Etiology and therapy indication for cochlear implantation in children with single-sided deafness : Retrospective analysis.

OBJECTIVE: The characteristics of children with single-sided deafness (SSD) who become candidates for unilateral cochlear implantation (uCI) were identified. STUDY DESIGN: In all, 118 children with SSD presenting from 2013-2019 to a tertiary pediatric children's hospital were retrospectively assessed regarding candidacy for uCI. RESULTS: Of the 118 children, 103 had completed uCI candidacy assessment, while 15 were undergoing this assessment at the time of review. More than half of children did not go on to implantation (63/103, 61%), with the 2 main reasons being (1) half (31/63) did not meet candidacy criteria for implantation, most commonly due to cochlear nerve aplasia/hypoplasia (31/82 who were assessed with MRI, 38%) and (2) families (30/103; 29%) declined participation in the surgical arm of the trial. The most common etiologies of SSD in the 37/103 (36%) children who both met candidacy and consented to implantation were congenital cytomegalovirus (cCMV; 16/37, 43%), unknown (6/37, 16%), cochleovestibular anomaly and trauma (each 5/37, 14%). CONCLUSIONS: Many children with SSD who present for implant candidacy assessment do not ultimately receive uCI. Major factors contributing to noncandidacy are cochlear nerve aplasia and parental acceptance of the intervention. While approximately half of children with SSD in our cohort were candidates for implantation, only 1/3 of the total cohort proceeded with implantation with the main predictors of acceptability of this intervention being an etiology (i.e., cCMV) that carries risk of progressive deterioration in the better hearing ear or SSD that was sudden in onset. These findings provide important insight into this new population of cochlear implant users and the emerging acceptance of intervention in children with SSD.

The combination of vestibular impairment and congenital sensorineural hearing loss predisposes patients to ocular anomalies, including Usher syndrome.

The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having USH. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was performed to determine the ocular phenotype. All the cases had undergone ocular examination and electroretinogram (ERG). Patients with an abnormal ERG underwent genetic testing for USH. We found an underlying ocular abnormality in 81.81% (27/33) of cases; of which 75% had refractive errors, and 50% of those patients showed visual improvement with refractive correction. A total of 14 cases (42.42%; 14/33) had generalized rod-cone dysfunction on ERG suggestive of Usher syndrome type 1, confirmed by mutational analysis. This work shows that adding vestibular impairment as a criterion for requesting an eye exam and adding the ERG to detect USH increases the chances of detecting ocular anomalies, when compared with previous literature focusing only on congenital SNHL.

Binaural responses to a speech syllable are altered in children with hearing loss: Evidence from the frequency-following response.

BACKGROUND & RATIONALE: In prior work using non-speech stimuli, children with hearing loss show impaired perception of binaural cues and no significant change in cortical responses to bilateral versus unilateral stimulation. Aims of the present study were to: 1) identify bilateral responses to envelope and spectral components of a speech syllable using the frequency-following response (FFR), 2) determine if abnormalities in the bilateral FFR occur in children with hearing loss, and 3) assess functional consequences of abnormal bilateral FFR responses on perception of binaural timing cues. METHODS: A single-syllable speech stimulus (/dα/) was presented to each ear individually and bilaterally. Participants were 9 children with normal hearing (MAge = 12.1 ± 2.5 years) and 6 children with bilateral hearing loss who were experienced bilateral hearing aid users (MAge = 14.0 ± 2.6 years). FFR temporal and spectral peak amplitudes were compared between listening conditions and groups using linear mixed model regression analyses. Behavioral sensitivity to binaural cues were measured by lateralization responses as coming from the right or left side of the head. RESULTS: Both temporal and spectral peaks in FFR responses increased in amplitude in the bilateral compared to unilateral listening conditions in children with normal hearing. These measures of "bilateral advantage" were reduced in the group of children with bilateral hearing loss and associated with decreased sensitivity to interaural timing differences. CONCLUSION: This study is the first to show that bilateral responses in both temporal and spectral domains can be measured in children using the FFR and is altered in children with hearing loss with consequences to binaural hearing.

