Cochlear implantation in patients with ossified cochleas.

PURPOSE: The timing of CI for postmeningitic deafness is controversial and differential outcomes have been reported. To review and share our surgical and auditory outcomes. MATERIALS AND METHODS: 17 patients with ossified cochleas who received CI were enrolled. Clinical data including the cause of cochlear ossification, preoperative examination, onset of deafness, age at implantation, surgical findings, and relevant auditory outcomes was analysed. RESULTS: Cochlear ossification was observed in 53% of patients with HRCT, whereas the corresponding value for MRI was 59%. Patients in both stage I and II received complete insertion of the electrode array, however, stage III patients only received partial insertion. 1 patient in stage II received bilateral CI. Hearing tests showed increased average hearing threshold for stage III patients than those in stage I and II (P < 0.05). CAP scores were much lower for stage III patients than those in stage I and II (P < 0.05). Postlingual deafness patients showed higher SIR scores than prelingual deafness children (P < 0.05). CONCLUSIONS: HRCT and MRI have comparable value in predicting the occurrence of ossification in cochleas. We recommend fast surgical intervention in the patients with bilateral profound postmeningitic deafness. If possible, bilateral cochlear implantation is recommended.

Rare case of bilateral aural atresia and cochlear dysplasia: when cochlear implantation is not the answer.

OBJECTIVE AND IMPORTANCE: Reports of patients with concurrent middle and inner ear anomalies are rare. These patients present a surgical challenge for cochlear implantation. The surgical risk must be weighed against the predicted benefit of the patient's hearing outcome and subsequent development of speech and language as well as their quality of life. CLINICAL PRESENTATION: Thirteen-year-old boy presented to the Otology clinic for auditory rehabilitation options. He has mild developmental delay, is non-verbal and communicates via American Sign Language. He was born with bilateral aural atresia and never wore amplification. On exam he has grade 1 microtia and complete ear canal atresia bilaterally. His behavioural hearing test shows profound sensorineural hearing loss of both ears. The computed tomography scan shows bilateral underdeveloped and completely opacified mastoid and middle ear, complete bony atresia of the ear canals, and an under-partitioned cochlea with poorly defined modiolus, among other abnormalities. The patient and his family were counselled on the available options as well as the need for any further studies. INTERVENTION: Counselling of patient and family. CONCLUSION: While there have been reports in the literature of performing cochlear implantations in patients with a concurrent atresia and cochlear dysplasia, these were patients whose degree of inner ear anomalies was relatively minor and their prognosis of a good audiological outcome was favourable. The presented case is that of a patient for whom the surgical approach to the cochlea alone would be difficult. More importantly, his quality of life would not significantly improve in light of the predicted limited hearing and language development outcomes, given the severity of his inner ear abnormalities, limited communication abilities, prolonged period of deafness and developmental delays.

Speech perception performance of double array multichannel cochlear implant users with standard and duplicated maps in each of the arrays.

OBJECTIVE: The present investigation evaluated the speech perception performance of patients with ossified cochlea implanted with the 24M Double Array cochlear implant, using standard and duplicated maps in each of the arrays. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Sixteen subjects received a Double Array cochlear implant. Among these, 9 fulfilled the following inclusion criteria: bilateral severe-to-profound postlingual deafness; bilateral obliterated cochlea, as shown by a computed tomographic scan; and a minimum age of 14 years to ensure reliable responses in the behavioral tests with the 3 tested maps. INTERVENTION: Rehabilitative. MAIN OUTCOME MEASURES: The speech perception performance with the 2 arrays was compared with that with a basal array duplicated map and an apical array duplicated map. Three maps were fitted: the default map with both arrays activated, a double channel map using only the electrodes of the basal array, and a double channel map programmed only with the electrodes of the apical array. The test battery was composed of a vowel test, a 4-choice word test, and sentence recognition in quiet. RESULTS: Statistical significance was reached in comparison the all tests in all programming conditions. Speech recognition in the standard map with both electrode arrays activated showed the highest scores. CONCLUSION: Performance with the 2 split electrode arrays was superior to those with the single arrays, regardless of the duplication of channels.

[The estimation of the efficacy of manual therapy included in the combined treatment of cochlear-vestibular disorders based on the results of computed stabilography].

The objective of the present study was to estimate the efficacy of the combined treatment of spondylogenic cochlear-vestibular disorders with the use of both medicamental and non-medicamental modalities. Computed static stabilometry was applied for diagnostics of postural disbalance and evaluation of the treatment outcomes. It was shown that the application of manual therapy for the management of 56 patients presenting with spondylogenic cochlear-vestibular disorders resulted in the decrease of tinnitus and the improvement of vestibular and cochlear functions.

