Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.

Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls (P = 0.001); half were previously unknown (AF < 0.000002). MYO6, MYO7A, PTPRQ, and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1N321I/+ mice, carrying the TMC1:p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy.

The Oticon Medical Neuro Zti cochlear implant and the Neuro 2 sound processor: multicentric evaluation of outcomes in adults and children.

Objective: This study evaluated the outcomes of the Oticon Medical Neuro Zti cochlear implant and the Neuro 2 sound processor.Design: Neuro One users were upgraded to Neuro 2. Monosyllabic word identification was evaluated in adults with Neuro One after ≥5 months, with Neuro 2 at upgrade, and with Neuro 2 after 3 months. Self-reported listening ability, satisfaction, and usability were measured in adults and children.Study sample: Participants were 44 adults and 26 children.Results: Speech identification scores in quiet and noise were 58% and 45% with Neuro One and 67% and 55% with Neuro 2 after 3 months, respectively. Hearing impairment duration and number of active electrodes significantly predicted speech identification in noise with Neuro 2. Significantly higher questionnaire ratings were obtained for Neuro 2 than Neuro One regarding listening ability in complex listening situations, comfort and music, as well as nine aspects of satisfaction and usability.Conclusion: This study demonstrates the clinical superiority of the Neuro 2 sound processor over Neuro One in terms of speech identification in quiet and in noise and reported patient benefit and satisfaction. Given the study design, sources of improvement may include factors unrelated to the sound processor itself.

Transtympanic pseudoaneurysm of the internal carotid artery complicating a myringotomy in a four-year old child: Case report and literature review.

We report the first case of a transtympanic iatrogenic internal carotid artery (ICA) pseudoaneurysm diagnosed in a 4-year-old child following a myringotomy. An endovascular treatment with a covered-stent was decided; spontaneous thrombosis was found during the therapeutic arteriography, and the procedure was aborted. Otoscopy and computed tomography (CT) scan monitoring showed a prolonged thrombosis and the disappearance of the pseudoaneurysm 18months after the diagnostic arteriography. Based on literature review, endovascular techniques seem to be preferred to the surgical approach for treatment of intrapetrous ICA pseudoaneurysm, however clinical and CT scan monitoring may also be a valid option.

Germline mutations in FH confer predisposition to malignant pheochromocytomas and paragangliomas.

Malignant pheochromocytoma (PCC) and paraganglioma (PGL) are mostly caused by germline mutations of SDHB, encoding a subunit of succinate dehydrogenase. Using whole-exome sequencing, we recently identified a mutation in the FH gene encoding fumarate hydratase, in a PCC with an 'SDH-like' molecular phenotype. Here, we investigated the role of FH in PCC/PGL predisposition, by screening for germline FH mutations in a large international cohort of patients. We screened 598 patients with PCC/PGL without mutations in known PCC/PGL susceptibility genes. We searched for FH germline mutations and large deletions, by direct sequencing and multiplex ligation-dependent probe amplification methods. Global alterations in DNA methylation and protein succination were assessed by immunohistochemical staining for 5-hydroxymethylcytosine (5-hmC) and S-(2-succinyl) cysteine (2SC), respectively. We identified five pathogenic germline FH mutations (four missense and one splice mutation) in five patients. Somatic inactivation of the second allele, resulting in a loss of fumarate hydratase activity, was demonstrated in tumors with FH mutations. Low tumor levels of 5-hmC, resembling those in SDHB-deficient tumors, and positive 2SC staining were detected in tumors with FH mutations. Clinically, metastatic phenotype (P = 0.007) and multiple tumors (P = 0.02) were significantly more frequent in patients with FH mutations than those without such mutations. This study reveals a new role for FH in susceptibility to malignant and/or multiple PCC/PGL. Remarkably, FH-deficient PCC/PGLs display the same pattern of epigenetic deregulation as SDHB-mutated malignant PCC/PGL. Therefore, we propose that mutation screening for FH should be included in PCC/PGL genetic testing, at least for tumors with malignant behavior.

Long-term results of middle fossa plugging of superior semicircular canal dehiscences: clinically and instrumentally demonstrated efficiency in a retrospective series of 16 ears.