Toward a method of achieving balanced stimulation of bilateral auditory nerves: Evidence from children receiving matched and unmatched bilateral cochlear implants simultaneously.

OBJECTIVES: To identify whether mismatched bilateral cochlear implants compromise balanced stimulation of the two auditory nerves and establish asymmetric hearing in children. METHODS: Behavioural and electrophysiological measures were completed in 47 children receiving bilateral CIs in the same surgery (simultaneously): 27 children received a peri­modiolar N24RE array in one ear and a 422 anti-modiolar array in the other (experimental group) and 20 children received 2 peri­modiolar arrays (control group). Differences in current levels between the two devices were measured by electrically evoked compound action potentials (ECAPs) at the time of surgery. These data were compared with minimum and maximum comfortably loud levels programmed in each speech processor (T-levels, C-levels, respectively) after 12 months of bilateral CI use. Asymmetries in functional hearing between arrays were measured in open set speech perception testing between 3 to 5 years of CI use. RESULTS: Higher current levels were required from the anti-modiolar than peri­modiolar array to evoke balanced interaural ECAP amplitudes (mismatched group: mean ± SD difference: -9.9 ± 22.6; matched group: -0.8 ± 26.5). This difference was larger in the experimental group than control group (t = -2.51; p = 0.016) and remained constant with increases in current level from ECAP threshold to maximum amplitudes (dynamic range) in many but not all children in both groups. T and C-levels were poorly predictive of levels needed to evoke balanced ECAP amplitudes in children with mismatched devices (F(1, 312) = 1.3, p = 0.263). Speech perception scores were more asymmetric between ears in children using bilateral mismatched arrays (mean ± SD: 73.8 ± 16.4 at the peri­modiolar array; 57.7 ± 26.4 at the anti-modiolar array), compared to children with bilateral matched arrays (right ear: 78.0 ± 10.4; left ear: 74.9 ± 13.5). CONCLUSION: Higher current level requirements at the anti-modiolar array compared to the peri­modiolar array in children with bilateral mismatched CIs are not fully accounted for in device programming. Mismatched electrodes in children receiving bilateral cochlear implants increases the risk of asymmetric hearing.

Use it or lose it? Lessons learned from the developing brains of children who are deaf and use cochlear implants to hear.

In the present paper, we review what is currently known about the effects of deafness on the developing human auditory system and ask: Without use, does the immature auditory system lose the ability to normally function and mature? Any change to the structure or function of the auditory pathways resulting from a lack of activity will have important implications for future use through an auditory prosthesis such as a cochlear implant. Data to date show that deafness in children arrests and disrupts normal auditory development. Multiple changes to the auditory pathways occur during the period of deafness with the extent and type of change being dependent upon the age and stage of auditory development at onset of deafness, the cause or type of deafness, and the length of time the immature auditory pathways are left without significant input. Structural changes to the auditory nerve, brainstem, and cortex have been described in animal models of deafness as well in humans who are deaf. Functional changes in deaf auditory pathways have been evaluated by using a cochlear implant to stimulate the auditory nerve with electrical pulses. Studies of electrically evoked activity in the immature deaf auditory system have demonstrated that auditory brainstem development is arrested and that thalamo-cortical areas are vulnerable to being taken over by other competitive inputs (cross-modal plasticity). Indeed, enhanced peripheral sight and detection of visual movement in congenitally deaf cats and adults have been linked to activity in specific areas of what would normally be auditory cortex. Cochlear implants can stimulate developmental plasticity in the auditory brainstem even after many years of deafness in childhood but changes in the auditory cortex are limited, at least in part, by the degree of reorganization which occurred during the period of deafness. Consequently, we must identify hearing loss rapidly (i.e., at birth for congenital deficits) and provide cochlear implants to appropriate candidates as soon as possible. Doing so has facilitated auditory development in the thalamo-cortex and allowed children who are deaf to perceive and use spoken language.

