Improvement in tinnitus distress, health-related quality of life and psychological comorbidities by cochlear implantation in single-sided deaf patients.

BACKGROUND: Patients with single-sided deafness (SSD) lack the ability localize sound sources and have difficulty with speech-in-noise hearing. In addition, there is a high prevalence of tinnitus distress. These problems may result in reduced health-related quality of life (HRQoL) and psychological comorbidities. This study aimed to investigate the influence of treatment with a cochlear implant (CI) on HRQoL, tinnitus distress, psychological comorbidities, and audiological parameters in SSD patients. METHODS: This retrospective study included 20 patients with postlingually acquired SSD (13 women, 7 men, mean age 57.0 years). Data on HRQoL were collected with the Nijmegen Cochlear Implant Questionnaire (NCIQ) and the Medical Outcome Study Short Form 36 Survey (SF-36). Tinnitus distress was assessed with the Tinnitus Questionnaire (TQ), psychological comorbidities were evaluated with validated questionnaires, and speech perception and hearing ability were measured with validated speech tests. RESULTS: Postoperatively, the mean total NCIQ score (p < 0.0001) and four subscores improved significantly, as did tinnitus distress (p < 0.05). Anxiety symptoms (Generalized Anxiety Disorder questionnaire, GAD-7) decreased significantly after CI. Speech perception improved significantly, particularly "hearing with background noise" (p < 0.05, Oldenburg Inventory, OI) and "localization" (p < 0.001, OI), as well as the Oldenburg Sentence Test (OlSa) scores for with vs. without CI when speech was presented from the SSD side and noise was presented from the normal hearing side (SSSDNNH; p < 0.005). CONCLUSION: CI in SSD patients is a powerful procedure to improve HRQoL, reduce tinnitus distress, and improve psychological comorbidities. Additionally, it is beneficial for hearing improvement, particularly in noise and for directional hearing.

[Improvement in tinnitus distress, health-related quality of life and psychological comorbidities by cochlear implantation in single-sided deaf patients. German version]. / Verbesserung von Tinnitusdistress, Lebensqualität und psychologischen Komorbiditäten durch Cochleaimplantation einseitig ertaubter Patienten.

BACKGROUND: Patients with single-sided deafness (SSD) lack the ability localize sound sources and have difficulty with speech-in-noise hearing. In addition, there is a high prevalence of tinnitus distress. These problems may result in reduced health-related quality of life (HRQoL) and psychological comorbidities. This study aimed to investigate the influence of treatment with a cochlear implant (CI) on HRQoL, tinnitus distress, psychological comorbidities, and audiological parameters in SSD patients. METHODS: This retrospective study included 20 patients with postlingually acquired SSD (13 women, 7 men, mean age 57.0 years). Data on HRQoL were collected with the Nijmegen Cochlear Implant Questionnaire (NCIQ) and the Medical Outcome Study Short Form 36 Survey (SF-36). Tinnitus distress was assessed with the Tinnitus Questionnaire (TQ), psychological comorbidities were evaluated with validated questionnaires, and speech perception and hearing ability were measured with validated speech tests. RESULTS: Postoperatively, the mean total NCIQ score (p < 0.0001) and four subscores improved significantly, as did tinnitus distress (p < 0.05). Anxiety symptoms (Generalized Anxiety Disorder questionnaire, GAD-7) decreased significantly after CI. Speech perception improved significantly, particularly "hearing with background noise" (p < 0.05, Oldenburg Inventory, OI) and "localization" (p < 0.001, OI), as well as the Oldenburg Sentence Test (OlSa) scores for with vs. without CI when speech was presented from the SSD side and noise was presented from the normal hearing side (SSSDNNH; p < 0.005). CONCLUSION: CI in SSD patients is a powerful procedure to improve HRQoL, reduce tinnitus distress, and improve psychological comorbidities. Additionally, it is beneficial for hearing improvement, particularly in noise and for directional hearing.

Validity of cochlear microphonics at high sound pressure levels as an important clinical aspect.