The objective of this study is to report the surgical outcome after middle fossa approach (MFA) plugging in patients suffering from a superior semi-circular canal dehiscence (SCD) syndrome. This is a retrospective case review. Tertiary referral center. Sixteen ears in 13 patients with a SCD syndrome suffering from severe and disabling vestibular symptoms with a bony dehiscence on CT scan >3 mm and decreased threshold of cervical vestibular evoked potentials (cVEMPs). We assessed preoperatively: clinical symptoms, hearing, cVEMPs threshold, size of dehiscence and videonystagmography (VNG) with caloric and 100 Hz vibratory tests. Postoperatively, we noted occurrences of neurosurgical complication, evolution of audiological and vestibular symptoms, and evaluation of cVEMP data. Tullio's phenomenon was observed in 13 cases (81.3 %) and subjectively reported hearing loss in seven (43.7 %). All patients were so disabled that they had to stop working. No neurosurgical complications were observed in the postoperative course. In three cases (16.6 %), an ipsilateral and transitory immediate postoperative vestibular deficit associated with a sensorineural hearing loss (SNHL) was noted, which totally resolved with steroids and bed rest. All patients were relieved of audiological and vestibular symptoms and could return to normal activity with a mean follow-up of 31.1 months (range 3-95). No patient had residual SNHL. cVEMPs were performed in 14 ears postoperatively and were normalized in 12 (85.7 %). Two of the three patients operated on both sides kept some degree of unsteadiness and oscillopsia. MFA plugging of the superior semi-circular canal is an efficient and non-hearing deteriorating procedure.

Lipochoristomas of the cerebellopontine angle and internal acoustic meatus: a seven-case review.

BACKGROUND: Intracranial lipomas are extremely rare tumors, with certain clinical and radiological characteristics. METHODS: We considered the diagnostic evaluation and treatment options of seven patients presenting with lipoma in the cerebellopontine angle or internal acoustic meatus in our department. RESULTS: Mean age was 51, with four out of seven cases being women. Balance disorders (vertigo, dizziness) were the predominant symptoms (in six out of seven patients), followed by hearing loss. The diagnosis of intracranial lipomas was based on the results of imaging studies, especially on magnetic resonance imaging. The management of lipomas of the CPA and of the IAM should initially be conservative, including close follow-up of the patient and evaluation of a potential change in tumor size. Surgical management of CPA lipomas should be reserved for patients with intractable clinical symptoms due to tumor overgrowth. CONCLUSIONS: Diagnosis of CPA and IAM lipomas is considered to be a clinical and imaging challenge. As the complete resection of such lipomas is a risky choice and taking into account the benign course of such tumors, the only absolute surgical indication should be uncontrolled tumor growth.

Endolymphatic sac tumors: experience of three cases.

OBJECTIVES: To describe the clinical features, radiological findings, treatment and outcomes of three cases of endolymphatic sac tumors (ELST). METHODS: Retrospective analysis of three cases of ELST. RESULTS: The first patient had a large ELST invading the labyrinth after a long history of vertigo. He was recurrence-free 1 year after retrolabyrinthine surgical removal. In the second case, an acute peripheral facial nerve paralysis associated with ipsilateral sensorineural hearing loss led to the diagnosis. A translabyrinthine approach was used to remove the tumor, which recurred three times over 10 years. The third patient was a young woman suffering from von Hippel-Lindau (VHL) disease and referred for a sudden sensorineural hearing loss due to an intralabyrinthine hemorrhage secondary to a 2 mm-large endolymphatic sac-confined ELST. Her hearing was totally lost after the deafness recurred 1 month after this first episode. MRI demonstrated a small bilateral ELST. The patient refused surgery on the deaf side. CONCLUSION: ELST are difficult to diagnose due to the wide variety of their presentations. Patients with ELST should be screened for VHL disease. Dural invasion and tumor hypervascularization increase the risk of local recurrences after surgery. Early surgical resection may lead to complete tumor removal and inner ear preservation.

Anatomo-radiological study of the superior semicircular canal dehiscence of 37 cadaver temporal bones.

