Successful cochlear implantation in Langerhans cell histiocytosis: A rare case

An 8-year-old girl presented with a 1-year history of bilateral progressive hearing loss and vertigo for 6 months. High-resolution computed tomography of the temporal bones demonstrated multiple lytic lesions. Histopathology examination confirmed a diagnosis of Langerhans cell histiocytosis (LCH). She underwent chemotherapy for 12 months. Following treatment, she was in remission. However, the bilateral profound sensorineural hearing loss persisted. She underwent right cochlear implantation with very good functional audiological outcomes. This is the first known reported case of successful auditory rehabilitation through cochlear implantation in a patient with bilateral profound hearing loss due to isolated bilateral temporal bone LCH.     

Bilateral cochlear implantation in Friedreich's ataxia: A case study

Abstract

With an incidence of 1:29 000 among Caucasians, Friedreich's ataxia (FRDA) is the most common inherited ataxia, leading to both sensory and motor degeneration. Despite many FRDA patients exhibiting normal or near normal sound detection thresholds, many individuals show abnormal neural conduction along their central auditory pathways. Electrophysiological testing can show abnormal or absent cochlear nerve and auditory brainstem recordings in the presence of normal pre-neural cochlear function (otoacoustic emissions or cochlear microphonics). This pattern of normal pre-neural cochlear function and disrupted neural conduction has been termed auditory neuropathy spectrum disorder (ANSD). Studies of FRDA patients with ANSD have shown that they exhibit severe deficits in temporal processing, impaired frequency discrimination, and deficits in speech perception. Rehabilitation of these auditory percept deficits remains difficult, as hearing aids may amplify sounds without adding clarity to the temporally disrupted or distorted signal that FRDA patients with ANSD may receive. There is limited data on the best intervention for patients with FRDA with ANSD, although personal radio aids (FM systems) have been shown to be beneficial. We report a case, where cochlear implantation has led to a dramatic improvement in speech perception in an individual with FRDA and ANSD. The majority of the literature on ANSD treatment has focused on paediatric patients with the ‘dyssynchrony’ type of ANSD, rather than the true neuropathy type underlying the hearing loss in FRDA patients.     

Binaural cochlear implantation after bilateral temporal bone fractures

Abstract

In this clinical note we discuss the indications, feasibility, and outcomes of binaural simultaneous cochlear implantation (CI) following bilateral transverse temporal bone (TB) fractures. A 41-year-old male, totally deaf after a bilateral TB fracture, underwent an audiological, electrophysiological, and imaging investigation in order to assess the integrity of the VIIIth cranial nerves. Five months later he received a simultaneous bilateral CI. Speech perception tests were conducted at different time points. A significant advantage by dichotic listening was observed since the beginning of the habilitation program. The patient achieved a 100% word and sentences recognition in quiet at 12 months. His listening skills in noisy conditions were improved by the use of two implants. A CI in TB fractures is feasible if the VIII nerve is intact and the cochlea is spared by the fracture rim. The early timing of the procedure probably contributed to its success by preventing cochlear fibrosis and ossification.

Sumario

En esta nota clínica discutimos las indicaciones, la factibilidad, y los resultados de la implantación coclear (CI) bilateral simultánea posterior a fracturas transversas bilaterales del hueso temporal (TB). Un varón de 41 años de edad, totalmente sordo después de una fractura bilateral del TB, fue sometido a una investigación audiológica, electrofisiológica y por imágenes, para evaluar la integridad de ambos octavos nervios craneales. Cinco meses después, el sujeto recibido IC bilaterales simultáneos. Se realizaron pruebas de percepción de lenguaje en diferentes momentos. Se observó una ventaja significativa en la audición dicótica desde el inicio del programa de habilitación. El paciente logró un 100% de reconocimiento de palabras y frases en silencio a los 12 meses. Sus habilidades para escuchar mejoraron con el uso de dos implantes. Una IC en fracturas del TB es factible si el VIII par está intacto y la cóclea no está involucrada en el trazo de la fractura. La oportuna realización del procedimiento probablemente contribuyó al éxito al prevenir la fibrosis coclear y la osificación.     

