Evaluation of the impact of hearing loss in adults: Validation of a quality of life questionnaire.

OBJECTIVES: The impact of hearing loss and of auditory rehabilitation (hearing aid, cochlear implant) on quality of life is a crucial issue. Commonly used questionnaires to assess quality of life in these patients (Nijmegen, APHAB, GBI) are time-consuming, difficult for patients to fill out, and show poor sensitivity to small improvements or deterioration. The objective of the present study was to validate a dedicated quality of life scale for hearing-impaired adults with or without auditory rehabilitation. MATERIAL AND METHODS: ERSA (Évaluation du Retentissement de la Surdité chez l'Adulte: Evaluation of the Impact of Hearing Loss in Adults) is a self-administered questionnaire. It is divided into 4 domains, each comprising 5 questions graded from 1 to 10. The questions are simple, and formulated so patients will answer according to how they feel at the actual time of the session. Test-retest reliability was measured in 38 patients. Internal coherence and validity against the APHAB questionnaire as gold standard and in relation to hearing performance were measured in 122 patients at auditory assessment. Sensitivity to change in hearing was measured in 36 cochlear implant patients, before and 6 or 12 months after implantation. RESULTS: Test-retest reliability was very satisfactory (ρ=0.88). Internal coherence was good for all questions. External validity, comparing ERSA to APHAB scores in the same non-implanted hearing-impaired patients, was good (ρ=0.52). Additionally, ERSA scores correlated with hearing performance in adverse conditions (monosyllabic words: ρ=0.22; sentences in noise: ρ=0.19). In patients tested before and after cochlear implantation, improvement in hearing performance in silence and in noise correlated with an improvement in ERSA score (ρ=0.37 to 0.59, depending on the test), but not to GBI score. CONCLUSION: The ERSA questionnaire is easy and quick to use, reliable, and sensitive to change in hearing performance after cochlear implantation.

[A report of three cases and review of auditory brainstem implants in children]. / Implantation auditive du tronc cérébral chez l'enfant. À propos de trois cas.

AIM OF THE STUDY: To present three pediatric cases of auditory brainstem implantation (ABI) and review literature data concerning this topic. PATIENTS: The first two children had a neurofibromatosis type II with bilateral sensorineural deafness; in both cases, the implant was inserted during the surgical removal of a vestibular schwannoma; the third patient had profound deafness due to bilateral cochlear nerve insufficiency associated with inner ear malformation. RESULTS: Two postoperative complications were observed: patient 1 had a persistent fever which required the replacement of the fat graft used to seal the translabyrinthine approach; patient 3 had a CSF leakage requiring additional surgery and lumbar external drainage. In our three patients, the numbers of active electrodes were 6/22 (Cochlear ABI 24M ABI), 11/12 (Medel Opus II ABI) and 11/12 (implant Medel), respectively. Due to additional major surgical procedures and to disappointing functional results of the ABI, patient 1 stopped wearing her implant 18 months after implantation. Nine months after surgery, patient 2 achieved open-set speech recognition and was very satisfied with the implant. Six months after implantation, patient 3 (cochlear nerve deficiency), who was 3.5 years-old at the time, clearly reacted to some environmental sounds but was not yet able to achieve speech recognition. CONCLUSIONS: ABI has now entered the list of treatments that can be proposed in pediatric profound sensorineural deafness. Its major risks of complications are CSF leakage and non-auditory side effects. Its outcomes are worse and less predictable than cochlear implants. Thus, its indications must remain restricted to cases meeting the following conditions: absence of alternative option to restore hearing, patients and parents high level of motivation and realistic expectations.

[Auditory brainstem implant: indications and results]. / Implant auditif du tronc cérébral: indications et résultats.

OBJECTIVES: To summarize the indications and evaluate the Auditory Brainstem Implant (ABI) performances in neurofibromatosis type 2 (NF2) and other otologics indications, as postmeningitis ossified cochlea. MATERIAL AND METHODS: Main and first indication of ABI is NF2. Emergent indications are bilateral total ossified cochlea, vestibular schwannoma with controlateral lesions, cochlear nerve aplasia or inner ear's malformations. The pre-operative evaluation includes clinical, radiological, lipreading, and psychological status. A translabyrinthine or retrosigmoid approach is performed, depending on tumoral or not tumoral status. The auditory perception with the ABI is evaluated by testing, the words recognition in open-set lists, and the speech understanding with usual sentences. RESULTS: In NF2 patients, best results are obtained in cases of smaller vestibular schwannoma and none, or short term, auditory deprivation. Negative prognostic factors are duration of total hearing loss (>10 years), tumor size (>30 mm), difficulties in electrode array placement, complications during post-operative course and number of active electrodes (<10). In cases of postmeningitis total deafness with totally ossified cochlea, results demonstrate a good benefit reaching these obtained with cochlear implant in post-meningitis deafness. CONCLUSION: These results show a clear benefit of ABI in NF2 patients, with or without previous tumor removal, in case of small tumor with a short duration of hearing loss. In case of postmeningitis ossified cochlea, results potentially reach those of cochlear implants.

