Management of first branchial anomalies in children: 20 years of experience.

PURPOSE: Branchial cleft anomalies (BCAs) are common pediatric head and neck lesions; however, only 1-4% involve the first branchial cleft. The rare occurrence of first BCAs, their presentation at a young age, and the possible facial nerve involvement make diagnosis and treatment challenging. METHODS: A retrospective chart review was conducted for children diagnosed with their first BCA between 2000 and 2020. Data on demographics, presenting symptoms, physical findings, imaging features, previous surgery, and treatment outcomes were collected and analyzed. RESULTS: The cohort included 17 patients with a median age of 5 years at presentation. Seven (41%) had undergone previous surgical intervention before definitive surgery. Eight were classified as Work Type II anomalies, and nine as Work Type I. Sixteen patients (94%) underwent definitive surgical excision at a median age of 6.9. A parotid approach was used in 10 (62%), with dissection of the mass from the facial nerve, and a retro-auricular or end-aural approach was used in 6 (38%). Complete excision was achieved in 14/16 patients (88%). Three patients had transient facial nerve paresis postoperatively. Recurrence was noted in 3/16 patients (18%). Enhancement in imaging was positively correlated with post-operative complications (R = 0.463, P = 0.018). CONCLUSIONS: First, BCA poses a diagnostic and surgical challenge; thus, definitive surgical treatment is often delayed. The surgical approach should be tailored to the type of anomaly (Work type I or II) and possible facial nerve involvement. Risk factors for post-operative complications are a history of recurrent infections and previous surgical interventions. The presence of contrast enhancement in preoperative imaging should alert surgeons to perioperative challenges and the risk of post-operative complications.

The impact of birth weight and gestational age on acute mastoiditis in children.

OBJECTIVE: Previous data correlate preterm and low birth weight (LBW) with acute otitis media, but there is a gap concerning the relations with acute mastoiditis (AM). This study investigates the effect of LBW and preterm birth on AM disease severity, neuro-otological complications, and recurrence. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary medical center. METHODS: The cohort is retrospective in nature consisting of 294 children with AM admitted between 1999 and 2020. Data collection included: patient gestational age and birth weight, signs and symptoms, physical examination, laboratory tests, imaging findings, and long-term outcomes. RESULTS: 294 cases of AM were included, 41/281 (15%) had LBW (< 2500 g), and 46/294 (15.7%) were preterm (gestational age < 37 weeks). We found no significant differences in laboratory tests, imaging studies (CT), rate of mastoidectomy performed, or late complications between LBW and normal birth weight (NBW) and between preterm and normal gestational age children. LBW children tended to develop AM at an older age compared with NBW children, 2.28 + 1.64 Vs. 1.84 + 1.4 years, respectively (p-value = 0.016). Additionally, preterm children were more prone to develop a second event of AM, with a shorter interval between these episodes. CONCLUSIONS: LBW and preterm birth are not independent risk factors for disease severity, need for intervention, or future complications in AM. Yet, LBW children present with AM at an older age, and preterm children are more prone for recurrent episodes of AM with a shorter interval between episodes suggesting a distinct disease course in these populations.

Acute mastoiditis in infants younger than 6 months: is an alternative treatment protocol needed?

OBJECTIVES: Several studies have reported that younger children suffer from increased incidence and more severe episodes of acute mastoiditis (AM) than older children, whereas other researchers have found the opposite. The aim of our study was to describe the occurrence, clinical, and microbiological aspects of AM in children 6 months or younger, and compare them with the results in an older reference age band. METHODS: The medical files of children hospitalized with a diagnosis of AM during 2001-2016 were retrospectively reviewed. Diagnosis of AM was based on the presence of clinical signs of acute otitis media (AOM) accompanied by two or more of the following: auricle protrusion, retro-auricular erythema, swelling, and local tenderness. Children were divided into two age bands, 6 months or younger, and older than 6 months. RESULTS: Fifty patients in the young age band and 335 in the older reference age band were included. Bilateral AOM was identified in 14 (28%) children under 6 months, and 50 (14.9%) in the reference age band (p < 0.001). Fever, mean WBC, and CRP values were similar in both age bands; 4 (8%) children under 6 months had pre-auricular/zygomatic area swelling, as compared to 1(0.02%) in the reference age band (p < 0.001). Complication rates (subperiosteal abscess, sinus vein thrombosis, and epidural abscess) were similar in both age bands. All children were treated with myringotomy and IV antibiotics. Cortical mastoidectomy with the insertion of ventilation tube was performed in 10 (20%) children in the younger age band and 58(17.3%) in the reference age band. Streptococcus pneumoniae was the most common (38%) isolated bacteria in the younger age band, and Group A streptococcous (GAS) (20.6%) in the reference age band. CONCLUSIONS: AM in children 6 months or younger has similar presentation and characteristics as in older children. Pre-auricular swelling and bilateral AOM are more typical in the younger age band; Streptococcus pneumoniae is the most common pathogen in the younger children. We suggest that the treatment approach should be the same for both groups.

