International Journal of Pediatric Otorhinolaryngology

Comparison of motor-phonetic versus phonetic-phonological speech therapy approaches in patients with a cleft (lip and) palate: a study in Uganda

10-01-2020 – Cassandra Alighieri, Kim Bettens, Laura Bruneel, Daniel Sseremba, Duncan Musasizi, Isaac Ojok, Kristiane Van Lierde

Journal Article

Introduction

At present, there is growing interest in combined phonetic-phonological approaches to treat active speech errors in children with a cleft (lip and) palate (CP ± L). Unfortunately, evidence for these type of speech interventions in this population is lacking. Therefore, the present study investigated the effectiveness of speech intervention in Ugandan patients with CP ± L. Moreover, a comparison was made between a motor-phonetic and a phonetic-phonological speech intervention.

Methods

Eight patients (median age: 11.26y) with an isolated CP ± L were assigned into a group receiving motor-phonetic treatment (n = 4) or a group receiving combined phonetic-phonological treatment (n = 4). The participants received 6h of individual speech therapy. In both groups, perceptual and instrumental speech evaluations were performed to evaluate the patients speech before and after the intervention.

Results

Speech therapy (irrespective of the used approach) was found to be effective in increasing consonant proficiency and in decreasing the occurrence of non-oral and passive CSCs. No statistically significant differences in outcome variables were found when comparing the two groups pre- and post-treatment. The descriptive results, however, revealed a larger increase in % correctly produced consonants, places and manners after the intervention in the group receiving a combined phonetic-phonological treatment compared to the group receiving a motor-phonetic treatment.

Conclusion

This study took a first step in providing evidence concerning the effectiveness of different speech therapy approaches in children with CP ± L. The present study holds some important implications for clinical practice suggesting that an additional phonological approach may be beneficial for the patients with CP ± L. Further research including randomized controlled trials with larger sample sizes is necessary to provide further evidence.

Case report of two children with auditory neuropathy spectrum disorder related to a neurofascin (NFASC) gene variant

17-01-2020 – Jonathan L. Harper, Theodore E. Wilson, Ryan M. Mitchell

Journal Article

We present a case of two siblings born to nonconsanguineous parents that presented with hypotonia, respiratory insufficiency, and auditory neuropathy spectrum disorder (ANSD) correlated with NFASC (MIM: 609145) and the homozygous loss of function variant p.
P924Rfs
X35. This appears to be the first two reported cases of NFASC correlated with ANSD. NFASC encodes for neurofascin which plays an important role in the formation, function and maintenance of axon initial segments and nodes of Ranvier. Due to the rarity of this gene variation, reports are sparse in the literature leading to delays in diagnosis which can impact patients language acquisition and spoken language skills.

Music and psychoacoustic perception abilities in cochlear implant users with auditory neuropathy spectrum disorder

17-01-2020 – Mustafa Yüksel, Ayça Çiprut

Journal Article

Objective

Auditory neuropathy spectrum disorder (ANSD) is a condition wherein the pre-neural or cochlear outer hair cell activity is intact, but the neural activity in the auditory nerve is disrupted. Cochlear implant (CI) can be beneficial for subjects with ANSD; however, little is known about the music perception and psychoacoustic abilities of CI users with ANSD. Music perception in CI users is a multidimensional and complex ability requiring the contribution of both auditory and nonauditory abilities. Even though auditory abilities lay the foundation, the contribution of patient-related variables such as ANSD may affect the music perception. This study aimed to evaluate the psychoacoustic and music perception abilities of CI recipients with ANSD.

Study design

Twelve CI users with ANSD and twelve age- and gendermatched CI users with sensorineural hearing loss (SNHL) were evaluated. Music perception abilities were measured using the Turkish version of the Clinical Assessment of Music Perception (T-CAMP) test. Psychoacoustic abilities were measured using the spectral ripple discrimination (SRD) and temporal modulation transfer function (TMTF) tests. In addition, the age of diagnosis and implantation was recorded.

Results

Pitch direction discrimination (PDD), timbre recognition, SRD, and TMTF performance of CI users with ANSD were concordant with those reported in previous studies, and differences between ANSD and SNHL groups were not statistically significant. However, the ANSD group performed poorly compared with SNHL group in melody recognition subtest of T-CAMP, and the difference was statistically significant.

Conclusion

CI can prove beneficial for patients with ANSD with respect to their music and psychoacoustic abilities, similar to patients with SNHL, except for melody recognition. Recognition of melodies requires both auditory and non-auditory abilities, and ANSD may have an extensive but subtle effect in the life of CI users.

Current management of congenital anterior cranial base encephaloceles

14-01-2020 – Harrison M. Thompson, Rodney J. Schlosser, Erika McCarty Walsh, Do-Yeon Cho, Jessica W. Grayson, Thomas T. Karnezis, Peter L. Miller, Bradford A. Woodworth

Journal Article

Objectives

Congenital encephaloceles provide unique diagnostic and reconstructive challenges for the pediatric rhinologist. The objectives of the current study were to evaluate contemporary treatment strategies for congenital encephaloceles focusing on presentation, surgical technique, and outcomes.

Methods

Multi-institutional retrospective chart review of congenital encephaloceles (2003–2019). Data regarding demographics, presenting symptoms, associated abnormalities, surgical technique, size, location, and complications were collected.

Results

Fourteen patients with 15 congenital encephaloceles were treated using endoscopic techniques (avg 6.0 years, range 2 months–22 years) with mean follow up of 23 months. The majority presented with nasal obstruction (n = 13); only one child had cerebrospinal fluid (CSF) rhinorrhea. Associated anomalies included nasal deformities, congenital hypopituitarism, and Morning Glory syndrome. Average encephalocele size was 2.44 cm (range 0.5–3.6 cm) with mean skull base defect size of 8.6 x 7.7 mm. Locations included the foramen cecum (n = 9), central sphenoid (n = 3), midline anterior cranial fossa (n = 1), orbital plate of frontal bone (n = 1), and ethmoid roof (n = 1). Because of favorable expansion from encephaloceles, it was unnecessary to postpone surgeries to allow nasal cavity growth. Three individuals had prior operations, including surgeries for “nasal polyp” or “adenoid cyst”. Two patients had post-operative complications (meningitis and CSF leak) effectively treated with no further sequelae.

Conclusions

In the current study, congenital encephaloceles in children as young as 2 months were successfully repaired using endoscopic techniques. Endoscopic approaches remain a safe and effective intervention for management of these lesions.

Recurrent laryngeal nerve monitoring during thyroidectomy and parathyroidectomy in children

15-01-2020 – M. Legré, E. Bois, S. Bernard, N. Teissier, T. Van Den Abbeele

Journal Article

Objective

Injury of the recurrent laryngeal nerve (RLN) is the most frequent complication of thyroid and parathyroid (TP) surgery. Monitoring of the RLN in children is not widely studied as this is not a common disease in the pediatric population. The aim of our study was to evaluate the reliability, feasibility and benefits of RLN monitoring during TP surgery in children.

Methods

We analyzed all children who underwent TP surgery in our department between January 2009 and March 2018. Patients were classified into three groups: without monitoring (group 1), monitoring with an endotracheal tube (group 2) and monitoring with a double-needle electrode placed through the cricothyroid ligament (group 3).

Results

We performed 53 surgeries (77 RLNs at risk) on 47 patients aged between 6 months and 18 years. Fourteen RLNs were in group 1, 47 in group 2 and 16 in group 3. We found one transient lesion of the RLN in group 1, three in group 2 and two in group 3. Seven patients in group 3 could not undergo monitoring with the tube because they need an orotracheal tube smaller than the smallest monitoring tube size available. The sensitivity and specificity values of the monitoring methods were 33% and 97% in group 2 and 67% and 100% in group 3, respectively. The positive and negative predictive values were 50% and 95% in group 2 and 100% and 92% in group 3, respectively. No complications arose during insertion of the electrode through the cricothyroid ligament.

Conclusion

Monitoring of the RLN with a double-needle electrode inserted through the cricothyroid ligament is a reliable method with no associated complication, which is achievable at any age.

Associated syndromes in patients with Pierre Robin Sequence

14-01-2020 – Peter Karempelis, Mitchell Hagen, Noelle Morrell, Brianne Barnett Roby

Journal Article

Objectives

Classically, Pierre Robin Sequence (PRS) is a triad of micrognathia, glossoptosis, and airway obstruction, although frequently associated with cleft palate. Current literature reports that Stickler syndrome is the most common syndrome associated with PRS, and 22q11 deletion syndrome (22q11 DS) as the second most common. This study identifies associations between PRS and genetic syndromes.

Methods

A retrospective chart review was performed to identify patients diagnosed with PRS over a 10-year period from 4/1/2007 to 4/1/2017 at a tertiary childrens hospital.

Results

4,052 consecutive charts were reviewed and 234 patients had a diagnosis of PRS confirmed with the triad of micrognathia, glossoptosis, and airway obstruction. Of note, all of these patients had cleft palate. Of the 234 patients with PRS, 65 patients had syndromic diagnoses (28%). One patient had 22q11 DS (0.43%), and 31 patients had Stickler syndrome (13.2%). Additionally, 3 patients had central hypoventilation syndrome, 3 patients had Duane syndrome, 2 patients had Cornelia de Lange syndrome, 2 patients had Emanuel syndrome, 2 patients had Gordon syndrome, 2 patients had Mobius syndrome, 2 patients had Nager syndrome. Multiple other syndromes were identified, but occurred in isolated cases.

Conclusion

This study supports literature that PRS is most commonly associated with Stickler Syndrome but rarely associated with 22q11 DS given that only 1 patient had both PRS and 22q11 DS.

Torticollis in children with enlarged vestibular aqueducts

14-01-2020 – Jacob R. Brodsky, Karampreet Kaur, Talia Shoshany, Juliana Manganella, Devon Barrett, Kosuke Kawai, Makenzie Murray, Greg Licameli, Victoria Albano, Amanda Stolzer, Margaret Kenna

Journal Article

Objectives

To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA).

Methods

An online/phone survey was administered to parents of 133 children diagnosed with the following disorders: EVA, GJB2 (Connexin 26) mutations associated congenital hearing loss and epistaxis (control). The survey included questions regarding symptoms of torticollis, vertigo, and hearing loss.

Results

Patients with EVA had a 10-fold greater odds of having torticollis than controls (31% vs. 4%; OR = 10.6; 95% CI: 2.9, 39.2). No patients with GJB2 had a reported history of torticollis. Torticollis preceded the diagnosis of hearing loss in most (87%) patients with EVA who had a reported history of torticollis. EVA patients were more likely to have reported motor delay than controls (40% vs. 15%; p = 0.002). EVA patients with prior torticollis (80%; 12/15) were more likely to have balance impairment than EVA patients without prior torticollis (12%; 4/33; p < 0.001). Twelve patients had a reported history of paroxysmal torticollis, all of whom had EVA.

Conclusion

Torticollis in infants may be a marker of EVA. Infants with torticollis should be monitored closely for hearing loss and motor delay, especially when the torticollis is paroxysmal.

Comparison of ABR and ASSR using NB-chirp-stimuli in children with severe and profound hearing loss

14-01-2020 – Katharina Eder, Maria Elke Schuster, Daniel Polterauer, Maike Neuling, Eva Hoster, John-Martin Hempel, Sebastian Semmelbauer

Journal Article

Introduction

Objective techniques for hearing threshold estimation in infants and children with profound or severe hearing loss play a key role in pediatric audiology to prevent speech acquisition disorders by choosing the adequate therapy. Auditory brainstem responses and auditory steady-state responses are available for frequency-dependent hearing threshold estimations and both techniques show strong correlations. However, various systems and stimuli are available, which is one reason why comparison is challenging, and, so far, no single “gold standard” could be established for hearing threshold estimation in children suffering from profound or severe hearing loss. The aim of the study was to compare hearing threshold estimations in children with profound or severe hearing loss derived with narrow-band CE-chirps evoked auditory brainstem responses and auditory steady-state response.

Subjects

and Methods: 71 children (121 ears) with an age from 3 month to 15 years were measured with the Interacoustics Eclipse EP25 ABR system® (Denmark) with narrow-band CE-chirps® at 500, 1000, 2000 and 4000 Hz under identical conditions.

Results

Auditory brainstem responses and auditory steady-state responses highly correlate (r = 0.694, p < 0.001). Correlation coefficients differ depending on the center frequency and patient age. Generally, auditory steady-state responses show a better hearing threshold than auditory brainstem responses or a remaining hearing threshold when auditory brainstem responses could not be obtained. In approximately 15% of cases this would have affected the therapeutic strategy when only taking one technique into account.

Conclusion

Auditory brainstem responses and auditory steady-state responses should be jointly used in the diagnostic approach in children with suspected profound or severe hearing loss.

Test-retest reliability of the Greek Speech-in-babble test (SinB) as a potential screening tool for auditory processing disorder

14-01-2020 – Valentinos Sofokleous, Maria Marmara, Georgios K. Panagiotopoulos, Stellina Mouza, Maria Tsofidou, Afroditi Sereti, Ioanna Grigoriadi, Εleftherios Petridis, Christos Sidiras, Michael Tsiourdas, Vasiliki (Vivian) Iliadou

Journal Article

Introduction

There seems to exist a specific group of people considered to be at higher risk of having Auditory Processing Disorders (APD). These patients are frequently initially referred to, or managed by various professionals such as Otolaryngologists, Speech Therapists, and Occupational Therapists. It is, therefore, essential to retain a low threshold of when to refer such individuals for a formal APD diagnostic evaluation. Under these circumstances, there might be a role for the Greek Speech-in-Babble (Sin
B) recognition test as a screening tool for abnormal auditory processing competency.

