Factors Predicting Subjective Satisfaction for Successful Hearing Aid Adaptation.

(1) Background: For successful hearing aid (HA) use during daily life, an objective parameter reflecting the subjective satisfaction is required. We explored the aided hearing status, hearing in noise test (HINT) scores, and subjective outcomes to predict performance improvements in everyday living. (2) Methods: A total of 406 patients with hearing loss (HL) who were prescribed HAs were included and were divided into two groups according to the symmetricity of HL. The relationship between audiometric data and subjective questionnaires under unaided and aided (3 months) conditions were investigated. (3) Results: Patients with symmetric HL showed a significant HINT signal-to-noise ratio (SNR) change and significant increase in their subjective satisfaction questionnaire score under the bilateral HA condition. On the other hand, the HINT SNR change and subjective questionnaire score showed various significances according to the side of HA (better or worse hearing) in asymmetric HL HINT SNR and was significantly correlated with the subjective questionnaire score in symmetric HL patients and AHL patients with unilateral HA in their better ear. (4) Conclusions: The HINT SNR improvement after long-term HA use could be an effective tool for predicting the subjective satisfaction of HA use and HA validation.

Mechanism underlying and prevention of electrode migration in cochlear implants.

PURPOSE: We investigate the clinical manifestations, mechanisms, and methods of preventing electrode migration in Cochlear Implantation (CI) patients, based on our practical experience with this problem. STUDY DESIGN: This is a retrospective study in a single center. METHODS: We retrospectively reviewed electrode migration in 4 (0.75%) of 532 patients who underwent CI at our tertiary institution from January 2002 to December 2022. Pre- and post-operative pure-tone audiometry, word recognition score, aided functional gain test, and sound field speech intelligibility test were evaluated. RESULTS: All four patients underwent CIs with the straight electrode type. The following events or symptoms were observed in the patients before confirming electrode migration: an increase in high-frequency thresholds during the post-operative aided functional gain test and a decline in scores on the sound field speech intelligibility test. Electrode migration was confirmed through transocular view X-ray or temporal bone computer tomography. Two patients showed coiled electrodes within the mastoid cavity; while in the others, the electrodes were observed to be floating inside the cavity. To prevent migration of electrodes due to these issues, we mixed bone paste collected during the drilling of the mastoid cavity with glue and used it to secure the electrodes in place. CONCLUSION: Electrode migration can result in a decrease in hearing ability and may necessitate a revision surgery to adjust the electrode placement. The main factors affecting electrode placement include the position of electrode within the mastoid cavity and the elasticity of straight electrodes. It is important for surgeons to recognize the factors that increase the risk of electrode migration and to take preventative measures to reduce this risk.

Objective and subjective efficacy of hearing aids in patients with mild-to-moderate unilateral hearing loss: a prospective study.

PURPOSE: In patients with unilateral sensorineural hearing loss (USNHL), we explored both objective functional audiological gains and subjective satisfaction, indicating when a unilateral hearing aid is valuable. METHODS: Thirty-seven patients with mild-to-moderate USNHL (mean pure-tone thresholds between 25 and 70 dB) were prescribed unilateral hearing aids. Functional gain, the aided speech discrimination score (SDS), the Hearing in Noise Test (HINT) score, and the sound localization test score were collected, and a questionnaire (the Hearing Handicap Inventory for the Elderly, HHIE) completed after 1, 2, and 3 months of hearing aid use. We classified the participants as having 'no handicap' (HHIE < 17), 'mild-to-moderate handicap' (17-42), and 'significant handicap' (> 42). RESULTS: The decrease in handicap afforded by unilateral hearing aids was largest in the 'significant handicap' group (the HHIE total score fell from 59.1 to 37.2; P = 0.007). There were no between-group differences in either functional gain or the aided SDS. Only the 'significant handicap' group evidenced an improved HINT score; the composite signal-to-noise ratio (SNR) fell from - 1.5 to - 2.2 dB [S/N] (P = 0.023). The HHIE usefully indicated when a hearing aid alleviated the discomfort of USNHL; patients with unaided HHIE scores ≥ 20 evidenced significant decreases in the composite SNR (- 1.7 to - 2.0 dB [S/N]; P = 0.045). CONCLUSIONS: When considering whether to prescribe a unilateral hearing aid for patients with mild-to-moderate USNHL, it is helpful to use the HHIE to evaluate discomfort. If the total score is ≥ 20, a hearing aid is appropriate.

