Quality of life in bimodal hearing users (unilateral cochlear implants and contralateral hearing aids).

The main objective was to evaluate the bimodal self-rated benefits on auditory performance under real conditions and the quality of life in two groups of cochlear-implanted adults, with or without a contralateral hearing aid. The secondary objective was to investigate correlations between the use of a hearing aid and residual hearing on the non-implanted ear. This retrospective study was realized between 2000 and 2010 in two referral centers. A population of 183 postlingually deaf adults, implanted with a cochlear experience superior to 6 months, was selected. The Speech, Spatial, and other Qualities of Hearing Scale were administered to evaluate the auditory performances, and the Nijmegen Cochlear Implant Questionnaire to evaluate the quality of life. The population was divided into two groups: a group with unilateral cochlear implants (Cochlear Implant-alone, n = 54), and a bimodal group with a cochlear implant and a contralateral hearing aid (n = 62). Both groups were similar in terms of auditory deprivation duration, duration of cochlear implant use, and pure-tone average on the implanted ear. There was a significant difference in terms of pure-tone average on low and low-to-mid frequencies on the non-implanted ear. The scores on both questionnaires showed an improvement in the basic sound perception and quality of social activities for the bimodal group. The results suggest that the bimodal stimulation (cochlear implant and contralateral hearing aid) improved auditory perception in quiet and the quality of life domain of social activities.

[Genotype--phenotype correlation limits in sensorineural hearing loss: case report of a three-year-old child with a bilateral cochleovestibular impairment and a molecular variant of the COCH gene]. / Les limites des relations phénotypes--génotypes dans les surdités neurosensorielles: a propos d'un enfant de 3 ans associant dysfonction cochléovestibulaire bilatérale et variant moléculaire du gène COCH.

Mutations of the COCH gene inherited in an autosomal dominant mode are responsible for late-onset cochleovestibular impairment on both sides. Our objective is to report the youngest patient (3 years) associating a molecular variant of the COCH gene and a cochleovestibular impairment on both sides. The clinical sequence has started with a vestibular dysfunction in a two-year-old child: recurrent rotatory dizziness during 12 months. At the age of 3, a sensorineural hearing loss on both sides has occured associated with spontaneous variation during 6 months. The lack of mutation of the connexin 26, connexin 30 and pendrin genes has reorientated the genetic investigation. A molecular variant of the COCH gene was found in the vWFA2 domain. It was an in-frame deletion predicting the synthesis of an abnormal protein in which 21 aminoacid were missing. Others family members with mutation were asymptomatics. In this isolated case report, the study was in favor of a non pathogenic molecular variant of the COCH gene. For all that, mutations of the COCH gene could be searched in progressive cochleovestibular dysfunctions on both sides in children, even without family affect.

Ibuprofen as risk-factor for complications of acute anterior sinusitis in children.

IMPORTANCE: Non-steroidal anti-inflammatory drugs (NSAIDs) are known to inhibit chemotaxis, oxidative burst and phagocytosis, bacterial killing in granulocytes as well as inhibiting neutrophil aggregation or degranulation, thereby interfering with the function of lymphocytes. On the other hand, ibuprofen is widely prescribed in pediatrics for its powerful analgesic and antipyretic effects. To our knowledge, no previous publication outlines the relationship between Ibuprofen therapy and an increased risk of intracranial and/or orbital complications of acute fronto-ethmoidal sinusitis in childhood. OBJECTIVE: To look for a relationship between ibuprofen and occurrence of intra-cranial and/or orbital complications of acute fronto-ethmoidal sinusitis in pediatrics. SETTING AND METHODS: The medical charts of patients younger than 18 years admitted into the E.N.T. departments of 4 academic care centers during 2 consecutive years for fronto ethmoidal sinusitis were reviewed retrospectively. The history of ibuprofen intake, the occurrence of complication (orbital or intracranial) as well as the usual demographic data were noted. A statistical analysis was performed in order to ascertain whether a relationship between taking NSAIDs and the onset of an intracranial and/or orbital complication exists. RESULTS: Intake of ibuprofen appeared to be a risk-factor of intracranial complications or associated orbital and intracranial complications of acute fronto-ethmoidal sinusitis in children. Neither gender nor age nor initial pain intensity were statistically related to the onset of complications. CONCLUSION AND RELEVANCE: This retrospective multicenter cohort study appears to suggest that ibuprofen increases the risk of orbital and/or intracranial complications of acute fronto-ethmoidal sinusitis in childhood. Therefore, we recommend not prescribing ibuprofen if one suspects an acute sinusitis in a child or adolescent.

