Metronome-guided cochlear implantation for slower and smoother insertions of lateral wall electrodes.

INTRODUCTION: Achieving a slow and smooth electrode array insertion is paramount for preserving structural and functional integrity during cochlear implantation. This controlled study evaluates the efficacy of a metronome-guided insertion technique in enhancing the smoothness and speed of electrode array insertions. METHODS: In a prospective cohort study, patients undergoing cochlear implant surgery between 2022 and 2023 with lateral wall electrode arrays were included. Metronome guidance was delivered through an acoustic signal via headphones during electrode array insertion in cochlear implantation and compared to a control group without metronome-guidance. RESULTS: In total, 37 cases were evaluated, including 25 conventional insertions and 12 metronome-guided insertions. The results indicate that metronome-guided insertions were significantly slower (- 0.46 mm/s; p < 0.001) without extending the overall procedure time. This can be attributed to fewer paused sections observed in the metronome-guided technique. Moreover, metronome-guided insertions exhibited superior performance in terms of insertion smoothness and a reduced number of re-gripping events. CONCLUSIONS: The findings support the recommendation for the systematic application of metronome guidance in the manual insertion of cochlear implant electrode arrays, emphasizing its potential to optimize surgical outcomes.

Treatment of sleep-disordered breathing with positional therapy: long-term results.

PURPOSE: The aim of the present study was to assess the efficacy of a sleep position trainer (SPT) in patients with an established diagnosis of positional obstructive sleep apnea and to evaluate the adherence after 1-year follow-up. METHODS: Polysomnography (PSG) was performed at baseline and after 1 year of SPT use. Patients received questionnaires to assess treatment satisfaction and subjective adherence. Data on objective adherence and number of vibrations initiated by the SPT were collected from the SPT device. RESULTS: Nine out of 58 patients stopped using the SPT during the first year of treatment (16%). Thirty-four middle-aged and overweight patients underwent a PSG after 1 year of SPT use (male/female ratio, 28/6; overall apnea/hypopnea index (AHI), 16/h). A significant reduction in overall AHI to 6/h was observed using treatment (p < 0.001). The median percentage of supine sleep decreased significantly to 1% with SPT (p < 0.001). The mean objective SPT use in 28 patients was 7.3 ± 0.9 h/night and 69 ± 26% of the nights. Furthermore, 75% of the patients reported a better sleep quality since the start of SPT treatment. CONCLUSIONS: Long-term treatment with the SPT was found to be effective in reducing overall AHI. Time spent sleeping in supine position was reduced to almost zero in the continuing users. Patient satisfaction was high when using the SPT.

Drug-induced sedation endoscopy in surgically naïve infants and children with obstructive sleep apnea: impact on treatment decision and outcome.

PURPOSE: Adenotonsillectomy (AT) is the first-line treatment for obstructive sleep apnea (OSA) in children irrespective of clinical upper airway (UA) findings. We aimed to investigate whether drug-induced sedation endoscopy (DISE) changes treatment decision and outcome in otherwise healthy children and infants with OSA. METHODS: Retrospective analysis of prospectively collected data on polysomnography, DISE, and treatment in surgically naïve, otherwise healthy infants (n = 34) and children (n = 75) with OSA. Treatment success is defined as post-treatment obstructive apnea/hypopnea index (oAHI) < 5 h-5-1, and cure is defined as oAHI < 2 h-1. RESULTS: Based upon UA findings during DISE, AT was performed in 22 infants and 57 children. oAHI improved from 16.5 h-1 (8.1-28.3) to 0.8 h-1 (0.3-4.2) (p = 0.01) in infants and from 28.6 h-1 (23.4-34.9) to 0.7 h-1 (0.4-1.8) (p < 0.001) in children. AT was successful in 84.2% of infants and 91.4% of children. A cure was obtained in 68.4% of infants and 78.7% of children. DISE changed the treatment decision in 1/3rd of infants and 1/4th of children, and they did not undergo AT. In the non-AT group, isolated adenoidectomy/tonsillectomy or non-surgical treatment was successful in 86.6% of children and in 100% of infants. Cure was achieved in 66.6% of children and 75% of infants. CONCLUSIONS: DISE performed in otherwise healthy and surgically naïve infants and children with OSA altered the therapeutic decision making in up to 1/3rd to 1/4th of the cases and resulted in comparable treatment outcomes as standard treatment by AT. The present data suggest that DISE may provide individually tailored treatment of OSA in otherwise healthy infants and children.