Determination of Tympanostomy Tube Types for Otitis Media with Effusion in Patients with Cleft Palate: Comparison between Paparella Type 1 and Type 2 Tubes.

This study examined the effects of different types of tympanostomy tubes in pediatric patients undergoing cleft palate (CP) surgery in order to provide guidance for the proper insertion of tympanostomy tubes in the management of otitis media with effusion (OME). A total of 101 ears with middle ear effusion in 51 patients with CP were included in this study. Patients underwent palatoplasty and tympanostomy tube surgery at the same time. The type of tube inserted (Paparella type 1 or 2), the severity of CP, and types of palatoplasty surgeries were investigated. All patients were followed up for at least 6 months, and recurrence rates, complications, and reinsertion surgery were evaluated. The rate of OME recurrence after spontaneous tube extrusion was significantly higher in the type 1 group than in the type 2 group (44.3% vs. 19.4%, respectively, p = 0.016). Persistent eardrum perforation was more common in the type 2 group than in the type 1 group (41.9% vs. 12.9%, respectively, p = 0.001). The tube reinsertion rate was higher in the type 1 group than in the type 2 group (22.9% vs. 3.2%, respectively, p = 0.015). The tube reinsertion rate decreased to 8.6% in cases of palatoplasty with Sommerlad's technique, even with type 1 tube insertion, which was not significantly different from the reinsertion rate in the type 2 group (3.7%, p = 0.439). The Paparella type 1 tube would be a better choice in cases of palatoplasty performed using Sommerlad's technique, particularly considering the higher rate of persistent eardrum perforation after extrusion associated with the Paparella type 2 tube. Alternatively, a larger size type 2 tube may be considered in other surgeries to decrease the frequency of recurrence and tube reinsertion.

Relationship Between Facial Bone Fractures and the Risk of Posttraumatic Complications: A Hypothesis on the Cushion Effect of the Facial Skeletons in Temporal Bone Fractures.

BACKGROUND: This study investigated whether concomitant facial bone (FB) fractures reduce temporal bone (TB) injuries, such as posttraumatic facial palsy and vertigo, through an impact absorbing effect, so-called "cushion effect," in severe trauma patients. METHODS: A total of 134 patients with a TB fracture were included. They were divided into two groups according to their concomitant facial fractures: group I (no FB fracture) and group II (FB fracture). We compared clinical characteristics, such as brain injury, trauma severity, and complications of TB fracture, between the two groups. RESULTS: In group II, immediate facial palsy was more frequent (11.6% vs. 1.5% in group I), and the Injury Severity Score was higher (19.0 ± 5.9 vs. 16.7 ± 7.3, P = 0.020). Delayed facial palsy (12.3% in group I vs. 4.3% in group II) and posttraumatic vertigo (24.6% vs. 7.2%) occurred more often in group I. FB fractures significantly decreased the incidence of posttraumatic vertigo (odds ratio [OR], 0.276; 95% confidence interval [CI], 0.083-0.914). Intraventricular hemorrhage (OR, 20.958; 95% CI, 2.075-211.677), facial nerve canal injury (OR, 12.229; 95% CI, 2.465-60.670), and FB fractures (OR, 16.420; 95% CI, 1.298-207.738) increased the risk of immediate facial palsy. CONCLUSION: Concomitant FB fractures reduced the risk of the occurrence of delayed facial palsy and posttraumatic vertigo in injured patients with TB fracture. Particularly, an anterior force may be reduced by the cushion effect of the bony fracture.

Efficacy and Safety of Co-Administered St. John's Wort and Ginkgo biloba Extracts in Patients with Subjective Tinnitus: A Preliminary Prospective Randomized Controlled Trial.

