International Pediatric ORL Group (IPOG) laryngomalacia consensus recommendations

Objective To provide recommendations for the comprehensive management of young infants who present with signs or symptoms concerning for laryngomalacia. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). Results Consensus recommendations include initial care and triage recommendations for health care providers who commonly evaluate young infants with noisy breathing. The consensus statement also provides comprehensive care recommendations for otolaryngologists who manage young infants with laryngomalacia including: evaluation and treatment considerations for commonly debated issues in laryngomalacia, initial work-up of infants presenting with inspiratory stridor, treatment recommendations based on disease severity, management of the infant with feeding difficulties, post-surgical treatment management recommendations, and suggestions for acid suppression therapy. Conclusion Laryngomalacia care consensus recommendations are aimed at improving patient-centered care in infants with laryngomalacia

Total ossiculoplasty in children: predictive factors and long-term follow-up.

International audienceOBJECTIVE: To evaluate the long-term results and predictive factors of a good outcome with the use of a total ossicular replacement prosthesis in children. DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: The study included 114 children (116 ears). INTERVENTIONS: A total of 116 ears underwent total ossicular chain reconstruction with a titanium prosthesis. Cartilage was always used for tympanic membrane reconstruction. MAIN OUTCOME MEASURES: Audiological results were evaluated according to the guidelines of the American Academy of Otolaryngology-Head and Neck Surgery. Predictive factors of audiological results were determined. Logistic regression and χ(2) tests were used for statistical analysis. RESULTS: The mean age at surgery was 9.8 years. Ossiculoplasty was performed during second-look surgery in 91 ears (78.4%) and during another stage in 25 ears (21.6%). The first-stage procedure was always performed for cholesteatoma. Audiometric results were available for 116 ears at 1 year, for 89 ears (76.7%) at 2 years, and for 42 ears (36.2%) at 5 years. Closure of the average air-bone gap (ABG) to within 20 dB was achieved in 65 ears (56%) at 1 year. The mean (SD) preoperative and postoperative (at 1 year) ABGs were 41.0 (9.5) dB and 22.4 (12.6) dB, respectively. There were no cases of extrusion, but 17 luxations of the prosthesis were confirmed by computed tomography. Luxation occurred on average at 31.4 months. Only three 4000-Hz degradations of bone conduction were reported, with no dead ears. We examined 3 predictive factors of auditory results: preoperative ABG, footplate status, and postoperative otoscopic findings. CONCLUSIONS: Total ossiculoplasty is a reliable technique in children. Long-term hearing outcomes are stable and satisfactory, but luxation can occur at any time. Preoperative ABG and footplate status are negative predictive factors of auditory results

Educational content and challenges encountered when training service user representatives as peer researchers in a mixed study on patient experience of hospital safety

International audienceAbstract Background and objectives In France, following the passing of a 2002 law, service user representatives (SURs) are part of hospital committees in charge of care quality and safety issues. Ten service user representatives (SURs) were recruited and trained as “peer researchers” to participate in all phases of a study aimed at outlining how patients experience hospital safety. This article aims to describe the study protocol and how peer researchers training was designed and implemented to prepare them to drive a qualitative and quantitative research. It also examines the challenges related to collaborative research and how these were resolved. Methods The way our training was conceived belongs to the field of “design-based research”, known for its pragmatic and collaborative scope, in which viewpoints of all participants are included. Our training was therefore based on peer researchers and research sponsors expectations, as well as on recommendations of the literature. Results A 45-h training was held. While the program was meant to train peer researchers to respect scientific norms, it also aimed to improve their sense of self-legitimacy as they navigated their new role. Peer researchers were particularly eager to understand meaning behind the instructions, especially in the field of ethical and scientific norms. Various challenges occurred related to project organization, recruitment and peer researchers involvement. Some issues were overcome by learning how to share control over the research process. Conclusion This experiment highlights the importance of a training program’s duration and quality to prepare SURs for their roles as peer investigators and to create a group dynamic around a research project, even with SURs familiar with patient involvement and our research theme (safety issues). Trainers overcame hurdles by being adaptive and by using educational approaches. They also learned to include trainees’ input, even when it forced them to reconsider their own assumptions

Otologic features in children with primary ciliary dyskinesia.: Otologic features in children with PCD

International audienceDespite continuous antibiotic therapy, the middle ear condition in PCD remained severe throughout childhood, with improvement only after age 18 years. Armstrong grommet placement did not improve the middle ear condition. Central complex defect is a marker of severity

Cholesteatoma secondary to temporal bone involvement by Langerhans cell histiocytosis: a complication amenable to curative surgery.

