Cochlear implantation in a patient with combined renal and liver transplantation

Abstract

Objective and importance

Patients who have undergone solid organ transplantation and continuing immunosuppressant medication are at a higher risk of wound problems and infections following cochlear implantation. This risk is theoretically even further increased in multi-organ transplant recipients due to the increased doses of immunosuppressive medications that these patients are administered.

Clinical presentation and intervention

Here, we present the first reported case of successful cochlear implantation in a patient who had previously undergone successful combined liver and kidney transplant. She had no significant complications from the surgery and had good audiological outcomes 3 months post-operatively.

Conclusion

As we continue our advances in the use of cochlear implant technology, our report adds to the growing evidence of its benefits in transplant recipients. However, there are important pre- and peri-operative considerations in this group of patients which can improve safety and outcome.     

Cochlear implant and delayed facial palsy

Abstract

Delayed facial nerve palsy following cochlear implant surgery is less documented though it poses diagnostic and therapeutic challenges. Apart from the functional, aesthetic and emotional concerns, it can raise important medico legal issues. The objectives of this study were: to report a case of delayed facial palsy following cochlear implant surgery in a patient who had positive viral antibody markers pre operatively; and to review the literature on delayed onset facial paralysis following viral reactivation and its relation to cochlear implant surgery. An extensive literature review was done using internet and medical search engines and library facilities. Important articles on the topic were identified and summarised. Data on delayed facial palsy following cochlear implant surgery were collected, constructed in a coherent way and details discussed. Postulated mechanisms of delayed facial palsy include neural oedema, vasospasm and viral reactivation. Of these, reactivation of previous herpes simplex virus infection has special significance, as many of these patients are positive for viral antibody markers. Manipulation of sensory branches of the facial nerve and chorda tympani can be a mechanism in such cases. Correlation of clinical presentation and pre operative positive viral antibody markers with positive polymerase chain reaction can be strongly suggestive of viral reactivation. It is concluded that patients with positive viral antibody markers are more susceptible to facial palsy from viral reactivation. Corticosteroids, antiviral agents and physiotherapy can be useful in producing a quicker and complete recovery. An experienced cochlear implant surgery team and pre operative radiological evaluations are mandatory to decrease the chances of direct facial nerve trauma. Proper irrigation lowers the risk of neural oedema. Copyright © 2009 John Wiley & Sons, Ltd.     

Cochlear implantation in keratitis–ichthyosis–deafness syndrome – 10-year follow-up of two patients

Abstract

Objective and importance

The objective of this study was to describe long-term outcomes after cochlear implantation in keratitis–ichthyosis–deafness (KID) syndrome, often caused by GJB2 mutations, in the context of other reported cases.

Clinical presentation and intervention

The authors conducted correlative clinical and molecular genetic analysis on two implanted patients with KID syndrome, and tabulated their clinical outcomes. Both children had initially successful surgery. In one case, due to skin-related problems, despite extensive salvage surgery cochlear explantation was required. This patient now communicates with sign language and lip-reading. This contrasts with the outcome for the other patient whereby at post-operative year 10 he is able to easily converse by telephone. Both patients each carry a de novo 148G > A GJB2 mutation.

Conclusion

Patients with KID syndrome appear to be good candidates for cochlear implantation but may face significant skin-related problems which could disrupt successful post-operative habilitation. Consultation with dermatological colleagues regarding any new therapies may be warranted.     

Chronic inflammatory reaction to bone wax in cochlear implantation: A case report and literature review

Background: Cochlear implantation is a well-established treatment for severe-to-profound sensorineural hearing loss. While bone wax is used commonly during mastoidectomy and other bony surgeries as a hemostatic agent, there have been reports of bone wax triggering foreign body reactions, months to years after surgery. This report describes the first known foreign body reaction to bone wax used in cochlear implantation surgery.

Case Presentation: A 6-year-old male presented with an unusual post-auricular inflammation two years after cochlear implantation. Extended treatment with oral and intravenous antibiotics failed to resolve the presumed infection. Upon exploration of the mastoid cavity, fragments of bone wax were discovered within the granulation tissue. Excision of bone wax fragments and local flap reconstruction resulted in complete resolution of the inflammatory process.