Changes in Swallowing-related Quality of Life After Endoscopic Treatment For Zenker's Diverticulum Using SWAL-QOL Questionnaire.

Dysphagia affects the most cardinal of human functions: the ability to eat and drink. The aim of this prospective study was to evaluate swallowing dysfunction in patients diagnosed with Zenker's diverticulum using the Swallowing Quality of Life (SWAL-QOL) questionnaire preoperatively. In addition, SWAL-QOL was used to assess changes in the outcome of swallowing function after endoscopic treatment of Zenker's diverticulum compared to baseline. Pre- and postoperative SWAL-QOL data were analyzed in 25 patients who underwent endoscopic treatment of Zenker's diverticulum between January 2011 and December 2013. Patients were treated by different endoscopic techniques, depending on the size of the diverticulum: CO2 laser technique or stapler technique, or the combination of both techniques used in larger diverticula. Their mean age was 69 years, and 28% of patients were female. The mean interval between endoscopic surgery and completion of the postoperative SWAL-QOL was 85 days. The median (min-max) preoperative total SWAL-QOL score was 621 (226-925) out of 1100, indicating the perception of oropharyngeal dysphagia and diminished quality of life. Following endoscopic treatment of Zenker's diverticulum, significant improvement was demonstrated in the postoperative total SWAL-QOL score of 865 (406-1072) out of 1100 (p < 0.001). On the majority of subscales of SWAL-QOL there was significant improvement between pre- and postoperative scores. To the authors' knowledge, this is the first report in the literature on the changes in pre- and postoperative SWAL-QOL scores for patients with Zenker's diverticulum before and after treatment. The results of this study indicate that endoscopic treatment of Zenker's diverticulum leads to significant symptom relief as documented by significant changes in the majority of the SWAL-QOL domains.

Drug-induced sedation endoscopy in children <2 years with obstructive sleep apnea syndrome: upper airway findings and treatment outcomes.

Few data are available about the pattern of upper airway (UA) obstruction in children <2 years with obstructive sleep apnea syndrome (OSAS). Also, the role of adenoidectomy versus adenotonsillectomy (AT) is poorly defined in this age group. We performed drug-induced sedation endoscopy (DISE) in young OSAS children to investigate the pattern of UA obstruction and the value of DISE in therapeutic decision making. Retrospective analysis of ≤2-year-old children undergoing DISE-directed UA surgery. OSAS severity and the treatment outcomes were documented by polysomnography. Data are available for 28 patients, age 1.5 years (1.3-1.8), BMI-z score 0.5 (-0.7 to 1.3) with severe OSAS, obstructive apnea/hypopnea index (oAHI) 13.8/hr (7.5-28.3). All but 3 had (>50%) obstruction at the level of the adenoids, and all but 5 had (>50%) tonsillar obstruction. DISE-directed treatment consisted of adenoidectomy (n = 4), tonsillectomy (n = 1), and AT (n = 23). There was a significant improvement in respiratory parameters. Twenty children (71.4%) had a postoperative oAHI <2/hr. None had palatal or tongue base obstruction. Five children had a circumferential UA narrowing (hypotonia), 2 of them had residual OSAS. DISE showed a collapse of the epiglottis in 6 and late-onset laryngomalacia in 4. These findings did not affect surgical outcome. Adenotonsillar hypertrophy is the major cause of UA obstruction, and DISE-directed UA surgery was curative in 71,4% of children ≤2 years. We suggest that DISE may be helpful in surgical decision making. Circumferential UA narrowing may result in less favorable surgical outcomes.

Safety and effectiveness of the Bonebridge transcutaneous active direct-drive bone-conduction hearing implant at 1-year device use.