Total Endoscopic Ear Surgery in management of cochleocele: A case series.

Cochleocele is an extrusion or herniation of the endosteum, through an incomplete stapes footplate, into the middle ear. The cochleocele may rupture resulting in a cerebrospinal fluid leak into the middle ear space causing a risk of menigitis. We report six cases of Incomplete Partition Type I with cochleocele which have all been successfully treated using a Totally Endoscopic Ear Surgery approach even during infancy. As the first two cases developed post-operative pseudomonas meningitis, preventative strategies are recommended.

Characterizing responses from auditory cortex in young people with several years of cochlear implant experience.

OBJECTIVE: To determine if cortical responses evoked by a cochlear implant in children who are deaf differ from normal and to characterize these differences in children who achieve good versus fair speech perception outcomes post-implantation. METHODS: Late latency-evoked potential responses were recorded at 28 scalp locations in 16 children who were deaf from infancy and experienced cochlear implant users. Speech perception measures indicated that 8 had good scores and 8 had fair scores. In each child, responses were evoked by 36ms electrical pulse trains delivered from a single-implant electrode at the apical and basal ends of the array and by 36ms tone bursts (0.5, 2, and 6kHz). Responses to the tone bursts were also recorded in 14 age-matched children with normal hearing. RESULTS: We found (1) a dominant positive wave in all implant users and (2) a larger than normal negative amplitude peak in users with fair speech perception scores which had similar scalp topography to N1 but did not show the expected changes in amplitude with stimulus frequency. CONCLUSIONS: Late latency-evoked potential responses in children using cochlear implants reflect abnormal and/or immature patterns of cortical activity. SIGNIFICANCE: Limitations in auditory skills with a cochlear implant in children may be due to developmental processes in the cortex which are either slow to mature or which mature abnormally.

Auditory brainstem activity and development evoked by apical versus basal cochlear implant electrode stimulation in children.

OBJECTIVE: The role of apical versus basal cochlear implant electrode stimulation on central auditory development was examined. We hypothesized that, in children with early onset deafness, auditory development evoked by basal electrode stimulation would differ from that evoked more apically. METHODS: Responses of the auditory nerve and brainstem, evoked by an apical and a basal implant electrode, were measured over the first year of cochlear implant use in 50 children with early onset severe to profound deafness who used hearing aids prior to implantation. RESULTS: Responses at initial stimulation were of larger amplitude and shorter latency when evoked by the apical electrode. No significant effects of residual hearing or age were found on initial response amplitudes or latencies. With implant use, responses evoked by both electrodes showed decreases in wave and interwave latencies reflecting decreased neural conduction time through the brainstem. Apical versus basal differences persisted with implant experience with one exception; eIII-eV interlatency differences decreased with implant use. CONCLUSIONS: Acute stimulation shows prolongation of basally versus apically evoked auditory nerve and brainstem responses in children with severe to profound deafness. Interwave latencies reflecting neural conduction along the caudal and rostral portions of the brainstem decreased over the first year of implant use. Differences in neural conduction times evoked by apical versus basal electrode stimulation persisted in the caudal but not rostral brainstem. SIGNIFICANCE: Activity-dependent changes of the auditory brainstem occur in response to both apical and basal cochlear implant electrode stimulation.

Auditory brainstem activity in children with 9-30 months of bilateral cochlear implant use.

Bilateral cochlear implants aim to restore binaural processing along the auditory pathways in children with bilateral deafness. We assessed auditory brainstem activity evoked by single biphasic pulses delivered by an apical or basal electrode from the left, right and both cochlear implants in 13 children. Repeated measures were made over the first 9-30 months of bilateral implant use. In children with short or long periods of unilateral implant use prior to the second implantation, Wave eV of the auditory brainstem response was initially prolonged when evoked by the naïve versus experienced side. These differences tended to resolve in children first implanted <3 years of age but not in children implanted at older ages with long delays between implants. Latency differences were projected to persist for longer periods in children with long delays between implants compared with children with short delays. No differences in right versus left evoked eV latency were found in 2 children receiving bilateral implants simultaneously and their response latencies decreased over time. Binaural interaction responses showed effects of stimulating electrode position (responses were more detectable when evoked by an apical than basal pair of implant electrodes), and duration of delay between implants (measured by latency delays). The trends shown here suggest a negative impact of unilateral implant use on bilateral auditory brainstem plasticity.