Objective

To explore the test-retest reliability of a diagnostically validated speech-in-babble test, the Greek Sin
B, as a potential screening tool.

Methods

Ten health professionals coming from various disciplines administered the Sin
B test twice, under conditions similar to those encountered when using it as a screening tool, and test-retest reliability was assessed. 93 Greek-speaking individuals, of whom 27 adults and 66 children or young adolescents aged five years old or more, served as our study sample.

Results

For the right ear, the Intraclass Correlation Coefficient (ICC) was 0.858 with a 95% confidence interval (CI) = 0.786–0.906. Slightly better conditions apply for the left ear, as the ICC was 0.873 with 95% CI = 0.809–0.916. These 95% CIs indicate a ‘good’ to ‘excellent’ level of reliability for both ears. Spearmans rho was 0.86 and 0.71 for the right and left ear, respectively.

Conclusion

Our results suggest that the test possesses the required reliability to evaluate a subjects hearing abilities under screening conditions. On these terms, it could be used to screen populations considered as being at risk for Auditory Processing Disorders. Forthcoming research should focus on establishing its efficiency by comparing the results of the screening test with that of diagnostic tests and on fine-tuning Sin
B as a screening tool.

Low cost, easy-to-replicate myringotomy tube insertion simulation model

10-01-2020 – Nicole Molin, Jerlon Chiu, Benjamin Liba, Glenn Isaacson

Journal Article

Introduction

Simulation is an established part of modern surgical education. Several training centers have proposed different simulation models for myringotomy tube (MT) placement and validated their effectiveness in medical student and resident training. None is widely used. Early models were simple tubes that lacked important microsurgical elements. Newer simulators are more comprehensive, but are difficult and expensive to build. We present a MT placement simulator that is low cost, easy to construct with basic power tools and allows for acquisition of the most necessary MT placement skills.

Methods

The model incudes a rotating spherical “head”, a 4 mm oval speculum, a drilled-out working shaft similar in size to the external auditory canal, and a realistic paper tympanic membrane target, set at an anatomically correct angle. To evaluate the models efficacy, we assessed the performance of 10 surgically naïve medical student volunteers before training and after 30 min of instruction with the model. Their speed was recorded and operative performance was assessed using a validated Global Rating Scale.

Results

After 30 min of practice on the model, there was significant improvement in MT placement skill scores and significant decrease in time for tube placement (p < 0.05).

Conclusion

This MT placement simulation model is inexpensive and easy to build. Unlike existing planar models, it simulates patient head orientation, and requires realistic hand positioning on a 4 mm speculum. Practice with the model for 30 min resulted in statistically significant improvement in MT placement skill scores for inexperienced student surgeons.

Subannular ventilation tubes in the pediatric population: Clinical outcomes of over 1000 insertions

10-01-2020 – Nathan Yang, Pier-Luc Beaudoin, Michael Nguyen, Hélène Maillé, Anastasios Maniakas, Issam Saliba

Journal Article

Objectives

Long-term transtympanic tube insertions for chronic middle ear disease are associated with high rates of complications. The objective of this study was to examine the clinical outcomes achieved with an alternate technique, the subannular tube insertion, by determining mean tube lifespan, cumulative incidence of post-operative events and complications, audiometric changes and risk factors associated with earlier tube extrusion in the pediatric population.

Methods

A retrospective chart review of all patients operated for subannular tube insertion between January 2007 and 2013 was conducted in a single pediatric tertiary care center. Exploratory Cox regression analysis was performed to identify potential risk factors.

Results

A total of 1014 tubes from 459 patients were included in the study. Mean subannular tube lifespan was 41.3 months with median time of 35.0 months. Cumulative incidence of post-operative events in decreasing frequency were otorrhea (21.7%), tube blockage (16.0%), tympanic membrane retraction (12.5%), otitis media with effusion (10.0%), acute otitis media (6.4%), perforation (4.6%) and cholesteatoma formation (1.1%). For patients with available pre- and post-operative audiograms, mean air-bone gap improved from 19.5 d
B to 7.0 d
B after subannular tube insertion (p < 0.01). Increasing age and previous subannular tube insertion carried hazard ratios of 1.029 (p < 0.01) and 1.749 (p < 0.01) for tube extrusion respectively, while craniofacial anomalies and concomitant tympanoplasty at the time of tube insertion had hazard ratios of 0.795 (p < 0.01) and 0.680 (p = 0.03).

Conclusions

Subannular tube insertion appears to be a safe and effective alternate technique for middle ear ventilation in cases of intractable disease.

Endoscopic type 1 cartilage tympanoplasty in children

10-01-2020 – Engin Dursun, Emine Demir, Suat Terzi, Özlem Çelebi Erdivanlı, Zerrin Özergin Coşkun, Gökçe Aydın Balaban, Metin Çeliker

Journal Article

Objectives

Endoscopic type 1 tympanoplasty using cartilage grafts for repair of chronic tympanic membrane perforation is increasing. The aim of this study was to evaluate the results of endoscopic type 1 cartilage tympanoplasty in children.

Materials and methods

Patients under 18 years of age who underwent type 1 cartilage tympanoplasty between January 2013 and February 2019 were retrospectively evaluated. Anatomic success rate was calculated according to the intact status of the graft. Air conduction (AC), bone conduction (BC), air-bone gap (ABG) and hearing gain were calculated using pure tone audiometry tests at pre-operative, and 6th month postoperative period. Patients with postoperative ABG ≤20 d
B were considered as functionally successful.

Results

The study included 56 patients (6 bilateral) and 62 ears. The anatomic success rate of our study was 91.9% (57/62). Preoperative AC was 36.4 ± 6.5 (21–50) d
B, BC was 7.8 ± 3.7 (5–25) d
B, and ABG was 28.6 ± 6.9 (10–41) d
B. Postoperative AC was 24.5 ± 8.8 (7–45) d
B, BC was 7.6 ± 3.4 (5–19) d
B, and ABG was 16.9 ± 7.4 (2–32) d
B. There was no change in postoperative BC (p: 0.683), whereas AC, and ABG significantly decreased (p < 0.001). Mean hearing gain was 12.1 ± 6.2 (3–26) d
B and the functional success rate was 72.5% (45/62).

Conclusion

Endoscopic type 1 cartilage tympanoplasty, a minimally invasive surgical procedure, provided successful functional and anatomical results at the pediatric population.

Pediatric Vocal Symptoms Questionnaire (PVSQ): Four new versions for parental evaluation and self-evaluation

10-01-2020 – Livia Lima, Mara Behlau

Journal Article

Objectives

To elaborate reduced versions of the Pediatric Vocal Symptoms Questionnaire (PVSQ) protocol for the two forms of application of the instrument: self-evaluation (SE) and parental evaluation (PE).

Methods

The Brazilian validation database of the PVSQ was used (Zip code: 758,309). Data were collected from 716 people, comprising 367 children and adolescents and 349 parents/guardians with and without voice complaint and/or vocal alteration. Three major procedures were adopted: 1. Verification of suitability of the database; 2. Determination of the extraction technique and the number of factors to be extracted, and 3. Decision on the type of factor rotation. For factor analysis, the Kaiser rule and Varimax rotation were used; Spearmans correlation analysis was used to verify the degree of relationship between the PVSQ variables, both of the SE and PE. The Mann-Whitney test was used to analyze the ROC curve of the general score of vocal symptoms of each version. A 5% significance statistical level was adopted.

Results

Factor analysis made it possible to elaborate four new versions of the PVSQ, called: “common core of the PVSQ” (SE and PE), “common core of the PVSQ reduced version” (SE and PE), “PVSQ SE reduced version” and “PVSQ PE reduced version”. The most complete version of the PVSQ has 7 domains in the SE and 5 in the PE. The common core of the PVSQ SE reduced version had a better area under the ROC curve (AUC), efficiency and sensitivity, and the PVSQ PE reduced version had better specificity.

Conclusions

Four new reduced PVSQ versions are available, containing 10 to 26 items. Aspects of specificity, efficiency and sensitivity, as well as discriminating capacity should be considered in the choice of the version and is a prerogative of the clinician who applies the instrument. As the questionnaire allows the comparison between the parental evaluation and the self-evaluation, it is recommended to apply the same version for parents and children.

Evaluation of the effect of Boyle-Davis mouth gag on intracranial pressure in patients undergoing adenotonsillectomy by using ultrasonographic optic nerve sheath diameter measurement

10-01-2020 – Elif Karali, Abdullah Demirhan, Akif Gunes, Ahmet Ural

Journal Article

Objective

We aimed to evaluate the effect of an increase in intracranial pressure (ICP) due to sympatho-adrenergic response caused by mouth gag and tongue depressor during adenotonsillectomy by measuring the optic nerve sheath diameter (ONSD) by ultrasonographic method.

Methods

Forty patients (age range 3–12 years) who underwent adenotonsillectomy were included in the study. All patients underwent surgery under general anesthesia with endotracheal intubation. Boyle-Davis mouth gag was used during the procedure. ONSD measurement was performed and a high-frequency linear probe. All ONSD measurements were performed by a single investigator experienced in the use of ultrasound. Ultrasonographically measured ONSD before induction was accepted as baseline (T0) value. Immediately after insertion of the mouth gag (T1), just before removal of the mouth gag (T2), and just before extubation (T3), ultrasonographic measurements of ONSD were recorded.

Results

When patients baseline ONSD values were compared with the values obtained in T1, T2, T3, and a statistically significant increase was detected. The ONSD value measured before removing the mouth gag (T2) was significantly higher than the ONSD value measured immediately after the insertion of the mouth gag (T1). The ONSD value measured just before extubation (T3) and after removal of the mouth gag was significantly lower than the ONSD value (T2) measured just before removal of the mouth gag. When heart rate, mean arterial pressure (MAP), peripheral oxygen saturation (Sp
O2) values of T0, T1, T2, T3 were compared, any statistically significant difference was not observed. When end-tidal carbon dioxide level (et
CO2) and peak inspiratory pressure (PIP) values of T1, T2, T3 were compared, any statistically significant difference was not observed.

Conclusion

This study showed that the Boyle-Davis mouth gag used during the adenotonsillectomy operation resulted in a significant increase in the diameter of the optic nerve sheath measured ultrasonographically and increased the ONSD even further during the time the mouth gag was remained in situ. In children with intracranial pathologies who will undergo adenotonsillectomy or those with increased ICP-related risk factors, the risks that may arise from the effect of the Boyle-Davis mouth gag on ICP should be considered.

Intracranial complications of pediatric rhinosinusitis: Identifying risk factors and interventions affecting length of hospitalization

07-01-2020 – Corina Din-Lovinescu, Ghayoour Mir, Conor Blanco, Kevin Zhao, Thomas Mazzoni, Arno Fried, Mostafa El Khashab, Giant Lin

Journal Article

Objective

To identify risk factors and interventions affecting length of hospitalization (LOH) and clinical outcome in children with intracranial complications of rhinosinusitis.

Methods

Retrospective chart review of 12 children hospitalized at 2 academic medical centers for intracranial complications of rhinosinusitis over the past 5 years.

Results

12 patients were identified with an average age at presentation of 13 years old. 92% were male and 75% were African American. The most common presenting symptoms were fever and headache. Localizing neurological symptoms including hemiparesis and aphasia, in addition to seizures occurred in 33% of patients and increased LOH significantly (33 versus 15 days, p = 0.03). Epidural (EA) and subdural abscesses (SA) were the most common intracranial complications. 58% of patients were initially treated with a combination of open neurosurgical (ON) intervention and endoscopic sinus surgery (ESS) and LOH was significantly shorter for these patients compared to those treated otherwise (14 versus 31 days, p = 0.02). Streptococcus species were the most common group of bacteria identified in 75% of cases, with S. anginosus accounting for 42% of cases. The overall average LOH was 21 days with 92% of patients having complete resolution of symptoms by time of discharge.

Conclusions

Treatment of intracranial complications of acute rhinosinusitis can have favorable outcomes after appropriate surgical management. Localizing neurologic symptoms and seizures portend longer hospital stay and recovery time. Shorter hospital stay was seen in those undergoing early combined ON and ESS interventions.

Post-tonsillectomy respiratory complications in children with sleep disordered breathing

07-01-2020 – Annie E. Moroco, Robert A. Saadi, Meghan N. Wilson

Journal Article

Objective

A common indication for tonsillectomy in children is clinically diagnosed sleep disordered breathing (SDB) without confirmation of obstructive sleep apnea (OSA) by polysomnography (PSG). Our goal was to review rates of post-tonsillectomy respiratory complications in children with SDB without prior PSG in order to develop recommendations for postoperative monitoring and safe hospital discharge in this population.