PURPOSE: To assess the presence of dehiscence of the superior semicircular canal (SSCC) on computed tomography (CT) scanning and to study the microscopic anatomo-radiological correlation. MATERIALS AND METHODS: Thirty-seven temporal bones preserved in formalin, regardless of the clinical history of cadavers, were studied. A microscopic anatomical study was conducted with an operative microscope (20×). The settings of the CT permitted to obtain 0.6 mm slices contiguous reconstruction in Pöschl plane and in Stenvers plane. Three-dimensional (3D) reconstructions were performed if a radiological dehiscence was observed. The apex thickness was measured in Pöschl plane. The radiological positive criterion of SSCC dehiscence was an absence of bone coverage of more than 1 mm long in Pöschl and Stenvers planes. RESULTS: We observed three dehiscences of the 37 temporal bones on CT in Pöschl and Stenvers planes. However, no dehiscence was found microscopically. The 3D reconstruction was also positive in these three cases. Reconstructions in the Pöschl plane offered good results up to a bone thickness of 0.6 mm. When it was lower than 0.6 mm, the interpretation of the images appeared to be subjective. CONCLUSION: This study emphasizes the limitations of CT imaging, with a risk of false positives to take into account when interpreting the images. The 3D reconstructions also give too many false positives to be used alone and make an accurate diagnosis. The diagnosis of SSCC dehiscence will therefore remain clinical. Complementary and instrumental radiological examinations should be performed only to confirm this clinical suspicion.

Unique case of malignant transformation of a vestibular schwannoma after fractionated radiotherapy.

OBJECTIVE: Malignant transformation of vestibular schwannoma is considered a rare clinical entity. Radiotherapy, as a treatment option for vestibular schwannoma, is regarded as a potential risk factor for secondary malignancy. Recently, radiotherapy with dose fractionation has been proposed, intended to diminish the risk of radiation-induced neuropathy. CASE PRESENTATION: The aim of the present study is to report the first case, to the best of our knowledge, of malignant transformation of a residual vestibular schwannoma 19 years after fractionated radiotherapy, describing its characteristics with regard to those previously reported in the literature. CONCLUSIONS: The main purpose of the present work is to state that the knowledge of the iatrogenic potential pitfalls of any technique of radiotherapy in clinical oncology is becoming a necessity. Finally, our report demonstrates that the irradiated patients must be monitored for life because a secondary malignancy may appear after a very long delay.

Spontaneous osteodural defects of the temporal bone: diagnosis and management of 12 cases.

OBJECTIVES: The objectives of the study were to describe the clinical presentation, diagnostic process, surgical treatment, and outcome of patients with spontaneous dural defect of temporal bone and to explore the possible contribution of idiopathic intracranial hypertension. METHODS: Medical records of consecutive patients with spontaneous defects of the temporal bone were reviewed. Clinical presentation, diagnostic process, exploration of benign intracranial hypertension, surgical management, and outcome of patients are presented. RESULTS: Six of the 12 patients presenting with spontaneous cerebrospinal fluid otorrhea were women. Ages ranged from 38 to 76 years. Seven patients presented with meningitis. The location and the extent of the occurring defect were detected by computed tomography in all cases. Radiologic signs of empty sella syndrome, indicator of benign intracranial hypertension, were revealed in 3 cases using magnetic resonance imaging. Six tegmen defects were repaired using a middle fossa approach without recurrence. Four patients received the combined approach. All patients had complete resolution of the cerebrospinal fluid leak, although 2 cases developed adverse effects attributable to surgical procedure. CONCLUSION: The diagnosis of spontaneous cerebrospinal fluid otorrhea requires clinical suspicion in the setting of meningitis and persistent serous otitis media. High-resolution computed tomography can confirm the diagnosis. The authors' findings advocate the multilayered closure technique through a middle fossa approach.

One Year Assessment of the Hearing Preservation Potential of the EVO Electrode Array.

BACKGROUND: A prospective longitudinal multicentre study was conducted to assess the one-year postsurgical hearing preservation profile of the EVOTM electrode array. METHODS: Fifteen adults presenting indications of electro-acoustic stimulation (pure-tone audiometry (PTA) thresholds ≤70 dB below 750 Hz) were implanted with the EVO™ electrode array. Hearing thresholds were collected at five time-points from CI activation to twelve months (12M) after activation. Hearing thresholds and hearing preservation profiles (HEARRING group classification) were assessed. RESULTS: All subjects had measurable hearing thresholds at follow-up. No case of complete loss of hearing or minimal hearing preservation was reported at any time point. At activation (Nact = 15), five participants had complete hearing preservation, and ten participants had partial hearing preservation. At the 12M time point (N12m = 6), three participants had complete hearing preservation, and three participants had partial hearing preservation. Mean hearing loss at activation was 11 dB for full range PTA and 25 dB for PTAs low-frequency (125-500 Hz). CONCLUSIONS: This study provides the first longitudinal follow-up on associated hearing profiles to the EVO™ electrode array, which are comparable to the literature. However, other studies on larger populations should be performed.

