Cochlear implantation in a patient with eosinophilic otitis media

Eosinophilic otitis media is an intractable middle ear disease with gelatinous mucoid fluid containing eosinophils associated with bronchial asthma and nasal allergy that sometimes induces deterioration of sensorineural hearing loss. Here, we report a case of eosinophilic otitis media in a 50-year-old woman who received a Nucleus 22 multi-channel cochlear implant in the right ear at the age of 42 years. She had received treatment for bronchial asthma, chronic sinusitis with nasal allergy and otitis media with effusion since the age of 30 years and had noted bilateral sudden deafness and vertigo at the age of 35 years. Preoperative CT and MRI showed cochlear ossification in the left ear, in which mastoidectomy was performed as treatment of eosinophilic otitis media. Long-term follow-up revealed that cochlear implant is indicated for deafness induced by eosinophilic otitis media, and an early decision for cochlear implant surgery is necessary. Steroid administration was remarkably useful in controlling eosinophilic otitis media in patients with bronchial asthma and chronic sinusitis with nasal allergy.     

Cochlear implantation in a patient with Epstein syndrome

Epstein syndrome is a rare disease which is accompanied by nephritis, sensorineural hearing impairment and macrothrombocytopenia. It has been suggested that this syndrome is a hereditary disease associated with mutations in MYH9, which encodes non-muscle myosin heavy chain IIA. We report a case of a patient with Epstein syndrome in whom bilateral profound hearing impairment developed and who had undergone cochlear implantation 9 years previously. Prior to this, the patient showed progressive sensorineural hearing impairment and had become completely deaf by the age of 25. A cochlear implant was successfully used with a speech discrimination score of 98% (sentence test). However, in the present case, peri- and postoperative complications occurred: tympanic perforation remained after a promontory stimulation test, followed by transitory otitis with purulent discharge. Therefore, tympanoplasty was performed simultaneously with cochlear implantation. These complications were considered to be caused by platelet dysfunction and delayed wound healing. Furthermore, cochlear destruction was observed 8 years postoperatively. In Epstein syndrome, the mechanism of osseous change remains uncertain. To the best of our knowledge, this is the first case report of Epstein syndrome in a patient with long-term use of a CI.     

Cochlear implantation in patients with substantial residual hearing

OBJECTIVES: Cochlear implantation is an effective means for providing auditory rehabilitation in adult patients with severe to profound sensorineural hearing loss. It has been hypothesized that patients with substantial, preoperative residual hearing would be excellent cochlear implant candidates because of surviving neural populations and a lack of auditory deprivation. The purpose of this study is to describe the outcomes of patients with substantial residual hearing who have undergone cochlear implantation. STUDY DESIGN: Retrospective chart review of patients with substantial preoperative residual hearing who underwent cochlear implantation. METHODS: Chart reviews were completed for patients with substantial residual hearing who underwent cochlear implantation (City University of New York Sentence Test [CUNY] > 60%, Hearing in Noise Test sentences presented in quiet [HINTQ] > 50%, or Consonant-Nucleus-Consonant [CNC] > 20% in the ear to be implanted). Preoperative and postoperative measures of audiologic performance as well as complications were assessed. RESULTS: All 12 patients who met inclusion criteria ultimately surpassed their preoperative aided performance level after implantation and gained significant benefit from their cochlear implant. At 6 months postimplantation, mean CUNY, HINTQ, and CNC scores were 93%, 78%, and 48% in the implant ear alone, respectively. However, progress was slower than expected for many patients, and at least one patient took 1 year to surpass his preoperative performance level. There were no complications from surgery in this selected group of patients. CONCLUSIONS: Patients with some degree of residual hearing do benefit from cochlear implantation. However, there may be an initial decline in performance as compared with preoperative levels. This decline is overcome in time in this patient population. These patients need to be counseled accordingly.     

Cochlear implantation in patients with MELAS syndrome

MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) is a rare congenital disorder of mitochondrial DNA (mt-DNA). Patients with this syndrome may present to the otolaryngologist with sensorineural hearing loss (SNHL) that is genetic in origin. Mitochondrial cytopathies can present with a variety of symptoms, but they occasionally present with SNHL as their first manifestation. Two cases of MELAS patients who responded well to cochlear implantation are presented. A review of the literature is also carried out focusing mainly on diagnosis, anesthetic considerations and management of these patients.     