Bilateral cochlear implantation in children with Noonan syndrome

Noonan syndrome is a mostly autosomal dominant inherited disorder, which can be accompanied by hearing disorders or deafness, coagulation disorders, combined heart defects and developmental disorders. We are reporting on two children with an established Noonan syndrome with a severe bilateral hearing loss of respectively 95 and 100 dB and proper findings in the CT/MRI of the petrous bone. After complete otologic and radiologic diagnostics, both children underwent bilateral cochlear implantation successfully. According to the authors’ knowledge, this is the first time that cochlear implant therapy is discussed in patients with Noonan syndrome.     

Evaluating inter-aural hearing preservation in bilateral paediatric cochlear implantation

Objective: To examine inter-aural hearing preservation results in children undergoing simultaneous bilateral cochlear implantation (CI).

Methods: Retrospective case review in tertiary referral centre. All children undergoing simultaneous bilateral CI between January 2013 and June 2014 (18 months). Patients eligible for inclusion in the study had pre-operative hearing thresholds of <90?dB at 250?Hz and ≥100?dB at 500?Hz. Patients with anatomical cochlear anomalies or missing data were excluded. Seven patients were included, 1 male, 6 female, mean age of 12 years 11 months at the time of surgery. All patients had simultaneous bilateral cochlear implant surgery, using the same implant and technique. All patients had pre- and post-operative unaided pure tone audiometry. Inter-aural hearing preservation results were compared in each patient.

Results: The achieved hearing preservation for 14 ears was complete in 5, partial in 7, and minimal in 2. Measurable hearing preservation was achieved in 86% overall. Inter-aural analysis revealed that only 2 (subjects 1 and 4) of the 7 patients had preservation results within the same preservation group (complete/partial/minimal). The mean inter-aural preservation difference was 30.7% with a range from 12.4% to 65.2%.

Conclusions: Several factors and techniques have already been identified in the wider literature to explain differences in hearing preservation results in CI. However, despite controlling for known factors, we demonstrate variable inter-aural results. This suggests that there may be more factors beyond the surgeon’s control influencing our ability to provide consistent results.     

Pneumocoele after cochlear implantation

Abstract

Objective and importance

Scalp pneumocoele is a rare complication of cochlear implantation surgery. We present the largest case series to date; consisting of four cases over 8 years from one centre. The presentation, diagnosis, and management of each case are reviewed. Previously unreported complications of pneumocoeles are highlighted.

Clinical presentation

Three of our four cases, consisting of two adults (aged 53 and 81 years) and one child (aged 26 months), developed scalp pneumocoeles within the first 3 months of implantation. Another child (aged 10) developed pneumocoele 7 years after surgery. The common diagnostic features include a soft, non-tender, fluctuant swelling over the receiver/stimulator package. In all cases, pneumocoeles were triggered by Valsalva's or Toynbee's manoeuvres. Complications include infection (case one) and reduction in cochlear implant (CI) function (cases three and four), secondary to displacement of the receiver/stimulator package or electrode displacement.

Intervention/technique

Diagnosis is made from clinical history and examination. Aspiration results in reduction of pneumocoeles. This is not necessary for smaller pneumocoeles as they may resolve spontaneously. Additional investigations may reveal complications of pneumocoeles, such as X-rays for implant displacement, and inflammatory marker to detect signs of infection. To prevent the recurrence of pneumocoeles, patients are advised to avoid Valsalva's or Toynbee's manoeuvres. There were no recurrences of pneumocoeles.

Conclusion

Post-cochlear implantation pneumocoeles can be associated with harmful complications such as infection and CI dysfunction. The mechanism of pneumocoele formation is discussed, and strategies for its prevention are examined.     