Spectrum of genes for inherited hearing loss in the Israeli Jewish population, including the novel human deafness gene ATOH1.

Mutations in more than 150 genes are responsible for inherited hearing loss, with thousands of different, severe causal alleles that vary among populations. The Israeli Jewish population includes communities of diverse geographic origins, revealing a wide range of deafness-associated variants and enabling clinical characterization of the associated phenotypes. Our goal was to identify the genetic causes of inherited hearing loss in this population, and to determine relationships among genotype, phenotype, and ethnicity. Genomic DNA samples from informative relatives of 88 multiplex families, all of self-identified Jewish ancestry, with either non-syndromic or syndromic hearing loss, were sequenced for known and candidate deafness genes using the HEar-Seq gene panel. The genetic causes of hearing loss were identified for 60% of the families. One gene was encountered for the first time in human hearing loss: ATOH1 (Atonal), a basic helix-loop-helix transcription factor responsible for autosomal dominant progressive hearing loss in a five-generation family. Our results show that genomic sequencing with a gene panel dedicated to hearing loss is effective for genetic diagnoses in a diverse population. Comprehensive sequencing enables well-informed genetic counseling and clinical management by medical geneticists, otolaryngologists, audiologists, and speech therapists and can be integrated into newborn screening for deafness.

[COCHLEAR IMPLANTS IN SINGLE-SIDED DEAFNESS].

INTRODUCTION: In normal hearing, the brain receives bilateral auditory input from both ears. In individuals with only one functioning ear listening in noisy environments and sound localization may become difficult. Historically, the impact of unilateral hearing loss in children had typically been minimized by clinicians, as it was assumed that one normal hearing ear provided sufficient auditory input for speech development and normal hearing experience. Data supporting the negative effects of unilateral deafness has been accumulating during the last decades. The effects of unilateral deafness extend beyond spatial hearing to language development, slower rates of educational progress, problems in social interaction and in cognitively demanding tasks. Until recently, treatments for single sided deafness were limited to routing signals from the deaf ear to the contralateral hearing ear either through conventional CROS aids or through bone anchored technologies. These technologies simply transfer sounds to the single functioning ear which allow sound awareness from the deaf side and minor improvement in hearing in noisy environments and localization. The cochlear implant is a surgically implanted electronic device that contains an array of electrodes which is placed into the cochlea, and stimulates the cochlear nerve. The cochlear implant bypasses the injured parts of the inner ear. Currently it is the only treatment to restore binaural hearing. This review aims to discuss the different aspects, the benefits and disadvantages of cochlear implantation in children with single sided deafness.

Surgical intervention for acute mastoiditis: 10 years experience in a tertiary children hospital.

PURPOSE: To evaluate the clinical course of children with acute mastoiditis (AM) who required surgical intervention. MATERIAL AND METHODS: Clinical and biochemical characteristics at the moment of hospital admission were reviewed for patients who required surgery for AM. Children who were successfully managed conservatively during the last 3 years of study were chosen as a comparison group. RESULTS: During 2008-2017, 570 children were admitted with AM: 82(14%) underwent cortical mastoidectomy, including 31(38%) with decompression of epidural space and sigmoid sinus. The comparison group consisted of 167 children with AM who did not require surgery. The surgical group had a higher rate of acute otitis media before admission. At the time of hospital admission, the surgical group had a higher rate of prolonged fever, otorrhea, and sub-periosteal abscess. Their average temperature, WBC, neutrophil count, and CRP were significantly higher (39.2 vs. 37.9°. C, 20 K vs. 16.5 K, 67 vs. 55.8 percent, 17 vs. 8.8, respectively, p = 0.0001). Fusobacterium necrophorum was the most common pathogen in the surgical group (50%), and group A streptococcus in the comparison group (22%). Sub-periosteal abscess, sinus venous thrombosis, and epidural involvement were diagnosed in 95, 35, and 38 percent of patients, respectively. Average length of IV antibiotic treatment was 20 days in operated children, compared to 5.6 days in the comparison group (p = 0.0001). Since 2013, a significantly higher percentage of children were diagnosed with Fusobacterium mastoiditis (p = 0.0001) who required surgery (p = 0.008). CONCLUSION: In children with AM presenting with, high fever, leukocytosis, elevated CRP, and sub-periosteal abscess, early CT and surgical intervention were frequently required. The increase in Fusobacterium infection might be an explanation for the increase in complicated AM requiring surgery.

