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.

Auditory brainstem responses to CE-Chirp® stimuli for normal ears and those with sensorineural hearing loss.

OBJECTIVE: Evaluation of the characteristic differences between click-and CE-Chirp-evoked auditory brainstem responses (ABRs) in normal hearing and sensorineural hearing loss. DESIGN: A prospective study. Ears with normal hearing and with sensorineural hearing loss were evaluated. Pure-tone audiometry and click-and CE-Chirp evoked ABRs exams were conducted for all ears. Visual detection levels, wave-V amplitudes, and latencies of the ABRs were assessed. STUDY SAMPLE: Twenty-two ears with normal hearing and 22 ears with sloping type sensorineural hearing loss were examined. RESULTS: In normal-hearing ears, mean amplitudes were larger for CE-chirps than for clicks at all intensities until 80 dB nHL, at which the amplitudes dropped off, presumably due to upward spread of excitation. In ears with sensorineural hearing loss, however the drop-off was less significant at 80 dB nHL. Comparisons with pure-tone audiometry findings revealed ABRs to CE-Chirps to correlate at 0.5, 1, 2, and 3 kHz, and to clicks at 1, 2, 3, and 4 kHz. CONCLUSIONS: The CE-Chirp has advantages over clicks for examining normal ears. However, under high-level stimulation, these advantages are no longer present. In ears with sensorineural hearing loss, the upward spread of excitation is less prominent. The CE-Chirps results correlate significantly to low frequency audiometric findings at 0.5 kHz, while clicks do not.

Destabilization and mislocalization of POU3F4 by C-terminal frameshift truncation and extension mutation.

Most X-linked nonsyndromic hearing loss is caused by various types of mutations of the POU domain class 3 transcription factor 4 gene (POU3F4). We found five unique missense and frameshift truncation and extension mutations in Korean patients. Two missense mutations (p.Thr211Met and p.Gln229Arg) disturbed transcriptional activity. Two frameshift extension mutations (p.Thr354GlnfsX115 and p.X362ArgextX113) were located outside of POU domain and nuclear localization signal (NLS) at the C-terminus. POU3F4 protein levels were low and could be restored by MG132, a proteasome inhibitor, in vitro. These mutant POU3F4 proteins were exclusively localized to the cytoplasm and did not have transcriptional activity. Frameshift mutation (p.Leu317PhefsX12) in POU3F4 leads to the truncation of the C-terminal 44 amino acids spanning the POU domain and NLS. This frameshift truncation mutant protein was located in both the nucleus and cytoplasm and was present at low protein levels. This mutant was also transcriptionally inactive, even in the presence of MG132. From these results, we conclude that frameshift truncation and extension mutations in the C-terminus of POU3F4 lead to cytoplasmic localization and subsequent proteosomal degradation due to structural aberrations, which cause transcriptional inactivity and thus nonsyndromic hearing loss.

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.

Lobule-specific membrane excitability of cerebellar Purkinje cells.

Cerebellar Purkinje cells (PCs) are the sole output of the cerebellar cortex and function as key to a variety of learning-related behaviours by integrating multimodal afferent inputs. Intrinsic membrane excitability of neurons determines the input-output relationship, and therefore governs the functions of neural circuits. Cerebellar vermis consists of ten lobules (lobules I-X), and each lobule receives different sensory information. However, lobule-specific differences of electrophysiological properties of PC are incompletely understood. To address this question, we performed a systematic comparison of membrane properties of PCs from different lobules (lobules III-V vs. X). Two types of firing patterns (tonic firing and complex bursting) were identified in response to depolarizing current injections in lobule III-V PCs, whereas four distinct firing patterns (tonic firing, complex bursting, initial bursting and gap firing) were observed in lobule X. A-type K(+) current and early inactivation of fast Na(+) conductance with activation of 4-aminopyridine-sensitive conductances were shown to be responsible for the formation of gap firing and initial bursting patterns, respectively, which were observed only in lobule X. In response to current injection, PCs in lobule X spiked with wider dynamic range. These differences in firing pattern and membrane properties probably contribute to signal processing of afferent inputs in lobule-specific fashion, and particularly diversity of discharge patterns in lobule X, as a part of the vestibulocerebellum, might be involved in strict coordination of a precise temporal response to a wide range of head movements.

