Cleft palate morphology, genetic etiology, and risk of mortality in infants with Robin sequence.

Robin sequence (RS) has many genetic and nongenetic causes, including isolated Robin sequence (iRS), Stickler syndrome (SS), and other syndromes (SyndRS). The purpose of this study was to determine if the presence and type of cleft palate varies between etiologic groups. A secondary endpoint was to determine the relationship of etiologic group, cleft type, and mortality. Retrospective chart review of patients with RS at two high-volume craniofacial centers. 295 patients with RS identified. CP was identified in 97% with iRS, 95% with SS, and 70% of those with SyndRS (p < .0001). U-shaped CP was seen in 86% of iRS, 82% with SS, but only 27% with SyndRS (p < .0001). At one institution, 12 children (6%) with RS died, all from the SyndRS group (p < .0001). All died due to medical comorbidities related to their syndrome. Only 25% of children who died had a U-shaped CP. The most common palatal morphology among those who died was an intact palate. U-shaped CP was most strongly associated with iRS and SS, and with a lower risk of mortality. RS with submucous CP, cleft lip and palate or intact palate was strongly suggestive of an underlying genetic syndrome and higher risk of mortality.

Long-term efficacy of triple semicircular canal plugging in the treatment of patients with ipsilateral delayed endolymphatic hydrops.

This study aims to explore the long-term efficacy of triple semicircular canal plugging (TSCP) in the treatment of intractable ipsilateral delayed endolymphatic hydrops (DEH), so as to provide an alternative therapy for this disease. Forty-eight patients diagnosed with ipsilateral DEH referred to vertigo clinic of our hospital between Dec. 2010 and Dec. 2017, were included in this study for retrospective analysis. All patients were followed up for 2 years. Vertigo control and auditory functions were measured and analyzed. Pure tone audiometry, caloric test, and vestibular evoked myogenic potential (VEMP) were performed in two-year follow-up. Forty-five patients who accepted intratympanic gentamicin (26.7 mg/mL) twice given one week apart were selected as a control group. The total control rate of vertigo in TSCP group was 97.9% (47/48) in the two-year follow-up, with complete control rate of 83.3% (40/48) and substantial control rate of 14.6% (7/48). The rate of hearing loss was 22.9% (11/48). The total control rate of vertigo in intratympanic gentamicin group was 80.0% (36/45), with complete control rate of 57.8% (26/45) and substantial control rate of 22.2% (10/45), and the rate of hearing loss was 20.0% (9/45). The vertigo control rate of TSCP was significantly higher than that of intratympanic gentamicin (χ2 = 6.01, p < 0.05). There was no significant difference of hearing loss rate between two groups. (χ2 = 0.12, p > 0.05). TSCP, which can reduce vertiginous symptoms in patients with intractable ipsilateral DEH, represents an effective therapy for this disorder.

Usefulness of vertebrobasilar artery radiological finding as a predictive and prognostic factor for sudden sensorineural hearing loss.

OBJECTIVE: The association between sudden sensorineural hearing loss (SSNHL) and radiological findings of the vertebrobasilar artery is not well-known and little research has been done. We hypothesized that the radiological features of the vertebrobasilar artery contribute to the incidence and prognosis of SSNHL. METHODS: We retrospectively enrolled patients diagnosed with unilateral SSNHL (SSNHL group) and those with acute vestibular neuritis (AVN; control group) in our hospital. All patients underwent magnetic resonance imaging and computed tomography. We measured the following parameters on the radiological images: basilar artery diameter, direction and distance of basilar artery deviation, direction and distance of vertebral artery deviation, and incidence of vertebral artery obstruction. Pure tone audiometry (PTA) was performed in all patients. Follow up PTA between 1 week and 1 month after treatment was performed in the SSNHL group. RESULTS: A total of 244 SSNHL patients and 62 AVN patients were included in the analysis. Age, body mass index, and basilar artery diameter were found to be significantly associated with SSNHL. In the SSNHL group, patients were divided into three subgroups based on the consistency between the basilar artery deviation site and disease site. No significant difference was noted in initial PTA, final PTA, PTA recovery, and symptom improvement among the three groups. In case of the basilar artery, when the deviation and disease sites were in the opposite direction and the basilar artery diameter was >3.5 mm, diameter of basilar artery was positively correlated with PTA recovery. CONCLUSIONS: The strength of this study is that radiological evaluation of the vertebrobasilar artery was performed. Further research on the association between SSNHL and radiological features of the vertebrobasilar artery should be conducted to emphasize the importance of vascular assessment in SSNHL.

