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[Cochlear prosthesis implantation in superficial leptomeningeal hemosiderosis].

Superficial leptomeningeal hemosiderosis is a rare disease of the central nervous system. Chronic bleeding into the subarachnoid space causes deposition of hemosiderin in glial cells and subsequent damage to adjacent brain tissue. There is a characteristic predilection for the cerebellum and eighth cranial nerve. Accordingly, among a variety of symptoms, cerebellar ataxia and sensorineural hearing loss progressing to total deafness commonly occur. To date, the hearing loss has been believed to be purely neural. We present a case of superficial hemosiderosis in a patient with total deafness who was successfully provided with a cochlear implant. Audiometry demonstrated total bilateral cochlear hearing losses but with preserved function of the eighth cranial nerve. These findings make us conclude that in contrast to current opinion, superficial hemosiderosis can initially damage the cochlea alone. Thus, patients with total deafness due to this disorder may benefit from cochlear implantation. All patients require careful audiometric assessment, including promontory stimulation.

Adult↗

[The usefulness of intraoperatively registered, electrically evoked stapedius reflex for the programming of cochlear implants in children].

The programming of a cochlear implant speech processor used by young children is often difficult, especially when the stimulus level associated with maximum auditory loudness (MAL) needs to be determined. Excessively high stimulation should be avoided as this can have a traumatic effect. The aim of this study was to determine if a relationship exists between the intraoperatively determined electrical stapedius reflex threshold (ESRT) and the postoperatively determined MAL and hearing threshold for 27 patients, each having one of three implant types. The question of whether the ESRT provides a practical technique to simplify, improve and accelerate speech processor programming was investigated. For the monopolar stimulation mode used for the Med-El and Clarion implant systems, the average MAL and threshold was expressed as a percentage of the average ESRT across all electrodes. For the "common ground" stimulation mode used for the Nucleus implant system, a parabolic transformation was used to relate MAL and ESRT to one another. These transformations between MAL values calculated from the ESRT and the actual MAL values, determined psychoacoustically, diverged considerably from one another. Therefore, it was not possible to determine the MAL from the ESRT with certainty. The ESRT does, however, provide a means to estimate an approximate upper boundary for the MAL, apart from its use to control implant function. The determination of the exact MAL will still need to be determined using behavioural techniques.

Adolescent↗

Cochlear implantation in patients with MELAS syndrome.

MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) is a rare congenital disorder of mitochondrial DNA (mt-DNA). Patients with this syndrome may present to the otolaryngologist with sensorineural hearing loss (SNHL) that is genetic in origin. Mitochondrial cytopathies can present with a variety of symptoms, but they occasionally present with SNHL as their first manifestation. Two cases of MELAS patients who responded well to cochlear implantation are presented. A review of the literature is also carried out focusing mainly on diagnosis, anesthetic considerations and management of these patients.

Adult↗

Short-term evaluation of over-under myringoplasty technique.

Over-under tympanoplasty is a technique aimed at eliminating the disadvantages of the two classical techniques of overlay and underlay myringoplasty, which are employed in repairing the tympanic membrane. In this retrospective study, a total of 104 patients underwent myringoplasty, which was performed by means of the underlay technique in 46 patients and over-under technique in 58 patients. The mean follow-up period was 11 months. In the first group of patients, the underlay technique was performed; the grafted membrane was placed medial to the remaining drum and the manubrium of the malleus. In the second group, the over-under technique was performed; the grafted membrane was placed under the remaining drum and over the malleus. The rate of success in the first group of 46 patients was 91.5%, and that of atelectasis was 19.5%. The rate of success in the second group of 58 patients was 94.9% and 12%, respectively. Lateralization of the graft was not observed in either of the groups. In the patients operated on by means of the underlay technique, the air-bone gap decreased by 16.55 dB this rate was 16.96 dB in those operated on by the over-under technique. The authors consider over-under tympanoplasty to be superior to the other two classical methods not only because of its effectiveness, but also because of the results achieved by this technique.

Adolescent↗

Cochlear implantation after subtotal petrosectomies.

Among cochlear implant candidates there are patients who have abnormal middle and/or inner-ear conditions that make them unsuitable for implantation. Insertion of a foreign body may also be contraindicated in the setting of an existing or potential intracranial communication, or when the ear is prone to infection. Five patients presented with such unfavorable conditions. These included a Mondini dysplasia with persistent cerebrospinal fluid leak, an atretic mastoid with meningocele, chronic otitis media, a transverse petrous bone fracture, and a temporal bone adenoma. All patients underwent subtotal petrosectomies and cochlear implantations. In four cases implantation was performed concomitantly with subtotal petrosectomy, while the remaining case required a two-stage procedure. No complications occurred. The technique is described in detail, and the cases and the indications for surgery are discussed. By obliterating and isolating the tympanomastoid cleft from the outer environment and utilizing the technique of subtotal petrosectomy, a broader spectrum of patients can now be implanted safely.