It is widely accepted that extracts of St. John's wort (Hypericum perforatum) improve depressive symptoms, and tinnitus patients commonly presented with either mild depression or anxiety. We investigated whether co-administration of St. John's wort and Ginkgo biloba extracts can suppress tinnitus. Participants with subjective tinnitus aged 30-70 years were randomly assigned to the experimental (co-administration of St. John's wort and Ginkgo biloba extract; n = 20) or control (Ginkgo biloba extract only; n = 26) group for 12 weeks. Participants were blinded to the group assignments. After 12 weeks of treatment, no significant change in the minimum masking level on the tinnitogram was observed in either group. In the co-administration group, the Tinnitus Handicap Inventory (THI) score decreased from 34.7 (SD, 15.9) to 29.6 (16.0) (p = 0.102). However, the control group showed a significant decrease in THI score, from 30.5 (16.7) to 25.6 (17.1) (p = 0.046). Regarding the Short Form-36 Health Survey (SF-36), only the "Social Functioning" domain score changed significantly after extract co-administration, from 74.5 (21.5) to 83.9 (20.5) (p = 0.047). Co-administration of St. John's wort and Ginkgo biloba extracts did not improve the symptoms of subjective tinnitus compared to administration of Ginkgo biloba extract alone.

What is the most important factor to preserve hearing in lateral semicircular canal fistula surgeries, fistula size or bony structure?

PURPOSE: Cholesteatoma on lateral semicircular canal (LSCC) fistula > 2 mm in size is likely to be unmanipulated due to the risk of sensorineural hearing loss. However, the matrix can be successfully removed without hearing loss when it is > 2 mm. The purpose of the study was to evaluate surgical experience over the past 10 years and to suggest the important factor for the hearing preservation in LSCC fistula surgeries. METHODS: According to the fistula size and symptoms, 63 patients with LSCC fistula were grouped as follows: Type I (fistula < 2 mm), Type II (≥ 2 mm and < 4 mm without vertigo), Type III (≥ 2 mm and < 4 mm with vertigo), Type IV (≥ 4 mm), and Type V (any size fistula but with deafness at the initial visit). The cholesteatoma matrix was meticulously manipulated and removed by experienced surgeons. RESULTS: Only two patients completely lost their hearing after surgery (4.5%). However, the loss was inevitable because their cholesteatomas were highly invasive and there was also facial nerve canal involvement; thus, the bony structure of the LSCC was already destroyed by the cholesteatoma. Unlike these two Type IV patients, Type I-III patients, and those with a fistula size < 4 mm, did not lose their sensorineural hearing. If the structure of the LSCC was maintained, hearing loss did not occur even if the fistula size ≥ 4 mm. CONCLUSIONS: The preservation of the labyrinthine structure is more important than the defect size of the LSCC fistula. If the structure is intact, cholesteatoma matrices lying on the defect can be safely removed, even though the size of bony defect is large.

Surgical Outcomes of Simultaneous Cochlear Implantation and Intracochlear Schwannoma Removal.

OBJECTIVE: Intracochlear schwannoma is very rare, and complete loss of hearing is inevitable after the removal of this tumor. Here, we discuss cochlear implantation (CI) performed simultaneously with the removal of an intracochlear schwannoma. STUDY DESIGN: Retrospective single-center study. SETTING: Tertiary medical institute. METHODS: Simultaneous CI and intracochlear schwannoma removal were performed in 4 subjects. After subtotal cochleostomy, the tumors were removed meticulously, with preservation of the modiolus. A new slim modiolar electrode (Nucleus CI632) was placed in a manner that hugged the modiolus. The surgical outcomes of functional gain, word recognition score (WRS), sound localization, and hearing in noise and speech intelligibility tests were investigated. RESULTS: Intracochlear schwannomas were removed successfully from the 4 patients, with no remnant tumor. The mean aided hearing threshold 6 months after surgery was 25.0 ± 1.8 dB, and the mean-aided WRS with a 60 dB stimulus was 36.0 ± 18.8% (range 16%-60%). The Categorical Auditory Performance (CAP) score of the 3 single-sided deafness patients under contralateral ear masking was 7. The CAP score of the patient with bilateral sensorineural hearing loss was 6, which improved from a preoperative score of 0. CONCLUSION: When an intracochlear schwannoma does not completely invade the modiolus, CI with simultaneous tumor removal can be performed successfully, resulting in good hearing performance. A slim modiolar electrode can be placed stably at the modiolus after schwannoma removal.