Cortical reorganization following auditory deprivation predicts cochlear implant performance in postlingually deaf adults.

Long-term hearing loss in postlingually deaf (PD) adults may lead to brain structural changes that affect the outcomes of cochlear implantation. We studied 94 PD patients who underwent cochlear implantation and 37 patients who were MRI-scanned within 2 weeks after the onset of sudden hearing loss and expected with minimal brain structural changes in relation to deafness. Compared with those with sudden hearing loss, we found lower gray matter (GM) probabilities in bilateral thalami, superior, middle, inferior temporal cortices as well as the central cortical regions corresponding to the movement and sensation of the lips, tongue, and larynx in the PD group. Among these brain areas, the GM in the middle temporal cortex showed negative correlation with disease duration, whereas the other areas displayed positive correlations. Left superior, middle temporal cortical, and bilateral thalamic GMs were the most accurate predictors of post-cochlear implantation word recognition scores (mean absolute error [MAE] = 10.1, r = .82), which was superior to clinical variables used (MAE: 12.1, p < .05). Using the combined brain morphological and clinical features, we achieved the best prediction of the outcome (MAE: 8.51, r = .90). Our findings suggest that the cross-modal plasticity allowing the superior temporal cortex and thalamus to process other modal sensory inputs reverses the initially lower volume when deafness becomes persistent. The middle temporal cortex processing higher-level language comprehension shows persistent negative correlations with disease duration, suggesting this area's association with degraded speech comprehensions due to long-term deafness. Morphological features combined with clinical variables might play a key role in predicting outcomes of cochlear implantation.

Association Between Incomplete Partition Type III and Abnormal Hypothalamic Morphology: Further Imaging Evidence.

PURPOSE: Incomplete partition III (IP-III), characterized by congenital mixed or sensorineural hearing loss, is a rare genetic disease transmitted through X-linked mode of inheritance. Inner ear findings of IP-III have been well described and allow an immediate diagnosis to be made. Recently, an association between IP-III and distinct hypothalamic malformations has been reported in some of the patients with IP-III. The purpose of this study was to investigate the morphologic abnormalities of the hypothalamus in IP-III. MATERIALS AND METHODS: Magnetic resonance imaging studies of 8 subjects, including 1 set of brothers, who were diagnosed with IP-III based on their clinical and inner ear imaging findings, were analyzed. RESULTS: Of the 8 subjects, 7 demonstrated some degree of morphologic abnormality of the hypothalamus. Of these, 2 showed asymmetrical thickening, 1 showed symmetrical thickening, and 4 showed mass-like enlargement of the hypothalamus. Six of 7 subjects with hypothalamic abnormalities showed asymmetry in caudal extension of the abnormalities, which was more discernible on coronal oblique T2-weighted images. Clinically, none of the subjects had endocrinologic or neurologic symptoms. CONCLUSIONS: This retrospective analysis presents further magnetic resonance imaging evidence on the association between the rare IP-III malformations and the presence of hypothalamic morphologic abnormalities.

A Case Series of X-Linked Deafness-2 with Sensorineural Hearing Loss, Stapes Fixation, and Perilymphatic Gusher: MR Imaging and Clinical Features of Hypothalamic Malformations.