Risk factors for severity of juvenile-onset recurrent respiratory papillomatosis at first endoscopy.

OBJECTIVE: The study objective was to determine risk factors for severity of juvenile-onset recurrent respiratory papillomatosis (RRP) at first endoscopic evaluation. METHODS: Based on a review of all cases undergoing surgery for juvenile-onset RRP in two pediatric otolaryngology departments in the USA and France, the following severity risk factors were analyzed: number of laryngeal levels involved, extension to the subglottis, and bilateral involvement. RESULTS: Thirty-two patients were included, with 571 endoscopic procedures. Number of endoscopies per patient varied according to initial extension: 30.67 procedures when all three levels were involved, 15.57 procedures when two and 14.08 procedures when only one (P=0.03). The odds ratio for risk of >14 procedures in 3-level involvement was 20.43 (P=0.047). Initial subglottic extension tended to be associated with more endoscopic procedures (23.67 vs 15.56, P=0.16). CONCLUSIONS: RRP severity correlated with initial laryngeal extension of papillomatous lesions at first endoscopy. This finding allowed a short 3-item assessment scale to be created for routine use, complementary to Derkay's assessment scale.

Computational Fluid Dynamics in the assessment of nasal obstruction in children.

OBJECTIVES: Nasal obstruction is a highly subjective symptom. It can be evaluated by combining clinical examination, imaging and functional measurements such as active anterior rhinomanometry (AAR). In pediatrics, AAR is often impossible because it requires the participation of the child. Airflow modeling by Computational Fluid Dynamics (CFD) has been developed since the early 1990s, mostly in adults. This study is the first to describe a methodology of "numerical rhinomanometry" in children using CFD and to evaluate the feasibility and the clinical interest of this new tool. MATERIALS AND METHODS: Five children aged from 8 to 15 years, complaining of nasal obstruction, underwent routine management including clinical evaluation, AAR, and CT-scanning. CT acquisitions were used for CFD calculations and numerical rhinomanometry. RESULTS AND CONCLUSIONS: In the 5 children, the results of CFD were concordant with clinical complaints and examination. In 3 children, AAR and CFD were concordant. In one patient, CFD corrected the results of AAR. In one patient, AAR was not feasible, unlike CFD, which contributed to diagnosis. This study highlighted the feasibility of CFD in children and that it can support or refute diagnosis of nasal obstruction with good reliability. These results indicate that CFD modeling could be widely used for functional exploration in pediatric rhinology.

The anterior laryngeal webs.

Anterior laryngeal webs can be congenital or acquired and occur secondary to a surgical procedure, intubation, or infection. Clinical presentation can include vocal and respiratory symptoms. The first step in managing a laryngeal web is performing flexible laryngoscopy, followed by a direct laryngoscopy under general anesthesia. Treatment remains a challenge. Endoscopically, the web can be incised with cold instruments or by laser, mitomycin-C can be applied, and a silastic keel can be inserted in the anterior commissure. Externally, an anterior cricothyrotomy is necessary and a silastic keel can be placed; anterior cartilage graft can be inserted in case of a thick web. Regardless of the technique used, the primary concern is recurrence.

[Dysphonia in children]. / Les dysphonies de l'enfant.

Dysphonia is a frequent reason for consultation in children. Most of the time, this presentation is the consequence of a vocal abuse, however, the physician has to be aware that an organic lesion, such as laryngeal papillomatosis can exist and so perform a flexible laryngoscopy. In case of laryngeal papillomatosis, surgery and several medical treatments will be proposed for a long time. In other cases, speech therapy, sometimes associated with surgery, will be performed.

