Sex change surgery for patients with gender identity disorders has been performed successfully during the last decade, especially for males becoming females. A troublesome problem for some of these individuals is the retention of a male voice. Castration and estrogen therapy may produce only minor changes in pitch, with patients requiring further surgical treatment. Techniques used include glottoplasty, crico thyroid approximation and/or anterior commissure advancement. Although the voice is highly gender-specific, the standards of care for transsexuals do not cover guidelines for a diagnostic approach and surgical therapy. For medical jurisprudence, questions concerning specific aspects of German social law are answered in a representative case of a male-to-female transsexual. A review of the literature shows that there is no common thought about whether and when a specific surgical procedure should be performed in a gender-transforming process and which method should be used for long-term results and patient satisfaction. Although worldwide experience with these operations and overall good results are assumed, further reports by phonosurgeons performing these operations are required concerning actual results. We propose that medical scientific societies should offer phonosurgery as an optional treatment for male-to-female transsexuals in their guidelines and standards of care.

Adult↗

PubMed1998

[Johanson-Blizzard syndrome. A complex dysplasia syndrome with aplasia of the nasal alae and inner ear deafness].

BACKGROUND: The Johanson-Blizzard syndrome is a rare autosomal recessive syndrome with ectodermal dysplasia. ENT findings in the syndrome include profound bilateral hearing loss, aplasia of the alae nasi and dental malformations. To date approximately 30 cases have been described. CASE REPORT: We report our findings in a female patient who was born as the second child of consanguine parents. Pregnancy was normal, birth weight 3620 g and body length 52 cm. She was hospitalized immediately after birth because of anal atresia and facial dysmorphism with aplastic alae nasi, mongoloid eye slant and slightly dystopic ears. Bilateral symmetric profound hearing loss was diagnosed by subjective hearing tests and confirmed by auditory evoked brainstem potentials. Otoacoustic emissions were absent. Hearing aids were successfully fitted. Other malformations were a duplex of the uterus and vagina and exocrine pancreatic insufficiency. The anal atresia was corrected surgically. DISCUSSION: In general, the exocrine pancreatic insufficiency in the Johanson-Blizzard syndrome requires careful medical management. The aplastic alae nasi require no specific therapy, while in our case in the hearing loss could be treated with hearing aids.

Abnormalities, Multiple↗

PubMed1998

[Evaluating the patient satisfaction questionnaire after inpatient treatment in phoniatrics and pediatric audiology--a contribution to quality control?].

PURPOSE: In Germany public hospitals are compelled to evaluate patient satisfaction with their treatment as a part of quality management. The purpose of this study was to assess by means of a short questionnaire inpatient satisfaction with medical care in a phoniatric-paedaudiologic department. MATERIALS AND METHODS: From April 1995 until March 1997, 638 patients underwent inpatient treatment for phoniatric or paedaudiologic diseases in our department. A confidential questionnaire was handed out to the patients or to accompanying parents. 13 questions covered different aspects of hospital treatment, such as satisfaction with diagnostic and therapeutic measures, nursing care, accommodation, catering and administration. The participants were asked to rate on a scale of 1 (very good) to 6 (very poor). RESULTS: 306 (48%) of the patients returned their questionnaires. 16% of them expressed substantial dissatisfaction both with administration and accommodation. Only 2.7% of the patients conveyed a poor overall impression of their inpatient treatment. 3% asserted they would not want to be treated in the department again as the inpatient. However, the mean overall assessment indicated a high degree of patient satisfaction. CONCLUSIONS: Although the self-explaining 13-item questionnaire is easy to evaluate at low cost, it appears to be an inadequate measuring device for routine assessment of inpatient satisfaction since only 48% of the questionnaires could be analysed.

Adolescent↗

PubMed1998

[Auditory speech-evoked cerebral cortex potentials in patients with stuttering syndromes].

BACKGROUND: The cause of stuttering is unknown. For clinical purposes it proved to be useful to assume a multifactorial genesis with organic psychological and social aspects. A longstanding organic theory focussed on the failure to develop left-hemispheric dominance for speech, whereas others favoured deficits of the speech motor system. Positron emission tomography (PET) studies support organic theories for the development of stuttering. The purpose of this study was to find out whether in stutterers auditory cortical potentials evoked by pure tones, noise and words are different to those of healthy controls. PATIENTS AND METHODS: 10 young adults having suffered from stuttering since infancy were examined. The potentials were measured and analysed as previously described. RESULTS: No correlation of clinical and electrophysiological findings were found. In one case the evoked potentials were normal, in all other patients heterogeneous results were obtained in respect of tone-evoked and both noise- and speech-evoked potentials. Cortical hemispheric differences could be detected. CONCLUSIONS: In agreement with PET findings reported in the literature the data obtained in this study indicate an organic, central nervous cause of stuttering. Obviously both speech motoric components and perception elements are affected. These facts have to be taken into account whenever a psychological cause of stuttering is suspected. Nevertheless, psychosomatic aspects of the disturbances must be considered since they influence the patients' ability to cope with their symptom.

