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Use of distortion-product otoacoustic emissions for auditory evaluation in Menière's disease.

Otoacoustic emissions are a relatively new tool for assessment of inner ear function. We studied 65 patients diagnosed as having Menière's disease. In order to obtain and characterize responses at different frequencies we used distortion-product otoacoustic emissions (DPOAE). In unilateral cases, the difference in response between ears on the diseased and healthy sides was evident at all frequencies. In the former the result was lower than expected for a group of normally hearing subjects, while in the latter it was better than expected according to pure tone averages. Reduction in amplitude and increment in threshold of the DPOAE in the diseased ear was a significant finding and could be correlated to the stage of the disease. In those subjects treated with Betahistine after 1 year's follow-up, DPOAE testing showed the recovery of response at low frequencies and a reduction of its threshold at all frequencies studied.

Acoustic Stimulation↗

Surgery of otosclerosis in the elderly.

Auditory results following stapes surgery in 26 patients with otosclerosis >60 years of age at the time of surgery were compared with those obtained in 140 patients <60 years undergoing stapes surgery during the same time period. Stapedotomy was performed in all cases. The mean follow-up period was 29 months. Hearing results as judged by postoperative air-bone gaps were as good in the older age group as in the younger patients. Moreover, complications of surgery, such as postoperative formation of perilymphatic fistula, did not occur more frequently in elderly vs younger patients. On the basis of the results obtained, it is concluded that stapes surgery should be offered to elderly patients with the same indications as younger patients with otosclerosis.

Adult↗

[Results in terms of hearing results after surgical closure of labyrinthine fistulas].

OBJECTIVES: We investigated the results achieved in terms of hearing in 36 patients who had each undergone resection of a cholesteatoma and closure of a labyrinthine fistula. PATIENTS AND METHODS: These patients included 28 who had a fistula of the semicircular canal, in 7 of them with an opened perilymphatic space. In 8 cases we found a fistula of the cochlea, with with a perilymphatic leak 7 in of them. We compared the sensorineural threshold before and after surgery and also the results in the two groups. Patients who were deaf preoperatively were excluded from further analysis. RESULTS: Hearing did not improve in any of the patients (n=5) who were deaf preoperatively. There was no significant difference in changes to the hearing threshold between patients with fistula of the cochlear and those with fistula of the semicircular canal with opened perilymphatic space; nor did patients with a fistula of the semicircular canal without perilymphatic leakage have a better outcome than patients with an open membranous labyrinth. CONCLUSIONS: The outcome of cholesteatoma resection and closure of the fistula in a single operation was good. Perilymphatic leakage seems to be predictive of a poorer result in terms of hearing.

Audiometry, Pure-Tone↗

[Auditory brainstem responses evoked by direct mechanical stimulation of the ossicular chain. Objective preoperative testing of candidates for implantable hearing aids].

BACKGROUND AND OBJECTIVE: The function of several implantable hearing systems is based on the vibratory excitation of the ossicular chain. For preoperative testing, malleus vibration audiometry has been described [7]. In special cases, it could be desirable to perform objective testing before implantation. We therefore studied the auditory brainstem responses (ABR) evoked by vibratory stimulation of the ear drum. METHOD: The ABR evoked by vibratory and acoustical click stimulation were recorded in 20 subjects with normal hearing at four different stimulus intensities. For the vibratory stimulation (v-ABR), the transducer rod was brought into mechanical contact with the eardrum under microscopic control. This setup was further used for the determination of the individual subjective threshold. The v-ABR were compared to conventional ABR recordings. RESULTS: In all subjects, the v-ABR could clearly be identified. At high stimulus levels, the complete wave pattern was observed in 15 of 20 cases. There is no essential difference between v-ABR and conventional ABR with respect to the incidence and parameters of the potentials. At high stimulus levels, a pronounced stimulus artefact impedes the identification of wave I in some cases. Moreover, the quality of v-ABR is limited by the uncomfortable situation and incomplete relaxation of the subject. CONCLUSIONS: The measurement of vibratory evoked ABR is practicable without hazards for the patient and it yields reliable results. The hearing threshold for click stimuli can be determined from the incidence and amplitude of the responses. The loudness associated with the click stimuli can be estimated from the response latency. Therefore, the investigation of v-ABR not only gives evidence of the individual suitability for implantable hearing aids but its outcome may also be useful as an orientation for the postoperative fitting.