Health-related quality of life in children with dysphonia and validation of the French Pediatric Voice Handicap Index.

OBJECTIVE: voice disorders are common in the pediatric population and can negatively affect children's quality of life. The pediatric voice handicap Index (pVHI) is a valid instrument to assess parental perception of their children voice but it is not translated into French language. The aim of the present study was to adapt a French version of the pVHI and to evaluate its psychometric properties including construct validity, reliability, and some aspects of external validity. PATIENTS AND METHOD: we performed a cross sectional study including 32 dysphonic children and 60 children with no history of voice problems between 3 and 12 years of age. The original pVHI was translated into French language according to forward-backward rules and then administered to parents or caregivers. Construct validity and internal consistency were explored using confirmatory factor analysis and Cronbach's alpha. The questionnaire was filled twice to assess test-retest reliability using the intra-class correlation coefficient. The external validity was explored by comparing the French pVHI total and subscales scores between dysphonic and asymptomatic children. Correlations between the French pVHI and both the perceptual GRBAS scale and the health-related quality of life (HRQOL) survey "Vécu et Santé Perçu de l'Adolescent et de l'Enfant" (VSP-Ap) were also performed. RESULTS: the structure of the French pVHI showed a good fit with excellent reliability (α = 0.929) and high test-retest reliability. Significant differences were found between the group of dysphonic children and the control group (p < 0.001). The French pVHI scores were positively correlated to all parameters of the GRBAS scale (p < 0.05). Significant negative correlations were found between the Functional domain of the pVHI and various domains of the VSP-Ap as Leisure Activities, Schooling and Sentimental Relationship (p < 0.05). CONCLUSION: the French pVHI is considered to be a valid and reliable instrument to assess voice-related quality of life in children with voice disorder. We recommend its use in the multidimensional protocols for assessing voice disorder in the pediatric population.

[Cochlear implants in children with mental disabilities]. / Implant cochléaire chez l'enfant présentant des troubles envahissants du développement.

INTRODUCTION: Nowadays, profoundly deaf children with associated psychotic disorders can sometimes benefit from a cochlear implant program. These fragile children who have a specific development need a reinforced observation and follow up, and active parental collaboration. METHODS: From a series of 8 children with psychotic disorders implanted between 1999 and 2006, we present 4 representative cases of our practise, which have at least 3 years post implant. RESULTS: The questions that should occur about the conditions of the cochlear implantation are discussed in the light of the results of our observations. No children deteriorated them selves. All of them use their cochlear implant continuously. CONCLUSION: Cochlear implant is a tool in a global approach towards mobilization of communication resources in such children.

Pediatric paranasal sinus mucoceles: etiologic factors, management and outcome.

OBJECTIVES: To study the local and general etiological factors of paranasal sinus mucoceles in the pediatric population and to evaluate and discuss the clinical management and the results of endonasal marsupialization. DESIGN: Retrospective study. PATIENTS AND METHODS: A series of 10 cases of paranasal sinus mucocele in children were managed. For imaging, CT-scan and, sometimes MRI were performed. An ophthalmologic evaluation was performed preoperatively in all cases, and post-operatively in case of preoperative trouble. All patients were treated with endoscopic surgical procedure. Authors also looked for etiological factors. RESULTS: Cystic fibrosis was found in 6 cases out of 10. Others etiological factors were trauma and inflammatory process; one case was strictly idiopathic. Three patients out of 10 had ophthalmologic trouble related with the mucocele. With a mean follow-up of 17 months, neither recurrence nor complication were noted. All patients with ophthalmologic complain were free of trouble after surgery. CONCLUSION: Paranasal sinus mucoceles in children are still rare. In our experience, 9 out of 10 patients had predisposing factors, especially cystic fibrosis. Imaging with CT-scan and MRI allow the physician to rule out other tumors such as meningoceles or rhabdomyosarcoma. Endoscopic endonasal surgery is nowadays the gold standard for the treatment of paranasal sinus mucoceles.

Congenital nasal pyriform aperture stenosis: Elaboration of a management algorithm from 25 years of experience.

