Intratympanic gentamicin therapy for vertigo in nonserviceable ears.

PURPOSE: Intratympanic ototoxic agents have become a widely accepted means of managing vertigo in patients with Meniere's disease while preserving residual hearing. We investigated expanding the indications for intratympanic gentamicin to include control of vertigo in patients without serviceable hearing in the involved ear caused by a variety of end-organ pathologies. MATERIALS AND METHODS: We present a retrospective series of 6 patients suffering from vertigo caused by end-organ disease, in an ear without serviceable hearing. Two patients suffered from delayed endolymphatic hydrops, 3 from Meniere's disease, and 1 from poststapedectomy vertigo. These patients chose unilateral vestibular ablation with serial intratympanic gentamicin injection rather than labyrinthectomy for a variety of reasons. Conventional electronystagmography (ENG) testing and audiometry were completed on all patients. The ENG testing included bithermal calorics and rotational testing. All patients had a magnetic resonance image with gadolinium to exclude retrocochlear or central pathology. Rotational testing was repeated before each injection and at the conclusion of therapy to assess changes in the peripheral vestibular response. The patients' subjective response to therapy was followed. RESULTS: Follow-up has been 10 to 69 months with successful control of vertigo in all patients. CONCLUSIONS: Intratympanic gentamicin therapy offers a minimally invasive, ambulatory, low morbidity, cost-effective means of managing vertigo in patients with nonserviceable hearing.

Otologic findings in a pediatric cohort with sickle cell disease.

OBJECTIVE: Sensorineural hearing loss (SNHL) has been associated with sickle cell disease (SCD) in older children and adults. Few studies have investigated this association in younger children. DESIGN: In a cohort of pediatric patients followed at our sickle cell clinic, with a mean age of 7.8 years, 154 audiograms were completed on 84 patients. RESULTS: This produced 22 subjects with abnormal audiograms. Middle ear effusion was the etiology of 19 subjects' audiometric findings. Three children were found to have mild SNHL. None of the subjects had hearing loss of severity which would warrant amplification. The prevalence rate for SNHL in our cohort of pediatric patients was 3.5%. CONCLUSION: This differs from past reports, and may reflect an age dependent prevalence of SNHL in this disease.

Congenital stapes ankylosis, broad thumbs, and hyperopia: report of a family and refinement of a syndrome.

We report on a family with conductive hearing loss due to congenital stapes ankylosis, and with hyperopia, broad thumbs, and broad first toes. Neither of the studied relatives had symphalangism, possibly distinguishing this syndrome as an entity separate from the facio-audio-symphalangism and proximal symphalangism syndromes. An alternative possibility is that this family falls within the spectrum of the facioaudio-symphalangism and proximal symphalangism syndromes. Visualization of the ossicular chain, and ophthalmologic and radiologic studies are important in the evaluation of families with congenital conductive hearing loss. A characteristic physiognomy in our patients is present; this autosomal dominant syndrome was first described by Teunissen and Cremers [1990: Laryngoscope 100:380-384].

Spontaneous labyrinthine hemorrhage in sickle cell disease.

We report the clinical and MR imaging findings in two African-American patients with manifestations of sickle cell disease affecting the inner ear. Both suffered sudden-onset sensorineural hearing loss and vestibular symptoms, and both had high labyrinthine signal on T1-weighted MR images attributed to labyrinthine hemorrhage. Follow-up studies of the first patient revealed a decrease in abnormal vestibular signal. Careful attention to the labyrinth on T1-weighted MR images can reveal vestibulocochlear clinical findings in sickle cell patients, with important implications for management and prognosis.

Bizarre "sawtooth" tympanogram in a patient with otitis media.

A patient evaluated for otitis media had a bizarre tympanogram with a "sawtooth" configuration. Acute otitis media with a pinpoint perforation was diagnosed. After resolution of the acute infection, a 2-mm diameter perforation remained and the presence of a patulous eustachian tube was documented. Tympanoplasty was eventually required, after which a normal tympanogram was recorded. Our interpretation of the tympanogram is discussed and the literature reviewed.

Historic perspective of the acoustic otoscope.

The acoustic otoscope, originally called the acoustic reflectometer, was developed and produced by John and David Teele in the early 1980s. Since initial production, two different instrument versions have been developed by two separate companies. During the period of time in which the acoustic otoscope has been in production, there have been numerous studies reported with the two instrument versions. We provide a historic summary of the acoustic otoscope, summarize the pertinent studies, and address the contrasting results found in the literature.

Utility of EMLA in the relief of pain associated with electroneurography.

