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1/1409. carbamazepine-induced sensorineural hearing loss.

    carbamazepine is a commonly prescribed anticonvulsant medication that affects various levels of the nervous system. We report a case of temporary sensorineural hearing loss in a patient after overdosing with 36 g of carbamazepine. Six days after the overdose, the patient complained of bilateral hearing loss and tinnitus. Audiograms revealed a 30- to 40-dB sensorineural hearing loss bilaterally. Another audiogram 2 weeks later showed a complete recovery in both ears accompanied by a clinical resolution in audiovestibular symptoms. carbamazepine is used to treat partial and generalized seizures, trigeminal neuralgia, and bipolar illness. Adverse effects are not common but most frequently include dizziness, drowziness, nausea, and skin rashes; rare complications are agranulocytosis, bradycardia, and heart block. Documented hearing loss as a side effect of carbamazepine has not been reported, to our knowledge. ( info)

2/1409. Minimal, progressive, and fluctuating hearing losses in children. Characteristics, identification, and management.

    Referring to specific types of hearing loss as "minimal" or "mild" seems to imply that their effects are equally mild or negligible. A growing body of literature, however, supports the notion that such losses can have a significant impact on the communicative and educational development of young children. Although OME is considered a common childhood ailment, mounting evidence suggests that it is not always benign and may contribute to significant educational and communicative difficulties in some young children when accompanied by conductive hearing loss. Even very mild bilateral and unilateral SNHL seems to contribute to problems in the areas of social and emotional function, educational achievement, and communication in some children. Because these hearing losses are so mild, they may not be immediately recognized as the source of such difficulties. The purpose of this report is to heighten the general pediatrician's awareness of the significance of even very mild or minimal hearing losses in children. As the gatekeepers for children's health care, pediatricians are typically the primary recipients of parental expressions of concern and the initiators of evaluations or referrals to address such. ( info)

3/1409. family with low-grade neuroendocrine carcinoma of salivary glands, severe sensorineural hearing loss, and enamel hypoplasia.

    Four sibs in a family on the Isle of Man, two brothers and two sisters ranging in age from 33 to 45 years, presented with low-grade malignant tumors of the submandibular gland in three cases and of the nasal cavities and maxillary sinuses in one. The neoplasms were all of the same histological type, apparently hitherto undescribed, showing well-differentiated neoplastic ducts, surrounded by neoplastic myoepithelial cells, together with sheets of epithelial cells expressing neuroendocrine markers by immunohistochemistry. Cervical neck node metastases have developed in all four cases. In the sib with a primary sinonasal neoplasm, widespread bloodstream metastases also became manifest and a single such metastasis in his brother. All four sibs have severe enamel hypoplasia and the same lesion is present in 5 of their 11 children. In the two male patients, severe sensorineural hearing loss has developed in adult life, unilateral in the left ear in one brother, bilateral in the other. In the brother with bilateral sensorineural hearing loss, magnetic resonance imaging revealed a vestibular schwannoma on the left side, which is currently under treatment. The inherited hearing loss is thought to be unilateral in this case also. ( info)

4/1409. Successful cochlear implantation in a patient with melas syndrome.

    OBJECTIVE: To describe methods of assessing cochlear implant candidacy in patients with potentially significant peripheral and central nervous system (CNS) degeneration. STUDY DESIGN: A patient with a degenerative CNS disease (melas syndrome) undergoing evaluation for cochlear implantation is described. SETTING: This study took place at a tertiary care center. PATIENT: A patient with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) who had cortical blindness and profound sensorineural hearing loss was evaluated and rehabilitated with cochlear implantation. INTERVENTIONS: Pure-tone audiogram, behavioral responses to promontory stimulation electrical auditory brainstem response, and electrically evoked middle-latency responses (MLRs) were used to assess eighth nerve, auditory brainstem, and cortical auditory pathways. cochlear implantation with Cochlear Corporation mini 22 implant was performed. RESULTS: Repeatable electrically evoked MLRs and behavioral responses to promontory stimulation documented the presence of auditory cortical responses. Successful implantation resulted in open set speech recognition and communication using the auditory/oral mode. CONCLUSION: This report describes successful implantation in a patient with melas syndrome and demonstrates the ability to preoperatively confirm the integrity of brainstem and cortical auditory pathways despite significant CNS degeneration. ( info)

5/1409. Case report and discussion of hearing preservation after translabyrinthine excision of small acoustic tumors.

    OBJECTIVE: Since 1991, three separate reports have shown how hearing may be salvaged after translabyrinthine excision of small acoustic tumors. The authors submit yet another report of a complete translabyrinthine excision of a 1.4-cm intracanalicular acoustic tumor with modest hearing preservation. An attempt is made to retrace the steps of the operation and recognize and discuss what particular events may have safeguarded the viability of the cochlea. With the availability of cochlear implantation, there should be added incentive to preserve the cochlear neurones if hair cells cannot be saved. STUDY DESIGN: The study design was a retrospective case review. SETTING: The study was conducted at a primary care hospital. INTERVENTION: Therapeutic and rehabilitative measures were performed. MAIN OUTCOME MEASURES: Hearing preservation was measured. CASE REPORT: A 55-year-old woman presented with a left-sided hearing loss and a 1.4-cm left acoustic tumor completely filling the internal auditory canal (speech reception threshold [SRT] 30 dB, discrimination [Pb] 28%). A successful translabyrinthine excision of the tumor was performed in November 1995. A 1-year postoperative audiogram showed a mixed hearing loss in the left ear with SRT 85 dB and Pb 0%. Average pure-tone threshold for 500 Hz, 1 kHz, and 3 kHz was 50 dB and aided SRT 40 dB with Pb 64%. Postoperative magnetic resonance imaging confirmed complete excision of the tumor. CONCLUSION: An exceptional case of hearing preservation after translabyrinthine excision of a small acoustic tumor illustrates how it may be possible to preserve cochlear hair cells and neurones simultaneously in certain selected cases. A review of the surgical events shows the value of sealing the cochlear duct with bone wax, selectively removing the vestibular nerves with the tumor by sharp dissection, and safeguarding the meatal segment of the anterior inferior cerebellar artery by a limited dural incision. ( info)

