales of behavioral/emotional assessment, including those concerning social skills, were within the normal ranges, although scores on the competence scale were lower than those on other scales. CONCLUSIONS: This long-term study of early-CI children shows that CI improves not only speech perception, but also behavioral/emotional development, including social skills.

Incus footplate assembly: Indication and surgical outcome.

OBJECTIVES/HYPOTHESIS: To review surgical findings and hearing outcomes of incus footplate assembly (IFA) for the patients with conductive hearing loss due to missing stapes superstructure with a mobile stapes footplate. STUDY DESIGN: Retrospective case review and survey. METHODS: Pre- and postoperative audiometric data and intraoperative findings were reviewed. Postoperative air-bone gap (ABG) and ABG closure (postoperative air-conduction threshold-preoperative bone-conduction threshold) were analyzed. RESULTS: The causes of missing stapes superstructure and conductive hearing loss were congenital ossicular anomaly (n = 5), chronic otitis media (n = 2), and congenital cholesteatoma (n = 1). The prosthesis was designed to fit between the medial side of the incus and stapes footplate and had a mean length of 3.6 ± 0.5 mm. The mean pre- and postoperative ABG were 38.3 ± 4.8 and 13.3 ± 10.0 dB, respectively. The postoperative ABG at frequencies of 0.25, 0.5, 1.0, 2.0, 3.0, and 4.0 kHz were 20.0 ± 15.4, 16.9 ± 11.9, 16.3 ± 10.3, 10.6 ± 7.3, 12.9 ± 14.0, and 23.1 ± 16.2 dB, respectively. The mean ABG closure was 9.5 dB (range, -1.3∼35.8 dB). Seven cases obtained the best results (mean ABG closure ≤10 dB). In the remaining patient, the mean ABG closure was 9.5 dB until 6 months after surgery, but was 35.8 dB 1 year after surgery. CONCLUSIONS: IFA seems to be a reasonable surgical option in patients with missing the stapes superstructure, but with a mobile footplate in which the long process of incus is preserved. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:2569-2573, 2016.

Transmastoid reshaping of the sigmoid sinus: preliminary study of a novel surgical method to quiet pulsatile tinnitus of an unrecognized vascular origin.

OBJECTIVE A dominant sigmoid sinus with focal dehiscence or thinning (DSSD/T) of the overlying bony wall is a commonly encountered, but frequently overlooked, cause of vascular pulsatile tinnitus (VPT). Also, the pathophysiological mechanism of sound perception in patients with VPT remains poorly understood. In the present study, a novel surgical method, termed transmastoid SS-reshaping surgery, was introduced to ameliorate VPT in patients with DSSD/T. The authors reviewed a case series, analyzed the surgical outcomes, and suggested the pathophysiological mechanism of sound perception. The theoretical background underlying VPT improvement after transmastoid SS-reshaping surgery was also explored. METHODS Eight patients with VPT that was considered attributable to DSSD/T underwent transmastoid SS-reshaping surgery between February 2010 and February 2015. The mean postoperative follow-up period was 9.5 months (range 4-13 months). Transmastoid SS-reshaping surgery featured simple mastoidectomy, partial compression of the SS using harvested cortical bone chips, and reinforcement of the bony SS wall with bone cement. Perioperative medical records, imaging results, and audiological findings were comprehensively reviewed. RESULTS In 7 of the 8 patients (87.5%), the VPT abated immediately after surgery. Statistically significant improvements in tinnitus loudness and distress were evident on numeric rating scales. Three patients with preoperative ipsilesional low-frequency hearing loss exhibited postoperative improvements in their low-frequency hearing thresholds. No major postoperative complications were encountered except in the first subject, who experienced increased intracranial pressure postoperatively. This subsided after a revision operation for partial decompression of the SS. CONCLUSIONS Transmastoid SS-reshaping surgery may be a good surgical option in patients with DSSD/T, a previously unrecognized cause of VPT. Redistribution of severely asymmetrical blood flow, reinforcement of the bony SS wall with bone cement to reconstruct a soundproof barrier, and disconnection of a problematic sound conduction route via simple mastoidectomy silence VPT.

