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.

The protective effect of aspirin-induced temporary threshold shift in an animal model of cisplatin-related ototoxicity.

PURPOSE: The purpose of this study was to evaluate whether induction of temporary threshold shift (TTS) with aspirin prior to cisplatin exposure can prevent or minimize cisplatin detrimental effects on hearing. METHODS: We randomly divided BALB mice into three groups: (1) cisplatin only, (2) aspirin only, and (3) combined aspirin/cisplatin. Cisplatin was administered as a single intraperitoneal injection of 14 mg/kg. Aspirin was administered for three weeks via intraperitoneal injection of 200 mg/kg sodium salicylate, twice daily. Air conduction thresholds were recorded using Auditory Brainstem Responses (ABR). Cochleae were harvested and cochlear hair cells were counted using a scanning electron microscope (SEM). RESULTS: Aspirin-induced TTS have reached an average of 30.05±16.9 dB after 2 weeks. At 60 days, cisplatin-only treated mice experienced an average threshold shifts of 50.7 dB at 4 kHz, 35.16 dB at 8 kHz, 70 dB at 16 kHz, 53.1 dB at 32 kHz. All threshold shifts were significantly worse than for cisplatin/aspirin treated mice with TTS of 11.85 dB at 4 kHz, 3.58 dB at 8 kHz, 16.58  dB at 16 kHz, 20.41 dB at 32 kHz (p < 0.01). Cochlear cell count with SEM has shown reduction in the number of both inner and outer hair cells in the mid-turn in cisplatin treated mice. CONCLUSION: Aspirin induced TTS can protect from cisplatin-induced ototoxicity. This beneficial effect was demonstrated by auditory thresholds as well as SEM. Larger pre-clinical and clinical studies are still needed to confirm these findings.

The effect of environmental noise isolation on bronchiolitis severity in hospitalised children.

AIM: Bronchiolitis is an infectious disease, with no effective treatment. Music and Mozart's works specifically are known to have a positive effect on physiological parameters, while noise is considered harmful. We aim to evaluate the short-term effect of environmental noise detachment and/or music listening on the course of bronchiolitis in hospitalised children. METHODS: This is a prospective, double-blinded randomised controlled trial. Patients were divided into three intervention groups: 1-Mozart's Sonata, 2-instrumental music, 3-silence. Music was heard via media players and soundproof headphones. Disease severity was evaluated before and after intervention using the Modified Tal score. RESULTS: Seventy music sessions were included in the analysis (Mozart n = 23, instrumental n = 22, silence n = 25). A one-point drop in the average bronchiolitis severity score was observed in all three groups from 7.1 (CI 95%, 5 to 9.2) to 6.1 (CI 95%, 4.3 to 7.9), p < 0.001. No significant difference was found between the three groups with respect to change in the severity score before and after the intervention. CONCLUSION: Isolation from disturbing sounds heard in paediatric departments could be considered a simple non-invasive intervention in children hospitalised with bronchiolitis. Further studies are warranted to evaluate long-term effects of this intervention and the specific effect of music.

Cortical Auditory Evoked Potentials Recorded Directly Through the Cochlear Implant in Cochlear Implant Recipients: a Feasibility Study.