INTRODUCTION: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare disease presenting with neonatal respiratory distress, often associated with other anomalies. MATERIALS AND METHODS: This study reports the clinical and radiological characteristics of the patients managed in The Department of Pediatric Otolaryngology Head and Neck Surgery of La Timone Children's Hospital in Marseille between 1988 and 2014. Pyriform aperture (PA) widths were measured on CT-scans, obtained by using hand calipers at the largest portion of the PA in a plan parallel to the Francfort plan. RESULTS: 10 patients were included. Average PA width was 6.6mm, 5/10 patients presented with single central maxillary median incisor, 6/10 patients had associated abnormalities. 8 patients underwent a surgical intervention and 2 patients were medically managed. All the patients had satisfactory nasal airway permeability on late follow-up. A management algorithm was elaborated. CNPAS should be evoked when breathing difficulties are associated with impossibility of passing fiberscope or nasogastric tube at the nasal inlet. Craniofacial CT-scanning is necessary to make the diagnosis and look for associated abnormalities. Medical treatment associating nasal wash and decongestants should be performed. Surgical intervention is necessary when failure of the medical management. DISCUSSION AND CONCLUSIONS: Our results were close to those found in the literature in terms of clinical characteristics, associated abnormalities and PA width. However, no objective criterion to decide whether a surgical intervention is necessary or not, has been established so far. The algorithm we propose offers guidelines from diagnosis to treatment, but the management should be adapted based on clinical tolerance.

Butterfly-cartilage tympanoplasty in children: A 28-case series and literature review.

OBJECTIVE: This study presents results for a pediatric series of tympanoplasty using the butterfly-cartilage technique, with comparison to literature reports. METHODS: A retrospective study included patients aged between 2 and 12 years receiving tympanoplasty performed by a single senior surgeon in our department between 2003 and 2012, for whom pre- and postoperative tympanum imaging was complete. Results were analyzed at a minimum 10 months' follow-up in terms of graft healing and auditory data, with pre- and postoperative audiograms and calculation of mean hearing loss. RESULTS: Twenty-eight tympanoplasties were performed in 27 children: 16 males, 11 females; in 1 case, perforation was bilateral. Perforation location was inferior or antero-inferior in 14 cases (50%), antero-superior in 9 (32.1%) and anterior in 5 (17.9%). Perforation size on otoscopy ranged from 11.1% to 36.4% of tympanum area (mean, 26.3%). Mean follow-up was 25 months. Closure of the perforation was achieved in 87.7% of cases. Mean hearing loss was 20.2dB (range, 10-31.25dB) preoperatively and 14dB (range, 5-31.25dB) postoperatively. DISCUSSION: We analyze our results and compare them with the international literature to refine indications for this technique of tympanic repair. CONCLUSION: Tympanoplasty using the butterfly-cartilage technique was reliable and easy to perform. It should be included in the therapeutic armamentarium for moderate non-marginal tympanic perforation. In the present series, the technique was reserved to perforations involving less than 40% of tympanum area.

Self- and parental assessment of quality of life in child cochlear implant bearers.

OBJECTIVES: The aim of this study was to assess quality of life in children fitted with cochlear implants, using combined self- and parental assessment. MATERIALS AND METHODS: Thirty-two children, aged 6 to 17 years, with prelingual hearing loss and receiving cochlear implants at a mean age of 22 months, were included along with their families. The KIDSCREEN-27 questionnaire was implemented, in face-to-face interview, in its parents and children-adolescents versions, with 27 items covering physical well-being ("physical activities and health"), psychological well-being ("general mood and feelings about yourself"), autonomy & parents ("family and free time"), peers & social support ("friends") and school environment ("school and learning"). Parent and child responses were compared with a general population database, and pairwise. RESULTS: Global scores were compared against the general population on Cohen d effect-size. For child self-assessment, the results were: physical well-being, 72.81 (d=0); psychological well-being, 78.13 (d=-0.4); autonomy & parents, 63.84 (d=-0.2); peers & social support, 61.72 (d=-0.4); and school environment 73.83 (d=0). For parent assessment, the respective results were 62.66 (d=-0.8), 74.89 (d=-0.3), 57.37 (d=-1.2), 51.56 (d=-0.8), and 68.95 (d=-0.4). Half of the children could not answer the questionnaire, mainly due to associated disability. Schooling and language performance were poorer in non-respondent than respondent children. Quality of life was comparable between implanted and non-implanted children: Cohen d, 0 to 0.4. Early cochlear implantation in children with pre-lingual hearting loss provides quality of life comparable to that of the general population.

