Dominant negative variants in IKZF2 cause ICHAD syndrome, a new disorder characterised by immunodysregulation, craniofacial anomalies, hearing impairment, athelia and developmental delay.

BACKGROUND: Helios (encoded by IKZF2), a member of the Ikaros family of transcription factors, is a zinc finger protein involved in embryogenesis and immune function. Although predominantly recognised for its role in the development and function of T lymphocytes, particularly the CD4+ regulatory T cells (Tregs), the expression and function of Helios extends beyond the immune system. During embryogenesis, Helios is expressed in a wide range of tissues, making genetic variants that disrupt the function of Helios strong candidates for causing widespread immune-related and developmental abnormalities in humans. METHODS: We performed detailed phenotypic, genomic and functional investigations on two unrelated individuals with a phenotype of immune dysregulation combined with syndromic features including craniofacial differences, sensorineural hearing loss and congenital abnormalities. RESULTS: Genome sequencing revealed de novo heterozygous variants that alter the critical DNA-binding zinc fingers (ZFs) of Helios. Proband 1 had a tandem duplication of ZFs 2 and 3 in the DNA-binding domain of Helios (p.Gly136_Ser191dup) and Proband 2 had a missense variant impacting one of the key residues for specific base recognition and DNA interaction in ZF2 of Helios (p.Gly153Arg). Functional studies confirmed that both these variant proteins are expressed and that they interfere with the ability of the wild-type Helios protein to perform its canonical function-repressing IL2 transcription activity-in a dominant negative manner. CONCLUSION: This study is the first to describe dominant negative IKZF2 variants. These variants cause a novel genetic syndrome characterised by immunodysregulation, craniofacial anomalies, hearing impairment, athelia and developmental delay.

Two-Year Outcomes After Pediatric In-Office Tympanostomy Using Lidocaine/Epinephrine Iontophoresis and an Automated Tube Delivery System.

OBJECTIVE: Evaluate 2-year outcomes after lidocaine/epinephrine iontophoresis and tympanostomy using an automated tube delivery system for pediatric tube placement in-office. STUDY DESIGN: Prospective, single-arm. SETTING: Eighteen otolaryngology practices. METHODS: Children age 6 months to 12 years indicated for tympanostomy were enrolled between October 2017 and February 2019. Local anesthesia of the tympanic membrane was achieved via lidocaine/epinephrine iontophoresis and tympanostomy was completed using an automated tube delivery system (the Tula® System). An additional Lead-In cohort of patients underwent tube placement in the operating room (OR) under general anesthesia using only the tube delivery system. Patients were followed for 2 years or until tube extrusion, whichever occurred first. Otoscopy and tympanometry were performed at 3 weeks, and 6, 12, 18, and 24 months. Tube retention, patency, and safety were evaluated. RESULTS: Tubes were placed in-office for 269 patients (449 ears) and in the OR for 68 patients (131 ears) (mean age, 4.5 years). The median and mean times to tube extrusion for the combined OR and In-Office cohorts were 15.82 (95% confidence interval [CI]: 15.41-19.05) and 16.79 (95% CI: 16.16-17.42) months, respectively. Sequelae included ongoing perforation for 1.9% of ears (11/580) and medial tube displacement for 0.2% (1/580) observed at 18 months. Over a mean follow-up of 14.3 months, 30.3% (176/580) of ears had otorrhea and 14.3% (83/580) had occluded tubes. CONCLUSION: In-office pediatric tympanostomy using lidocaine/epinephrine iontophoresis and automated tube delivery results in tube retention within the ranges described for similar grommet-type tubes and complication rates consistent with traditional tube placement in the OR.

High frequency of otolaryngology/ENT encounters in Canadian primary care despite low medical undergraduate experiences.

