Brainstem hemorrhage presented as audiovestibular syndromes.

Brainstem hemorrhage usually presented with acute multiple neurologic dysfunction, and the prognosis was poor. Rarely, it can manifest with audiovestibular symptoms only. Here, we report a case of brainstem hemorrhage involving the right middle cerebellar peduncle and dorsal lateral pons presented with constant nonpulsatile tinnitus and rotatory vertigo. We believed that rotatory nystagmus should be regarded as a central sign until proven otherwise even if the neurologic signs are subtle.

Treatments and outcomes of malignant tumors of external auditory canal.

PURPOSE: Primary cancer of the external auditory canal (EAC) is a rare disease with poor prognosis. Because of the rarity of this entity, there is no large series for staging, treatments, and outcomes. The purpose of this study is to evaluate the treatment modalities and outcomes for malignant tumors of EAC at our institute, comparing with other studies. METHODS: We retrospectively reviewed the patients treated for primary cancer of EAC between 1991 and 2002 at our hospital, which is a tertiary referral center. Ten males and 2 females were enrolled in this study. The median follow-up period was 56.5 months. The interventions included sleeve resection, radical mastoidectomy, or lateral temporal bone resection combined with neck dissection or parotidectomy. Adjuvant radiotherapy was performed in case of incomplete resection. All patients were staged according to the Pittsburgh staging system. RESULTS: Ten patients had squamous cell carcinoma and 2 had adenoid cystic carcinoma (ACC). Five patients had stage I disease, 2 with stage II, 1 with stage III, and 4 with stage IV. All patients (n = 7) with early stage (I or II) were disease-free, but only 1 of 5 patients with late stage (III or IV) was disease-free. The recurrences occurred in 50% (2/4) of patients with incomplete resection, despite of the adjuvant radiotherapy. Only one patient with complete resection had recurrence and the histologic diagnosis of this particular patient was ACC. CONCLUSIONS: Patients with early-stage cancer can benefit from less aggressive surgical interventions without significant morbidity or mortality. No recurrence occurred in patients with complete resection except the one with ACC. The result of our institute was comparable or superior to those of other studies in early-stage disease. However, patients with advanced cancer had high recurrence rate despite of adjuvant radiotherapy, suggesting more aggressive surgical approach for complete resection was necessary.

A novel mutation in the WFS1 gene identified in a Taiwanese family with low-frequency hearing impairment.

BACKGROUND: Wolfram syndrome gene 1 (WFS1) accounts for most of the familial nonsyndromic low-frequency sensorineural hearing loss (LFSNHL) which is characterized by sensorineural hearing losses equal to and below 2000 Hz. The current study aimed to contribute to our understanding of the molecular basis of LFSNHL in an affected Taiwanese family. METHODS: The Taiwanese family with LFSNHL was phenotypically characterized using audiologic examination and pedigree analysis. Genetic characterization was performed by direct sequencing of WFS1 and mutation analysis. RESULTS: Pure tone audiometry confirmed that the family members affected with LFSNHL had a bilateral sensorineural hearing loss equal to or below 2000 Hz. The hearing loss threshold of the affected members showed no progression, a characteristic that was consistent with a mutation in the WFS1 gene located in the DFNA6/14/38 locus. Pedigree analysis showed a hereditarily autosomal dominant pattern characterized by a full penetrance. Among several polymorphisms, a missense mutation Y669H (2005T>C) in exon 8 of WFS1 was identified in members of a Taiwanese family diagnosed with LFSNHL but not in any of the control subjects. CONCLUSION: We discovered a novel heterozygous missense mutation in exon 8 of WFS1 (i.e., Y669H) which is likely responsible for the LFSNHL phenotype in this particular Taiwanese family.

reducing false positives in newborn hearing screening program: how and why.

OBJECTIVE: To compare the initial referral rate, the accurate identification rate of congenital hearing loss, and the cost between one step with transient evoked otoacoustic emissions (TEOAEs), two steps with TEOAE and automated auditory brainstem response (AABR), and one step with AABR in newborn hearing screening program. The aim of this study is to compare their efficacy between our three different protocols and to see which one is most cost-effective. STUDY DESIGN: From November 1998 to April 2006, 25,588 healthy newborns were screened for hearing loss in Mackay Memorial Hospital, Taipei. In the periods from November 1998 to January 2004, from February 2004 to February 2005, and from March 2005 to April 2006, the screening tools used were TEOAE alone (n = 18,260), TEOAE plus AABR (n = 3,540), and AABR (n = 3,788), respectively. RESULTS: A statistically significant decrease in referral rate was achieved in the group using AABR as screening tools when compared with TEOAE plus AABR and TEOAE alone (0.8 versus 1.6 versus 5.8%). The accurate identification rate of congenital hearing loss was 0.42% in AABR protocol, 0.25% in TEOAE and AABR protocol, and 0.45% in TEOAE protocol, which was not statistically significant. The total direct costs (including predischarge screening and postdischarge follow-up costs) per screening were US $10.04 for the program using TEOAE alone, US $8.60 for TEOAE plus AABR, and US $7.33 for AABR. The intangible cost (parental anxiety) was much higher in the earlier program due to higher referral rate. CONCLUSION: In the efficacy of the hearing screening program using the one-step TEOAE, two-step TEOAE and AABR, and one-step AABR programs, the latter significantly decreased the referral rate from 5.8, to 1.6, and to 0.8%. No significant difference was noted between their accurate identification rates of congenital hearing loss. The total costs, including expenditures and intangible cost, were much lower in the protocol with AABR due to reduction in false positives.