INTRODUCTION: Cochlear microphonics are electrical stimulus responses of the inner ear, generated by mechanical displacement of the hair cells caused by acoustic stimulation. As cochlear microphonics are often used in the diagnosis of hearing impairment and deafness, in preliminary investigations it was seen that obliteration or ossification have no effect on the extent to which cochlear microphonics can be recorded at high sound pressure levels. As artifacts at high sound pressure levels suggested, measurements were subsequently conducted using temporal bone specimens. METHODS: In a test setup equivalent to that for electrocochleography, a needle electrode was placed on the cochlear promontory and used to record potentials following application of an acoustic stimulus. RESULTS: Curves comparable to cochlear microphonics were registrable down to a threshold of 80 dB HL. Additional measurements conducted on damp cloths yielded comparable findings. CONCLUSIONS: Registration of cochlear microphonics at high sound pressure levels does not serve as an indicator of hair cell function, but should instead be regarded as artifacts. The possible sources are discussed.

The influence of post-meningitic obliteration and ossification of the cochlea on cochlear microphonics.

Cochlear microphonics are electrical stimulus responses of the inner ear. They are generated by mechanical displacement of the hair cells caused by acoustic stimulation and can be recorded from the cochlear promontory via a needle electrode. In individuals with post-meningitic deafness, fibrous obliteration or ossification of the cochlea may occur. The aim of the present investigation was to establish whether obliteration or ossification leads to any alteration in the extent to which cochlear microphonics can be recorded (as compared with that in patients with a fluid-filled cochlea whose deafness was not post-meningitic), as an indication of changes in the mechanical displacement of the remaining hair cells. Cochlear microphonics in 15 individuals with proven obliteration or ossification of the cochlea were compared with those in 15 control subjects (deaf individuals whose hearing loss was not post-meningitic, and who had a fluid-filled cochlea). Our findings reveal no statistically significant differences between the two groups in the degree to which cochlear microphonics can be recorded. This study does not demonstrate evidence of a change in this property due to ossification or obliteration of the cochlea.

Impact of low-frequency hearing.

Today, cochlear implantation is the treatment of choice in the case of severe to profound hearing loss, but the speech perception abilities of many recipients in noisy conditions are still poor and the overall sound quality and ease of listening still require improvement. Residual low-frequency hearing has been associated with improved hearing performance in cochlear implant patients, especially in difficult listening environments (i.e. cocktail party effect). It seems that low-frequency information can enhance the segregation of competing voices, which leads to better speech understanding in noise. We investigated the effect of low-frequency hearing on speech perception performance in a group of subjects being implanted with the Nucleus Hybrid-L device. The Hybrid-L device is a modified Nucleus Freedom implant, which instead of the standard electrode carries a very delicate electrode array that allows preservation of residual hearing to a great extent. Sentence test results from 22 subjects are presented here. Additionally, for 8 subjects, the acoustically presented frequency range was limited to 300, 500 and 700 Hz, and speech perception tests with a single competing talker were conducted. The Hybrid-L study group achieved a speech reception threshold of 15.9 dB in the hearing aid alone condition, 10.8 dB in the cochlear implant alone condition, and 3.9 dB when using the combination of cochlear implant and hearing aid. Differences between the 3 conditions are statistically significant. Results from the additional experiment on the acoustically presented frequency range suggest that very limited residual hearing below 500 Hz is already sufficient to produce a significant improvement in speech perception performance in conjunction with a cochlear implant.

Experimental middle ear surgery in rabbits: a new approach for reconstructing the ossicular chain.

This experimental animal study aimed at evaluating a new prosthesis to replace the ossicular chain; we developed a new technique for surgical implantation into the middle ear of rabbits. The rabbit middle ear is, owing to the relative anatomical dimensions involved, an ideal environment for implantation procedures involving the ossicles, as the surgical conditions are similar to those of the human middle ear. This study included a total of 34 approximately six-month-old female white rabbits (New Zealand) weighing between 3.2 and 4.4 kg. The implants used were constructed of ceramic materials (titania, TiO(2)) of various pore sizes. Directly prior to implanting the total ossicular reconstruction prostheses (TORPs), as well as at 28, 84 and 300 days after implantation, electric response audiometry was used to determine the hearing thresholds of the animals (bone conduction; click stimulus nHL). An erbium:YAG laser was used to excise the original ossicular chain. Following implantation, we were unable to detect any stenosis of the outer ear canal or perforation of the tympanic membrane. The conductive hearing threshold was in the range of 4.21 +/- 6.68 dB nHL (n = 131). The hearing level showed no significant difference before and after surgery (P < 0.05).