Otolaryngology involves the treatment of patients with diseases and disorders of the ear, nose, throat (ENT), and related structures of the head and neck. Many medical students in Canada have limited experiences in ENT and a vast majority of these students go on to pursue a career as primary care physicians. Physicians at a primary care facility classified patient's visits as either being "ENT" related or not, to assess the amount of ENT related concerns they typically encounter. The data was collected separately in the summer and winter months to assess any seasonal variability. One in eight patient encounters presented with an ENT related concern. The percentage of ENT related symptom presentation visits in the pediatric population for both data collection periods (29%) was more than three times that of the adult population (9%). The rate of ENT symptom presentation in both adult and pediatric populations was not affected by seasonality. Primary care physicians will encounter new patients presenting with ENT related concerns quite frequently. This is especially true in the pediatric patient population. Increased ENT medical education is both necessary and essential for undergraduate medical students, residents, and primary care physicians.

L'oto-rhino-laryngologie (ORL) concerne les maladies et les troubles de l'oreille, du nez, de la gorge et des structures connexes de la tête et du cou. De nombreux étudiants au Canada n'ont qu'une expérience limitée de cette spécialité alors que la grande majorité d'entre eux poursuivent une carrière de médecin de soins primaires. Les médecins d'un établissement de soins primaires ont classé les visites des patients afin de déterminer le volume de consultations en lien avec l'ORL. Les données ont été recueillies séparément pendant les mois d'été et d'hiver pour évaluer la variabilité saisonnière. D'après les données, une consultation sur huit était liée à la présence de symptômes ORL. Le pourcentage de consultations chez la population pédiatrique pour les deux périodes de collecte de données (29 %) était plus de trois fois supérieur à celui de la population adulte (9 %). La survenance de symptômes ORL n'était pas affectée par la saisonnalité, ni chez l'une ni chez l'autre. Les médecins de soins primaires voient assez souvent de nouveaux patients présentant des problèmes ORL, particulièrement des enfants. Un renforcement de l'enseignement de la médecine ORL est à la fois nécessaire et essentiel pour les étudiants en médecine de premier cycle, les résidents et les médecins de soins primaires.

Spontaneous Resolution of Ear Lidding in Newborns: A Prospective Observational Cohort Study.

ABSTRACT: Ear lidding is a cosmetic outer ear shape deformity commonly observed in newborns. Although lidding is considered a benign condition, psychological concerns such as bullying and depression have been observed in older children supporting correction of the condition. Nonsurgical correction of lidding using molding and splinting techniques has become increasingly popular, achieving successful outcomes in the majority of cases. Spontaneous resolution of the condition has also been reported in the literature however there is minimal prospective data available on the natural progression of ear lidding. In our case series of 11 closely followed newborns, we aimed to characterize the natural progression and resolution of lidding. Ten consecutive newborns participated in the observation plan and all 10 had complete spontaneous resolution of lidding within an average of 40 days. One other newborn's parents self-selected to have molding and splinting treatment. These results suggest that cosmetic treatment for less severe cases of ear lidding may be unnecessary as they have the potential to resolve on their own. Future research in this area could include controlled study designs and more work is needed to identify, which infants will require treatment. Our study may provide helpful reassurance to families and physicians that many newborns may see complete resolution of lidding without intervention.

Intranasal Dexmedetomidine increases the successful sedation of children with autism for out-patient auditory brainstem response hearing tests.

INTRODUCTION: The auditory brainstem response (ABR) hearing test can be challenging in children with autism spectrum disorder (ASD) due to the inherent behavioral challenges associated with this condition. To attempt to increase our success in sedating ASD patients, we added the use of intranasal Dexmedetomidine (Dexmed) to be used alone or with oral Chloral Hydrate (CH) in an ambulatory care setting, with monitoring by a specialist nurse. OBJECTIVES: To determine the success and safety of a protocol for ABR testing performed under sedation with intranasal Dexmed and oral chloral hydrate in ASD patients. To compare the success rate, the occurrence of adverse events and time needed to initiate ABR between Dexmed-CH protocol and previous CH-alone protocol in ASD patients. METHODS: Retrospective review. ASD patients in Dexmed-CH sedation protocol were age- and sex-matched to ASD patients who underwent CH-alone sedation protocol, for comparison. RESULTS: 74 ABR records in ASD children were included, 37 patients using Dex-CH protocol and 37 patients using CH-alone protocol. In the Dexmed-CH protocol group, Dexmed was used in 2 different ways: alone as a first choice in patients who refused to swallow CH (9/37), or combined with CH as a rescue (28/37). Under this sedation protocol, 89% of the attempted ABRs were completed successfully with no major adverse effects. In comparison, in ASD patients sedated using the protocol of CH-alone, the success rate significantly lower (69% vs. 89%). The time needed to initiate the test was not significantly different. CONCLUSION: The use of intranasal Dexmed by itself or in combination with CH was a safe and reliable method of performing sedated ABR in ASD patients. Modifying our previous oral CH protocol to include intranasal Dexmed, substantially improved our success rate of sedation in ASD patients in an ambulatory setting. This study may be of significant value to centers worldwide exploring alternatives to general anesthesia for ABR testing in ASD patients.