The objective of this study is to evaluate the safety and efficacy of a new transcutaneous bone-conduction implant (BCI BB) in patients with conductive and mixed hearing loss or with single-sided deafness (SSD), 1 year after surgical implantation. The study design is multicentric prospective, intra-subject measurements. Each subject is his/her own control. The setting is nine university hospitals: 7 French and 2 Belgian. Sixteen subjects with conductive or mixed hearing loss with bone-conduction hearing thresholds under the upper limit of 45 dB HL for each frequency from 500 to 4000 Hz, and 12 subjects with SSD (contralateral hearing within normal range) were enrolled in the study. All subjects were older than 18 years. The intervention is rehabilitative. The main outcome measure is the evaluation of skin safety, audiological measurements, benefit, and satisfaction questionnaires with a 1-year follow up. Skin safety was rated as good or very good. For the mixed or conductive hearing loss groups, the average functional gain (at 500 Hz, 1, 2, 4 kHz) was 26.1 dB HL (SD 13.7), and mean percentage of speech recognition in quiet at 65 dB was 95 % (vs 74 % unaided). In 5/6 SSD subjects, values of SRT in noise were lower with BB. Questionnaires revealed patient benefit and satisfaction. The transcutaneous BCI is very well tolerated at 1-year follow up, improves audiometric thresholds and intelligibility for speech in quiet and noise, and gives satisfaction to both patients with mixed and conductive hearing loss and patients with SSD.

Prospective case-controlled sound localisation study after cochlear implantation in adults with single-sided deafness and ipsilateral tinnitus.

OBJECTIVES: To analyse the sound localisation skills of subjects with profound single-sided deafness (SSD) and accompanied ipsilateral tinnitus who are using a cochlear implant (CI) for between 4 and 11 years. DESIGN: Sound localisation skills were tested using nine loudspeakers in a frontal semicircle ranging from -90° to +90°. Subjects were tested in the CION and the CIOFF conditions via 3 localisation stimuli: broadband noise (BB), low-pass noise (LP) and high-pass noise (HP). PARTICIPANTS: The test group consisted of 10 adult subjects with profound sensorineural SSD, ipsilateral tinnitus and a CI. Normative data of a control group of 30 normal hearing subjects were used for comparison. MAIN OUTCOME MEASURES: Sound location accuracy was analysed via the root-mean-square error (RMSE), the mean absolute error (MAE), the localisation bias ('b') and the bias-adjusted deviation ('db '). Subjective dynamic aspects of hearing were assessed via a reduced version of the Speech, Spatial and Qualities of Hearing Scale (SSQ5 ). RESULTS: For all 3 stimuli, the RMSE improved significantly in SSD subjects in the CION condition compared to the CIOFF condition. The localisation accuracy of subjects with SSD improved significantly for BB and HP stimuli. A significant bias-adjusted deviation 'db ' was found for the BB and HP stimuli. Subjects' mean SSQ5 scores were significantly higher in the CION condition at test date than in the CIOFF condition preoperatively. CONCLUSIONS: Subjects can better locate sound in the CION condition than in the CIOFF condition.

Dutch health-related quality of life measure for chronic otitis media.

OBJECTIVE: Chronic otitis media (COM) describes a variety of symptoms and physical findings that result from prolonged damage to the middle ear by infection and inflammation. The Health-Related Quality of Life measure for COM (COMQ-12) is a new questionnaire for the assessment of COM that evaluates the overall burden of disease from the patient's perspective. The aim of this study was to develop and appraise the psychometric properties of the Dutch version of the COMQ-12. MATERIALS AND METHODS: The Dutch version of the COMQ-12 was obtained through translation and back-translation. Fifty adult patients with a history of active COM completed the Dutch version of the COMQ-12. The internal consistency of this questionnaire was evaluated using Cronbach's alpha coefficient. RESULTS: The average COMQ-12 score was 22.4 (SD 11.9). The internal consistency of the Dutch version of the COMQ-12 was high, with a Cronbach's alpha value of 0.833. CONCLUSIONS: The Dutch version of the COMQ-12 provides appropriate health-related quality of life outcome measures in patients with a history of COM. This questionnaire is a useful tool to evaluate the overall burden of disease from the patient's perspective.