Chronic suppurative otitis media following paediatric cochlear implantation.

The objective of this study was to report and discuss the management of chronic suppurative otitis media (CSOM) following cochlear implantation in children. The study was a retrospective review of 650 patients receiving an implant at two paediatric tertiary referral centres for cochlear implantation. Nine patients were identified who developed CSOM following cochlear implantation (incidence 1.38%). The mean time interval between implantation and symptom development was 3.66 years (range 2-8 years) and the mean time interval between implantation and CSOM surgery was 5.02 years (range 2.2-8 years). All patients presented with otorrhoea and/or abscess formation over the implant site. Two patients underwent a modified radical mastoidectomy and seven underwent a combined approach tympanoplasty, three of whom required posterior canal wall reconstruction with cortical bone and one with cartilage. In four cases it was possible to remove the cholesteatoma without removing the implant. All but two patients were fitted with a contralateral implant. In the explanted ears the cochlear implant electrode was cut at the cochleostomy site, which was then covered with muscle. Chronic suppurative otitis media following cochlear implantation may occur either as a result of a posterior canal wall defect related to surgery or possibly de novo. Attempts should be made to save the implant, but explantation with reimplantation of the contralateral ear may be the only option. In these cases the intracochlear part of the electrode array should be left in situ to facilitate possible future reimplantation. Surgical options for management of CSOM should be individualized and may include both canal-wall up and canal-wall down techniques. To reduce the incidence of CSOM following implantation the authors recommend: (1) prompt treatment and careful follow-up of patients with a history of otitis media with effusion, (2) avoidance of excessive thinning of the posterior canal wall during mastoidectomy and (3) reconstruction of any accidental trauma to the annulus or posterior canal wall during posterior tympanotomy.

Stimulation parameters differ between current anti-modiolar and peri-modiolar electrode arrays implanted within the same child.

OBJECTIVE: To compare stimulation parameters of peri-modiolar and anti-modiolar electrode arrays using two surgical approaches. METHODS: Impedance, stimulation thresholds, comfortably loud current levels, electrically evoked compound action potential thresholds and electrically evoked stapedial reflex thresholds were compared between 2 arrays implanted in the same child at 5 time points: surgery, activation/day 1, week 1, and months 1 and 3. The peri-modiolar array was implanted via cochleostomy in all children (n = 64), while the anti-modiolar array was inserted via a cochleostomy in 43 children and via the round window in 21 children. RESULTS: The anti-modiolar array had significantly lower impedance, but required higher current levels to elicit thresholds, comfort, electrically evoked compound action potential thresholds and electrically evoked stapedial reflex thresholds than the peri-modiolar array across all time points, particularly in basal electrodes (p < 0.05). The prevalence of open electrodes was similar in anti-modiolar (n = 5) and peri-modiolar (n = 3) arrays. CONCLUSION: Significant but clinically acceptable differences in stimulation parameters between peri-modiolar and anti-modiolar arrays persisted four months after surgery in children using bilateral cochlear implants. The surgical approach used to insert the anti-modiolar array had no overall effect on outcomes.

Delayed myocardial laceration after intrapericardial pneumonectomy.

Curative resection for large central bronchogenic tumors may require radical or intrapericardial pneumonectomy. Myocardial herniation through the pericardial defect is a rare early postoperative complication. Prevention of cardiac herniation and subsequent hemodynamic compromise is always necessary after intrapericardial pneumonectomy.

Diverticuloplasty for severe mucosal injury after cricopharyngeal myotomy.