Cochlear implantation and far-advanced otosclerosis.

OBJECTIVE: To evaluate results of cochlear implantation in patients with far-advanced otosclerosis. METHODS: Sixteen patients with far-advanced otosclerosis had undergone unilateral (n = 13) or bilateral (n = 3) cochlear implantation. Surgical difficulties, incidence of complications and postoperative benefit were analyzed. RESULTS: A full electrode insertion was achieved in all patients without surgical difficulties. All patients demonstrated excellent benefit of cochlear implantation. Binaural implantation still improves speech performances, compared to unilateral implantation. In case of residual cochlear function of one nonoperated side, a stapes surgery, performed during the same surgical time as cochlear implantation, can improve speech scores and restore bilateral hearing. Facial nerve stimulation occurred only in 1 patient. CONCLUSION: Cochlear implantation is the method of choice for rehabilitation of patients with otosclerosis, presenting profound or total hearing loss. Patients obtain excellent benefit with a low rate of complications.

[Performances and complications of cochlear implant in 134 adult patients implanted since 1990]. / Performances et complications de l'implant cochléaire chez 134 patients adultes implantés depuis 1990.

OBJECTIVE: The aim of this study was to analyse performance of cochlear implants in a retrospective series of adults with postlingually in order to search for predictive factors and identify complications. METHODS: Between 1990 and 2003, 134 adult patients were implanted: 129 patients on one side and 5 patients on both sides. Hearing benefit at 6, 12, 24 and 36 months and performance at 12 months were analyzed as function of the etiology, age at implantation, lipreading and speech coding strategy. RESULTS: Compared to performances before implantation, cochlear implants provided significant and rapid improvement at 6 months (p<0.0001). The results were not correlated with age at implantation, etiology or lipreading ability before implantation. Performances were worse in patients with meningitis (p<0.01), but the percentage of improvement was similar between the different etiologies. Major complications were rare: one case of regressive postoperative facial nerve weakness. CONCLUSION: Cochlear implants provide significant and rapid improvement, with low morbidity. Results are not correlated with age at implantation, etiology or lipreading ability.

[Beneficial effect of cochlear implants in the elderly]. / Bénéfice de l'implant cochléaire chez le sujet âgé.

OBJECTIVE: To evaluate the benefit of cochlear implantation in older adults aged 60 Years and over. METHODS: Fifty-six profoundly or totally hearing-impaired patients, aged 60 Years and over, were studied retrospectively. At the end of the preoperative evaluation, 28 patients received a cochlear implant. The mean age was 66 Years and the median follow-up was 22.5 Months. Speech perception scores before and after implantation were analyzed in order to evaluate the benefit of cochlear implantation. The speech perception score before implantation was compared to that of the non-implanted patients. RESULTS: There was a significant improvement of the dissyllabic words and sentences scores after implantation. The patients who are over 70 Years performed as well as those who are younger (between 60 and 70 Years). One patient developed a postoperative vertigo due a perilymphatic fistula. There was no flap-related problems. In the non-implanted group (mean age: 68 Years), 18 patients declined the cochlear device because they thought the subjective benefit of their hearing aid was sufficient and 5 patients declined because of surgical risk. The mean age, the cause and the duration of the deafness, and the speech perception scores were similar between implanted and non-implanted patients. CONCLUSION: This study demonstrates the beneficial effect of cochlear implantation in the elderly. These results suggest that a similar benefit could have been obtained in the patients who declined surgery. An early implantation could reduce the duration of the deafness and preserve binaural sound perception allowing increased performance in older people.

[Progressive bilateral hearing loss with superficial hemosiderosis of the central nervous system: contribution of cochlear implantation]. / La surdité dans l'hémosidérose superficielle du système nerveux central. Intérêt de l'implantation cochléaire.

Two cases of progressive bilateral hearing loss associated with superficial hemosiderosis of the central nervous system are reported. This is a rare disease caused by repetitive hemorrhage in subarachnoid spaces with hemosiderin deposits on the brain surface and cranial nerves. MRI provided the etiologic diagnosis in both cases based on typical low-density signals from the brain, the brainstem, and the cerebellar surfaces on T1 and T2 sequences. In one case a fourth ventricle ependymoma, which was the probable cause of hemosiderosis, was also discovered. Based on a literature review, we discuss the pathophysiological hypotheses, the modalities for treating hearing loss including cochlear implantation despite retrocochlear sensorineural hearing loss.