HDR syndrome: Large cohort and systematic review.

HDR syndrome is a rare disease characterized by hypoparathyroidism, deafness, and renal dysplasia. An autosomal dominant disease caused by heterozygous pathogenic GATA3 variants, the penetrance of each associated condition is variable. Literature reviews have provided some answers, but many questions remain, in particular what the relationship is between genotype and phenotype. The current study examines 28 patients with HDR syndrome combined with an exhaustive review of the literature. Some conditions such as hearing loss are almost always present, while others described as rare initially, do not seem to be so rare after all (genital malformations and basal ganglia calcifications). By modeling pathogenic GATA3 variants found in HDR syndrome, we found that missense variations appear to always be located in the same area (close to the two Zinc Finger domain). We describe new pathogenic GATA3 variants, of which some seem to always be associated with certain conditions. Many audiograms were studied to establish a typical audiometric profile associated with a phenotype in HDR. As mentioned in the literature, hearing function should always be assessed as early as possible and follow up of patients with HDR syndrome should include monitoring of parathyroid function and vesicoureteral reflux in order to prevent complications.

Pediatric Cholesteatoma Follow-Up: Residual and Recurrence in 239 Cases with Over 5-Year Hindsight.

INTRODUCTION: Pediatric cholesteatoma is an aggressive disease which requires long-term evaluation to assess management strategies. The objective was to determine optimal follow-up duration in pediatric cholesteatoma to detect residual and recurrent diseases. METHODS: This cohort study was set in a tertiary referral center. All consecutive patients with a minimum 5-year follow-up were included. Medical history, initial extension, surgical procedures, and follow-up were collected. The main outcome measure were Kaplan-Meier survival curves of residual and recurrence cumulative incidence. RESULTS: Totally 239 ears with the first tympanoplasty between 2008 and 2014 were studied including 25% congenital. At first surgery (S1), mean age was 8.4 years and mean follow-up time 7.9 years. Mastoidectomy was performed in 69% and stapes was absent in 38% of cases. Notably, 83% (199 ears) had a second procedure (S2) of which 186 were planned. After S1, maximum cumulated incidence of residual was 45% [95%CI, 38%; 52%] at 74 months, with probability of residual of 39% at 3 years. After S2 (n = 199), maximum cumulated residual incidence was 21% [95%CI, 12%; 32%] at 62 months and 16% at 3 years. Concerning recurrence, maximum cumulated incidence after S1 (n = 239) was reached at 98 months with 21% [95%CI, 12%; 32%], 13% at 3 years and 16% at 5 years. Congenital disease had significantly less residuals after S1 (p = 0.02), but similar recurrence rate (p = 0.66) compared with acquired. CONCLUSIONS AND RELEVANCE: We recommend MRI follow-up of at least 5 years after the last surgery for residual disease and clinical follow-up of at least 10 years to detect recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.

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.

Robot-Assisted Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma.

Congenital cholesteatoma accounts for 25% of cholesteatoma cases in children. Transcanal Endoscopic Ear Surgery (TEES) is ideal for these patients because it offers a wide endoscopic view of the middle ear and a minimally invasive approach. The two main limitations are the loss of one operative hand and a narrow external auditory canal in younger children. Here, we present the case of a 3-year-old patient with a Potsic stage III congenital cholesteatoma adherent to the incus and branches of the stapes. A robotic-assisted TEES procedure was performed, during which a robotic arm with 6 degrees of freedom held a 0°, 2.9 mm wide endoscope, enabling the surgeon to work in a narrow environment with both hands. The procedure's duration was 2 h and 9 min, including 16 min for the installation and draping of the robotic arm. After a trans-canal approach, the cholesteatoma was dissected from the ossicles using both a needle (or sickle knife) and suction to stabilize the ossicles and limit the risk of hearing trauma. The cholesteatoma was debulked to reduce its size, allowing it to be pushed under the malleus anteriorly and then separated from other adherences before removal. A tragal cartilage graft was used to reinforce the tympanic membrane.

Retinal Phenotype of Patients with CLRN1-Associated Usher 3A Syndrome in French Light4Deaf Cohort.