OBJECTIVES: The use of objective measures in cochlear implant (CI) mapping, has greatly contributed to the refinement of the setting of audible and comfortable stimulation levels, which serve as the basis of the mapping process, especially in cases of infants and young children. In addition, objective measures can also confirm the integrity of the CI system. Current CI objective measures mainly reflect neural activity from the auditory nerve and brainstem site. An objective cortical CI measure that reflects directly central auditory activity is greatly needed, especially since it is closely related to CI outcomes in both children and adults. Recording the brain activity currently requires an external evoked potential (EP) system including scalp electrodes, rendering it impractical for widespread clinical use. This study aimed to assess the feasibility of recording cortical auditory evoked potentials (CAEPs) directly and solely through the cochlear implant in response to external acoustic stimulation in the non-implanted ear. DESIGN: A total of nine CI users (four females and five males) participated, including seven post-lingual adults (23 to 72 years), and two pediatric cases, one teenager (15 years), and one child (8 years)-both pre-lingual. All participants had a residual hearing in the ear contralateral to the ear with CI. CAEPs were recorded in the implanted ear in response to acoustic stimulation of the non-implanted ear, consisting of a brief tonal stimulus at comfortable listening levels. Recordings used an intracranial montage consisting of an intracochlear apical electrode (active) and one of the two ( case and ring ) extra-cochlear implanted electrodes serving as reference electrodes. The CI CAEPs were compared with a single-channel conventional CAEP recording obtained simultaneously via scalp electrodes (Fz-mastoid) using a standard EP system and an external trigger from the CI system. Statistical comparisons were made between the CI and the scalp recorded CAEPs and for differences between the CI CAEP measures acquired using the ring and the case as the reference electrode. RESULTS: CAEPs recorded directly and solely through the CI were equivalent to the standard scalp recorded CAEP responses. CAEP responses acquired using the case electrode as the reference were highly correlated in terms of morphology, latencies, and amplitudes of the CAEP components. The CI CAEP latencies of the two pediatric cases were consistent with their normal developed age group and delayed relative to adult CAEP latencies, as expected. CONCLUSIONS: This study demonstrated the feasibility of recording long latency CAEPs directly and solely through CI in adults with residual hearing, in response to acoustic stimulation of the non-implanted ear. The CI CAEPs closely resembled the CAEPs recorded simultaneously by an external EP system and via scalp electrodes. The ability to record directly from the implant, without the need of an external recording system, presents an innovative method with many clinical and research implications.

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.

The Impact of Superior Canal Dehiscence on Power Absorbance, Otoacoustic Emissions, and Hearing in Fat Sand Rats.

BACKGROUND: Superior Semicircular Canal Dehiscence (SSCD) may lead to vestibular and auditory impairments. OBJECTIVE: To study the effects of power absorbance (PA), Distortion Product Otoacoustic emissions (DPOAE), and hearing thresholds in normal ears of fat sand rats, after a bullotomy, creation and patching. METHODS: SSCD was performed unilaterally in eight normal hearing animals while the contra-lateral un-operated ear was used as a control. Measures included auditory brain stem responses thresholds for air and bone conduction stimuli, DPOAEs and PA at peak pressure. RESULTS: The normal PA pattern of the animals grossly resembled that of human ears. A bullotomy generated specific, large and significant (p < 0.0001) changes in PA without altering hearing thresholds. SSCD significantly decreased PA at low (p < 0.02) and increased at high frequencies (p < 0.03), but on a smaller scale than the bullotomy. SSCD, induced a mean air-bone gaps of 24.3 for clicks, and 31.2 dB for 1 kHz TB. SSCD also increased the DPOAEs levels by mean of 10.1 dB SPL (p < 0.03). Patching the dehiscence, reversed partially the PA changes, the auditory threshold shifts, and the DPOAEs levels to pre-SSCD values. CONCLUSIONS: SSCD affects both incoming and emitting sounds from the ear, probably due to its effect on cochlear impedance and stiffness of the middle and inner ear. The presence of DPOAEs and ABGs indicated a "third window" disease, i.e., SSCD. Due to similar PA patterns after bullotomy and SCCD, PA alone has limited diagnostic yield for patients with SCCD.

[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.

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.

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.

Treatment of congenital cytomegalovirus beyond the neonatal period: an observational study.

