Clinical characteristics and hearing loss etiology of cochlear implantees undergoing surgery in their teens, 20s, and 30s.

PURPOSE: This study aimed to investigate the etiology of hearing loss, including genetic variants, in individuals who underwent cochlear implantation (CI) in their teens to thirties. It also sought to analyze post-CI speech performance and identify prognostic factors affecting CI outcomes in this age group. METHODS: We conducted a retrospective review of 421 cochlear implant patients at Seoul National University Bundang Hospital, focusing on 63 subjects aged 10-39 years who underwent their first CI by a single surgeon between July 2018 and June 2022. The study included audiologic evaluation, molecular genetic testing, and analysis of speech performance post-CI. Statistical analyses were performed using SPSS 25 and GraphPad Prism 7. RESULTS: Among 63 participants (M:F, 24:39), nine underwent CI in their teens, 24 in their 20 s, and 30 in their 30 s. Most of them (40, 63.5%) had postlingual deafness. The study found that 65.2% (40/63) of subjects received a genetic diagnosis, with DFNB4 being the most common etiology (37.5%, 15/40). Post-CI speech evaluation showed an average sentence score of 80% across all subjects. Factors such as the onset of hearing loss, duration of deafness (DoD), and preoperative Speech Intelligibility Rating (SIR) significantly influenced CI outcomes. Notably, longer DoD was associated with poorer CI outcomes, but this did not affect individuals with postlingual hearing loss as much. CONCLUSION: The study concludes that in individuals aged 10-39 undergoing CI, the onset of hearing loss and preoperative SIR are critical predictors of postoperative outcomes. CI is recommended for those with postlingual hearing loss in this age group, irrespective of the DoD. The study highlights the importance of genetic factors especially DFNB4 in hearing loss etiology and underscores the value of the relatively easy-to-evaluate factor, preoperative SIR in predicting CI outcomes.

Comparing pronunciation challenges in South Korean preschoolers with unilateral single-sided deafness due to cochlear nerve deficiency to a norm-referenced standard.

This study aimed to compare the development of pronunciation in South Korean preschoolers with unilateral cochlear nerve deficiency (CND) to that of age-matched preschoolers with normal hearing, a topic that has not been explored previously. In a retrospective analysis, 25 preschoolers with unilateral CND who had undergone a speech evaluation battery, including a pronunciation and vocabulary test, were enrolled. Utilizing the Urimal Test of Articulation and Phonation and customized language ability tests, pronunciation and vocabulary were assessed. The subjects' speech evaluation scores were converted into age-adjusted z-scores using normal controls' data. While vocabulary performance was within normal limits, their average pronunciation z-score was -2.90, significantly lower than both the zero reference point and their vocabulary z-scores. None of the subjects scored above average in pronunciation. Thirteen patients were recommended for articulation therapy, seven were considered as potential candidates for this therapy, and the remaining five were within normal limits. There was no observed correlation between the development of pronunciation and vocabulary. Notably, some subjects' pronunciation scores did not improve, even after serial follow-up during their preschool years. Despite typical vocabulary development, preschoolers with unilateral CND exhibit significant delays in pronunciation. These findings emphasize the necessity for vigilant monitoring of their language development.

Sleep disturbance and dysregulation of circadian clock machinery in sudden sensorineural hearing loss.

BACKGROUND: The cochlea contains a robust biological clock associated with auditory function, exhibiting diurnal sensitivity to noise or ototoxicity. OBJECTIVES: We examined the relationship between disrupted circadian rhythm and altered expression of circadian clock genes in patients with sudden sensorineural hearing loss (SSNHL) and explored whether the circadian clock genes serve as prognostic biomarkers. MATERIAL AND METHODS: Twelve patients with SSNHL were enrolled study group. Twelve people with normal hearing were enrolled voluntarily for comparison. Audiological evaluation was performed to evaluate hearing thresholds. Korean version of the Pittsburgh Sleep Quality Index Questionnaire was performed to evaluate sleep quality and patterns. Circadian clock genes including for PERI, PER2, PER3, CRYI, CRY2, CLOCK, ARNTL, CSNKIE, and TIMELESS expression in blood were evaluated using real-time quantitative PCR method. RESULTS: Compared with healthy controls without hearing loss, most of the circadian clock genes were markedly downregulated, coupled with low sleep quality and disturbing patterns, in patients with SSNHL. Intriguingly, a weak correlation between hearing improvement following steroid treatment and altered levels of circadian clock genes was observed. CONCLUSIONS AND SIGNIFICANCE: This study provides an additional basis for the relevance of disrupted circadian rhythm to SSNHL and suggests a possible prognostic biomarker for SSNHL treatment.