X-linked deafness-2 (DFNX2) is an X-linked recessive disorder characterized by profound sensorineural hearing loss and a pathognomonic temporal bone deformity. Because hypothalamic malformations associated with DFNX2 have been rarely described, we aimed to further describe these lesions and compare them with features of a nonaffected population. All patients diagnosed with DFNX2 between 2006 and 2019 were included and compared with age-matched patients with normal MR imaging findings and without hypothalamic dysfunction. MR imaging features differing between groups were selected to help identify DFNX2. Sensitivity and specificity were calculated for these features. Agreement among 3 radiologists was quantified using the index κ. Information on the presence or absence of gelastic seizures, precocious puberty, or delayed puberty was also gathered. We selected distinctive MR imaging features of hypothalamic malformations in DFNX2. The feature selected on axial T2 images was the folded appearance of the ventromedial hypothalamus (sensitivity, 100%; specificity, 95.8%) characterized by an abnormal internal/external cleft (sensitivity, 100%; specificity, 95.7%). On coronal T2, the first distinctive feature was a concave morphology of the medial eminence (sensitivity, 100%; specificity, 97.1%), the second feature was at least 1 hypothalamic-septum angle ≥90° (sensitivity, 90%; specificity, 72.5%), and the third feature was a forebrain-hypothalamic craniocaudal length of ≥6 mm (sensitivity, 70%; specificity, 79.7%). Clinical features were also distinctive because 9 patients with DFNX2 did not present with gelastic seizures or precocious puberty. One patient had delayed puberty. The κ index and intraclass correlation coefficient ranged between 0.78 and 0.95. Imaging and clinical features of the hypothalamus suggest that there is a hypothalamic malformation associated with DFNX2. Early assessment for pubertal delay is proposed.

Different prognoses in patients with profound sudden sensorineural hearing loss.

Background: Inner ear hemorrhage is increasingly recognized as a cochlear lesion that can cause profound sudden sensorineural hearing loss (SSNHL). Objectives: To investigate changes of cochlear and vestibular function and to compare therapeutic recovery from profound SSNHL induced by different etiologies. Material and methods: Eighty patients with profound SSNHL (≥90 dB) were divided into an inner ear hemorrhage group and a non-inner ear hemorrhage group by MRI. Statistical analysis was performed to compare the therapeutic effects from vertigo and hearing loss and the outcomes of follow-up in the two groups. Results: There were significant differences between the two groups in terms of the overall 14-day therapeutic response rate (20 vs. 48%), the incidence of imbalance (26.7 vs. 6%), the incidence of semicircular canal dysfunction on the affected side (60 vs. 20%), the incidence of abnormal C-VEMP and O-VEMP on the affected side (63.3 vs. 38%; and 60 vs. 30%, respectively), the average hearing threshold (74.2 ± 10.7 vs. 53.6 ± 11.4 dB), and the word recognition score (65.5 ± 21.7 vs. 83.5 ± 24.5%) at a 12-month follow-up. Conclusions and significance: A higher percentage of patients with profound SSNHL induced by inner ear hemorrhage were associated with vertigo and had a poor prognosis.

Genetic, Radiologic, and Clinical Variability in Brown-Vialetto-van Laere Syndrome.

Brown-Vialetto-van Laere syndrome is characterized by a progressive sensorimotor neuropathy, optic atrophy, hearing loss, bulbar dysfunction, and respiratory insufficiency. Mutations in SLC52A2 and SLC52A3, encoding riboflavin transporters RFVT2 and RFVT3, respectively, are the genetic basis of this disorder, often referred to as riboflavin transporter deficiency types 2 and 3, respectively. We present cases of both types of riboflavin transporter deficiency, highlighting the distinguishing clinical features of a rapidly progressive motor or sensorimotor axonal neuropathy, optic atrophy, sensorineural hearing loss, and bulbar dysfunction. One child presented with isolated central apnea and hypoventilation, not previously described in genetically confirmed Brown-Vialetto-van Laere, later complicated by diaphragmatic paralysis secondary to phrenic nerve palsy. Magnetic resonance imaging showed T2 hyperintensity in the dorsal spinal cord in 2 children, as well as previously unreported cervical nerve root enlargement and cauda equina ventral nerve root enhancement in 1 child. Novel homozygous mutations were identified in each gene-a NM_024531.4(SLC52A2):c.505C > T, NP_078807.1(SLC52A2):p.(Arg169Cys) variant in SLC52A2 and NM_033409.3(SLC52A3):c.1316G > A, NP_212134.3(SLC52A3):p.(Gly439Asp) variant in SLC52A3. Both treated children showed improvement on high-dose riboflavin supplementation, highlighting the importance of early recognition of this treatable clinical entity.