Comparison of hearing screening programs between one step with transient evoked otoacoustic emissions (TEOAE) and two steps with TEOAE and automated auditory brainstem response.

OBJECTIVE: To compare the efficacy between one step with transient evoked otoacoustic emissions (TEOAE) and two steps with TEOAE and automated auditory brainstem response (AABR) in a newborn hearing screening program. We investigated their differences in referral rate, the accurate identification rate of congenital hearing loss (HL), and cost effectiveness. METHOD: From November 1998 to December 2004, a total of 21,273 healthy newborns were screened for HL in Mackay Memorial Hospital, Taipei. In the periods from November 1998 to January 2004 and from February 2004 to December 2004, the screening tools used were TEOAE alone (n = 18,260) and TEOAE plus AABR (n = 3,013), respectively. RESULTS: A statistically significant decrease of referral rate was achieved in the group using TEOAE and AABR as screening tools when compared with TEOAE alone (1.8% vs. 5.8%). The accurate identification rate of congenital HL was 0.45% in TEOAE protocol and 0.3% in TEOAE and AABR protocol, which was not statistically significant. The total direct costs per screening were 10.1 U.S. dollars for the program using TEOAE alone and 8.9 U.S. dollars for the TEOAE plus AABR program. The intangible cost, however, was much higher in the earlier program because of the higher referral rate. CONCLUSION: In terms of the efficacy of a hearing screening program using the one step TEOAE and two step TEOAE and AABR programs, the later significantly decreased the referral rate from 5.8% to 1.8%. No significant difference was noted between the accurate identification rates of congenital HL. The total costs, including expenditures and intangible cost, were lower in the protocol with TEOAE plus AABR.

Effectiveness of intratympanic dexamethasone injection in sudden-deafness patients as salvage treatment.

OBJECTIVE: To study the effectiveness of intratympanic dexamethasone (IT-DEX) in patients with severe or profound sudden sensorineural hearing loss (SSNHL) after treatment failure with conventional therapy. STUDY DESIGN: Randomized, controlled study. METHODS: Patients who met the criteria for SSNHL, with a severity of severe to profound, underwent 10 days of standard treatment with oral steroid and other facilitating agents. Patients showing poor response to standard treatment were assigned randomly to a control group or to a group receiving IT-DEX. IT-DEX injections were performed once a week for 3 consecutive weeks. Pure-tone audiometry was obtained before each injection. Minimum follow-up time was 1 month. Successful treatment was defined as a hearing improvement of greater than 30 dB. RESULTS: Thirty-nine patients meeting the inclusion criteria were studied. After treatment with oral steroid, 10 of 39 (26%) patients demonstrated hearing improvement, whereas the remaining 29 (74%) patients showed a hearing improvement of less than 30 dB. For those without hearing improvement, 15 received IT-DEX, and 14 received further standard treatment (except oral steroid and carbogen inhalation). Hearing improved in 8 of 15 (53.3%) compared with 1 of 14 (7.1%), with an average decrease in threshold of 28.4 dB and 13.2 dB for the IT-DEX group and the control group, respectively (P <.05). Prognostic factors such as age, treatment delay time, and sex did not significantly affect the response to therapy. CONCLUSIONS: IT-DEX injection effectively improves hearing in patients with severe or profound SSNHL after treatment failure with standard therapy and is not associated with major side effects. It is therefore a reasonable alternative as salvage treatment.

A universal newborn hearing screening program in Taiwan.

