French adaptation and validation of the Speech, Spatial and Qualities of Hearing scale for Parents (SSQ-P) and for Children (SSQ-Ch).

OBJECTIVES: Hearing loss can seriously impact children's daily life. This study aims to translate and validate the French versions of the hearing performance questionnaires, SSQ-Parent (for 5-18 years old children), and SSQ-Children (for 11-18 years old children). DESIGN: This controlled prospective trial was conducted between April and October 2020. The forward-backward translation method was used, and a test-retest procedure was carried out on a case and a control population. Cases had at least 30 dBHL hearing loss. STUDY SAMPLE: 54 cases (mean age 10.4 years old) and 32 controls (mean age 12.5 years old) answered the SSQ-Parent. 35 cases (mean age 13.1 years old) and 35 controls (mean age 14.3 years old) answered the SSQ-Children. RESULTS: Spearman's correlation coefficients between global scores of the test and re-test were 0.91 (p < 0.001) for SSQ-Parent, and 0.89 (p < 0.001) for SSQ-Children. Both tests were discriminant (respectively, global score 57.8 vs 92 p < 0.001, 61.2 vs 92.6 p < 0.001), and internally consistent (Cronbach's alpha 0.94 and 0.97). Items-global score correlation was satisfactory. ROC curves showed high area under curve for the SSQ-Children (0.990), and SSQ-Parent (0.988). CONCLUSION: The SSQ-Parent and SSQ-Children revealed excellent statistical properties, and can be used for the evaluation of hearing performance of children.

French adaptation and validation of parents' evaluation of aural/oral performance of children (PEACH) scale in children.

OBJECTIVE: Hearing loss can seriously impact children's quality of life. Disease-specific questionnaires are required to optimise medical care. This study aims to translate, adapt and validate the French version of the PEACH score for the auditory performance of children. DESIGN: This is a controlled, prospective study, conducted between April and October 2020. The translation was conducted using a forward-backward technique, and statistical validation was conducted with a test and re-test, on a patient population and a control population. STUDY SAMPLE: Patients were included if they were 1-11 years old, and had at least 30 dB hearing loss in one ear. The mean age was 6 years for the 39 patients and 3.9 years for the 34 controls. RESULTS: Reproducibility, measured by Spearman's coefficient between global scores of the test and re-test was 0.78 (p < 0.001). The test was internally consistent (Cronbach's alpha was 0.89) and item per item construct validity was satisfactory. The ROC curve showed a moderate area under the curve (0.74 p < 0.001) with 67% sensitivity and 73% specificity. CONCLUSIONS: The French PEACH had good statistical properties, although a brief 13-item questionnaire, and can be used for evaluation of the disease-specific quality of life for young children with hearing loss.

Long-term outcomes of retrospective case series of middle ear implantation with Vibrant Soundbridge in children with congenital aural atresia.

PURPOSE: to assess audiological performance in quiet and noise, quality of life and side effects of Vibrant Soundbridge (VSB) in children with congenital aural atresia (CAA). METHODS: A retrospective study including consecutive patients with unilateral or bilateral CAA implanted with VSB from 2009 to 2020 in a tertiary referral centre. RESULTS: 18 patients with CAA and a present stapes were included (3 simultaneous bilateral VSB implants) and 21 ears: 17 VSB were attached to the short incus process, and four to the stapes. Age at implantation ranged from 4.7 to 15.8 years. Average follow-up was 6.5 years (± 3.7 years). In unilateral VSB, air conduction pure tone average (ACPTA) thresholds increased from 75.3 ± 15.2 to 32.6 ± 8.3 dB post-operatively (VSB activated) (n = 15; p < 0.01). The speech reception threshold (SRT) and the word recognition score (WRS) were significantly improved from 81.5 ± 10.4 to 43.9 ± 7.6 dB and 0% to 84.8 ± 8.5% postoperatively (n = 15; p < 0.01). The signal to noise ratio (SNR) was significantly improved from 2.1 ± 2.9 dB VSB inactivated to 0.3 ± 2.7 dB VSB activated (n = 15; p < 0.01). There was no significant difference in performance according to floating mass transducer (FMT) placement. 5/15 children were non-users at last follow-up in unilateral VSB and 0/3 in bilateral. CONCLUSIONS: CAA ears with VSB activated had a significant improvement of ACPTA, WRS, SRT and SNR. A third of patients with unilateral CAA became non-users at last follow-up. The main challenge is to target the indications for the implantation of the VSB to avoid its discontinuation.