Adolescent↗

PubMed1997

[Chronic tinnitus in children and adolescents].

BACKGROUND: The problem of tinnitus in adults is reviewed systematically in nearly all standard otolaryngology reference works, whereas textbooks and monographs that focus on pediatric otorhinolaryngology or audiology and hearing in children and adolescents provide only little information concerning the epidemiology, etiology and therapy of tinnitus. The purpose of this study was to evaluate the psychosomatic aspects of chronic tinnitus in this younger age group. A rational diagnostic approach is discussed as to which diagnostic measures are necessary in the pediatric group for deciding which therapeutic option to chose. The therapeutic outcome of tinnitus counselling in non-severe cases and of parenteral lidocaine infusions in cases of a troublesome tinnitus is presented. PATIENTS AND METHODS: From January 1992 to December 1995, 31 children and adolescents in the age range from 6 to 17 years were treated for a chronic tinnitus without a measurable hearing loss. In 20 cases the tinnitus was bilateral; in 11 cases it was unilateral, without side preference. In 24 patients the case history gave no hint of a major annoyance by the tinnitus or significant psychological components. In these cases tinnitus counselling was carried out. In 7 cases-3 girls and 4 boys in the age range from 10 to 17 years-the kind and grade of symptom satisfied the ICD-10 criteria of a depressive episode. These patients were hospitalized for 10 days and a lidocaine infusion therapy (2 mg/kg Xylocain Cor in 500 ml HAES 6%) was performed as treatment for the somatic component of the disorder. Data were analyzed catamnestically using the patients' files. RESULTS: In all cases normal hearing threshold and speech intelligibility were ascertained by pure-tone and speech audiometry. Auditory evoked brainstem potentials gave no further information. The measurement of transient evoked otoacoustic emissions gave no consistent results in either of the two groups. Tinnitus measurement and audiometric masking could only be carried out in patients older than 10 years and showed non-reproducible results. In all cases with no major symptoms tinnitus disappeared. During a follow-up of 12-44 months, 4 cases treated with lidocaine achieved complete remission; in 3 cases the tinnitus eased off to such an extent that it was no longer regarded as annoying. In one girl of the lidocaine group a somatisation disorder developed independently of the tinnitus and was treated by psychotherapy. No side-effects of the lidocaine occurred. CONCLUSIONS: All aspects of chronic tinnitus in children and adolescents can be covered best when regarding this symptom as a psychosomatic disorder. The diagnostic approach in this age group has to include a detailed case history embracing both organic and psychological and social aspects. It should also include pure-tone and speech audiometry. Only in cases with an uncertain hearing threshold auditory evoked brainstem potentials have to be measured. Otoacoustic emissions give no further information about the development and therapeutic outcome of the tinnitus. In this age group tinnitus measurement and masking is of no diagnostic value. In patients with no signs of a hearing loss and no other organic symptoms there is no need for further diagnostic measures such as imaging or serological investigations. In cases with severe annoyance, tinnitus counselling is sufficient therapy. In cases with severe symptoms, lidocaine infusion therapy may be a therapeutic option for the somatic component of the disorder. In adolescents with chronic tinnitus psychotherapy will be necessary only in rare cases. The overall prognosis of this disorder is good.

Adolescent↗

PubMed1997

[Angelman syndrome: genetic defect with delayed language development].

Explore the source record for details and available documents.

Angelman Syndrome↗

PubMed1996

[Early indications for surgical vocal cord medial relocation in unilateral recurrent nerve paralysis in advanced age].

BACKGROUND: Unilateral vocal cord paralysis is usually treated conservatively. Phonosurgery is not indicated before 1 year after the onset of the palsy as during this period spontaneous recovery can be expected. In the elderly patient conservative voice rehabilitation is often limited by a restricted general condition. In these cases a severe communication disorder may result unless vocal cord function recovers spontaneously. PATIENTS AND METHODS: In 4 patients aged 68 to 79 years who had suffered from latrogenic unilateral vocal cord paralysis for 6 to 9 months, surgical medialisation of the paralysed vocal cord was carried out in local anaesthesia as an Isshiki-Type-I-operation after unsuccessful conservative treatment prior to surgery. RESULTS: In all cases a definite amelioration of voice function could be reached. There were no local or general complications. No spontaneous recovery of nerve function could be detected by electromyography between 6 to 9 months after the operation. Voice function remained stable during this period. CONCLUSIONS: We conclude that in elderly patients with unilateral vocal cord paralysis with no signs of spontaneous recovery and unsuccessful conservative treatment, surgical voice rehabilitation may be indicated before 1 year after the onset of the palsy.