Implication of bony cochlear nerve canal on hearing in patients with congenital unilateral sensorineural hearing loss.

This study was designed to evaluate the dimensions of the bony cochlear nerve canal (BCNC) in congenital unilateral sensorineural hearing loss (USNHL) patients with normal inner ears, and to analyze the correlation between BCNC and hearing status. Medical records and temporal bone computed tomography (TBCT) findings of 380 patients who visited Seoul National University Hospital between January 1999 and December 2007 were reviewed retrospectively. The length and width of the BCNC at the fundus of the internal auditory canal were measured in millimeters based on the axial view of TBCT for three ear groups: normal inner ears of control group subjects (group A, 179 ears), normal inner ears on the contralateral side of USNHL patients (group B, 201 ears), and the affected inner ears in USNHL (group C, 201 ears). The mean values of length and width in group C (0.79 ± 0.36 and 1.58 ± 0.83 mm, respectively) were significantly smaller than those in group A (1.07 ± 0.10 and 2.38 ± 0.28 mm, respectively; p < 0.001) and group B (1.04 ± 0.23 and 2.33 ± 0.39 mm, respectively; p < 0.001). The receiver operating characteristic curves for BCNC were conducted to estimate the cutoff values from which the proportions of profound hearing loss increased, compared to those of mild to severe hearing loss. The areas under the curve were 0.487 ± 0.044 (p = 0.781) and 0.622 ± 0.041 (p = 0.011) for length and width, respectively. The cutoff value of width with a sensitivity of 90% for profound USNHL was 1.16 mm in otherwise normal inner ears. Clinicians would be recommended to take a close look at BCNC as one of the possible causes of hearing loss in otherwise normal inner ears of USNHL on TBCT.

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.

Early combination treatment with intratympanic steroid injection in severe to profound sudden sensorineural hearing loss improves speech discrimination performance.

The objective of the study was to determine the therapeutic efficacy of early combined treatment with intratympanic steroid injection (ITSI) in patients with severe to profound sudden sensorineural hearing loss (SSNHL), who did not respond to initial systemic steroid treatment (SST). The study design included historical controlled trials (retrospective analysis for the SST group). Patients (n = 73; 38 women and 35 men) diagnosed with severe to profound SSNHL at the time of their first visit in tertiary referral centers were recruited. Among these 73 patients, 30 patients who showed no early response within a week after the start of initial SST were prospectively included as the ITSI group. ITSI was performed twice a week for two consecutive weeks. In contrast, 43 patients with the same condition who did not receive ITSI were retrospectively included as the SST group. For each group, pure-tone audiogram (PTA) and speech discrimination score (SDS) tests were performed before SST and more than 2 months after termination of treatment in each group. After termination of each treatment, the final average gain on PTA showed no significant difference between the two groups; however, the final average gain of SDS demonstrated a significantly better recovery in the ITSI group. ITSI as part of an early combined therapy represents an effective treatment in terms of the improvement of SDS in severe to profound SSNHL showing no early response to initial SST.

P2Y4-mediated regulation of Na+ absorption in the Reissner's membrane of the cochlea.

The epithelial cells of Reissner's membrane (RM) are capable of transporting Na(+) out of endolymph via epithelial Na(+) channel (ENaC). However, much remains to be known as to mechanism of regulation of Na(+) absorption in RM. We investigated P2Y signaling as a possible regulatory mechanism of ENaC in gerbil RM using voltage-sensitive vibrating probe technique and immunohistochemistry. Results showed that UTP induced partial inhibition of the amiloride-sensitive short-circuit current but did not change short-circuit current when applied in the presence of amiloride. The inhibitory effect of UTP was not completely reversible in minutes. The response to UTP was inhibited by reactive blue-2 and 2',3'-O-(4-benzoylbenzoyl)adenosine 5'-triphosphate but not by suramin or pyridoxalphosphate-6-azophenyl-2', 4'-disulfonic acid, which indicates this P2Y receptor as the P2Y(4) subtype. The phospholipase C (PLC) inhibitors 1-[6[[(17beta)-3-methoxyestra-1,3,5(10)-trien-17-yl]amino]hexyl]-1H-pyrrole-2,5-dione and 1-O-octadecyl-2-O-methyl-rac-glycero-3-phosphocholine markedly inhibited the effect of UTP on ENaC. In contrast, neither modulation of protein kinase C nor application of 2-aminoehoxydiphenyl borate affected P2Y(4)-mediated inhibition of ENaC. Immunoreactive staining for P2Y(4) was observed in the RM, apical membrane of stria vascularis, spiral ligament, and organ of Corti, including outer hair cell, inner hair cell, outer pillar cell, Deiters' cell, and Hensen cell. These results suggest that the physiological role of P2Y(4) receptor in RM is likely to regulate Na(+) homeostasis in the endolymph. The acute inhibition of ENaC activity by activation of P2Y(4) receptor is possibly mediated by decrease of phosphatidylinositol 4,5-biphosphate in the plasma membrane through PLC activation.

