Paul R. Kileny

Effects of cortical lesions on middle-latency auditory evoked responses (MLR)

Middle-latency auditory evoked responses (MLRs) were recorded simultaneously at 3 or 4 electrode locations in the coronal plane in 5 normal subjects, 11 patients with temporal lobe lesions and in 5 patients with cortical lesions not involving the temporal lobes. In patients with unilateral temporal lobe lesions, the amplitude of Pa and hence that of the Na-Pa complex was reduced over the involved hemisphere but remained intact over the contralateral hemisphere. No MLR asymmetries were demonstrated in patients with cortical lesions that did not affect the temporal lobes or in 2 cases with unilateral anterior temporal lobectomy. The latency of wave V of the auditory brain-stem response was within normal limits in the majority of the patients studied regardless of the site of their cortical lesion.

Pediatric cochlear implant patient performance as a function of age at implantation.

Objective: The objective of this study was to examine the effect that age at implantation has on performance of children who received multichannel cochlear implants. Study Design: This is a retrospective study of 295 children who were broken down into 5 age groups based on age at implantation: 1–3 years, 3–5 years, 5–7 years, 7–9 years, and 9–11 years. Speech perception test scores obtained 12, 24, and 36 months postactivation were compared for the 5 groups using repeated-measures analysis of variance. Setting: This study was carried out at a tertiary academic medical center. Patients: Subjects consisted of 295 children who ranged in age from 12 months to 10 years 11 months at the time they obtained their cochlear implant. Intervention: All patients received their cochlear implant at a single implant facility. Main Outcome Measures: Performance on several speech perception tests was compared 12, 24, and 36 months postactivation. Performance was evaluated as a function of age at implantation. Results: Patients in all 5 groups demonstrated improved scores when compared with scores obtained preoperatively with hearing aids. Repeated-measures analysis of variance (ANOVA) revealed a significant group by time interaction for 3 of the 5 measures. For all three of these measures, children implanted at younger ages demonstrated greater gains in speech perception over time than children implanted at older ages. Conclusions: These results are in agreement with those of previous studies indicating that early implantation facilitates improved development of speech perception skills in profoundly deaf children.

Longitudinal Effects of Botulinum Toxin Injections on Voice-Related Quality of Life (V-RQOL) for Patients with Adductory Spasmodic Dysphonia

Adductory spasmodic dysphonia is a focal dystonia of laryngeal muscles. Patients with this disorder typically have severe vocal difficulties, with significant functional, social, and emotional consequences. There is no widely accepted cure for this condition, however, botulinum toxin injections of the thyroarytenoid muscles are considered by most voice clinicians to be the state of the art treatment. Based on extensive experience treating patients for adductory spasmodic dysphonia, we feel that traditional means of voice assessment do not adequately measure either the disease severity or the treatment outcomes. That is, listening to or acoustically analyzing limited phonatory samples does not capture the functional, social, and emotional consequences of this disorder. These consequences will be reflected in a patients voice-related quality of life (V-RQOL). Using a validated voice outcomes instrument, the V-RQOL Measure, the purpose of this study was to quantify longitudinal changes in the V-RQOL of patients with adductory spasmodic dysphonia who are undergoing botulinum toxin injections. Twenty-seven consecutive new patients presenting with dysphonia to our institution during an 18-month period were diagnosed with adductory spasmodic dysphonia, and treated patients were evaluated prospectively using the V-RQOL Measure. Results indicated that (1) V-RQOL was initially very low for these patients, (2) botulinum toxin injections improved it significantly for each injection cycle studied, and (3) the magnitude of the treatment effect appears to change across injections.