Effects of phase duration and pulse rate on loudness and pitch percepts in the first auditory midbrain implant patients: Comparison to cochlear implant and auditory brainstem implant results.

The auditory midbrain implant (AMI), which is designed for stimulation of the inferior colliculus (IC), is now in clinical trials. The AMI consists of a single shank array (20 contacts) and uses a stimulation strategy originally designed for cochlear implants since it is already approved for human use and we do not yet know how to optimally activate the auditory midbrain. The goal of this study was to investigate the effects of different pulse rates and phase durations on loudness and pitch percepts because these parameters are required to implement the AMI stimulation strategy. Although each patient was implanted into a different region (i.e. lateral lemniscus, central nucleus of IC, dorsal cortex of IC), they generally exhibited similar threshold versus phase duration, threshold versus pulse rate, and pitch versus pulse rate curves. In particular, stimulation with 100 mus/phase, 250 pulse per second (pps) pulse trains achieved an optimal balance among safety, energy, and current threshold requirements while avoiding rate pitch effects. However, we observed large differences across patients in loudness adaptation to continuous pulse stimulation over long time scales. One patient (implanted in dorsal cortex of IC) even experienced complete loudness decay and elevation of thresholds with daily stimulation. Comparing these results with those of cochlear implant and auditory brainstem implant patients, it appears that stimulation of higher order neurons exhibits less and even no loudness summation for higher rate stimuli and greater current leakage for longer phase durations than that of cochlear neurons. The fact that all midbrain regions we stimulated, which includes three distinctly different nuclei, exhibited similar loudness summation effects (i.e. none for pulse rates above 250 pps) suggests a possible shift in some coding properties that is affected more by which stage along the auditory pathway rather than the types of neurons are being stimulated. However, loudness adaptation occurs at multiple stages from the cochlea up to the midbrain.

Performance and preference for ACE stimulation rates obtained with nucleus RP 8 and freedom system.

Cochlear recently released the Nucleus Freedom System which has been based on the Nucleus Research Platform 8. Both systems make use of the same implant, the CI24RE, which includes expanded total stimulation rates up to 32 kHz. In this study the performance of the ACE strategy at 500, 1200 and 3500 pps/channel was investigated using an ABC-CBA design. At the end of each period speech tests were performed. In the CBA phase the patients completed a comparative questionnaire to determine the subjective rate preference. Preliminary results in 13 recipients indicate no differences in for the ACE strategy at rates ranging from 500 pps to 3500 pps/channel.

The results in patients implanted with the nucleus double array cochlear implant: pitch discrimination and auditory performance.

OBJECTIVE: In patients with total or surgically inaccessible cochlear obliteration, only a reduced number of active electrodes can be inserted with standard cochlear implants, resulting in below average auditory performance. Therefore, a special implant with two electrode arrays was developed on the basis of the Nucleus 22 cochlear implant, the socalled Double Array. One electrode array with 11 active electrodes is inserted into the basal turn of the cochlea, while the second array with 10 active electrodes is inserted into the second turn. The Double Array is now available on the basis of the more advanced Nucleus 24 with 11 active electrodes on each array and two reference electrodes, one at the case and the second one an additional ball electrode, which is placed under the temporalis muscle. For device description and surgical technique see Lenarz et al. (2001). This paper presents psychophysical data on pitch discrimination and auditory performance of patients implanted with a Double Array on the basis of the Nucleus 22. STUDY DESIGN: A prospective intra-individual study using a Latin square paradigm was performed in six adult patients with obliterated cochlea who received the Nucleus 22 Double Array. After appropriate fitting and loudness balancing, patients were tested either with the basal, the apical or both electrode arrays. Apart from auditory performance tests including numbers and monosyllable word tests, pitch discrimination was determined with a defined procedure. RESULTS: When activating each array alone, auditory performance was better with the basal array than with the apical array. Both arrays together showed marked improvement compared with the basal array, indicating an additional effect of the second array. Pitch discrimination was significantly better for the electrodes in the basal turn than in the second turn, indicating differences in electrical excitation of the auditory nerve fibers. Pitch discrimination was positively correlated with auditory performance data. CONCLUSION: The additional apical array leads to significant improvement in auditory performance in patients with obliterated cochleae by increasing the number of intracochlear electrodes. Despite reduced pitch discrimination, the apical array provides important information for speech recognition. For this reason the Double Array provides a profound advantage for patients with obliterated or surgically inaccessible cochleae.