Traumatic perilymphatic fistula with pneumolabyrinth: diagnosis and management.

A perilymphatic fistula (PLF) is an abnormal communication between the inner and middle ear resulting in vestibular or cochlear symptoms. We review three pediatric traumatic temporal bone fractures with pneumolabyrinth, confirmed radiologically by the presence of air within the cochlea (pneumocochlea) or vestibule (pneumovestibule). Patients were treated conservatively with complete resolution of vestibulopathy. Hearing outcome was variable and worse in two patients with pneumocochlea. A pneumolabyrinth on radiologic imaging confirms a PLF and obviates the need for exploration to reach a diagnosis. We suggest exploration be reserved for patients with persisting cerebrospinal fluid leakage, progressive sensorineural hearing loss, or vestibular symptomatology.

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. STUDY DESIGN: Case presentation. This study was conducted at Gruppo Otologico, Rome, Italy, a private referral center for neurotology and skull base surgery. METHODS: The subject of our study was a 12-year-old female child with postmeningitic deafness and bilaterally ossified cochleae. This case is the first brainstem implantation performed at our center with the indication of severe ossification of the cochlea. RESULTS: Successful brainstem implantation of a device was carried out, and the hearing of the patient was restored to the degree that she can freely use the telephone after 8 months of implantation. CONCLUSION: Although more cases are needed before establishing the exact outcome of brainstem implantation in cases of deafness in the presence of severe bilateral cochlear ossification, preliminary results show the superiority of brainstem implants to conventional or even customized cochlear implants.

[Obliterated cochlea in Cogan's syndrome -- implications for cochlear implant surgery]. / Obliteration der Cochlea beim Cogan-Syndrom -- Bedeutung für die Cochlear-Implant-Chirurgie.

BACKGROUND: Acute, often bilateral deafness in Cogan's syndrome or other autoimmune diseases is caused by autoimmune mediated inflammatory attack on the membranous labyrinth. Auditory rehabilitation in case of bilateral deafness can be achieved by cochlear implant surgery. METHODS: A retrospective analysis of all patients suffering from Cogan's syndrome that had received a cochlear implant, was carried out. RESULTS: 6 of 295 adult patients (2.6 %) that had received a cochlear implant, had become deaf due to Cogan's syndrome. Partial obliteration or ossifikation was encountered in all cases and influenced surgical procedure. In one case a fibrous obliteration of the scala tympani was found 8 weeks after acute onset of complete deafness. CONCLUSIONS: The course of obliteration is unknown. With regard to our results a fibrous obliteration may occur as early as 8 weeks after complete deafness. This has to be considered in counseling of patients. Only early cochlear implant surgery facilitates best possible rehabilitation results.

Auditory brainstem implant in bilateral and completely ossified cochleae.

OBJECTIVE: To report and evaluate the results of auditory brainstem implantation in a case of postmeningitis hearing loss with totally ossified cochleae on both sides. STUDY DESIGN: Case report. SETTING: Tertiary referral center. PATIENT: A 49-year-old man was referred to the authors' department for bilateral total hearing loss subsequent to bacterial meningitis 2 years earlier. Pure tone audiometry, auditory brainstem response tests, and promontory tests did not reveal any cochlear activity. Computed tomography showed bilateral and totally ossified cochleae. Magnetic resonance imaging confirmed the diagnosis by showing no cochlear signal on T2-weighed images and ruled out brainstem and cerebellopontine angle abnormalities. INTERVENTION: A left auditory brainstem implantation was performed through a translabyrinthine route, using a Nucleus 22-channel device. MAIN OUTCOME MEASURES: Word and sentence recognition tests in sound-only and sound plus vision modes. RESULTS: No postoperative complication was observed. Twelve electrodes could be activated, and their tonotopy was defined. Nine other electrodes were inactivated because of an absence of auditory response (4 electrodes) or paresthesia (5 electrodes). At the last follow-up visit, 26 months after the implantation, 50% of disyllabic word scores and 60% of sentence scores were achieved using auditory brainstem implant sound only. These scores reached 80% and 93%, respectively, with lip-reading. CONCLUSION: Auditory brainstem implantation is an efficient means of auditory rehabilitation in cases of bilateral total hearing loss with totally ossified cochleae. It should be considered in cases of predictable failure in cochlear implantation.

[Evaluation of hearing performance in cochlear implant patients]. / Koklear implantasyonlu hastalarda isitme performanslarinin karsilastirilmasi.