Zinc protection against pneumolysin toxicity on rat cochlear hair cells.

Streptococcus pneumoniae can induce local and systemic diseases such as meningitis, otitis media, and pneumonia. One third of these meningitis cases can be associated with irreversible sensorineural hearing loss whose mechanisms likely involves the exotoxin pneumolysin (PLY) that irreversibly damages cochlear hair cells (HCs). In the respiratory system and in neuron it has been demonstrated that zinc deficiency increases severity and mortality of such infections in animal models and in children. Moreover, zinc supplementation can decrease the severity of pneumococcal respiratory infections. The aim of our study was to assess the potential protective effect of zinc against PLY toxicity on HCs in culture. Our results showed that in the presence of zinc at concentration as low as 1 microM, the toxicity of PLY was largely reduced by about 50% for both inner and outer HCs. At 300 microM of zinc, protection significantly increased with 62 and 55.2% for IHCs and OHCs, respectively. Our results suggest that the protective effect of zinc is likely due to an inhibition of the toxin incorporation and aggregation into the plasma membrane, thus preventing calcium influx through the toxin pores. Our findings raise the possibility that treatments with zinc may help to prevent debilitating otological sequelae from pneumococcal infection.

Multifrequency immittancemetry in experimentally induced stapes, round window and cochlear lesions.

OBJECTIVES: To establish that admittance (Y) and susceptance (B) conductance (G) tympanograms at 2 kHz can reflect the status of the annular ligament and the cochlear pressure. METHODS: Seven experiments were set up in 22 guinea pigs: ventilation of the bulla, blockage of the stapes and round window membrane (RWM), fistula, fluid removal from the cochlea, injection of saline in the scala tympani and acoustic trauma. Resonance frequency, Y, B and G at 2 kHz and curve shapes were analyzed before and after lesions. RESULTS: A supplementary peak was observed in Y/G tympanograms in all RWM fistulas and in some cases of acoustic trauma; injection of saline into the scala tympani induced constant, immediate and reproducible changes; RWM and stapes blockages induced foreseeable peaking at 2 kHz; fluid removal from the cochlea induced multiple peak curves. CONCLUSION: Experimentally induced modifications at the AL result in noticeable, constant and reproducible changes in tympanogram curves at 2 kHz and seem to reflect inner ear pressure.

Intraorbital apocrine hidrocystoma.

A 33-year-old man reported an 18 months history of a progressive right eyelid swelling, a sensation of eye pain and headaches. He noted a visual discomfort. Physical examination showed right eyelid edema, without skin lesion. The patient's acuity was unchanged from the baseline; neither exophthalmia nor deficit in the visual field was noted. Magnetic resonance image showed an intraorbital, extraconal cystic lesion. Histopathologic examination revealed apocrine hidrocystoma.

Transpetrosal approaches for meningiomas of the posterior aspect of the petrous bone Results in 43 consecutive patients.

OBJECTIVE: To assess outcome following excision of meningiomas of the posterior aspect of the petrous bone through transpetrosal approaches. MATERIAL AND METHOD: We carried out a retrospective case-series study in a multidisciplinary tertiary care center on all patients who underwent meningiomas removal from January 1989 to September 2005. Surgical approaches were transpetrosal: widened retrolabyrinthine, translabyrinthine, transotic and transcochlear, occasionally combined with a subtemporal transtentorial approach. Epidemiology, symptoms, preoperative evaluation, surgery, postoperative complications and facial and auditory results were analyzed using standardized grading systems. The Desgeorges and Sterkers classification was used to assess tumor size and location. RESULTS: Forty women and three men underwent surgery (mean age: 56.7). Medium-sized tumors stages 2 and 3 (84%) and AM and P localization (34% and 20.4%) predominated. In 65% of cases, the tumor extended beyond the CPA. Main presenting symptoms were balance disorders (72%) and sensorineural hearing loss (53.5%). Mortality was nil. A preoperative facial nerve paresis was present in 14% of patients. Tumor removal was complete in 79.1% of cases. At 1-year post-op, 73% of patients had a normal or subnormal facial function and 55% had serviceable hearing. A cerebrospinal fluid leakage occurred in 6.9%. DISCUSSION: Posteriorly attached meningiomas are less symptomatic and of better prognosis than medially inserted ones. Transpetrosal approaches are reliable for the removal for all types and sizes of such tumors, and can be easily combined in the same procedure with a subtemporal transtentorial approach to remove extensions to the clivus and tentorium. They offer low morbidity and a high proportion of facial nerve and hearing preservation.