Preliminary study to determine an optimal mode for favorable residual hearing at low frequencies: Full electrical stimulation, electric acoustic stimulation, and electrical complement.

Objective: In this prospective study, each subject experienced three modes electric acoustic stimulation (EAS), full electrical stimulation (FES), and electrical complement (EC), and the performance of each mode and subject preference were evaluated. Methods: Eight ears (seven patients) with successfully preserved residual hearing after cochlear implantation (CI) were included. EAS, FES, and EC programs were set up on each patient's device, and each mode was used for at least 1 h per day for a month. The Speech Intelligibility test, the Speech, Spatial and Qualities of Hearing Scale, and the Hearing in Noise test (HINT) results in each stimulation mode. Results: The mean monosyllabic word score (EAS: 90.3 ± 4.0; FES: 81.2 ± 16.1) and the mean sentence score (EAS: 98.3 ± 1.7; FES: 95.0 ± 3.0) were significantly higher in the EAS mode than in the FES mode. The mean bisyllabic word score (EAS: 95.6 ± 5.6; EC: 90.1 ± 5.6) was higher in the EAS mode than in the EC mode. In HINT, the signal-to-noise ratios under the noise front (EAS: 4.7 ± 2.5; FES: 7.9 ± 4.4) and noise composite conditions (EAS: 4.2 ± 2.7; FES: 6.6 ± 4.0) were significantly smaller in the EAS mode than in the FES mode. After trials of the three modes, five subjects preferred EAS, one preferred EC, and two preferred FES. Conclusion: Among the three stimulation modes, EAS produced slightly better results, and subjects generally preferred EAS (five of seven patients, 71.4%). The use of hearing aids before CI was considered an important factor in mode preference. FES may be preferred when CI was performed at a young age and subjects had little experience with hearing aids. However, adults may prefer EC over EAS if there was little or no hearing-aid use before CI.

Transtympanic soft tissue tympanoplasty can replace conventional techniques elevating tympanic membranes.

PURPOSE: Most traditional tympanoplasties require elevating the tympanic membrane (TM). These techniques are rather complicated and success rates are not perfect. Therefore, the authors developed a novel technique, transtympanic soft tissue (TST) tympanoplasty, which does not require raising eardrums, and evaluated its surgical efficiency compared to perichondrium underlay (PU) tympanoplasty. STUDY DESIGN: A retrospective study was conducted in a single center. METHODS: 152 cases who underwent TST tympanoplasty (n = 70) or PU tympanoplasty (n = 82) between 2011 and 2020 were included in the study. Perforation location, pure tone audiometry, complications, and closure rates were analyzed according to the size of the TM perforations: moderate perforation (25-40%, n = 100) and large perforation (≥ 40%, n = 52). RESULTS: For the moderate perforations, the closure rates of the TST (n = 45) and PU (n = 55) groups were 93.3% and 89.1%, respectively (p = 0.461), and even for the large perforations, the success rates were 88.0% in the TST group (n = 25) and 81.5% in the PU group (n = 27) (p = 0.515). The mean postoperative air-bone gap (ABG) values of the TST group for moderate and large perforations were 5.3 ± 5.8 dB and 6.6 ± 5.7 dB, respectively. There was no significant difference in postoperative ABG between the two surgical procedures (p > 0.05). The total operation time for TST tympanoplasty was significantly shorter than that for PU tympanoplasty (p = 0.002). CONCLUSIONS: TST tympanoplasty is considered a novel, simple technique to replace traditional tympanoplasty techniques involving raising eardrums, even for large-sized perforations.