Vestibular function analysis of sudden sensorineural hearing loss with dizziness.

BACKGROUND: A considerable number of patients with sudden sensorineural hearing loss (SSNHL) have been reported to have dizziness. OBJECTIVE: To analyze vestibular functions and identify the clinical characteristics of SSNHL with dizzinessMETHODS:71 patients with SSNHL who complained of dizziness were investigated retrospectively. The patients underwent vestibular function tests consisting of video-nystagmography, video head impulse test and vestibular evoked myogenic potentials. RESULTS: Among 35 patients with spontaneous nystagmus (SN), 21 showed ipsilesional posterior canal gain deficit, 11 showed a gain deficit in the posterior canal only and 9 showed both horizontal and posterior canal gain deficit in video head impulse test. In only one subject with bilateral horizontal canal gain deficit with contralesional SN, AICA infarction was observed.Among 36 patients without SN, 10 were diagnosed as benign paroxysmal positional vertigo and 5 had ipsilesional canal paresis in caloric test. Other 21 patients showed no abnormal signs in vestibular function tests. CONCLUSIONS: In VFT analysis of SSNHL with dizziness, diverse patterns were identified. In the absence of SN, no definite vestibular organ involvement was the most frequent. But in the case with SN, posterior canal deficit was most common and cerebellar ischemic stroke was rare.

Comparing the Suppression Head Impulse Paradigm and the Head Impulse Paradigm in Vestibular Neuritis.

OBJECTIVE: To compare the results of suppression head impulse paradigm (SHIMP) and head impulse paradigm (HIMP) in acute vestibular neuritis (AVN). STUDY DESIGN: Retrospective study. SETTING: Tertiary otology clinic. PATIENTS AND INTERVENTIONS: We tested 21 patients with AVN with the HIMP, SHIMP, and caloric tests, and we analyzed the relationships between the tests' results. MAIN OUTCOME MEASURES: For this study, we adopted vestibulo-ocular reflex (VOR) gains in the SHIMP and HIMP, peak saccade velocity (PSV) of SHIMP which is another indicator of residual vestibular function, and canal paresis of caloric test. RESULTS: VOR gains showed significant correlation (R = 0.926, p < 0.001) between the SHIMP and HIMP, but VOR gains were slightly lower in the SHIMP than in the HIMP (mean difference 0.07 ±â€Š0.09, p < 0.001). The difference between the HIMP and SHIMP gains was slightly larger on the affected side (0.10 ±â€Š0.09) than on the healthy side (0.03 ±â€Š0.09). The PSV of SHIMP had significant correlation with HIMP gain and canal paresis. Sixteen of 21 patients showed 100% ipsilesional caloric canal paresis, and eight (50%) of them showed no anti-compensatory saccade (direction toward head rotation) in the SHIMP. However, they showed not extremely low VOR gain but variable VOR gain. CONCLUSION: The new parameters of SHIMP might be used as complement for evaluating vestibular function in AVN. However, the clinical impact of the saccades of SHIMP in AVN has not been revealed clearly yet. This question should be investigated in further studies.

Management of complications in cochlear implantation.