Cricopharyngeal myotomy with either diverticulopexy or diverticulectomy is the recommended therapy for Zenker's diverticulum. Mucosal injury during myotomy is rare and usually can be managed by direct mucosal closure. A technique is described to repair a serious mucosal defect that may occur during cricopharyngeal myotomy.

Sinonasal disease in cystic fibrosis: clinical characteristics, diagnosis, and management.

Cystic fibrosis is an autosomal recessive genetic disorder that causes dysfunction of exocrine glands, and has several clinical manifestations. Among those, sinonasal involvement is almost universal, with or without chronic sinusitis and/or nasal polyposis. This review will detail the pathophysiologic changes of the sinonasal mucosa, and the clinical manifestations, diagnosis, and treatment. Developmental anatomic abnormalities, which are identified radiologically, will also be demonstrated. Medical management is the first treatment for patients with cystic fibrosis, but effective treatment of sinonasal disease in cystic fibrosis relies heavily on surgery. In the past, nasal polyposis was the main indication for surgery, and consisted mostly of polypectomy alone. This procedure was associated with a high recurrence rate. The development of functional endoscopic sinus surgery has contributed to decreasing the morbidity of sinonasal surgery and the recurrence of nasal polyposis in cystic fibrosis. The evolution of the surgical techniques will be discussed and a review of the literature will be provided.

Frequency selectivity in patients with acoustic neuroma.

Frequency selectivity was compared in subjects with hearing loss due to acoustic neuroma and cochlear pathology, and normal listeners. A particular interest was the role of probe tone parameters on the shape of the tuning curve. Psychophysical tuning curves (PTCs) were measured for each of two equal energy 2000-Hz probe tones (10 dB SL/300 msec and 17 dB SL/60 msec), using simultaneous 1/3-octave narrow-band noise maskers centered at 1, 1.25, 1.6, 2.5, 3.15, and 4 kHz. The results showed that the critical masker levels obtained for impaired listeners were significantly greater than those from normal subjects. The slope of the low-frequency limb of the PTC was steeper for normal compared to hearing-impaired listeners but there was no difference due to site of lesion. In all three groups, the critical masker levels obtained with the short probe were significantly greater than those for the long probe, negating the hypothesis that equal energy probes would yield the same outcomes. Tuning in listeners with hearing loss was highly correlated with audiometric threshold but not with tumor size, width of the internal auditory canal, or tumor location within the cerebellopontine angle. The main conclusion was that cochlear and retrocochlear hearing loss are similar with respect to their effect on frequency selectivity.

Orbicularis oris muscle injury in brass players.

The embouchure of the brass player is critical to tone production and largely depends on the integrity of the orbicularis oris muscle. Injury to this muscle can cripple the professional musician by causing fatigue, pain, and tonal deterioration. Ten brass players presented with muscular defects in the orbicularis oris muscle. Examination identified areas of abnormality within the muscle and electromyography (EMG) ruled out a neurologic deficit. All patients underwent exploration under neuroleptic anesthesia, and 9 patients underwent repair. The technique is described. The repaired patients reported improvement after the operation and all resumed playing at their premorbid level. The 10th patient was found to have thinning of the entire orbicularis oris muscle (presumably congenital) and was not able to be repaired. There were no complications of the procedure and no recurrences.

Surgical experience with bone-anchored hearing aids in children.

Titanium osseointegrated implants for bone-anchored hearing aids (BAHAs) have been in use since 1977. A series of 32 children who received implantation since 1990 is reported. The report focuses on the surgical aspects of BAHAs, predisposing factors, and prevention of complications in an unusual pediatric population. The records of 32 children who had undergone two-stage implantation of a BAHA were retrospectively reviewed. The majority of the patients had craniofacial abnormalities. Of the 32 implantees, 29 use their BAHA at present. Five children failed to achieve osseointegration, and eight patients have had revision surgery for lost abutments, trauma, or chronic skin problems. There were no differences between preimplantation and postimplantation bone or air conduction thresholds. The pediatric BAHA carries with it a unique set of challenges and problems but can be successfully implanted and maintained.