Tinnitus modifications after cochlear implantation

Tinnitus can be defined as a phantom sensation in the absence of an external sound. In our study, we evaluated the effect of cochlear implant on tinnitus evolution. Among adult, postlingually deaf patients who underwent cochlear implantation at our clinic, we selected 20 subjects with pre-implantation tinnitus (group A) and 10 subjects without pre-implantation tinnitus (group B). Pre- and post-surgery tinnitus was assessed through two questionnaires: the first one dealing with tinnitus characteristics and psychosocial impact, and the second one represented by THI, an internationally validated score of evaluation of the effects of tinnitus on patient’s emotions and activities of daily living. None of the patients belonging to group B developed tinnitus after surgery. As for group A, 40% of patients declared suppression of tinnitus, 30% attenuation of tinnitus after surgery, 25% reported tinnitus was unchanged and 5% reported worsening of tinnitus. In the nine patients with bilateral tinnitus (45%), after implantation tinnitus disappeared from both sides in four patients and attenuated bilaterally in four patients. A comparison between pre- and post-implantation THI scores showed decreased score in 65% of cases, unchanged score in 30% and increased score in 5%. The beneficial effect of cochlear implant on tinnitus, reported by a majority of patients, could be due to acoustic masking, to direct electrical stimulation of the acoustic nerve, and above all to a possible cochlear implantation dependent reorganization of the central auditory pathways and associative cerebral areas. In the light of these results, the authors propose (1) to include tinnitus in the selection criteria of which ear to implant; (2) to consider implantation eligibility for patients with bilateral severe hearing loss associated with severe tinnitus; and (3) to inform patients about the small risk of post-operative tinnitus worsening.     

Parental perspectives on decision-making and outcomes in pediatric bilateral cochlear implantation

Abstract

Objective: Bilateral cochlear implantation is becoming standard care to improve outcomes for children with profound or severe to profound hearing loss. This study examined parents’ perceptions of the decision process and of the benefits of two implants. Design: The study adopted a qualitative approach, examining parents’ views through individual semi-structured interviews. Study sample: The participants consisted of the parents of 15 children at one centre in Ontario, Canada, where 26 children had received bilateral implants. Children ranged in age from 1.8 to 11.9 years and their experience with two implants ranged from 0.3 to 4.2 years. Results: Parents described decision-making as relatively straightforward. However, many parents shared concerns related to surgery. Primary reasons for choosing two implants were neuroplasticity, preference for a backup device, and maximizing potential through technology. Expected outcomes included better speech understanding in noise and the ability to locate the source of sounds. Perceived benefits of bilateral implantation included improved speech understanding in noise, the availability of a backup implant, and parents’ assurance that they had provided their children with the best possible access to hearing. Conclusions: Overall, parents were satisfied with the second implant and identified benefits that are not easily quantifiable through traditional clinical measures.

Sumario

Objetivo: La implantación coclear bilateral se está convirtiendo en un procedimiento estándar para mejorar los resultados en niños con problemas auditivos profundos, o entre severos y profundos. Este estudio examinó la percepción de los padres sobre el proceso de decisión y sobre los beneficios de dos implantes. Diseño: El estudio adoptó un enfoque cualitativo, para examinar los puntos de vista de los padres por medio de entrevistas individuales semi-estructuradas. Muestra de estudio: Participaron 15 padres de niños en un centro de Ontario, Canada, en el que habían sido implantados bilateralmente 26 niños. La edad de los niños varió de 1.8 a 11.9 años y su experiencia con dos implantes, de 0.3 a 4.2 años. Resultados: Los padres describen la toma de su decisión como relativamente sencilla. No obstante, muchos padres compartieron sus preocupaciones sobre la cirugía. Las razones fundamentales para seleccionar dos implantes fue la neuroplasticidad, la preferencia por un dispositivo de apoyo y la maximización del potencial a través de la tecnología. Los resultados esperados incluyeron la mayor comprensión del lenguaje en ruido y la habilidad para la localización de las fuentes sonoras. La percepción de beneficios de la implantación bilateral incluyó la mayor comprensión del lenguaje en ruido, la disponibilidad de un implante de apoyo y la seguridad de los padres de que han proporcionado a sus hijos la mayor posibilidad de acceso a la audición. Conclusiones: La impresión general es de que los padres están satisfechos con el segundo implante y que identificaron beneficios que no son fácilmente cuantificables por medio de las mediciones clínicas tradicionales.     

Bilateral cochlear implantation after ethylene glycol intoxication: a case report

Abstract

Objective

To report a case of deafness and blindness caused by ethylene glycol poisoning and treated by bilateral simultaneous cochlear implantation.

Study design

Case report.

Setting

University teaching hospital, tertiary referral center.

Patient

37 years old man poisoned with ethylene glycol.

Intervention

Bilateral simultaneous Medel Sonata cochlear implants.

Outcome measure

BKB sentences (Bamford–Kowal–Bench sentence tests); AB words (Arthur Boothroyd monosyllabic words).

Results

Successful rehabilitation of hearing in a case of deafness and blindness of acute onset.