Sound localization in patients with a unilateral hearing aid: Discordance between the right and left ears.

Objectives: Patients with unilateral hearing loss (UHL) have difficulty in recognizing the direction of a sound. Previous studies have shown that hearing aids (HAs) could improve the directional perception of sound. In this study, we analyzed the results of sound localization tests in patients using a unilateral HA. Methods: All patients with UHL who had performed sound localization tests since 2018 were included in this study. Sound localization tests, functional gain tests, and the speech discrimination scores (SDSs) were analyzed. The tests were obtained at 1-, 3-, and 6-month after fitting the HA. Results: Of the 32 patients with UHL, 13 were right-sided and 19 were left-sided. After 6 months of using a HA, the results of the sound localization test were significantly better in patients with right than left UHL (percent correct [PCT], 61.9 ± 24.0% vs. 37.9 ± 24.6%, p = .011; mean absolute error (MAE), 41.4 ± 23.9° vs. 65.5 ± 28.6°, p = .018; root-mean-square error (RMS), 25.8 ± 17.6° vs. 48.8 ± 24.5°, p = .007]. The aided SDSs were not different between the two groups (78.7 ± 16.5% vs. 77.2 ± 18.5%, p = .825). Conclusion: The side of the hearing loss could have a substantial effect on sound localization in UHL patients using a HA. Sound localization test results should be interpreted with the consideration of this discordance. Level of Evidence: 4.

Comparison of surgical results between 'atticosinuplasty' and canal wall up mastoidectomy for early-stage cholesteatoma.

BACKGROUND: We devised a surgical technique called 'atticosinuplasty' (AS) for the treatment of early-stage cholesteatoma. OBJECTIVE: This study analyzed the adequacy and applicability of AS compared to canal wall-up mastoidectomy (CWU) in patients treated for early-stage cholesteatoma. MATERIALS AND METHODS: A total of 187 patients with either AS (n = 89) or CWU (n = 98) were compared in terms of postoperative hearing outcome, recurrence and re-operation rate, and radiologic outcome. RESULTS: Hearing gain was significant in the AS group (p < 0.001) but not in the CWU group. Air conduction change, air-bone gap (ABG) change, and ABG closure did not significantly differ between the two groups. The frequencies of cholesteatoma recurrence and revision ossiculoplasty were also similar. Of the 32 patients in the AS group with only attic/sinus involvement preoperatively, 20 (62.5%) showed no haziness and 6 (18.8%) had haziness extending to the mastoid postoperatively. Among the 67 patients in the CWU group who had haziness extending to the mastoid preoperatively, in 54 (80.6%) there was no change postoperatively. CONCLUSIONS AND SIGNIFICANCE: AS is a surgical technique worth trying in patients with early stage of attic/sinus cholesteatoma in terms of the rate of recurrence and hearing improvement.

Early management for traumatic benign paroxysmal positional vertigo in traumatically injured patients.

OBJECTIVES: The purpose of this study was to identify the clinical features of posttraumatic benign paroxysmal positional vertigo (t-BPPV) in traumatically injured patients, investigating the effectiveness of the early diagnosis and management including canalith repositioning procedures (CRPs). PATIENTS AND METHODS: The subjects of the present study were 74 patients who were hospitalized in the Trauma Center, Ajou University Hospital. We investigated the relationship between injury mechanisms and t-BPPV. Patients with t-BPPV were categorized into mild (typical BPPV) and severe (bilateral, recurrent, or persistent) types. RESULTS: Of the 74 patients, 41 were diagnosed with t-BPPV. Nineteen were mild and 22 were severe types. 'A fall' (36%) and 'pedestrian car accident' (32%) were common as the injury mechanisms provoking severe t-BPPV. In the severe t-BPPV group, they were hospitalized longer (as median value, 20 days in the severe group vs. 10 days in the mild group, P = 0.004), stayed longer in intensive care unit (3 days vs. 0 days, P = 0.016), and needed more days until the BPPV management (13.5 days vs. 6 days, P = 0.021). Major trauma (the Injury Severity Score >15) patients had a longer time to implementation of the first CRPs (10 days in major trauma and 3 days in minor trauma patients, P = 0.019). CONCLUSIONS: Severity of trauma and longer duration of ICU treatment were factors delaying BPPV management. This delay could negatively affect the progress of t-BPPV. Diagnostic and therapeutic maneuvers including CRPs should be performed as early as possible, even in severely injured patients.