International audienceOBJECTIVES: To describe secondary acquired cholesteatoma in patients previously treated for Langerhans cell histiocytosis (LCH). To focus on misleading symptoms suggesting LCH relapse. PATIENTS: This study involved 3 patients aged 12, 20, and 58 months whose conditions were diagnosed with LCH. All 3 had involvement of the mastoid and of the skin of the external ear canal (EAC) at diagnosis. They were treated with steroids and vinblastine. INTERVENTIONS: Serial computed tomographic (CT) scans and clinical follow-up. MAIN OUTCOME MEASURE: Exploratory surgery of the mastoid. RESULTS: Otorrhea recurred in all 3 patients at 24, 17, and 26 months, respectively, with difficulties to clinically identify a hole in the posterior part of the EAC. The otorrhea was considered a sign of a new occurrence of LCH, leading to systemic chemotherapy in 2 cases. A CT scan showed a defect in the posterior wall of the EAC and suggested cholesteatomatous invasion of the mastoid (2 true cholesteatomas and 1 precholesteatomatous case).Surgery (canal wall up mastoidectomies) successfully removed the cholesteatoma (bilateral in 2 cases) and reconstructed the bony defect using cartilage. Biopsies ruled out LCH recurrence. CONCLUSION: Secondary acquired cholesteatoma (through a bony defect of the EAC) may occur in patients previously treated for LCH. Recurrence of symptoms and bone destruction on CT may wrongly suggest LCH recurrence. Surgery allows removal of the cholesteatoma as well as EAC reconstruction and control biopsy

Congenital stapes ankylosis: study of 28 cases and surgical results.

OBJECTIVE: The objective of this study was to analyze functional results after stapes surgery in patients with congenital nonprogressive conductive deafness resulting from an isolated fixation of the stapes according to age and surgical procedure. STUDY DESIGN: The authors conducted a retrospective case series from March 1993 to December 2003 in patients from two tertiary referral centers. METHODS: Twenty-eight patients were operated on by stapedotomy or partial stapedectomy using Teflon stapes prostheses. The median age at surgery was 14.2 years (range, 8.3-29.1 years). Main outcome measures were clinical and audiometric evaluation before and after surgery. Mean air conduction (MAC) and bone conduction (MBC) thresholds were recorded at 0.5, 1, 2, and 4 kHz. The evaluation of functional outcome was based on the MAC gain, the MBC comparison, and the mean postoperative and residual air-bone gaps. RESULTS: The median preoperative MAC was 50 dB (range, 19.0-65.0 dB) with a 35.0 dB median dB air-bone gap. With a mean follow up of 19 months, postoperative hearing improvement was statistically significant: median gain of 32.5 dB (P<.001) and median residual air-bone gap of 3.5 dB. The MBC was also statistically improved with median pre- and postoperative MBC of 11.5 and 6.5 dB, respectively (P<.001). Results were not dependent on the age group or type of surgery (stapedotomy or partial stapedectomy). No perceptive hearing loss was observed despite one gusher case. CONCLUSION: Surgical treatment of isolated congenital stapes ankylosis allows good functional results regardless of age or type of surgery

Temperature-sensitive auditory neuropathy associated with an otoferlin mutation: Deafening fever!

International audienceTransient deafness associated with an increase in core body temperature is a rare and puzzling disorder. Temperature-dependent deafness has been previously observed in patients suffering from auditory neuropathy. Auditory neuropathy is a clinical entity of sensorineural deafness characterized by absent auditory brainstem response and normal otoacoustic emissions. Mutations in OTOF, which encodes otoferlin, have been previously reported to cause DFNB9, a non-syndromic form of deafness characterized by severe to profound prelingual hearing impairment and auditory neuropathy.Here we report a novel mutation in OTOF gene in a large family affected by temperature-dependent auditory neuropathy. Three siblings aged 10, 9 and 7 years from a consanguineous family were found to be affected by severe or profound hearing impairment that was only present when they were febrile. The non-febrile patients had only mild if any hearing impairment. Electrophysiological tests revealed auditory neuropathy. Mapping with microsatellite markers revealed a compatible linkage in the DFNB9/OTOF region in the family, prompting us to run a molecular analysis of the 48 exons and of the OTOF intron–exon boundaries. This study revealed a novel mutation p.Glu1804del in exon 44 of OTOF. The mutation was found to be homozygous in the three patients and segregated with the hearing impairment within the family. The deletion affects an amino acid that is conserved in mammalian otoferlin sequences and located in the calcium-binding domain C2F of the protein