Cases reported "Hearing Disorders"

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1/20. Otoacoustic emissions and auditory brainstem responses after neonatal hyperbilirubinemia.

    Severe hyperbilirubinemia often results in hearing loss. Behavioral audiometry, auditory-evoked brainstem responses (ABRs) and otoacoustic emissions (OAEs) were performed in three such patients in an attempt to localize the pathophysiology of this hearing loss. Behavioral audiometric findings in these patients (all male, 4, 15 and 25 years old) ranged from severe in the 4-year-old, moderate in the 15-year-old and slight in the 25-year-old. Where obtained, ABR wave V thresholds were elevated or ABR were absent. However, absolute and inter-wave latency measurements were not indicative of brainstem pathology. OAEs (transient and distortion product) could only be obtained in the high- or low-frequency ranges in these patients. Our findings suggest that at least some lesions producing hearing loss in severe hyperbilirubinemia are in the cochlea, especially at the outer hair cells. Finally, we found that only moderately elevated serum bilirubin levels (<20 mg/dl) may contribute to the development of sensorineural hearing loss.
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keywords = wave
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2/20. The role of radiation in delayed hearing loss in nasopharyngeal carcinoma.

    Although radiation damage to the inner ear can be induced experimentally in animals, its incidence in humans as a complication of therapy for nasopharyngeal carcinoma (NPC) remains a matter of conjecture due to inadequate histological validation. A case of advanced NPC with a mixed conductive and neurosensory hearing loss is presented that at necropsy showed tumour invasion of the VIIIth cranial nerve in the internal auditory meatus with associated infection. The architecture of the organ of corti was well-preserved despite heavy doses of radiation but degeneration, the cause of which could not be substantiated, was present in the auditory nerve pathway. A diagnosis of radiation damage to the end-organ of hearing in the absence of supporting histological evidence should be made with caution.
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ranking = 0.71974481049767
keywords = radiation
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3/20. The effect of midline petrous apex lesions on tests of afferent and efferent auditory function.

    OBJECTIVES: Historically, audiological procedures have focused on the assessment of the afferent (ascending) cochlear-VIIIth nerve system and have, for the most part, ignored the efferent (descending) auditory system. We report afferent and efferent auditory test results for two cases with a cholesterol cyst of the right petrous apex; one lesion involves the afferent segment of the auditory system, and the second lesion involves both the afferent and efferent segments of the auditory system. These "natural experiments" provide a unique opportunity to study the effect of a space-occupying lesion of the petrous apex on afferent and efferent function of the auditory system. DESIGN: Transient evoked otoacoustic emission (TEOAE) suppression studies were performed to assess the effect of the cholesterol cyst on the efferent system of the two cases. In addition, three complementary afferent tests of brain stem auditory function were administered: 1) acoustic reflex thresholds (ARTs); 2) masking level difference (MLD); and 3) auditory brain stem response (ABR). These tests are complementary because the superior olivary complex (SOC) is involved not only in the mediation of the sound evoked efferent reflex assessed in TEOAE suppression, but in the mediation of the ARTs, the MLD, and the ABR. RESULTS: The two cases with midline petrous apex lesions, one not involving the VIII-cochlear efferent auditory system, differed from each other with regards to TEOAEs suppression, and ARTs. Specifically, the case with only afferent involvement produced normal TEOAE suppression, a normal MLD, normal ARTs, and abnormal waves III and V of the ABR, whereas the case with both afferent and efferent involvement produced abnormal TEOAE suppression, a normal MLD, abnormal ARTs, and abnormal waves III and V of the ABR. CONCLUSIONS: These cases illustrate that although several auditory tests can be mediated within the same or adjacent anatomical structures, i.e., the SOC, they may not be equally affected by the same lesion due to different physiology. Further, the TEOAE suppression paradigm is a clinically relevant test to assay the sound evoked efferent reflex that is mediated by the medial olivocochlear system of the SOC.
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4/20. Pineal region tumor manifesting initially as hearing impairment.

    An 18-year-old male presented with a pineal region germinoma with hearing impairment as the chief complaint. Magnetic resonance image demonstrated a well-enhanced multi-cystic tumor extending into the upper fourth ventricle and wall of the bilateral lateral ventricles. audiometry revealed bilateral mild hearing impairment in the low frequencies. Auditory brainstem response recording showed low amplitudes in all waves (IV-V/I ratio < 1) with prolong latencies (I-V and III-V) on the right but no discernable wave at 60 dB clicks on left. Hearing impairment and audiometric findings were improved after ventriculoperitoneal shunt operation. The hearing impairment appeared to be a mixed (conductive and sensorineural) type. The tumor was responsible for the sensorineural deafness because of invasion and compression of the central auditory structures. The inferior brachium was maximally compressed anterolaterally by the dilated bilateral lateral ventricles and posteromedially by the tumor. hydrocephalus caused conductive deafness by halting or arresting the footplate of stapes movement, as a consequence of high-pressure transmission through the cochlear aqueduct.
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5/20. Long-term results and experience with the first-generation semi-implantable electromagnetic hearing aid with ossicular replacement device for mixed hearing loss.

