Maurizio Guida

Hearing Rehabilitation in Neurofibromatosis Type 2 Patients: Cochlear versus Auditory Brainstem Implantation

Objective: We aimed to evaluate and compare the auditory performance of neurofibromatosis type 2 (NF2) patients with bilateral total deafness fitted with cochlear or auditory brainstem implants. Patients and Methods: A retrospective case review was performed. Nine patients suffering from NF2 who underwent hearing rehabilitation by means of cochlear (4 patients) or auditory brainstem (5 patients) implantation participated in the study. Postoperative auditory performance was assessed using closed- and open-set tests. Results: In the group of patients fitted with a cochlear implant, 3 subjects achieved open-set speech recognition abilities comparable to those of standard adult postlingual implant patients; the remaining patient scored 0% in all open-set format tests, reporting benefits only in environmental sound detection and lip-reading. Among the 5 patients who underwent auditory brainstem implantation, 1 reached good open-set speech recognition skills, scoring 70% in the common phrases comprehension test, and she was able to communicate on the telephone. Two other patients achieved open-set speech understanding (respectively, 33 and 41% in the common phrases comprehension test), reporting daily use of their device. The remaining 2 patients did not achieve any level of open-set speech perception, obtaining only improved access to environmental sound and lip-reading skills. Conclusions: Our study confirmed literature data reporting that cochlear implantation may offer open-set speech communication in NF2 patients. In this small cohort, cochlear implant patients performed better than auditory brainstem implant patients, even if variability in auditory performance was observed with both devices. More studies are needed in order to clarify the role and reliability of electrophysiological tests in predicting the residual functionality of the cochlear nerve after tumor removal.

Favorable outcome of cochlear implant in VIIIth nerve deficiency.

Objective: To report on the outcomes of cochlear implantation (CI) in a child with cochleovestibular nerves (CVN) hypoplasia. Study Design: Retrospective case review. Setting: Tertiary referral center, University hospital. Patients: An 18-month-old child with profound bilateral congenital hearing loss and bilateral hypoplasia of the CVN at imaging. Intervention: Left CI at age 29 months with a Nucleus Contour device (Cochlear Ltd., Lane Cove, New South Wales, Australia) after unsatisfactory results of hearing aid use for 10 months. Main Outcome Measures: Speech perception tests, behavioral observation, electrophysiologic tests, and cognitive evaluation. Results: Although the child scores poorly in every perceptive category with the CI alone, the device greatly enhances his speech understanding with the hearing aid in the opposite ear. In the bimodal condition, his words and sentences identification, recognition, and comprehension far exceed the monaural figures. The Meaningful Auditory Integration Scale (MAIS) tests reaches a score of 26/40, and the MacArthurs questionnaires confirm the improvement of language production and comprehension. These results became noticeable after 5 to 6 months and continued to improve up to the 10th month. The childs cognitive scores and overall performance competences greatly benefit from the CI, with the mental age overcoming the chronological age. Conclusion: We can confirm the chance of achieving satisfactory results by CI even when the imaging of CVN is doubtful and the electrophysiological tests are disappointing. In our experience, a CI in Type IIb dysplasia of the CVN is a feasible option, provided that the candidate shows some responses at aided audiogram and at least minimal signs of language development. Adequate counseling is necessary for these children because the expected outcome is somewhat lower than that of their deaf peers with normal appearance of the nerves.

Cochlear Implantation and Cogan Syndrome

Objective To evaluate outcomes and issues pertaining to cochlear implantation in a group of subjects affected by Cogan syndrome. Study Design Prospective cohort. Setting Department of Ophthalmology and Otorhinolaryngology, University of Parma. Patients Five postlingually deafened adults suffering from a typical form of Cogan syndrome who underwent cochlear implantation. Main Outcome Measures Benefit from cochlear implantation as measured by word and everyday sentence recognition tests. Surgical issues and postoperative complications were also evaluated. Results In two cases, intracochlear electrodes were inserted into the scala vestibuli because of the ossification of the scala tympani. Two patients experienced a recurrence of keratitis the day after surgery. To date, with a follow-up of 1 to 4 years, no patient has experienced flap complications or other local or systemic complications. At the 12-month postoperative evaluation, all patients had gained useful open-set speech perception, achieving a mean score of 91% and 95% on word and everyday sentence recognition tests, respectively. Conclusions Patients deafened by Cogan syndrome demonstrated high levels of speech understanding after undergoing cochlear implantation. Obliteration of the cochlea may complicate electrode implantation, requiring modifications of the surgical technique. Stress consequent to the surgical procedure may instigate an acute phase of the basic illness.