Pediatric Cochlear implant soft failure.

PURPOSE: Hard cochlear implant failures are diagnosed by objective tests whereas soft failures are suspected on the basis of clinical signs and symptoms. This study reviews our experience with children in tertiary pediatric medical center who underwent revision cochlear implantation, with emphasis on soft failures. MATERIALS AND METHODS: Children (age<18years) who underwent revision cochlear implantation from 2000 to 2012 were identified by database search. Pre- and post-explantation data were collected. RESULTS: Twenty-six revision surgeries were performed, accounting for 7.4% of all cochlear implant surgeries at our center during the study period. The pre-explantation diagnosis was hard failure in 7 cases (27%), soft failure in 12 (46%), and medical failure in 7 (27%). On post-explantation analysis, 7/12 devices from the soft-failure group with a normal integrity test had abnormal findings, yielding a 63% false-negative rate (12/19) for the integrity test. All children regained their initial performance. Compared to hard failures, soft failures were associated with a shorter median time from first implantation to symptom onset (8 vs 25months) but a significantly longer time from symptom onset to revision surgery (17.5 vs 3months; P=0.004). CONCLUSIONS: Soft cochlear implant failure in young patients poses a diagnostic challenge. A high index of suspicion is important because a delayed diagnosis may have severe consequences for language development. A normal integrity test does not unequivocally exclude device failure and is unrelated to functional outcome after revision surgery. Better education of parents and rehabilitation teams is needed in addition to more accurate diagnostic tests.

Long-term outcomes and electrophysiological measures of children with inner ear malformations and cochlear implants.

OBJECTIVES: To compared auditory and speech performance outcomes of children with cochlear implants (CI), between those with inner ear malformations (IEMs) and with normal ear anatomy; and to describe differences in electrophysiological measurements. STUDY DESIGN: A retrospective study. SETTING: A tertiary care pediatric medical center. PATIENTS: Forty-one children with IEMs who underwent CI during 2003-2017, and 41 age-matched CI recipients with normal ear anatomy (control group). MAIN OUTCOME MEASURES: Post-CI auditory performance outcomes including educational setting, Categories of Auditory Performance (CAP), and Speech Intelligibility Rating (SIR); and electrophysiological measurements, Including maximal comfortable electrical levels (CLs) and impedances along CI electrodes. RESULTS: The ANOVA on ranks revealed lower CAP scores in the study than control group: H3 = 18.8, P < 0.001. Among children with IEMs, CAP scores were better in children with enlarged vestibular aqueduct (EVA) (P < 0.04). SIR scores of the control group did not differ from those with isolated EVA; however, SIR scores of the IEMs without EVA subgroup were lower than all the other study subgroups (P < 0.01). The proportion of the control group that was integrated with full inclusion educational settings into the regular mainstream schools was higher than for those with IEMs without EVA (47 % vs. 15 %, P < 0.05), but similar to those with isolated EVA. For the study group versus control group, maximal comfortable electrical levels (CLs) were higher)P > 0.03) while impedance measurements were similar. CONCLUSIONS: Outcomes of pediatric recipients with normal anatomy were better than those with IEMs. Among pediatric recipients of CI with IEMs, auditory performance was better and CLs were lower among children with isolated EVA than all other IEM subgroups.

Discovery of a Novel Missense Variant in NLRP3 Causing Atypical Cryopyrin-Associated Periodic Syndromes With Hearing Loss as the Primary Presentation, Responsive to Anti-Interleukin-1 Therapy.