Purpose: Biallelic variants in CLRN1 are responsible for Usher syndrome 3A and non-syndromic rod-cone dystrophy (RCD). Retinal findings in Usher syndrome 3A have not been well defined. We report the detailed phenotypic description of RCD associated with CLRN1 variants in a prospective cohort. Methods: Patients were clinically investigated at the National Reference Center for rare ocular diseases at the Quinze-Vingts Hospital, Paris, France. Best-corrected visual acuity (BCVA) tests, Goldmann perimetry, full-field electroretinography (ffERG), retinal photography, near-infrared reflectance, short-wavelength and near-infrared autofluorescence, and optical coherence tomography (OCT) were performed for all patients. Results: Four patients from four unrelated families were recruited. Mean follow-up was 11 years for three patients, and only baseline data were available for one subject. Median BCVA at baseline was 0.2 logMAR (range, 0.3-0). ffERG responses were undetectable in all subjects. The III4e isopter of the Goldmann visual field was constricted to 10°. The retinal phenotype was consistent in all patients: small whitish granular atrophic areas were organized in a network pattern around the macula and in the midperiphery. OCT showed intraretinal microcysts in all patients. Upon follow-up, all patients experienced a progressive BCVA loss and further visual field constriction. Four distinct pathogenic variants were identified in our patients: two missense (c.144T>G, p.(Asn48Lys) and c.368C>A, p.(Ala123Asp)) and two frameshift variants (c.176del, p.(Gly59Valfs*13) and c.230dup, p.(Ala78Serfs*52)). Conclusions: RCD in Usher 3A syndrome has some distinctive features. It is a severe photoreceptor dystrophy with whitish granular posterior pole appearance and cystic maculopathy.

Auditory processing disorder in children: the value of a multidisciplinary assessment.

PURPOSE: Auditory processing disorder (APD) may affect 0.2-5% of the paediatric population. The diagnosis of APD remains difficult because of polymorphic symptoms possibly entangled with other difficulties. The purpose of this study was to evaluate a new multi-disciplinary assessment in the French language. METHODS: The battery of tests was composed of: (a) APD targeted speech assessment: speech perception in noise, a dichotic test, temporal processing tests (patterns); (b) Psychometric assessment: sustained auditory attention, sustained visual attention, evaluation of cognitive functions; (c) phonemic identification and discrimination; (d) ENT examination, tonal and vocal audiometry and ABR recordings. The diagnosis was made if two of the targeted speech tests were 2 standard deviations (SDs) below the mean or if only one of the tests was 3 SDs below. The auditory attention tests, as well as the phonemic identification and discrimination tests were complementary to the diagnostic battery. However, they did not allow for the diagnosis of APD. RESULTS: 50 children suspected of APD benefited from this protocol, and 12 were excluded from the study. A diagnosis of APD was confirmed in 17 children (45%). 59% of the patients had associated disorders. The most effective tests for diagnosing APD were dichotic testing (p = 0.001) and pattern recognition (frequency, p = 0.001). The sustained auditory attention test (p = 0.01) and the phonemic identification and discrimination test reinforced the diagnosis of APD. CONCLUSION: It seems important to evaluate children suspected of APD with a multi-disciplinary protocol. It makes it possible to diagnose APD children, but also to identify attentional difficulties and cognitive disorders that may be associated.

Malformations Associated With Pediatric Congenital Cholesteatomas.

OBJECTIVE: To describe malformations associated with pediatric congenital cholesteatomas of the middle ear. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: One hundred and seventy-three cases of middle ear congenital cholesteatoma (CC) in 171 children operated between 2007 and 2017. INTERVENTIONS: Demographic, clinical, and surgical data were collected from operative reports. MAIN OUTCOME MEASURES: We first described the type and rate of malformations associated with CC. Secondly, we compared cholesteatoma features in two subgroups: anterior superior (AS) versus posterior superior (PS) starting point. Third, we compared demographic, clinical, and surgical data between patients with and without malformation. RESULTS: CC was associated with malformations in 17 cases (17/173; 9.8%). The main malformation was preauricular fistula (8/173; 4.6%). Other malformations were: one first branchial cleft, two labio palatine cleft, one nasal cyst, two preauricular fibrochondroma, and five other malformations. PS congenital cholesteatomas were diagnosed in older children (4.6 versus 8.6 years, p < 0.05) and had greater extension in middle ear than the AS cholesteatoma (39.7% versus 95.8%, p < 0.05). We did not find any significant difference between these two groups regarding the associated malformations. We did not find a difference in clinical presentation of CC between patients with and without associated malformation. CONCLUSIONS: We found various associated malformations in 9.8% of CC cases with no statistical difference in the malformation rate between AS and PS groups. All the malformations were located in the craniofacial region suggesting that genes implicated in craniofacial development may play a role in the pathophysiology of CC.

Mutation m.3395A > G in MT-ND1 leads to variable pathologic manifestations.