Intracochlear hemorrhage after gamma knife radiosurgery.

PURPOSE: To describe an acute complication after gamma knife stereotactic radiosurgery (GKRS) for vestibular schwannoma (VS) in a neurofibromatosis type 2 (NF2) patient. STUDY DESIGN: Case report. SETTING: Tertiary care center. PATIENT: A 20-year-old man, who had bilateral VS and was having right-sided profound deafness, underwent GKRS for a 2-cm left-sided VS in an attempt to preserve his only hearing ear. He received a margin dose of 13 Gy to the 50% isodose line. Twenty-four hours after treatment, he presented with spinning vertigo, left-sided dead ear, and ipsilateral mild facial paralysis (House-Brackmann grade 3). RESULTS: Magnetic resonance imaging demonstrated an intracochlear hemorrhage at the level of the basal turn of the left cochlea. Hearing did not recover, and the patient had to resort to lip reading. The facial paralysis regressed completely after 3 months. CONCLUSION: Many cases of hemorrhage caused by GKRS have previously been reported, but all were related to meningiomas or brain metastases. Because the patient had no coagulation defect, sign of trauma, or any history of infection, the hemorrhage might have been caused by a direct thermal effect on the endothelial cells or to an immediate tumoral swelling, inducing an increase in intravascular outflow resistance and leading to venous obliteration. To our knowledge, this is the first report of acute intracochlear hemorrhage after GKRS for VS.

Necrotizing external otitis: a report of 46 cases.

OBJECTIVES: To define germs involved, clinical presentation, treatment regimen, and prognostic factors in necrotizing external otitis. PATIENTS AND METHODS: Retrospective study reviewing a series of 46 patients treated during 10 years in a tertiary care center. Diagnosis was confirmed by using otomicroscopy, computed tomographic and/or magnetic resonance imaging scan and bone scintigraphy (Te 99 and Ga 26 bone scan). Patients were provided ceftazidime and ciprofloxacin intravenously and monitored using a Ga-67 bone scan. The following were assessed: presenting symptoms, general context, bacteriological analysis, imaging protocol sensitivity, complications, delay to healing, and cure rate. RESULTS: Sex ratio was 2.29 (mean age, 73.6 yr). The most common presenting symptoms were otalgia and otorrhea in 97.8 and 91.3%, respectively. Facial paralysis was present in 19.6% of cases. Thirty patients had diabetes mellitus (65.2%), and 8 were immunocompromised (17.4%). Pseudomonas aeruginosa was isolated in 69.2% of cases and was resistant to ciprofloxacin in 18.5%, but was susceptible to ceftazidime in all cases. Four patients died during the treatment (4.4%), but only 2 of disease. The mean delay to healing was 14 weeks (SD, 9.7). Healing rate was 95.6%. No relapse was observed after a mean follow-up of 78.4 weeks (SD, 36.5 wks). Two factors significantly influenced the prognosis: facial paralysis and existence of systemic factors for immune deficiency (p = 0.023 and 0.038, respectively). CONCLUSION: The association of ciprofloxacin and ceftazidime was efficient in countering the increasing resistance of P. aeruginosa to quinolones. We propose a prognostic classification of necrotizing external otitis based on the presence of facial paralysis and/or systemic factors.

Outcome-based assessment of endolymphatic sac decompression for Ménière's disease using the Ménière's disease outcome questionnaire: a review of 90 patients.