Selection of the optimal first ear for sequential bilateral cochlear implantation in children.

OBJECTIVES: When there is a difference in hearing on both ears, where to perform the first cochlear implantation (CI) becomes an important issue. The purpose of the study was to evaluate which ear should be chosen for the first implantation in sequential bilateral CI with a long inter-implant period. METHODS: The study population consisted of 34 severe-to-profound sensorineural hearing loss pediatrics with the inter-implant period of ≥3 years between the first CI (CI-1) and the second CI (CI-2) before the age of 19 (mean of inter-implant period: 7.1-year). The patients were classified into Group A (CI-1 was performed on the ear with better hearing), Group B (CI-1 on the ear with worse hearing), or Group C (symmetrical hearing in both ears). Speech intelligibility test results were compared between the groups. RESULTS: The monosyllabic word scores of CI-1 were excellent in Groups A (91.7±7.9%) and B (92.5±3.6%) but slightly lower in Group C (85.7±14.9%) before the second implantation (P = .487). At 3 years after the second implantation, all groups demonstrated excellent scores in the bilateral CI condition (95.9±3.0% in Group A; 99.1±.8% in Group B; 97.5±2.9% in Group C, P = .600). However, when the patients were tested in using CI-2 only in Groups A and B after using bilateral CI for 3 years, the scores were inconsistent in Group A (79.6±23.9%; range: 22.2-94.4%), while those were higher and more constant in Group B (92.9±4.8%; 86.8-100.0%). CONCLUSIONS: The first CI is strongly recommended to perform on a worse hearing ear if they had different hearing levels between ears. Even with the first CI on a worse hearing ear, its performance never deteriorates. In addition, if they receive the second CI several years later, it will be likely that the second one functions better.

Effect of Age at Cochlear Implantation in Educational Placement and Peer Relationships.

OBJECTIVES: The education and school life of children who have undergone cochlear implantation (CI) is very important and should be monitored continuously. This study assessed auditory performance in children with cochlear implants over time, along with educational placement and peer relationship, and compared the results based on the age at CI and the presence of additional disabilities. DESIGN: In total, 77 children who had undergone CI at the Hearing Center in Ajou University Hospital at less than 10 years old and who were presently attending school or had already graduated from school within 3 years were enrolled in this study. All children had congenital bilateral severe or profound hearing loss at the diagnosis. They were classified based on the age at which they received CI: the "early-implanted group," younger than 3.5 years (n = 38), and the "late-implanted group," between 3.5 and 10 years old (n = 39). RESULTS: The early-implanted group had worse short-term auditory performance than the late group, but after 2 years of the implant use, auditory performance was similar in both groups. The early and late groups did not differ significantly in terms of the proportions of children who were enrolled in a regular school (94.7% and 89.7%, respectively). However, the early-implanted group had a larger proportion of children who were enrolled in a regular school without additional needs (73.0%), compared with the late group (48.6%) (p = .034). Children with multiple disabilities showed poorer performance (word score of 57.7% and sentence score of 44.7%) than children with hearing disability only (91.8% and 87.2%, respectively), which affected full-time enrollment in regular schools. With regard to peer relationships, 19.0% of children in the early-implanted group required close observation and assistance, and 9.5% even required help and counseling. Children who underwent early CI had a high tendency toward social restraint, apathy, and over-commitment. No correlations were observed between audiological factors and the aspects of peer relationships assessed in this study. CONCLUSIONS: Early CI and the absence of other disabilities were the two main factors that increased the likelihood of full-time enrollment in mainstream classes at regular schools. Nevertheless, many children who underwent earlier CI still encountered difficulties in peer relationships.