A non-synonymous mtDNA mutation, m.3395A > G, which changes tyrosine in position 30 to cysteine in p.MT-ND1, was found in several patients with a wide range of clinical phenotypes such as deafness, diabetes and cerebellar syndrome but no Leber's hereditary optic neuropathy. Although this mutation has already been described, its pathogenicity has not been demonstrated. Here, it was found isolated for the first time, allowing a study to investigate its pathogenicity. To do so, we constructed cybrid cell lines and carried out a functional study to assess the possible consequences of the mutation on mitochondrial bioenergetics. Results obtained demonstrated that this mutation causes an important dysfunction of the mitochondrial respiratory chain with a decrease in both activity and quantity of complex I due to a diminution of p.MT-ND1 quantity. However, no subcomplexes were found in cybrids carrying the mutation, indicating that the quality of the complex I assembly is not affected. Moreover, based on the crystal structure of p.MT-ND1 and the data found in the literature, we propose a hypothesis for the mechanism of the degradation of p.MT-ND1. Our study provides new insights into the pathophysiology of mitochondrial diseases and in particular of MT-ND1 mutations.

PHENOTYPIC CHARACTERISTICS OF ROD-CONE DYSTROPHY ASSOCIATED WITH MYO7A MUTATIONS IN A LARGE FRENCH COHORT.

PURPOSE: To document the rod-cone dystrophy phenotype of patients with Usher syndrome type 1 (USH1) harboring MYO7A mutations. METHODS: Retrospective cohort study of 53 patients (42 families) with biallelic MYO7A mutations who underwent comprehensive examination, including functional visual tests and multimodal retinal imaging. Genetic analysis was performed either using a multiplex amplicon panel or through direct sequencing. Data were analyzed with IBM SPSS Statistics software v. 21.0. RESULTS: Fifty different genetic variations including 4 novel were identified. Most patients showed a typical rod-cone dystrophy phenotype, with best-corrected visual acuity and central visual field deteriorating linearly with age. At age 29, binocular visual field demonstrated an average preservation of 50 central degrees, constricting by 50% within 5 years. Structural changes based on spectral domain optical coherence tomography, short wavelength autofluorescence, and near-infrared autofluorescence measurements did not however correlate with age. Our study revealed a higher percentage of epiretinal membranes and cystoid macular edema in patients with MYO7A mutations compared with rod-cone dystrophy patients with other mutations. Subgroup analyses did not reveal substantial genotype-phenotype correlations. CONCLUSION: To the best of our knowledge, this is the largest French cohort of patients with MYO7A mutations reported to date. Functional visual characteristics of this subset of patients followed a linear decline as in other typical rod-cone dystrophy, but structural changes were variable indicating the need for a case-by-case evaluation for prognostic prediction and choice of potential therapies.

High prevalence of congenital deafness on Reunion Island is due to a founder variant of LHFPL5.

Reunion Island is a French oversea department in the Indian Ocean with 1.6/1000, an estimated prevalence of deafness that is almost double as compared to the mainland France. Twelve children having isolated bilateral prelingual profound deafness along with motor delay attributed to vestibular areflexia were enrolled. Their mean walking age was 19 months. Electroretinography and temporal bone CT-scans were normal in all cases. A novel homozygous frameshift lipoma HMGIC fusion partner-like 5 (LHFPL5) variant c.185delT p.(Phe62Serfs*23) was identified using whole-exome sequencing. It was found in seven families. Four patients from two different families from both Reunion Island and mainland France, were compound heterozygous: c.185delT p.(Phe62Serfs*23) and c.472C > T p.(Arg158Trp). The phenotype observed in our patients completely mimics the hurry-scurry (hscy) murine Tmhs knock-out model. The recurrent occurrence of same LHFPL5 variant in Reunion Island is attributed to common ancestor couple born in 1693.

Mutations in TUBB4B Cause a Distinctive Sensorineural Disease.

Leber congenital amaurosis (LCA) is a neurodegenerative disease of photoreceptor cells that causes blindness within the first year of life. It occasionally occurs in syndromic metabolic diseases and plurisystemic ciliopathies. Using exome sequencing in a multiplex family and three simplex case subjects with an atypical association of LCA with early-onset hearing loss, we identified two heterozygous mutations affecting Arg391 in ß-tubulin 4B isotype-encoding (TUBB4B). Inspection of the atomic structure of the microtubule (MT) protofilament reveals that the ß-tubulin Arg391 residue contributes to a binding pocket that interacts with α-tubulin contained in the longitudinally adjacent αß-heterodimer, consistent with a role in maintaining MT stability. Functional analysis in cultured cells overexpressing FLAG-tagged wild-type or mutant TUBB4B as well as in primary skin-derived fibroblasts showed that the mutant TUBB4B is able to fold, form αß-heterodimers, and co-assemble into the endogenous MT lattice. However, the dynamics of growing MTs were consistently altered, showing that the mutations have a significant dampening impact on normal MT growth. Our findings provide a link between sensorineural disease and anomalies in MT behavior and describe a syndromic LCA unrelated to ciliary dysfunction.