Clinical observations and molecular variables of patients with hearing loss and incomplete partition type III.

OBJECTIVES/HYPOTHESIS: To analyze the clinical manifestations and genetic features of patients with hearing loss (HL) and incomplete partition (IP) type III malformation, and to evaluate speech performance after cochlear implantation (CI) in these patients. STUDY DESIGN: Individual retrospective cohort study. METHODS: Of 206 probands with inner ear malformations (IEMs), we constructed a homogeneous cohort of 11 genetically documented IP type III (DFNX2). Mutations affecting POU3F4 were classified as extension (n = 2), truncation (n = 3), large genomic deletion (n = 2), or missense substitution (n = 4). Postoperative outcomes were rigorously assessed with focus on POU3F4 genotypes and compared with 80 age-matched implantees without IEMs. RESULTS: HL in our cohort was prelingual in onset irrespective of degree. Serviceable hearing was obtained by wearing conventional hearing aids in three, and eight subjects required CI. No correlation was found between mutation types and initial auditory phenotype. As for the eight cochlear implantees, average Categories of Auditory Perception score was <1 preoperatively and 3.9 at 2 years post-CI. Speech performances improved over the first 3 months postoperatively in a manner comparable to implantees without IEMs. However, it then tended to slow down until 1 year postoperatively, leading to worse scores at 2 years than implantees without IEMs. Furthermore, this was more evident in those with a truncation or deletion mutation. CONCLUSIONS: CI surgeons should be aware that postoperative auditory performance may be not as good in IP type III patients as in patients without IEMs. In particular, our study implies that certain types of POU3F4 mutations may have poorer prognoses than other types after CI. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:E123-E128, 2016.

Long-term outcome of cochlear implant in patients with chronic otitis media: one-stage surgery is equivalent to two-stage surgery.

This study compared long-term speech performance after cochlear implantation (CI) between surgical strategies in patients with chronic otitis media (COM). Thirty patients with available open-set sentence scores measured more than 2 yr postoperatively were included: 17 who received one-stage surgeries (One-stage group), and the other 13 underwent two-stage surgeries (Two-stage group). Preoperative inflammatory status, intraoperative procedures, postoperative outcomes were compared. Among 17 patients in One-stage group, 12 underwent CI accompanied with the eradication of inflammation; CI without eradicating inflammation was performed on 3 patients; 2 underwent CIs via the transcanal approach. Thirteen patients in Two-stage group received the complete eradication of inflammation as first-stage surgery, and CI was performed as second-stage surgery after a mean interval of 8.2 months. Additional control of inflammation was performed in 2 patients at second-stage surgery for cavity problem and cholesteatoma, respectively. There were 2 cases of electrode exposure as postoperative complication in the two-stage group; new electrode arrays were inserted and covered by local flaps. The open-set sentence scores of Two-stage group were not significantly higher than those of One-stage group at 1, 2, 3, and 5 yr postoperatively. Postoperative long-term speech performance is equivalent when either of two surgical strategies is used to treat appropriately selected candidates.

De novo large genomic deletions involving POU3F4 in incomplete partition type III inner ear anomaly in East Asian populations and implications for genetic counseling.

OBJECTIVE: The aim of this study was to understand the prevalence and molecular genetic etiology of incomplete partition type III (IP type III) anomaly in Koreans. We also attempted to verify the prevalence of genomic deletions in the DFNX2 locus and to look for association between inheritance patterns and mutation type in East Asian IP type III subjects. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Subjects with IP type III anomaly and their biological mothers. INTERVENTIONS: Sanger sequencing, array-comparative genomic hybridization (aCGH), and PCR were performed. We also analyzed the type and inheritance of the causative genetic abnormality in East Asian DFNX2 patients. MAIN OUTCOME MEASURE: Mutation type and occurrence. RESULTS: We identified IP type III in 10 (4.8%) of 206 patients with an inner ear abnormality. We confirmed an etiologic homogeneity, DFNX2, of the IP type III in this Korean population. Two (20%) of the 10 DFNX2 carried a large genomic deletion affecting POU3F4, as proved by aCGH. PCR confirmed that the 2 deletions occurred de novo. Genetic alteration occurred de novo in 29.4% (5/17) of all reported Korean IP type III cases. From this study and literature review, we observed a striking difference of de novo occurrence rate (75% versus 12.5%, p = 0.032) between large genomic deletions and point mutations in East Asian population. CONCLUSIONS: Our data suggest that different POU3F4 mutations might show different recurrence rate in siblings of the IP type III families, especially in East Asian population. Genetic counseling should be provided accordingly.