Changes in Vestibular Symptoms and Function After Cochlear Implantation: Relevant Factors and Correlations With Residual Hearing.

OBJECTIVES: The aim of this study was to evaluate vestibular function loss after cochlear implantation (CI) and the relationship between vestibular function and hearing changes. METHODS: Seventy-five patients with CI were enrolled and divided into those with normal preoperative caloric function (group I) and those with a normal preoperative waveform in cervical vestibular evoked myogenic potential (c-VEMP) testing (group II). The relationship between hearing and changes in the vestibular system was analyzed preoperatively and at 3 and 6 months postoperatively. RESULTS: In group I, unilateral weakness on the implanted side was detected in five (7.7%) and eight (12.3%) patients at 3 and 6 months post-CI, respectively. By 3 months post-CI, the total slow-phase velocity (SPV; warm and cold stimulations) was significantly different between the implanted and non-implanted sides (P=0.011), and the shift in total SPV from pre- to post-CI was significantly correlated with the average hearing threshold at 6 months post-CI. In group II, an abnormal c-VEMP was detected on the implanted side in six patients (16.2%) at 3 months post-CI, and in six patients (16.2%) at 6 months post-CI. Significant changes were noticed in the P1 and N1 amplitude at 3 months postCI (P=0.027 and P=0.019, respectively). CONCLUSION: Vestibular function and residual hearing function should be afforded equal and simultaneous consideration in terms of preservation.

Cochlear Implantation via the Transmeatal Approach in an Adolescent with Hunter Syndrome-Type II Mucopolysaccharidosis.

Type II mucopolysaccharidosis (MPS II) commonly known as Hunter syndrome, is a rare X-linked lysosomal storage disorder caused by iduronate-2-sulfatase deficiency, which in turn causes otorhinolaryngological manifestations, including sensorineural hearing loss (SNHL). Previously, the median survival age of patients with MPS was approximately 13.4 years. However, in the era of enzyme replacement therapy and other multidisciplinary care modalities, the life expectancy has increased. Herein, we report a rare case of an adolescent with MPS II who underwent SNHL treatment with cochlear implantation (CI). Based on unexpected findings of mastoid emissary veins and overgrowth of the vessels around the temporal bone, CI was performed using the transmeatal approach instead of the conventional transmastoid method, to avoid damage to the vessels. The average hearing threshold after CI was 35 dB and no surgical complications were encountered. Adolescent MPS II may present vessel abnormalities, which can reduce the success rate of surgery. In patients with MPS II with SNHL, CI should be performed under careful monitoring of vessel overgrowth. Moreover, with regard to feasibility of CI in adolescent patients with MPS II with SNHL, surgical techniques such as the transmeatal approach should be selected based on adequate assessment of the case.

A New CT Parameter for Predicting Residual Hearing Preservation in Cochlear Implantation: The "Basal Turn-Facial Ridge Angle".

OBJECTIVES: We suggest a simple measurement, called the "basal turn-facial ridge (BT-FR) angle," for determining the electrode insertion axis using preoperative temporal bone computed tomography (CT) to predict hearing preservation (HP) in cochlear implantation (CI). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Eighty-two ears that underwent CI between 2010 and 2018 were included. Ears with preoperative thresholds less than or equal to 80 dB HL at 125, 250, and 500 Hz were enrolled and grouped using the criteria of Skarzynski et al.: Group 1, complete or partial HP; Group 2, minimal HP or complete hearing loss. INTERVENTION: All subjects underwent CI with soft surgery techniques through the round window approach. MAIN OUTCOME MEASURES: The BT-FR angle is the angle between the basal turn line (BT-line), which is a straight line passing through the center of the longitudinal axis of the BT, and the facial ridge line, which is a straight line running from the endpoint of the BT-line to a point just above the facial ridge. RESULTS: The BT-FR angle was 2.5 ±â€Š2.9 degrees in Group 1 and -0.3 ±â€Š2.7 degrees in Group 2 (p = 0.003). The angle and hearing loss showed a significant negative correlation (r = -0.401, p = 0.002). In multiple linear regression, "age at operation" (ß coefficient 0.260; p = 0.001) and the "BT-FR angle" (-1.967; p = 0.001) were significant variables affecting the degree of residual hearing loss. CONCLUSIONS: The BT-FR angle, which can be measured simply, may be useful to predict residual HP after CI.