CONCLUSION: Cochlear implantation (CI) remains a safe procedure with a low complication rate. Nevertheless, advances in surgical techniques and the optimization of treatment modalities would further reduce complications. OBJECTIVE: To assess the complications of CI and describe the management of each complication encountered at our hospital. PATIENTS AND METHODS: This study involved 720 patients that underwent implantation from November 1988 through April 2007. Mean age at implantation was 13.6 years (range 12 months to 83 years). Patients were followed up regularly with a mean follow-up of 42 months (range 4-81 months). RESULTS: No death was attributed to device implantation. Major complications included: device failure in 12 patients, misplaced electrodes in 4, hematoma in 3, flap necrosis in 3, magnet displacement in 2, facial nerve twitching in 2, electrode exposure in 2, external auditory canal keratoma in 1, immediate facial nerve paralysis in 1, and skin flap irritation due to the suture material in 1. The overall major complication rate was 4.2% (30/720), and there were 37 minor complications (5.1%), which were resolved by conservative treatment or minor intervention. Minor complications included temporary vertigo in 17 patients, facial twitching in 11, hematoma in 4, subcutaneous emphysema in 3, and temporary facial nerve paralysis in 2.

Does the Width of the Bony Cochlear Nerve Canal Predict the Outcomes of Cochlear Implantation?

A narrow bony cochlear nerve canal (BCNC) is associated with sensorineural hearing loss necessitating cochlear implantation (CI). This study evaluated the implications of BCNC width for post-CI outcomes. A total of 56 children who had received CIs were included. The patients were divided into three groups according to the width of the BCNC (Group 1: diameter < 1.4 mm, n = 17; Group 2: diameter 1.4-2.0 mm, n = 14; Group 3: diameter > 2.0 mm, n = 25). The post-CI speech performances were compared among the three groups according to BCNC width. The correlation between BCNC width and post-CI speech performance was evaluated. Logistic regression analysis was also performed to investigate factors that can impact post-CI speech performance. Cochlear nerve deficiency (CND) occurred more frequently in Group 1. Groups 1 and 2 had significantly worse post-CI outcomes. Patients with intact cochlear nerves had significantly better post-CI outcomes than those with CND. When the cochlear nerve was intact, patients with a narrower BCNC showed less favorable results. Therefore, patients with either a narrow BCNC or CND seemed to have poorer outcomes. A narrow BCNC is associated with higher CND rates and poor outcomes. Measurement of BCNC diameter may help predict CI outcomes.

Changes in P2Y4 receptor expression in rat cochlear outer sulcus cells during development.

Extracellular adenosine triphosphate (ATP) released from cellular sources plays an important role in variety of the cochlear physiologic processes. The primary purinergic receptor subtype in the cochlea is the P2X2 receptor, which is a subtype of P2X receptor. This receptor appears to mediate a protective decrease in the electrical driving force in response to acoustic overstimulation. Outer sulcus cells (OSCs) in the cochlear lateral wall appear to maintain an adequate K+ concentration in the cochlear endolymph in response to varying intensities of auditory stimulation. However, little is known about developing OSCs. The purpose of this study was to investigate subtypes of purinergic receptors in developing rat OSCs using a voltage-sensitive vibrating probe. Results showed that only two P2 receptors (P2Y4 and P2X2) contributed to the regulation of short circuit currents in neonatal OSCs. ATP increased cation absorption via apical nonselective cation channels after activating P2Y4 receptors in early neonatal OSCs. P2Y4 expression rapidly declined postnatally and reached near adult levels on postnatal day 14. P2X2 was co-expressed with P2Y4 in early neonatal OSCs. Temporal changes in P2Y4 during OSC development might be involved in the establishment of the endolymphatic ion composition needed for normal auditory transduction and/or specific cellular differentiation.

Diverse clinical and laboratory manifestations of bilateral vestibulopathy.