A Case of Post-Traumatic Meniere's Disease.

There are only a few reports of post-traumatic Meniere's disease and there is few literature that contains detailed data associated with the disease. We report a case of post-traumatic Meniere's disease. He suffered from tinnitus, fluctuating sensorineural hearing loss, and recurrent vertigo. Symptomatic medical treatment was not helpful and neither was soft tissue plugging around the oval and round windows during exploratory tympanotomy. Three months after soft tissue plugging, endolymphatic sac decompression surgery was performed. The patient's symptoms improved markedly thereafter. The clinical significance of post-traumatic Meniere's disease is described and we present a brief review of the literature.

Audiological and surgical evidence for the presence of a third window effect for the conductive hearing loss in DFNX2 deafness irrespective of types of mutations.

The objective of this study was to clarify the cause of the air-bone gap in incomplete partition (IP) type III cases according to the POU3F4 gene (DFNX2) mutation type. A retrospective analysis of patient medical records was done in a tertiary referral medical center. Five IP type III patients proved to be carrying a mutation in or affecting POU3F4. The hearing and the middle ear status at either exploratory tympanotomy or cochlear implantation from these DFNX2 cases was reviewed. Four of five unrelated IP type III patients harbored a point mutation of POU3F4 and the fifth patient carried a large genomic deletion upstream to POU3F4. Two of the four DFNX2 patients carrying a point mutation had moderate to severe mixed hearing loss with a substantial amount of air-bone gap. These patients underwent exploratory tympanotomy to identify the cause of their hearing loss. The other three patients, including one carrying a large deletion, had profound hearing loss at presentation and received a cochlear implant. In the exploratory tympanotomy group with a substantial amount of air-bone gap and a point mutation (n = 2), one patient had a perfect ossicular chain with normal mobility, a positive ipsilateral stapedial reflex, and a positive round window reflex. In the cochlear implantation group (n = 3), we found a stapes with normal mobility and a positive round window reflex in one patient who harbored a large genomic deletion upstream to POU3F4. We concluded that the probable presence of the third window effect is not limited to the particular type of POU3F4 mutation.

Clinical characteristics of patients with narrow bony cochlear nerve canal: is the bilateral case just a duplicate of the unilateral case?

OBJECTIVES/HYPOTHESIS: To evaluate whether there is a genetic contribution to the development of narrow bony cochlear nerve canal and analyze the differences between unilateral and bilateral narrow bony cochlear nerve canal. STUDY DESIGN: Retrospective review. METHODS: Patients diagnosed with unilateral (n=44) or bilateral (n=13) sensorineural hearing loss with narrow bony cochlear nerve canals from October 2004 to July 2011 were evaluated. The segregation ratio of hearing loss among siblings of probands was calculated. The differences in segregation ratio and other clinical characteristics were also compared. RESULTS: The segregation ratio of hearing loss in siblings of patients with unilateral narrow bony cochlear nerve canal was close to zero, whereas the ratio in siblings of patients with bilateral canals was 20%. Internal auditory canal narrowing was significantly more frequently combined in unilateral than bilateral narrow bony cochlear nerve canal cases. Cochlear nerve deficiency based on magnetic resonance imaging was found in 63.6% of bilateral narrow bony cochlear nerve canal cases. Milder hearing loss was exclusively detected in bilateral cases. CONCLUSIONS: Narrow bony cochlear nerve canal is not likely to have a simple Mendelian inheritance pattern, especially in unilateral cases. The contribution of a Mendelian genetic etiology to bilateral narrow bony cochlear nerve canals remains elusive. More frequent coexistence of internal auditory canal narrowing among unilateral narrow bony cochlear nerve canal cases suggests that bilateral narrow bony cochlear nerve canals are not just a duplicate of the unilateral narrow bony cochlear nerve canal.

