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OBJECTIVE. This project aims to prospectively and objectively assess otolaryngological manifestations and quality of life of children with primary ciliary dyskinesia (PCD) and compare these findings with healthy pediatric controls. STUDY DESIGN. Cross-sectional. SETTING. Two high-volume pediatric PCD specialty centers. METHODS. Standardized clinical assessment; Sino-Nasal Outcome Test 22 (SNOT-22); Hearing Environment and Reflection Quality of Life (HEAR-QL); Reflux Symptom Index (RSI); standardized physical examination of the sinonasal, laryngeal, and otological systems; and investigations including pure-tone audiograms (PTAs) and sinonasal cultures were collected. RESULTS. Forty-seven children with PCD and 25 control participants were recruited. Children with PCD had more upper airway symptoms than healthy children. They had significantly higher scores in both SNOT-22 and RSI, indicating worse sinonasal and reflux symptoms, with worse quality of life on the HEAR-QL index compared to healthy children (P < .05). Fifty-two percent of children with PCD-related hearing loss were not aware of their hearing deficit that was present on audiological assessment, and only 23% of children who had ventilation tubes had chronic otorrhea, most of which was easily controlled with ototopic drops. Furthermore, although all children with PCD had chronic rhinosinusitis, only 36% of them were using topical nasal treatment. The most common bacteria cultured from the middle meatus were Staphylococcus aureus in 11 of 47 (23%), followed by Streptococcus pneumoniae in 10 of 47 (21%). CONCLUSION. This multisite cohort highlights the importance of otolaryngology involvement in the management of children with PCD. More rigorous otolaryngological management may lead to reductions in overall morbidity and improve quality of life for children with PCD.

A wide variety of pathologies can affect the palatine tonsils. Ultrasound is a commonly used modality for assessing head and neck masses in children; however, its use in tonsillar evaluation has not been widely explored. The objective of this study was to measure 3-dimensional tonsillar size with ultrasound, in centimeters, and correlate these measurements with actual ex vivo dimensions on pathology specimens.We performed a prospective cohort study.The study was set in a tertiary care children's hospital.Children undergoing tonsillectomy were included in the study. Transcervical high-frequency ultrasonography (HFU) was performed prior to surgery to obtain 3-dimensional measurements of the right and left palatine tonsils. Mean sizes were compared to ex vivo tonsil measurements and correlations were obtained.Seventy-five consecutive children underwent a transcervical HFU, with a total of 150 tonsils analyzed. The mean differences between HFU and pathology measurements were -0.08 cm and -0.24 cm for the right and left craniocaudal axes, -0.19 cm and -0.18 cm for the right and left mediolateral axes, and 0.05 cm and 0.03 cm for the right and left anteroposterior axes. Correlation coefficients between ultrasound and pathology measurements were all above 0.5.HFU can accurately measure the size of pediatric tonsils in 3 dimensions.

The present study reviewed all cases reported of isolated Langerhans cell histiocytosis bone lesions in the head and neck of pediatric patients. The objective was to create a treatment algorithm to facilitate the diagnosis and management of these lesions.Eligible articles were identified through a comprehensive search of the following electronic databases: PubMed, Ovid Medline, Embase, and Cochrane Library.Two authors independently reviewed the titles and abstracts retrieved by the electronic search concordant with the criteria for study eligibility. The lists of articles from each author were jointly reviewed and a common list created. All relevant articles were reviewed in hard copies and as full texts to justify inclusion.A total of 201 patients (67% male, mean age: 8.1 ± 4.3 years) were included from 45 studies. Clinical presentations included swelling (64%), pain (9%), or both (18%). The skull (61%) and the orbit (24%) were the most common location for isolated bone lesions in the head and neck. The most frequently documented management option was resection, followed by observation, chemotherapy, and intralesional steroid injection.Due to its rarity and variability in presentation and severity, treatment of these lesions has yet to be standardized. Based on the review, a diagnosis and treatment algorithm was created for head and neck surgeons when encountering these types of lesions.