    OBJECTIVE: To document the long-term surgical and audiologic results of the implantation of a first-generation semi-implantable hearing aid for mixed hearing loss, consisting of a magnetic partial or total ossicular replacement prosthesis and an electromagnetic driver fitted in the ear canal. The short-term results have previously been published as excellent in both surgical and audiologic terms. To analyze and discuss reasons of treatment failure, as well as future perspectives. STUDY DESIGN: Patient file review, follow-up otomicroscopic examination and audiometry, as well as patient interview. SETTING Tertiary referral center at a University hospital. patients: Of nine patients operated on, six with a mixed hearing loss after chronic otitis media could be evaluated primarily. Two underwent implantation with a partial and four with a total ossicular replacement prosthesis. MAIN OUTCOME MEASURES: Use of and satisfaction with the hearing aid, apparatus function and integrity, hearing without electromagnetic driver, prostheses containment, drum abnormalities, nonaudiologic ear symptoms, e.g., discharge. RESULTS: All patients attended follow-up for a mean of 9.5 years after implantation (range 8.8-10 years). No patients used the semi-implantable hearing aid at follow-up. The mean semi-implantable hearing aid using time was 24 months (range 3-60 months). The main reasons for discontinuation of use were problems fitting the electromagnetic driver correctly in the ear canal and prosthesis extrusion or dislocation. CONCLUSIONS: Despite excellent short-term surgical and audiologic results, it is concluded that these first long-term results of the first-generation electromagnetic semi-implantable hearing aids with ossicular replacement prosthesis for mixed hearing loss are disappointing. However, improvements in the surgical approach and in the reshaping, refinement, and overall improvement of the semi-implantable hearing aid could lead to satisfactory treatment of this group of patients.
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ranking = 88517.614223731
keywords = electromagnetic
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6/20. Delayed visual maturation associated with auditory neuropathy/dyssynchrony.

    Delayed visual maturation is a term used to describe infants who initially seem blind but subsequently have a marked improvement. The mechanism of visual loss and the subsequent improvement remains unknown. Auditory neuropathy/dyssynchrony is a condition of hearing impairment associated with absent or severely abnormal brainstem auditory evoked potentials but normal cochlear functions as measured by otoacoustic emissions. In this report, a 9-month-old infant who had no visual fixation for the first 3 months of life and congenital hearing impairment is described. Her brainstem auditory evoked potential study at 2.5 months of age showed no response to click stimuli presented at 90 dB nHL, whereas her otoacoustic emissions were normal. Subsequently, her vision and hearing improved. A brainstem auditory evoked potential study at 9 months of age showed reproducible waveforms. This case suggests the need for a detailed hearing evaluation of children with delayed visual maturation. Furthermore, this case highlights the need for follow-up brainstem auditory evoked potential testing prior to pursuing any audiologic intervention.
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7/20. The feasibility of using oto-acoustic emissions to monitor cochlear function during acoustic neuroma surgery.

    The feasibility of using evoked oto-acoustic emission (EOAE) measurement for intra-operative monitoring of cochlear function was assessed during removal of an acoustic neuroma in a 53-year-old woman with normal hearing on the operated side prior to surgery. The high level of noise in the operating theatre was the only material problem encountered and this was not sufficient to prevent recording of identifiable waveforms. During manipulation of the brainstem, damage to the cochlea was indicated by an increase in EOAE latency and its eventual disappearance. A total hearing loss in the operated ear was revealed after surgery. Monitoring cochlear function with EOAEs is probably best considered at present as an adjunct to auditory brainstem response monitoring of the composite cochlea and eighth nerve, thus providing differential information.
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8/20. aicardi syndrome accompanied by auditory disturbance and multiple brain tumors.

    We described a 5-month-old girl with aicardi syndrome accompanied by auditory disturbance and multiple brain tumors. She was admitted to our hospital because she suffered from intractable flexor spasms. physical examination revealed craniofacial asymmetry, left auricular deformity, scoliosis, and remarkable hypotonia with psychomotor retardation. Abnormal ophthalmological findings included chorioretinopathy with pale and round-shaped peripapillary lacunae, and there was modified hypsarrhythmia in her EEG. MRI revealed multiple brain tumors in the 3rd and the lateral ventricles which are considered to be choroid plexus papilloma with agenesis of the corpus callosum. ACTH therapy was administered because of the intractable seizures. After ACTH therapy, the thresholds of waves I and V were much improved. The interpeak latency of waves I-V of the left ear and the peak latency of wave I of the right ear had been lengthened. Acoustic reflex with contralateral stimulation showed no response in the left ear. These findings indicate that the auditory system is also involved in the aicardi syndrome and that ACTH is effective for its dysfunction.
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ranking = 1.5
keywords = wave
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9/20. glomus jugulare tumours. (A series of 21 cases).

    Twenty-one cases of glomus jugulare tumours were analysed in retrospect. Six had been treated surgically, ten by irradiation, and five by a combination of incomplete surgical excision followed by irradiation. Recurrences appeared in four cases, but no patient has died of the tumour during the follow-up period which is from a few months to eleven years. Metastases were not found in any case. The optimal time of irradiation in combined treatment is discussed.
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ranking = 0.30846206164186
keywords = radiation
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10/20. Hearing impairment in two boys with I-cell disease.

    Auditory brainstem responses (ABR) were analyzed in 2 boys with I-cell disease. Both patients showed a prolonged latency of wave I, a normal I-V interpeak latency and an elevated threshold of wave V. The intensity-latency curve had a steep slope. These abnormal ABR findings reflect the presence of conductive hearing impairment associated with the cochlear component in I-cell disease.
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