Methods

Following Institutional Review Board (IRB) approval at Penn State Milton S. Hershey Medical Center, a database query using Current Procedural Terminology (CPT) codes for tonsillectomy with or without adenoidectomy (42820, 42821, 42825, 42826) between January 1, 2012 and December 31, 2017 was performed. International Classification of Diseases (ICD) codes for sleep disordered breathing (G47.30), snoring (R06.83), and obstructive sleep apnea (G47.33) were applied for further selection. Charts were individually reviewed to confirm the inclusion criteria of pediatric patients (≤18 years) who underwent tonsillectomy without prior PSG and were monitored overnight. Demographic, operative, and relevant postoperative hospital course data (including desaturations, supplemental oxygen requirements, and upgraded level of care) were collected.

Results

A total of 1874 unique patient encounters were identified by our database search and 364 children met inclusion criteria. The average age of the patient population was 6.5 ± 3.1 years and 52.2% of children were female. Mean z-score for the population was 0.6. The rate of overnight oxygen desaturation events (<95%) was 2.2%, with no severe complications found in this population. Children with desaturation events were supplemented with oxygen and resolved prior to hospital discharge. Only race was found to be significantly related to risk of mild overnight desaturations (P = 0.023).

Conclusion

A lack of significant postoperative respiratory complications or alterations in the clinical management of children with SDB without prior PSG supports the idea that such patients may safely be discharged from the hospital following tonsillectomy without overnight oxygen monitoring.

Binaural interaction component of the auditory brainstem response in children with autism spectrum disorder

07-01-2020 – Doaa ElMoazen, Ossama Sobhy, Rania Abdou, HebatAllah AbdelMotaleb

Journal Article

Introduction

There is ample evidence that auditory dysfunction is a common feature of autism spectrum disorder (ASD). Binaural interaction component (BIC) manifests binaural interaction and is valid and proven response which reflects ongoing binaural processing.

Objectives

To investigate the differences in binaural interaction component of auditory brainstem response (ABR-BIC) between children with autism spectrum disorder (ASD) and normal peers and to correlate between ABR-BIC amplitudes and the acquired communication skills in ASD children.

Methods

ASD was diagnosed according to the criteria of 5th edition of diagnostic and statistical manual of mental disorders (DSM-V) and all children with ASD underwent test of acquired communication skills (TACS). Click evoked ABRs were elicited by left monaural, right monaural and binaural stimulation at intensity of 65 d
Bn
HL in all participants. ABR-BIC was then calculated as the difference between the binaurally evoked ABR waveform and a predicted binaural waveform created by algebraically summing the left and right monaurally evoked ABRs. The difference in amplitudes that gives rise to ABR-BIC is at IV-VI waves.

Results

ABR-BIC amplitudes were demonstrated to be significantly reduced in the ASD group compared to the control group. There was significant positive correlation between ABR-BIC amplitude and the language and social scores in TACS.

Conclusion

This study provided an objective evidence of binaural processing disorder in children with ASD.

Postoperative respiratory complications and disposition in patients with type 1 laryngeal clefts undergoing injection or repair – A single institution experience

07-01-2020 – Vandra C. Harris, Nicholas M. Dalesio, James Clark, Jason C. Nellis, David E. Tunkel, Andrew H. Lee, Margaret Skinner

Journal Article

Objective

Identify incidence and factors associated with respiratory complications after type 1 cleft repair.

Methods

Retrospective chart review of patients who underwent cleft repair over a 5-year period performed by a single surgeon. Primary endpoint was respiratory complications (oxygen desaturation <90%). Fishers exact test was used to identify differences between repair types (endoscopic carbon dioxide laser-assisted repair and injection laryngoplasty). Logistic regression was used to identify predictors of respiratory events.

Results

Fifty-five patients were included. Thirty-four (62%) patients underwent endoscopic carbon dioxide laser-assisted repair and 21 (38%) underwent injection laryngoplasty. Average hospital stay for each group was 1.6 days (SD = 3.1) and 0.6 days (SD = 0.9), respectively. Desaturations occurred in three patients (9%) in the laser-assisted repair group and one patient (4%) in the injection group. All occurred within 3 h after surgery and resolved with supplemental oxygen, oral airway placement, and/or mask ventilation. Two affected patients had comorbid diagnosis of asthma (one had poor medication compliance), and one had a history of developmental delay and hypotonia. In the injection group, desaturations occurred in one patient with a history of tracheal stenosis and double aortic arch. No correlation existed between repair type and desaturation (p = 0.57). No variables were significant predictors of events.

Conclusions

In this cohort, respiratory events after type 1 laryngeal cleft repair occurred early in the postoperative period, in children with cardiac and pulmonary comorbidities. This suggests postoperative admission may only be necessary for a select group of patients undergoing type 1 cleft repair. However, further research is needed to determine criteria for same-day discharge.

Can endoscopic ear surgery replace microscopic surgery in the treatment of acquired cholesteatoma? A contemporary review

17-01-2020 – Yi Hu, Bing Mei Teh, Guillermo Hurtado, Xu Yao, Juntao Huang, Yi Shen

Journal Article, Review

Acquired cholesteatoma leads to significant morbidities while current surgical options remain a challenge. The principles of surgery include complete removal of disease, prevention of recurrence, and restoration of hearing function when possible. Traditionally, this has been performed using microscopes; however, a novel technique using endoscopes offers a new perspective on our understanding of anatomy, pathogenesis and surgical approaches. In recent years, various studies have demonstrated good outcomes with transcanal endoscopic ear surgery (EES) in cholesteatoma surgery. Nevertheless, the use of EES is not universal and remains controversial due to the efficacy of microscopes, specific limitations of endoscopes and the need to learn new skills. This review focuses on recent advances in EES for the treatment of acquired cholesteatoma, benefits, current challenges, and a discussion on the indications and contraindications of EES.

Manifestations of Pediatric Extranodal Rosai Dorfman Disease in the head and neck

07-01-2020 – Mohamedkazim M. Alwani, Alhasan N. Elghouche, Elizabeth A. Schueth, Vincent J. Campiti, Bruce H. Matt, Abideen O. Yekinni

Journal Article, Review

Objectives

To systematically evaluate the clinico-diagnostic profile and management outcomes of otorhinolaryngologic manifestations of Extranodal Rosai-Dorfman Disease (ENRDD) in the pediatric population.

Methods

The search terms Rosai Dorfman Disease and Sinus Histiocytosis were used to query PubMed, Ovid/Medline, and Scopus databases from inception through September 30, 2018. Studies were systematically reviewed in accordance to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol. All reports of ENRDD involving at least one otorhinolaryngologic subsite in children less than 18 years were eligible for inclusion.

Results

A total of 31 studies met inclusion criteria resulting in identification of 31 ENRDD cases with a mean age of 11.5 years. Of the 31 patients, 23 were male (74.2%) and 7 were female (22.6%). Extranodal lesions were limited to otorhinolaryngologic subsites in 24 patients (77.4%), while 7 patients (22.5%) were found to have extranodal lesions simultaneously involving otorhinolaryngologic and nonotorhinolaryngologic sites. The nasal vault was the most common otorhinolaryngologic site involved (n = 13, 41.9%), followed by the paranasal sinuses (n = 10, 32.3%). The most common non-otorhinolaryngologic site that was concurrently involved was the orbit (n = 4, 57.1%). Concurrent cervical lymphadenopathy was present in 19 patients (61.2%). While not documented for 2 cases, emperipolesis on histopathology was confirmed in 29 patients (93.5%). Single therapy with surgical excision was the most common modality of treatment (n = 15, 53.6%) and yielded highest remission rates (80%). In 11 instances (34.36%), ENRDD was misdiagnosed.

Conclusion

Pediatric ENRDD is a rare disease entity that maintains a high misdiagnosis potential. The most common otorhinolaryngologic location for extranodal manifestation is the sinonasal compartment. Surgical excision remains the most common treatment modality yielding lowest persistence and/or recurrence rates.

The impact of auditory nerve functional states on the correlations between human and computer decisions for electrically evoked compound action potential threshold

17-01-2020 – Qiang Li, Chen Zhang, Tianhao Lu, Changjian Xu, Zengjun Sun, Wei Fan, Zhengmin Wang, Shufeng Li

Journal Article

Objectives

The electrically evoked compound action potential (ECAP) is widely used in clinical to reflect the functional states of the auditory nerve in cochlear implant (CI) recipients, especially in pediatric CI users. Currently, the software can automatically provide the ECAP threshold, which is convenient and not affected by the subjective judgement of the clinicians. However, it remains unclear whether the correlations between human and computer decisions for ECAP threshold can be affected by auditory nerve functional states, which is also the main purpose of our present study.

Methods

Intracochlear electrical stimulation, which can decrease the excitability of the auditory nerve, was used to change the auditory nerve functional states of guinea pigs. Ten normal-hearing guinea pigs were implanted with CIs unilaterally. ECAPs were recorded both before and after the electrical stimulation, representing different functional states of the auditory nerve. Forward masking (Fwd
Msk) and alternating polarity (Alt
Pol), two most commonly-used artifact-reduction methods, were applied to the measurements. All measurements recorded by the software were saved for computer and human analysis with linear regression and visual detection methods.

Results

The correlations between human and computer performance in the peak-picking process were not affected by auditory nerve states and artifact-reduction methods. However, complicated findings were observed for ECAP threshold. With Fwd
Msk utilized, weaker correlations between human and computer performance were observed in abnormal state compared to those in normal state. Regardless of the functional states of the auditory nerve, the results revealed stronger correlations in Alt
Pol than those in Fwd
Msk. Furthermore, when compared with human decision, computer linear-regression threshold (C-LRT) was always less accurate than computer visual-detection threshold (C-VDT), which was not affected by auditory nerve states.

Conclusions

(1) the functional states of the auditory nerve can definitely affect the correlations between human and computer decisions for ECAP threshold, but the impact is limited to the Fwd
Msk method; (2) Alt
Pol can produce stronger correlations compared with Fwd
Msk, which is not affected by auditory nerve states; and (3) regardless of the auditory nerve states, C-VDT can always show higher consistency with human decision, while C-LRT reveals more variability.

Trimodal embolization of juvenile nasopharyngeal angiofibroma with intracranial extension

22-12-2019 – Andrew J. Maroda, Nicholas A. Beckmann, Anthony M. Sheyn, Lucas Elijovich, L. Madison Michael, Julie M. DiNitto, Sanjeet V. Rangarajan

Journal Article

Objectives/purpose

1. Understand three different techniques for embolization of juvenile nasal angiofibroma (JNA) and assess their combined efficacy. 2. Perform successful endoscopic intralesional embolization of highly vascular sinonasal neoplasms.

Methods

In this study, we present the case of a 10-year-old male patient diagnosed with juvenile nasal angiofibroma (JNA) who successfully underwent trimodal embolization and resection at a tertiary academic medical center after failed coil embolization in his home country. We examine the clinical details of the case and a review of pertinent literature.

Results

Preoperative embolization is common in the treatment of JNA, but there is little consensus as to the proper timeframe and techniques utilized. In our case, preoperative imaging revealed a vascular tumor with intracranial extension consistent with UPMC Stage V JNA. Diagnostic angiogram revealed significant arborization from the internal and external carotid systems. A trimodal embolization technique, utilizing transarterial, percutaneous, and direct endoscopic intralesional injection of n-Butyl Cyanoacrylate (n-BCA) was performed. A two-staged endoscopic and open resection was subsequently performed one week later with minimal blood loss. In our case, combining intralesional embolization with traditional transarterial techniques resulted in an improved operative field and a successful clinical result.

Conclusion

Embolization of highly vascular sinonasal tumors with n-BCA is not limited to endovascular techniques, but can be safely combined with percutaneous and endoscopic intralesional embolization up to one week prior to surgical resection.

Acute mastoiditis in a newborn with 11 days of life: Case report

08-12-2019 – Ana Sousa Menezes, Daniela Ribeiro, Sara Pereira, Albina Ramires, Luís Dias

Journal Article

Acute mastoiditis is a potentially life-threatening complication and extremely rare in children under six months. We herein report the case of a 11-days-old newborn with acute mastoiditis complicated by subperiosteal abscess, submitted to surgical and medical treatment. A transient hypogammaglobulinemia was finally diagnosed and successfully managed without complications. Early recognition of this rare immunological disorder and prompt intervention are critical to prevent further complications.

International Pediatric ORL Group (IPOG) Robin Sequence consensus recommendations

04-01-2020 – Pierre Fayoux, Sam J. Daniel, Gregory Allen, Karthik Balakrishnan, An Boudewyns, Alan Cheng, Alessandro De Alarcon, Dimple Goel, Catherine K. Hart, Nicolas Leboulanger, Gi Lee, Eric Moreddu, Harlan Muntz, Reza Rahbar, Richard Nicollas, Carolyn R. Rogers-Vizena, John Russell, Michael J. Rutter, Richard J.H. Smith, Michelle Wyatt

Journal Article

Objective

To provide recommendations for the comprehensive management of airway obstruction in patients with Robin Sequence.

Methods

Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG).

Results

The consensus statement provides recommendations for medical specialists who manage infants with Robin Sequence including: evaluation and treatment considerations for commonly debated issues in post-natal airway obstruction, assessment of antenatal obstruction and perinatal airway management.

Conclusion

Consensus recommendations are aimed at improving management of airway obstruction in patients with Robin Sequence.

Parents’ satisfaction with a trial of a newborn hearing screening programme in Jordan

31-12-2019 – Maha Zaitoun, Amjad Nuseir

Journal Article

Objective

This study examines parents satisfaction level toward a trial of a newborn hearing screening programme (NHSP) that was applied in King Abdullah II University Hospital (KAUH) in Jordan over one year. This is the first study that investigated parents’ satisfaction toward a hearing screening programme in the Arab countries, and the results will improve any future screening programmes in the Arabian region.