Auditory brainstem implants in NF2 patients: results and review of the literature

Objective Neurofibromatosis Type 2 (NF2) patients have multiple central nervous system tumors and, specifically, bilateral vestibular schwannomas (VSs) causing bilateral deafness. If the cochlear nerve is not preserved during tumor removal, the only hearing rehabilitation in these patients could be via an auditory brainstem implant (ABI). Study Design Retrospective case study and literature review. Setting Tertiary referral cranial base center. Patients In 24 NF2 patients, 25 ABIs were placed in the lateral recess of the fourth ventricle after VS surgery via a translabyrinthine approach. Results In this series, a large range of results are observed: from open speech and use of the telephone to no ABI use, because of the poor sound identification ability. Of the 24 patients, 19 use their ABI on a daily basis, 4 are nonusers, and 1 died of NF2 progression. A multivariate analysis did not reveal a good predictor for ABI outcome. In literature, the results of ABI in NF2 are difficult to compare, and the overall outcome was poor compared with cochlear implantation results. Conclusion Auditory brainstem implantation in NF2 patients directly after tumor removal is a safe procedure and the best means of hearing rehabilitation if the cochlear nerve is not preserved. The results in NF2 cases in the literature and these series are poor compared with cochlear implantation. If a cochlear implant is possible, it has the preference over an ABI, also in NF2. Nevertheless, the majority of the patients have benefit of the ABI during daily life particularly in combination with lip reading.

Cochlear implantation in children with cerebral palsy. A preliminary report

OBJECTIVES The aim of this study is to assess the post-implantation speech perception and intelligibility of speech produced by five profoundly deaf children with cerebral palsy. METHODS This study is derived by a review of a prospectively maintained data collection on all patients entering the cochlear implant program. Five children with cerebral palsy who underwent cochlear implantation participated in this study. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. The follow-up of the series ranged from 12 to 45 months. RESULTS At the last follow-up, two children who were placed into speech perception category 1 (detection of a speech signal) preoperatively progressed to category 6 (open-set word recognition with familiar words) postoperatively. Two children moved from preoperative category 2 (pattern perception) to postoperative category 6. One child placed into category 0 (no detection of speech) preoperatively progressed to category 4 (word identification) postoperatively. Before implantation, three children had connected speech unintelligible, and two subjects had connected speech intelligible to a listener who concentrates and lip-reads. At the last follow-up, one child had connected speech unintelligible, two children had connected speech intelligible to a listener who concentrate and lip-reads, one child had connected speech intelligible to a listener who has little experience of a deaf persons speech, and one child had connected speech intelligible to all listeners. CONCLUSIONS Cochlear implantation allowed these patients to dramatically improve their quality of life, increasing their self-confidence, independence and social integration.

Auditory brainstem implant in a child with severely ossified cochlea.

Objective: The hearing outcome after implanting a severely ossified cochlea has always been less satisfactory than implanting a patent one. The aim of our study is to present a case where brainstem implantation was successfully performed as an alternative to cochlear implantation in a child with bilateral severe ossification of the cochlea.

Comparison of speech perception benefits with SPEAK and ACE coding strategies in pediatric Nucleus CI24M cochlear implant recipients

Nine congenitally deaf children who received a Nucleus CI24M cochlear implant and who were fitted with the SPrint speech processor participated in this study. All subjects were initially programmed with the SPEAK coding strategy and then converted to the ACE strategy. Speech perception was evaluated before and after conversion to the new coding strategy using word and Common Phrase speech recognition tests in both the presence and absence of noise. In quiet conditions, the mean percent correct scores for words were 68.8% with SPEAK and 91% with ACE; for phrases the percentage was 66.6% with SPEAK and 85.5% with ACE. In the presence of noise (at +10 dB signal-to-noise ratio), the mean percent correct scores for words were 43.3% with SPEAK compared to 84.4% with ACE; for phrases the percentage was 41.1% with SPEAK and 82.2% with ACE. Statistical analysis revealed significant improvement in open-set speech recognition with ACE compared to SPEAK. Preliminary data suggest that converting children from SPEAK to the ACE strategy improves their performance. Subjects showed significant improvements for open-set word and sentence recognition in quiet as well as in noise when ACE was used in comparison with SPEAK. The greatest improvements were obtained when tests were presented in the presence of noise.

Nucleus multichannel cochlear implantation in partially ossified cochleas using the Steenerson procedure.