Conclusions: Bone wax has been implicated in foreign body reactions in many different surgical specialties. This is the first reported case of an adverse reaction to bone wax after cochlear implantation. Clinically, the bone wax reaction was misinterpreted as an implant infection, which resulted in extended antibiotic use and delay of appropriate treatment. As a result, bone wax should be used judiciously during mastoidectomy, and particularly in the area of the electrode and soft tissue closure.     

Bilateral sensorineural hearing loss and labyrinthitis ossificans secondary to neurosarcoidosis

Abstract

Objective

To report a case of cochlear ossification as a result of neurosarcoidosis in a patient with bilateral profound sensorineural hearing loss.

Study design

Case report.

Setting

University teaching hospital, tertiary referral center.

Patient

Forty-year-old man with neurosarcoidosis and bilateral profound sensorineural hearing loss.

Intervention

Unilateral cochlear implantation.

Outcome measure

Aided thresholds speech perception tests.

Results

Marked improvement in hearing following implant.

Conclusion

Patients with neurosarcoidosis are at risk of labyrinthitis ossificans. Early imaging of these patients is recommended and if early cochlear ossification is identified they should be offered rapid access to rehabilitation with a cochlear implant.     

Bilateral cochlear implantation under local anaesthesia in a young adult – A case report

Objective: Cochlear implantation is routinely performed under general anaesthesia in most centres in the world. There have been reports of this surgery being performed under local anaesthesia and sedation for unilateral cochlear implantation. Our case study reports the first bilateral cochlear implantation carried out under local anaesthesia with sedation. This paper outlines the protocol used and discusses the challenges and advantages of the procedure being performed under local anaesthesia.

Case study: A 28-year-old female with a history of multidrug-resistant tuberculosis presented with bilateral severe to profound sensorineural hearing loss. On evaluation, she was found to be a suitable candidate for cochlear implantation. However, she was clinically unfit to undergo the surgery under general anaesthesia. After counselling the patient, the decision to undertake the procedure under local anaesthesia was made. The procedure took 105 min and was uneventful. The patient was discharged the next day

Conclusion: In the hands of an experienced surgeon and anaesthesiology team, bilateral cochlear implantation is possible under local anaesthesia. Patient motivation and cooperation are extremely important for the procedure to be done under local anaesthesia. This is an option for patients needing cochlear implantation who are medically unfit for general anaesthesia.     

Cochlear implant revision surgeries in children

IntroductionThe surgery during which the cochlear implant internal device is implanted is not entirely free of risks and may produce problems that will require revision surgeries.ObjectiveTo verify the indications for cochlear implantation revision surgery for the cochlear implant internal device, its effectiveness and its correlation with certain variables related to language and hearing.MethodsA retrospective study of patients under 18 years submitted to cochlear implant surgery from 2004 to 2015 in a public hospital in Brazil. Data collected were: age at the time of implantation, gender, etiology of the hearing loss, audiological and oral language characteristics of each patient before and after cochlear implant surgery and any need for surgical revision and the reason for it.ResultsTwo hundred and sixty-five surgeries were performed in 236 patients. Eight patients received a bilateral cochlear implant and 10 patients required revision surgery. Thirty-two surgeries were necessary for these 10 children (1 bilateral cochlear implant), of which 21 were revision surgeries. In 2 children, cochlear implant removal was necessary, without reimplantation, one with cochlear malformation due to incomplete partition type I and another due to trauma. With respect to the cause for revision surgery, of the 8 children who were successfully reimplanted, four had cochlear calcification following meningitis, one followed trauma, one exhibited a facial nerve malformation, one experienced a failure of the cochlear implant internal device and one revision surgery was necessary because the electrode was twisted.ConclusionThe incidence of the cochlear implant revision surgery was 4.23%. The period following the revision surgeries revealed an improvement in the subject's hearing and language performance, indicating that these surgeries are valid in most cases.     