Cochlear implantation in children with otitis media

OBJECTIVE: To describe the outcome of cochlear implantation in a cohort of pediatric patients with a history of otitis media. STUDY DESIGN: Retrospective chart review and follow-up phone survey. METHODS: The medical records of patients younger than 48 months of age who underwent cochlear implantation were reviewed for demographics, complications, and history of otitis media and bilateral myringotomy and ventilation tubes. A follow-up phone survey was performed to determine the long-term natural history of otitis media. RESULTS: The records of 126 profoundly deaf pediatric patients were reviewed. Seventy-two percent had a history of at least one episode of otitis media, 31% had a history of greater than three episodes, and 2% had a history of chronic serous otitis media. Twenty-three percent underwent bilateral myringotomy and tubes in the distant past, 6 to 8 weeks before implantation, at the time of implantation, or after implantation. A phone survey was successfully completed for 76 patients with a mean follow-up period of 46 months. There was a significant decrease in the incidence of otitis media after the cochlear implantation in the majority of patients with a history of otitis media with and without a history of bilateral myringotomy and tubes. CONCLUSIONS: Placement of ventilation tubes either before or at the time of cochlear implantation seems to adequately prevent infectious disease-related complications in otitis-prone children. Therefore, implantation should not be delayed, and subsequent episodes of otitis media can be managed by conventional therapy.     

伴有听力障碍的多残儿童人工耳蜗疗效分析

目的 探讨多残儿童人工耳蜗植入手术的经验及问题。方法 我院近年来开展多导人工耳蜗植入术600余例，对其中8名多残儿童开展了多导人工耳蜗植入手术。8名患者中，深度感音性聋同时合并智障与脑瘫者1例，余合并智障者2例，其中一例智障患者合并Mondini畸形，合并脑瘫者3例，Usher’s综合症2例。对这8例患者进行回顾性分析，随访3～5年。就术后开机调试特点，术后听力言语康复效果以及存在的问题等进行讨论。结果 （1）术后无面瘫、脑脊液漏、脑膜炎等手术并发症；（2）手术后护理比常规人工耳蜗术后病人复杂：病人哭闹、卧床困难、输液困难、不配合医护人员工作等；（3）开机调试后所有术后多残儿童都有听觉反应，除1例听到声音后极为恐惧外，其余患者都非常兴奋，但调试比较困难，患者配合程度差，有时一个病人需要多次调试才能获得结果，增加了调机人员的工作难度；（4）经过艰苦的康复训练，言语辨别率比术前明显提高，家长对此很满意。结论 伴有多种残疾的深度感音性聋患者可以行人工耳蜗植入，术后增加了一个全新的康复手段，从而为整个康复计划起到了推动作用，减轻了患者家庭和社会的负担，但增加了医护人员术后护理、调机以及康复上的工作难度。     

Cochlear implantation in a patient with osteogenesis imperfecta

Osteogenesis imperfecta (OI) is a connective tissue disorder characterized by a deficit in the synthesis of type I collagen. Hearing loss affects 42–58% of OI patients and progresses to deafness in 35–60% of these patients. For OI patients, cochlear implantation (CI) is the only promising treatment option. However, literature on CI in patients with OI is relatively rare. After CI, speech perception is generally good. However, among patients with severe demineralization of the cochlea, most patients are reported to have complications of facial nerve stimulation (FNS), preventing some patients from using the cochlear implant on a daily basis. Here we report a successful CI using a Nucleus CI24 Contour Advance cochlear implant in a patient with OI. Although high-resolution computed tomography (HRCT) showed extensive demineralization of the cochlea, intracochlear electrodes were inserted properly. The use of a modiolus-hugging device and the advance off-stylet technique contributed to the successful implantation, with no complications such as FNS or misplacement of electrodes. Therefore, CI can be used for treating deaf patients with OI.     

Cochlear implantation in patients with ossified cochleas

Purpose

The timing of CI for postmeningitic deafness is controversial and differential outcomes have been reported. To review and share our surgical and auditory outcomes.