Method

The main tool for this study was a questionnaire that was translated and modified from the original version of the Parental Satisfaction with the Newborn Hearing Screening Programme (PSQ-NHSPs1). The questionnaire consisted of 19 items covering five main aspects of the NHSP. The parents responses were not anonymously given where the parents whose children had undergone the hearing screening were contacted by phone using the data record of the hospital.

Results

The majority of the parents were very satisfied with the programme overall and showed great support and appreciation for the effort in testing their babies and increasing their awareness. The satisfaction levels varied among the specific aspects of the programme. Good portion of the parents did not receive the brochure containing information about the screening, and almost half of them did not know the results of the hearing screening.

Conclusion

Parents were overall satisfied with neonatal hearing screening programme that was conducted at KAUH. However, parents were less satisfied with information related to the test procedure and results. Parents responses in this study could be used to improve any future hearing screening program in Jordan or in the Arab countries.

Age-related variability in pediatric scalp thickness: Implications for auditory prostheses

31-12-2019 – Sunil D. Sharma, Edward Park, Patricia L. Purcell, Karen A. Gordon, Blake C. Papsin, Sharon L. Cushing

Journal Article

Objectives

Minimizing soft tissue reduction during the placement of percutaneous implants has been encouraged to improve cosmesis and perhaps decrease soft tissue complications. However, the fidelity of sound transmission and retention of transcutaneous magnet devices relates to the intervening soft tissue thickness. The objective of this study was to examine age-related variability in scalp thickness in patients receiving either percutaneously implanted or transcutaneous magnet-retained auditory prostheses.

Methods

Children undergoing percutaneous implant retained prostheses or cochlear implantation underwent two scalp thickness measurements: 1) a superior measurement at the conventional marked site for cochlear implant receiver stimulator and 2) an inferior measurement 1.5 cm below the superior measurement representing the conventional marked site for cochlear implant fantail. Linear regression was used for statistical analysis.

Results

Eighty-five children (median age 6 years; range 9 months–17 years) were examined. Scalp thickness at the two sites ranged from 1 to 10 mm (superior mean = 4.1 mm; inferior mean = 4.3 mm). A strong correlation was observed between age and scalp thickness at the superior (R = 0.62, p < 0.01) measurement, while a moderate correlation was observed at the inferior (R = 0.45, p < 0.01) sites. Children younger than 7 years displayed little variability in scalp thickness, and no correlation between age and scalp thickness (R = 0.002, p = 0.74).

Conclusion

In older children, scalp thickness varies significantly with age. However, children younger than 7 years consistently have scalp thicknesses of 3–4 mm. Lack of variability in scalp thickness in children younger than 7 years argues against the use of an age-adjusted approach to soft tissue management in this age group.

Turkish adaptation of quality of life questionnaire for children with hearing loss and the assesment of the effects of hearing loss on quality of life among children aged 7-12

01-01-2020 – Nazan Nemli, İsmet Bayramoğlu, Yusuf Kemal Kemaloğlu

Journal Article

Monitoring the effects of Hearing Loss on Quality of Life, which is frequently seen on children and causes inefficiency in speech, education, and social development, is not only important for listening skills, using hearing devices, and linguistic and speech skills but also important for the treatment. And, if no treatment possible, then it is also critical for rehabilitation with different equipment. By making use of the Quality of Life Questionnaire, developed by Streufert at University of Washington in 2008 to Turkish and Turkish culture, this study was conducted in order to assess the effect of hearing loss of children between the age of 7 and 12 on their quality of life, as well as determining the effects of the hearing loss-related variables on the quality of life.

The study was carried out on 115 kids between the ages of 7 and 12; 35 with unilateral HL, 45 with bilateral HL, and 35 of them without any hearing loss. The questionnaire was named İŞYAK (İşitme Kayıplılar için Yaşam Kalitesi Ölçeği – as with Turkish initials – Quality of Life Questionnaire for Hearing Loss). If the İŞYAK was confirmed on the sample in Turkish culture was examined by using Confirmatory Factor Analysis (CFA). In addition to CFA, the data obtained from the İŞYAK and Quality of Life Scale for Children (ÇİYKÖ) were compared by using variance analysis, and it was determined that there were significant differences between the normally hearing children and the children having hearing loss in terms of school, physical activity scores of ÇİYKÖ and all the dimensions of İŞYAK (environment, activity, emotions). In conclusion, besides the general similarities with the results obtained from the study on developing İŞYAK, differences were observed in the demographic variables.

The statistical analyses conducted on İŞYAK showed that İŞYAK can be used both in clinical and as well as in rehabilitation centers as a special questionnaire for determining the quality of life for the kids with hearing loss with reliable and valid results.

Acute pediatric tracheitis: Distinguishing the disease by tracheostomy status

31-12-2019 – Jonathan S. Ni, Jocelyn Kohn, Udayan K. Shah, Jessica R. Levi

Journal Article

Objectives

Tracheitis is an upper airway infection that often presents in patients with tracheostomies and can potentially cause airway obstruction. This study aims to use a nationwide database to identify a large cohort of pediatric patients admitted with tracheitis to elucidate the management and resource utilization associated with the disease both with and without tracheostomies.

Methods

The Kids’ Inpatient Database (KID) 2012 was used to identify 2394 weighted discharges with acute tracheitis, with or without obstruction, as the primary diagnosis. Data on prior tracheostomy status, demographics, hospital characteristics, management, and resource utilization were obtained. Two groups of interest, based on presence of prior tracheostomy, were studied. Linear regression was performed to determine independent predictors of total charges.

Results

The mean age was 5.52 years (SD: 5.54), mean length of stay (LOS) was 6.37 days (SD: 10.18), and mean total charges were $60,996.61 (SD: 107,798.41). Patients with prior tracheostomy had lower rates of endoscopy and endotracheal intubation than patients without (p < 0.0005). There was no significant difference in LOS (p = 0.076) or total charges (p = 0.210) between the groups based on prior tracheostomy status.

Conclusion

Pediatric tracheitis should be differentiated on the basis of tracheostomy status. We propose that tracheitis diagnosis codes should be distinguished by the presence of tracheostomy as “open” and the absence of tracheostomy as “closed.”

Trajectory of change in the swallowing status in spinal muscular atrophy type I

17-01-2020 – Young-Ah Choi, Dong In Suh, Jong-Hee Chae, Hyung-Ik Shin

Journal Article

Objectives

This study aimed to elucidate the change in progressive swallowing dysfunction from birth up to 2 years of age to provide clinical insights into the management of swallowing difficulty in patients with spinal muscular atrophy (SMA) type I.

Methods

Data of 11 patients with SMA type I were retrospectively reviewed. The Neuromuscular Disease Swallowing Status Scale (Nd
SSS) scores and videofluoroscopic swallowing study (VFSS) were used.

Results

Swallowing function deteriorated in patients with SMA type I at an approximate age of 6 months. Tube feeding was initiated at the median age of 6 months (interquartile range, 3–7 months). The transition period for switching the feeding route from totally oral to tube feeding varied widely among patients (5–12 months). In four patients, aspiration was observed in VFSS, even when nutrition was provided orally. In two patients, the evidence of laryngeal aspiration was obtained via the VFSS during the very early stages of the disease at 3 and 4 months. Conversely, in one patient, total oral feeding was maintained for up to 12 months, and evidence of aspiration was not observed in the VFSS.

Conclusion

An individualized approach is essential, as the timeline of deterioration of swallowing function varies widely in patients with SMA type I.

Carbon dioxide laser versus cold-steel supraglottoplasty: A comparison of post-operative outcomes

31-12-2019 – Jeffrey C. Yeung, Syed O. Ali, Mallory G. McKeon, Samantha Grenier, Kosuke Kawai, Reza Rahbar, Karen F. Watters

Journal Article

Objective

Supraglottoplasty is the mainstay of surgical treatment for laryngomalacia, and is commonly performed via two methods: cold steel or carbon dioxide (CO2) laser. The degree of post-operative monitoring following supraglottoplasty varies, both within and between institutions. The aim of this study was to compare the post-operative monitoring and interventions required by patients undergoing cold-steel versus CO2 laser supraglottoplasty.

Design

Retrospective cohort of pediatric patients (age < 18 years) undergoing supraglottoplasty at a tertiary care pediatric hospital. The primary exposure was the surgical instrument(s) used during supraglottoplasty. The primary outcome was prolonged intensive care unit (ICU)-stay (defined as >24 h).

Results

155 cases were eligible for inclusion. Fifty-eight (37.4%) patients had a comorbid condition. Common indications for surgery included feeding difficulty (56.1%), severe respiratory distress (33.5%), and obstructive sleep apnea (25.2%). CO2 laser was employed in 49 cases and cold-steel in 106 cases. Prolonged ICU-stay (>24 h) was observed in 14 CO2 laser cases (28.6%) and 11 cold-steel cases (10.4%) (adjusted OR 3.42; 95% CI 1.43, 8.33). CO2 laser cases were more likely to require post-operative intubation, non-invasive positive pressure ventilation, and nebulized racemic epinephrine. Concomitant neurological condition was associated with an increased risk of prolonged ICU-stay, while extent of surgery and age were not.

Conclusions

CO2 laser supraglottoplasty is associated with an increased risk of prolonged ICU-stay and need for ICU-level airway intervention, compared to the cold-steel technique. While this association should not be misconstrued as a causal relationship, the current study demonstrates that specific surgical factors may influence the patient monitoring requirements following supraglottoplasty, particularly the choice of instrument and the extent of surgery.

Novel OTOF pathogenic variant segregating with non-syndromic hearing loss in a consanguineous family from tribal Rajouri in Jammu and Kashmir

26-12-2019 – Raja A.H. Kuchay, Yaser Rafiq Mir, Xue Zeng, Asima Hassan, Kazunori Namba, Mustafa Tekin

Journal Article

Background

Hereditary hearing loss is characterized by a very high genetic heterogeneity. The OTOF (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder.

Methods

In this study, whole exome sequencing was employed for detection of novel pathogenic variant that segregates with autosomal recessive nonsyndromic hearing loss in a tribal family from Rajouri, Jammu and Kashmir. Proband was a 9-year-old male born to first-cousin parents and presented with sensorineural hearing loss since birth. Family resides in an area with high consanguinity and lack of basic health care facilities including genetic counselling services.

Results

We report a novel OTOF pathogenic variant NM_194248.2:c.4249_4250ins
G (p.
Ser1417Cysfs
Ter4) co-segregating with hearing loss in this family and not present in any public databases.

Conclusions

Our findings not only extend the geographical and mutational spectrum of autosomal recessive nonsyndromic hearing loss but also support the need for introducing genetic counselling services to rural and tribal areas in India with high consanguinity.

How sacculo-collic function assessed by cervical vestibular evoked myogenic Potentials correlates with the quality of postural control in hearing impaired children?

07-01-2020 – Eugen Ionescu, Pierre Reynard, Nathalie Goulème, Cécile Becaud, Karen Spruyt, José Ortega-Solis, Hung Thai-Van

Journal Article

Objective

Functional integrity of vestibular end organs is essential for gaze stabilization, dynamic visual acuity, postural control and spatial orientation. Some authors hypothesized on the importance of saccules for postural control and motor development in children, including achievements such as standing up and walking. The purpose of this article was to observe how saccular dysfunction assessed by cervical Vestibular Evoked Myogenic Potentials (cVEMPs) correlates with the quality of postural control in non-syndromic deaf children.

Method

Seventy-six non-syndromic hearing-impaired children were retrospectively included. Sacculo-collic pathway was assessed with cVEMPs elicited in bone conduction. The response was quoted “normal” if a reproducible wave P13–N23 of at least 50μV in amplitude was present, if not, it was quoted “absent”. The sample was divided in 3 groups depending on the presence of the sacculo-collic responses: normal bilateral group (Group 1), normal unilateral (Group 2) and absent bilaterally group (Group 3). Motor assessment was achieved with Movement Assessment Battery for Children, second edition (MABC-2). Postural control (PC) was assessed using the dynamic Balance Quest platform. The scores obtained with MABC-2, and the postural parameters recorded on the Balance Quest platform (sway of Centre of Pressure and spectral power index) were analyzed and compared throughout the groups.

Results

Group 1 (normal bilateral) showed the best scores regarding motor abilities and postural stability within available normative data. Group 3 (absent bilateral) had the lowest motor and postural control skills. A good correlation between the scores obtained by MABC-2 motor test and dynamic posturography (Balance Quest) was observed.

Conclusions

The presence of at least one sacculo-collic response would predict satisfactory static and dynamic motor and postural control skills in non-syndromic hearing-impaired children. MABC-2 and Dynamic Posturography Balance Quest appears reliable and comparable tools for PC assessment in hearing impaired children. In the light of these results, it appears that in young children candidates for uni- or bilateral CI whose walking is not yet acquired, should receive a vestibular assessment before surgery to avoid the risk of bilateral sacculo-collic function impairment.

Nasopharyngeal cultures in children; when, what and why?