A national study of choanal atresia in tertiary care centers in Canada - part II: clinical management.

BACKGROUND: To evaluate the clinical management of choanal atresia (CA) in tertiary centers across Canada. METHODS: Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with choanal atresia to a participating center. RESULTS: The health charts of 215 patients (59.6% female) with choanal atresia (CA) were reviewed. Mean age of initial surgical repair was 0.8 months for bilateral CA, and 48.6 months for unilateral CA. Approaches of surgical repair consisted of endoscopic transnasal (31.7%), non-endoscopic transnasal (42.6%), and transpalatal (25.2%). Stents were used on 70.7% of patients. Forty-nine percent of patients were brought back to the OR for a planned second look; stent removal being the most common reason (86.4%). Surgical success rate of initial surgeries was 54.1%. Surgical technique was not associated with rate of restenosis [χ2 (2) = 1.6, p = .46]. CONCLUSIONS: The present study is the first national multi-institutional study exploring the surgical outcomes of CA over a 30-year period. The surgical repair of CA presents a challenge to otolaryngologists, as the rate of surgical failure is high. The optimal surgical approach, age at surgical repair, use of stents, surgical adjuncts, and need for planned second look warrant further investigation.

A national study of choanal atresia in tertiary care centers in Canada - part I: clinical presentation.

BACKGROUND: To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. METHODS: Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. RESULTS: The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. CONCLUSIONS: The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted.

Mastoid pressure dressing - Current practice amongst Canadian pediatric cochlear implant surgeons.

OBJECTIVES: Mastoid pressure dressing (MPD) has routinely been used following major ear surgery, such as cochlear implant (CI) surgery, to prevent postoperative wound complications. To date, controlled studies have suggested no difference in the incidence of wound complications following MPD use. However, there is a variation in the practice of MPD usage across pediatric CI surgeons. In this study, we aimed to identify the most common type of postoperative dressing management after pediatric cochlear implantation and the factors in the decision-making process for post-surgical care amongst Canadian pediatric CI surgeons. METHODS: Canadian Otolaryngologists who perform pediatric CI surgery were identified (n = 18) and contacted via email to complete a short online questionnaire regarding current post-operative head dressing practice following CI surgery. Descriptive statistics were used to analyze the response data. RESULTS: The participants provided an approximate number of CI's they performed in 2016. 100% of the recipients completed the survey. Approximately 376 CI's were completed in 2016 with an average of 21 CI's per surgeon. 61% of participants routinely used MPDs following surgery justified by reasons such as wound protection, institutional standard of care, and physician's original training practice. CONCLUSION: There is no clear consensus on the use of MPDs amongst Canadian pediatric CI surgeons. Since the current evidence in the literature suggests no difference in wound complication incidence post-surgery with MPD use, a change in postoperative dressing management to non-use in those that employ this practice may be justified. Eliminating the usage of a MPD may also have potential economic benefits. Further prospective controlled studies may be warranted.

In-Office Tympanostomy Tube Placement in Children Using Iontophoresis and Automated Tube Delivery.