Patients undergoing radiotherapy (RT) often present with serious bacterial infections requiring the use of antibiotic treatment. Gentamicin is a commonly used aminoglycoside antibiotic, whose ototoxicity remains a major problem in clinical use. The objective of this study was to determine whether radiation exposure can influence gentamicin-induced ototoxicity.Prospective animal study.Animal care facilities of the Montreal Children's Hospital Research Institute.Sixteen guinea pigs received low-dose RT unilaterally for 4 weeks (total: 48 Gy). Animals then received low or high doses of gentamicin (40 mg/kg/d and 80 mg/kg/d) for 10 days. The ears were divided into 4 groups: gentamicin 40 mg, gentamicin 80 mg, gentamicin 40 mg + RT, and gentamicin 80 + RT. Auditory brainstem responses and distortion products otoacoustic emissions were assessed at baseline and before and after gentamicin treatment. Cochlear morphology using light and scanning electron microscopy were evaluated.High-dose gentamicin caused significant auditory brainstem response threshold shifts (P = .020), with greater hearing loss in the irradiated ear (difference of 23.6 + 7.5 dB). All animals exposed to high-dose gentamicin had head tilts toward the radiated side. Cochlear morphology revealed the greatest hair cell damage in the gentamicin 80 + RT group followed by gentamicin 80.Results suggest that radiation can exacerbate the ototoxicity of gentamicin at high doses.

Acute mastoiditis is an uncommon but challenging condition when it occurs in children with cochlear implant. The literature is scarce as to the management of this condition with regards to explantation. The objective of the study is to determine the need for explantation in patients with cochlear implants who suffer from acute mastoiditis.Online medical databases-PubMed, Ovid Medline, Ovid Medline in process, Embase, Cochrane Library, CINAHL, Biosis, Google Scholar, and Scopus.A systematic review of all publications addressing the treatment of mastoiditis in cochlear implant children prior to November 2013 was conducted. Data were collected from online medical databases-PubMed, Ovid Medline, Ovid Medline in process, Embase, Cochrane Library, CINAHL, Biosis, Google Scholar, and Scopus. The review was performed in 3 phases; an initial screening review of abstracts was performed, followed by a detailed review of full articles based on inclusion and exclusion criteria, and lastly a final review to extract data from selected articles.Twelve articles were found eligible for this systematic review including a total of 43 patients. Subperiosteal abscess was present in 14.3%. All patients received intravenous antibiotics as an initial treatment, and if needed, surgical intervention was performed. Only 1 patient required explantation (2.3%).Prompt, aggressive medical and if needed surgical therapy can help in saving the implant and result in a favorable outcome.

There is currently no treatment available to prevent radiation-induced sensorineural hearing loss. Metformin has antineoplastic effects and is able to regulate the mitochondrial production of reactive oxygen species after cellular stress, which is one of the mechanisms involved in apoptosis after radiation damage. The objective of this study was to determine the safety and radioprotective properties of metformin against radiation-induced cochlear damage both in vitro and in vivo.In vitro and prospective animal study.Animal Care Facilities of the Montreal Children's Hospital Research Institute.Cultured auditory hair cells (HEI-OC1) were exposed to different concentrations of metformin to determine its safety. Cells were incubated with different metformin concentrations and subjected to radiation. Cell viability after experiments was determined with the 3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium (MTS) assay. Sixteen guinea pigs were divided in 2 groups: drinking tap water (n = 8) and drinking water containing metformin (n = 8). The animals were unilaterally irradiated for 20 days (total dose 70 Gy), and the ears were divided in 4 groups: control (n = 8), irradiated (n = 8), metformin (n = 8), and experimental (n = 8). Auditory brainstem responses were assessed before and 1, 6, and 16 weeks after completion of radiotherapy.Metformin was not cytotoxic or radioprotective in cultured auditory hair cells. Experimental ears had less hearing loss than radiated ones; however, differences were not statistically significant (P.05).Metformin is not ototoxic or radioprotective in vitro or in vivo. Ears solely subjected to metformin had better hearing thresholds than the rest of the groups.

Objective To determine the ototoxic potential of ciprofloxacin/dexamethasone eardrops. Methods Ventilation tubes were inserted in the ears of 15 chinchillas. One ear was randomized to receive four drops of ciprofloxacin/dexamethasone. A control solution (0.45% NaCl) was applied in the contralateral ear. The eardrops were delivered twice daily for 7 consecutive days. Auditory brain stem response (ABR) measurements were recorded at baseline (post–tympanostomy tube insertion), and on days 4, 8, 15, 22, and 60. Results An initial transient increase of 8.11 dB nHL was noted in the mean thresholds of the experimental ears at day 4. On the last ABR evaluation (day 60), the mean thresholds were 16.33 ± 6.93 dB nHL in the experimental ears and 13.66 ± 6.65 dB nHL in the control ears. The difference in the mean thresholds between the experimental and control ears at day 60 was not significant (95% confidence interval, 3.12, −8.02). Scanning electron microscopy demonstrated normal morphology in all experimental ears that were assessed. Conclusion Ciprofloxacin/dexamethasone eardrops did not appear ototoxic when used in chinchillas post–ventilation tube insertion.