Unusual association of congenital middle ear cholesteatoma and first branchial cleft anomaly: management and embryological concepts.

OBJECTIVE: To report two cases of an undescribed association of first branchial cleft fistula and middle ear congenital cholesteatoma and to discuss management and embryological hypothesis. DESIGN: Retrospective study and review of the literature RESULTS: Both patients were young girls free of past medical or surgical history. Surgical removal of the first cleft anomaly found in the two cases a fistula routing underneath the facial nerve. Both cholesteatomas were located in the hypotympanum, mesotympanum. In one case, an anatomical link between the two malformations was clearly identified with CT scan. DISCUSSION: The main embryological theories and classification are reviewed. A connection between Aimi's and Michaels' theories (congenital cholesteatoma) and Work classification might explain the reported clinical association.

Nasal and sinus polyposis in children.

Nasal and sinus polyposis in the pediatric population is uncommon and its etiology is unclear. In this 11-year retrospective study, the authors describe the etiologic features and evaluate the effectiveness of endoscopic sinus surgery in 46 children. Patients were divided into three groups according to whether nasal and sinus polyposis was isolated (n = 14), or associated with either asthma (n = 5) or cystic fibrosis (n = 27). An allergy was present in 10% of patients with isolated polyposis, 80% of patients with polyposis associated with asthma, and 22% of patients with polyposis associated with cystic fibrosis. The indications for surgery were disabling symptoms, especially chronic nasal obstruction, rhinorrhea, and mouth breathing, and failure to respond to medical treatment. No surgical complications were encountered. Most patients reported improvement in quality of life with reduction of nasal obstruction in 83% of cases and rhinorrhea in 61%. Minor asymptomatic recurrence (i.e., a few micropolyps localized on the roof of the ethmoid cavity) was observed in 24% of the cases in this series, and major recurrence with the same functional symptoms as before surgery in 12%. However, recurrences were higher in patients with cystic fibrosis, because minor recurrence with no clinical manifestation was observed in 32% of these cases and major recurrence in 16%. Endoscopic sinus surgery must be decided in collaboration with the pediatric and pulmonary physicians, and must be performed skillfully. With a mean follow-up of 3.7 years, results in this series are encouraging.

Airway growth after cricotracheal resection in a rabbit model and clinical application to the treatment of subglottic stenosis in children.

OBJECTIVE: To evaluate the effects of a partial resection of the anterior cricoid arch on the growth of the laryngotracheal complex in an animal model and to study the lumen caliber and cross sectional surface area following resection as the animals are allowed to grow. Laryngeal, subglottic, and tracheal measurements in the infant and pediatric larynges in autopsy specimens are also investigated. DESIGN: A controlled animal study was done comparing the operated rabbit group to the nonoperated group. Twenty-nine infant rabbits were compared to a control group of ten rabbits. In addition, autopsy dissections were performed on infant and pediatric larynges to obtain measurements of various laryngeal and cricoid relationships. METHODS: Thirty-nine pasteurella-free New Zealand white female rabbits between the ages of 8 and 11 weeks were used in this study. In the experimental group, cricotracheal resections were performed by removing the anterior cricoid cartilage and the first tracheal ring. A primary anastomosis was then performed. The animals were kept alive for 4 months and then humanely euthanized. The laryngeal complex was then removed and histological sections of the cricotracheal region were mounted on glass slides and stained with H&E. Measurements were taken of the cricoid and upper tracheal lumen and cross-sectional area. RESULTS: Twenty-seven of the study group survived until the time of sacrifice. All of the control group survived during the study period. The two groups were comparable in regard to weight, lumen size, and cross-sectional area There were no statistical differences between the groups. CONCLUSIONS: The experimental data support the hypothesis that partial cricotracheal resections can be performed safely and effectively in young rabbits. The potential clinical significance is described in light of the autopsy data.