A cross-sectional analysis of otitis media with effusion in children with Down syndrome.

UNLABELLED: Children with Down syndrome are at risk to develop otitis media with effusion (OME). We performed a retrospective and cross-sectional analysis to evaluate the prevalence of OME in children with Down syndrome (DS) for consecutive age categories between 6 months and 12 years. Clinical and audiometric data were available for 107 children followed in a multidisciplinary Down team. A high prevalence of OME was found at the age of 1 year (66.7 %), with a second peak prevalence of 60 % at 6-7 years. A declining trend was observed in children ≥8 years. Overall, 52.3 % of DS children had either OME or ventilation tubes at the time of evaluation. Hearing thresholds were significantly higher in children with bilateral OME (median 36.7 decibel hearing level (dB HL), range 26.7-46.1) compared to those with at least one normally ventilated middle ear (median 28.3 dB HL, range 22.8-3.3), p = 0.013. CONCLUSION: We found a high prevalence of OME in children with Down syndrome, with a peak of ≥60 % around 1 and 6-7 years. A declining trend is seen in older children. Mild to moderate hearing loss was present in children with bilateral OME.

Evaluation of the impact of a clinical pathway on the organization of a multidisciplinary dental sleep clinic.

PURPOSE: Clinical pathways are used to organize complex care processes by providing structure and standardization. The multidisciplinary approach of oral appliance (OA) therapy for sleep-disordered breathing (SDB) is a complex and dynamic process suitable for such a structured pathway approach. METHODS: A clinical pathway for patients referred for OA therapy was developed and implemented. The aim of this study was to evaluate the impact of this clinical pathway on the time to delivery of the OA and the organization of the multidisciplinary dental sleep clinic (MDSC). The latter was achieved using the care process self-evaluation tool (CPSET). RESULTS: First, development and implementation of the clinical pathway gave structure and shortened the mean time to delivery by 102 days (240 ± 70 vs. 138 ± 33 days) (Mann-Whitney U: P < 0.001). Second, the CPSET scores were obtained in a cohort of 49 healthcare professionals involved in the pathway. Overall, patient-focused organization received the highest scores (80.5 ± 9.0%), whereas cooperation with primary care received the lowest score (66.7 ± 12.4%). CONCLUSIONS: This is the first project on clinical pathways in OA therapy for SDB. The implementation of the pathway in our MDSC has created a significant shortening of the time to delivery. A first evaluation of the clinical pathway using the CPSET scores indicates that all disciplines involved should be thoroughly informed in an ongoing approach.

Validation and translation of the Dutch tinnitus functional index.

OBJECTIVES: Several questionnaires are used to survey how tinnitus affects quality of life, making comparisons across studies difficult. The questionnaires also are used to measure treatment outcome but were not designed for this purpose. To address these issues, a new questionnaire has been suggested, the tinnitus functional index (TFI), which is highly responsive to treatment-related change. The current study aim was to translate and validate the TFI for a Dutch-speaking population. Factor analysis was performed to characterize the TFI profile in a large tinnitus population. METHODS: The questionnaire was translated using a translation-back translation procedure, and 263 patients in the ENT department of Antwerp University Hospital with tinnitus-related complaints completed it. Factor structure was assessed using exploratory analysis with oblique rotation and compared with the original questionnaire. Internal consistency was measured using Cronbach's alpha coefficient. Spearman correlations with the percentage of time aware of the tinnitus and the visual analogue scales (VAS) for maximum tinnitus loudness and mean tinnitus loudness were calculated to investigate convergent validity. RESULTS: The original eight-factor structure could be confirmed in the Dutch version of the TFI. Internal consistency (a=0.96) and convergent validity showed good results. Statistically significant correlations were found with the VAS for maximum loudness (r=0.59; p<0.001), VAS for mean loudness (r=0.66; p<0.001), and percentage of time aware of tinnitus (r=0.58; p<0.001). CONCLUSIONS: The Dutch version of the TFI is suitable for measuring in clinical and research settings how tinnitus affects daily life, with psychometric properties in line with the original version.