Conclusion

Bilateral CI is an effective method of hearing rehabilitation in presence of additional disability (blindness).     

Outcome for bilateral cochlear implantation in patients with congenital Cytomegalovirus infection

ObjectivesTo analyze the impact of bilateral cochlear implantation (CI) on perceptual and linguistic development in hearing-impaired children with congenital Cytomegalovirus (CMV) infection.Patients and methodA retrospective study was performed for the period 1991-2016 in a pediatric CI reference center. Closed Set Word (CSW) recognition scores, Categories of Auditory Performance (CAP) and linguistic level on the MT Lenormand scale (MTL) were compared between bilateral (Bi) and unilateral (Uni) groups 12, 24 and 36 months after first CI (CI-1).Results84 patients with congenital CMV infection who underwent CI were included, in 2 groups: sequential or simultaneous bilateral CI (Bi) (N = 20), and unilateral CI (Uni) (N = 64). Twelve, 24 and 36 months after CI-1, CSW scores were 35.56%, 64.52% and 82.93% in Uni and 60.3%, 85% (P = 0.0084*), and 100% (P = 0.00085*) in Bi. CAP scores 12, 24 and 36 months after CI-1 were 2.57, 3.85 and 4.3 in Uni and 3.91 (P = 0.0068*), 5.00 (p = 0.029*) and 5.50 (P = 0.051*) in Bi. MTL linguistic level scores at 12, 24 and 36 months were 0.72, 1.25 and 1.65 in Uni, and 1.72, 3 (P = 0.033) and 3.11 (P = 0.045) in Bi. These significantly better scores in Bi at 24 and 36 months after CI-1 were also found on analysis of subgroups with no associated neurologic disorder (P = 0.046* and P = 0.032*), no associated psychiatric pathology (P = 0.0055* and P = 0.0073*), and no other associated disorder (P = 0.0018* and P = 0.035*), and for all subgroups together (P = 0.0036 and P = 0.037).ConclusionBilateral CI is a faster way than unilateral CI for patients with congenital CMV infection to achieve structured fluent oral language. 50% of the series showed cerebral abnormalities on MRI, without difference between groups. This was not in itself predictive of poor progression of oral communication, unless associated with major neurologic disorder. Some children made little or no use of their CI in the medium term.     

人工耳蜗植入术后X线投照位置的探讨

目的:确定耳蜗在颅骨的空间位置,设计最佳耳蜗X线摄片投照角度,用于评价电极位置。方法:测量26例耳蜗植入候选患儿术前CT和32例正常成人中内耳CT片上,耳蜗底周与正中矢状线的夹角;并根据测量的X线投射角度拍摄耳蜗位X线平片。结果:蜗轴中线与正中矢状线的角度为40°到68°,平均52.66°;其中儿童组的平均值为52.52°(s=7.07),成人组的平均值为52.80°(s=5.78),两者间差异无显著性意义(P>0.05)。结论:按所测的X线投射角度拍摄的耳蜗平片,能清晰完整显示电极系列的整体和所有的单个电极,确定电极的插入深度和准确的位置,为频率的匹配定位、语言处理方案的优化提供帮助。     

Tinnitus and cochlear implantation

Tinnitus is a common experience, but there is very marked heterogeneity of aetiology, perception and the extent of distress among individuals who experience tinnitus. In view of this, a modern approach to tinnitus should consider homogeneous groups of individuals. This review considers tinnitus experiences in patients undergoing cochlear implantation, this being of interest because the prevalence of tinnitus in this patient group prior to surgery may shed some light on the link between cochlear dysfunction and tinnitus. Second, any change in tinnitus experience as a result of electrode placement surgery or cochlear implant activation has relevance for patient counselling and informed consent. Finally, in recent tinnitus retraining therapy literature there has been the suggestion that unilateral sound therapy for tinnitus patients may set up an unhelpful asymmetry of input to the auditory system, with possible exacerbation of contralateral tinnitus. Unilateral cochlear implant use represents the most dramatic asymmetry possible and hence is a test of that hypothesis. Relevant papers (n = 32) were identified from literature databases. The standard of reporting tinnitus results was inconsistent. Tinnitus is experienced by up to 86% of adult cochlear implant candidates, but is not universal and is only troublesome in a small proportion (reported as 27% in one study). Electrode insertion may induce tinnitus in a small (up to 4%) number of patients, but this is rare. Cochlear implant device use is associated with reduction of tinnitus intensity and awareness in up to 86% of patients, and rarely with exacerbation (up to 9%). There are some indications in the literature that the more complex the simulation strategy, the larger that effect. Specifically, unilateral cochlear implant use was generally associated with reduction of contralateral tinnitus (in up to 67% of individuals) rather than exacerbation, and so the assertion that unilateral sound therapy for tinnitus is contraindicated is not proven.     