Hearing aids are still beneficial to patients, even if they have a low speech discrimination.

PURPOSE: A speech discrimination score (SDS) is a predictor for the successful use of hearing aids (HAs). This study is to evaluate the efficacy of HAs in patients with a low or poor SDS. METHODS: We enrolled 186 ears using HAs, with an unaided SDS ≤ 64%. They were categorized into four groups by their unaided SDS: 0-16% for Group 1, 20-32% for Group 2, 36-48% for Group 3, and 52-64% for Group 4. Aided SDS was measured 1, 3, 6, and 12 months after the use. The Hearing In Noise Test (HINT), the Hearing Handicap Inventory for the Elderly (HHIE), and the International Outcome Inventory for Hearing Aids (IOI-HA) were assessed. RESULTS: The SDS increased by 27.4% (12.0 to 39.4%) in Group 1, 26.4% (26.9 to 53.3%) in Group 2, 24.6% (42.2 to 66.8%) in Group 3, and 10.5% (59.5% to 70.0%) in Group 4. HINT composite scores significantly decreased from 22.5 to 15.1 in Group 1, 9.4 to 7.0 in Group 2, and 4.4 to 2.4 in Group 4. Total HHIE score changed from 48.2 to 24.2 in Group 1, 64.0 to 32.8 in Group 2, 37.1 to 16.6 in Group 3, and 55.8 to 40.1 in Group 4 (P < 0.05 in Groups 2, 3, and 4). CONCLUSION: In patients with a low SDS, a significant increase in SDS was achieved after the use of HAs, and subjective satisfaction was also acceptable. Low SDS might not be a contraindication for HAs.

Vertical Nystagmus in the Bow and Lean Test may Indicate Hidden Posterior Semicircular Canal Benign Paroxysmal Positional Vertigo: Hypothesis of the Location of Otoconia.

The 'Bow and Lean Test' (BLT) was developed for proper diagnosis of horizontal semicircular canal benign paroxysmal positional vertigo (HSC-BPPV). Occasionally, down- and/or up-beating vertical nystagmus is observed during the BLT. This study analyzed patients who exhibited vertical nystagmus in the BLT to comprehend the clinical significance of this sign. Of 2872 patients with vertigo between 2010 and 2015, 225 patients who showed vertical nystagmus in the BLT were enrolled. All patterns of vertical nystagmus were described based on their types of BPPV. After performing therapeutic maneuvers for BPPV, remnant symptoms in the BLT findings were investigated. Of the 225 patients with vertical nystagmus, 163 were posterior semicircular canal BPPV (PSC-BPPV). Down-beating in the bowing position and no nystagmus in the leaning position ('Down/-') was the most common type (190 of 225 patients). In addition, the nystagmus occurred in the form of '-/Up', 'Down/Up', and '-/Down'. The pattern of vertical nystagmus may be related to the position of otoconia in the canals. The location of the otoconia enables the diagnosis of hidden PSC-BPPV. Even after treatment for BPPV, patients with vertical nystagmus in the BLT tended to complain remnant vertigo symptoms (44.8% vs. 23.9%, P = 0.022, in PSC-BPPV; 70.0% vs. 24.0%, P = 0.020, in HSC-BPPV). We thought that they actually had hidden PSC-BPPV and the otoconial debris may still in the PSC; this untreated PSC-BPPV might cause the remnant symptoms. In conclusion, vertical nystagmus in the BLT may indicate the presence of PSC-BPPV. Moreover, vertical nystagmus during the BLT may occur in patients with hidden PSC-BPPV who complain of remnant vertigo symptoms. Vertical nystagmus shown in the BLT may not include the possibility of central vertigo.