Life-threatening macroglossia after posterior fossa surgery: a surgical positioning problem?

A 55-year-old woman was operated in the lateral park bench position with significant neck flexion and oral packing. Macroglossia was noticed immediately postoperatively after endotracheal extubation. The patient was reintubated for 13 days and subsequently required a tracheostoma. After the placement of the tracheostoma and the removal of the endotracheal tube, the congestion of the tongue decreased markedly within 24 hours. Macroglossia is a rare complication following posterior fossa procedures with few cases reported so far. It can cause airway obstruction, which could be a life-threatening complication, and it therefore requires prompt treatment. The aetiology of postoperative macroglossia remains uncertain and has been attributed to arterial, venous and lymphatic compression, mechanical compression, or neurogenic causes. This article describes new insights into aetiology and also describes preventive measures and possible treatment.

Middle ear capillary haemangioma causing vestibulocochlear symptoms: a case report.

PROBLEM: A 58-year-old man presented with transient vertigo and pulsatile tinnitus. METHODS: High-resolution computed tomography, magnetic resonance imaging, excision, and subsequent immunohistochemical assays were performed. RESULTS: Imaging showed a soft tissue mass in the epitympanum and mastoid with bone erosion of the tegmen tympani and a dural tail sign, suggesting meningioma. Subsequently, because of signs of clinical progression, a canal-wall-up attico-antromastoidectomy was performed, with near-complete removal of a granulomatous, ossifying, haemorrhagic mass. CONCLUSIONS: Radiological imaging was critical in determining the extent of the mass and excluding other pathologies. Due to the atypical clinical and radiological signs, the final diagnosis of capillary haemangioma of the middle ear and temporal bone was made only after surgical resection and histopathological examination with immunohistochemistry, which excluded meningioma. The contiguous occurrence of cutaneous capillary haemangioma of the lateral face and neck was an important clue to the diagnosis.

Impact of type D personality on adherence to oral appliance therapy for sleep-disordered breathing.

PURPOSE: Type D personality, defined as a combination of social inhibition and negative affectivity, has been associated with poor medication adherence and lower adherence to continuous positive airway pressure in patients with sleep-disordered breathing. Up to this date, the association of patient's personality with adherence with a mandibular advancement device (MAD) has not been studied. The purposes of this study were to examine the association between type D personality and poor adherence to MAD treatment and to examine the impact of type D personality on perceived side effects during this treatment. METHODS: Eighty-two patients out of 113 patients with a known baseline type D scale who have started MAD treatment between June 2006 and December 2009 were included. Information about side effects and adherence were collected via a postal questionnaire. Thirty-three patients were using a monobloc MAD and 49 patients were using a duobloc MAD. RESULTS: Forty-five percent of type D patients discontinued MAD treatment, whereas only 15 % of non-type D patients reported treatment discontinuation. The odds ratio for treatment discontinuation was 6.03 (95 % confidence interval 1.22-29.81; p = 0.027) for type D personality, adjusted for age, gender, MAD type (monobloc or duobloc), and decrease in apnea severity. In continuing MAD users, no significant difference in perceived side effects was reported between the personality types. CONCLUSION: This is the first study to examine the relationship between type D personality and adherence to MAD treatment. Type D patients reported a significantly higher discontinuation rate when compared to patients without type D personality.

Treatment of obstructive sleep apnea using a custom-made titratable duobloc oral appliance: a prospective clinical study.