Cochlear implantation in patients with Jervell and Lange-Nielsen syndrome, and a review of literature

Jervell and Lange-Nielsen syndrome is characterized by a long QT interval in electrocardiography, syncopal attacks and congenital sensorineural hearing loss. Upon diagnosis, beta-blockers is recommended as the first choice of medication, which is considered to reduce the occurrence rate of syncopal attack and sudden death during life. Cochlear implantation is indicated for hearing rehabilitation in this syndrome with profound deafness. The auditory and language outcome after cochlear implantation in this syndrome is not worse than those in patients with non-syndromic sensorineural deafness. The life quality in this syndrome could be improved greatly with cochlear implantation only if the cardiologic event has been controlled effectively.     

Cochlear implantation in a patient with bilateral deep brain stimulators

OBJECTIVE: We report the case of a patient successfully implanted with a Nucleus Contour cochlear implant after placement of a deep brain stimulator for Parkinson disease. METHODS: The authors conducted a case report and literature review. RESULTS: Successful hookup and mapping of the device was performed 1 month after implantation without evidence of aberrant activity of the deep brain stimulators. CONCLUSIONS: To our knowledge, this is the first reported case of successful implantation of both a cochlear implant and a deep brain stimulator in the same patient. We have outlined one approach to avoiding detrimental interactions between cochlear implant and deep brain stimulator devices.     

Cochlear implantation in patients with otosclerosis of the otic capsule

Objective

To evaluate outcomes of cochlear implantation of patients with otosclerosis of the otic capsule.

Cochlear implantation in a bilateral endolymphatic sac tumor patient. A case report

At the present time, the optimal management of patients with locally aggressive ELSTs includes: early diagnosis, surgical complete excision and long term follow up to monitor for recurrent disease. The presence of bilateral tumors produces a significant risk for bilateral profound hearing loss. We report the unusual case of bilateral endolymphatic sac tumor in a 14-year-old girl who presented bilateral anacusis and in whom a cochlear implant was performed after removal of the second tumor.     

Cochlear implantation as a successful rehabilitation for radiation-induced deafness

Radiotherapy of the head and neck can be associated with conductive and/or sensori-neural hearing loss. We report the case of a 67-year-old man who developed complete bilateral deafness caused by labyrinthitis and radiation-induced neuritis of the acoustic nerve after postoperative radiotherapy of a nasopharyngeal carcinoma. Two years postoperatively extensive clinical workup including computed tomography and magnetic resonance imaging showed no recurrence or secondary brain tumors. To facilitate sound perception a Combi 40 cochlear implant was implanted. Because of fibrosis the insertion depth of the stimulating electrode into the scala tympani was limited and therefore a “short electrode version” was used. Six months after implantation the patient had achieved an excellent enviromental sound recognition and moderate speech intelligibility. Present experiences has shown that although radiotherapy can cause damage to the labyrinth and acoustic nerves and central hearing pathways, there may still be surviving auditory nerve fibers that can be stimulated successfully by a cochlear implant. Received: 30 July 1997 / Accepted: 28 October 1997     

Cochlear implantation in irradiated tympanojugular paraganglioma

Objective: Indications for cochlear implantation have extended progressively over the years both from an audiological and clinical point of view, including the introduction of cochlear implants (CI) for hearing rehabilitation following skull base surgeries and neurotological procedures. In the past, cochlear implantation has been used for hearing rehabilitation in radiation-induced sensorineural hearing loss in nasopharyngeal and tonsillar carcinoma with successful outcomes. Here we describe a similar outcome following total deafness after tympanojugular paraganglioma irradiation, which is also the first such report in literature. Design: The irradiated ear of this patient was implanted with a CI through standard posterior tympanotomy. Study sample: A 26 year old male with multiple paragangliomas with bilateral deafness, secondary to surgery on one ear and radiotherapy for the opposite ear. Results: After a follow-up of 48 months, no local complications have occurred and the hearing results have remained stable with 100% sentence recognition. Conclusions: Bilateral and multiple paragangliomas are rare tumors. Despite the modality of treatment, the hearing is almost always compromised. In these patients, cochlear implantation offers a new perspective for hearing restoration. This report demonstrates that cochlear implantation can be effectively performed after tympanojugular paraganglioma irradiation with long-lasting, satisfactory results, even in the presence of residual tumor.     

