Differences in perception of hearing handicap between cochlear implant users and their spouses.

OBJECTIVES/HYPOTHESIS: To examine the relationship between patient and proxy ratings of health-related quality of life (HRQL) in patients with cochlear implants. STUDY DESIGN: Prospective survey. METHODS: Eighty-two adult cochlear implant recipients and their spouses/partners were asked to complete the Nijmegen Cochlear Implant Questionnaire (NCIQ) and the Hearing Participation Scale (HPS) that were mailed to them. RESULTS: For the NCIQ, there were 38 useable responses from recipients and 31 useable responses from the partners. There were 27 paired and useable questionnaires (i.e., from both the recipient and his/her partner). For the HPS, there were 42 useable responses from recipients and 36 useable responses from the partners. Of these, there were 35 paired questionnaires. There was good agreement between partners and patients with both the HPS (r = 0.62) and the NCIQ (r = 0.80). There was a significant statistical difference in the scoring of the Basic Sound Perception subscale between the two groups (P = .039), with spouses under-rating performance in this subscale. CONCLUSIONS: For patients with cochlear implants, patients' partners corroborate patients' self-reported HRQL. In cases in which patients have sensory impairment, there may be an additional role for proxy ratings of HRQL to give a more complete assessment of functional limitations.

Refractory effects of the N1 event-related potential in experienced cochlear implant patients.

OBJECTIVE: To determine if cochlear implant (CI) patients exhibit a temporal processing deficit for auditory stimuli, by examining refractory effects of the N1 event-related potential (ERP) component. DESIGN: CI patients and normally-hearing controls were tested in an auditory refractory period paradigm while ERP recordings were collected across the scalp. Participants were presented with brief 500-Hz tones that were separated by inter-stimulus intervals (ISIs) of 500, 1000, or 3000 ms. The amplitude of the N1 was examined as a function of ISI within each group. STUDY SAMPLE: Ten adult CI patients and 13 age-matched normally-hearing controls were tested. Patients had long-lasting severe or profound sensorineural hearing loss prior to implantation, and a minimum of two years experience with CI activation. RESULTS: Unlike normally-hearing controls, CI users showed no refractory effect for tones at 500 ms ISIs compared to 1000 ms. However, similar to controls, recovery from refractoriness was observed in anterior locations at 3000 ms. CONCLUSION: The refractory period threshold, defined as the minimum ISI where different N1 amplitudes are elicited, is greater than 1000 ms in CI patients; at least double that of normally-hearing controls.

Cochlear implant rehabilitation for patients with vestibular schwannoma: report of two cases.

OBJECTIVE AND IMPORTANCE: The objective of this paper is to highlight two main points. The primary aim is to demonstrate that cochlear implants can function in the presence of retrocochlear pathology, even after stereotactic radiosurgery (SRS), and hence to introduce this as a management option in selected patients with retrocochlear pathology, such as Neurofibromatosis type II (NFII) patients. A secondary aim is to act as a caveat that computed tomography (CT) scanning alone may not be sufficient imaging in subjects undergoing cochlear implantation (CI). CLINICAL PRESENTATION: In this paper we report two patients who underwent cochlear implant despite the presence of a vestibular schwannoma (VS) on the same side. The first case is a 59-year-old male, diagnosed with VS after 9 months of good hearing with a cochlear implant. The second case is 26-year-old female known case of NFII, received a cochlear implant after controlling the tumor growth with a SRS. CONCLUSION: We show the consequences of missing important pre-implant pathology prior to CI in one case. In both cases, we add to the literature showing that cochlear implants can work well in the presence of VS, even in the presence of previous SRS. This adds significantly to the management options available to NFII patients, and the results seem to be better than those expected for auditory brainstem implant (ABI), and with a much simpler and safer intervention.

Cochlear implant magnet extrusion with subsequent surgical replacement and restoration of full implant use without the need for device explantation.

OBJECTIVE AND IMPORTANCE: We recount the unusual case of a young boy whose removable cochlear implant magnet extruded through the skin after becoming displaced after several episodes of direct but minor head trauma. This article outlines a course of clinical management that resulted in the successful re-implantation of a new magnet without infective sequelae and the need for device explantation. CLINICAL PRESENTATION: The child presented with recurrent erythema and swelling at the implant site. In retrospect, subtle magnet displacement was not readily identified on initial imaging. The external magnet required an increase in strength while local swelling settled, but subsequently had to be reduced as the magnet was drawn gradually to the skin surface. Full extrusion took place, but in the absence of any obvious infection, the site healed quickly. INTERVENTION AND TECHNIQUE: The external device was retained with a self-adhesive disc for 1 year, during which time the patient was observed closely for signs of local infection. The child was subsequently admitted for surgical re-exploration and insertion of a new magnet. Care was taken to re-establish the integrity of the fibrous pocket that surrounded the cochlear implant. CONCLUSION: This uneventful intervention was followed by the resumption of full and unimpaired implant use. At the time of this article submission, the child is now 5 years post-extrusion and continues to do well in full-time education.

Cochlear implantation in Cockayne syndrome: our experience of two cases with different outcomes.

Cockayne syndrome is a rare autosomal recessive defect in DNA repair resulting in a classic facies with potential visual and auditory impairment. The hearing loss begins peripherally and may become central as the condition progresses. Coexisting sensory deprivation from visual impairment and the possibility of progressive deterioration in mental function conspire with a lack of published experience to produce many challenges for the cochlear implant team. To the best of our knowledge, we present the first case reports with documented follow-up of cochlear implantation in two patients with different manifestations of Cockayne syndrome.

Refractory severe facial nerve cross-stimulation and loss of auditory sensation after ten years of uneventful cochlear implant use. A rare and challenging case.

Facial nerve stimulation after cochlear implantation has been well described. When the implant is first activated it is usually possible to 'programme out' the unwanted cross-stimulation. We report an exceptional case of delayed facial nerve stimulation and auditory failure after ten years of uncomplicated implant use. This sudden change proved refractory to reprogramming strategies. The patient refused contralateral implantation as he felt there was residual aided hearing. The implant was removed and replaced. Integrity testing of the explanted device showed it to be functioning normally. Severe facial nerve cross-stimulation persisted and the patient failed to regain his previous auditory sensation. Subsequently, despite extensive investigation and a trial of fluoride therapy, after a third implant he has performed equally badly leading to non-use.