Recently, valganciclovir treatment of symptomatic congenital cytomegalovirus (cCMV) disease, commenced during the neonatal period (≤ 4 weeks), was found to improve hearing and developmental outcome. However, many children (symptomatic or asymptomatic at birth) present only after 4 weeks of age. The purpose of this observational retrospective study was to describe the outcome and safety of valganciclovir therapy in infants with cCMV who started treatment > 4 weeks of life. Of the 91children who started antiviral treatment > 4 weeks of age, 66/298 (22.2%) were symptomatic at birth; 25/217 (11.5%) were asymptomatic at birth. Treatment was initiated on average at 14 weeks of age (range 5-77 weeks) and at 53.3 weeks (range 12-156 weeks), respectively. Of the 45 affected ears in the symptomatic group, 30 (66.7%) improved and only 2 (4.4%) deteriorated, with most of the improved ears (27/30, 90%) returning to normal. In the asymptomatic group, late-onset treatment was initiated and out of the 42 deteriorated ears, 38 (90.5%) improved after at least 1 year of follow-up. Hematological adverse events, i.e., neutropenia, were noted in a minority of cases (4.4%).Conclusion: Our study demonstrates the benefits and safety aspects of treating symptomatic and asymptomatic children with cCMV even beyond the recommended neonatal period.What is Known:• Valganciclovir treatment of symptomatic congenital cytomegalovirus (cCMV) disease, commenced during the neonatal period, is beneficial in improving hearing and developmental outcome.• However, data of treatment started beyond the neonatal period is lacking.What is New:• Our study demonstrates the benefits of treating symptomatic children with cCMV as well as asymptomatic children that develop late-onset hearing loss even beyond the recommended neonatal period.• This was true for symptomatic children who presented > 4 weeks as well as to those were asymptomatic at birth but experienced late hearing deterioration.

Cochlear Implantation and Social-Emotional Functioning of Children with Hearing Loss.

This study examined the contribution of cochlear implants (CIs) to the social-emotional functioning of children who are deaf or hard of hearing (dhh). Sixty-three parents of children who are dhh participated in the study. Thirty children were CI users and 32 used hearing aids (HAs). They completed the Strengths and Difficulties Questionnaire and a background questionnaire. Parents of children with CIs reported lower levels of hyperactivity/inattention and higher levels of pro-social behavior compared to parents of children with HAs. Additionally, older age when hearing loss was detected was related to more pro-social behavior, and age at implantation among CI users was negatively correlated with children's hyperactivity/inattention and conduct problems. These findings add to the existing knowledge about the many benefits of CIs for individuals with hearing loss and emphasize the possible impact of early implantation to children's social-emotional functioning.

Valganciclovir Is Beneficial in Children with Congenital Cytomegalovirus and Isolated Hearing Loss.

OBJECTIVE: To evaluate the efficacy of antiviral treatment for infants with congenital cytomegalovirus (cCMV) with isolated sensorineural hearing loss (SNHL). STUDY DESIGN: Data were reviewed retrospectively for infants with isolated SNHL who received prolonged antiviral treatment between 2005 and 2017. Hearing status was evaluated for infants who had been followed for >1 year. RESULTS: Among 329 infants treated for cCMV, 59 (18%) were born with isolated SNHL. Hearing impairment was unilateral in 38 (64.4%) infants and bilateral in 21 (35.6%). Of the 80 affected ears at baseline, 55 (68.8%) improved, and only 2 (2.5%) deteriorated. Most of the improved ears (53/55 = 96.3%) returned to normal hearing with no deterioration observed in the ears that were unaffected at baseline. On best ear evaluation, of 21 infants who had bilateral hearing loss, 16 (76.1%) improved (93.7% regaining normal functional hearing); none deteriorated. CONCLUSION: Infants born with isolated SNHL due to cCMV were found to benefit from prolonged antiviral treatment. These children (and ears) showed significant improvement in hearing status and no deterioration of unaffected ears at baseline. Our data serve as observational evidence of the benefits of antiviral treatment in these children. Avoiding treatment of these children due to the lack of prospective data is debatable.

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.

Congenital Cytomegalovirus Infection After a Multiple Birth Pregnancy.

BACKGROUND: Congenital cytomegalovirus (cCMV) infection is an important cause of hearing loss and neurodevelopment delay. While data on vertical transmission and neonatal outcome after singleton pregnancy with cCMV are well established, only scarce reports have addressed cCMV in multiple birth pregnancies. Furthermore, no studies have yet compared the outcome after birth and long-term follow-up of children with cCMV born after a singleton versus multiple pregnancies. METHODS: Infant outcome after birth of symptomatic versus asymptomatic infection was compared for infants born with cCMV after multiple (study group) and singleton (control group) pregnancies in a 1:2 ratio. RESULTS: Of 508 infants diagnosed with cCMV, 25 (4.9%) were born after a multiple pregnancy. Children in the study and control groups did not differ in terms of specific prenatal CMV investigations including amniocentesis and brain magnetic resonance imaging studies. However, prematurity rates were significantly higher in the study compared with control group (52% vs. 4%, P < 0.001). There was a higher rate of symptomatic cCMV infection in the study group than in the controls (48% vs. 14%, P < 0.001). Hearing impairment at birth was also more frequent in the study group (32% vs. 8%, P = 0.016). A long-term follow-up demonstrated that children in the study group had higher rates of neurologic sequelae (hearing impairment or neurodevelopmental delay) compared with children in the control group (20% vs. 4%, P = 0.016). CONCLUSIONS: Infants with cCMV born after multiple birth pregnancies are born earlier and have a higher risk of symptomatic disease at birth and worse long-term neurologic outcome than those born after a singleton pregnancy. This important group of children warrants meticulous prenatal and postnatal care.