Congenital abnormalities associated with microtia: A 10-YEARS retrospective study.

OBJECTIVE: Microtia is a congenital auricular malformation, often part of a syndromic form (35%-55% of cases). The accurate prevalence of associated malformations remains to be determined with regard to the heterogeneous results of the previous studies. This study aims to describe in a large population cohort the abnormalities associated with microtia and to determine the most suitable assessment for these children. METHODS: This is a retrospective and observational cohort study collecting data from the medical records of children affected by microtia, diagnosed or followed-up between 2007 and 2017. Data were collected via a computer database. Clinical data, as well as imaging or genetic results, were noted. RESULTS: Six hundred ninety four children were included, 587 (84.6%) with unilateral and 107 (15.4%) with bilateral microtia. Inner ear malformations were observed in 14.1% of the ears. The main associated anomalies were hemifacial microsomia (29%), velopharyngeal insufficiency (9%), ophthalmologic (6.2%), vertebral (5.9%), cardiac (5.5%) and kidney (3%) abnormalities. Main identified entities were Goldenhar, Treacher-Collins and Guion-Almeida syndromes. CONCLUSION: A comprehensive clinical assessment must be completed when microtia is diagnosed. Besides screening well-known oculo-auriculo-vertebral spectrum malformations, velopharyngeal insufficiency should be systematically sought. Specialized care must be provided to the very frequently associated hemifacial macrosomia. Mild forms of this last malformation may correspond to Guion-Almeida syndrome, especially in cases of learning disability.

Pain After Cochlear Implantation: An Unusual Complication?

OBJECTIVE: To analyze clinical signs and better define the underestimated long-term pain after implantation, to discuss etiological hypothesis, and to propose our department treatment algorithm and results. STUDY DESIGN AND SETTING: Retrospective review of children implanted with complaints of atypical pain in the area of device implantation, not in the immediate postoperative period, more than or equal to 4 on the Visual Analog Pain Scale (VAPS: 0-10) in the ENT pediatric department of Trousseau and Necker Enfants Malades Hospitals between 1998 and 2015. PATIENTS: All patients had full clinical and electrophysiological checking, and had normal functioning device. Exclusion criteria were: related history of local trauma, ongoing skin infection, magnet displacement, and device failure. INTERVENTION(S): Treatments and outcomes were reviewed, with a minimum of 6 months follow up. Two groups were analyzed: Group PS: pain associated with local swelling (n = 9) and Group P: isolated pain (n = 11). The first-line treatment was medical. In Group PS, anti-inflammatory, pain medication, and antibiotics were used at the same time; in Group P, only anti-inflammatory and pain medication was used. MAIN OUTCOME MEASURE(S): Success was defined when complete resolution of pain and swelling (pain scale = 0). RESULTS: Twenty cases out of the 1,448 implanted patients in our department (1.4%) were included. Average age at first occurrence of pain was 15 years (3-22 yr). Mean delay between surgery and pain complaint was 5.8 years (0.25-14). Mean follow up was 2 years (0.5-5 yr).First line medical treatment was successful in eight cases (40%). Of the 12 patients who failed medical treatment and required surgery, two had resolution of pain with magnet change and 10 had resolution with reimplantation. (5/9 patients in Group PS and 7/11 in Group P).Microbiology was performed in 10/20 cases and analysis of explanted devices was performed in 7/10 cases. Positive microbiological culture of soft tissues was positive for 3/10 cases, biofilm was positive for 5/7 cases.Pain in the area of the implanted device can occur shortly after surgery or on long-term follow up. It has been seen in all device types. Pain may be clinically underestimated, as low VAPS grade (<5) or intermittent pain may be not reported. Low-grade infections might be a hypothesis to explain these pain. Management may include medical or surgical intervention. CONCLUSION: Pain in the implanted area can be a major complication leading to implant non-use. Rate may be underestimated because of the lack of medical report. At explantation, we recommend systematic evaluation of biofilm and device failure regardless of the suspected etiology of the pain.

Velopharyngeal insufficiency managed by autologous fat grafting in patients with aberrant courses of internal carotid arteries.