The Impact of Lateral Relaxing Incision on Middle Ear Function in Cleft Palate Repair.

Children with cleft palate are susceptible to otitis media with effusion. This study aimed to investigate the effect of lateral relaxing incision (RI) on middle ear function in cleft palate patients who underwent palatoplasty using double-opposing Z-plasty (DOZ). This is a retrospective study of patients who underwent bilateral ventilation tube insertion concurrently with DOZ, wherein RI was selectively performed on the right side of the palate (Rt-RI group) or not (No-RI group). The frequency of VTI, duration of the first ventilation tube retention, and hearing outcomes at the last follow-up were reviewed. Outcomes were compared using the χ 2 test and t test. A total of 126 treated ears from 63 non-syndromic children (18 male, 45 female) with cleft palate were reviewed. The mean age at surgery was 15.8±6.17 months. There were no significant differences in the frequency of ventilation tube insertion between the right and left ears within the Rt-RI group or between the Rt-RI and no-RI groups in the right ear. Subgroup analysis for ventilation tube retention time, auditory brainstem response thresholds, and air-conduction pure tone averages showed no significant differences. In the DOZ, the use of RI had no significant effects on middle ear outcomes during 3 years of follow-up. Relaxing incision seems to be safe without concern for middle ear function in children with cleft palate.

Hearing loss in neonates and infants.

Hearing in neonates and infants is crucial for their development of language and communication skills. Unless hearing loss is appropriately managed early, it can cause a significant socioeconomic burden considering its detrimental impact on the child's development and its common nature. It is also the most common congenital sensory deficit, with an approximate incidence of 1.5 per 1,000 newborns. Its etiologies are heterogeneous: genetic causes are reportedly involved in up to 80% of cases, while congenital cytomegalovirus infection is the leading environmental factor contributing to congenital hearing loss. The introduction of newborn hearing screening using automated auditory brainstem response and/or automated otoacoustic emission in many developed countries has helped detect and manage hearing loss early. Current auditory rehabilitation options such as cochlear implantation implementing cutting-edge technologies can treat almost all degrees of hearing loss, emphasizing the importance of early hearing detection and intervention. Rapidly developing genetic diagnostic technologies and future cutting-edge treatment options, including gene therapy, will shed light on the future management of hearing loss in neonates and infants.

A comparative study of audiological outcomes and compliance between the Osia system and other bone conduction hearing implants.

PURPOSE: To examine the subjective and objective audiological benefits of the Osia system compared to devices commonly implanted prior to the introduction of this system. METHODS: Osia recipients with either conductive hearing loss (CHL/MHL) (n = 9) or single-sided deafness (SSD) (n = 8) who underwent surgery from February 2021 to March 2022 were prospectively recruited. The audiological outcomes and usage rate of Osia implantees were compared with those of retrospectively recruited patients implanted with other devices (n = 50). The subjective satisfaction of the Osia implantees was also evaluated through questionnaires. RESULTS: All users of the Osia system were classified as regular users. In the CHL/MHL group, the effective gain of the Osia system (11.1 ± 14.9 dB) surpassed that of the Baha and Bonebridge (- 2.7 ± 12.6 dB) at 2 kHz (p = 0.01, Mann-Whitney U test). Among the devices, the Osia system tended to tolerate the worst bone conduction thresholds, up to the level of 61 dB. In the SSD group, the functional gain of Osia at 4 kHz (37.5 ± 3.1 dB) was higher than that of the Baha and Bonebridge group (26.9 ± 3.0 dB) (p = 0.05, Mann-Whitney U test). CONCLUSION: The Osia system yielded larger audiological gain than the Baha Attract and Bonebridge devices, especially at high frequencies, leading to substantially higher compliance. The Osia system tended to have the strongest tolerance to aggravated bone conduction thresholds among the available transcutaneous bone conduction hearing implants. Therefore, the Osia system could potentially be a good option for CHL/MHL patients with bone conduction thresholds of 50 dB HL or more, as well as patients with SSD.

Functional Outcomes of Cochlear Implantation in Children with Bilateral Cochlear Nerve Aplasia.