Some clinicians use hydrogen peroxide (H2O2) to clear the lumen of ventilation tubes that become blocked postoperatively. The ototoxicity associated with H2O2 has been controversial.We designed an experiment to test if H2O2 damages the cochlear hair cells using a Chinchilla laniger animal model.Nine chinchillas (18 ears) were included in this study. Each animal was used as its own control. Following the insertion of ventilation tubes in both ears and baseline recordings of the auditory brain stem responses (ABR), we instilled 2 ml of 3 percent H2O2 into their right external auditory canals (experimental ears). H2O2 was left in the external auditory canal for a total of 5 minutes and then was drained. We instilled a normal saline control solution in their left ears (control ears) in a similar fashion. ABR recordings were performed 1 day after the last instillation of H2O2 and 5 days later.There was no statistically significant difference in the ABR thresholds of the experimental and control ears.H2O2 did not appear to cause ototoxicity in chinchilla ears with tympanostomy tubes exposed to H2O2 instillation using a standard clinical protocol.

Objectives:Determine whether wideband reflectance (WBR) measurements in babies who were born by vaginal delivery are different from those of babies born by C-section. Our working hypothesis is that babies delivered by Caesarian section have higher reflectance scores on WBR, as they have more fluid and debris in the outer and middle ear. This can have an impact on universal hearing screening passing rates.Methods:Following standard newborn hearing screening, WBR was measured in 225 healthy babies. Sixty-six babies were delivered by Caesarean section compared to 159 babies delivered vaginally. A total of 123 ears were included in the C-section group and were compared to a total of 288 ears in the second group. The results were obtained for 60 frequencies.Results:The mean screening age for babies in the C-section delivery group was 27.5 hours compared to 24.3 hours for babies in the vaginal delivery group. For both groups reflectance was high between 0.24 to 0.79 kHz and 2.50 to 4.80 kHz, with the highest at...

Objectives:There is controversy as to whether eye color has an effect on acquired sensorineural hearing loss. The objectives of this review were to analyze the literature evaluating the association between eye color and acquired causes of sensorineural hearing loss and to propose the potential mechanisms of this association.Methods:Six databases were searched to identify clinical studies relating eye color and acquired causes of sensorineural hearing loss such as noise, cisplatin ototoxicity, radiation, and presbycusis. Included articles were independently assessed for quality by two authors using the Downs and Black rating scale.Results:Seventeen articles were eligible for review. Eye color was not found to have an effect in the non-exposed population or in presbycusis. In noise-induced sensorineural hearing loss, light-eyed patients had more significant loss following noise exposure, although the variability reported due to eye color was modest (r2 = 1-14%). Two out of three studies reported that dark e...

Objective: To systematically review the literature to characterize radiation-induced sensorineural hearing loss for head and neck tumors.Method: Seven databases were searched to identify studies assessing the characteristics of sensorineural hearing loss caused exclusively by radiotherapy for head and neck cancer.Results: Fourteen articles were evaluated. The reported incidence of sensorineural hearing loss was higher on frequencies above 4 Hz (up to 95%) when compared to the speech frequencies. Radiation dose and follow-up time were important factors affecting incidence rates.Conclusion: Evidence demonstrates that the resulting SNHL is irreversible, progressive, and dose-dependent. High-quality literature regarding SNHL due to radiotherapy is lacking. Improved evaluation protocols should be adopted in the standard of care for patients undergoing radiotherapy.

Objective: 1) Evaluate the commonly used ototoxicity criteria. 2) Develop a method for establishing comparisons among studies using different criteria. 3) Use this comparison method to evaluate the effect of treatment and assessment factors (ie, chemotherapy agent, cumulative dose, head and neck radiotherapy, and follow-up period) on the reported incidence of ototoxicity.Method: A review was performed on studies regarding platinum compounds and hearing loss published between January 1, 2000, and July 15, 2011. A total of 47 pediatric and 42 adult studies were evaluated. Data analyzed included patient demographics, treatment modalities (surgery, radiotherapy and chemotherapy), and auditory outcomes.Results: Ten methods for defining ototoxicity were identified, and each was critically reviewed. Grades within each criteria were then classified as either “mild/moderate” or “severe” ototoxicity, based on their definitions and on the agreement between these criteria in the literature. The total incidence of oto...