24-12-2019 – Samuel Flyman, Ann Hermansson, Marie Gisselsson-Solén

Journal Article

Introduction

Nasopharyngeal cultures are commonly used to determine the causative bacteria in upper airway infections. However, several bacteria can occupy the nasopharynx simultaneously and most healthy children are asymptomatic carriers of presumptive pathogens. This makes the interpretation of nasopharyngeal cultures difficult. Knowledge about which bacteria reside in the nasopharynx can assist the physician in the choice of antibiotic treatment and might also predict the risk of complications. Today, little is known about how nasopharyngeal cultures are being used in clinical practice.

Objectives

The aim of this study was to explore how nasopharyngeal cultures are used in clinical practice, when and why they are performed, what they show, and what impact they have on the treatment of the patient.

Methods

The results of all nasopharyngeal cultures taken from children aged 0–12 years in the county of Skåne, Sweden, during 2018 were obtained. Medical charts from hospitals and primary care centres were used to determine why the cultures were taken and whether they resulted in a change of treatment.

Results

During 2018, 2200 nasopharyngeal cultures were taken, most of them during the winter season. Forty-one percent of children had on-going antibiotic treatment or had been treated with antibiotics in the previous two months. Acute otitis media (AOM) was the most common reason for taking a culture. The most frequently identified bacteria were Moraxella catarrhalis and Haemophilus influenzae. There was a positive correlation between M. catarrhalis on one hand and Streptococcus pneumoniae and H. influenzae on the other. Overall, bacterial resistance was rare. The presence of beta-lactamase negative ampicillin resistant H. influenzae was associated with recent or on-going antibiotics, whereas S. pneumoniae with decreased penicillin susceptibility were found less frequently in the same group of children. A positive culture resulted in a change of treatment in 29% of the cases.

Conclusion

Apart from playing a confirmatory role and monitoring the incidence of resistant bacteria, almost a third of the nasopharyngeal cultures analysed in this study contributed to decision-making. It therefore appears that bacterial sampling have a role in clinical practice. It would be valuable to study more closely why nasopharyngeal cultures are taken in during AOM and how the result affects the treatment.

Endoscopic repair of septal perforation in children

22-12-2019 – Rusetsky Yury, Mokoyan Zhanna, Meytel Irina, Spiranskaya Olga, Malyavina Ulyana

Journal Article

Objective

Being increasingly faced with the problem of pediatric nasal septal perforations, we have found that the surgical management of nasal septal perforations in children is not widely described in the litrature. The objective of our study was to demonstrate the results of different surgical techniques, including two original endoscopic techniques, in the septal perforation repair in children.

Methods

24 children, ranging between 6 and 17 years of age, with nasal septal perforations were operated using different endoscopic techniques from February 2015 to May 2019 at the special tertiary referral clinic. Apart from well-known techniques, such as anterior ethmoidal artery flap, intranasal bipedicled advancement flap, sublabial flap, free temporal fascia graft, we used two original techniques – inverted edges technique and cross-septal returned flap.

Results

The total rate of complete perforation closure was 79% (19 of 24 patients). Regarding the reduction of symptoms, the efficacy of surgery was approaching 100%.

The combination of inverted edges technique and anterior ethmoidal artery septal flap demonstrated the best results with no reperforations in all 10 cases. Using cross-septal returned flap, we achieved complete closure of perforation in 5 (83%) of 6 patients. The remaining techniques were performed rare and showed relatively low rates of success. There were 2 cases of complications (oronasal fistula), both developed in patients with sublabial mucosal flap.

Conclusion

Use of endoscopic assistance, vascularized mucoperichondrial flaps and bilateral closure demonstrates high effectiveness in septal perforation surgical repair in children.

Level of evidence

4.

Slide tracheoplasty: Predictors of outcomes and literature review

22-12-2019 – Aileen Wertz, Stephanie M. Fuller, Christopher Mascio, Steven E. Sobol, Ian N. Jacobs, Luv Javia

Journal Article

Objective

Determine preoperative comorbidities and intraoperative parameters associated with adverse postoperative outcomes.

Methods

Retrospective case series at a single tertiary care childrens hospital from 2010 through 2017.

Results

Twenty-six patients with median age of 6 months and median weight of 7.1 kg underwent slide tracheoplasty. Median time to extubation, length of intensive care unit admission, and length of hospitalization were 7, 27, and 30 days, respectively. Twenty-two (85%) required no additional intervention. Overall success was 87%. One (4%) patient required open revision, and 3 (11%) required tracheostomy. Concomitant cardiac surgery was associated with postoperative tracheostomy (p = 0.04). Age and weight at surgery were inversely correlated with length of intubation (p = 0.03) and length of hospital stay (p = 0.001, p = 0.002) respectively. Hospital stay was 2.2 times longer if preoperative mechanical ventilation was required (p = 0.01) and 39% longer for every 1 mm decrease in airway diameter at the narrowest portion of the stenosis (p = 0.005). There were no deaths related to persistent tracheal stenosis with a median follow-up of 24 months.

Conclusion

Slide tracheoplasty is safe and effective. Concomitant cardiac surgery was associated with postoperative tracheostomy. Lower age and weight at surgery were correlated with longer length of intubation and hospital stay. Preoperative mechanical ventilation and smaller airway diameter were associated with longer hospital stay. This information may be helpful in counseling families and planning future prospective studies.

Laryngeal sequelae secondary to surgical treatment for recurrent respiratory papillomatosis in children

18-12-2019 – M.L. Scatolini, G. Labedz, A. Cocciaglia, C.G. Pérez, M.E. Nieto, M. Rodríguez D′Áquila, H.A. Rodríguez

Journal Article

Objective

to describe the frequency and predictive factors associated with laryngeal scarring caused by surgical treatment of recurrent respiratory papillomatosis (RRP) in children.

Introduction

RRP is an important cause of hoarseness and respiratory obstruction in children. The current standard of care for RRP is the systematic and repetitive surgical interventions. The repetitive surgeries may increase the risk of sequelae. A larger number of surgeries, the surgical technique used, and disease severity are related to an increased risk of scarring.

Material and methods

A retrospective, descriptive review of the medical charts of patients with RRP younger than 18 years was conducted. Between 2014 and 2017, 79 patients were identified; five patients were excluded. Demographic and clinical data were recorded and analyzed. The patients were divided into two groups, one with and the other without surgical sequelae, for comparison to identify sequela-associated factors.

Results

75 patients, 40 (53.3%) male, were analyzed. Age at symptom onset ranged from 2 months to 13 years. Median age at the time of diagnosis was 42 months. Overall, 44% presented with disseminated disease. A median of nine (range, 1 to 86) surgeries were performed in each patient with a median of two (range, 0.6 to 10) of the average number of surgeries per year per patient. 29 patients (38.7%) had laryngeal sequelae. When comparing the patients with and without sequelae, statistically significant differences were found in the variables of dissemination during the course of the disease, overall number of surgeries and mean number of surgeries per year, history and number of previous surgeries at an outside institution, urgent surgeries, and CO2 laser use. Patients who underwent more than 10 surgeries or who had a history of previous surgeries at an outside institution had a higher frequency of laryngeal scarring in multivariate analysis.

Conclusion

Scarring secondary to surgical treatment for RRP is common. Surgery-related variables seem to be predisposing factors. A greater number of surgeries and surgeries performed at less specialized centers are strongly related to this complication. Future studies with a larger sample size are necessary to determine whether other factors are involved.

Hearing screenings for preschool children: A comparison between whispered voice and pure tone audiogram tests

16-12-2019 – Lukas Skoloudik, Jan Mejzlik, Michal Janouch, Jakub Drsata, Jan Vodicka, Viktor Chrobok

Journal Article

Introduction

This prospective study compares the efficiency of two hearing screening tests performed on preschool children. These tests are known as whispered voice test and pure tone audiometry.

Methods

Standard hearing screenings were performed on five-year old children using a whispered voice test followed by ENT examination with pure tone audiometry.

Results

A total of 827 children were included in the study. Hearing loss (>25 d
B) was observed in 5.8% of the evaluated children (n = 48), being bilateral in only 1.6% (n = 13) of these cases. Slight hearing impairment (hearing loss of 16–25 d
B) was observed in 25.4% (n = 210) of the children, with 14.5% bilateral cases (n = 120). Interestingly, 62 children (7.5%) were under suspicion of hearing loss by their parents; however, an audiogram revealed the poor consistency of this diagnosis (sensitivity 20.8%, Cohens kappa coefficient of 0.048). The whispered voice test (6 m distance) throwed a hearing impairment diagnosis in 807 (48.8%) of examined ears; however, its sensitivity was of only 56.5%, with a specificity of 51.6% and Cohens kappa coefficient of 0.0254 (poor).

Conclusion

The hearing loss incidence in preschool children coupled with the low efficacy of whispered voice tests and the parents’ unreliability during the hearing impairment survey advocate for a more efficient audiometric hearing screening before beginning school attendance.

“Mothers emotional experiences related to their childs diagnosis of deafness and cochlear implant surgery: Parenting stress and childs language development”

17-12-2019 – Marinella Majorano, Letizia Guerzoni, Domenico Cuda, Marika Morelli

Journal Article

Objectives

The present study aims to assess the emotional experiences, specifically parenting stress, of mothers of children with cochlear implants (CIs), and their childrens language development before surgery and at three and six months after CI activation.

Methods

Twenty mothers of children with CIs were interviewed before their childrens surgery about their experiences in connection with the diagnosis of deafness, the surgery and the activation of the CI. The Parenting Stress Index questionnaire and the Mac
Arthur-Bates-Communication Development Inventory were administered before the surgery and at 3 and 6 months after the CI activation.

Results

Analysis of the qualitative data resulting from the interviews showed that the mothers’ emotional experiences before the CI surgery were complex. Mothers reported both positive and negative emotions related to deafness, diagnosis and surgery, benefits of the CI, coping strategies and future expectations.

The mothers of children with more advanced lexical production six months after CI activation displayed a high frequency of themes related to positive emotions, thoughts and coping strategies before the surgery. Distress on the part of the mothers, perceptions of difficulties in their child and instances of parent-child dysfunctional interaction were negatively and significantly related to the childs language and communication development.

Conclusions

The findings support the importance of assessing the mothers emotional experience in relation to diagnosis and CI activation before the surgery.

Implications for clinical practice are discussed

specifically, the importance of the support offered to the parents, aimed at enhancing both their awareness of their expectations about their childs rehabilitation process and their self-efficacy in supporting the childs adaptation to the use of the CI.

Effect of drug induced sleep endoscopy on intraoperative decision making in pediatric sleep surgery

14-12-2019 – Julia Dmowska, Stephen Reed Larson, M. Boyd Gillespie, Anthony Sheyn

Journal Article

Objectives

To demonstrate the effect of drug induced sleep endoscopy (DISE) on intra-operative decision making during pediatric sleep surgery for obstructive sleep apnea (OSA).

Methods

A retrospective chart review was performed on pediatric (3–17 years) patients with moderate-to-severe OSA (7.2–71.8) who underwent drug induced sleep endoscopy at the time of initial sleep surgery. The characteristics evaluated included age, race, gender, site of obstruction, type of surgical intervention, pre- and post-operative apnea and hypopnea index. Of the 26 patients that were identified, 18 had both a pre- and post-operative polysomnograms result.

Results

All patients underwent DISE immediately prior to surgical treatment. The mean pre-operative AHI for the 18 patients with post-operative polysomnogram results was 21.3 (7.2–71.8). The mean post-operative AHI for the 18 patients was 7.6 (0.7–25.1). There was a significant difference between pre- and post-operative AHI (p < 0.001). Of the 26 patients, the most common area of collapse was the soft palate, occurring in 17/26 (65.4%) patients. Base of tongue involvement was found to be present in 11/26 (42.3%) patients, and the epiglottis was involved in 4/26 (15.4%). Evidence of multilevel collapse was observed in 6/26 (23.1%) patients. Patients observed to have palatal collapse underwent a pharyngoplasty (20/26; 76.9%) at the time of adenotonsillectomy. Three (11.5%) patients underwent a tongue reduction.

Conclusion

This study provides additional evidence that DISE can affect intra-operative decision making, with the potential for improved post-operative outcomes. A randomized controlled study is needed to determine if these outcomes are better than what can be achieved without DISE.

Classification of the concha-type microtia and their new suitable treatment strategies without autogenous costal cartilage grafting

11-12-2019 – Fengfeng Guo, Lin Lin, Xiaobo Yu, YuPeng Song, Qinghua Yang, LeRen He, Bo Pan, Haiyue Jiang

Journal Article

Objective

Numerous corrective methods have been successfully applied in concha-type microtia reconstruction over the past several decades, and autogenous rib cartilage grafting has become a routine technique in a two or three-stage operation. However, it still remains a challenge due to the effective use of the large volume of the remnant cartilage and skin involved. The objective of this study was to clarify how this remnant cartilage and skin could be manipulated for new suitable treatment strategies without autogenous costal cartilage grafting.

Methods

A total of 424 patients with concha-type microtia operated at our Center from January of 2012 to June of 2019 have been reviewed and analyzed cases. At the same time, a classification system for grading the severity of concha-type microtia was created on the basis of anatomical findings and ear size.