OBJECTIVES/HYPOTHESIS: Evaluate technical success, tolerability, and safety of lidocaine iontophoresis and tympanostomy tube placement for children in an office setting. STUDY DESIGN: Prospective individual cohort study. METHODS: This prospective multicenter study evaluated in-office tube placement in children ages 6 months through 12 years of age. Anesthesia was achieved via lidocaine/epinephrine iontophoresis. Tube placement was conducted using an integrated and automated myringotomy and tube delivery system. Anxiolytics, sedation, and papoose board were not used. Technical success and safety were evaluated. Patients 5 to 12 years old self-reported tube placement pain using the Faces Pain Scale-Revised (FPS-R) instrument, which ranges from 0 (no pain) to 10 (very much pain). RESULTS: Children were enrolled into three cohorts with 68, 47, and 222 children in the Operating Room (OR) Lead-In, Office Lead-In, and Pivotal cohorts, respectively. In the Pivotal cohort, there were 120 and 102 children in the <5 and 5- to 12-year-old age groups, respectively, with a mean age of 2.3 and 7.6 years, respectively. Bilateral tube placement was indicated for 94.2% of children <5 and 88.2% of children 5 to 12 years old. Tubes were successfully placed in all indicated ears in 85.8% (103/120) of children <5 and 89.2% (91/102) of children 5 to 12 years old. Mean FPS-R score was 3.30 (standard deviation [SD] = 3.39) for tube placement and 1.69 (SD = 2.43) at 5 minutes postprocedure. There were no serious adverse events. Nonserious adverse events occurred at rates similar to standard tympanostomy procedures. CONCLUSIONS: In-office tube placement in selected patients can be successfully achieved without requiring sedatives, anxiolytics, or papoose restraints via lidocaine iontophoresis local anesthesia and an automated myringotomy and tube delivery system. LEVEL OF EVIDENCE: 2b Laryngoscope, 130:S1-S9, 2020.

Subjective and objective vestibular changes that occur following paediatric cochlear implantation: systematic review and meta-analysis.

OBJECTIVE: Cochlear implantation can result in post-operative vestibular dysfunction of unknown clinical significance. The objective of this study was to characterize the presence, magnitude, and clinical significance of vestibular dysfunction that occurs after pediatric cochlear implantation. DATA SOURCES: The databases Embase, Medline (OvidSP), and PubMed were used. Only articles published in English were included. Grey literature and unpublished sources were also reviewed. STUDY SELECTION: Articles published from 1980 until the present which documented pre-operative and post-operative vestibular testing on children under the age of 18 were used. DATA EXTRACTION: Parameters that were assessed included number of patients, pre- and post-operative vestibular-evoked myogenic potentials (VEMPs), head impulse testing (HIT), calorics, and posturography, timing of pre- and post-operative testing, symptomatology, and other demographic data such as etiology of the hearing loss. DATA SYNTHESIS: Ten articles were included. Relative risk values evaluating the effect of cochlear implantation on vestibular function were calculated for VEMPs and caloric testing due to the availability of published data. I2 values were calculated and 95% confidence intervals were reported. Separate analyses were conducted for each individual study and a pooled analysis was conducted to yield an overall relative risk. Assessment on risk of bias in individual studies and overall was performed. CONCLUSION: Pediatric cochlear implantation is associated with a statistically significant decrease in VEMP responses post-operatively (RR 1.8, p < 0.001, I2 91.86, 95%CI 1.57-2.02). Similar results are not seen in caloric testing. Insufficient data is available for analysis of HIT and posturography. Further studies are necessary to determine the effect of cochlear implantation on objective vestibular measures post-operatively and whether any changes seen are clinically relevant in this population.

Improving vaccination uptake in pediatric Cochlear implant recipients.

BACKGROUND: An Infectious Disease vaccine specialist joined our institution's Cochlear Implant Team in 2010 in order to address the high percentage of non-compliance to immunization prior to surgery identified previously from an internal review. The purpose of this study was to (1) review the immunization status of cochlear implant recipients in 2010-2014, (2) assess if introducing a vaccine specialist made a significant change in vaccination compliance and (3) elucidate any barriers to vaccination compliance. METHODS: Retrospective chart review and a telephone survey. Medical records of 116 cochlear implant recipients between 2010 and 2014 were reviewed. A telephone survey was conducted to obtain the current vaccination status in children who required post-operative vaccinations with incomplete records on chart review and, if applicable, the reason for non-compliance. RESULTS: Between 2010 and 2014, 98% of children were up-to-date at the time of surgery, compared to 67% up-to-date at the time of surgery between 2002 and 2007. 27 children were included in our post-operative immunization analysis. 29.6% (8/27) failed to receive necessary vaccinations post-surgery. Pneumovax-23, a vaccine for high-risk patients (such as cochlear implant candidates) was missed in all cases. CONCLUSION: Pre-operative vaccination for cochlear implant recipients improved dramatically with the addition of a vaccine specialist. However, a significant proportion of patients requiring vaccinations post-surgery did not receive them. The main reason for non-compliance was due to parents being unaware that their children required this vaccine postoperatively by being "high-risk". Although improvement was demonstrated, a communication gap continued to impede the adequacy of vaccination uptake in pediatric cochlear implant recipients following surgery at age 2 when the high-risk vaccine was due.