OBJECTIVE: Cryopyrin-associated periodic syndromes (CAPS), also known as NLRP3-associated autoinflammatory diseases, are a spectrum of rare autoinflammatory diseases caused by gain-of-function variants in the NLRP3 gene, resulting in inflammasome hyperactivation and dysregulated release of interleukin-1ß (IL-1ß). Many patients with CAPS develop progressive sensorineural hearing loss (SNHL) because of cochlear autoinflammation, which may be the sole manifestation in rare cases. This study was undertaken to establish the suspected diagnosis of CAPS in a family presenting with autosomal-dominant progressive/acute SNHL and a novel missense variant in the NLRP3 gene of unknown significance (NM_001079821.3:c.1784G>A p.Ser595Asn). METHODS: We conducted an ex vivo functional assessment of the NLRP3 inflammasome in heterozygous individuals (n = 10) and healthy family members (n = 5). RESULTS: The assay revealed hyperactivation of the inflammasome among heterozygous individuals, supporting the hypothesis that this missense variant is a pathogenic gain-of-function variant. Administration of IL-1 receptor antagonist resulted in a substantial clinical improvement among pediatric patients, who exhibited near resolution of hearing impairment within 1 to 3 months of treatment. CONCLUSION: Our findings highlight the crucial role of early diagnosis and treatment with an anti-IL-1 agent in reversing cochlear damage. Furthermore, our results suggest that high- and ultrahigh-frequency ranges need to be included in the auditory assessment to enable early detection of subclinical SNHL. Finally, incorporating functional inflammasome assessment as part of the clinical evaluation could establish the diagnosis in inconclusive cases.

Post-operative MRI detection of residual cholesteatoma in pediatric patients - The yield of serial scans over a long follow-up.

OBJECTIVES: Non-echo-planar diffusion weighted magnetic resonance imaging (Non-EPI DWI MRI) is commonly used for follow-up after cholesteatoma surgery. MRI has a critical role in the evaluation of residual disease, where physical examination will commonly demonstrate an intact tympanic membrane. The aim of our study was to assess the timing of residual cholesteatoma identification on serial MRI scans and the yield of MRI follow up after canal wall up tympano-mastoidectomy. METHODS: A retrospective chart review of children that underwent canal wall up tympano-mastoidectomy due to cholesteatoma in Schneider Children's Medical Center during 2004-2016, and were followed up both clinically and with MRI. RESULTS: Seventy-seven children (89 ears) were included, who altogether underwent 166 surgeries (77 revisions). Average follow-up was 66 ± 34.4 months. During follow up, 244 scans were performed; 19 cases of residual disease were diagnosed by MRI and confirmed in surgery. The mean time from surgery and an MRI positive for residual disease was 29.7 ± 16 months (range: 10-66). In 9/19 cases (47%), at least one negative MRI preceded the scan positive for residual disease, and in 4 cases at least two initial scans were negative. CONCLUSIONS: MRI plays an important role in the diagnosis of residual disease after cholesteatoma surgery. In our cohort. Almost half of the cases diagnosed with residual disease had at least one negative scan prior to the positive one, emphasizing the importance of close radiological follow-up with serial scans after surgery.

Congenital cholesteatoma: Clinical features and surgical outcomes.

OBJECTIVES: A typical presentation of congenital cholesteatoma (CC) is asymmetric conductive hearing loss (CHL). As CHL is usually associated with middle ear effusion, diagnosis of CC is frequently delayed. This study aimed to describe the clinical characteristics, treatment and outcomes of children with CC. METHODS: The medical files of children diagnosed with CC at a large tertiary pediatric medical center during 2000-2019 were reviewed. The primary outcome measures were: presenting symptoms, surgical findings, stage of disease, recurrence rate and hearing outcome. Imaging findings and the size of mastoid air cells were assessed in CT scans. RESULTS: Thirty-nine children were diagnosed with CC. The presenting symptom was unilateral CHL in 85%, with an average speech reception threshold of 41.5 ± 13.7 dB in the affected ear. The mean time from first symptoms to diagnosis was 1.3 years. The surgical approach was exploratory tympanotomy in 25% and canal wall up mastoidectomy in 69%. Seventy percent of the children presented with Potsic stage III-IV. The mean postoperative speech reception threshold was 26.4 ± 12.2 dB (P = 0.002). Recurrence of cholesteatoma occurred in 38% of the patients, mostly in stage III-IV. Mastoid air cell size was significantly smaller on the affected than the unaffected side. CONCLUSIONS: In children with persistent unilateral or asymmetric conductive hearing loss, CC should be suspected. Late diagnosis of CC is associated with a high recurrence rate. This highlights the need to promote awareness to the disease among primary physicians in the community health care system.