Transclavicular approach to the neck, thoracic inlet, and axilla.

OBJECTIVE: To describe claviculotomy and claviculectomy for access to lesions of the lower part of the neck, with simultaneous reconstruction. METHODS AND MATERIALS: A retrospective review of 15 patients who underwent claviculotomy for access to the lower part of the neck. RESULTS: Eight patients underwent plate reconstruction, and one patient required subsequent removal. Three patients underwent total claviculectomy, and four patients underwent partial claviculectomy. Ten patients are alive and free of disease, three have died of disease, and two are alive with disease. All but two patients are satisfied with shoulder function, and one patient is dissatisfied with the cosmetic result. CONCLUSIONS: The claviculotomy and claviculectomy technique for tumors that transgress the neck, thoracic inlet, and axilla offers maximal exposure for excision, vascular control, and preservation of vital structures. Modern plating techniques have allowed clavicular reconstruction with improved cosmesis and preservation of shoulder stability.

Assessment of vocabulary development in children after cochlear implantation.

OBJECTIVES: To assess vocabulary development in children following cochlear implantation and to evaluate the effect of age at implantation on performance. DESIGN: Retrospective study (mean follow-up, 3(1/2) years). SETTING: Tertiary center. PATIENTS: Children with prelingual deafness provided with a cochlear implant between 1988 and 1999, who serially performed the Peabody Picture Vocabulary Test-Revised (60 patients) and the Expressive One-Word Picture Vocabulary Test-Revised (52 patients). The children were subgrouped into those receiving implants at younger than 5 years and at 5 years or older. OUTCOME MEASURES: Age-equivalent vocabulary test score and gap index (chronological age minus the age-equivalent score, divided by the chronological age at the time of testing) were calculated. For each test, the following were performed: calculation of rate of change for age-equivalent score; comparison of earliest and latest gap indices means (the cohort and intergroup and intragroup comparison); and multiple regression analysis demonstrating the effect of age at implantation, sex, communication mode, etiology of deafness, and residual hearing on the rate of vocabulary development. RESULTS: Expressive and receptive vocabulary development rates were 0.93 and 0.71 (age-equivalent scores per year), respectively. Subgrouped by age at implantation, the children's rates (for both vocabularies) were not statistically different (Peabody Picture Vocabulary Test-Revised, P =.90; Expressive One-Word Picture Vocabulary Test-Revised, P =.23). The global latest gap indices were significantly less than the earliest (Peabody Picture Vocabulary Test-Revised, P =.048; Expressive One-Word Picture Vocabulary Test-Revised, P<.001), indicating an improvement in age-appropriate vocabulary development over time. The age subgroups demonstrated similar results, except for the younger group's receptive gap index. On multiple regression analysis, the significant predictive variables were residual hearing (Expressive One-Word Picture Vocabulary Test-Revised) and male sex and oral communication mode (Peabody Picture Vocabulary Test-Revised). CONCLUSIONS: Children with cochlear implants developed their vocabularies at rates that were sufficient to prevent an increase in their gap indices as related to ideal scores at testing. A late age at implantation does not singularly preclude beneficial development of vocabulary.

Otologic and laryngologic manifestations of mucopolysaccharidoses after bone marrow transplantation.

INTRODUCTION: Children with mucopolysaccharidosis have significant functional impairment of a number of systems because of deposition of glycosaminoglycans. The otolaryngologic system is affected, resulting in conductive and sensorineural hearing loss and voice abnormality. METHOD: Eleven children who had undergone bone marrow transplantation had their hearing and voices assessed. Unmatched data from patients who had not undergone transplantation were collected for nonstatistical comparison. RESULTS: The hearing loss in children who had undergone bone marrow transplantation was variable but on average was less severe than in the younger children who had not undergone transplantation. The incidence of otitis media with effusion was diminished in the transplanted group. Approximately half had normal voices. DISCUSSION: Diminution of the progression of hearing loss and voice abnormality probably occurs in patients after treatment by bone marrow transplantation.