Auditory brainstem implant: part I. Auditory performance and its evolution over time.

OBJECTIVE: Evaluation of auditory performance and its evolution over time in patients with the auditory brainstem implant. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS AND METHODS: Between May 1996 and April 2000, 14 patients with neurofibromatosis type 2 underwent implantation with a multichannel auditory brainstem implant. Auditory performance data were obtained in 13 patients who had used their device on a regular daily basis for 1 to 41 months (average 19 months). Hearing evaluation was based on the results of four tests (vowel confusion, consonant confusion, Freiburger numbers, and speech-tracking test), which were performed with and without lip-reading at regular intervals after device activation. RESULTS: 12 patients received auditory sensation through the auditory brainstem implant immediately after device activation. In one patient, because of postoperative electrode migration, device activation was not successful. In this case, after the electrode array was repositioned, activation was successful. The results of the audiovisual mode 2 weeks after device activation revealed a lip-reading enhancement above the chance level in about 50% of the patients in the vowel confusion and speech-tracking tests and in 70% of the patients in the consonant confusion test. Lip-reading enhancement improved within the first 6 months and then entered a plateau phase, which was more prominent in the monosyllabic vowel and consonant tests. In the auditory alone mode, more than half of the patients showed their first positive result in the vowel test 3 months after device activation, but it took about 6 months until half of the patients revealed a result above the chance level in the consonant and Freiburger numbers tests. Open set speech recognition in the auditory alone mode (in the speech-tracking test) was not common and happened relatively late (within 1 year or later). DISCUSSION AND CONCLUSION: Although auditory sensation appeared immediately after device activation, a period of 6 months was necessary for relearning and adaptation of the central auditory system to the altered form of auditory information presented by the auditory brainstem implant.

[Subjective deafness in case of peri-synaptic audiopathy. Isolated defects of the inner haircells?]. / Funktionelle Taubheit bei peri-synaptischer Audiopathie - Isolierte Störungen der inneren Haarzellen?

BACKGROUND: Damage to or functional impair of the inner hair cells, synapsis or dendrites of the ganglion cells of the auditory nerve result in specific audiometric findings. Due to the normal function of the outer hair cells otoacoustic emissions can be registered, ABR and ECochG show at least elevated thresholds or are absent. PATIENTS: We demonstrate 5 cases with these audiological findings described in the literature as Auditory Neuropathy. RESULTS: All patients have profound to severe hearing loss with poor speech understanding under best aided conditions with conventional hearing aids. 3 patients, which were implanted with a cochlear implant have speech understanding but one prelingually adult, also implanted, has only sound identification. CONCLUSIONS: Hypoxia, carboplatin, ototoxicity and metabolic disorders are possible etiologies for damage to the inner hair cells or synapsis. The results will be discussed with reference to the localisation of the pathology and the definition as Auditory Neuropathy.

The nucleus double array cochlear implant: a new concept for the obliterated cochlea.