OBJECTIVES/HYPOTHESIS: To identify the clinical and laboratory characteristics of bilateral vestibulopathy (BVP) on the video head impulse test (vHIT). STUDY DESIGN: Retrospective chart analysis. METHODS: During 23 months, 1,789 patients with dizziness underwent vHIT in our tertiary referral hospital. Of these patients, 65 (3.6%) patients had bilaterally positive catch-up saccades. Based on the caloric test, 15 (group 1) had bilateral caloric weakness, 13 (group 2) had unilateral caloric weakness, and 37 (group 3) had normal caloric responses on both ears. We collected data on these patients regarding demographics, symptoms, gain, and type of saccade on horizontal canal plane vHIT, as well as gain and time constant on velocity step of the rotatory chair test. RESULTS: The average age of group 2 (70.38 ± 11.96 years) and group 3 (69.03 ± 11.01 years) were significantly older than that of group 1 (54.80 ± 11.96 years) (P = 0.029, P = 0.003, respectively). Although all patients had bilaterally positive vHIT, 10 of 15 in group 1 were finally diagnosed as classical BVP by clinical features. On comparison of average gain on bilateral horizontal vHIT, groups 2 (0.71 ± 0.17) and 3 (0.80 ± 0.14) had higher gain compared to group 1 (0.45 ± 0.22) (P = 0.001, P = 0.000, respectively). On velocity step test, time constant and gain of group 3 (11.60 ± 3.07, 0.49 ± 0.13) was significantly higher than those of group 1 (4.92 ± 1.36, 0.22 ± 0.17) (P = 0.000, P = 0.004, respectively). On the receiver operating characteristic curve analysis, vHIT alone seemed to be a discordant method for diagnosis of BVP compared to the caloric and step velocity tests. CONCLUSION: About 3.6% patients with dizziness showed bilateral vestibular ocular reflex deficit during high-frequency acceleration, which was prevalent especially in elderly patients. Also, positive bilateral vHIT does not always correlate with caloric or rotatory chair test results. This may imply that a diverse spectrum of vestibulopathies exist according to the stimulation frequency of deficit. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:E42-E49, 2017.

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.

Reactive blue 2, an antagonist of rat P2Y4, increases K+ secretion in rat cochlea strial marginal cells.

Extracellular ATP decreases K+ secretion in strial marginal cells via apical P2Y4 receptors. We investigated the effect of reactive blue 2 (RB-2), an antagonist of rat P2Y4, on rat strial marginal cells using a voltage-sensitive vibrating probe. The application of RB-2 increased K+ secretion in a dose-dependent manner, and this increase was characterized as a peak followed by a partial relaxation to a steady-state. Moreover, this response was similar to that caused by 4,4'-diisothiocyanatostilbene-2,2'-disulfonic acid (DIDS). Suramin had no similar effect, except at high concentration. Thus, we tested the effects of these chemicals on P2Y4 receptors in strial marginal cells. Both RB-2 and DIDS had antagonistic activities at P2Y4, and the antagonist potency at P2Y4 paralleled the potency of K+ secretion. Interestingly, 2'- and 3'-O-(4-benzoyl-benzoyl)adenosine 5'-triphosphate (BzATP) exhibited an agonistic effect at P2Y4 receptor, which was blocked by RB-2, but not by pyridoxalphosphate-6-azophenyl-2',4'-disulfonic acid (PPADS). Based on these results, we speculate that direct and/or indirect inhibitory mechanisms between P2Y4 and KENQ1/KCNE1 K+ channels exist in strial marginal cell.

Analysis of the long-term hearing results after the surgical repair of aural atresia.

OBJECTIVES: Careful surgical candidate selection guarantees a high probability of serviceable hearing postoperatively in congenital aural atresia (CAA) patients. The authors analyzed hearing results after CAA surgery with long-term follow-up (F/U) with respect to several clinical factors. STUDY DESIGN: This was a retrospective study. METHODS: The medical records of 93 CAA patients (100 ears) who underwent operations from January 1987 through December 2002 at Seoul National University Hospital were reviewed. Mean duration was 56.9 months. The authors evaluated the results of hearing after surgery over 3 year F/U with a view to clarifying the factors accounting for unsuccessful results. RESULTS: Approximately 64% of patients treated surgically achieved a considerable hearing gain over long-term F/U. Postoperative hearing remained relatively stable over the period from 6 months to 3 years postoperatively, yielding only 2.75 dB of aggravation. However, hearing results in revision cases deteriorated with time, which led to statistically higher air-conduction thresholds than those of primary cases at the 1 and 3 year F/Us. Resultantly, only 26.6% of patients having achieved a poor hearing gain post first surgery benefited from revision audiologically. The severity of microtia was found to help predict poor long-term hearing outcomes after CAA surgery. CONCLUSIONS: Nonrevision cases and cases with mild microtia appear to have acceptable and stable long-term hearing results. Disappointing long-term hearing results in revision, and severe microtia cases should lead to considerations of alternative options in these cases, such as bone-anchored hearing aids, which offer reliable and stable results.

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.