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1/26. 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.
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2/26. 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.
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3/26. 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.
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4/26. diagnosis and treatment of a severe psychotic illness in a man with dual severe sensory impairments caused by the presence of Usher syndrome.

    The present paper reports the case history of a 50-year-old man born with Usher syndrome, who developed a psychotic illness later in life, to illustrate the specific diagnostic problems, and the value of direct observation and a detailed assessment of communication. The subject had had a significant hearing impairment since birth, problems with balance and developed retinitis pigmentosa, leaving him with progressively limited vision in adult life. A pattern of bizarre and aggressive behaviour, and a disintegration in his ability to communicate using signs developed over 3 months. An initial diagnosis of depression was made, but it later became clearer that the subject had developed a psychotic illness. This condition responded well to a combination of antidepressant and antipsychotic medication. The possible association between Usher syndrome and psychotic illness is also discussed.
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5/26. Multi-channel cochlear implantation in patients with a post-traumatic sensorineural hearing loss.

    There are few accounts of cochlear implantation in adults with post-traumatic sensorineural hearing loss. We report our experience with multichannel implantation in three such patients. Two patients experienced no cognitive or communication deficits as a result of their head injury. At nine months post-implant, compared with our experience of non-head-injured implantees, these patients achieved average or above average scores on audiological performance tests. The third patient presented with cognitive, behavioural and communicative deficits. The level of improvement achieved by this patient, when lip-reading was supplemented with electrical stimulation, in both BKB sentence and connected discourse tracking (CDT) tests was comparable with that of the non-head-injured group. However, his absolute performance at nine months post-implant was well below average. Performance at 18 months on BKB sentences and environmental sound recognition showed little change, and was again well below average, however his score on CDT with lip-reading and electrical stimulation improved considerably and was similar to the average achieved by the non-head-injured group. The major difficulties experienced with this patient were increasing depression and low implant use. Considerably more time was spent in the assessment and rehabilitation of this patient and involved liaison with a number of other agencies. When considering such patients for cochlear implantation it is strongly recommended that these additional requirements are taken into account.
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6/26. cochlear implantation of auditory neuropathy.

    Auditory neuropathy (AN) is a hearing disorder that presents with a grossly abnormal or absent neural response as measured by evoked potentials in the presence of normal outer hair cell function evidenced by present otoacoustic emissions or cochlear microphonics. rehabilitation for patients with AN is challenging due to abnormal temporal encoding at the auditory nerve leading to severely impaired speech perception. Although patients with AN may demonstrate improvement in thresholds with amplification, temporal encoding dysfunction, and consequently speech perception degradation, is not alleviated by amplification. Another issue is the heterogeneity of the AN population in terms of audiologic and neurologic findings, in addition to uncertain etiology and pathophysiology. For children with prelingual onset of AN, development of auditory and oral communication skills is particularly compromised. All children with hearing loss in the severe-to-profound range who do not benefit from conventional amplification can be considered candidates for a cochlear implant (CI). This paper presents a case study of a child with AN who received a CI. Whereas no synchronous neural response auditory brainstem response could be elicited to acoustic stimuli, an electrically evoked auditory nerve action potential was evident following implantation, suggesting restoration to some degree of neural synchrony. Significant improvement in speech perception was found post-CI. Recommendation to implant all patients with AN would be premature, but these findings suggest that electrical stimulation in some cases of auditory neuropathy can be a viable option.
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7/26. Auditory neuropathy: case study with hyperbilirubinemia.

    Auditory neuropathy (AN) has been described in the literature as presenting with a combination of audiometric findings that include elevated behavioral audiometric thresholds, auditory brainstem response findings that are not consistent with audiometric findings, poor speech recognition, and present otoacoustic emissions (OAEs) and/or cochlear microphonics. Since the availability of clinical OAE testing, AN has come to be identified with increasing frequency; however, incidence and prevalence figures are unavailable. There is a great deal of discussion about the accurate diagnosis of AN, its characteristics, and its treatment. Some of this discussion is occurring on the internet and over the telephones. The need to continue to provide information in accessible peer-reviewed journals is paramount. Following a review of the literature, a case study is presented of a boy who was diagnosed with AN as a newborn. He experienced hyperbilirubinemia and other neonatal health complications. His educational intervention was managed elsewhere until recently. Information is presented about the progression of the case over a 5-year period that includes audiologic data and communication development results.
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8/26. Auditory manifestations of keratitis-ichthyosis-deafness (KID) syndrome.

    OBJECTIVE: Evaluation of the auditory manifestations of keratitis-ichthyosis-deafness (KID) syndrome, a rare genodermatosis characterized by follicular hyperkeratosis, vascularizing keratitis, and congenital hearing loss. STUDY DESIGN: Five individuals with sporadic KID syndrome were evaluated in the outpatient audiology clinic at the Warren Grant Magnuson Clinical Center of the National Institutes of health. methods: Audiologic examinations included pure-tone audiometry, speech audiometry, and middle ear immittance testing. Auditory brainstem responses and otoacoustic emissions were analyzed in 2 subjects. RESULTS: Four subjects had prelingual, bilateral, profound sensorineural hearing loss, whereas the fifth subject had significant residual hearing that exhibited no progression on serial audiograms. All 5 subjects had a history of non-erosive keratosis obturans and cutaneous cysts in the external ear canals that prevented continuous use of ear molds. CONCLUSIONS: The sensorineural hearing loss in KID syndrome is generally prelingual and profound. This combination of auditory and cutaneous phenotypes is similar to those previously reported for KID syndrome. KID syndrome presents a difficult challenge for communication rehabilitation because keratitis may impair the perception of sign and spoken language, and the cutaneous manifestations routinely curtail use of external amplification devices.
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9/26. alstrom syndrome with acute pancreatitis: a case report.

    We report the case of a 21-year-old female with alstrom syndrome who also suffered from acute pancreatitis of obscure manifestation. The patient had underlying cone-rod dystrophy of the retinas, nystagmus, obesity, progressive sensorineural hearing impairment, diabetes mellitus, and hypertriglyceridemia, compatible with the clinical diagnosis of alstrom syndrome. Serial examinations showed liver dysfunction and pancreatitis. In treating a patient with poor communication (i.e. cone-rod dystrophy and hearing impairment) suffering from acute illness, understanding the underlying disease and the potential for pancreatitis with hypertriglyceridemia is necessary. It is also a challenge to treat a patient with multiple system involvement. In conclusion, alstrom syndrome is a disease of systemic multi-organ involvement, and hepatic disease and pancreatitis, possibly due to dyslipidemia, appear to be manifestations of alstrom syndrome.
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10/26. genetic counseling for the deaf.

    genetic counseling is a process that emphasizes accurate diagnosis of hereditary conditions and communication of information to families. genetic counseling involves systematic collection of family and medical history, a physical examination by a certified clinical geneticist, sharing of information with the family, and follow-up and support services. The issues that arise in genetic counseling can differ for every family and are often dependent on the degree of deafness present in the family, age of onset, and linguistic and cultural orientation. It is important for the genetic counselor to consider these factors in the provision of genetic services. With the increasing application of molecular genetics to the diagnosis and management of hereditary deafness and the increasing participation of families with deafness in research studies, the involvement of genetic counselors to provide information and education to consumers as well as medical professionals and researchers is becoming even more critical. The success of genetic counseling for the provision of information to families and the delineation of types of hereditary deafness through clinical and laboratory research is dependent on appropriate referrals by medical professionals, including otolaryngologists. A working relationship between otolaryngologists and clinical geneticists for the referral and evaluation of patients with hereditary deafness or deafness of "unknown" etiology is important.
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