Adult↗

Auditory sequence learning: differential sensitivity to task relevant and task irrelevant sequences.

Using a serial reaction time task, this study examines whether learning of auditory sequences is possible without a corresponding motor response, i.e., by listening alone. The dual sequence paradigm used by Mayr (in Journal of the Experimental Psychology: Learning memory and cognition 22:350-354, 1996, Experiment 1) was adapted to the auditory domain. Four different actors spoke the same four colour words. These were presented such that speaker identity followed one sequence, and the word spoken followed a different sequence. Subjects were asked to respond (with a key press) to one of these dimensions (identity or word), and ignore the other. Results showed learning for either type of stimulus, but only when it was responded to. No learning of either type of auditory sequence by listening alone was found. The results add evidence to visual implicit learning studies that have failed to find learning of event sequences when spatial or response selection was not an important factor in processing. The findings are discussed in the context of implicit learning as a general and fundamental cognitive process.

Awareness↗

An evaluation of two signal-processing hearing aids.

Various forms of signal processing are used in modern ASP hearing aids. The present study investigated the relationship between sentence recognition ability and two types of signal processing used in commercially available hearing aids. Results indicated a significant improvement in sentence recognition ability employing an instrument with adaptive compression (variable release time) versus an instrument with an adaptive high-pass filter with short attack and release times. Data were obtained for a single-talker competing message at several message-to-competition ratios. The adaptive compression system may prove to be beneficial to hearing-impaired listeners in certain background noise environments.

Aged↗

Evaluation of a dereverberation technique.

A two-microphone dereverberation technique was evaluated by obtaining speech recognition measures and preference judgments from normal-hearing and hearing-impaired listeners. Monaural speech recognition performance was measured for two reverberation conditions (0.4 second and 1.2 seconds) with and without processing. Binaural speech recognition performance was also measured for the unprocessed conditions. In addition, paired-comparison judgments of preference were obtained for all combinations of the processed and unprocessed monaural stimuli. For both groups of subjects, scores at the shorter reverberation time were significantly higher than scores for the longer reverberation time. For the normal-hearing subjects, processing to dereverberate had no significant effect on speech recognition performance. Binaural presentation of the unprocessed signal yielded significantly higher scores. For the hearing-impaired subjects, performance was significantly better in the unprocessed condition than the processed condition, but was not significantly different from the binaural condition. Paired-comparison judgments revealed differences in patterns of preference between normal-hearing and hearing-impaired listeners.

Adult↗

Continuous discourse tracking: an analysis of the procedure.

The method of continuous discourse tracking was evaluated in terms of the strategies used by the talker and the types of responses elicited from the listener. Talker utterances were classified into four categories: 1. Complete repetition (of the initial utterance); 2. Partial repetition; 3. Repetition with change in emphasis; and 4. A combined strategy using two or more correction strategies. Listener responses were classified into three categories: 1. Correct repetition of intended utterance; 2. Partially correct repetition; and 3. Totally incorrect repetition or no response. The listeners showed small but statistically significant differences in their response patterns. Much larger differences were observed in the pattern of correction strategies used by the talkers. Differences in correction strategy were also observed between the early and later stages of a talker-listener exchange.

Attention↗

Psychological and somatic distress in relation to perceived hearing disability, hearing handicap, and hearing measurements.

Acquired hearing loss has frequently been reported to cause strains in everyday life, but few attempts have been reported where the affliction is related to the concept of stress. In this within-subjects investigation of 48 middle-aged to elderly hearing impaired patients, the relationships between hearing measurements, experienced disability and handicap (HMS), and psychological and somatic distress symptoms (SCL-90(R)) were analyzed. The variations in experienced hearing disability was found to correlate to the occurrence of a few, but more severe distress symptoms, and by loss of hearing for high frequencies. The experienced handicap, defined as emotional responses and personal opinions, was found to correlate to a general level of distress. In particular those distress items expressing insecurity were related to handicap experience. It was suggested that insecurity in social settings, and diminished hearing for contextual sounds, could cause a perceived loss of control, which could induce stress reactions.

Adaptation, Psychological↗

Electrophysiological evidence for early interaction between talker and linguistic information during speech perception.

This study combined behavioral and electrophysiological measurements to investigate interactions during speech perception between native phonemes and talker's voice. In a Garner selective attention task, participants either classified each sound as one of two native vowels ([epsilon] and [ae]), ignoring the talker, or as one of two male talkers, ignoring the vowel. The dimension to be ignored was held constant in baseline tasks and changed randomly across trials in filtering tasks. Irrelevant variation in talker produced as much filtering interference (i.e., poorer performance in filtering relative to baseline) in classifying vowels as vice versa, suggesting that the two dimensions strongly interact. Event-related potentials (ERPs) were recorded to identify the processing origin of the interference: an early disruption in extracting dimension-specific information or a later disruption in selecting appropriate responses. Processing in the filtering task was characterized by a sustained negativity starting 100 ms after stimulus onset and peaking 200 ms later. The early onset of this negativity suggests that interference originates in the cognitive effort required by listeners to extract dimension-specific information, a process that precedes response selection. In agreement with these findings, our results revealed numerous dimension-specific effects, most prominently in the filtering tasks.