Cochlear implantation in a pacemaker patient.

The authors present their experience with cochlear implantation in a 22-year-old patient who had a cardiac pacemaker because of complete atrioventricular block. The question of compatibility between the cochlear implant (multichannel Digisonic DX10AE) and the pacemaker (Biotronik Nanos-01AE) was raised. After consulting the two manufacturers regarding the risk of electrical interference, the authors decided to program the pacemaker in a locked mode (VOO) and then perform cochlear implantation. When the cochlear implant was switched on for the first time, electrocardiograms were obtained to ensure that the two devices were compatible. Absolutely no interference was detected. After a 3-year follow-up, cochlear implant performance remains excellent.

Objective aerodynamic and acoustic measurement of voice improvement after phonosurgery.

HYPOTHESIS: Objective analysis of the outcome of phonosurgery is needed to allow comparison of different surgical techniques or indications, or both. We tried to demonstrate that measurement of mean oral airflow during the production of a sustained vowel could be used in combination with acoustic measurements such as jitter or shimmer for assessment of voice improvement after phonosurgery. STUDY DESIGN: This prospective study included 27 consecutive patients who underwent phonosurgery during a 3-month period. METHOD: Perceptual analysis served as the "gold standard." Objective measurements (oral airflow, jitter, shimmer) on the most stable portion of a sustained vowel /a/ were made using the EVA system (SQ-Lab, Aix-en-Provence, France). RESULTS: Oral airflow was significantly greater in patients with poor perceptual results than in patients with good perceptual results. Jitter and shimmer were not significantly different because of the dispersion of the values. Discriminant factorial analysis showed that a combination of jitter, shimmer, and oral airflow was able to identify patients with good or poor perceptual results in 77% of cases. CONCLUSIONS: Oral airflow allows simple, quick, and reliable assessment of the outcome of phonosurgery and can be used in everyday clinical practice.

Severe laryngomalacia: surgical indications and results in 115 patients.

Between 1987 and 1993, 115 children were operated on for severe forms of laryngomalacia in two pediatric ear, nose, and throat (ENT) departments. The criteria used to determine the severity of the illness were selected following short hospitalization periods during which the children received both pediatric and ENT checkups. Based on clinical manifestations and/or the results of pH monitoring gastroesophageal reflux was found to be present in 68% of the children in the study. Detailed analysis and endoscopy were used to differentiate the symptoms that were related to laryngomalacia from those that were caused by other conditions, including mixed-breathing, swallowing, and sucking difficulties. Endoscopic resection of the aryepiglottic folds, with or without the use of a carbon dioxide laser, resulted in rapid improvement of both ventilation and swallowing. The success rate of this simple and effective procedure, which has no inherent morbidity, was 98% in an average follow-up period of 30 months. Only seven children required an additional similar procedure. The procedure failed in only two children, who needed to be tracheotomized. Given these excellent results, endoscopic resection can be considered an effective technique for the management of severe laryngomalacia.

Surgical removal of subglottic hemangiomas in children.

OBJECTIVE: To examine the indications and the results of surgical excision of severe subglottic hemangiomas. DESIGN: Retrospective study and case series. SETTING: Four academic tertiary care centers of pediatric otolaryngology. PATIENTS: Twenty children were included from 1991 to 1997. All presented with severe subglottic hemangiomas resistant to classical treatments such as corticosteroids and/or CO2 laser. INTERVENTION: Six children were operated on using laryngotracheal reconstruction and prolonged stenting by a reinforced Silastic roll and 14 children were operated on by a single-stage laryngotracheoplasty and postoperative intubation in a pediatric intensive care unit. RESULTS: All patients were successfully decannulated or extubated and have been free from recurrent hemangiomas. CONCLUSION: The surgery of severe subglottic hemangiomas is a reliable technique in selected patients and should be considered in corticoresistant or corticodependent, circular, or bilateral hemangiomas.