Tonotopic organisation of the auditory cortex in sloping sensorineural hearing loss.

Although the tonotopic organisation of the human primary auditory cortex (PAC) has already been studied, the question how its responses are affected in sensorineural hearing loss remains open. Twenty six patients (aged 38.1 ± 9.1 years; 12 men) with symmetrical sloping sensorineural hearing loss (SNHL) and 32 age- and gender-matched controls (NH) participated in an fMRI study using a sparse protocol. The stimuli were binaural 8s complex tones with central frequencies of 400 HzCF, 800 HzCF, 1600 HzCF, 3200 HzCF, or 6400 HzCF, presented at 80 dB(C). In NH responses to all frequency ranges were found in bilateral auditory cortices. The outcomes of a winnermap approach, showing a relative arrangement of active frequency-specific areas, was in line with the existing literature and revealed a V-shape high-frequency gradient surrounding areas that responded to low frequencies in the auditory cortex. In SNHL frequency-specific auditory cortex responses were observed only for sounds from 400 HzCF to 1600 HzCF, due to the severe or profound hearing loss in higher frequency ranges. Using a stringent statistical threshold (p < 0.05; FWE) significant differences between NH and SNHL were only revealed for mid and high-frequency sounds. At a more lenient statistical threshold (p < 0.001, FDRc), however, the size of activation induced by 400 HzCF in PAC was found statistically larger in patients with a prelingual, as compared to a postlingual onset of hearing loss. In addition, this low-frequency range was more extensively represented in the auditory cortex when outcomes obtained in all patients were contrasted with those revealed in normal hearing individuals (although statistically significant only for the secondary auditory cortex). The outcomes of the study suggest preserved patterns of large-scale tonotopic organisation in SNHL which can be further refined following auditory experience, especially when the hearing loss occurs prelingually. SNHL can induce both enlargement and reduction of the extent of responses in the topically organized auditory cortex.

Contrasting results of tests of peripheral vestibular function in patients with bilateral large vestibular aqueduct syndrome.

OBJECTIVE: To analyze and summarize the effect of bilateral large vestibular aqueducts in peripheral vestibular organ function. METHODS: Eighteen patients with bilateral large vestibular aqueduct syndrome (LVAS; Study Group) and 18 healthy volunteers (Control Group) were investigated using audiometry, caloric test, sensory organization test (SOT), and vestibular-evoked myogenic potential (VEMP) tests. RESULTS: All 18 patients (36 ears) exhibited sensorineural hearing loss. For cervical VEMP (cVEMP), the Study Group showed lower thresholds (Study Group vs. CONTROL GROUP: 71.4vs. 75.3dBnHL; p=0.006), N1 latencies (24.1vs. 25.2ms; p=0.026) and shorter P1 (15.3vs. 16.6ms; p=0.003), and higher amplitudes (400.7vs. 247.2µV; p<0.001) than the Control Group. For ocular VEMP (oVEMP), the Study Group had lower thresholds (79.3vs. 81.8dBnHL; p=0.046) and higher amplitudes (40.6vs. 14.4µV; p<0.001) than the Control Group. Fourteen of 16 patients (87.5%) who completed caloric tests had abnormal results, and 10 of 18 patients (55.6%) exhibited abnormal results in SOTs. CONCLUSIONS: The hyperfunction of vestibular test in otolithic organs and the hypofunction of vestibular test in semicircular canals, as well as the dysfunction in the balance test were demonstrated in patients with LVAS. SIGNIFICANCE: Our findings can help clinicians gain a better understanding of the characteristics of vestibular organ function in patients with LVAS, which can facilitate optimal targeted treatment.

Early cortical metabolic rearrangement related to clinical data in idiopathic sudden sensorineural hearing loss.