Acoustic Stimulation↗

Effects of noise and filtering on the intelligibility of speech produced during simultaneous communication.

UNLABELLED: This study investigated the effects of noise and filtering on the intelligibility of speech produced during simultaneous communication (SC). Four normal hearing, experienced sign language users were recorded under SC and speech alone (SA) conditions speaking Boothroyd's forced-choice phonetic contrast material designed for measurement of speech intelligibility. Twenty-four normal hearing listeners audited the speech samples produced by the four speakers under the SC and SA conditions, three listeners in noise and three listeners in filtered listening conditions for each of the four speakers. Although results indicated longer sentence durations for SC than SA, the data showed no difference in the intelligibility of speech produced during SC versus speech produced during SA under either the noise or filtered listening condition, nor any difference in pattern of phonetic contrast recognition errors between the SA and SC speech samples in either listening condition. This conclusion is consistent with previous research indicating that temporal alterations produced by SC do not produce degradation of temporal or spectral cues to speech intelligibility or disruption of the perception of specific English phoneme segments. LEARNING OUTCOMES: As a result of this activity, the participant will be able to (1) describe simultaneous communication; (2) explain the role of simultaneous communication in communication with children who are deaf; (3) discuss methods of measuring speech intelligibility under filtered and noise conditions; and (4) specify the ability of listeners to perceive speech produced during simultaneous communication under noise and filtered listening conditions.

Adult↗

Effects of cytomegalovirus (CMV) related deafness on pediatric cochlear implant outcomes.

OBJECTIVE: Human cytomegalovirus (CMV) is a commonly recognized viral cause of perinatal sensorineural hearing loss. CMV-infected infants are also at risk for developmental neurological deficits. This retrospective study assesses the impact of CMV-induced deafness on pediatric cochlear implant outcomes. STUDY DESIGN AND SETTING: Thirteen patients from the Johns Hopkins pediatric cochlear implant database were identified with CMV-related deafness. A retrospective review of the medical records of the Johns Hopkins Hospital was performed. RESULTS: The mean age at implantation was 5.6 years. Follow-up audiometric data ranged from 6 to 48 months postoperatively. Mean speech perception scores were 4.5 (out of 6) following implantation. CONCLUSION: We have shown that cochlear implants can provide useful speech comprehension to patients with CMV-related deafness. Speech recognition scores were within the range established by our overall pediatric implant population. SIGNIFICANCE: This observation underscores the importance of a multidisciplinary rehabilitation program following implantation in these patients at risk for cognitive delay.

Antibodies, Viral↗

Intraoperative auditory monitoring in acoustic neuroma surgery.

Intraoperative auditory monitoring is a useful adjunct that is currently evolving. Near-field monitoring techniques such as electrocochleography and direct eighth nerve compound action potential are being used more frequently. The use of these two techniques is compared in 26 patients undergoing hearing preservation acoustic neuroma resection. Overall, 9 (35%) of the 26 patients had their hearing preserved. Three (23%) of the 13 with electrocochleography monitoring and 6 (46%) of the 13 with direct compound action potential monitoring had hearing preserved after surgery. Although there was a suggestion of improved results with direct compound action potential monitoring, the results were not statistically different. It was noted that lack of electrical response at the completion of the procedure (regardless of monitoring technique) was correlated with poor postoperative hearing, whereas the presence of a waveform at termination in no way predicted satisfactory postoperative hearing. The two techniques and their advantages and disadvantages are discussed.

Action Potentials↗

Preoperative hearing predicts postoperative hearing.

Optimal hearing is one goal of otologic surgery. It is generally presumed that hearing thresholds after tympanoplasty-mastoidectomy surgery are determined by remaining anatomy. We assessed the effect of the disease process on hearing after surgery, and our data do not support this presumption. We studied the relationship between preoperative and postoperative hearing across tympanoplasties with differing anatomies in 124 patients without ossicular reconstruction with prostheses or bone. Our results indicate that poor hearing before surgery is associated with poor hearing after surgery, regardless of anatomy (type of tympanoplasty). This relationship holds for both the airbone gap and the speech-reception threshold. When considering ossicular reconstruction, the surgeon should remember that the disease process itself affects hearing in addition to the physical attributes of the ear. These results should be considered in preoperative counseling, as well as in the interpretation of the otologic literature.

Audiometry↗