Paediatric otogenic sinus venous thrombosis: the role of Fusobacterium necrophorum.

Objectives: Sinus venous thrombosis (SVT) is a rare complication of acute otitis media (AOM) with acute mastoiditis (AM), which during recent years has been associated with Fusobacterium necrophorum (Fn) infection. Our objective was to review clinical, microbiologic, and hematologic features of paediatric otogenic SVT, with a specific focus on the role of Fn. Methods: A retrospective database review in a tertiary paediatric hospital between 2000-2019. Results: Fifty children aged 6-155 months were treated for AM with SVT. Forty-seven (94%) underwent cortical mastoidectomy. Forty-six children received low-molecular-weight heparin (LMWH). Follow-up imaging revealed recanalisation in 92% of cases. No long-term neurologic or haematologic complications were observed. Since 2014, when anaerobic cultures and PCR were routinely used in our institute, Fn was isolated from 15/21 children with SVT. Their time to recanalisation was longer, and the rate of lupus anticoagulant antibodies (LAC) was higher than in the 6 non-Fn patients. Children positive for LAC also had a longer time to recanalisation. Conclusions: Fn is a common pathogen in AM with SVT; its thrombogenic role was demonstrated by a higher prevalence of LAC and a longer time to recanalisation.

Cochlear implant failure: diagnosis and treatment of soft failures.

OBJECTIVE: Early diagnosis of cochlear implant failures (CIF) is a critical part of post-implantation follow-up. Diagnosis is challenging and time consuming. Our study aimed to describe diagnoses of CIF with emphasis on soft failures (SF), focusing on symptoms, time from symptoms to replacement, and differences between SF and hard failures (HF). METHODS: A retrospective review of medical records in a tertiary care referral paediatric medical centre including all patients who experienced CIF during 2000-2020. RESULTS: Of 1004 CI surgeries, 72 (7.2%) cases of CIF were included, of which 60 CIF were in children (mean age 3.1 years). Twenty-five cases were due to HF, 26 SF, and 21 due to medical reasons. Patients with SF were more likely to present with headache, dizziness, or tinnitus compared with those with HF. Facial stimulation and disconnections were more common in implants from Advanced Bionics, dizziness and tinnitus in Cochlear, and poor progression in Med-El. Mean time from symptoms to implant replacement surgery was longer in cases with SF compared to HF. CONCLUSIONS: SF poses a diagnostic challenge. Symptoms such as headache, dizziness, and tinnitus are common. Diagnosis of failure should often be based on assessments of the implant and rehabilitation teams.

Medical and surgical characteristics of fusobacterium necrophorum mastoiditis in children.

OBJECTIVES: To evaluate and compare characteristics of Fusobacterium necrophorum mastoiditis (FnM) to characteristics of acute mastoiditis (AM) caused by other bacteria in tertiary children hospital Methods : Children with FnM (N=43) and non FnM (N=88). Assess medical, microbiologic and imaging characteristics, surgical findings and postoperative recovery. RESULTS: Children with FnM had a positive history of otitis media, ear discharge and sub-periosteal abscess (p=0.0004, 0.09, 0.0003, respectively) at presentation. Their temperature, WBC and CRP were significantly higher (39.8 vs. 37.9, 19.4K vs. 16.1K, 21 vs. 8.7, p=0.0001). Positive culture was found in 46% of patients; 64% were diagnosed by PCR. CT scan was indicated in 95% and surgical intervention in 93% of children with FnM, compared to 15% and 9.7% of children in the non-FnM group (p=0.0001). Complex post-operative course was frequent for the FnM group: 88% of children had persistent fever, 46% had additional imaging and 14% additional surgical intervention. Children with FnM were treated with IV antibiotics for an average of 22 compared to seven days for non-FnM children (p=0.0001). CONCLUSIONS: Fusobacterium should be suspect in a child presenting with AM, a septic appearance, high fever and increased inflammation markers. A regimen of two antibiotics should be empirically started, then proceed to imaging and early surgical intervention. A relatively slow post-operative recovery process should be expected; however, prognosis is good.