6/1409. Sensorineural hearing loss associated with otitis media with effusion in children.

    Sensorineural hearing loss (SNHL) is known to occur in various types of otitis media. Although the mechanism by which SNHL develops in association with otitis media with effusion (OME) is unknown, several hypotheses have been advocated up to now. We reviewed the clinical records of children with otitis media with effusion (OME) to reveal the association with sensorineural hearing loss. The material consisted of 71 children (119 ears) who were diagnosed as having OME and gave reliable audiograms in our clinic during an 11 month period from February 1997 through January 1998. From these cases those which showed bone conduction loss of 25 dB or higher at any one of the frequencies of 250 through 4 kHz were selected and considered to be cases of SNHL. Eight cases (9%) which had temporary threshold shift (TTS) or permanent threshold shift (PTS) were considered to be etiologically related to OME. The clinical course in each of these cases with SNHL was reviewed and evaluated in detail. We noted that all children with TTS improved completely. The result of this study indicates that we have to be aware of a possible development of SNHL during the course of OME. ( info)

7/1409. Labyrinthine involvement in Langerhans' cell histiocytosis.

    BACKGROUND: Langerhans' cell histiocytosis, a rare condition caused by the proliferation of abnormal Langerhans' cells ('LCH cells') and an accompanying granulomatous infiltrate, can affect several organs including the ear. External and middle ear involvement are common with a reported incidence as high as 61%. The bony labyrinth is resistant to erosion by the granulation tissue, thereby protecting the cochlea and vestibular structures. Probably for this reason, involvement of the inner ear is rare, with few case reports in the literature. patients: We report two girls, one with bilateral and the other with unilateral mastoid involvement, in whom there was invasion of the labyrinth. The first girl had 'single system' LCH affecting only bone and developed an acute hearing loss due to invasion of the cochlea, while the second had both bone and skin involvement and labyrinthine involvement was diagnosed on imaging prior to the onset of labyrinthine symptoms. CONCLUSION: Inner ear involvement can lead to permanent deafness, which may be prevented by early institution of treatment. Threatened inner ear involvement requires urgent systemic medical therapy with steroids, possibly combined with chemotherapy. ( info)

8/1409. Persistent bilateral hearing loss after shunt placement for hydrocephalus. Case report.

    Transient hearing decrease following loss of cerebrospinal fluid (CSF) has been reported in patients undergoing lumbar puncture, spinal anesthesia, myelography, and/or different neurosurgical interventions. The authors present the first well-documented case of a patient with persistent bilateral low-frequency sensorineural hearing loss after shunt placement for hydrocephalus and discuss the possible pathophysiological mechanisms including the role of the cochlear aqueduct. These findings challenge the opinion that hearing decreases after loss of CSF are always transient. The authors provide a suggestion for treatment. ( info)

9/1409. Effects of acknowledging a hearing loss on social interactions.

    An important part of the counseling process is to offer guidance to people with hearing impairments on accepting their disability and adjusting to the negative attitudes held by some communications partners. The purpose of this study was to determine subjects' (1) preferences for interacting with individuals with a hearing impairment who acknowledged their hearing loss, and (2) perceptions of personality, employability, intelligence, and adjustment related to acknowledgment of hearing loss. Two male students with hearing impairments were videotaped under two experimental conditions. During one condition students acknowledged their hearing losses, spoke about adjustment, concerns, etc., while during the other condition they did not acknowledge their hearing loss. Results indicated that subjects preferred speakers when they discussed their hearing loss. Speakers who acknowledged their hearing loss also received more favorable ratings on personality, employability, and adjustment. Clinical implications for aural rehabilitation programs are discussed. ( info)

10/1409. Study of systemic lupus erythematosus in temporal bones.

    Despite some reports of sensorineural hearing loss with systemic lupus erythematosus (SLE), its pathologic correlate has remained unidentified due to the scarcity of human temporal bone studies. We here present findings in 14 temporal bones from 7 patients with SLE, examined histologically and immunohistochemically for pathologic conditions in the cochlea that might relate to their otologic histories. Blue-staining concretions were seen in the stria vascularis of 6 ears. Most of the cases showed a loss of spiral ganglion cells, with various degrees of hair cell loss and atrophy of the stria vascularis. One ear demonstrated formation of fibrous tissue and bone throughout the cochlea, with complete loss of the membranous labyrinth. Cochlear hydrops was found in only 1 ear. These findings in temporal bones from patients with SLE are discussed in relation to autoimmune disease of the inner ear. ( info)
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