Results

A total of 436 ear cases (involving 424 patients), showing concha-type microtia, were included in our study and reviewed through medical records, photographs, analysis of surgical methods, and postoperative outcomes. The concha-type microtia were classified into four graded types: Grade I (n = 151), Grade II (n = 101), Grade III (n = 93), and Grade IV (n = 79). A total of 352 ears in 345 patients with Grade I to III concha-type microtia were followed up for 1 month to 7 years (average, 14.7 months). 329 patients (95.4%) were satisfied with the aesthetic outcomes of the corrected ear.

Conclusions

Individual corrective methods and aesthetic outcomes for patients with Grade I to III of deformity were described in this study. The authors present new suitable approaches according to a progressive classification system which provide conservative and individualized methods of treatment in early stages of life.

Detection of nasal microbiota in pediatric patients with antrochoanal polyps by TLDA

15-12-2019 – Beibei Song, Huiwen Zheng, Shujing Han, Lixing Tang, Xiaojian Yang, Ping Chu, Pengpeng Wang, Jie Lu, Wentong Ge, Xin Ni

Journal Article

Objective

To evaluate and compare the microbiological features in ACPs groups and control subjects in pediatric group, further to explore the potential role of microbial in the etiology of ACPs.

Methods

A total of 32 patients with ACPs, and 10 control subjects were enrolled in this study. Demographic datas were collected. The Taq
Man low-density array assays were used to detect the microbial of swab specimens and nasal tissue samples from ACPs patients.

Results

A total of 15 species were identified in all groups. Of all the species, Mycoplasma pneumoniae was the most common species in ACP patients, but was negative in control group. Of all the viruses detected, Adenovirus positivity was significantly higher in control group than that in ACPs middle meatus on unaffected side, ACPs middle meatus on affected side, and ACPs polypous surface group (P < 0.05). Cytomegalovirus positivity was significantly higher in control group than that in ACP polypous group (P < 0.05). Human herpesvirus 6 (HHV-6) was absent in control goup, and positive in ACP middle meatus on affected side was significantly higher than that in ACP polypous surface and ACP polyp group (P < 0.05). The expression of other microbial differed not significantly in unaffected side, affected side of ACPs, ACPs polypous surface, and ACPs polyp.

Conclusions

Mycoplasma pneumoniae was the most common species in ACP patients. Streptococcus pneumonia and Moraxella catarrhalis were the only bacteria detected at certain frequency in nasal polyps and control subjects. Human herpesvirus 6 and
Mycoplasma pneumoniae may have potential role in the development of ACPs. The isolates rate of microbial differed in middle meatus on unaffected and affected side of ACPs, ACPs polypous surface, ACPs polyp, and their role in the etiology of ACPs need to be further studied.

Prevalence of hearing impairment among primary school children in the Kilimanjaro region within Tanzania

10-12-2019 – Solvang Iselin Ertzgaard, Naalsund Kristin, Tønder Sofie, Hansen Giske Sindberg, Hagan Tobias Bang, Mnyanyi Cosmas, Tronstad Tron Vedul, Nkya Aslam, Øygarden Jon

Journal Article

Introduction

Hearing impairment is becoming a public health concern and a widespread problem. The World Health Organization estimates that globally there are about 466 million people with hearing impairment, of which 34 million represent children. We examined the prevalence and etiology of hearing impairment among primary school children in the Kilimanjaro region within Tanzania.

Method

A cross-sectional study of primary school children aged 6–17 was conducted to determine the prevalence of hearing impairment. Tanzanian primary school children were screened for hearing loss and an otoscopy was performed on those who failed the screening.

Results

There were 403 children screened from three schools in rural areas of Tanzania. The prevalence of hearing loss was found to be 7.1%, 10.8% and 16.7% in the different schools. Cerumen was proven to be the most common possible cause of hearing impairment. Other possible causes of hearing impairment were found to be (in descending order) otitis (all types), no abnormalities, and perforation of the tympanic membrane.

Conclusions

These results show a need for better identification of hearing impairment in school-going children and implementation of prevention and treatment options in Tanzania and other developing countries. This can further improve childrens potential for language development, communication and academic achievement.

Evaluation of sagittal airway dimensions after face mask therapy with rapid maxillary expansion in Class III growing patients

10-12-2019 – Elisabetta Cretella Lombardo, Lorenzo Franchi, Roberta Lione, Alice Chiavaroli, Paola Cozza, Chiara Pavoni

Journal Article

Objective

The aim of this investigation was to analyze the effects of RME and FM on the airway dimensions in Class III patients compared with untreated controls.

Methods

A sample of 47 Class III patients treated with RME and FM was analyzed. The treated group was compared with 18 untreated controls. Lateral cephalograms for each patient at T1, T2, and T3 were digitized.

Results

At long-term evaluation a significant increase in airway size and a significant decrease in adenoid size were found in the treated group as well as an improvement in the pharynx dimension. During active treatment the treated group showed a significant improvement in lower airway size and in lower pharynx dimension. A significant decrease in adenoid size was also found.

Conclusion

The treatment with RME and FM produced favorable stable changes in the airway dimensions in Class III subjects when compared with untreated controls.

Secondhand Smoke exposure and risk of Obstructive Sleep Apnea in Children

10-12-2019 – Rajeev Subramanyam, Ignacio E. Tapia, Bingqing Zhang, Janell L. Mensinger, Annery Garcia-Marcinkiewicz, Denis H. Jablonka, Jorge A Gálvez, Karina Arnez, Robert Schnoll

Journal Article

Objectives

Obstructive sleep apnea (OSA) has a prevalence of 4% in children. Few studies have explored the role of secondhand smoke (SHS) on OSA severity and have shown contradicting results. Most studies have focused on the effect of SHS on snoring. This study explored the association of SHS exposure and OSA severity in children aged 3–18 years.

Methods

This is a retrospective single center IRB-approved study. Electronic Medical Records (EMR) were queried between 1/24/2015 and 1/24/2018 to obtain data on SHS exposure with standard questionnaires from perioperative database. SHS was analyzed as a binary variable and OSA was measured using obstructive apnea hypopnea index (OAHI) from polysomnography (PSG) as a continuous variable. Analyses were done on all children and in those with severe OSA (OAHI≥10/h) as a subgroup.

Results

EMR query yielded 101,884 children of whom 3776 had PSG. Limiting baseline PSG in 3–18-year-old and reliable information on SHS yielded 167 analyzable children of whom 70 had severe OSA. Children exposed to SHS had significantly more public insurance than non-exposed (p < 0.0001). Among children with severe OSA, median OAHI was significantly higher in SHS exposed compared to non-exposed (29.0vs.19.5,p = 0.04), but not across all children. In multivariable analysis SHS exposure increased OAHI by 48% in severe OSA subgroup (95%CI: 8%–102%; p = 0.01) when adjusted for race, body mass index, and adjusted household income.

Conclusion

Children aged 3–18 years with severe OSA who were exposed to SHS were found to have 1.48 increase in odds of OAHI than those without SHS exposure. Results could be limited by retrospective nature of study and EMR tools.

Current evaluation and management of plastic bronchitis in the pediatric population

10-12-2019 – Youjin Li, Ryan J. Williams, Natasha D. Dombrowski, Karen Watters, Kevin P. Daly, Alexandria L. Irace, Gary A. Visner, Reza Rahbar, Francis Fynn-Thompson

Journal Article

Objective

To describe a multidisciplinary approach for the treatment of plastic bronchitis (PB) in children.

Methods

Retrospective chart review of children with PB between 1997 and 2017. Data regarding clinical presentation, diagnosis, management, and outcomes were analyzed.

Results

Of 34 patients presenting with PB, 24 had single ventricle (SV) heart disease, 9 had pulmonary disease, and one had no underlying disease. Median (IQR: interquartile range) age at the time of PB diagnosis was 5.5 years (IQR: 9.0). Presenting symptoms included cough productive of casts (n = 27, 79%), wheezing (n = 5, 15%), dyspnea (n = 18, 53%), hypoxia (n = 31, 91%), and respiratory failure (n = 9, 26%). Diagnosis was made based on clinical evaluation, bronchoscopy findings, and/or pathology of casts. Treatment methods included bronchoscopy for cast removal (25% of SV patients, 91% of non-SV patients), chest physiotherapy (SV: 92%, non-SV: 45%), albuterol (SV: 79%, non-SV: 73%), inhaled steroids (SV: 75%, non-SV: 18%), nebulized hypertonic saline (SV: 29%, non-SV: 9%), nebulized heparin (SV: 8%, non-SV: 55%), nebulized tissue plasminogen activator (t
PA; SV: 33%, non-SV: 9%), inhaled Dornase Alfa (SV: 54%, non-SV: 9%), antibiotics (SV: 46%, non-SV: 45%), systemic steroids (SV: 13%, non-SV: 45%), and lymphatic embolization (SV: 8%, non-SV: 45%). Of SV patients, 11 had no recurrence, 5 underwent heart transplantation, one awaits transplant, and 3 died due to cardiac disease. Three patients with respiratory disease had recurrent PB and one died from MRSA pneumonia.

Conclusion

PB is a highly morbid disease with limited treatment options. Bronchoscopy and chest physiotherapy for airway clearance are among the most-utilized therapies.

Association between vertigo, cognitive and psychiatric conditions in US children: 2012 National Health Interview Survey

07-12-2019 – Robin T. Bigelow, Yevgeniy R. Semenov, Howard J. Hoffman, Yuri Agrawal

Journal Article

Objectives

Small studies have suggested an association between vertigo and psychiatric comorbidity. The purpose of this study is to evaluate the associations between vertigo and cognitive and psychiatric conditions among a large sample of U.
S. children.

Methods

We conducted a cross-sectional analysis of the 2012 National Health Interview Survey (NHIS) Child Balance Supplement administered to parents/caregivers of children aged 3–17 years. Multivariable logistic regression models were used to evaluate the association between vertigo and specific cognitive and psychiatric conditions.

Results

The 1-year prevalence of vertigo was 1.56% in this nationally-representative sample (N = 10,823) of U.
S. children aged 3–17 years. After adjusting for demographic and confounding health variables (otitis media and headaches/migraine), children with vertigo had significantly higher odds of attention deficit disorder (OR = 1.73, 95%CI: 1.06–2.81), learning disability (OR = 3.45, CI: 2.18), developmental delay (OR = 2.59, CI: 1.34–4.98), intellectual disability (OR = 6.60, CI: 2.60–16.79), and are more likely to utilize special education services (OR = 2.46, CI: 1.48–4.10) relative to the rest of U.
S. children. Children with vertigo also had higher odds of having difficulty with emotions, concentration, or behavior (OR = 2.92, CI 1.85–4.61), and having a poor attention span (OR = 1.68, CI: 1.01–2.80).

Conclusions

Vertigo is associated with significantly increased odds of cognitive and psychiatric comorbidity in U.
S. children. These findings support the hypothesis that the vestibular system is important for normal cognitive and psychiatric development in children.

Endoscopic endonasal transsphenoidal approach for pediatric craniopharyngiomas: A case series

08-12-2019 – Juliana Carolina Schelini, Sergio Cavalheiro, Patrícia Alessandra Dastoli, Élcio Roldan Hirai, Camila Atallah, Marcos Costa, Jardel Nicacio, Andrea Maria Capellano, Nasjla Silva, Samuel Zymberg, Rodrigo de Paula Santos

Journal Article

Objectives

This study aims to analyze our series of pediatric patients who underwent craniopharyngioma resection using the endoscopic endonasal transsphenoidal approach (EETA).

Methods

We collected clinical and surgical data from the charts of 20 children who underwent craniopharyngioma removal surgery using the EETA from 2007 to 2017. From the charts, we collected demographic information, results of imaging tests (size and extension of the tumor), and information regarding the surgical procedure and postoperative complications.

Results

From the 20 patients included in this series (12 women and eight men), 17 underwent EETA as a primary procedure, and the remaining three underwent EETA as a secondary procedure due to a relapsing tumor following previous transcranial surgery. The mean age of the patients at the time of the surgical procedure was 7.5 years (range 3–18 years). Regarding their location, 12 tumors were in the sellar and suprasellar regions, three extended into the third ventricle, and five were exclusively intrasellar. We achieved a gross total resection (GTR) of the tumor in 14 patients (70%), subtotal in five (25%), and partial in one (5%). One patient (5%) developed a cerebrospinal fluid fistula after the surgical procedure. In the postoperative follow-up period (mean time = 5.3 years; range = 2–9 years), 11 (55%) patients developed panhypopituitarism, and a relapsing tumor was later found in three (15%) patients. Regarding visual impairment, four patients had visual abnormalities preoperatively (amaurosis, n = 2; bilateral visual acuity decrease, n = 1; bilateral visual field defect, n = 1), and those did not improve or worsened postoperatively. None of the patients who did not have vision problems before the surgery developed those postoperatively.

Conclusion

Our results showed that the EETA is a safe and effective approach for removing craniopharyngiomas in children, as it associated with low operative morbidity and complication rates. Also, our data demonstrated that the EETA may be performed regardless of the size of the nasal cavity, pneumatization of the sphenoid sinuses, and location or extension of the tumors.

Usefulness of cochlear implantation in children with single sided deafness

07-12-2019 – Désirée Ehrmann-Mueller, Anja Kurz, Heike Kuehn, Kristen Rak, Robert Mlynski, Rudolf Hagen, Wafaa Shehata-Dieler

Journal Article

Objectives

Children with single sided deafness (SSD) show a poorer performance at school, which is attributable to reduced speech discrimination in noise, to reduced localization ability, and to a decreased power of concentration due to faster hearing exhaustion. Therefore, it is important to provide children with SSD with adequate hearing amplification to restore binaural hearing. This can only be achieved by provision with a cochlear implant (CI). But these treatment option in children with SSD is still under discussion.