Double challenge: cochlear implantation in the only hearing ear with progressive hearing loss following meningitis and vestibular dysfunction after implantation

ObjectiveVestibular dysfunction associated with cochlear implantation is rare. It is usually seen in patients with otosclerosis due to spread of electrical activity throughout the demineralized bone. A 17-year old female with progressive hearing loss 2 years after meningitis and vestibular dysfunction in the implanted ear is presented in this study.FindingsThe patient had mild hearing loss in the right ear and total hearing loss on the left side because of complete ossification of the cochlea following meningitis. She had to have cochlear implantation in the right ear because of progression of hearing loss. She had successful implantation but she experienced vestibular dysfunction following activation of cochlear electrodes. Closure of two electrodes caused disruption of auditory programming. Then the patient was subjected to long term vestibular rehabilitation program.ConclusionTiming for implantation before the completion of cochlear ossification is crucial not to miss the chance for hearing restoration. However, difficulties in hearing rehabilitation due to extensive ossification can be doubled by vestibular problems triggered by stimulation of the vestibular nerve by cochlear electrodes. Attempts to reduce the balance problem will complicate auditory programming. Vestibular rehabilitation for long term helps to carry on hearing progress.     

Vestibular function after cochlear implant surgery

Objective: The aim of this study was to analyze vestibular function in cochlear implant (CI) patients for iatrogenic damage to vestibular function.

Methods: Prospective clinical study. Tertiary care audiological center. Twenty-five subjects receiving surgery for cochlear implantation during 2012 and 2013 were analyzed. Both vestibulo-ocular and vestibulo-spinal responses before and 2 months after CI surgery were evaluated using several tests: analysis of spontaneous nystagmus, head shaking test (HST), and head impulse test recorded by videooculography; caloric stimulation at 44° and 30° in both ears; cervical evoked myogenic potentials and static stabilometry. Residual cochlear function was tested by air-conduction pure-tone audiometry.

Results: Our tests showed damage to vestibular receptors after CI surgery in 12% of the patients; in particular, horizontal semicircular canal function and saccular function had lower responses after surgery. Audiometric results showed poorer thresholds after CI surgery. The static stabilometry results indicate good vestibulo-spinal responses and patients did not report disequilibrium nor postural deficit.

Conclusion: All data suggest an efficient vestibular compensation mechanism in CI patients.     

Can HIV-infected patients undergo cochlear implantation?

Objective: HIV-infected patients have an increased risk of developing sensorineural hearing loss. Cochlear implantation can be a mean of rehabilitating such patients.

Methods: A 36-year-old HIV-infected male with bilateral profound sensorineural hearing loss underwent cochlear implantation after exclusion of medical and radiological contraindications. The patient was followed up for 2 years to assess the audiological outcome.

Results: After a follow up of 2 years, the patient has attained good functional results not experienced any complications.

Conculsion: Cochlear implantation can restore hearing to HIV-infected patients with profound hearing loss due to cochlear impairment. This helps in both social and vocational rehabilitation and can improve the quality of life of such patients.     

Bilateral cochlear implantation in bilateral Langerhans cell histiocytosis

Abstract

Background

Langerhans’ cell histiocytosis (LCH) is a rare proliferative disorder that can have otologic manifestations in up to 30% of patients. Treatment of local and systemic disease may include medical, surgical, and radiation therapies. Involvement of the temporal bone can lead to conductive and, rarely, sensorineural hearing loss. Post-labyrinthectomy cochlear implantation can be an effective treatment option for sensorineural hearing loss in the setting of persistent LCH.

Methods

A retrospective case review at a tertiary academic medical center. Hearing in Noise Test (HINT) performed before and after bilateral cochlear implantation was examined.

Results

Following bilateral partial labyrinthectomy, post-operative testing showed a HINT in quiet of 17%. Left-sided cochlear implant followed by immunosuppressive therapy for persistent disease showed marked improvement with post-operative HINT in a quiet room of 80% and 63% at 1 and 2 years. Fifty-five months after left implantation, and 10 months after right cochlear implantation, binaural post-operative HINT in quiet was 81%.

Conclusion

Surgical excision of LCH lesion remains a mainstay of treatment for temporal bone involvement. Bilateral cochlear implant with adjuvant immunosuppression in our patient demonstrated both immediate and delayed improvement in auditory function after staged cochlear implantation in the setting of persistent disease.     