Facial nerve aberrations encountered during cochlear implantation.

CONCLUSION: In patients with cochleovestibular malformations, surgeons should always consider the possibility of an aberrant course or shape of the facial nerve (FN). In our series, the majority of cases could be successfully implanted without major complications through careful handling of the FN and ossicles. OBJECTIVES: To assess the frequency, type, and surgical implications of FN aberrations encountered during cochlear implantation (CI). METHODS: Medical records and radiologic findings of 972 patients who underwent CI from 1988 to 2009 were reviewed retrospectively. Radiologic and intraoperative findings demonstrating malformations of the FN, ossicles, and inner ear were evaluated. Intraoperative events and postoperative FN outcome were reviewed. RESULTS: Seven patients (0.7%) had an aberrant course or shape of the FN during CI. All these patients were pediatric. Four showed abnormal anterior or inferior location of the vertical segment, two had a bifurcated horizontal or vertical segment, and one had an inferiorly located horizontal segment. All seven patients displayed combined malformations of the cochlea and had malformed or missing ossicles. CIs were successfully performed via the facial recess approach and cochleostomy. There was no postoperative FN paralysis except in one case with immediate iatrogenic FN paralysis that recovered completely 1 month after FN decompression.

Stage progression of congenital cholesteatoma in children.

This study aimed to investigate the most prevalent stage in each age-group of children with congenital cholesteatoma (CC) and verify the correlation between the stage and the age of the patients for the type of CC ("closed" keratotic cyst and "open" infiltrative types). Patients diagnosed with CC between 2004 and 2009 (n = 156; 116 boys and 40 girls; mean age, 5 years and 5 months; range, 12 months-16 years and 7 months) were enrolled retrospectively. Assessment of stage and type of CC was performed with preoperative high-resolution temporal bone computed tomography and intraoperative findings. The stage of CC was determined using Potsic's staging system classified into four stages according to ossicular involvement and mastoid extension. The patients consisted of groups divided on the basis of a 2-year interval. The prevalence of stage I began to decline from the age-group of 1-2 years. In contrast, the prevalence of stages III increased from the age-group of 3-4 years and that of stage IV from the age-group 5-6 years. The prevalence of "open" infiltrative type CC increased from approximately the age of 7 years, thus showing some correlation with age but not with stage. The prevalence of CC with the advanced stage increased since the age of 2 years. Therefore, the importance of early diagnosis and treatment of CC should be more emphasized.

Anxiolytics reduce residual dizziness after successful canalith repositioning maneuvers in benign paroxysmal positional vertigo.

CONCLUSIONS: This study suggests that adjuvant anti-anxiety medication may be helpful for patients with benign paroxysmal positional vertigo (BPPV) even after a successful canalith repositioning procedure (CRP). OBJECTIVE: Although the CRP is an effective treatment for BPPV, many patients suffer from persistent dizziness despite successful CRPs. The aim of this study was to evaluate the effect of adjuvant anxiolytic medication on residual dizziness after successful CRP. METHODS: Between January 2011 and March 2011, 73 patients were diagnosed with BPPV and they underwent successful treatment with CRPs. The patients were randomly assigned to either the medication group or the control group. The patients in the medication group were prescribed low dose etizolam for 2 weeks whereas the patients in the control group were not prescribed any medication. All patients were scheduled to return 2 weeks after evaluation of subjective visual vertical. Subjective symptoms before and after CRP were measured using the Dizziness Handicap Inventory (DHI) and the Activities-specific Balance Confidence (ABC) scale. RESULTS: Both groups demonstrated a significant improvement in DHI scores. However, the medication group showed significantly greater decrease in the functional (p = 0.018) and emotional (p = 0.030) subscale scores, as well as in the total DHI (p = 0.038) score.

Surgical management options and postoperative functional outcomes of petrous apex cholesteatoma.