The aim of the present study is to evaluate audiological and clinical results in children with SSD following cochlear implantation. A special focus was placed on the duration of deafness before implantation and on the frequency of CI-use in everyday life.

Methods

Seven children with SSD of different etiologies who were provided with a CI between 3 and 16 years of age were evaluated. Every child underwent multiple audiological tests before and after cochlear implantation. After cochlear implantation speech recognition tests in noise using the HSM (Hochmair, Schulz and Moser 1997) test and localization tests were performed. Furthermore, the frequency of implant use was evaluated.

Results

Speech recognition in noise with CI compared to the unaided condition significantly improved in all children in different settings. Improvement of the localization ability measured by the root mean square error (RMSE) was shown in all children. All children are very satisfied with the decision to have undergone cochlear implantation and are all full-time users.

Conclusions

Cochlear implantation benefits speech recognition in noise and sound localization ability in children with SSD at different ages. All implanted children are full-time users regardless of age or duration of deafness before implantation.

The clinical and genetic research of Waardenburg syndrome type I and II in Chinese families

08-12-2019 – Qin Liu, Jing Cheng, Yu Lu, Jia Zhou, Li Wang, Changliang Yang, Guang Yang, Hui Yang, Jingyuan Cao, Zhao Zhang, Yi Sun

Journal Article

Objective

Waardenburg Syndrome (WS) is a neurocristopathy with an autosomal dominant mode of inheritance and highly genetic heterogeneity. To date, mutations of PAX3, SOX10, MITF, EDNRB, EDN3 and SNAI2 have been implicated in the pathogenesis of WS. In this study, we aimed to identify pathogenic genes among WS families and to analyze the pathogenic relationship between genotypes and phenotypes.

Methods

In this study, all six families studied were from Hubei province, China.
WS patients underwent screening for all deafness genes including PAX3, SOX10, MITF, EDNRB, EDN3 and SNAI2 using Massively Parallel Sequencing (MPS) and validation of mutations using Sanger sequencing.

Results

Clinical evaluation revealed prominent phenotypic variability in Hubei WS patients. Two WS1 families and four WS2 families were diagnosed in six families. Sensorineural hearing loss was the most common, followed by iris pigmentary abnormality. Molecular genetic analysis of the WS genes for six families revealed five novel heterozygous mutations. Two mutations occurred in the PAX3 gene: one nonsense mutation c.667C > T(p.
Arg223Ter) and one missense mutation c.220C > T(p.
Arg74Cys).
One missense mutation c.331T > C (p.
Phe111Leu) and one nonsense mutation c.346C > T(p.
Gln116Ter) were detected in the SOX10 gene. Two mutations were detected in the MITF gene: one splice site mutation c.859-1G > A and one nonsense mutation c.859G > T(p.
Glu287Ter). Among them, the mutations (SOX10 c.331T > C and MITF c.859G > T) were de novo mutations.

Conclusion

In this study, six mutations were found to be associated with the phenotype of patients. Our data helped illuminate the phenotypic and genotypic spectrum of WS in Hubei province and could have implications for the genetic counseling of WS in Hubei province.

Trend and patterns in the antibiotics prescription for the acute otitis media in Korean children

06-12-2019 – Shin Hye Kim, Jeong-Rok Kim, Jae-Jun Song, Sung-Won Chae

Journal Article

Background

The Korean Otologic Society developed guidelines for treatment of acute otitis media (AOM) in 2010, which advocated limiting the prescription of antibiotics. However, it is not known whether this has influenced the antibiotic prescription rate. Thus, this study aimed to examine the impact of these guidelines on the patterns in antibiotic prescription for AOM in Korean children.

Methods

AOM patients aged less than 15 years, included in the Korean Health Insurance Review and Assessment Service database from 2009 to 2017, were retrospectively identified. We estimated the overall antibiotic prescription rate, antibiotic prescription rates for suppurative and non-suppurative AOM, and types of antibiotics prescribed. Moreover, we analyzed differences in antibiotic prescription rates according to age group, hospital type, and medical department.

Results

The overall antibiotic prescription rate decreased significantly from 2009 to 2017. The antibiotic prescription rate for non-suppurative AOM decreased much more than the rate for suppurative AOM. Overall, amoxicillin/clavulanate was the most commonly prescribed antibiotic, followed by cephalosporin. The antibiotic prescription rate decreased by a similar margin in all age groups. Tertiary referral hospitals and otorhinolaryngology department showed the lowest antibiotic prescription rate every year.

Conclusions

This study demonstrated the changes in antibiotic prescription rates for AOM following implementation of the Korean AOM treatment guidelines. The practice of antibiotic prescription for children with AOM appears to have decreased every year. However, the overall antibiotic prescription rate is still high. Therefore, clinicians should understand and adhere to the guidelines, and merely observe children with mild AOM symptoms.

Hot nasal packing with hot saline irrigation for hemostasis after adenoidectomy: A prospective randomized controlled study

07-12-2019 – Seyit Mehmet Ceylan, İlyas Dişikırık, Mahmut Alper Kanmaz, Altan Yıldırım, Efe Sezgin

Journal Article

Objective

This study aimed to investigate the efficacy of hot posterior nasal packing and hot saline irrigation in bleeding control after adenoidectomy.

Methods

A total of 130 patients scheduled for adenoidectomy were included in the study, and randomized into two groups at the beginning of the surgical operation. After adenoidectomy, saline impregnated tampon, and saline irrigation at room temperature (22 °C) was applied to the patients in one group while 50 °C saline impregnated tampon and saline irrigation at the same temperature were applied to the patients in the other group. We recorded hemostasis for up to 3 min after tamponade for bleeding control, and the amount of bleeding into the nasopharynx.

Results

The age of the patients ranged from 1.5 to 13 years (mean ± SD: 6.07 ± 3.08 years, and 5.33 ± 2.55 years, 22 °C and 50 °C saline irrigation groups, respectively). There were 37 males and 28 females in the 22 °C saline group, while 34 males and 31 females in the 50 °C saline group. When comparing the two groups, there was no statistically significant difference in terms of duration of hemostasis (p = 0.64). However, bleeding scores at 2 nd min after the tamponade were significantly lower in the 50 °C saline group (p = 0.007). The amount of bleeding in the 50 °C saline group was also significantly lower than the 22 °C saline group (p = 0.015).

Conclusion

In this study, application of 50 °C saline impregnated tampon, and hot saline irrigation was found to be more effective in the control of bleeding after adenoidectomy by reducing the amount of bleeding compared to 22 °C saline impregnated tampon application and saline irrigation at 22 °C. However, hot nasal packing and hot saline irrigation did not affect duration of hemostasis and cauterization.

Efficacy of crenotherapy by Politzer in the treatment of otitis media with effusion in children with down syndrome

04-12-2019 – Massimo Mesolella, Brigida Iorio, Filippo Ricciardiello, Gaetano Motta

Journal Article

Safety of nasal stenting in pharyngeal flap surgery for pediatric velopharyngeal dysfunction

04-12-2019 – R. Araslanova, R. Instrum, A. Dzioba, M. Husein

Journal Article

Introduction

The pharyngeal flap procedure is an effective surgery for velopharyngeal dysfunction (VPD) yet carries approximately a 3.2% post-operative airway obstruction risk. Life threatening airway compromise occurs in first 24 h post-operatively. Nasopharyngeal airway has been shown to decrease these complications but its routine use is not commonplace. At our centre, surgical technique involves routine placement of bilateral nasopharyngeal airway referred to as nasal stenting. Our objective was to demonstrate safety of nasal stenting for children with VPD undergoing pharyngeal flap surgery.

Methods

A retrospective review of pediatric patients aged 1 through 18 years at the time of VPD diagnosis, who underwent superiorly based pharyngeal flap surgery at London Health Sciences Centre (LHSC), was conducted. Patients were admitted to a regular ward with bihourly oxygen saturation checks. Nasal stents were removed on post-operative day two. Demographic data along with patient outcomes and perioperative complications were collected and analyzed.

Results

Eighty-five pediatric patients underwent superiorly based pharyngeal flap surgery at LHSC from November 2004 through February 2017 that met inclusion criteria. Mean age at the time of surgery was 11.0 years, whereas average age at diagnosis was 8.7 years. The majority of patients (60.0%) had history of cleft palate repair. Only 28.2% had additional comorbidities, the most common being Pierre-Robin Sequence. Average length of hospital stay was 2.7 days. No patients required admission to an intensive care unit. Surgical complication rate was 8.2%. No post-operative airway obstruction events were encountered. Two patients (2.3%) underwent pharyngeal flap reversal for obstructive sleep apnea and one for persistent nasal obstruction. Five (5.9%) minor stent-related complications were captured. Two patients developed self-limiting epistaxis. Two patients had partially extruded stents prior to their removal. One patient developed nasopharyngeal port granuloma which resolved with a course of intranasal corticosteroids.

Conclusion

Nasal stenting for pharyngeal flap surgery is safe. Stent related complications were minor and did not influence length of hospital stay. A prospective study to determine if routine stenting would lead to decreased serious airway complications is warranted.

Factors affecting persistent tympanic membrane perforation after tympanostomy tube removal in children

02-12-2019 – Clarice Brown, Philomena Behar

Journal Article

Objectives

Evaluate and compare the rates of persistent tympanic membrane (TM) perforations between short-term vs long-term tympanostomy tubes. In addition, to determine which demographic, anatomical, and physical factors affect ear drum healing after tympanostomy tube (TT) removal and simultaneous paper patch myringoplasty (PPM) in children.

Study design

Retrospective chart review.

Methods

Charts were reviewed from the Women and Childrens of Buffalo hospital and our pediatric otolaryngology practice electronic medical record. Data was retrieved from patients less than 18 years old who underwent surgical removal of a TT and concomitant PPM between January 2005 and January 2017.

Results

343 ear drums were studied that underwent tympanostomy tube removal and paper patch myringoplasty. 45/343 (13%) of ears had a persistent perforation after PPM. The rate of persistent perforation with short-term tubes and long-term tubes was significantly different (6.6% and 20% respectively). Patient characteristics significantly associated with persistent perforations were: age at time of tube removal and number of tubes that patient received. Ear drum characteristics that significantly impacted persistent perforation included: size of ear drum perforation, and presence of tympanosclerosis. Length of intubation, ear drum atrophy, retraction, granulation tissue, middle ear effusion and thickened ear drum were not found to be significant factors.

Conclusion

Overall, we found an 87% perforation closure rate after surgical removal of the TT and PPM. Persistent perforations occurred significantly more often in patients with long-term tubes than short term tubes. Our data also suggests that several patient and ear drum characteristics may be important factors that contribute to persistent perforation after tympanostomy tube placement and removal.

Pediatric adenoidectomy in the very young child and indications for postoperative inpatient admission

04-12-2019 – Stephen R. Chorney, Julia F. Dailey, Karen B. Zur

Journal Article

Objectives

To determine the rate of significant respiratory events following adenoidectomy in young patients and to identify factors that would prompt inpatient admission postoperatively.

Methods

A retrospective chart review was performed of consecutive adenoidectomy surgeries at a high-volume, tertiary-care childrens hospital between 2016 and 2018. Children under 3.5 years of age who had surgery for obstructive symptoms were included. Patients were grouped by age (youngest ≤1.5 years, middle 1.6–2.5 years, and oldest 2.6–3.5 years). We excluded patients having revision surgery, a concomitant tonsillectomy, or additional major surgical procedure.

Results

There were 353 patients that met inclusion criteria. The three age groups were similar with respect to all characteristics except age (p < .001), body mass index (p < .001), and percentage of Black or African American children (p = .02). Patients under 1.5 years more often had preoperative polysomnography (p = .02) with a lower oxygen saturation nadir (p = .04), and were more likely to have surgery for obstructive sleep apnea (p < .001). No differences were found between age groups with respect to recovery room issues, nurse triage calls, or readmissions within 30 days of surgery. An elective admission rate in the cohort was 35.1%, and this was age-group dependent with 79.5% of the youngest group being admitted (p < .001). On admission, 16.9% of all patients had admission events requiring positive pressure support, intensive care unit admission, or prolonged hospitalization, which was similar across all age groups (p = .67). Events were more common in younger patients (17 mos. vs 20 mos., p = .07), those with more comorbidities (74.8% vs 51.5%, p = .06) and significantly higher in those with severe preoperative polysomnogram variables (p < .001). Based on multivariate regression analysis, younger children (OR: 13.7, 95% CI: 6.5 – 29.0, p < .001) or children with an AHI over 5 events/hr (OR: 32.3, 95% CI: 3.4 – 303.2, p = .005) were more likely to have significant events on admission.

Conclusions

Significant respiratory events are uncommon after adenoidectomy for obstructive symptoms, even in very young children. However, for children under 1.5 years of age or those with AHI scores above 5 events/hr, postoperative admission for monitoring is recommended. Clinical judgement should be used when considering outpatient surgery for older children or those with comorbidities.

National questionnaire on skills and techniques in pediatric tracheotomy surgery in Turkey

01-12-2019 – Murat Gumussoy, Ibrahim Cukurova, Sinan Atmaca

Journal Article

Objective

The aim of the study was to determine the differences in surgical preferences of ENT Surgeons in Turkey with regard to pediatric tracheotomy.