INTRODUCTION: Velopharyngeal insufficiency (VPI) is usually managed, besides speech therapy, by performing a velopharyngoplasty. An alternative approach is autologous fat grafting (AFG) of the posterior pharyngeal wall. About 5% of the population has internal carotid arteries (ICA) with an aberrant course. This anatomic variation can be responsible for surgical difficulties while when performing a velopharyngoplasty, and therefore lead surgeons to only consider a speech reeducation of VPI. However, AFG is does not bear such surgical morbidity. OBJECTIVE: The aim of this study is to retrospectively determine AFG efficiency on VPI in patients with aberrant ICA's courses who cannot benefit from a velopharyngoplasty, by comparing pre- and postoperative Borel Maisonny score (BMS) and intelligibility (Intell). METHODS: We conducted a retrospective study in 2 centers, including children with VPI and aberrant ICA's courses who underwent an AFG of the posterior pharyngeal wall from 2004 to 2015, in addition to speech therapy. RESULTS: Nine patients (4-11 years old) underwent the surgical procedure, 8 of them presenting a 22q11 microdeletion. All improved their VPI by AFG of the pharyngeal wall according to BMS and Intelligibility after a 10 months follow-up. The effect was stable after 3 years of follow-up. No severe complication (apnea, vascular injury) occured. CONCLUSION: AFG of the pharyngeal wall, associated with speech therapy, seems to be a safe procedure for patients with VIP and aberrant ICA's courses. Multiple procedures are possible if needed.

Otogenic Temporomandibular Arthritis in Children.

Importance: Septic arthritis of the temporomandibular joint (SATMJ) is a very rare but potentially severe complication of pediatric middle ear infections because it presents risks of TMJ ankylosis. Objective: To describe the clinical, radiological, biological, and microbiological characteristics and evolution of SATMJ complicating middle ear infections (otogenic SATMJ) in children. Design, Setting, and Participants: This multicenter retrospective study included all children younger than 18 years referred between January 1, 2005, and December 31, 2015, for otogenic SATMJ or for TMJ ankylosis that occurred a few months to a few years after an acute mastoiditis. Nine children were included in the study. Review of the children's medical charts was conducted from February 1, 2016, to April 1, 2016. Main Outcomes and Measures: Patients' demographic characteristics and symptoms; radiological, biological, and bacteriological findings, including reanalysis of initial imaging; and treatment and outcome of SATMJ. Results: Of the 9 children, 6 were boys and 3 were girls; the mean age was 2.1 years (range, 6 months to 4.7 years). In 7 cases (78%), the primary middle ear infection was acute mastoiditis. Clinically, 5 children (55%) had preauricular swelling and only 1 (11%) had trismus. Associated thrombophlebitis of the lateral sinus or intracranial collections was present in 7 cases (78%). An initial computed tomographic scan was performed for all but 1 patient, and second-line analysis detected clear signs of TMJ inflammation in all 8 children who had a computed tomographic scan. However, SATMJ was diagnosed in only 3 cases at the time of the initial middle ear infection, leading to the recommendation of TMJ physical therapy for several months. The most frequently involved bacteria was Fusobacterium necrophorum, which was found in 4 cases. Long-term ankylosis was identified in 6 cases (67%), and 5 of these children required surgical treatment. Conclusions and Relevance: Clinicians and radiologists must thoroughly look for signs of SATMJ in children with acute mastoiditis to detect this complication, which can lead to disabling and hard-to-treat TMJ ankylosis.

Cone beam computed tomography and histological evaluations of a straight electrode array positioning in temporal bones.

CONCLUSION: CBCT allows a precise evaluation of the dimensions and of the shape of the cochlear duct that is of primary importance for the choice of the length and the design of the electrode array. This radio-histological study confirms that CBCT is a reliable tool to be used in clinical studies to evaluate the position of straight electrode arrays within the cochlear scala. OBJECTIVE: To validate the reliability of cone beam computed tomography (CBCT) in the evaluation of cochlear anatomy and positioning of a straight electrode array, by comparing radiological images to histological analysis of cochlear implanted temporal bones. METHODS: Eight temporal bones for four subjects were analysed before and after cochlear implantation with a straight electrode array. The size of the cochlea, the dimensions, and shape of the cochlear duct at 180° and 360° were evaluated on CBCT performed before implantation. Temporal bones then underwent histological analysis to determine the position of the electrode array. Scalar localization of the electrode array was assessed on CBCT performed after implantation, and compared with histological study. RESULTS: CBCT is a reliable radiological technique to differentiate variability not only of cochlear dimensions, but also of size and shape of the cochlea. Histological analysis confirmed the scalar position of the electrode array assessed by the CBCT in 7/8 temporal bones, but soft tissue trauma such as dissection of the spiral ligament was not identified by the CBCT.