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.

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.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient.

OBJECTIVE: To provide recommendations for the workup of hearing loss in the pediatric patient. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group. RESULTS: Consensus recommendations include initial screening and diagnosis as well as the workup of sensorineural, conductive and mixed hearing loss in children. The consensus statement discusses the role of genetic testing and imaging and provides algorithms to guide the workup of children with hearing loss. CONCLUSION: The workup of children with hearing loss can be guided by the recommendations provided herein.

Hearing rehabilitation with the closed skin bone-anchored implant Sophono Alpha1: results of a prospective study in 15 children with ear atresia.

OBJECTIVE: To study gain and cutaneous tolerance of the Sophono Alpha1 implant, used for unilateral hearing rehabilitation in children with ear atresia, and to demonstrate non-inferiority compared to the referral closed skin device, BAHA® on a test-band. METHODS AND MATERIALS: Fifteen children included in a prospective clinical trial from September 2010 to November 2012. Tertiary care center. MAIN OUTCOMES: Pure tone and speech audiometry before surgery and at M6 and 12; Speech-in-noise tests with and without Alpha1 at M6. Quality of life questionnaire completed before surgery and at M6; children and parent's satisfaction assessed at M6. Cutaneous appearance assessed at M6, M12 and during the last clinical assessment. RESULTS: Patients' ages ranged from 61 to 129 months. They had a pure conductive deafness with a mean air conduction pure-tone average (ACPTA) of 69.02±9.31dB, and a mean SRT of 71.73±9.20dB. Follow-up was 12-32 months. At M6, the mean aided ACPTA was 33.49±4.89dB, the mean aided SRT 38.27±4.54dB and the mean aided SRT in noise was statistically improved (-7.80dB). Sophono Alpha1 demonstrated non-inferiority compared to BAHA® on a test-band. At M12, mean aided ACPTA was 2.94dB higher (p<0.001) but the mean SRT variation (+0.73dB) was not significantly different (p=0.35). At M12, all children used the implant 5 to 12h daily (mean: 10) without cutaneous complications. Both children and parents reported being satisfied or very satisfied. The score for 7/10 questions in silence or noisy environment was statistically improved when wearing the device. CONCLUSIONS: This prospective study demonstrate non inferiority, good cutaneous tolerance, satisfaction of children and parents and improvement of the quality of life with the Sophono Alpha1 compared to BAHA® on a test-band. In the light of this study and of the previously published series, the aided threshold is between that obtained with BAHA on a softband and percutaneous BAHA. The Sophono Alpha1 device needs to be part of the hearing solutions proposed for the hearing rehabilitation in children with ear atresia.

Pediatric cochlear implantation in residual hearing candidates.

OBJECTIVES: To propose categories for the various types of residual hearing in children and to review the outcomes of cochlear implantation (CI) in children with these different hearing conditions. METHODS: We identified 53 children with residual hearing who had received a cochlear implant. Five groups were arbitrarily defined based on auditory features: G1, characterized by low-frequency residual hearing (n=5); G2, characterized by severe sensorineural hearing loss (SNHL) and low speech discrimination (n=12); G3, characterized by asymmetric SNHL (n=9); G4, characterized by progressive SNHL (n=15); and G5, characterized by fluctuating SNHL (n=12). The main audiometric features and outcomes of the groups were analyzed. RESULTS: The mean age at implantation was 10.15 years (range, 2.5-21 years). The mean preoperative score for the discrimination of open-set words was 48%; this score increased to 74% at 12 months and 81% at 24 months after the CI procedure (G1 to G5, respectively: 79/62/77%, 50/81/88%, 59/75/86%, 35/74/67%, and 39/69/80%). Children who were implanted after 10 years of age did not improve as much as those who were implanted at a younger age (open-set word list speech perception [OSW] score at 12 months: 62% vs 83%; P=.0009). Shorter delays before surgery were predictive of better performance (P=.003). Inner ear malformation and SLC26A4 mutations were not predictive of the outcome. CONCLUSIONS: CIs provide better results compared with hearing aids in children with residual hearing. Factors that may impact the benefits of CIs in patients with residual hearing are age, delay in performing the CI procedure, which ear is implanted, and initial underestimation of the patient's hearing difficulties.

Cochlear implant failure and revision surgery in pediatric population.