Results in studies concerning cortical changes in idiopathic sudden sensorineural hearing loss (ISSNHL) are not homogeneous, in particular due to the different neuroimaging techniques implemented and the diverse stages of ISSNHL studied. Considering the recent advances in state-of-the-art positron emission tomography (PET) cameras, the aim of this study was to gain more insight into the neuroanatomical differences associated with the earliest stages of unilateral ISSNHL and clinical-perceptual performance changes. After an audiological examination including the mean auditory threshold (mean AT), mean speech discrimination score (mean SDS) and Tinnitus Handicap Inventory (THI), 14 right-handed ISSNHL patients underwent brain [18F]fluorodeoxyglucose (FDG)-PET within 72 h of the onset of symptoms. When compared to an homogeneous group of 35 healthy subjects by means of statistical parametric mapping, a relative increase in FDG uptake was found in the right superior and medial frontal gyrus as well as in the right anterior cingulate cortex in ISSNHL patients. Conversely, the same group showed a significant relative decrease in FDG uptake in the right middle temporal, precentral and postcentral gyrus as well as in the left posterior cingulate cortex, left lingual, superior, middle temporal and middle frontal gyrus and in the left insula. Regression analysis showed a positive correlation between mean THI and glucose consumption in the right anterior cingulate cortex and a positive correlation between mean SDS and glucose consumption in the left precentral gyrus. The relative changes in FDG uptake found in these brain regions and the positive correlation with mean SDS and THI scores in ISSNHL could possibly highlight new aspects of cerebral rearrangement, contributing to further explain changes in those functions that support speech recognition during the sudden impairment of unilateral auditory input.

Revisiting the relationship of three-dimensional fluid attenuation inversion recovery imaging and hearing outcomes in adults with idiopathic unilateral sudden sensorineural hearing loss.

BACKGROUND AND PURPOSE: Three-dimensional fluid attenuation inversion recovery (3D FLAIR) may demonstrate high signal in the inner ears of patients with idiopathic sudden sensorineural hearing loss (ISSNHL), but the correlations of this finding with outcomes are still controversial. Here we compared 4 3D MRI sequences with the outcomes of patients with ISSNHL. MATERIALS AND METHODS: 77 adult patients with ISSNHL underwent MRI with pre contrast FLAIR, fast imaging employing steady-state acquisition images (FIESTA-C), post contrast T1WI and post contrast FLAIR. The extent and degree of high signal in both cochleas were evaluated in all patients, and asymmetry ratios between the affected ears and the normal ones were calculated. The relationships among MRI findings, including extent and asymmetry of abnormal cochlear high signals, degree of FLAIR enhancement, and clinical information, including age, vestibular symptoms, baseline hearing loss, and final hearing outcomes were analyzed. RESULTS: 54 patients (28 men; age, 52.1±15.5years) were included in our study. Asymmetric cochlear signal intensities were more frequently observed in pre contrast and post contrast FLAIR (79.6% and 68.5%) than in FIESTA-C (61.1%) and T1WI (51.9%) (p<0.001). Age, baseline hearing loss, extent of high signal and asymmetry ratios of pre contrast and post contrast FLAIR were all correlated with final hearing outcomes. In multivariate analysis, age and the extent of high signals were the most significant predictors of final hearing outcomes. CONCLUSION: 3D FLAIR provides a higher sensitivity in detecting the asymmetric cochlear signal abnormality. The more asymmetric FLAIR signals and presence of high signals beyond cochlea indicated a poorer prognosis.

Bilateral simultaneous sudden sensorineural hearing loss.

OBJECTIVE: This study adopted an inner ear test battery and MR imaging in patients with bilateral sudden sensorineural hearing loss (SSNHL) to investigate their causes, disease extent, and evaluate hearing outcome. PATIENTS AND METHODS: From 1995 to 2014, 16 patients with bilateral SSNHL received audiometry, caloric test and MR imaging. Vestibular-evoked myogenic potential (VEMP) test was added to the test battery after 2000. RESULTS: Percentages of abnormal mean hearing level (MHL), cervical VEMP test, ocular VEMP test, and caloric test in patients with bilateral SSNHL were 100% (32/32), 100% (12/12), 100% (4/4), and 81% (26/32), respectively, implying that not only the cochlear part but also the vestibular part was severely affected in both ears. Causes of bilateral SSNHL were neoplasm in 5 patients, stroke in 5, meningitis in 1, and unknown in 5. Post-treatment MHL did not significantly differ from pre-treatment MHL indicating poor hearing outcome. Seven patients (44%) had passed away within 5years after onset, 2 patients were lost, and 7 patients survived. CONCLUSION: Via MR imaging, causes of bilateral SSNHL were identified for 69% of cases. Both cochlear and vestibular endorgans/afferents were identified to be severely affected bilaterally by the vestibular test battery and resulted in poor hearing outcome. A high mortality rate (44%) indicates that bilateral SSNHL is an ominous sign for a more sinister underlying disease.