Assessment of Angular Insertion-Depth of Bilateral Cochlear Implants Using Plain X-ray Scans.

OBJECTIVE: To evaluate in cochlear implant patients, the feasibility and reliability of angular depth of insertion (aDOI) measurements using plain x-ray scans. STUDY DESIGN: Retrospective study where three observers independently evaluated and compared intraoperative anterior-posterior and oblique x-ray scans. SETTING: A tertiary pediatric medical center. PATIENTS: Included were 50 children (100 ears) who underwent bilateral simultaneous cochlear implantation during 2008 to 2015. MAIN OUTCOME MEASURES: Inter-rater agreement of aDOI measured in plain x-ray scans; effect of head position on measured aDOI; and symmetry of aDOI between patients' ears in bilateral simultaneous cochlear implantations. RESULTS: Differences in the average aDOI measurements among the three observers ranged between 2 and 7 degrees. There was high inter-rater agreement (R = 0.99, p < 0.01) among all observers, and strong correlations between each pair of observers (0.92-0.99). Head rotation of 45 degrees (between the two views) resulted in a median difference in aDOI of 14 degrees, with excellent correlation among the observers. The rate of asymmetry was high, with a median difference of 39 degrees and up to 220 degrees between ears. CONCLUSIONS: Assessment of aDOI using intraoperative plain x-rays is efficient and reliable. The effect of head positioning on measurement is small. Further studies are needed to evaluate the effect of aDOI and insertion symmetry on functional outcomes.

Hearing in Noise With Unilateral Versus Bilateral Bone Conduction Hearing Aids in Adults With Pseudo-conductive Hearing Loss.

OBJECTIVE: The role of bilateral bone conduction amplification in patients with bilateral conductive hearing loss is unclear because cranial attenuation is usually considered negligible, and both cochleae can be stimulated with similar efficacy by each device. The aim of the study was to determine if bilateral bone-conduction hearing aids can improve hearing in noise in a homogeneous group of normal-hearing subjects with bilateral pseudo-conductive hearing loss. STUDY DESIGN: Prospective, comparative. SETTING: Department of Communication Sciences and Disorders in University of Haifa, Israel. SUBJECTS: Department of Communication Sciences and Disorders in University of Haifa, Israel. INTERVENTIONS: Induction of bilateral pseudo-conductive hearing loss of more than 35 dB using earplugs and earmuffs. MAIN OUTCOME MEASURES: Hearing quality on blinded comparison of unilateral versus bilateral amplification with bone-conduction hearing aids under different locations of noise. RESULTS: Unilateral and bilateral amplification had similar efficacy when both signal and noise were presented from the front. However, bilateral amplification was significantly better when signals were presented from the front and noise was presented from both sides (SNR -10: 92% vs. 84%, p = 0.001; SNR -15: 84% vs. 78%, p = 0.005). Analysis of subject responses on blinded questionnaires revealed that 81% found hearing easier with bilateral amplification. Noise was reported to be more disturbing with unilateral amplification by 55% of the subjects and with bilateral amplification, by 9%. CONCLUSIONS: Bilateral amplification with bone conduction devices can improve understanding in noise in the binaural squelch setting. Subjective improvements with bilateral bone-conduction aids included better sound quality and reduced noise disturbance. These findings are consistent with the binaural mechanism of spectral release from masking.

Vestibular and balance function is often impaired in children with profound unilateral sensorineural hearing loss.

RATIONALE: Children with unilateral deafness could have concurrent vestibular dysfunction which would be associated with balance deficits and potentially impair overall development. The prevalence of vestibular and balance deficits remains to be defined in these children. METHODS: Twenty children with unilateral deafness underwent comprehensive vestibular and balance evaluation. RESULTS: Retrospective review revealed that more than half of the cohort demonstrated some abnormality of the vestibular end organs (otoliths and horizontal canal), with the prevalence of end organ specific dysfunction ranging from 17 to 48% depending on organ tested and method used. In most children, impairment occurred only on the deaf side. Children with unilateral deafness also displayed significantly poorer balance function than their normal hearing peers. CONCLUSIONS: The prevalence of vestibular dysfunction in children with unilateral deafness is high and similar to that of children with bilateral deafness. Vestibular and balance evaluation should be routine and the functional impact of combined vestibulo-cochlear sensory deficits considered.

Emberger syndrome: A rare association with hearing loss.