Results of VSB implantation at the short process of the incus in children with ear atresia.

OBJECTIVE(S): To describe a new and more simple surgical procedure for implanting the Vibrant Soundbridge (VSB) on the short process of the incus in children with ear atresia and atretic plate and present the audiometric results. METHODS: Since 2014, pre- and post-operative audiometric tests with tonal and vocal audiometryat the maximal follow up, respectively 33, 22 and 12 months were performed after VSB implantation at the ENT department, Necker Enfants Malades Hospital, Paris, France. 3 children aged 11, 9 and 15 years with conductive hearing loss due to high grade ear atresia and absent ear canal were implanted. The malformed and fused malleus-incus complex was found to be immobile in two of the subjects, who then underwent a superior tympanotomy to carefully remobilize the malleus. Stapes were mobile in all cases. The FMT clip was customized and crimped on the short process, stabilized by the metallic wire. RESULTS: At the maximum follow-up time, the bone conduction was unchanged, and the mean aided ACPTA was 21 dB, 29 dB and 30 dB, compared to 66 dB, 63 dB and 68 dB unaided, respectively. The word recognition score (WRS) at 65 dB SPL increased from 10%, 10% and 70% (unaided) respectively to 100% (aided). CONCLUSIONS: The long process of the incus is usually very hypoplastic in patients with ear atresia with a fused malleus-incus complex lateral to the stapes. VSB coupling to the long process of the incus or the stapes may be challenging in small mastoids. Coupling to the short process of the incus is comparatively a simple procedure, since there is no need for posterior tympanotomy and has no proximity to the facial nerve. The results of this study are similar to other ear atresia cases in the literature with classic FMT placement on the stapes or long process of the incus. Outcomes should be confirmed in a larger number of patients.

Mastoid fascia kite flap for cryptotia correction.

OBJECTIVE: Cryptotia is one of the most common malformations of the upper auricle with aesthetic and functional consequences, however there is no standard treatment. We present the surgical technique and results of a kite flap procedure which can be used in the different cryptotia subtypes. METHODS: We reviewed all patients treated in our department from 2010 to 2015, using a mastoid fascia kite flap technique. The incision of this local flap follows the retro-auricular sulcus along the rim of the helix superiorly and drawing a skin paddle inferiorly. The mastoid fascia is exposed and a superiorly and posteriorly based flap is drawn and detached from the skull. Finally, the skin paddle is rotated and sutured between the superior helix and temporal skin creating the superior sulcus. The retro-auricular incision is closed directly inferiorly. RESULTS: Six patients (mean age 12) and seven ears were studied. One patient had bilateral cryptotia and only two had a normal contralateral ear. Mean follow-up was of 45 months. There was no skin necrosis, no complications reported and no revision surgery. CONCLUSIONS: We describe a reliable flap with a simple design and improved aesthetic result, as the thickness of the flap projects the helix well, the scar is entirely hidden in the retro-auricular sulcus and the direct suture induces a harmonious medialization of the inferior part of the ear and earlobe.

Superparamagnetic nanoparticles as vectors for inner ear treatments: driving and toxicity evaluation.

Conclusion Super paramagnetic nanoparticles (MNP) are a promising vector to achieve controlled drug delivery into the cochlea. Objective The goal of the study was to evaluate the toxicological risk of MNP upon the inner ear. Methods Fe3O4-MNP displacement was studied in various catheter materials, shape, and solvent with a local magnetic field. EC5V cells (derived from the inner ear) were cultured with MNP (100 and 500 nm) at various concentrations or without MNP. Cell survival was assessed with a flow cytometry analysis. Localization of MNP within the cells was studied with confocal microscopy. In vivo, a single intra-cochlear administration of 200 nm MNP (3 × 10(10)MNP/mL, n = 8; 1.5 × 10(12) MNP/mL, n = 6) or saline (n = 14) was performed in guinea pigs. Hearing thresholds were assessed with auditory brainstem responses at Day 7. Results MNP could be concentrated at different locations of the catheter with sequential activation of solenoids. MNP were internalized in the cytoplasm, but not in the nuclei nor in endosomes at 48 h. After 48 h of incubation, no difference for cell survival between the groups was observed, whatever the MNP concentration. A size effect was observed with less survival in the 100 nm group. In guinea pigs at day 7, hearing threshold shift was not different in the three groups.