OBJECTIVES: To determine the speech perception levels of cochlear implant patients and to evaluate the possible effects of implant type, duration of deafness, and bacterial meningitis on the patients' hearing performance. PATIENTS AND METHODS: A three-section test battery on speech perception was administered to 21 cochlear implant patients (12 males, 9 females; mean age 25 years; range 9 to 40 years). The patients' performance was assessed with respect to single- or multi-channel implant design, duration of hearing loss, and etiologic factors of hearing loss. RESULTS: The overall mean success score was 74.6 (range 49-100). No significant difference was found between patients using single- or multi-channel implants. The mean score of 12 patients whose duration of hearing loss was below the average (8.2 years) was significantly higher than those of nine patients with a longer history of hearing loss (85.7 versus 59.6, p=0.001). Patients with meningitis-associated hearing loss exhibited a significantly lower performance than those presenting with other etiologic factors (61.9 versus 84.1, p=0.001). CONCLUSION: Early diagnosis and rehabilitation are essential to achieve better performance following cochlear implantation.

The Vienna Cochlear Implant in patients with obliteration of the cochlea.

Twelve deaf patients with obliterated or ossified cochleas received the extracochlear version of the Vienna Cochlear Implant. Four patients, 1 of them a child, developed open speech comprehension. Obliteration of the cochlea could not always be predicted by conventional tomography of the temporal bone. Short duration of deafness, wide dynamic range, and good ability of time resolution (small temporal difference limen [TDL]) are predictors for good postoperative results. Obliteration or ossification of the cochlea per se is no contraindication to cochlear implantation.

Radiologic evaluation of multichannel intracochlear implant insertion depth.

Postoperative plain film x-rays are necessary in all multichannel cochlear implant patients to confirm intracochlear position, detect possible electrode kinking, and provide a reference if postoperative slippage occurs. In addition, precise documentation of multichannel intracochlear electrode insertion depths is necessary for comparison of speech recognition results among patients and may be of use for future speech processing strategies. In the present study, a method has been devised, using a modified Stenver's view, to more accurately document insertion depths of the electrode array and location of individual electrodes on 50 multichannel cochlear implant patients. Surgical estimates of insertion depth are shown to have great variability in regard to distance along the basilar membrane when compared with x-ray documentation. Additionally, there is preliminary evidence that insertion depth, as determined by x-ray studies, has a strong correlation with open-set speech discrimination.

The perception of temporal modulations by cochlear implant patients.

The perception of temporal modulations of pulsatile electric stimuli was measured in seven cochlear implant patients using the Cochlear Pty. Limited prosthesis. Four patients were postlingually deafened adults and three patients were young adults who were deafened very early in life. The first study measured detection thresholds for modulated pulse duration for a series of modulation frequencies and pulse rates. The shape of the detection thresholds as a function of modulation frequency, the temporal modulation transfer function, often resembled a low-pass filter with a 50-100-Hz cut-off frequency. Thresholds did not markedly vary across the different pulse rates for most patients. Thresholds were less than 10%-20% of the range of usable hearing for most patients. The second study compared detection thresholds for modulated pulse durations around different reference pulse durations: 50, 100, and 300 microseconds. Detection thresholds were generally proportional to the different reference pulse durations. The third study measured difference limens for the discrimination of modulation depth. The difference limens were similar to the detection thresholds for the same reference pulse duration and pulse rate. The three patients deafened very early in life showed more within-group variation in performance, and their overall levels of performance were poorer than those of the postlingually deafened adults.

Importance of patient and processor variables in determining outcomes with cochlear implants.

Within-subjects comparisons of processing strategies for cochlear implants are reviewed. Compressed analog strategies were compared to interleaved pulses strategies in tests with one group of 8 subjects, and to continuous interleaved sampling strategies in tests with another group of 11 subjects. The tests included open-set recognition of words and sentences. The results show that, while different strategies may produce quite different outcomes across subjects, individual performances with one processing strategy are significantly correlated with those of alternative strategies. These findings emphasize the importance of patient variables in determining outcomes across a variety of prosthesis designs.

The bionic ear and the mythology of paediatric implants.

Parents face a daunting task when deciding whether an implant is an optimal, or even a viable choice for their deaf child. Much of the confusion is as a result of vested interest by both proponents and critics of cochlear implantation.

[Indications and results of cochlear implants in young children]. / Indications et résultats de l'implant cochléaire chez le jeune enfant.

In order to determine the criteria for patient selection and the preoperative prognostic factors for hearing recovery after cochlear implants in young totally dead children, the results of hearing rehabilitation were studied in 15 children who had undergone cochlear implantation at from 2 to 9 years of age. The choice of implant is determined by the permeability of the cochlear duct. A multi-system should be used, except when the cochlea is totally ossified. The reliability and efficacy of mono and multi system implants make it possible to offer a solution to the urgent therapeutic problems posed by total deafness in the young child.