Revisiting Place-Pitch Match in CI Recipients Using 3D Imaging Analysis.

OBJECTIVE: To improve the estimation of the perceived pitch in a single-sided deaf cochlear implant (CI) listener by using accurate 3-dimensional (3D) image analysis of the cochlear electrode positions together with the predicted tonotopical function for humans. METHODS: An SSD CI user underwent a Cone-Beam computed tomography (CBCT) scan. Electrode contacts were marked in 3D space in relation to the nearest point on the cochlear lateral wall. Distance to the base of the lateral wall was calculated and plotted against the place-pitch function for humans. An adaptive procedure was used to elicit the perceived pitch of electrically evoked stimulation by matching it with a contralateral acoustic pitch. RESULTS: The electrically evoked pitch percept matched well with the calculated frequency. The median mismatch in octaves was 0.12 for our method in comparison to 0.69 using the conventional Stenvers view. CONCLUSION: A method of improved image analysis is described that can be used to predict the pitch percept on corresponding cochlear electrode positions. This method shows the potential of 3D imaging in CI fitting optimization.

Binaural stimulation through cochlear implants in postlingual deafness: a positron emission tomographic study of word recognition.

HYPOTHESIS: Bilateral stimulation through cochlear implants induces a brain activity pattern closer to the normal one than unilateral stimulation. BACKGROUND: Although it has been shown that speech comprehension through bilateral cochlear implants leads to better performances than after unilateral implantation, the existence of neural underpinnings of this improvement remains to be studied. METHODS: We performed an H2O positron emission tomographic study of word recognition in 5 patients with bilateral cochlear implants and 5 normal-hearing controls. Subjects had to distinguish words from nonwords in binaural and monaural conditions. RESULTS: There was no overactivation in patients for binaural stimulation, with a hypoactivation in the right temporal cortex. For monaural stimulation, patients demonstrated more activation contralaterally to the stimulation side in the posterior temporal cortex and in the cerebellum. CONCLUSION: Binaural stimulation through cochlear implants is advantageous compared with the monaural at the neurofunctional level because the pattern of brain activity is closer to the normal one.

Auditory cortical activity during cochlear implant-mediated perception of spoken language, melody, and rhythm.

Despite the significant advances in language perception for cochlear implant (CI) recipients, music perception continues to be a major challenge for implant-mediated listening. Our understanding of the neural mechanisms that underlie successful implant listening remains limited. To our knowledge, this study represents the first neuroimaging investigation of music perception in CI users, with the hypothesis that CI subjects would demonstrate greater auditory cortical activation than normal hearing controls. H(2) (15)O positron emission tomography (PET) was used here to assess auditory cortical activation patterns in ten postlingually deafened CI patients and ten normal hearing control subjects. Subjects were presented with language, melody, and rhythm tasks during scanning. Our results show significant auditory cortical activation in implant subjects in comparison to control subjects for language, melody, and rhythm. The greatest activity in CI users compared to controls was seen for language tasks, which is thought to reflect both implant and neural specializations for language processing. For musical stimuli, PET scanning revealed significantly greater activation during rhythm perception in CI subjects (compared to control subjects), and the least activation during melody perception, which was the most difficult task for CI users. These results may suggest a possible relationship between auditory performance and degree of auditory cortical activation in implant recipients that deserves further study.

Assessment of changes in cochlear function with pneumolabyrinth after middle ear trauma.