Background and Objectives: Many otologists face a dilemma in the decision-making process of surgical management of patients with cochlear nerve (CN) aplasia. The goal of this study is to provide fresh evidence on cochlear implantation (CI) results in patients with CN aplasia. Materials and Methods: We scrutinized functional outcomes in 37 ears of 21 children with bilateral CN aplasia who underwent unilateral or bilateral CI based on cross-sectional and longitudinal assessments. Results: The Categories of Auditory Performance (CAP) scores gradually improved throughout the 3-year follow-up; however, variable outcomes existed between individuals. Specifically, 90% of recipients with a 1-year postoperative CAP score ≤1 could not achieve a CAP score over 1 even at 3-year postoperative evaluation, while the recipients with a 1-year postoperative CAP score >1 had improved auditory performance, and 72.7% of them were able to achieve a CAP score of 4 or higher. Meanwhile, intraoperative electrically evoked compound action potential was not correlated with postoperative CAP score. Conclusions: Our results further refine previous studies on the clinical feasibility of CI as the first treatment modality to elicit favorable auditory performance in children with CN aplasia. However, special attention should be paid to pediatric patients with an early postoperative CAP score ≤1 for identification of unsuccessful cochlear implants and switching to auditory brainstem implants.

Outcome of cochlear implantation in the worse ear of post-lingual asymmetric hearing loss: elucidation of prognostic markers.

BACKGROUND: Specific correlations between the outcomes of cochlear implantation (CI) and hearing thresholds of the both ears in post-lingual asymmetric hearing loss (AHL) patients were not clear. AIMS/OBJECTIVES: To identify the variables influencing the outcome of CI in post-lingual AHL patients. METHOD: We included 18 adult subjects who had CI in the worse ear due to post-lingual AHL with average hearing asymmetry of 36 dB. Speech perception scores were evaluated in the sound field with hearing aid on the better ear before CI, and with the cochlear implant in the worse ear at 3, 6, and 12 months after CI switch-on. RESULTS: Average increases in phonetically balanced word score, spondee word score, and everyday sentence score at 12 months from CI switch-on compared with those before CI were 38.9%p, 46.2%p, and 52.4%p, respectively. Multiple linear regression analysis showed that speech perception scores were negatively influenced by age at implantation and hearing threshold difference of both ears (HTD-Both) itself, rather than the worse or better ear hearing thresholds. CONCLUSIONS AND SIGNIFICANCE: Post-lingual AHL subjects with high average hearing asymmetry can benefit from CI in the worse ear, while CI outcomes can be adversely influenced by HTD-Both and age at implantation both in the short- and long-term follow-up.

House Dust Mite Sublingual Immunotherapy in Children Versus Adults With Allergic Rhinitis.

BACKGROUND: There are only a few studies in which the clinical efficacy of SLIT has been compared between children and adults. In addition, there is a lack of research on other factors, associated with the treatment, including immunological parameters and quality of life (QOL). OBJECTIVE: To compare the effects of sublingual immunotherapy (SLIT) in adults and children on various factors: clinical efficacy, quality of life (QOL), satisfaction, immunological parameters, and adverse events. METHODS: Subjects who were sensitized to house dust mites and treated with SLIT for at least 2 years were enrolled. Seventy patients who completed questionnaires measuring nasal symptoms and Rhinoconjunctivitis Quality of Life Questionnaire (RQLQ) scores and underwent serologic tests for immunological parameters at initial, 1-year, and 2-year follow-up were selected and divided into two groups based on age: a child group (age 4-12 years, n = 44) and an adult group (age 19-59 years, n = 26). RESULTS: The Total Nasal Symptom Score (TNSS) was significantly decreased after 2 years of SLIT in both the child and adult groups (p < 0.001, both); however, changes in TNSS from baseline did not significantly differ between the two groups (p = 0.365). More patients in adult group were satisfied with SLIT than those in child group (p = 0.050), and changes in RQLQ score from baseline tended to be larger in adult group (p = 0.089). The levels of immunological parameters at baseline were significantly higher in the child group than in the adult group; however, changes in the levels of these parameters were not significantly different. CONCLUSION: Although more adult patients were satisfied with SLIT, the clinical effects of SLIT on nasal symptoms were comparable between child and adult groups. Despite different immunological values at baseline between the two groups, changing patterns of immunological parameters did not differ.

Beware of a Fragile Footplate: Lessons from Ossiculoplasty in Patients with Ossicular Anomalies Related to Second Pharyngeal Arch Defects.