Hearing preservation following suboccipital removal of acoustic neuromas

Advances in the diagnosis and intraoperative management of acoustic neuromas have greatly reduced the incidence of neurologic deficits following their removal. Ninety‐three patients underwent acoustic tumor removal during a 4½‐year period, and hearing preservation was attempted in 20 cases. Hearing was preserved in 65% of the entire series, and excellent results were obtained in tumors less than 1.5 cm. No patient with a tumor greater than 1.5 cm had serviceable hearing preserved when total tumor removal was performed. Two patients, one with neurofibromatosis and one with an acoustic neuroma in an only‐hearing ear, had planned partial tumor removal with preservation of hearing. Preoperative auditory brainstem response results were not predictive of postoperative hearing preservation. Intraoperative auditory brainstem response monitoring demonstrated that loss of wave V consistently correlated with loss of hearing postoperatively, whereas persistence of wave V (with a latency prolongation not exceeding 3.00 ms) was predictive of successful hearing preservation regardless of latency increases.

Self-report of cochlear implant use and satisfaction by prelingually deafened adults.

Objective: Prelingually deafened adults tend to demonstrate smaller improvements in speech recognition after cochlear implantation than do postlingually deafened adults, which had led some professionals to believe that prelingually deafened adults receive only minimal benefit from a cochlear implant. The primary objective of this study was to evaluate cochlear implant use and satisfaction by prelingually deafened adults. Design: A questionnaire was administered to 12 prelingually deafened adult cochlear implant patients to evaluate cochlear implant efficacy and satisfaction. Questionnaire results were contrasted with performance on speech recognition tasks. Results: Although these patients demonstrated little or no improvements in speech recognition 12 mo postoperatively, most patients reported that they used their device regularly, that they were satisfied with their device, and that using the cochlear implant improved both their expressive and receptive communication skills. Conclusions: Procedures other than traditional speech recognition measures should be used to evaluate cochlear implant benefit, particularly with prelingually deafened adults.

Cochlear implantation of children with minimal open-set speech recognition skills

Objective: The purpose of this study was to evaluate the postoperative performance of 12 children who demonstrated some open‐set speech recognition skills before receiving a Nucleus multichannel cochlear implant with a view toward expanding the selection criteria for cochlear implant candidacy to include children who derive minimal benefit from amplification. Design: Pre‐ and postoperative performance of two groups of children were compared. Group 1 consisted of 12 children who demonstrated some open‐set speech recognition skills before receiving a Nucleus multichannel cochlear implant (Borderline group). Group 2 consisted of 12 children who demonstrated no open‐set speech recognition skills before implantation with a Nucleus device (Traditional group). In all children, candidacy was determined based on preimplant binaural aided performance. For most subjects, the poorer ear was selected for implantation. Mean pre‐ and postoperative speech recognition scores of the Borderline subjects were compared to determine the benefit provided by their cochlear implants. Secondly, matched‐pair analyses were used to compare the mean speech recognition scores obtained by the Borderline and Traditional subjects. Results: The scores of the Borderline group improved significantly on five of six speech recognition measures when 6 mo postoperative scores obtained with the implant were compared with preoperative test scores obtained with hearing aids. By the 12 mo postoperative interval, the scores of the Borderline group had improved significantly (p < 0.05) on all six measures. In contrast, scores obtained by the Traditional group had improved significantly on three of six measures at both the 6 and 12 mo postoperative intervals. Comparison of postoperative test scores revealed that the Borderline group scored significantly higher than the Traditional group on three of six measures at the 6 mo test interval and on six of six measures at the 12 mo test interval (p < 0.05). Conclusions: The findings of this study indicate that both groups derive significant benefit from their cochlear implants. Although the mean preoperative audiograms for the implanted ears did not differ significantly for the two groups of subjects, members of the Borderline group exhibited significantly better speech recognition skills than the Traditional group during the first year after implantation. These findings suggest that the increased auditory experience of the Borderline subjects positively influenced their performance with a cochlear implant. The authors advocate that the selection criteria used to determine pediatric cochlear implant candidacy be broadened to include consideration of children who demonstrate minimal open‐set speech recognition skills.

Effects of Preoperative Electrical Stimulability and Historical Factors on Performance with Multichannel Cochlear Implant

We investigated the relationship between results of preoperative transtympanic electrical promontory stimulation, duration of deafness, postoperative implanted psychophysical results, and postoperative speech and speech sound recognition as indicated by a battery of five tests. Our subjects were 10 patients implanted with the Cochlear Corporation multielectrode implant, 1 year postimplantation, with a minimum of 17 active electrodes programmed in the bipolar + 1 mode. The results indicated that preoperative promontory thresholds, the slope of the threshold function, and the duration of auditory deprivation are excellent predictors of postoperative speech and speech sound recognition in the auditory (processor alone) mode. These results have significant implications for patient selection and counseling.