OBJECTIVE: This study aimed to report pediatric cochlear reimplantation data focusing on failure of the device and to assess surgical challenges and functional outcomes. METHODS: Retrospective case review from 1990 to 2012. Tertiary referral academic center. A retrospective review of medical and surgical records, audiologic results, and report of the manufacturer's analysis was performed. The causes of revision cochlear implantation were divided into hard failure and soft failure. Results compared speech perception in open set or closed set words before and after revision surgery with cochlear implant only. RESULTS: During the study period, 877 cochlear implantations were performed. Our reimplantation rate for failure of the device is 5.7% (50 of 877). The main reasons for hard failure were loss of hermeticity and cracked casing following head trauma. Surgical difficulties might be due to neo-osteogenesis related to the use of bone dust. Initial atraumatic insertion is important to ensure that revision surgeries are less problematic. In postmeningitis deafness, initial choice of electrode array is of utmost importance. We would suggest a plain shape and a larger diameter array, to ensure sufficient subsequent insertion. Post-reimplantation audiologic results were the same or better than preoperative levels in 86% of cases. CONCLUSION: Failure might be difficult to diagnose in children with limited language skills. The surgical team and patients should be aware of the surgical difficulties that can be encountered, especially in cases of meningitis or initial traumatic insertion. Auditory performances after reimplantation are the same or better in most cases.

Cochlear implantation and vestibular function in children.

OBJECTIVES: To analyze vestibular function Q2 (VF) after cochlear implantation (CI) in children. METHODS: Retrospective cohort study at a tertiary pediatric referral center. 43 children that had vestibular testing (VT) after unilateral cochlear implantation, from 2001 to 2010, were included. CT scan of the temporal bone was done systematically before surgery. VT included three tests: head-impulse test, caloric tests, vestibular evoked myogenic potentials. VF was graded in: normal (type 1), partial dysfunction (type 2), severe dysfunction and areflexia (type 3). In 12 cases, VT was done before and after CI. Vestibular function was analyzed looking to side, etiology and preoperative status. RESULTS: Mean age at CI was 2.9 years. Bilateral inner ear malformation were retrieved in 16%. Before surgery, 50% of children had normal vestibular responses, 4/12 had bilateral type 2, two had asymmetrical VF. In this group, after surgery, 2 children had VF worsening, none on the CI side only. Considering all 43 patients, post operative VT showed normal response in 48.8% and type 2&3 in 16.2%. Children had asymmetrical poorer vestibular function on the side of CI in 19%. Among them, 75% had normal contralateral VF. Sensorineural hearing loss etiologies known to be associated with vestibulopathy (Usher/Meningitis/Inner Ear Malformations/CMV) were associated to abnormal vestibular function more frequently than in other causes (p=0.01). CONCLUSION: Half of the children had initial vestibular dysfunction. In our study, 20% of cochlear implantation could have worsened vestibular function. As vestibular function should be part of the choice in cochlear implantation, side of implantation and survey; and vestibular tests are uneasy to achieve in pediatric population, we propose a three-steps evaluation and gradation which allow easier comparison.

Benefits from upgrade to the CP810 sound processor for Nucleus 24 cochlear implant recipients.

The objective of this study was to measure performance benefits obtained by upgrading recipients of the Cochlear Nucleus CI24 cochlear implant to the new CP810 sound processor. Speech recognition in quiet and in spatially separated noise was measured in established users of the Cochlear ESPrit 3G (n = 22) and Freedom (n = 13) sound processors, using the "Everyday" listening program. Subjects were then upgraded to the CP810 processor and were re-assessed after a 3-month period, using both the "Everyday" program and the new "Noise" program, which incorporates several pre-processing features including a new directional microphone algorithm ("Zoom"). Subjective perceptions were also recorded using the abbreviated profile of hearing aid benefit (APHAB) questionnaire. Mean scores for monosyllables in quiet, presented at 50 and 60 dB SPL, increased by 11% (p < 0.0001) and 8% (p < 0.001), respectively, after upgrade, for all subjects combined. Significant increases were also recorded for both processor groups. In noise, the mean scores were 60.0 and 67.4% for the original and CP810 Everyday programs, respectively (difference not significant). With the CP810 Noise programs the mean score increased to 82.5% (p < 0.01), with significant increases in both processor groups. There was evidence of slightly greater upgrade benefit in users of the ESPrit 3G processor and in relatively poor performers. The APHAB questionnaire also indicated significant reduction in perceived difficulty in the background noise and reverberation sub-scales after upgrade. The findings of the study appear to support the expectation of increased benefit from the new CP810 sound processor.