Cases reported "cochlear diseases"

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11/57. scala vestibuli cochlear implantation in patients with partially ossified cochleas.

    Partial cochlear obstruction is a relatively common finding in candidates for cochlear implants and frequently involves the inferior segment of the scala tympani in the basal turn of the cochlea. In such patients, the scala vestibuli is often patent and offers an alternative site for implantation. The current report describes two patients with such partial obstruction of the inferior segment of the basal cochlear turn, caused in one case by systemic vasculitis (Takayasu's disease) and in the other by obliterative otosclerosis. A scala vestibuli implantation allowed for complete insertion of the electrode array. No problems were encountered during the surgical procedures and the good post-operative hearing and communicative outcomes achieved were similar to those reported in patients without cochlear ossification. The importance of accurate pre-operative radiological study of the inner ear is underscored, to disclose the presence and define the features of the cochlear ossification and ultimately to properly plan the surgical approach. ( info)

12/57. 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. ( info)

13/57. Canavan's leukodystrophy is associated with defects in cochlear neurodevelopment and deafness.

    The authors present the temporal bone histopathology of two siblings (4 months old and 6 months old at autopsy) with Canavan's disease, an autosomal recessive leukodystrophy that is variably associated with sensorineural hearing loss. The histopathology demonstrated bilateral absence of the organ of corti throughout the apical and basal cochlea and mild secondary atrophy of the spiral ganglia neurons. The vestibular end organs and ganglia were normal. These findings implicate a role of aminoacylase II in the neurodevelopment of the organ of corti. ( info)

14/57. Binaural cochlear implants.

    With the success of monaural cochlear implantation, patients frequently ask about having a second implant. We have performed binaural cochlear implants in 12 adult patients. Desire not to disrupt a functioning implant was the primary consideration in implanting the contralateral ear. Seven patients received a second 3M/House single-channel implant to upgrade to a magnetic external receiver. Four patients with a 3M/House device in one ear elected to place a Nucleus multichannel implant in the opposite ear. One patient with a poorly functioning Nucleus device elected to have a second Nucleus device. Four patients with a Nucleus and a 3M/House implant, one with binaural 3M/House implants, and one with binaural Nucleus implants were tested for auditory discrimination in order to quantify monaural versus binaural differences. The functional benefit of the second implant was mixed, but all patients showed some degree of objective improvement on one or more tests. Five of the six are regular users of both devices; the patient with binaural Nucleus implants wears only one. Despite the differing processing schemes, patients with a Nucleus device in one ear and a 3M/House device in the other ear are able to combine the two signals to advantage. We feel that cochlear implantation in the contralateral ear is an acceptable option in selected patients desiring an upgraded implant without placing a functioning implant at risk. ( info)

15/57. A new autosomal dominant syndrome of idiopathic progressive vestibulo-cochlear dysfunction with middle-age onset.

    A kindred is described with progressive autosomal dominant vestibulo-cochlear dysfunction resulting in instability in the dark, head movement dependent oscillopsia and hearing loss. The first symptoms appeared in the 4th decade and progressed to vestibular areflexia, presumably in the 5th decade and to almost total deafness in the 6th-7th decade of life. The history was negative for other neurological, otological or infectious diseases, or the use of neuro-ototoxic drugs. The affected subjects showed remarkable compensation for the loss of vestibular function. ( info)

16/57. Cochlear implant failure: is an auditory brainstem implant the answer?

    OBJECTIVE: To investigate the auditory rehabilitative results achieved in five patients with cochlear implants (CIs) who subsequently received, due to poor results, auditory brainstem implants (ABIs). MATERIAL AND methods: Between April 1997 and March 2003, 37 patients (age range 14 months to 70 years) were fitted with ABIs in our ENT Department. Fourteen subjects had neurofibromatosis type 2 and 23 were non-tumor patients who had cochlea or cochlear nerve disease. Five subjects had previously been treated with a CI and received an ABI owing to the poor results achieved. One child had bilateral undiagnosed cochlear nerve aplasia and one was suffering from auditory neuropathy; three adults had total cochlear ossification. RESULTS: The open-set sentence recognition score (auditory-only mode) 6-8 months after ABI activation ranged from 0% to 100% in adults. In 1 subject the speech-tracking score was 56 words/min with the ABI. The two children who had achieved no hearing ability with their CI were able to detect sounds and words as early as 3 months after activation of the ABI. CONCLUSION: CI failure as a result of anatomical abnormalities can be remedied by an ABI. ( info)

17/57. Recurrent acute otitis media associated meningitis in a patient with a contralateral cochlear implant and bilateral cochleovestibular dysplasia.