The influence of age at implantation on performance with a cochlear implant in children.

Objective This study involved the assessment of speech recognition abilities as a function of age at implantation and length of cochlear implant use in children who received the Nucleus CI22M cochlear implant. Study Design Two separate analyses were performed. The first analysis involved the assessment of speech recognition performance as a function of length of time with a cochlear implant in 48 patients evaluated at 7 years of age. The second analysis involved the assessment of speech recognition performance as a function of age at implantation in 53 patients evaluated 36 months after implantation. Patients were divided into four groups based on length of implant use or age at implantation, and the results were analyzed by a repeated-measures analysis of variance. Setting This study was carried out at a tertiary academic medical center. Patients Patients consisted of children implanted with a Nucleus Multi Channel cochlear implant programmed with the SPEAK encoding strategy. Their ages at the time of evaluation ranged from 5.5 to 7.8 years. Their ages at implantation ranged from 2.4 to 14.5 years. Interventions All patients received a Nucleus Multi Channel cochlear implant programmed with the SPEAK encoding strategy. Word and sentence recognition tests were administered at various ages and at several postimplantation intervals. Main Outcome Measures Performance as a function of length of cochlear implant use and as a function of age at implantation. Results Patients performed significantly better as length of cochlear implant use increased and age at implantation decreased. When patients were tested at a fixed postimplantation time interval (36 months), there was an overall trend for patients who received the implant at a younger age to perform better in spite of being younger at the time of evaluation. However, these effects were not statistically significant for all speech recognition tests that were administered. Conclusions These results confirm previous findings indicating continued improvement of speech recognition with time in implanted children. Furthermore, the results support the concept of the advantage of a younger age at implantation.

Arytenoid subluxation: diagnosis and treatment.

Both arytenoid subluxation and recurrent laryngeal nerve paralysis (RLNP) may result from injury to the larynx, and they may be difficult to distinguish clinically. A patient with arytenoid subluxation who was initially believed to have RLNP was treated with medialization laryngoplasty 1 year after the injury. Preoperative magnetic resonance imaging and computed tomography effectively demonstrated the cricoarytenoid subluxation, which was confirmed by intraoperative electromyography (EMG) showing normal electrical activity in the thyroarytenoid muscle. Photographs from preoperative fiberoptic laryngoscopy are presented to identify the appearance of arytenoid subluxation. Computed tomographic findings and photographs from laryngoscopy of two patients with RLNP documented by intraoperative EMG evaluation are presented to help distinguish the clinical appearance of this disorder from arytenoid subluxation. An integrated approach to the diagnosis and treatment of arytenoid subluxation is presented.

Prognostic Value of Evoked and Standard Electromyography in Acute Facial Paralysis

Ninety-one patients with idiopathic (n = 62) and traumatic (n = 29) facial paralyses were available for evaluation at least 1 year after the onset of paralysis. In nine cases of idiopathic paralysis and in 12 cases of traumatic paralysis, total intratemporal nerve decompression was performed. The remaining patients were treated with steroids alone. All patients underwent evoked electromyography (EEMG) testing within 2 weeks of the onset of paralysis. Facial nerve recovery was graded using the House-Brackmann facial nerve recovery scale. Subjects were grouped according to maximal decline of compound muscle action potential (CAP), as determined by EEMG, and by level of recovery 1 year after onset of paralysis. Among patients who did not undergo surgical decompression of the facial nerve, incomplete clinical recovery (grade III or higher) was significantly associated with CAP decline of >90% (p < 0.05) for idiopathic paralysis. In contrast, there was no significant association between CAP decline of >90% and clinical outcome in traumatic paralysis. These findings support previous reports of the prognostic value of EEMG in idiopathic facial paralysis, but suggest that this test may have less predictive value in the evaluation of facial paralysis as a result of trauma.