Acoustic Stimulation↗

[Clinical and molecular genetic analysis of monozygotic twins displaying stapes gusher syndrome (DFN3)].

BACKGROUND: DFN3 ( "stapes gusher") is the most frequent form of X-linked hearing impairment. It accounts for up to 0.5% of all cases of severe childhood hearing disorders. PATIENTS AND METHODS: Monozygotic twins with suspected stapes gusher syndrome, their mother, and control individuals were analyzed clinically and genetically. RESULTS: The clinical investigations confirmed a DFN3 phenotype in both brothers who displayed all typical symptoms. A molecular genetic investigation of the POU3F4 gene, which plays an essential role in the development of DFN3, was also performed. No chromosomal aberrations within the coding region of POU3F4were detected. Since several authors have described mutations in the 5' untranslated region of the gene also resulting in a DFN3 phenotype, we screened this area by microsatellite analysis and detected a double deletion localized in the critical interval. This is the first description of a double deletion in the non-coding region of POU3F4 leading to DFN3 phenotype. CONCLUSION: Interestingly, in spite of having an identical genotype, the twins displayed significant phenotypic differences. This underlines the importance of exogenous factors in the development of inherited pathological processes.

Audiometry, Pure-Tone↗

["One shot" CO2 laser stapedotomy].

BACKGROUND: In order to further optimize the surgical technique with CO(2) laser in stapes surgery, a scanner system was used to obtain a footplate perforation of 0.5-0.6 mm with only one laser application ("one-shot" stapedotomy). PATIENTS AND METHODS: A total of 255 patients with otosclerosis were submitted to primary CO(2) laser stapedotomy with a SurgiTouch scanner. This study discusses the surgical technique and clinical results. RESULTS: An adequately large perforation diameter could be achieved with a single shot in 68% of the patients treated. In 14% of the patients, a second laser application at the same site was necessary. In 18% the perforation had to be enlarged by several slightly overlapping laser applications without using the scanner. There was no evidence of laser dependent inner ear affections. CONCLUSION: CO(2) laser, combined with modern scanner systems, is well suited for application in stapes surgery.

Adolescent↗

[Measuring vibration properties of middle ear implants with the mechanical middle ear model. Initial results].

With the aid of a mechanical middle ear model (MMM) the sound transmission properties of different middle ear implants were investigated. Input of the MMM involved a broad-band signal from 100 to 5000 Hz that was supplied by a miniaturized loudspeaker. Displacement of an artificial stapes footplate was measured by a fiberoptic probe. The transfer functions of four different total ossicular replacement prostheses (TORPs) of different materials and shapes were compared. Three of the devices revealed similar transfer functions which corresponded to the typical curve of the normal middle ear. One of the prostheses demonstrated a high-frequency deterioration of approximately 5 dB. This effect was explained by a 3- to 6-times higher mass of the implant when compared to the others. Altogether, the weight of the prosthesis seems to have the most marked impact on sound transmission to the inner ear, whereas stiffness of the implant itself is less crucial as long as it can be regarded as a rigid body.

Acoustic Impedance Tests↗

Fibromyalgia as a disorder of perceptual organization? An analysis of acoustic stimulus processing in patients with widespread pain.

We examined to what extent patients with fibromyalgia differ from painfree control subjects in the perception and processing not only of somatosensory but also of external stimuli. For this purpose the acoustic perception of 30 patients with fibromyalgia was compared with that of 36 generally pain-free age and gender matched subjects. The groups were also controlled for organic disease of pathological dysfunction of the ear and auditory nerves. Thresholds of unpleasantness and hearing thresholds were determined autiometrically for various frequencies. In addition the participants rated their experience of daily noise, vulnerability to acoustic stress, and functional and affective complaints associated with fibromyalgia. As expected the results show reduced unpleasantness thresholds for all frequencies and a nonsymptomatic hearing loss for higher frequencies. The elevated hearing threshold correlated significantly with experience of noise at the place of work, which was also elevated in the fibromyalgia group. Generalized pain had a high impact on the interaction between threshold of unpleasantness and daily noise experience. We interpret the differences in thresholds of hearing and of unpleasantness in patients with fibromyalgia as a form of either preconscious or conscious acts to protect against disturbing stimulation. Our results support the notion of a generalized disturbancy of perceptual thresholds in patients with fibromyalgia not restricted to the perception of pain.