    OBJECTIVE: To illustrate that a patient with a cochlear implant may be at increased risk of meningitis secondary to developmental anatomic abnormality associated with the underlying sensorineural hearing loss, as opposed to the implant itself. methods: Case report. RESULTS: Our 12-year-old patient has bilateral cochlear dysplasia, profound sensorineural hearing loss and no prior history of recurrent acute otitis media or meningitis. He underwent a left cochlear implant at 8 years of age and subsequently experienced three episodes of right acute otitis media and meningitis over the next 4 years. Middle ear exploration revealed a cerebrospinal fluid leak. A right radical mastoidectomy with closure of the external auditory canal, removal of the tympanic membrane, malleus, and incus, closure of the eustachian tube, and obliteration of the mastoid and middle ear with abdominal fat has prevented further episodes. CONCLUSION: meningitis in a patient with a cochlear implant is not necessarily related to the implant. ( info)

18/57. temporal bone histopathology in dominantly inherited audiovestibular syndrome.

    OBJECTIVE: To describe the clinical and pathologic features of a new dominantly inherited audiovestibular syndrome. methods: history, examination, and audiometric testing in the proband, brother, and son; quantitative rotational testing in the proband and son; histopathology of the cochlea and vestibular labyrinth in the proband; sequencing candidate genes COCH and MYO7A in the brother and son. RESULTS: Affected family members developed slowly progressive hearing loss beginning in their late 30s and progressive imbalance in their early 70s. Three of four affected had brief (minutes) episodes of vertigo typically occurring a few times per year. Auditory and vestibular function testing documented a slowly progressive loss of auditory and vestibular function. Postmortem examination showed a loss of hair cells in the cochlea and vestibular receptor organs. There were no cellular infiltrates or acidophilic deposits. No mutations were found in the COCH or MYO7A genes. CONCLUSIONS: This dominantly inherited audiovestibular syndrome results in a selective loss of hair cells in the auditory and vestibular end organs. Finding the causative gene could have important implications for understanding the pathophysiology of presbycusis and dysequilibrium of aging. ( info)

19/57. An unusual disease presenting at an unusual age: Susac's syndrome.

    Susac's syndrome is a rare disease of unknown aetiology affecting the small vessels of the retina, brain, and cochlea. We present the case of a 55-year-old female, the oldest patient yet described with the condition, and highlight the syndrome's clinical features. ( info)

20/57. Paget disease and sensorineural hearing loss associated with spiral ligament degeneration.

    HYPOTHESIS: Previously unreported cystic degeneration of the spiral ligament in cases of Paget disease. BACKGROUND: About 70% of cases of Paget disease involve the skull, with hearing affected in approximately 50% of these. The hearing impairment may be sensorineural, mixed, or, rarely, only conductive. The etiology and pathogenesis of the hearing loss are not yet understood, and reports in the literature are inconsistent regarding the pathologic changes responsible for sensorineural hearing loss. Of six pairs of temporal bones from patients with Paget disease in the temporal bone collection of a research institution, two pairs have abnormalities not previously associated with sensorineural hearing loss or Paget disease. We report the histopathologic findings in these temporal bones. methods: The temporal bones were fixed in formalin, decalcified in ethylenediaminetetraacetic acid, embedded in celloidin, and sectioned in the horizontal plane at a thickness of 20 microm. Every 10th section was stained with hematoxylin-eosin and mounted on glass slides. The sections were examined by light microscopy. RESULTS: Cystic degeneration of the spiral ligament, primarily in the basal segment, was found in both cases. endolymphatic hydrops and a small endolymphatic sac with calcification of the perisaccular tissue were found in one case. CONCLUSIONS: Cystic degeneration of the spiral ligament has not been previously reported and may be unique to Paget disease. This is consistent with recent literature showing a previously unsuspected role of the spiral ligament in sensorineural hearing loss. ( info)
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