Design

Questionnaire study.

Participants

ENT Surgeons.

Materials and methods

The national multiple-choice questionnaire study included a total of 16 questions about physicians technical preferences, different methods and complications in pediatric tracheotomy surgery. It was planned and implemented with the support of a professional survey company (www.surveymonkey.com).

Results

The questionnaire was answered by 591 ENT Surgeons; the percentage of the physicians performing pediatric tracheotomies in the previous year was 59.6%. Forty point four percent (40.4%) of the physicians had not performed tracheotomies in the pediatric age group and 57.9% had not performed tracheotomies in patients under one year old. Seventy point six percent (70.6%) of the physicians who had performed tracheotomies had made vertical skin incisions, 69.5% of them had removed subcutaneous adipose tissue, 81.4% of them had retraction the thyroid isthmus area from their field of view; 83.9% of them had made a vertical incision to the trachea, 82.5% of them had applied a stay suture to the trachea and 4.7% of them had used additional techniques for stoma maturation. The intraoperative mortality, early complication, late complication, and total complication rates were 3.9%, 32.7%, 21.2% and 53.9 respectively.

Conclusions

This is the first large-scale questionnaire study with data on pediatric tracheotomy techniques and the practices of ENT Surgeons at a national level. Common approaches were observed among the.

Cochlear implant outcomes in children with attention-deficit/hyperactivity disorder: Comparison with controls

01-12-2019 – Shaghayegh Omidvar, Zahra Jeddi, Afsaneh Doosti, Seyed Basir Hashemi

Journal Article

Objectives

The coincidence of attention-deficit/hyperactivity disorder (ADHD) and hearing loss in the children has adverse effects in speech, cognition, communication and motor development. This can influence cochlear implant (CI) outcomes negatively. The aim of this study was to compare auditory, language, speech, cognition, communication and motor outcomes between CI children with versus without ADHD.

Methods

Nineteen CI children with ADHD and twenty-three age and gender matched children without ADHD at the Shiraz CI center ranging in age from 37 to 60 months were participated in this cross-sectional study. The developmental quotient in auditory, receptive and expressive language, speech and cognition skills was evaluated through Newsha Developmental Scale. The Persian version of the Ages and Stages Questionnaire (ASQ) was used to assess childrens developmental status in fine and gross movements, communication, problem solving, and personal-social domains. A comparison of the results between two groups was made by the Mann-Whitney test.

Results

CI children with ADHD had significantly lower Newsha developmental quotients in cases of auditory, receptive and expressive language, speech and cognition skills compared to the control group (P = 0.027 to <0.001). A significant difference was observed between children with and without ADHD in fine and gross movements, communication, problem solving, and personal-social domains of ASQ at 60 months (P = 0.029 to 0.003).

Conclusion

Children with ADHD showed decreased ability in auditory, language, speech, cognition, motor and communication skills following CI compared to children without ADHD. It can guide clinician to provide these children with more specific rehabilitation program to improve their skills.

The immunology of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; what can the tonsils reveal. A literature review

02-12-2019 – Jostein Førsvoll, Einar Klæboe Kristoffersen, Knut Øymar

Journal Article, Review

Objectives

Tonsillectomy (TE) or adenotonsillectomy (ATE) may have a beneficial effect on the clinical course in children with the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. However, an immunological reason for this effect remains unknown. This literature review summarizes the current knowledge regarding the immunological role of the tonsils in the PFAPA syndrome.

Methods

We searched PubMed, Medline, EMBASE and Cochrane for papers written in English dated from 1 January 1987 to 30 April 2019. The search included all studies reporting histological, immunological or microbiological workup of tonsil specimens from children aged 0–18 years with PFAPA.

Results

Thirteen articles reported histological, immunological or microbiological workup of tonsil specimens in children with PFAPA. The histology of tonsil specimens from children with PFAPA displayed chronic tonsillar inflammation with lymphoid hyperplasia. No uniform immunological pattern was identified, but some studies found fewer B-lymphocytes and smaller germinal centers in PFAPA compared to controls. A difference in tonsillar microbiota between PFAPA and controls was found in one study.

Conclusion

A uniform immunological or microbiological pattern explaining the clinical effect of TE in children with PFAPA has not been revealed. Future targeted immunological studies of tonsils in PFAPA patients could possibly illuminate the understanding of the immunology in this disease.

Panel 1: Biotechnology, biomedical engineering and new models of otitis media

07-01-2020 – Marie Gisselsson-Solén, Paula A. Tähtinen, Allen F. Ryan, Apoorva Mulay, Shin Kariya, Anne G.M. Schilder, Tulio A. Valdez, Steve Brown, Ryan M. Nolan, Ann Hermansson, Gijs van Ingen, Tal Marom

Journal Article

Objective

To summarize recently published key articles on the topics of biomedical engineering, biotechnology and new models in relation to otitis media (OM).

Data sources

Electronic databases: PubMed, Ovid Medline, Cochrane Library and Clinical Evidence (BMJ Publishing).

Review methods

Articles on biomedical engineering, biotechnology, material science, mechanical and animal models in OM published between May 2015 and May 2019 were identified and subjected to review. A total of 132 articles were ultimately included.

Results

New imaging technologies for the tympanic membrane (TM) and the middle ear cavity are being developed to assess TM thickness, identify biofilms and differentiate types of middle ear effusions. Artificial intelligence (AI) has been applied to train software programs to diagnose OM with a high degree of certainty. Genetically modified mice models for OM have further investigated what predisposes some individuals to OM and consequent hearing loss. New vaccine candidates protecting against major otopathogens are being explored and developed, especially combined vaccines, targeting more than one pathogen. Transcutaneous vaccination against non-typeable Haemophilus influenzae has been successfully tried in a chinchilla model. In terms of treatment, novel technologies for trans-tympanic drug delivery are entering the clinical domain. Various growth factors and grafting materials aimed at improving healing of TM perforations show promising results in animal models.

Conclusion

New technologies and AI applications to improve the diagnosis of OM have shown promise in pre-clinical models and are gradually entering the clinical domain. So are novel vaccines and drug delivery approaches that may allow local treatment of OM. IMPLICATIONS FOR PRACTICE: New diagnostic methods, potential vaccine candidates and the novel trans-tympanic drug delivery show promising results, but are not yet adapted to clinical use.

Panel 5: Impact of otitis media on quality of life and development

31-12-2019 – Preben Homøe, Christian H. Heidemann, Roger AMJ. Damoiseaux, Susen Lailach, Judith E.C. Lieu, John S. Phillips, Roderick P. Venekamp

Journal Article

Objective

To summarize recent advances in knowledge on otitis media (OM) and quality of life (QoL) and development by synthesizing relevant research in this field published between June 1., 2015 until June 1., 2019.

Data sources

Systematic searches of PubMed, Embase and the Cochrane Library using predefined database-specific syntaxes.

Review methods

Articles selected were randomized controlled trials and observational studies with an adequate control group estimating treatment effects of OM including acute OM (AOM), recurrent AOM (RAOM), OM with effusion (OME), chronic OM (COM) and chronic suppurative OM (CSOM). Items included were Health Status, Health Status Indicators, Quality of Life, Functional Status, Specific Learning Disorder, Developmental Disabilities, Language Development Disorders, and Problem Behavior.

Results

The electronic database searches yielded a total of 699 records. After screening titles and abstracts, we identified 34 potentially eligible articles. Of these, 18 were excluded. This left 15 articles suitable for inclusion.

Conclusions

Although evidence is accumulating that OM may significantly impair childrens QoL and development as well as caregivers QoL, studies on this topic are relatively scarce and vary substantially in terms of methodological quality and outcome measurement instruments (OMI) used. In this review, studies have used 10 different OMIs capturing a wide range of OM symptoms as well as generic and disease-specific QoL outcomes. OM was associated with negative effects on auditory processing, language and speech development, school readiness, social competence, psychosocial wellbeing, and sleep. We found only four relevant randomized controlled trials, which mostly failed to demonstrate superiority of interventions in terms of QoL improvement and reports on reversibility are lacking. This underpins the urgent need for high quality studies in this field using validated and uniform OMIs. To facilitate interpretation and harmonization of study findings, we suggest and support the development of a core outcome set for the various OM entities that should include the most reliable and meaningful QoL and developmental OMIs.

Chapter 7 – Pathogenesis of otitis media – A review of the literature between 2015 and 2019

28-12-2019 – R.B. Thornton, A. Hakansson, D.W. Hood, J. Nokso-Koivisto, D. Preciado, K. Riesbeck, P.C. Richmond, Y.C. Su, W.E. Swords, K.L. Brockman

Journal Article

Objective

To perform a comprehensive review of the literature from July 2015 to June 2019 on the pathogenesis of otitis media. Bacteria, viruses and the role of the microbiome as well as the host response are discussed. Directions for future research are also suggested.

Data sources

PubMed database of the National Library of Medicine.

Review methods

PubMed was searched for any papers pertaining to OM pathogenesis between July 2015 and June 2019. If in English, abstracts were assessed individually for their relevance and included in the report. Members of the panel drafted the report based on these searches and on new data presented at the 20th International Symposium on Recent Advances in Otitis Media.

Conclusions

The main themes that arose in OM pathogenesis were around the need for symptomatic viral infections to develop disease. Different populations potentially having different mechanisms of pathogenesis. Novel bacterial otopathogens are emerging and need to be monitored. Animal models need to continue to be developed and used to understand disease pathogenesis.

Implications for Practice

The findings in the pathogenesis panel have several implications for both research and clinical practice. The most urgent areas appear to be to continue monitoring the emergence of novel otopathogens, and the need to develop prevention and preventative therapies that do not rely on antibiotics and protect against the development of the initial OM episode.

Panel 2- recent advance in otitis media bioinformatics

04-01-2020 – Diego Preciado, Jian-Dong Li, Kensei Komatsu, Arwa Kurabi, Gustavo Nino, Stephanie Val, Shyan Vijayasekaran, Oren Ziv, Ann Hermansson

Journal Article

Objectives

To update the medical literature on recent large-scale studies employing bioinformatics data analysis tools in otitis media (OM) disease models with a principal focus on developments in the past 5 years.

Data sources

Pubmed indexed peer-reviewed articles.

Review methods

Comprehensive review of the literature using the following search terms: ‘genomics, inflammasome, micro
RNA, proteomics, transcriptome, bioinformatics’ with the term ‘otitis media’, and ‘middle ear’. Included articles published in the English language from January 1, 2015–April 1, 2019.

Implications for practice

Large scale bioinformatics tools over the past five years lend credence to the paradigm of innate immune response playing a critical role in host defense against bacteria contributing to Otitis Media (OM) progression from acute to chronic. In total, genomic, mi
RNAomic, and proteomic analyses all point to the need for a tightly regulated innate immune and inflammatory response in the middle ear. Currently, there is an urgent need for developing novel therapeutic strategies to control immunopathology and tissue damage, improve hearing and enhance host defense for both acute and chronic OM based on full understanding of the basic molecular pathogenesis of OM.

Recent advances in understanding the natural history of the otitis media microbiome and its response to environmental pressures

28-12-2019 – Robyn L. Marsh, Celestine Aho, Jemima Beissbarth, Seweryn Bialasiewicz, Michael Binks, Anders Cervin, Lea-Ann S. Kirkham, Katherine P. Lemon, Mary P.E. Slack, Heidi C. Smith-Vaughan

Journal Article

Objective

To perform a comprehensive review of otitis media microbiome literature published between 1st July 2015 and 30th June 2019.

Data sources

PubMed database, National Library of Medicine.

Review methods

Key topics were assigned to each panel member for detailed review. Draft reviews were collated and circulated for discussion when the panel met at the 20th International Symposium on Recent Advances in Otitis Media in June 2019. The final draft was prepared with input from all panel members.

Conclusions

Much has been learned about the different types of bacteria (including commensals) present in the upper respiratory microbiome, but little is known about the virome and mycobiome. A small number of studies have investigated the middle ear microbiome; however, current data are often limited by small sample sizes and methodological heterogeneity between studies. Furthermore, limited reporting of sample collection methods mean that it is often difficult to determine whether bacteria detected in middle ear fluid specimens originated from the middle ear or the external auditory canal. Recent in vitro studies suggest that bacterial interactions in the nasal/nasopharyngeal microbiome may affect otitis media pathogenesis by modifying otopathogen behaviours. Impacts of environmental pressures (e.g. smoke, nutrition) and clinical interventions (e.g. vaccination, antibiotics) on the upper respiratory and middle ear microbiomes remain poorly understood as there are few data.

Implications for practice

Advances in understanding bacterial dynamics in the upper airway microbiome are driving development of microbiota-modifying therapies to prevent or treat disease (e.g. probiotics). Further advances in otitis media microbiomics will likely require technological improvements that overcome the current limitations of OMICs technologies when applied to low volume and low biomass specimens that potentially contain high numbers of host cells. Improved laboratory models are needed to elucidate mechanistic interactions among the upper respiratory and middle ear microbiomes. Minimum reporting standards are critically needed to improve inter-study comparisons and enable future meta-analyses.

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome: A debate about diagnosis and treatment in children continues

24-12-2019 – Donato Rigante, Luigi Corina

Editorial