Objective The purpose of this study was to report hearing results obtained in a group of subjects who received scala vestibuli implantation because of cochlear ossification and to compare these results to those in patients with scala tympani implantation. Study Design Retrospective analysis of consecutive cochlear implant procedures. Setting Department of Otolaryngology, University of Parma. Patients Five postlingually deafened adults with an electrode array placed into the scala vestibuli were compared with the speech performance of matched controls who had the electrode array inserted into the scala tympani. Main Outcome Measures Measures included vowel and consonant speech identification scores, bisyllabic word and sentence speech recognition scores, and common phrases comprehension scores. Results No significant difference was detected on speech performances between the subjects with scala vestibuli implantation and the control group. Conclusion Scala vestibuli implantation appears to be an excellent alternative in cases in which scala tympani is found to be not patent.

Multichannel cochlear implantation in radical mastoidectomy cavities

OBJECTIVE: We report on our experience in cochlear implantation in patients with radical mastoidectomy cavities. STUDY DESIGN, SETTING, AND METHODS: Retrospectively, records of patients from the Department of Otolaryngology, University of Parma between December 1991 and March 2000 were reviewed, and 6 postlingually deafened adults who received a cochlear implant in a radical cavity were identified. Speech performances were evaluated in terms of bisyllabic word and sentence recognition and common phrase comprehension. RESULTS: To date, with a follow-up of 1 to 9 years, no patient has experienced extrusion of electrodes or other local or intracranial complications. Mean bisyllabic word and sentence recognition scores were 74% and 80%, respectively. Mean comprehension score for common phrases was 86%. CONCLUSION: By obliterating and isolating the radical mastoidectomy cavity from the outer environment, patients who previously had undergone radical surgery of the middle ear can be safely implanted with satisfactory hearing results. Multichannel cochlear implantation (CI) is a treatment accepted worldwide for patients with total or profound deafness. In the presence of normal temporal bone anatomy, CI surgery is a safe and relatively simple procedure with a low complication rate. 1–3 However, some conditions, such as malformations of the middle or inner ear, cochlear ossification, chronic otitis media, and previous middle ear surgery, represent a technical challenge to CI surgeons. Patients with a bilateral radical mastoidectomy cavity who are otherwise suitable for implantation represent a certainly more problematic group to manage than the “standard” CI patients. Under these circumstances, various potential problems must be considered: extrusion of the electrode array by breakdown of the thin epithelial lining of the mastoid cavity, risk of recurrent cholesteatoma, and possibility of spreading of inflammation to the implant with potential labyrinthitis and meningitis. During recent years various surgical strategies have been proposed in the literature to avoid such complications. Many surgeons suggested overcoming these problems by performing an obliterative technique, 4–6 whereas others preferred to maintain the benefits of an open technique 7 or to rehabilitate the cavity 8 ; it has also been suggested that the cavity be bypassed via a middle cranial fossa approach. 9 We describe the experience at the CI Center of the University of Parma in managing 6 CI patients with radical mastoidectomy cavities.

Cochlear implantation in children with cochlear nerve deficiency

OBJECTIVE The aim of this study was to report on auditory performance after cochlear implantation in children with cochlear nerve deficiency. METHODS A retrospective case review was performed. Five patients with pre-lingual profound sensorineural hearing loss implanted in an ear with cochlear nerve deficiency participated in the study. Postoperative auditory and speech performance was assessed using warble tone average threshold with cochlear implant, speech perception categories, and speech intelligibility ratings. All patients underwent high resolution computed tomography and magnetic resonance imaging. RESULTS According to Govaerts classification, three children had a type IIb and two a type IIa cochlear nerve deficiency. Preoperatively, four patients were placed into speech perception category 1 and one into category 2. All patients had an improvement in hearing threshold with the cochlear implant. Despite this, at the last follow-up (range 18-81 months, average 45 months), only one girl benefited from cochlear implantation; she moved from speech perception category 2 to 6 and developed spoken language. Another child developed closed set speech perception and had connected speech that was unintelligible. The other 3 children showed little benefit from the cochlear implant and obtained only an improved access to environmental sounds and improved lipreading skills. None of these 4 children developed a spoken language, but they were all full-time users of their implants. CONCLUSIONS The outcomes of cochlear implantation in these five children with cochlear nerve deficiency are extremely variable, ranging from sporadic cases in which open set speech perception and acquisition of a spoken language are achieved, to most cases in which only an improved access to environmental sound develops. Regardless of these limited outcomes, all patients in our series use their device on a daily basis and derive benefits in everyday life. In our opinion, cochlear implantation can be a viable option in children with cochlear nerve deficiency, but careful counseling to the family on possible restricted benefit is needed.