OBJECTIVE: To evaluate the quality of life (QOL) of patients treated by endolymphatic sac decompression (ELSD) for Ménière's disease (MD) with a dedicated questionnaire, while recording the results of this surgery on vertigo attacks using the American Academy of Otolaryngology-Head and Neck Surgery criteria. STUDY DESIGN: Retrospective case review in a tertiary referral care center. MATERIAL AND METHODS: Ninety patients who have MD were treated by ELSD at our institution between 1986 and 2004. They were sent a 40-question Ménière's Disease QOL dedicated questionnaire (Ménière's Disease Outcome Questionnaire) concerning physical, mental, and social well-being. Five scores out of 100 were calculated (S1-S5): S3 for preoperative QOL score, S4 for postoperative QOL score, S5 for change in QOL score (S5 = S4 - S3), and S1 and S2 representing the answers to two other paired questions about the "overall" feeling of the patients. In parallel, results of ELSD on vertigo attacks and hearing were evaluated using the Academy of Otolaryngology-Head and Neck Surgery criteria. RESULTS: Fifty-nine interpretable answers (65.6%) to the Ménière's Disease Outcome Questionnaire were obtained from 28 men and 31 women (mean age, 56 yr and 9 mo), all suffering from "definite" MD. The mean S3 was 32.5 (range, 9.7-84.7), and the mean S4 was 60.8 (range, 0-98.6). Consecutively, S5 score was 28.3 (range, -34.7 to 77.4). QOL improved in 81.4% of cases with an average duration of follow-up of 57.5 months (range, 4-156 mo) (p < 0.001), worsened in 11.9% and was stable in 6.8%. There was no statistically significant difference as a function of sex (p = 0.7) or length of follow-up (p = 0.6). There was a significant correlation between S1 and S3 (p < 0.0001), and S2 and S4 (p < 0.0001), validating the whole questionnaire. In addition, 71% of satisfactory control of vertigo at 2 years postoperatively was noted. Hearing was improved or unchanged in 79% of cases at 3 months postoperatively. CONCLUSION: These results show that ELSD significantly improves the health of patients. It represents a first-line procedure that preserves the vestibular and afferent structures, unlike vestibular neurotomy and chemical labyrinthectomy, which may be indicated as a second line.

Multifrequency admittancemetry in Ménière's Disease: a preliminary study for a new diagnostic test.

OBJECTIVE: To assess the qualitative and quantitative aspects of multifrequency tympanograms in patients with Ménière's disease. STUDY DESIGN: Prospective study of adults. SETTING: University medical center. PATIENTS: Forty patients with Ménière's disease outside of episodes and 24 normal-hearing subjects with no otologic history. INTERVENTIONS: Resonance frequency sweep and tympanograms of admittance, conductance, and susceptance at 2 kHz and at resonance frequency were performed in each ear. MAIN OUTCOME MEASURES: A qualitative assessment was performed to find reproducible and comparable tympanograms (Student's t test) as a prerequisite for quantitative assessment. Four measurement criteria were compared between the groups to establish a diagnostic test (chi Pearson test). A p value less than 0.05 was considered statistically significant. RESULTS: Resonance frequency was decreased in Ménière's disease. Only conductance tympanograms at 2 kHz had a constant pattern. Width of conductance tympanograms at 2 kHz was increased in symptomatic Ménière's disease compared with normal ears. Using conductance width at 2 kHz as a diagnostic test with a threshold of 235 daPa, we found a statistically significant difference between symptomatic ears of patients with Ménière's disease and ears of normal subjects (p < 10). More than 95% of normal subjects' ears had a negative test; 56.5% of affected ears of patients with Ménière's disease and 45.8% of nonaffected ears of patients with Ménière's disease had a positive test. CONCLUSION: Width of conductance at 2 kHz seems to be a sensitive, simple diagnostic test in Ménière's disease. It would also be of great interest in studying Ménière's disease bilaterality.

Effects of body tilt on multifrequency admittance tympanometry.

INTRODUCTION: Hydrops and abnormalities of inner fluid pressure are involved in some otologic diseases such as Ménière's disease (MD). However, demonstrating abnormal perilymphatic or endolymphatic pressure is challenging. Multifrequency tympanometry studies in MD patients demonstrated an increase of the width of conductance tympanograms (outside an attack) compared with controls. To confirm that the increase in conductance width is caused by hyperpressure and not hypopressure in these patients tested outside an attack, we assessed the effect of changes in inner ear fluid pressure caused by body tilt on the results of multifrequency admittancemetry tympanograms. MATERIALS AND METHODS: A multifrequency tympanometry including conductance (G) tympanogram at 2 kHz and resonance frequency measurements were performed in 20 volunteers (40 ears) free of otologic or neurologic disease. The measures were collected in three different positions: vertical, supine, and Trendelenburg positions. RESULTS: Changes in inner ear fluid pressure caused by body tilt induced an increase in the width of G tympanograms. In the vertical position, the mean value was 141.7 ± 56.5 daPa; in the supine position, it increased to 158 ± 58.3 daPa; and increased even more in the Trendelenburg position (20 degrees), with a mean of 184 ± 69.6 daPa (p < 0.01). Resonance frequency also increased in the Trendelenburg position.We conclude that the increased width of G tympanograms in MD patients outside an attack may be caused by an increase in inner ear fluid pressure.