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.

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.

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.

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.

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.

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.

[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.

Postoperative Intracochlear Electrocochleography in Pediatric Cochlear Implant Recipients: Association to Audiometric Thresholds and Auditory Performance.

OBJECTIVES: The aim of this study was to compare intracochlear-recorded cochlear microphonics (CM) responses to behavioral audiometry thresholds in young children, with congenital hearing loss, 2 to 5 years after cochlear implantation early in life. In addition, differences in speech and auditory outcomes were assessed among children with and without residual hearing. DESIGN: The study was conducted at a tertiary, university-affiliated, pediatric medical center. CM responses by intracochlear electrocochleography technique were recorded from 102 implanted ears of 60 children and those responses correlated to behavioral audiometry thresholds at 0.125 to 2 kHz frequencies. All children had received Advanced Bionics cochlear implant with High Focus J1 or MidScala electrodes, along with extensive auditory rehabilitation before and after implantation, including the use of conventional hearing aids. Speech Spatial and Hearing Qualities, Category of Auditory Performance scale, and educational settings information were obtained for each participant. Those cochlear implantation (CI) outcomes were compared between children with or without residual CM responses. RESULTS: Two distinctive CM responses patterns were found among the implanted children. Of all ears diagnosed with cochlear hearing loss (n = 88), only in 29 ears, clear CM responses were obtained. In all other ears, no CM responses were obtained at the maximum output levels. The CM responses were highly correlated with coefficients ranging from 0.7 to 0.83 for the audiometric behavioral thresholds at 0.125 to 2 kHz frequency range. Of all ears diagnosed with auditory neuropathy syndrome disorder (n = 14), eight ears had residual hearing and recordable CM postimplantation. The other six ears showed no recordable CM responses at maximum output levels for all tested frequencies. Those showed supposedly better CM responses than the behavioral audiometry threshold, however with poor correlations with tested frequency thresholds. Children with residual hearing showed significantly better auditory outcomes with CI, compared with those without residual hearing. CONCLUSIONS: In children with congenital cochlear hearing loss, the objective CM intracochlear responses can reliably predict the residual audiometric threshold. However, in children with auditory neuropathy syndrome disorder, the CM thresholds did not match the behavioral audiometric responses. Postoperatively, children with recordable CM responses, showing preserved residual hearing, demonstrated better CI outcomes.

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.

Tympanomastoidectomy for Cholesteatoma in Children: Audiometric Results.

OBJECTIVES: The aim of this study was to investigate the audiometric outcomes of tympanomastoidectomy in children with cholesteatoma and to evaluate factors that may affect outcomes. METHODS: A retrospective cohort study was conducted. All pediatric patients diagnosed with cholesteatoma who underwent primary canal wall-up tympanomastoidectomy with or without ossicular reconstruction from 2009 to 2016 at a tertiary university-affiliated pediatric medical center were included. Pure tone average (PTA) and air-bone gap (ABG) at 0.5, 1, 2, and 4 kHz were compared before and after surgery. RESULTS: The cohort included 100 children (104 tympanomastoidectomies) of mean age 10.35 ± 3.6 years. The mean duration of follow-up was 35.4 ± 24.1 months. At surgery, 22 patients (21.2%) had intact ossicular chains, and 82 (78.8%) had ossicular discontinuity. Fifty-three patients (51%) underwent ossicular reconstruction, either partial (34 patients [64.1%]) or total (19 patients [35.8%]). In comparison with preoperative audiometry, there was a statistically significant improvement in PTA-ABG at the first postoperative examination (27.5 ± 12.7 vs 21.1 ± 11.1 dB, P = .001) and at the end of follow-up (20.2 ± 12.7 dB, P = .003) . Overall, values of less than 20 dB were measured in 34% of patients preoperatively and 73% postoperatively. On subgroup analysis, in patients who underwent any ossicular reconstruction, PTA-ABG was significantly improved relative to the preoperative value (28.1 ± 14.7 dB) at both the first (19.1 ± 11.9 dB, P = .006) and last (22.4 ± 15.1 dB, P = .011) audiometry examinations. CONCLUSIONS: Hearing outcomes after pediatric cholesteatoma surgery are diverse and related to several factors. The authors found that ossicular reconstruction improves audiometric outcomes, and the changes remain stable over time. LEVEL OF EVIDENCE: IIB.