Rare case of bilateral aural atresia and cochlear dysplasia: when cochlear implantation is not the answer

Objective and importance: Reports of patients with concurrent middle and inner ear anomalies are rare. These patients present a surgical challenge for cochlear implantation. The surgical risk must be weighed against the predicted benefit of the patient’s hearing outcome and subsequent development of speech and language as well as their quality of life.

Clinical presentation: Thirteen-year-old boy presented to the Otology clinic for auditory rehabilitation options. He has mild developmental delay, is non-verbal and communicates via American Sign Language. He was born with bilateral aural atresia and never wore amplification. On exam he has grade 1 microtia and complete ear canal atresia bilaterally. His behavioural hearing test shows profound sensorineural hearing loss of both ears. The computed tomography scan shows bilateral underdeveloped and completely opacified mastoid and middle ear, complete bony atresia of the ear canals, and an under-partitioned cochlea with poorly defined modiolus, among other abnormalities. The patient and his family were counselled on the available options as well as the need for any further studies.

Intervention: Counselling of patient and family.

Conclusion: While there have been reports in the literature of performing cochlear implantations in patients with a concurrent atresia and cochlear dysplasia, these were patients whose degree of inner ear anomalies was relatively minor and their prognosis of a good audiological outcome was favourable. The presented case is that of a patient for whom the surgical approach to the cochlea alone would be difficult. More importantly, his quality of life would not significantly improve in light of the predicted limited hearing and language development outcomes, given the severity of his inner ear abnormalities, limited communication abilities, prolonged period of deafness and developmental delays.     

人工耳蜗植入术中EABR监测的应用

目的探讨人工耳蜗植入术中进行EABR监测的方法，以了解耳蜗电刺激下听觉传导通路的神经反应情况。方法20例人工耳蜗植入患者，男14例，女6例，平均年龄13．6岁，语前聋患者14例，语后聋患者6例。全麻后安置体表记录电极，将PPS与听觉诱发电位仪触发端口连接，并选定听觉诱发电位仪的外触发模式。人工耳蜗电极植入后，先行常规NRT监测，然后将NRT刺激参数改为EABR模式，采用Basic双极刺激，脉宽50μs，强度由200CL起以10CL为步长递减至反应阈值。结果20例患者均记录到EABR，阈上20CL时Ⅲ波．Ⅴ波的平均潜伏期分别为2．04±0．20ms．3．96±0．41ms。相同刺激条件下的EABR反应平均阈值为148．46±11．63CL，NRT反应平均阈值为160．72±13．56CL。一例脑白质轻度发育异常患儿，术中NRT波形引出良好，EABRⅠ～Ⅳ波分化良好，Ⅴ波波形低钝，Ⅴ波／Ⅲ波振幅比〈1／2，考虑可能存在耳蜗核上性神经发育不良，现正在语言康复训练随访中。结论人工耳蜗植入术中进行EABR监测比NRT能提供更完整的．更接近听觉中枢的神经反应信息，能更进一步了解听觉传导通路的功能状态，以期对患者听力康复的效果提供更准确的预测。     

Percutaneous pedestals in cochlear implantation

Percutaneous pedestals have been integral to the development of cochlear implants since 1969. By enabling direct electrical access to implanted electrodes or other devices, they allow optimization of control of stimulation strategies. Similarly, technology not validated for implantable use can be safely tested. These advantages have facilitated the development of cochlear implants and also resulted in their inclusion in trials investigating electronic implants developed for other organs. Surgery is straightforward, but post-operative care, in particular, skin-care is crucial to ensure complications are minimized. This review discusses the history of percutaneous pedestal use in cochlear implants and other electronic devices. Surgical technique, aftercare, and complications of surgery are discussed along with possibilities for future development.