Aged↗

PubMed1996

[Speech-specific cortical potentials--methodologic aspects and initial clinical results].

BACKGROUND: The purpose of this study was to find out whether specific cortical potentials can be evoked and identified after word stimulation. The clinical relevance was to be investigated in patients with aphasic syndromes. MATERIALS AND METHODS: In 20 young adults with no signs of hearing impairment and in patients with manifest aphasic syndromes, word-evoked cortical potentials were compared with those after an equivalent noise stimulus. The test words were selected from the Freiburger Speech Comprehension Test. The duration of the words was between 450 and 640 ms. The stimulus was presented monaurally. The peak level was 70 dB HL. The noise stimulus was produced by modifying a low-band noise. Potentials were measured between the ipsilateral mastoid and the contralateral forehead. Data were analysed offline. RESULTS: In healthy persons, the potentials after word and noise stimulation did not differ until 100 ms after the stimulus onset. After noise stimulation a negative maximum could be seen 100 ms after the stimulus onset, and a positive maximum 200 ms after the stimulus onset. After word stimulation, a positive maximum of higher amplitude than after noise stimulation was measured 150 ms after the stimulus onset, and a negative maximum was measured 270 ms after the stimulus onset. In all test persons the difference curve of word-and noise-evoked potentials revealed a speech-specific component 170 ms (N 170) after the stimulus onset. The single-word analysis showed that the potentials depend on the phonemes of the test word. The potentials do not alter when the stimulus side is changed. In patients with aphasia the potentials depend on the grade of the disturbance of speech perception: global and Wernicke's aphasia show no significant difference of speech-and noise-evoked potentials, whereas in Broca's aphasia a speech specific maximum is apparent. CONCLUSION: The speech-specific component may be regarded as a paradigm of cortical speech detection processes. Electrophysiological speech audiometry by means of word-evoked cortical potentials seems possible and may be used for clinical purposes.

Adult↗

PubMed1996

[In situ sound pressure measurement in a professional violinist with bilateral tinnitus].

BACKGROUND: A case of a professional violinist suffering from a bilateral tinnitus is presented. The musician reported the tinnitus to be louder and more straining when playing his Vuillaume violin (France 1840) as compared with his Carcassi violin (Italy 1763). CASE REPORT: In the 42-year-old musician, audiometry revealed a normal hearing threshold in the right ear and a slight hearing loss in the left ear of up to 20 dB between 2 kHz and 8 kHz. Transitory evoked otoacoustic emissions could only be measured in the right ear. The tinnitus could be masked (distance type); residual inhibition was only seen in the right ear. By sound intensity measurements in both external auditory canals, the different sound spectra of both violins could be demonstrated. When playing with "forte" intensity, sound pressure reached peaks of over 90 dB. The tinnitus was ameliorated by lidocaine infusions. DISCUSSION AND CONCLUSIONS: The different sound spectra of both violins may be the reason for the enhancement of the musician's tinnitus. Interference of air and bone-conducted sound could lead to a cochlear overlap and thus influence the tinnitus although such a phenomenon can not be verified. It was previously reported that high-pitched instruments may cause tinnitus sensations in performing musicians. A review of the literature surprisingly reveals that although professional musicians are exposed to sound pressure levels that may cause hearing impairment, only very few do develop one. This fact has to be taken into account whenever an occupational disease is suspected.

Adult↗

PubMed1996

[Juvenile xanthogranuloma of the auricle].

BACKGROUND: Juvenile xanthogranulomas are rare benign tumors of histiocytic origin with cutaneous manifestation predominantly in the head and neck. A case of a 12-month-old girl is presented. CASE REPORT: A tumor of the inferior crus of the left auricle had been noticed three weeks after birth. It was of yellowish color, bosselated and firm, and not mobile. During operation the tumor seemed lipomatoid and affected both cutis and subcutis. Histologically cutaneous structures were regular. Subcutaneously, cell-rich connective tissue with foamy histiocytes and Touton giant cells were observed as characteristic features of a juvenile xanthogranuloma. DISCUSSION: The pathogenesis of this tumor is unclear. The definite diagnosis is established by histological examination. Histological differentiation should include malignant tumors of histiocytic origin (dermatofibro-sarcoma protuberans, histiocytoma, atypical fibrous histiocytoma, fibro-xanthosarcoma) and malignant melanoma. Surgery is the treatment of choice only in cases in which the clinical diagnosis is doubtful, since juvenile xanthogranulomas usually heal spontaneously.

Diagnosis, Differential↗

PubMed1996

[Metastases of germ cell tumors to the ENT area--a rare differential diagnosis of lymph node metastases in unknown primary tumor].