Adult↗

Laser excision of glomus tympanicum tumours: long-term results.

The purpose of this study was to evaluate the long-term results of laser excision of glomus tympanicum tumours in a series of patients who had been referred to the senior author (DAM) in a retrospective case review. Nine patients underwent complete excision of their glomus tympanicum tumours using a Diode or KTP laser. There were no cases of tumour recurrence. There were no complications during the peri- or postoperative period. Follow-up time was 31.8 months. In our experience, laser excision can be performed with minimal bleeding and morbidity, and it provides excellent tumour control in glomus tympanicum surgery.

Adolescent↗

Short-term results using Kurz titanium ossicular implants.

The efficiency of titanium middle ear prosthesis for ossicular reconstruction in chronic ear disease is investigated in a Scandinavian two-center retrospective study from a Norwegian tertiary otology referral center and a Finnish otology referral center. Retrospective chart reviews were performed for procedures involving 73 titanium prostheses between 1999 and 2004. All patients that underwent surgery including the Kurz Vario titanium prosthesis were included in the study, 38 procedures including the partial ossicular replacement prosthesis (PORP) and 35 procedures including the total ossicular replacement prosthesis (TORP). Mean follow-up was 14 months. The ossiculoplasty was performed alone (29 patients) or in combination with other chronic ear surgery procedures (34 patients). Comparisons of preoperative and postoperative pure tone averages (0.5, 1, 2, and 3 kHz) according to AAO-HNS guidelines are presented, as well as data for different PTA definitions. Otosurgery procedures, complications, revisions, and extrusion rates are reported. A postoperative air-bone gap (ABG) of <or=20 dB was obtained in 77% of the patients, 89% for the Bell (Porp) prosthesis, and 63% for the Arial (Torp) prosthesis. Overall mean pure tone averages improved 20.6 dB with ABG improvement of 19.3 dB. The overall extrusion rate was 5% (4 patients). Titanium prostheses have been easy and fast to handle and effective implants for reconstruction of the ossicular chain. We found no difference between reconstruction with or without cholesteatoma surgery during the same procedure. The combination of CWD and Torp gave significant inferior hearing thresholds as compared to Torp/CWU and Porp/CWD combinations.

Audiometry↗

Primary bone tumours of the spine: a 42-year survey from the Leeds Regional Bone Tumour Registry.

We conducted a review of the Leeds Regional Bone Tumour Registry for primary bone tumours of the spine since establishment in 1958 until year 2000. To analyse the incidence of primary tumours of the spine and to record the site of occurrence, sex distribution, survival and pathology of these tumours. Primary tumours of the spine are particularly rare, accounting for between 4 and 13% of published series of primary bone tumours. The Leeds Bone Tumour Registry was reviewed and a total of 2,750 cases of bone tumours and tumour-like cases were analysed. Consultants in orthopaedic surgery, neurosurgery, oncology and pathology in North and West Yorkshire and Humberside contribute to the Registry. Primary bone tumours of the osseous spine constitute only 126 of the 2,750 cases (4.6%). Chordoma was the most frequent tumour in the cervical and sacral regions, while the most common diagnosis overall was multiple myeloma and plasmacytoma. Osteosarcoma ranked third. The mean age of presentation was 42 years and pain was the most common presenting symptom, occurring in 95% of malignant and 76% of benign tumours. Neurological involvement occurred in 52% of malignant tumours and usually meant a poor prognosis. The establishment of Bone Tumour Registries is the only way that sufficient data on large numbers of these rare tumours can be accumulated to provide a valuable and otherwise unavailable source of information for research, education and clinical follow-up.

Adolescent↗

Computer-integrated finite element modeling of human middle ear.

The objective of this study was to produce an improved finite element (FE) model of the human middle ear and to compare the model with human data. We began with a systematic and accurate geometric modeling technique for reconstructing the middle ear from serial sections of a freshly frozen temporal bone. A geometric model of a human middle ear was constructed in a computer-aided design (CAD) environment with particular attention to geometry and microanatomy. Using the geometric model, a working FE model of the human middle ear was created using previously published material properties of middle ear components. This working FE model was finalized by a cross-calibration technique, comparing its predicted stapes footplate displacements with laser Doppler interferometry measurements from fresh temporal bones. The final FE model was shown to be reasonable in predicting the ossicular mechanics of the human middle ear.

Acoustic Stimulation↗