Detection of abnormally shaped ears by a trained non-specialist allows for early non-surgical intervention.

INTRODUCTION: Many children are born with abnormally-shaped ears, including protruding ears or unusually-shaped outer ears. While the majority are benign, these may cause significant issues with self-esteem and bullying in childhood. Early molding can resolve some of these abnormalities, avoiding the need for future corrective surgery. However, newborns with these abnormalities are rarely identified early, within the first few days of life, when molding is most effective. In this study, we investigate whether a trained non-specialist can correctly identify ear shape abnormalities in newborns. METHODS: A non-specialist (medical student) was trained on normal and abnormal ear anatomy using photographs and descriptions. Newborns <72 h of age were recruited from maternity wards. Newborns' ears were photographed and these images were assessed independently by two specialists and the non-specialist. External ear shape was classified as either normal or abnormal based on pre-determined criteria. RESULTS: A total of 661 ears of 334 newborns were photographed and assessed. High inter-rater agreement was achieved with a kappa statistic of 0.863 (SE 0.078). The non-specialist detected abnormally-shaped ears with a sensitivity and specificity of 90.9% and 91.1% respectively. CONCLUSIONS: Our study illustrates that non-specialist can be trained to accurately detect newborn ear abnormalities, providing a cost-effective means of ensuring that these children's health care needs are met in a timely fashion. Specifically, we recommend the integration of ear shape assessment into currently established programs such as the newborn hearing screening program.

Krazy Glue® in the ear: A case report of child abuse.

Krazy Glue® or cyanoacrylate glue is an acrylic resin that polymerizes in less than a minute when in contact with moisture or water. We present a case of a one month old referred to our tertiary pediatric otolaryngology clinic from an outside emergency department with a history of application of cyanoacrylate glue in the external ear canals. This report presents the management of this case along with the medical and legal outcomes surrounding this case of child abuse.

Health care professionals' neckties as a source of transmission of bacteria to patients: a systematic review.

BACKGROUND: There is growing concern that neckties worn by health care professionals may contribute to infections contracted in health care settings. We evaluated the evidence for health-care-associated infections resulting from neckties and whether the evidence is sufficient to warrant a tieless policy in Canada. METHODS: We performed a systematic review to determine whether neckties worn by health care professionals colonize harmful pathogenic bacteria and whether they contribute to the spread of infection to patients in the inpatient or outpatient setting. We searched PubMed (1966 to 2017) and Embase (1974 to 2017). The level of evidence was appraised according to the Oxford Centre for Evidence-Based Medicine levels of evidence. We evaluated the quality of evidence and the risk of bias using the Jadad scale or the Newcastle-Ottawa Scale. RESULTS: We screened 1675 citations, of which 6 were ultimately included in the systematic review. Only 1 study gave level 1b evidence (randomized controlled trial). Neckties were more likely than shirt pockets to colonize bacteria. There is limited evidence that neckties may be contaminated with pathogenic bacteria (e.g., methicillin-resistant Staphylococcus aureus) and very limited evidence that contaminated neckties may transmit bacteria (in a controlled experimental setting to a mannequin). INTERPRETATION: There is no evidence of increased rates of health-care-associated infections related to the wearing of neckties by health care professionals. There is weak evidence that neckties are contaminated with pathogenic (and nonpathogenic) bacteria. The level of evidence was weak and the studies were heterogeneous. Evidence to support the need for a tieless dress code policy is lacking.