OBJECTIVE: To increase the number of intracochlear electrodes that may be inserted into a totally obliterated cochlea, a special implant has been developed in collaboration with Cochlear Limited. This implant features two separate electrode carriers containing 11 and 10 active electrodes, respectively, as well as a reference electrode located on the receiver-stimulator package. The potential stimulation modes available with this device therefore include monopolar and bipolar stimulation, and stimulation between both arrays. SURGICAL TECHNIQUE: A cochleostomy anterior to the round window provides access to the basal turn (both the scala tympani and the scala vestibuli), and new built connective tissue and bone can be removed until the anterior wall of the basal turn is approached. A second cochleostomy is performed at the second turn caudal of the cochleariform process and 2 mm anterior of the oval window after removal of the incus. New tissue should also be removed if necessary. The two electrode carriers are then placed into the scala tympani of the basal and the scala vestibuli of the second turn, respectively. The remaining surgical procedure is identical with that used for cochlear implantation in patients without obliterated cochleas. PATIENTS: In this clinical study, 10 patients aged 32 to 66 years with an obliterated cochlea each received a double array cochlear implant. All patients had total obliteration of the basal turn either on preoperative imaging or during surgery. Intraoperatively, the second turn was not obliterated in only 4 of 10 patients. Postoperatively, a standard audiologic test battery was used to determine auditory improvement over time. POSTOPERATIVE RESULTS: All patients achieved significantly improved speech understanding when the additional apical electrode array was used, compared with the use of each electrode array independently. No complications occurred. CONCLUSION: In patients with a totally obliterated cochlea, the number of intracochlear electrodes can be increased by use of the Nucleus double array implant. As a result, patients achieve significantly better auditory results.

Extensive monitoring during auditory brainstem implant surgery.

In patients with reduced auditory nerve function, for example due to tumour removal or an accident, hearing rehabilitation can be elicited by an auditory brainstem implant (ABI). The electrode array of the ABI manufactured by Cochlear Ltd., Sydney, consists of 21 circled contacts in a silicon carrier. This is inserted in the lateral recess of the fourth ventricle. Since 1996, in Hannover eight patients have been implanted with a cochlear ABI Nucleus 21 + 1. All of them were profoundly deaf on both sides due to neurofibromatosis type 2 (NF2). To find the optimal electrode position during surgery, a multimodal monitoring by auditory evoked potentials (AEP), electromyography (EMG) and somatosensory evoked potentials (SEP) was performed. When monitoring AEPs, the function of the implant can be checked first by the stimulus artefact. By analysing the AEPs in more detail, the optimal positioning of the electrode on the cochlear nucleus can be found. If systems other than the auditory system are stimulated this will be revealed in one or more of the AEP, EMG and SEP recordings. According to the literature, AEPs stimulated by an ABI consist of three vertex positive peaks with latencies shorter than 4 ms. Typical AEPs are correlated with good post-operative hearing sensation. Comparing these AEPs with AEPs stimulated acoustically or electrically at different sites of the auditory system, it can be assumed that the first peak corresponds to J3, the second to J4 and the last to J5. From this comparison it can also be concluded that no potentials should occur later than 5 ms. This corresponds to our findings. Post-operatively, side-effects occurred when areas of the electrode array were stimulated that showed potentials with latencies longer than 5 ms intra-operatively. Our results indicate that monitoring is an essential aid for the surgeon in finding the optimal electrode position. Positioning solely with reference to anatomical landmarks may not be enough to find the optimal functional position.

Auditory brainstem implant in auditory rehabilitation of patients with neurofibromatosis type 2: Hannover programme.

An auditory brainstem implant (ABI) is indicated for patients suffering from bilateral neural deafness. The most affected patients are those with neurofibromatosis type 2 (NF2). An implantation is possible either at the same time as, or after, surgical removal of an acoustic neuroma. This paper demonstrates the results of eight out of 11 patients with NF2, seven of whom received an ABI after tumour removal. Pre-operatively, all of them were deaf. Post-operatively, the first fitting served to determine the individual stimulation parameters for each electrode. The stimulation-dependent side-effects were eliminated by reducing the stimulus intensity without causing negative effects on the hearing with the ABI. Only in one case was an open set understanding achieved within the first year. However, all patients had a better speech understanding when they combined their hearing with the ABI and their lip-reading abilities. There is no correlation between the performance with ABI and the tumour size or the duration of deafness.