Iatrogenic facial nerve exposure in cochlear implant surgery: incidence and clinical significance in the absence of intra-operative nerve monitoring

Objectives: Iatrogenic facial nerve injury is one of the most feared complications of cochlear implantation. Intraoperative facial nerve monitoring is used as an adjunctive modality in a variety of neurotologic surgeries including cochlear implantation. With the lack of nerve monitoring, there is a theoretically higher risk of iatrogenic fallopian canal dehiscence with facial nerve exposure, particularly the mastoid portion, during cochlear implant surgery. The purpose of this study is to determine the incidence of iatrogenic exposure of the facial nerve and its relation to the incidence of post-operative facial paralysis in the absence of facial nerve monitoring.

Methods: This was a retrospective study. Medical charts of 307 patients who underwent cochlear implantation without facial nerve monitoring, from 2012 to 2017 were reviewed to identify cases with a reported iatrogenic defect over the mastoid facial nerve. The incidence of post-operative facial palsy was determined and compared to the incidence with the use of intra-operative monitoring which has been reported in the literature.

Results: The incidence of iatrogenic dehiscence with facial nerve exposure was 46.58%. However, the incidence of post-operative facial palsy was only 2.1% which decreased to 0.72% in cases without injury of the facial neural sheath. This was not significantly different from the 0.73% rate reported in the literature with the use of intra-operative facial monitoring (P?=?0.99).

Conclusion: The incidence of iatrogenic facial nerve exposure during cochlear implantation may be relatively high. However, no additional risk of post-operative facial nerve paralysis was found, provided that the integrity of the neural sheath was preserved, even with the lack of intra-operative monitoring.     

Cochlear implantation following cerebellar surgery

Abstract

Cochlear implantation in patients with known central nervous system conditions can result in wide-ranging outcomes. The aim of this study is to report two cases of cochlear implantation outcomes in patients with acquired cerebellar ataxia following cerebellar surgery. The first is a female implanted with the Nucleus 24 implant in September 2000 and the second is a male implanted with a MED-EL Sonata Flexsoft electro-acoustic stimulation in July 2009. Programming these patients resulted in significant non-auditory stimulation which resulted in less than optimum map fittings. The patients did not gain any open set speech perception benefit although both of them gained an awareness of sound with the device. However, patient 2 elected to become a non-user because of the limited benefit.     

Systematic review of cochlear implantation in CHARGE syndrome

Objective: CHARGE syndrome presents with a collection of congenital anomalies affecting multiple organs. Ear and temporal bone anomalies, including hearing loss are highly prevalent.

We present an aid to management of this challenging condition and report the strategies and outcomes of cochlear implantation.

Methods: Systematic review of Medline, EMBASE, Web of Science, CENTRAL and clinicaltrials.gov was performed up to 21/10/2018

The review and meta-analysis of studies were performed according to the PRISMA statement.

Patient demographics, comorbidity, anatomical factors, details of cochlear implantation and audiological outcome were extracted and summarized. Anatomical and surgical factors were evaluated by meta-analysis. Audiological outcomes reported were too heterogeneous for meta-analysis.

All statistics were calculated with SPSS v23.0 (IBM, New York, USA).

Results: Thirty-one studies reported 165 cochlear implants in 156 patients with CHARGE syndrome. Temporal bone and facial nerve anomalies were common.

Discussion: The assessment and management of patients with CHARGE syndrome undergoing cochlear implantation is challenging. Outcomes may be affected by cochlear nerve deficiency, inner ear anomalies, and developmental delay. Surgery is almost invariably complicated by abnormal anatomy, and increased incidence of complications.

Conclusion: A careful, case-by-case assessment of an individual's requirements within a multi-disciplinary setup is essential for achieving the best possible outcome.     

Feasibility of hearing preservation for residual hearing with longer cochlear implant electrodes

Abstract

Background: Hearing preservation is thought to be achievable following atraumatic surgery with thin cochlear implant electrodes; therefore, the surgical approach and implant electrode design are crucial considerations.

Objective: To assess the feasibility of hearing preservation with long electrodes for patients meeting the criteria for conventional cochlear implantation.

Methods: One hundred and two patients (132 ears) who underwent cochlear implant surgery were analyzed. Inclusion criteria included measurable residual hearing in the low frequency before implantation and not meeting the criteria for electric acoustic stimulation (EAS).

Results: Of the 18 patients with residual hearing in the low frequency enrolled, 17 subjects (94.4%) retained low frequency hearing. A younger age at surgery tended to contribute to better hearing preservation than that observed in older patients. There was no clear trend regarding the influence of insertion depth angle of the electrode on hearing preservation.