Electroneurography (ENoG) is an electrodiagnostic procedure used to evaluate facial nerve deficit. A problem with application, however, is pain. Several studies have demonstrated that topical application of the eutectic mixture of lidocaine and prilocaine has an anesthetic effect on superficial and deep tissues. The present study was intended to determine if successful anesthesia of the trigeminal nerve could be achieved without altering the impulse transmission on the facial nerve. ENoG responses were measured at stimulation levels of 8, 16, 24, and 30 to 40 ma before and after Eutectic Mixture of Local Anesthetic (EMLA) application. A significant reduction in pain sensation was observed at stimulation levels of 8 and 16 ma while the amplitude of the response was not significantly changed. At the higher stimulation levels, namely, 24 and 30 to 40 ma, no significant reduction in pain or amplitude was observed. We concluded that, at moderate stimulation levels (> or =16 ma), EMLA reduces the pain associated with ENoG testing without affecting the amplitude of the muscular response.

Inner ear volumetric measurements using high-resolution 3D T2-weighted fast spin-echo MR imaging: initial experience in healthy subjects.

BACKGROUND AND PURPOSE: Adult size is achieved in the inner ear labyrinth by approximately 25 weeks' gestation, and minimal variability in age, sex, side, and race is found after birth. In this study, we opted to determine the reproducibility of inner ear volumetric measurements generated from high-resolution heavily T2-weighted 3D fast spin-echo MR images. METHODS: The temporal bones of 23 volunteers were imaged using a heavily T2-weighted 3D fast spin-echo MR imaging technique. The images were assessed by a neuroradiologist for the presence of inner ear configurational anomalies and, most important, for complete coverage of the inner ear labyrinth. Subsequently, the volume of the fluid in the inner ear was determined by two observers using a semiautomated segmentation algorithm. The mean, SD, range, and coefficient of variation of fluid volume in the inner ear were calculated. Age-, sex-, and side-related differences in the inner ear volumetric measurements were evaluated using analysis of variance. Interrater consistency in the inner ear volumetric measurements was evaluated by comparing the calculated coefficients of reliability. RESULTS: Volumetric measurements were available from 46 inner ears in 23 volunteers. The mean volume was 227.8 mm3 (SD, 24.4 mm3), and the coefficient of variation was 10.7%. No age-, sex-, or side-related differences in the inner ear volumetric measurements were found (F ratios were 4.33, 5.04, and 0.26, respectively). Interrater consistency, as assessed by the coefficient of reliability, was 5.3%. CONCLUSION: Reproducible volumetric measurements of the inner ear labyrinth can be obtained by applying a semiautomated segmentation algorithm to a heavily T2-weighted 3D fast spin-echo MR imaging data set. These volumetric measurements may help identify patients with congenital sensorineural hearing loss and normal inner ear configuration.

An approach to imaging the dizzy patient.

Disease of the vestibular system involves a lengthy differential diagnosis, from the relatively trivial to potentially life-threatening conditions. As a definitive specific diagnosis is often difficult to make, imaging may be used simply to "rule out" serious pathology. In this context, suboptimal imaging choices may be unnecessarily expensive and may fail to document the pathology. In contrast, an orderly investigation includes appropriate audiometric and vestibular tests, which when reviewed in the clinical context, indicate the most pertinent imaging modalities. This enables the clinician to plan a cost-effective, definitive imaging strategy. This review considers the role of imaging in an integrated approach to investigating the dizzy patient. A discussion of vestibular pathology is presented to assist in the review, and several cases are provided as examples.

Use of acoustic reflectometry in the detection of middle ear effusion.

The purpose of the present investigation was to compare the performance of the acoustic otoscope/DPU-411 printer and acoustic immittance with otoscopic examinations by a physician for the detection of middle ear effusion (MEE). Three hundred and two patients (11 months to 69 years) were evaluated with the acoustic otoscope, acoustic immittance, and otoscopic examinations. The patients were divided into two age groups for data analysis: 1 to 12 years (children) and 13 to 69 years (adults). Reflectivity and angle data were evaluated at different cut-off points and receiver operating characteristics (ROC) curves were determined. The variables were analyzed in different combinations for each study group. Sensitivity and specificity were poorest for the acoustic otoscope in children. Furthermore, acoustic immittance data compared more favorably with otoscopy than did the acoustic otoscope for all ages.

Large vestibular aqueduct syndrome: a tutorial and three case studies.

The large vestibular aqueduct (LVA) syndrome is a congenital malformation that predisposes the patient ultimately to a loss of hearing and possible continuing vestibular disorder. If the LVA patient is diagnosed, it typically is not until later life, when he/she exhibits profound sensorineural hearing loss. To better understand this disorder and to bring it to the attention of audiologists, we provide a brief tutorial of LVA and present three case studies that illustrate the syndrome.

Patient with audible popping sounds emanating from the ear canal.

We describe a most unusual case of a 40-year-old male who presented with audible popping sounds emanating from his left ear canal. The process of identifying the source of the sounds and treating the underlying cause is presented in detail.