An Objective Estimation of Air-Bone-Gap in Cochlear Implant Recipients with Residual Hearing Using Electrocochleography.

Although, cochlear implants (CI) traditionally have been used to treat individuals with bilateral profound sensorineural hearing loss, a recent trend is to implant individuals with residual low-frequency hearing. Notably, many of these individuals demonstrate an air-bone gap (ABG) in low-frequency, pure-tone thresholds following implantation. An ABG is the difference between audiometric thresholds measured using air conduction (AC) and bone conduction (BC) stimulation. Although, behavioral AC thresholds are straightforward to assess, BC thresholds can be difficult to measure in individuals with severe-to-profound hearing loss because of vibrotactile responses to high-level, low-frequency stimulation and the potential contribution of hearing in the contralateral ear. Because of these technical barriers to measuring behavioral BC thresholds in implanted patients with residual hearing, it would be helpful to have an objective method for determining ABG. This study evaluated an innovative technique for measuring electrocochleographic (ECochG) responses using the cochlear microphonic (CM) response to assess AC and BC thresholds in implanted patients with residual hearing. Results showed high correlations between CM thresholds and behavioral audiograms for AC and BC conditions, thereby demonstrating the feasibility of using ECochG as an objective tool for quantifying ABG in CI recipients.

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.

Positive IgM in Congenital CMV Infection.

Neonatal serum detection of cytomegalovirus (CMV) immunoglobulin M (IgM) has low sensitivity in identifying congenital cytomegalovirus (cCMV). Several reports have endeavored to associate the presence/absence of IgM to disease severity. Data were collected for all infants with cCMV followed in our clinic. Infant outcome after birth was compared between infants who tested positive or negative. Sensitivity of positive IgM in diagnosing cCMV was 40.7%. The rate of symptomatic disease in those who tested positive was statistically higher (67.7%, P < .001). Odds ratio for symptomatic disease in infants with positive IgM born after a maternal primary infection was 3.47 (95% confidence interval = 1.7-7.1). Positive IgM was found in only 48.8% of symptomatic and 22.1% of asymptomatic children. Our results demonstrated a low sensitivity of IgM in diagnosing cCMV. However, while a positive IgM antibody for CMV is associated with a more symptomatic disease, it does not serve as a precise laboratory marker for a severity.

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.

Cochlear implantation via round window or cochleostomy: Effect on hearing in an animal model.

OBJECTIVES/HYPOTHESIS: Cochlear implantation in patients with residual hearing has increased interest in hearing preservation. Two major surgical approaches to implantation have been devised: via the round window membrane and through cochleostomy. However, the advantages of either approach on hearing preservation have not been established. Due to the great inter- and intravariability among implantees, the current study used a normal-hearing animal model to compare the effect of the two methods on hearing. STUDY DESIGN: Animal study. METHODS: Thirteen fat sand rats were studied, in which 13 ears were implanted through cochleostomy and 13 via the round window. Hearing thresholds were determined by auditory brainstem responses to air and bone conduction at low and high auditory stimuli. RESULTS: The results indicated that each stage of the surgery, primarily the opening of the membranous labyrinth, was accompanied by significant deterioration in hearing. Hearing loss was mainly conductive, with no significant differences between the surgical approaches. CONCLUSIONS: Both surgical approaches carry similar risk of hearing loss. LEVEL OF EVIDENCE: NA Laryngoscope, 126:E375-E378, 2016.