Conclusion: It is possible to achieve hearing preservation in the lower frequency by the use of longer electrodes. This study underscores the importance of atraumatic surgery, even for patients with only limited residual hearing, and longer electrodes should be adopted for EAS.     

Cochlear implantation in a previously irradiated temporal bone — a case report

Abstract

A case of successful cochlear implantation following irradiation of the temporal bone is presented. Loss of cochlear architecture is demonstrated and is attributable to previous radiation. Viability of cochlear nerve and spiral ganglion following radiation is questioned. To date there has been only one previously published report on the subject     

Translabyrinthine vestibular schwannoma resection with simultaneous cochlear implantation

Objectives: (1) Report a rare case of translabyrinthine resection of a sporadic vestibular schwannoma (VS) and concurrent cochlear implantation (CI). (2) Discuss pre-, intra-, and post-operative considerations in this unique patient population. (3) Describe surgical and audiologic outcomes reported in this population.

Methods: Case report and review of the literature. PubMed search ‘Cochlear Implantation’[Mesh] AND (‘Neuroma, Acoustic’[Mesh] OR ‘VESTIBULAR SCHWANNOMA’[All Fields] OR ‘SCHWANNOMA’[All Fields]) limited to humans and English language. Returned 64 search results, abstracts and references of relevant papers reviewed.

Results: A 75-year-old male with longstanding history of slowly progressive severe hearing loss and tinnitus presented for evaluation of worsening imbalance, vertigo, and nausea. Workup revealed a 7?mm right intracanalicular mass on MRI concerning for vestibular schwannoma. Audiogram showed bilateral, symmetric, severe-to-profound sensorineural hearing loss, with poor open-set speech comprehension while bilaterally aided. He underwent successful concurrent right translabyrinthine resection of his VS with complete preservation of the cochlear nerve and uncomplicated cochlear implantation.

Discussion: Literature review revealed few previous reports of simultaneous VS and CI. The vast majority of these were in patients with neurofibromatosis Type 2 in whom auditory outcomes were poor. This patient represents one of the few cases of concurrent translabyrinthine tumor removal and CI for a spontaneous VS.

Conclusion: Single-stage cochlear implantation and translabyrinthine tumor resection is a feasible and safe option to consider for auditory rehabilitation in rare situations.     

Single-sided deaf tactical unit officer treated with cochlear implantation

Objectives: To demonstrate the efficacy of cochlear implantation for management of a severe single-sided deaf tactical unit officer. Showing that hearing improvement provided by cochlear implantation was sufficient for him to return to work as a police tactical officer.

Methods: Case report.

Results: A 43 years-old man working as a tactical unit officer suffered from a work-related severe single-sided hearing loss. He tried unsuccessfully many types of hearing aids including a contralateral routing of sound (CROS) system and a bone conduction hearing aid with a headband. He was finally treated with cochlear implantation which provided enough localization and hearing abilities for a complete return to work as a tactical unit officer.

Discussion: This case report demonstrates that cochlear implantation was very successful for a patient suffering from severe single-sided hearing loss. The current literature demonstrates benefits from cochlear implant over CROS and osseointegrated devices in unilateral hearing loss. Nevertheless, literature does not report any case of patient suffering from single-sided deafness that received a cochlear implant and a complete bilateral hearing rehabilitation that allowed him to return to work as a police tactical officer.

Conclusion: Cochlear implantation was the only effective treatment to provide our patient enough localization and hearing abilities for a complete return to work as a police tactical officer. As no consensus has been established about selection criteria for cochlear implantation in single-sided deafness, we suggest that the patient’s occupation should be considered when reviewing his candidacy for cochlear implantation.     

Case Report: Early bilateral simultaneous cochlear implantation in a five-year-old following head injury complicated by acute bacterial meningitis

Abstract

Meningitis is an important cause of profound sensorineural hearing loss, especially in children. In this case, a five-year-old suffered a head injury complicated by bacterial meningitis and developed a profound hearing loss. Magnetic resonance imaging at four weeks following injury showed evidence of developing labyrinthitis ossificans and a decision was made to perform bilateral cochlear implantation at an early stage. This report outlines the progress of this interesting case to date and discusses the rationale for the decision to implant in this way.