PURPOSE: This prospective clinical study investigates the efficacy of a specific custom-made titratable mandibular advancement device (MAD) for the treatment of obstructive sleep apnea (OSA). This MAD has attachments in the frontal teeth area that allow for progressive titration of the mandible. METHODS: Sixty-one adult OSA patients were included (age, 46.7 ± 9.0 years; male/female ratio, 45/16; apnea-hypopnea index (AHI), 23.2 ± 15.4 events/h sleep; body mass index, 27.9 ± 4.1 kg/m²). After an adaptation period, titration started based on a protocol of symptomatic benefit or upon reaching the physiological limits of protrusion. As a primary outcome, treatment response was defined as an objective reduction in AHI following MAD treatment of ≥50 % compared to baseline, and treatment success as a reduction in AHI with MAD to less than 5 and 10 events/h sleep. Compliance failure was defined as an inability to continue treatment. RESULTS: A statistically significant decrease was observed in AHI, from 23.4 ± 15.7 at baseline to 8.9 ± 8.6 events/h with MAD (p < 0.01). Treatment response was achieved in 42 out of 61 patients (68.8 %), whereas 42.6 % met criteria of AHI < 5 and 63.9 % achieved an AHI < 10 events/h sleep, respectively. Four patients (6.6 %) were considered as "compliance failures." CONCLUSIONS: The present study has evaluated the efficacy of a specific custom-made titratable MAD in terms of sleep apnea reduction.

Nonsyndromic hearing impairment is associated with a mutation in DFNA5.

Nonsyndromic hearing impairment is one of the most heterogeneous hereditary conditions, with more than 40 loci mapped on the human genome, however, only a limited number of genes implicated in hearing loss have been identified. We previously reported linkage to chromosome 7p15 for autosomal dominant hearing impairment segregating in an extended Dutch family (DFNA5). Here, we report a further refinement of the DFNA5 candidate region and the isolation of a gene from this region that is expressed in the cochlea. In intron 7 of this gene, we identified an insertion/deletion mutation that does not affect intron-exon boundaries, but deletes five G-triplets at the 3' end of the intron. The mutation co-segregated with deafness in the family and causes skipping of exon 8, resulting in premature termination of the open reading frame. As no physiological function could be assigned, the gene was designated DFNA5.

Mutations in the human alpha-tectorin gene cause autosomal dominant non-syndromic hearing impairment.

The tectorial membrane is an extracellular matrix of the inner ear that contacts the stereocilia bundles of specialized sensory hair cells. Sound induces movement of these hair cells relative to the tectorial membrane, deflects the stereocilia, and leads to fluctuations in hair-cell membrane potential, transducing sound into electrical signals. Alpha-tectorin is one of the major non-collagenous components of the tectorial membrane. Recently, the gene encoding mouse alpha-tectorin (Tecta) was mapped to a region of mouse chromosome 9, which shows evolutionary conservation with human chromosome 11q (ref. 3), where linkage was found in two families, one Belgian (DFNA12; ref. 4) and the other, Austrian (DFNA8; unpublished data), with autosomal dominant non-syndromic hearing impairment. We determined the complete sequence and the intron-exon structure of the human TECTA gene. In both families, mutation analysis revealed missense mutations which replace conserved amino-acid residues within the zona pellucida domain of TECTA. These findings indicate that mutations in TECTA are responsible for hearing impairment in these families, and implicate a new type of protein in the pathogenesis of hearing impairment.

Auditory neuropathy: a challenge for diagnosis and treatment.

In current terminology, auditory neuropathy spectrum disorder (ANSD) is a disease involving the disruption of the temporal coding of acoustic signals in auditory nerve fibres, resulting in the impairment of auditory perceptions that rely on temporal cues. There is debate about almost every aspect of the disorder, including aetiology, lesion sites, and the terminology used to describe it. ANSD is a heterogeneous disease despite similar audiological findings. The absence of an auditory brainstem response (ABR) and the presence of otoacoustic emissions (OAE) suggest an ANSD profile. However, to determine the exact anatomical site of the disorder, more in-depth audiological and electrophysiological tests must be combined with imaging, genetics and neurological examinations. Greater diagnostic specificity is therefore needed to provide these patients with more adequate treatment.

Intracranial complications of acute otitis media and Gradenigo's syndrome.

We describe the case of a 12-year-old girl with acute otitis media complicated by acute mastoiditis, epidural empyema, thrombosis of the sigmoid sinus and paralysis of the abducens nerve. The patient underwent a mastoidectomy on the left side combined with drainage of the epidural empyema through an extended burr holl and received intravenous antibiotics for 6 weeks and anticoagulation for 12 weeks. This report discusses the intracranial complications of acute otitis media, which were a common problem before the advent of adequate antibiotic drugs but have become rare since their introduction.