BACKGROUND AND OBJECTIVES: We review the intraoperative findings and postoperative outcomes of ossiculoplasty in subjects with second pharyngeal arch (SPA)-derived ossicular anomalies. We summarize potential intraoperative complications and recommend precautions that may reduce the risk of fracture. MATERIALS AND METHODS: Twenty-four patients with SPA-derived ossicular anomalies were included, and pre- and postoperative audiometric results were compared. RESULTS: The mean air conduction threshold (56.0 ± 12.4 dB HL) was significantly improved 1 month (27.6 ± 10.1 dB HL) and 6 months (23.8 ± 13.2 dB HL) after surgery (p < 0.001). The preoperative air-bone gap (ABG) (40.4 ± 7.4 dB HL) was significantly decreased at 1 month (15.1 ± 5.9 dB HL) and 6 months (11.3 ± 8.9 dB HL) postoperation. ABG closure was successful (<20 dB HL) in 21 (87.5%) patients 6 months after surgery. Intraoperative footplate fractures occurred in 3 of 24 patients. The fractures were managed successfully, and the ABG closure was successful in all cases. CONCLUSIONS: The stapes footplate is likely to be relatively thin in subjects with SPA-derived ossicular anomalies because the footplate is partially or totally derived from the SPA. Thus, a fragile footplate should be expected, and care is needed when handling the footplate. However, when complications are overcome, the audiological outcomes are excellent in most cases.

Revisiting the Clinical Scoring System for the Prognosis of Chronic Rhinosinusitis with Nasal Polyps.

PURPOSE: To evaluate the Japanese Epidemiological Survey of Refractory Eosinophilic Chronic Rhinosinusitis (JESREC) classification, a clinical scoring system, for predicting disease control status in chronic rhinosinusitis with nasal polyps (CRSwNP) and to investigate prognostic factors. MATERIALS AND METHODS: In total, 134 CRSwNP patients who underwent functional endoscopic sinus surgery after maximal medical treatment were enrolled. These patients were categorized into four groups according to JESREC classification: 1) non-eosinophilic CRSwNP (non-ECRSwNP), 2) mild eosinophilic CRSwNP (ECRSwNP), 3) moderate ECRSwNP, and 4) severe ECRSwNP. Disease control status among the patients was evaluated at 1 year after surgery, and the patients were divided into two groups (disease-controlled and disease-uncontrolled groups) for the investigation of prognostic factors. RESULTS: There was no significant difference in disease control status between non-ECRSwNP and ECRSwNP groups (p=0.970). Age, Lund-Mackay CT scores, global osteitis scores, tissue neutrophil count, and tissue eosinophil count were associated with disease control status. In subgroup analysis of the non-ECRSwNP group, only high tissue neutrophil count was related with disease control status, whereas for the ECRSwNP group, young age, high Lund-Mackay CT scores, high global osteitis scores, and high tissue and blood eosinophil counts were associated with disease control status. CONCLUSION: No difference in disease control status was identified between non-ECRSwNP and ECRSwNP cases. Tissue neutrophilia, however, appeared to be associated with disease control status in non-ECRSwNP cases, whereas tissue and blood eosinophilia was associated with ECRSwNP cases.

Prediction of Obstructive Sleep Apnea Based on Respiratory Sounds Recorded Between Sleep Onset and Sleep Offset.

OBJECTIVES: To develop a simple algorithm for prescreening of obstructive sleep apnea (OSA) on the basis of respiratory sounds recorded during polysomnography during all sleep stages between sleep onset and offset. METHODS: Patients who underwent attended, in-laboratory, full-night polysomnography were included. For all patients, audio recordings were performed with an air-conduction microphone during polysomnography. Analyses included all sleep stages (i.e., N1, N2, N3, rapid eye movement, and waking). After noise reduction preprocessing, data were segmented into 5-s windows and sound features were extracted. Prediction models were established and validated with 10-fold cross-validation by using simple logistic regression. Binary classifications were separately conducted for three different threshold criteria at apnea hypopnea index (AHI) of 5, 15, or 30. Prediction model characteristics, including accuracy, sensitivity, specificity, positive predictive value (precision), negative predictive value, and area under the curve (AUC) of the receiver operating characteristic were computed. RESULTS: A total of 116 subjects were included; their mean age, body mass index, and AHI were 50.4 years, 25.5 kg/m2 , and 23.0/hr, respectively. A total of 508 sound features were extracted from respiratory sounds recorded throughout sleep. Accuracies of binary classifiers at AHIs of 5, 15, and 30 were 82.7%, 84.4%, and 85.3%, respectively. Prediction performances for the classifiers at AHIs of 5, 15, and 30 were AUC, 0.83, 0.901, and 0.91; sensitivity, 87.5%, 81.6%, and 60%; and specificity, 67.8%, 87.5%, and 94.1%. Respective precision values of the classifiers were 89.5%, 87.5%, and 78.2% for AHIs of 5, 15, and 30. CONCLUSION: This study showed that our binary classifier predicted patients with AHI of ≥15 with sensitivity and specificity of >80% by using respiratory sounds during sleep. Since our prediction model included all sleep stage data, algorithms based on respiratory sounds may have a high value for prescreening OSA with mobile devices.