Emberger Syndrome (ES) is a rare genetic disorder characterized by lymphedema and myelodysplasia. It is also associated with hearing loss. The genetic mutations associated with ES are not part of the comprehensive 80 gene next generation sequencing (NGS) panel. As a result, the otolaryngologist should maintain an index of suspicion for ES in any child with SNHL who presents repeatedly with recurrent infections, lymphedema and/or cutaneous warts. This paper describes the clinical evolution and management of two children who were followed up for hearing loss and eventually were diagnosed with ES.

Acute mastoiditis in children with a cochlear implant.

OBJECTIVES: Cochlear implantation is performed at a young age, when children are prone to acute otitis media. Acute mastoiditis is the most common complication of otitis media, but data on its management in the presence of a cochlear implant are sparse. The objective of this study was to assess the characteristics, treatment, and outcome of acute mastoiditis in children with a cochlear implant. METHODS: The medical files of all children who underwent cochlear implantation at a pediatric tertiary medical center in 2000-2014 were retrospectively reviewed. Those diagnosed with acute mastoiditis after implantation were identified, and data were collected on demographics, history, presentation, method of treatment, complications, association with untreated otitis media with effusion, and long-term middle-ear sequelae. RESULTS: Of the 370 children (490 ears) who underwent cochlear implantation, 13 (3.5%) were treated for acute mastoiditis (median age at acute mastoiditis, 32 months). Nine had a pre-implantation history of chronic secretory or acute recurrent otitis media, and 5 had been previously treated with ventilation tubes. In all 9 children who had unilateral cochlear implant, the acute mastoiditis episode occurred in the implanted ear. The time from implantation to mastoiditis was 5-61 months. The same treatment protocol as for normal-hearing children was followed, with special attention to the risk of central nervous system complications. Primary treatment consisted of myringotomy with intravenous administration of wide-spectrum antibiotics. Surgical drainage was performed in 8 out of 13 patients, with (n=7) or without (n=1) ventilation-tube insertion, to treat subperiosteal abscess or because of lack of symptomatic improvement. There were no cases of intracranial complications or implant involvement or need for a wider surgical approach. No middle-ear pathology was documented during the average 3.8-year follow-up. CONCLUSIONS: The relatively high rate of acute mastoiditis and subperiosteal abscess in children with a cochlear implant, predominantly involving the implanted ear, supports the suggestion that recent mastoidectomy may be a risk factor for these complications. Despite the frequent need for drainage, more extensive surgery is usually unnecessary, and recovery is complete and rapid. As infections can occur even years after cochlear implantation, children with otitis media should be closely followed, with possible re-introduction of ventilation tubes.

Is It Necessary to Treat Otitis Media With Effusion (OME) Prior to Cochlear Implantation? Results Over a Long-term Follow-up.

OBJECTIVES: Evaluate the intra- and postoperative effects of untreated otitis media with effusion (OME) in cochlear implant (CI) patients, and to assess the role of ventilation tube (VT) introduction before implantation. STUDY DESIGN: A retrospective chart review. SETTING: Tertiary referral center. PATIENTS: CI patients, aged 10 years or younger, implanted during 2009 to 2013. INTERVENTIONS: Cases were divided into three groups: 1) normal aerated middle ear before CI, 2) OME treated with VT, and 3) untreated OME. MAIN OUTCOME MEASURE(S): Intraoperative and postoperative findings and complications. RESULTS: One hundred ninety-four cases (implanted ears) were included. Ninety-nine aerated, 39 treated with VT, and 56 with untreated OME. Mean age at implantation was 3.1, 2.1, and 1.6 years, respectively. Granulations and edema were significantly more common in untreated OME than aerated ears (62% vs. 7%, p <0.001). VT reduced the rate of these findings (46%) but not with statistical significance (p = 0.1) compared with untreated OME. Intraoperative findings were all manageable and were not associated with higher perioperative complication rates. The rates of early and late postoperative complications were low in all groups, with no significant differences between groups. Tympanic membrane perforations were encountered in two patients after VT extrusion. Rate of otorrhea was 20% during the first year after implantation and 5% at last follow up. CONCLUSION: Our results suggest that CI candidates with OME can be safely implanted without preimplantation VT insertion. Implanting patients with untreated OME allows earlier implantation. CI surgery can be more challenging in the presence of effusion; however, intraoperative findings are manageable.