OBJECTIVE: To describe a case of pneumolabyrinth secondary to tympanic membrane/ossicular trauma and the subsequent recovery of sensorineural hearing loss managed with conservative measures. PATIENTS: A 15-year-old boy presented to an outside hospital with signs and symptoms of acute hearing loss, vertigo, and tinnitus after penetrating injury to his right tympanic membrane. In addition, computed tomography demonstrated air density within the vestibule. INTERVENTIONS: The patient was managed conservatively with bed rest, avoidance of straining, corticosteroids, and antibiotics. MAIN OUTCOME MEASURES: Computed tomography, audiologic testing. RESULTS: Patient recovered near-normal hearing subjectively. There was closure of the air-bone gap (

Auditory cortical activation in severe-to-profound hearing-impaired patients monitored by SPET.

Single photon emission tomography was used to map blood flow increase in temporal and parietal cortex after auditory stimulation in 25 subjects: 10 normal-hearing, 10 severe-profound hearing-impaired and 5 totally deaf. After a 500 Hz pure tone stimulation, a marked perfusion increase was observed, particularly at the level of the contralateral auditory temporal cortex. Blood flow increase in temporal and parietal cortical areas of normal subjects was significantly higher than that observed in severe-to-profound hearing-impaired patients. In all cases, following 500 Hz pure tone acoustic stimulation, the most lateral sagittal slice tomograms (48.75 and 56.25 mm) showed the highest blood flow increase. Statistically significant differences were also observed between normal subjects and hearing-impaired patients in the 48.75 mm sagittal tomogram. In 2 hearing-impaired patients, the single photon emission tomography pattern showed activation of the intermediate sagittal tomogram, suggesting a possible new tonotopic cortical arrangement. No significant activation was present in totally deaf patients. In conclusion, Single Photon Emission Tomography appears to be a useful tool in the evaluation of auditory cortical activation and cortical plasticity, in severe-to-profound hearing-impaired patients. Moreover, it could be a useful test for the study of auditory central pathways.

Doppler ultrasonography of the vertebrobasilar circulation in patients with sudden sensorineural hearing loss.

BACKGROUND: The etiology of sudden sensorineural hearing loss (SSNHL) is still unclear and is most probably diverse. OBJECTIVE: To determine the relationship between vertebrobasilar circulation and hearing in patients with SSNHL treated with pentoxifylline. DESIGN: Case-control study of 32 consecutive patients with SSNHL. METHODS: Patients with onset of SSNHL within 72 hours were treated with pentoxifylline infusions for 10 days. Transcranial Doppler (TCD) ultrasonography of vertebrobasilar circulation and hearing levels were examined at the onset of SSNHL (before treatment), after 10 days of therapy, and after 3 months. MAIN OUTCOME MEASURES: Hearing levels and TCD ultrasonographic findings at the onset of SSNHL, after 10 days, and after 3 months. RESULTS: Patients with SSNHL had more frequent pathologic TCD ultrasonographic findings compared with a normal population. Thirteen patients with SSNHL had normal TCD ultrasonographic findings, eight had borderline findings, and six had pathologic findings, and in five patients, initially, pathologic TCD ultrasonographic findings improved during therapy. Patients with normal TCD ultrasonographic findings had an average hearing improvement of 17 dB, whereas patients with borderline and pathologic TCD ultrasonographic findings had 3 and 0 dB of average hearing improvement, respectively. The greatest improvement (28 dB) was noticed in patients who showed improvement in TCD ultrasonographic findings. CONCLUSION: Although the number of patients is small, our results led to the conclusion that SSNHL has vascular etiology, at least in some patients. Consequently, the therapy for SSNHL should depend on etiology; that is, vasoactive medications should be reserved for patients with supposed vascular etiology. TCD ultrasonography could have a role in the decision regarding the optimal treatment of SSNHL.

[Radionuclide tests of cerebral circulation in the assessment of treatment of sensorineural hearing loss]. / Radionuklidnye metody issledovaniia krovoobrashcheniia golovnogo mozga v otsenke rezul'tatov lecheniia neirosensornoi tugoukhosti.

Examination and treatment results are available for 59 patients with acute and chronic neurosensory hypoacusis. Cerebral circulation was investigated at radionuclide carotid arteriography and radionuclide single-photon emission computed tomography. Arterial flow to the brain, microcirculation of the gray matter and venous outflow from the cranial cavity were studied before and after the course of quantum hemotherapy.