OBJECTIVE: Mackay Memorial Hospital and the Children's Hearing Foundation established a pilot universal newborn hearing screening program in November 1998. Our objective was to assess the feasibility, accuracy and cost effectiveness of implementing universal newborn hearing screening in Taiwan. METHOD: Between November 1998 and October 2000 a total of 6765 newborns were screened for hearing loss prior to discharge from the wellborn nursery at Mackay Memorial Hospital. The average age of the subjects at the initial screening test was 52 h. The program employed a three stage hearing screening protocol using transient evoked otoacoustic emmisions (TEOAE) screening with referral for diagnostic auditory brainstem response assessment. RESULTS: The mean TEOAE screening time per ear was 41.43 s. The overall pass rate at the time of hospital discharge was 93.6%. Thus achieving an acceptable referral rate of 6.4% for diagnostic audiological assessments. Nine newborns were identified with permanent bilateral hearing impairment. 26 newborns were identified with permanent unilateral hearing impairment. Infants identified with bilateral hearing loss were immediately referred to the Children's Hearing Foundation for hearing aid assessment and fitting. Infants as young as 5 weeks of age were successfully fitted with hearing instruments and enrolled in the family centered early intervention program at the Children's Hearing Foundation. CONCLUSION: The frequency of bilateral congenital hearing loss requiring amplification in this population is shown to be approximately 1 in 752 newborns. This finding is consistent with previous research, which has indicated hearing loss to be the most frequently occurring birth defect. Universal newborn hearing screening using TEOAEs proved to be a cost effective and feasible method of identifying congenital hearing loss in Taiwan. The existence of many successful screening programs worldwide and the availability of fast, objective, reliable and inexpensive hearing screening procedures means that universal newborn hearing screening is becoming the standard of care.

Basal cell carcinoma of the external auditory canal.

Basal cell carcinomas of the EAC are known to be locally aggressive, although they are not associated with regional lymph node metastasis.

Acoustic characteristics of different target vowels during the laryngeal telescopy.

OBJECTIVE: The aim of this study was to investigate the acoustic characteristics of target vowels phonated in normal voice persons while performing laryngeal telescopy. The acoustic characteristics are compared to show the extent of possible difference to speculate their impact on phonation function. METHODS: Thirty-four male subjects aged 20-39 years with normal voice were included in this study. The target vowels were /i/ and /ɛ/. Recording of voice samples was done under natural phonation and during laryngeal telescopy. The acoustic analysis included the parameters of fundamental frequency, jitter, shimmer and noise-to-harmonic ratio. RESULTS: The sound of a target vowel /ɛ/ was perceived identical in more than 90% of the subjects by the examiner and speech language pathologist during the telescopy. Both /i/ and /ɛ/ sounds showed significant difference when compared with the results under natural phonation. There was no significant difference between /i/ and /ɛ/ during the telescopy. CONCLUSION: The present study showed that change in target vowels during laryngeal telescopy makes no significant difference in the acoustic characteristics. The results may lead to the speculation that the phonation mechanism was not affected significantly by different vowels during the telescopy. This study may suggest that in the principle of comfortable phonation, introduction of the target vowels /i/ and /ɛ/ is practical.

Otomastoiditis with acute left facial nerve paralysis caused by Mycobacterium chelonae.

Mycobacterium chelonae usually causes soft-tissue and skin infection. It is a rare cause of otomastoiditis. The clinical presentation and operative findings are similar to those of other nontuberculous mycobacterial infections. We describe a case of left-sided otomastoiditis with acute facial nerve paralysis caused by this organism in a previously well middle-aged woman. Her facial palsy totally resolved after tympanomastoidectomy plus a 7-week regimen of clarithromycin and moxifloxacin. To our knowledge, a case of otomastoiditis with acute facial nerve paralysis caused by M chelonae has not been reported previously.

Cholesterol granuloma of the external ear canal: A rare presentation.

Almost all aural cholesterol granulomas develop in the mucosa of the middle ear. We describe the case of a 14-year-old girl who presented with an asymptomatic, nontender, dark-blue, cystic lesion in the posteroinferior portion of the left ear canal. The mass was excised via a postauricular approach. Postoperatively, the mass was identified as a cholesterol granuloma on pathologic examination. To the best of our knowledge, this is the first reported case of a human cholesterol granuloma limited to the external ear canal.

High-dose intratympanic gentamicin instillations for treatment of Meniere's disease: long-term results.

CONCLUSIONS: Administration of high-dose gentamicin for intractable Meniere's disease appears to be effective in achieving long-term control of vertigo. However, the safety of this route of administration with respect to the patient's hearing has not yet been sufficiently established. OBJECTIVES: The study aimed to analyze the long-term results of patients receiving high-dose intratympanic gentamicin (ITG) instillation for refractory Meniere's disease. PATIENTS AND METHODS: Fourteen patients with Meniere's disease according to 1995 American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines who had failed medical (12 subjects) or surgical (2 subjects) treatment were included. Intratympanic injections of 27 mg/ml gentamicin were performed three times daily for 4 days. Vertigo control, the patients' functional level, and their hearing threshold were all analyzed. Criteria described in 1995 by AAO-HNS were used. RESULTS: The overall successful vertigo control rate was 92.9% over the 2-year follow-up and 85.7% at long-term follow-up (average 10 years). Hearing level as pure-tone average was worse in four patients (28.5%) after 2 years follow-up and in six patients (42.8%) after long-term follow-up, respectively. Profound sensorineural hearing loss occurred as a result of gentamicin injection in one patient (7%).