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.

Auditory Performance and Electrical Stimulation Measures in Cochlear Implant Recipients With Auditory Neuropathy Compared With Severe to Profound Sensorineural Hearing Loss.

OBJECTIVES: The aim of the study was to compare auditory and speech outcomes and electrical parameters on average 8 years after cochlear implantation between children with isolated auditory neuropathy (AN) and children with sensorineural hearing loss (SNHL). DESIGN: The study was conducted at a tertiary, university-affiliated pediatric medical center. The cohort included 16 patients with isolated AN with current age of 5 to 12.2 years who had been using a cochlear implant for at least 3.4 years and 16 control patients with SNHL matched for duration of deafness, age at implantation, type of implant, and unilateral/bilateral implant placement. All participants had had extensive auditory rehabilitation before and after implantation, including the use of conventional hearing aids. Most patients received Cochlear Nucleus devices, and the remainder either Med-El or Advanced Bionics devices. Unaided pure-tone audiograms were evaluated before and after implantation. Implantation outcomes were assessed by auditory and speech recognition tests in quiet and in noise. Data were also collected on the educational setting at 1 year after implantation and at school age. The electrical stimulation measures were evaluated only in the Cochlear Nucleus implant recipients in the two groups. Similar mapping and electrical measurement techniques were used in the two groups. Electrical thresholds, comfortable level, dynamic range, and objective neural response telemetry threshold were measured across the 22-electrode array in each patient. Main outcome measures were between-group differences in the following parameters: (1) Auditory and speech tests. (2) Residual hearing. (3) Electrical stimulation parameters. (4) Correlations of residual hearing at low frequencies with electrical thresholds at the basal, middle, and apical electrodes. RESULTS: The children with isolated AN performed equally well to the children with SNHL on auditory and speech recognition tests in both quiet and noise. More children in the AN group than the SNHL group were attending mainstream educational settings at school age, but the difference was not statistically significant. Significant between-group differences were noted in electrical measurements: the AN group was characterized by a lower current charge to reach subjective electrical thresholds, lower comfortable level and dynamic range, and lower telemetric neural response threshold. Based on pure-tone audiograms, the children with AN also had more residual hearing before and after implantation. Highly positive coefficients were found on correlation analysis between T levels across the basal and midcochlear electrodes and low-frequency acoustic thresholds. CONCLUSIONS: Prelingual children with isolated AN who fail to show expected oral and auditory progress after extensive rehabilitation with conventional hearing aids should be considered for cochlear implantation. Children with isolated AN had similar pattern as children with SNHL on auditory performance tests after cochlear implantation. The lower current charge required to evoke subjective and objective electrical thresholds in children with AN compared with children with SNHL may be attributed to the contribution to electrophonic hearing from the remaining neurons and hair cells. In addition, it is also possible that mechanical stimulation of the basilar membrane, as in acoustic stimulation, is added to the electrical stimulation of the cochlear implant.

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.

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.

Towards a Unified Testing Framework for Single-Sided Deafness Studies: A Consensus Paper.