Cochlear implantation in a pediatric patient with thalassemia major

We report the first known cochlear implantation in a pediatric patient with thalassemia major. After bilateral profound hearing loss was diagnosed a cochlear implantation was performed in the right ear when he was 24 months old. No complications occurred during the surgery and the postoperative period. Free-field audiometry and evaluation of auditory responses to speech (EARS) battery tests were used to evaluate perceptual auditory abilities, speech recognition and language growth. The patient demonstrated encouraging improvement in threshold levels that reached similar values to those of his peers. In contrast his level of language growth and recognition stayed at low levels. Results are discussed in the light of the specificity of this hereditary disease and its complications as we share our experience for better management of those type of patients in future.     

Cochlear implantation in children with cerebral palsy

Objective

Few studies have looked at the outcomes of children with complex needs following cochlear implantation. Increasing evidence supports the case for implantation in these children. To date there is very little evidence available evaluating the role of cochlear implantation in children with cerebral palsy. In this paper we look at the Manchester Cochlear Implant Programme's experience of implantation in 36 children with cerebral palsy.

Methods

A retrospective review of prospectively collected data for all children with cerebral palsy was undertaken. Cognitive and physical disability was scored by members of the cochlear implant team. A modified version of Geers and Moogs 1987 Speech Reception Score was used to assess outcome. Data was analysed looking at the relationship between cognitive and physical impairment, age at implantation and the SRS outcomes.

Results

This study demonstrated that children with cerebral palsy and a mild cognitive impairment do significantly better following implantation than those with a severe impairment (p = 0.008). Children with mild physical impairment did not appear to do significantly better than those with moderate or severe impairments (mild versus severe p = 0.13). Age at implantation was not a significant prognostic factor in this study group.

Conclusions

Children with complex needs are increasingly being referred for consideration of cochlear implantation. Further research is required to help guide candidacy, but each case must be considered individually. Higher functioning does appear to be the most important prognostic indicator regarding outcome but the effect of modest improvement in sound perception should not be underestimated.     

Cochlear implantation in a patient with Paget's disease

Hearing loss in Paget's disease, a metabolic bone disease, has been reported in as many as 60% of cases, and is presumably related to changes in mineral density of the otic capsule. We describe a 59-year-old man with Paget's disease who had severe to profound hearing loss and was referred to us for cochlear implantation. Preoperative evaluation revealed poor communication skills despite significant residual hearing, raising concerns about postimplantation outcome. Nevertheless, implantation was successful. As our literature search yielded only one report of cochlear implantation in Paget's disease, we record our clinical experience and discuss the hearing rehabilitation dilemmas in this case.     

Cochlear implantation in children with congenital inner ear malformations

OBJECTIVE/HYPOTHESIS: To assess the audiologic and surgical outcomes for pediatric cochlear implant patients with inner ear malformations. STUDY DESIGN: Retrospective review of 315 pediatric cochlear implant cases from 1994 to 2002. METHODS: Twenty-eight pediatric cochlear implant patients with known inner ear malformations determined on high-resolution computed tomography (HRCT) of the temporal bone were the subjects of review. Results of HRCT findings, intraoperative findings, postoperative complications, and objective measures of both closed- and open-set testing of speech perception were analyzed. RESULTS: Patients with the constellation of an incompletely partitioned (IP) cochlea, enlarged vestibular aqueduct (EVA), and a dilated vestibule (i.e., Mondini's malformation) as well as those with an isolated EVA or partial semicircular canal aplasia have relatively good levels of speech perception. Patients with total semicircular canal aplasia, isolated IP, cochlear hypoplasia, or common cavity demonstrated lower levels of performance. Poor performance may be related to associated developmental delays rather than labyrinthine anatomy alone. Complications of surgery were relatively limited. CONCLUSIONS: Cochlear implantation can be successfully performed in children with inner ear malformations. These children and their parents can expect significant auditory benefits from this intervention. The various types of inner ear malformations may have quite different prognoses for good auditory performance.