CONCLUSION: In cases of petrous apex (PA) cholesteatoma, radical removal should be prioritized over an unreasonable sparing of hearing or facial symmetry. Nevertheless, for patients with serviceable hearing and spared inner ear structures, conservative approaches may be applicable. Restoration of facial nerve (FN) function is achievable by reanimation procedures. OBJECTIVES: To analyze clinical manifestations, surgical techniques, and postoperative functional results of PA cholesteatoma. METHODS: From 1987 to 2010, 13 cases of PA cholesteatoma underwent operations. Clinical, audiological, and radiological findings, surgical approach, and postoperative functional outcomes were analyzed retrospectively. RESULTS: The most common symptoms were hearing loss and FN paralysis. All PA cholesteatomas extended to the middle ear; 10 (76.9%) through the anterior-superior route, whereas the other 3 (23.1%) were through the posterior-superior route. In 10 patients, labyrinthectomy was inevitable because of inner ear invasion. However, three limited cholesteatomas were removable via the middle cranial fossa or transmastoid approach without changing the bone-conduction threshold. Among six cases with FN paralysis, one underwent interposition graft, two FN decompression, and the others hypoglossal-FN anastomosis. One FN decompression case recovered to House-Brackmann grade I, and all other reanimation cases finally presented with grade IV. There were no major complications or recurrences during the follow-up period.

International classification of reliability for implanted cochlear implant receiver stimulators.

OBJECTIVE: To design an international standard to be used when reporting reliability of the implanted components of cochlear implant systems to appropriate governmental authorities, cochlear implant (CI) centers, and for journal editors in evaluating manuscripts involving cochlear implant reliability. STUDY DESIGN: The International Consensus Group for Cochlear Implant Reliability Reporting was assembled to unify ongoing efforts in the United States, Europe, Asia, and Australia to create a consistent and comprehensive classification system for the implanted components of CI systems across manufacturers. SETTING: All members of the consensus group are from tertiary referral cochlear implant centers. INTERVENTIONS: None. MAIN OUTCOME MEASURE: A clinically relevant classification scheme adapted from principles of ISO standard 5841-2:2000 originally designed for reporting reliability of cardiac pacemakers, pulse generators, or leads. RESULTS: Standard definitions for device failure, survival time, clinical benefit, reduced clinical benefit, and specification were generated. Time intervals for reporting back to implant centers for devices tested to be "out of specification," categorization of explanted devices, the method of cumulative survival reporting, and content of reliability reports to be issued by manufacturers was agreed upon by all members. The methodology for calculating Cumulative survival was adapted from ISO standard 5841-2:2000. CONCLUSION: The International Consensus Group on Cochlear Implant Device Reliability Reporting recommends compliance to this new standard in reporting reliability of implanted CI components by all manufacturers of CIs and the adoption of this standard as a minimal reporting guideline for editors of journals publishing cochlear implant research results.

Role of surgical management in temporal bone fibrous dysplasia.

CONCLUSION: Surgical treatment of fibrous dysplasia of the temporal bone (TBFD) revealed relatively good outcomes. Also, active surgical intervention is necessary to prevent the formation of secondary cholesteatoma or other complications. In particular, FD involving the inner ear may require greater caution when deciding on the resection area and timing of surgery. OBJECTIVES: The purpose of this study was to analyze clinical manifestations of TBFD and its surgical outcomes. PATIENTS AND METHODS: Nine patients diagnosed with TBFD after surgery at three tertiary referral centers were included. Pre- and postoperative clinical, audiological, and radiological findings were analyzed retrospectively. RESULTS: Most patients (88.9%) showed FD involving the mastoid and adjacent area. Radiological patterns consisted of six pagetoid and three sclerotic forms; seven patients were monostotic, whereas two patients were polyostotic. Canalplasty (CP) was performed in six cases and intact canal wall mastoidectomy was performed in three of them; hearing gain was achieved in five and one patient maintained preoperative normal hearing. Two patients underwent revision CP and showed no recurrence. In addition, one case had secondary cholesteatoma formed by an obstruction of the external auditory canal; another with a fast-growing cystic mass in the petrous bone underwent transmastoid labyrinthectomy and had no recurrence for 11 months.