BACKGROUND: Germ cell tumors of the testis usually metastasize to retroperitoneal lymph nodes, lungs, and liver. Other lymphatic or organ metastases especially in the head and neck are rare. CASE REPORTS: The cases of two men, ages 21 and 35, are presented. In both cases, the histologic examination of cervical lymph node specimen lead to the correct tumor diagnosis of testicular cancer. Multimodal urooncologic therapy led to long-lasting complete remissions in both cases. CONCLUSIONS: In young men between 20 and 35 years of age with cervical metastases of tumors of unknown primary site, germ cell tumors must be suspected. Histologic findings of non-squamous epithelial metastases (seminoma, embryonal carcinoma, chorionic carcinoma, teratoma, yolk sack tumor, and their combined forms) with or without retroperitoneal, mediastinal, or lung metastases; elevated tumor markers (human-chorionic gonadotropin, alpha-feto-protein, placental alkaline phosphatase, lactate dehydrogenase); and palpable intratesticular mass lead to the correct tumor diagnosis. Early detection of the disease is essential for successful therapy and long-term remission.

Adult↗

PubMed1995

[Multimodal carotid occlusion test for determining risk of infarct before therapeutic internal carotid artery occlusion].

UNLABELLED: In patients with head and neck carcinoma and extensive cervical metastasis, the topographic and functional relationship of the tumor to the carotid artery is highly important. In case of suspected carotid infiltration, the possibility of a carotid resection or a prosthetic replacement has to be considered preoperatively. Treatment of cavernous carotid aneurysms may also require sacrificing the internal carotid artery (ICA). An interdisciplinary test occlusion of the ICA was performed to assess cerebral collateral circulation prior to permanent carotid occlusion. MATERIALS AND METHODS: Fifty-two patients with cervical tumors (n = 45) or inoperable aneurysms (n = 7) were examined. The endovascular balloon test occlusion (BTO) of the ICA was combined with monitoring of the neurological status, cardiovascular status (EKG, blood pressure), cortical function (EEG), and single photon emission CT (SPECT) imaging of the regional cerebral blood flow (rCBF) with 99mTechnetium-HMPAO. In the last 24 patients, transcranial Doppler sonography (TCD) of the ipsilateral middle cerebral artery (MCA) was added for direct hemodynamic monitoring during BTO. In order to improve the diagnostic value of the test results and to simulate hemodynamic crisis, the cerebrovascular reserve capacity was then evaluated with acetazolamide (Diamox). RESULTS: BTO could be performed without neurological complications or carotid dissection. In eight (15%) patients BTO had to be interrupted previously due to neurological symptoms or a delta-EEG. These patients and patients with highly pathological test results in SPECT imaging (n = 9) or TCD (n = 3) were excluded from permanent carotid occlusion. Ten (19%) patients were definitely occluded without hemodynamic complications, but two patients suffered embolic infarctions, which can not be predicted by this procedure. In two patients with a severe hypoperfusion in SPECT imaging, the ICA had to be ligated under emergency conditions following a carotid rupture. Predictably, a hemodynamic infarction occurred postoperatively in both patients. CONCLUSIONS: The multimodal BTO with brain perfusion imaging (HMPAO-SPECT) and quantitative blood flow monitoring (TCD) allows a hemodynamic stroke risk assessment prior to permanent occlusion of the ICA. The procedure is important for planning of the therapeutic strategy and for the preoperative dialogue with the patient. Embolic ischemic complications can not be predicted.

Adult↗

PubMed1995

[Familial bilateral atresia of the auditory canal with hypertelorism].

The incidence of malformations of the ear is 1:5000, the incidence of stenosis and atresia is 1:12,000. 30% of the malformations are bilateral. In only 0.3% it is the only malformation. Inherited bilateral atresia of the external auditory canal associated with hypertelorism as the only additional malformation has not yet been described. Basing on a case report the way of interdisciplinary rehabilitation of children affected by bilateral hearing impairment is discussed. Special emphasis is on the discussion of the necessity of radiological examinations for the exclusion of cholesteatoma in an atretic ear which could be found in a 5-year old boy of the described family.

Child, Preschool↗

PubMed1995

[Esophagomediastinal fistula and recurrent laryngeal nerve paralysis after radiotherapy of Hodgkin's disease].

The case of a female patient 21 years old at the time of diagnosis is reported. The patient suffered from stage IV Hodgkin's disease of the nodular sclerotic type with head and neck manifestations in cervical lymph nodes and in the esophagus. After radiotherapy; she suffered from an esophageal/mediastinal fistula and unilateral paralysis of the recurrent laryngeal nerve. Fifteen years later, there are no signs of recurrent tumor growth or a secondary neoplasm. Dysphonia was ameliorated by speech therapy, and surgery was not necessary.

Adult↗