BACKGROUND: While hearing aids for a contralateral routing of signals (CROS-HA) and bone conduction devices have been the traditional treatment for single-sided deafness (SSD) and asymmetric hearing loss (AHL), in recent years, cochlear implants (CIs) have increasingly become a viable treatment choice, particularly in countries where regulatory approval and reimbursement schemes are in place. Part of the reason for this shift is that the CI is the only device capable of restoring bilateral input to the auditory system and hence of possibly reinstating binaural hearing. Although several studies have independently shown that the CI is a safe and effective treatment for SSD and AHL, clinical outcome measures in those studies and across CI centers vary greatly. Only with a consistent use of defined and agreed-upon outcome measures across centers can high-level evidence be generated to assess the safety and efficacy of CIs and alternative treatments in recipients with SSD and AHL. METHODS: This paper presents a comparative study design and minimum outcome measures for the assessment of current treatment options in patients with SSD/AHL. The protocol was developed, discussed, and eventually agreed upon by expert panels that convened at the 2015 APSCI conference in Beijing, China, and at the CI 2016 conference in Toronto, Canada. RESULTS: A longitudinal study design comparing CROS-HA, BCD, and CI treatments is proposed. The recommended outcome measures include (1) speech in noise testing, using the same set of 3 spatial configurations to compare binaural benefits such as summation, squelch, and head shadow across devices; (2) localization testing, using stimuli that rove in both level and spectral content; (3) questionnaires to collect quality of life measures and the frequency of device use; and (4) questionnaires for assessing the impact of tinnitus before and after treatment, if applicable. CONCLUSION: A protocol for the assessment of treatment options and outcomes in recipients with SSD and AHL is presented. The proposed set of minimum outcome measures aims at harmonizing assessment methods across centers and thus at generating a growing body of high-level evidence for those treatment options.

Pattern of hearing loss following cochlear implantation.

Cochlear implantation is associated with deterioration in hearing. Despite the fact that the damage is presumed to be of sensory origin, residual hearing is usually assessed by air-conduction thresholds alone. This study sought to determine if surgery may cause changes in air- and bone-conduction thresholds producing a mixed-type hearing loss. The sample included 18 patients (mean age 37 years) with an air-bone gap of 10 dB over three consecutive frequencies and measurable masked and reliable bone-conduction thresholds of operated and non-operated ears who underwent cochlear implant surgery. All underwent comprehensive audiologic and otologic assessment and imaging before and after surgery. The air-bone gap in the treated ears was 17-41 dB preoperatively and 13-59 dB postoperatively over 250-4,000 Hz. Air-conduction thresholds in the treated ears significantly deteriorated after surgery, by a mean of 10-21 dB. Bone-conduction levels deteriorated nonsignificantly by 0.8-7.5 dB. The findings indicate that the increase in air-conduction threshold after cochlear implantation accounts for most of the postoperative increase in the air-bone gap. Changes in the mechanics of the inner ear may play an important role. Further studies in larger samples including objective measures of inner ear mechanics may add information on the source of the air-bone gap.

Pediatric otogenic sigmoid sinus thrombosis: 12-Year experience.

OBJECTIVES: Otogenic sigmoid sinus thrombosis is a rare complication of acute otitis media. Treatment remains controversial particularly regarding extent of surgical intervention. The aim of the study was to review the 12-year experience of a major medical center with the treatment of sigmoid sinus thrombosis in children. METHODS: Retrospective case series identified by database review in a tertiary university-affiliated pediatric medical center. Twenty-four children aged 7-155 months were treated for sigmoid sinus thrombosis from 2000 through 2011. RESULTS: The transverse sinus was also involved in 10 patients, and the jugular vein, in 4. Acute otitis media with mastoiditis was the causative factor in all cases. Subperiosteal abscess was diagnosed in 21 patients, 11 with epidural involvement. Treatment in all cases consisted of broad-spectrum antibiotics and ventilation tube insertion. Twenty-one children (87.5%) underwent mastoidectomy with removal of bone covering the sigmoid sinus to drain pus and remove granulations from the epidural cavity, without aspiration or sinus drainage. Twenty-two patients received low-molecular-weight heparin for 3-6 months postoperatively. Children infected with Fusobacterium necrophorum had a longer and more severe course with coexisting osteomyelitis. There were no neurologic sequelae or hematologic complications. Follow-up imaging, performed in 15 children, revealed partial or full recanalization in 87%. CONCLUSIONS: Relatively conservative surgical intervention appears to yield good results in children with sigmoid sinus thrombosis consequent to acute otitis media. Anticoagulants are safe if correctly administered and may prevent extension of the thrombus.