Unique Case of Hearing Recovery After Otic Capsule Destruction and Complete Sensorineural Hearing Loss Caused by Langerhans Cell Histiocytosis.

: A 14-year-old woman presented with right-sided otologic and vestibular symptoms after presenting with hormonal disturbances earlier that year. Imaging showed a gross destruction of the temporal bone, mastoid air cells, and external acoustic meatus with invasion into the otic capsule. The patient experienced complete sensorineural hearing loss in the right ear. Biopsy diagnosed Langerhans cell histiocytosis (LCH) and the patient was treated with chemotherapy. After 1 year of treatment, the patient's hearing partially recovered and imaging showed reconstitution of the temporal bone including the otic capsule. Our case is the first report of complete sensorineural hearing loss with partial recovery after LCH treatment.

Health-Related Quality of Life Among Young Children With Cochlear Implants and Developmental Disabilities.

OBJECTIVE: The present study examined differences in health-related quality of life (HRQoL) between deaf children with cochlear implants (CI) with and without developmental disabilities (DD) and differences across HRQoL domains within both groups of children. METHODS: Ninety-two parents of children with CI aged 3-7 years participated in this cross-sectional study. Of these children, 43 had DD (i.e., CI-DD group) and 49 had no DD or chronic illness, demonstrating overall typical development (i.e., CI-TD group). Parents of children in both groups completed the KINDL, a generic HRQoL questionnaire. Parents also provided anecdotal comments to open-ended questions, and parent comments were evaluated on a CI benefits scale to assess parent-perceived benefits of CI for the deaf children with and without disabilities. RESULTS: Children in the CI-DD group had significantly lower HRQoL compared to children in the CI-TD group, including lower scores on the self-esteem, friend, school, and family HRQoL subscales. No significant differences among groups were found on the physical well-being and emotional well-being subscales. For the CI-TD group, age at implantation correlated negatively with self-esteem and school HRQoL subscales. In the CI-DD group, children's current age correlated negatively with family and with the total HRQoL scores. Parent anecdotal comments and scores on the CI-benefits scale indicated strong parent perceptions of benefits of implantation for children in both groups. CONCLUSION: Based on parents' proxy report, findings suggest that having DD affects multiple domains of HRQoL among young children with CIs above and beyond that of the CI itself. Parents of deaf children with DD may need greater support through the CI process and follow-up than parents of deaf children without DD.

Risk of sensorineural hearing loss with macrolide antibiotics: A nested case-control study.

OBJECTIVES: To determine the association between a diagnosis of sensorineural hearing loss (SNHL) and the prescription of a macrolide antibiotic. STUDY DESIGN: Retrospective nested case-control study. METHODS: From the LifeLink (IMS, Danbury, CT) health claims database, we randomly selected a cohort of subjects 15 to 60 years old from 2006 to 2014. Cases were identified as patients diagnosed with SNHL, each matched by age and calendar time to 10 controls selected from the same cohort. All macrolide prescriptions (erythromycin, azithromycin, clarithromycin, and telithromycin) were identified, and statistical comparison of usage was compared between cases and controls. Amoxicillin and fluoroquinolone antibiotics were used as positive controls to further investigate confounding by infection. Albuterol was used as a negative control because this is a drug class not expected to be associated with SNHL or with a confounding condition potentially causing SNHL. RESULTS: From a cohort of 6,110,723 subjects, we identified 5,989 cases of SNHL and 59,890 corresponding controls. The rate ratio for one prescription of a macrolide was 1.36 (95% confidence inteval [CI]: 1.24-1.49) and for multiple prescriptions was 1.66 (95% CI: 1.42-1.94). Similar rate ratios were observed with multiple prescriptions of amoxicillin and fluoroquinolones. CONCLUSION: A significant association between SNHL and macrolide use was likely due to confounding by indication for antibiotic treatment because the risk was also observed with fluoroquinolones and amoxicillin, antibiotics with no known ototoxic potential. Therefore, there does not appear to be an increased risk of SNHL in patients treated with macrolide antibiotics. LEVEL OF EVIDENCE: 3b. Laryngoscope, 127:229-232, 2017.