Objective Bone-anchored hearing aid (BAHA™) is a proven tool to improve hearing. Nevertheless, there are patients who are candidates for BAHA™ implants that end up refusing the surgery. The objective of this study is to review our BAHA™ experience with particular emphasis on reasons behind the refusal of some candidates. Methods A prospective cohort of 100 consecutive new candidates referred to The BAHA™ program in a tertiary health care center. Candidates’ demographics, hearing status, Co-morbidities and audiometeric tests were all recorded. Patients’ acceptance or refusal was noted alongside the reasons to refuse BAHA™. Results 100 new candidates were seen for BAHA™ assessment, 10 patients were excluded due to incomplete data. There were 68 children and 22 adults. Unilateral Conductive Hearing Loss was the most common reason for consultation (40%), followed by unilateral SNHL (23.3%). Aural Atresia was the commonest clinical finding (36.6%). The commonest reason for refusal was social acceptance by the parents due to concern with cosmesis. Conclusion The main reason of BAHA™ surgery refusal, in otherwise eligible candidates, is related to cosmesis. Patients with congenital anomalies were the most likely candidates to accept BAHA™ implants.

Objective: Determine the prevalence of asthma in children who underwent rigid bronchoscopy (RB) for a suspected foreign body aspiration (sFBA) in the tracheobronchial tree, and to identify characteristics of patients who could benefit from a trial of anti-asthma treatment prior to undergoing a diagnostic bronchoscopy.Method: Retrospective chart review of children with sFBA in the tracheobronchial tree who underwent RB at the Montreal Children’s Hospital (2001-2009). Patient characteristics such as delay between initial choking episode and first otolaryngology evaluation, clinical findings, radiologic findings, bronchoscopic findings, and history of asthma were analyzed.Results: A total of 55 children underwent a RB for sFBA. Foreign bodies (FB) were found in 36 of these, one of which was asthmatic (2.78%, P < .05). In the 19 children who had a negative bronchoscopy (no FB), four were asthmatics (21.1%, P < .05). The mean time from the suspected initial choking event to the first otolaryngology evaluation ...

Objective: Conduct an updated systematic review on the outcome of Bell Palsy (BP) in children following steroid treatment.Method: Medline, Embase, Cochrane Library, and BIOSIS Previews databases were searched obtaining articles from years 2000 to 2010 without any language restriction. Articles describing children aged 0 to 18 years with BP treated solely with corticosteroids were included. The outcome measure was facial movements based on clinical scales.Results: A total of 2293 papers were initially identified. Following review by 2 authors, 68 papers were analyzed in a hard-copy format. Finally, 6 studies were eligible to be included in the systematic review. Four of the studies exclusively included children with BP where in 2; various etiologies of facial palsy were described. The nontreated groups were smaller than the treated groups. Type of steroid and duration of treatment were inconsistently specified. Outcome measures used include the House-Brackmann scale, Yanagihara grading system, and clinical...

Objective: Cisplatin is a commonly used chemotherapeutic agent. One of its major dose limiting side effects is ototoxicity. No treatment has yet been approved for this condition. The objective of this study was to determine the potential protective effect of a systemic administration of erdosteine against cisplatin-induced ototoxicity.Method: Guinea pigs were assigned to 4 groups, each receiving a different concentration of intraperitoneal erdosteine: 0, 100, 200, and 500 mg/kg/d for 4 days. All of the animals received 12 mg/kg/d of intraperitoneal cisplatin. Auditory brainstem response (ABR), scanning electron microscopy, and outer hair cell counts were performed.Results: Significant otoprotection was observed in groups receiving 200 and 500 mg/kg/d of erdosteine as demonstrated by high frequency (25 kHz) ABR threshold shifts. These findings are supported by outer hair cell counts by scanning electron microscopy. Guinea pigs receiving 100 mg/kg/d did not present otoprotection.Conclusion: A systemic admin...

Objective: Characterize the demographics, etiologies, treatment and outcome of children presenting with facial paresis at two tertiary care pediatric hospitals. Study Design and Setting: Retrospective chart review. Children 0 18 years old admitted for facial nerve palsy between April 1989 and April 2005 were identified and pertinent information was recorded from their medical records. The facial palsy severity was scored using the House-Brackmann grading scale. Results: 280 cases of facial nerve paralysis were identified. Etiologies of facial nerve palsy were otologic infections (n = 56), congenital (n= 43), neoplasms (n=40), forceps use at birth (n=28), Bell’s palsy (n= 22), post-operative complication (n=16) and trauma (n=16). Other etiologies were found in the 59 remaining patients. Outcome varied between 100% complete recovery for forceps trauma cases to 23% complete recovery in postoperative complication cases. Conclusion: This represents the largest case series on facial nerve palsy in children. The etiologies of facial palsy in children are different from the ones reported in adults and are associated with a more favourable outcome. A better understanding of this condition and its various etiologies is necessary to obtain an accurate diagnosis and adjust the treatment accordingly. Results Summary of results by etiology